Common γ-chain blocking peptide reduces in vitro immune activation markers in HTLV-1-associated myelopathy/tropical spastic paraparesis.

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Massoud, Raya; Enose-Akahata, Yoshimi; Tagaya, Yutaka; Azimi, Nazli; Basheer, Asjad; Jacobson, Steven

2015-09-01

Human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a progressive inflammatory myelopathy occurring in a subset of HTLV-1-infected individuals. Despite advances in understanding its immunopathogenesis, an effective treatment remains to be found. IL-2 and IL-15, members of the gamma chain (γc) family of cytokines, are prominently deregulated in HAM/TSP and underlie many of the characteristic immune abnormalities, such as spontaneous lymphocyte proliferation (SP), increased STAT5 phosphorylation in the lymphocytes, and increased frequency and cytotoxicity of virus-specific cytotoxic CD8(+) T lymphocytes (CTLs). In this study, we describe a novel immunomodulatory strategy consisting of selective blockade of certain γc family cytokines, including IL-2 and IL-15, with a γc antagonistic peptide. In vitro, a PEGylated form of the peptide, named BNZ132-1-40, reduced multiple immune activation markers such as SP, STAT5 phosphorylation, spontaneous degranulation of CD8(+) T cells, and the frequency of transactivator protein (Tax)-specific CD8(+) CTLs, thought to be major players in the immunopathogenesis of the disease. This strategy is thus a promising therapeutic approach to HAM/TSP with the potential of being more effective than single monoclonal antibodies targeting either IL-2 or IL-15 receptors and safer than inhibitors of downstream signaling molecules such as JAK1 inhibitors. Finally, selective cytokine blockade with antagonistic peptides might be applicable to multiple other conditions in which cytokines are pathogenic.

Hepatic Myelopathy in a Patient with Decompensated Alcoholic Cirrhosis and Portal Colopathy

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Madhumita Premkumar

2012-01-01

Full Text Available Cirrhotic or hepatic myelopathy is a rare neurological complication of chronic liver disease usually seen in adults and presents as a progressive pure motor spastic paraparesis which is usually associated with overt liver failure and a surgical or spontaneous systemic portocaval shunt. We describe the development of progressive spastic paraparesis, in a patient with alcoholic cirrhosis with portal hypertension and portal colopathy who presented with the first episode of hepatic encephalopathy. The patient had not undergone any shunt procedure.

Mise au point myelopathie cervicarthrosique revelee par un ...

African Journals Online (AJOL)

Degenerative cervical myelopathy is a progressive spinal cord disease caused by mecanical compression from different structure of a spinal stenosis in pathologics conditions which brings histological damade. In addition acut myelopathy is frequently revealed by cervical spine injury with dynamic injury mechanism.

Remission of HIV-associated myelopathy after highly active antiretroviral therapy

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Fernandez-Fernandez F

2004-07-01

Full Text Available HIV-associated myelopathy is the leading cause of spinal cord disease in HIV-infected patients. Typically, it affects individuals with low CD4 T cell counts, presenting with slowly progressive spastic paraparesis associated with dorsal column sensory loss as well as urinary disturbances. Other aetiologies must be first ruled out before establishing the diagnosis. We report here the case of a 37-year-old woman with advanced HIV disease, who developed HIV-associated myelopathy. The patient showed a gradual improvement after beginning with highly active antiretroviral therapy and, finally, she achieved a complete functional recovery. In addition, neuroimaging and neurophysiological tests normalized.

Tropical spastic paraparesis and HTLV-1 associated myelopathy: clinical, epidemiological, virological and therapeutic aspects.

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Gessain, A; Mahieux, R

2012-03-01

In 1980, Human T cell leukemia/lymphoma virus type 1 (HTLV-1) was the first oncogenic human retrovirus to be discovered. HTLV-1 belongs to the Retroviridae family, the Orthoretrovirinae subfamily and to the deltaretrovirus genus. HTLV-1 preferentially infects CD4(+) lymphoid cells in vivo. Three molecules have been identified for binding and/or entry of HTLV-1: heparan sulfate proteoglycans, neuropilin-1, and glucose transporter 1. An efficient transfer of the virus from an infected cell to a target cell can occur through the formation of a viral synapse and/or by virofilm structure. As for all retroviruses, HTLV-1 genome possesses three major ORFs (gag, pol and env) encoding the structural and enzymatic proteins. HTLV-1 encodes also some regulatory and auxillary proteins including the tax protein with transforming activities and the HBZ protein which plays a role in the proliferation and maintenance of the leukemic cells. HTLV-1 is present throughout the world with clusters of high endemicity including mainly Southern Japan, the Caribbean region, areas in South America and in intertropical Africa. The worldwide HTLV-1 infected population is estimated to be around 10-20 million. HTLV-1 has three modes of transmission: (1): mother to child, mainly linked to prolonged breast-feeding; (2): sexual, mainly occurring from male to female and (3): contaminated blood products. HTLV-1 possesses a remarkable genetic stability. HTLV-1 is the etiological agent of mainly two severe diseases: a malignant T CD4(+) cell lymphoproliferation, of very poor prognosis, named Adult T cell Leukemia/Lymphoma (ATLL), and a chronic neuro-myelopathy named Tropical spastic paraparesis/HTLV-1 Associated Myelopathy (TSP/HAM). The lifetime risk among HTLV-1 carriers is estimated to be around 0.25 to 3%. TSP/HAM mainly occurs in adults, with a mean age at onset of 40-50 years and it is more common in women than in men. Blood transfusion is a major risk factor for TSP/HAM development. Clinically

Large armored bridging over fractured vertebra with intraspinal tumor mimicking bony mass caused by migrated fragments of burst cervical vertebra presenting with severe cervical myelopathy

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Satyarthee Guru Dutta

2017-06-01

Full Text Available Vertebral body may get displaced anterior or posteror with elements of rotation. However, burst cervical spine vertebral fracture may migrate anteriorly and posteriorly simultaneously. However anterior displaced fragment forming armor like mass is very rare. Similarly, the posteriorly propelled fragments migrating caudally and posterolaterally producing a large osseous mass inside spinal canal mimicking bony tumour causing severe cervical canal stenosis and presenting with marked myelopathy is extremely rare. To the best knowledge of authors, association of such traumatic dual pathology represents first of its kind in western literature, who was neglected early medical advice and presenting with marked compressive cervical myelopathy. She underwent successful surgical decompression with gradual recovery of spastic limb weakness and recovery of sensation. Authors also highlights the importance of early resuscitation and adequate maintainace of mean arterial pressure following acute spinal cord injury. Pertinent literature is briefly reviewed.

Cervical myelopathy: magnetic imaging findings

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Kholin, A.V.; Makarov, A.Yu.; Gurevich, D.V.

1996-01-01

69 patients with clinical signs of cervical myelopathy were examined using magnetic imaging (T1- and T2-suspended tomograms of the sagittal and transverse section using a device with 0.04 T field intensity). Vertebral disk hernias were revealed in 35 patients, compression of the spinal cord with metastases into vertebral body in 2, extramedullary tumor in 11, intramedullary tumor in 9, and syringomyelia in 12 patients. T2-suspended tomograms proved to be more informative due to their higher sensitivity to aqueous content. T1-suspended tomograms help assess the degree of spinal cord compression and the direction of the disk protrusion. Magnetic imaging is an informative method used for objective identification of the cases of myelopathy of cervical localization [ru

Prediction of surgical outcome in compressive cervical myelopathy: A novel clinicoradiological prognostic score

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Rishi Anil Aggarwal

2016-01-01

Full Text Available Context: Preoperative severity of myelopathy, age, and duration of symptoms have been shown to be highly predictive of the outcome in compressive cervical myelopathy (CCM. The role of radiological parameters is still controversial. Aims: Define the prognostic factors in CCM and formulate a prognostic score to predict the outcome following surgery in CCM. Settings and Design: Retrospective. Materials and Methods: This study included 78 consecutive patients with CCM treated surgically. The modified Japanese Orthopaedic Association (mJOA scale was used to quantify severity of myelopathy at admission and at 12-month follow-up. The outcome was defined as "good" if the patient had mJOA score ≥16 and "poor" if the score was <16. Age, sex, duration of symptoms, comorbidities, intrinsic hand muscle wasting (IHMW, diagnosis, surgical technique, Torg ratio, instability on dynamic radiographs, and magnetic resonance imaging (MRI signal intensity changes were assessed. Statistics: Statistical Package for the Social Sciences (SPSS (version 20.0 was used for statistical analysis. The association was assessed amongst variables using logistic regression analysis. Parameters having a statistically significant correlation with the outcome were included in formulating a prognostic score. Results: Severity of myelopathy, IHMW, age, duration, diabetes, and instability on radiographs were predictive of the outcome with a P value <0.01. Genders, diagnosis, surgical procedure, Torg ratio, and intensity changes on MRI were not significantly related to the outcome. A 8-point scoring system was devised incorporating the significant clinicoradiological parameters, and it was found that nearly all patients (97.82% with a score below 5 had good outcome and all patients (100% with a score above 5 had poor outcome. The outcome is difficult to predict with a score of 5. Conclusions: Clinical parameters are better predictors of the outcome as compared to radiological findings

Cervical Myelopathy in Rheumatoid Arthritis

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N. Mukerji

2011-01-01

Full Text Available Involvement of the cervical spine is common in rheumatoid arthritis. Clinical presentation can be variable, and symptoms may be due to neck pain or compressive myeloradiculopathy. We discuss the pathology, grading systems, clinical presentation, indications for surgery and surgical management of cervical myelopathy related to rheumatoid arthritis in this paper. We describe our surgical technique and results. We recommend early consultation for surgical management when involvement of the cervical spine is suspected in rheumatoid arthritis. Even patients with advanced cervical myelopathy should be discussed for surgical treatment, since in our experience improvement in function after surgery is common.

MR imaging of spinal factors and compression of the spinal cord in cervical myelopathy

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Kokubun, Shoichi; Ozawa, Hiroshi; Sakurai, Minoru; Ishii, Sukenobu; Tani, Shotaro; Sato, Tetsuaki.

1992-01-01

Magnetic resonance (MR) images of surgical 109 patients with cervical spondylotic myelopathy were retrospectively reviewed to examine whether MR imaging would replace conventional radiological procedures in determining spinal factors and spinal cord compression in this disease. MR imaging was useful in determining spondylotic herniation, continuous type of ossification of posterior longitudinal ligament, and calcification of yellow ligament, probably replacing CT myelography, discography, and CT discography. When total defect of the subarachnoid space on T2-weighted images and block on myelograms were compared in determining spinal cord compression, the spinal cord was affected more extensively by 1.3 intervertebral distance (IVD) on T2-weighted images. When indentation of one third or more in anterior and posterior diameter of the spinal cord was used as spinal cord compression, the difference in the affected extension between myelography and MR imaging was 0.2 IVD on T1-weighted images and 0.6 IVD on T2-weighted images. However, when block was seen in 3 or more IVD on myelograms, the range of spinal cord compression tended to be larger on T1-weighted images. For a small range of spinal cord compression, T1-weighted imaging seems to be helpful in determining the range of decompression. When using T2-weighted imaging, the range of decompression becomes large, frequently including posterior decompression. (N.K.)

Synovial chondromatosis of the lumbar spine with compressive myelopathy: a case report with review of the literature

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Abdelwahab, Ibrahim Fikry; Contractor, Daniel; Bianchi, Stefano; Hermann, George; Hoch, Benjamin

2008-01-01

Synovial chondromatosis has been rarely reported to occur in the spine with only one case found in the lumbar spine. We describe another case of synovial chondromatosis in the lumbar spine in a 41-year-old man who presented with compressive myelopathy. The tumor was located in the left ventrolateral corner of the epidural space just below the L 4 -L 5 intervertebral space. Besides being extremely rare, our case was unusual in that the juxtaposed facet joint was radiologically normal. (orig.)

Solitary Osteochondroma of the Thoracic Spine with Compressive Myelopathy; A Rare Presentation

International Nuclear Information System (INIS)

Mehrian, Payam; Karimi, Mohammad Ali; Kahkuee, Shahram; Bakhshayeshkaram, Mehrdad; Ghasemikhah, Reza

2013-01-01

A 19-year-old man presented with a 5-year history of back pain radiating to the lower extremities and paresthesis of the toes during the last year. Plain X-ray revealed a large cauliflower shaped exophytic mass at the level of T8, T9 and T10 vertebrae. Computed tomography (CT) and magnetic resonance imaging (MRI) showed an abnormal bony mass arising from the posterior arch of T9 with protrusion to the spinal canal and marked cord compression. The cortex and medulla of the lesion had continuity with those of the T9 vertebra. Surgical en bloc resection was performed and the patient’s symptoms resolved. The histopathologic diagnosis was osteochondroma. In patients with symptoms of myelopathy, in addition to more common etiologies, one should also be aware of rare entities such as osteochondroma

[Autoimmune syndrome in the tropical spastic paraparesis/myelopathy associated with human T-lymphotropic virus infections].

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Domínguez, Martha C; Torres, Miyerlandi; Tamayo, Oscar; Criollo, William; Quintana, Milton; Sánchez, Adalberto; García, Felipe

2008-12-01

Previous reports have given evidence that in tropical spastic paraparesis (TSP)/human T-lymphotrophic virus (HTLV-I)-associated myelopathy (HAM), an autoimmune process occurs as part of its pathogenesis. The roles of autoimmunity and the molecular mimicry was evaluated in TSP/HAM patients. Plasma samples were characterized from patients in the Pacific coastal region of Colombia. Thirty-seven were identified as TSP/HAM, 10 were diagnosed with adult T-cell leukemia virus, 22 were asymptomatic carriers but seropositive for HTLV-I and 20 were seronegative and served as negative controls. Plasmatic levels of the following were determined: antinuclear antibody (ANA) levels, anticardiolipine-2 (ACL-2), interferon- (IFN-gamma) and interleukin-4 (IL-4). Using Western blot, the crossreactivity of the seropositive and seronegative samples was evaluated against proteins extracted from several central nervous system components of non infected Wistar rats. The HTLV-I seropositive plasmas were crossreacted with a monoclonal tax (LT4 anti-taxp40) from spinal cord neurons of non infected Wistar rats. Of the TSP/HAM patients, 70.2% were reactive against ANA and 83.8% against ACL-2, in contrast with those ATL and asymptomatic seropositives subjects that were not reactive (P<0.001). Moreover, 70.3% had detectable levels of IFN and 43.2% had detectable IL-4. LT4 anti-taxp40 and plasma of TSP/HAM exhibited cross reactivity with a MW 33-35 kDa protein from the rat spinal cord nuclei. Support was provided for the existence of an autoimmune syndrome mediated by molecular mimicry; the syndrome was responsible for some of the axonal degeneration observed in TSP/HAM patients.

High signal in the spinal cord on T2-weighted images in rapidly progressive tropical spastic paraparesis

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Watanabe, M.; Hara, A.; Murakami, T.; Ando, Y.; Uyama, E.; Mita, S.; Uchino, M. [Kumamoto Univ. (Japan). School of Medicine; Yamashita, T. [Kumamoto Univ. (Japan). School of Medicine; Dept. of Neurology, Kumamoto Univ. (Japan)

2001-03-01

We report a 59-year-old woman with human T-cell lymphotrophic virus type-I (HTLV-I) associated myelopathy/tropical spastic paraparesis who showed high signal in the cervical and thoracic spinal cord on T2-weighted and contrast enhancement on T1-weighted images. (orig.)

Atlantoaxial subluxation. Radiography and magnetic resonance imaging correlated to myelopathy

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Yamashita, Y.; Takahashi, M.; Sakamoto, Y.; Kojima, R.

Twenty-nine patients with atlantoaxial subluxation (18 with rheumatoid arthritis, 2 due to trauma, 4 with os odontoideum, and one each with polyarteritis nodosa, rheumatic fever, Klippel-Feil syndrome, achondroplasia, and cause unknown) were evaluated using a 0.22 tesla resistive MRI unit. Cord compression was classified into four grades according to the degree on magnetic resonance imaging. There were 7 patients with no thecal sac compression (grade 0), 10 with a minimal degree of subarachnoid space compression without cord compression (grade 1), 7 with mild cord compression (grade 2), and 5 with severe cord compression or cord atrophy (grade 3). Although the severity of myelopathy showed poor correlation with the atlantodental interval on conventional radiography, high correlation was observed between MR grading and the degree of myelopathy. The high signal intensity foci were observed in 7 or 12 patients with cord compression (grades 2 and 3) on T2 weighted images. Other frequently observed findings in rheumatoid arthritis included soft tissue masses of low to intermediate signal intensity in the paraodontoid space, erosions of the odontoid processes, and atlanto-axial impaction on T1 and T2 weighted images.

Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses.

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Zalewski, Nicholas L; Flanagan, Eoin P; Keegan, B Mark

2018-01-09

To evaluate specific myelopathy diagnoses made in patients with suspected idiopathic transverse myelitis (ITM). A total of 226 patients 18 years and older were referred to Mayo Clinic Neurology for suspected ITM from December 1, 2010, to December 31, 2015. Electronic medical records were reviewed for detailed clinical presentation and course, laboratory and electrophysiologic investigations, and neuroimaging to determine the etiology. Current diagnostic criteria for ITM and alternative myelopathy diagnoses were applied. All cases where any discrepancy was suspected from the final reported clinical diagnosis were reviewed by each author and a consensus final diagnosis was made. The diagnostic criteria for ITM were met in 41 of 226 patients (18.1%). In 158 patients (69.9%), an alternative specific myelopathy diagnosis was made: multiple sclerosis or clinically isolated syndrome, 75; vascular myelopathy, 41; neurosarcoidosis, 12; neuromyelitis optica spectrum disorder, 12; myelin oligodendrocyte glycoprotein myelopathy, 5; neoplastic, 4; compressive, 3; nutritional, 3; infectious, 2; and other, 2. A myelopathy was not confirmed in 27 patients. Time from symptom onset to final clinical diagnosis in patients without ITM was a median of 9 months (range 0-288). Fifty-five patients (24%) required treatment changes according to their final clinical diagnosis. The majority of patients with suspected ITM have an alternative specific myelopathy diagnosis. A presumptive diagnosis of ITM can lead to premature diagnostic conclusions affecting patient treatment. Copyright © 2017 American Academy of Neurology.

Severity score system for progressive myelopathy: development and validation of a new clinical scale

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R.M. Castilhos

2012-07-01

Full Text Available Progressive myelopathies can be secondary to inborn errors of metabolism (IEM such as mucopolysaccharidosis, mucolipidosis, and adrenomyeloneuropathy. The available scale, Japanese Orthopaedic Association (JOA score, was validated only for degenerative vertebral diseases. Our objective is to propose and validate a new scale addressing progressive myelopathies and to present validating data for JOA in these diseases. A new scale, Severity Score System for Progressive Myelopathy (SSPROM, covering motor disability, sphincter dysfunction, spasticity, and sensory losses. Inter- and intra-rater reliabilities were measured. External validation was tested by applying JOA, the Expanded Disability Status Scale (EDSS, the Barthel index, and the Osame Motor Disability Score. Thirty-eight patients, 17 with adrenomyeloneuropathy, 3 with mucopolysaccharidosis I, 3 with mucopolysaccharidosis IV, 2 with mucopolysaccharidosis VI, 2 with mucolipidosis, and 11 with human T-cell lymphotropic virus type-1 (HTLV-1-associated myelopathy participated in the study. The mean ± SD SSPROM and JOA scores were 74.6 ± 11.4 and 12.4 ± 2.3, respectively. Construct validity for SSPROM (JOA: r = 0.84, P < 0.0001; EDSS: r = -0.83, P < 0.0001; Barthel: r = 0.56, P < 0.002; Osame: r = -0.94, P < 0.0001 and reliability (intra-rater: r = 0.83, P < 0.0001; inter-rater: r = 0.94, P < 0.0001 were demonstrated. The metric properties of JOA were similar to those found in SSPROM. Several clinimetric requirements were met for both SSPROM and JOA scales. Since SSPROM has a wider range, it should be useful for follow-up studies on IEM myelopathies.

Heterotopic ossification associated with myelopathy following cervical disc prosthesis implantation.

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Wenger, Markus; Markwalder, Thomas-Marc

2016-04-01

This case report presents a 37-year-old man with clinical signs of myelopathy almost 9 years after implantation of a Bryan disc prosthesis (Medtronic Sofamor Danek, Memphis, TN, USA) for C5/C6 soft disc herniation. As demonstrated on MRI and CT scan, spinal cord compression was caused by bony spurs due to heterotopic ossification posterior to the still moving prosthesis. The device, as well as the ectopic bone deposits, had to be removed because of myelopathy and its imminent aggravation. Conversion to anterior spondylodesis was performed. Copyright © 2015 Elsevier Ltd. All rights reserved.

Treatment of cervical myelopathy in patients with the fibromyalgia syndrome: outcomes and implications

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Ross, Ruth E.; Shade-Zeldow, Yvonne; Kostas, Konstantinos; Morrissey, Mary; Elias, Dean A.; Shepard, Alan

2007-01-01

Some patients with fibromyalgia also exhibit the neurological signs of cervical myelopathy. We sought to determine if treatment of cervical myelopathy in patients with fibromyalgia improves the symptoms of fibromyalgia and the patients’ quality of life. A non-randomized, prospective, case control study comparing the outcome of surgical (n = 40) versus non-surgical (n = 31) treatment of cervical myelopathy in patients with fibromyalgia was conducted. Outcomes were compared using SF-36, screening test for somatization, HADS, MMPI-2 scale 1 (Hypochondriasis), and self reported severity of symptoms 1 year after treatment. There was no significant difference in initial clinical presentation or demographic characteristics between the patients treated by surgical decompression and those treated by non-surgical means. There was a striking and statistically significant improvement in all symptoms attributed to the fibromyalgia syndrome in the surgical patients but not in the non-surgical patients at 1 year following the treatment of cervical myelopathy (P ≤ 0.018–0.001, Chi-square or Fisher’s exact test). At the 1 year follow-up, there was a statistically significant improvement in both physical and mental quality of life as measured by the SF-36 score for the surgical group as compared to the non-surgical group (Repeated Measures ANOVA P somatization disorder, and the anxiety and depression scores exclusively in the surgical patients (Wilcoxon signed rank, P < 0.001). The surgical treatment of cervical myelopathy due to spinal cord or caudal brainstem compression in patients carrying the diagnosis of fibromyalgia can result in a significant improvement in a wide array of symptoms usually attributed to fibromyalgia with attendant measurable improvements in the quality of life. We recommend detailed neurological and neuroradiological evaluation of patients with fibromyalgia in order to exclude compressive cervical myelopathy, a potentially treatable

Finger-tapping motion analysis in cervical myelopathy by magnetic-sensor tapping device.

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Miwa, Toshitada; Hosono, Noboru; Mukai, Yoshihiro; Makino, Takahiro; Kandori, Akihiko; Fuji, Takeshi

2013-08-01

Case-control study. The purpose of this study is to determine finger motion of patients with cervical myelopathy during finger-tapping cycles. A major symptom of patients with compressive cervical myelopathy is finger clumsiness. Therefore, understanding finger motion is prerequisite in assessing the severity of myelopathy. The popular grip-and-release test evaluates only the number of motion cycles, which is insufficient to fully describe complex finger motion. Forty-three patients with cervical myelopathy and 41 healthy controls tapped their index fingers against their thumbs as rapidly as possible for 30 seconds and the motion was recorded by a magnetic-sensor coil attached to the nail surface. Output signals were stored in a computer, which automatically calculated tapping frequency, distance moved, ratio of opening/closing velocity and the SD of the tapping interval. The SD of the tapping interval was significantly greater and all other measures were significantly smaller in patients with cervical myelopathy, than in healthy controls. All indices significantly improved after surgical decompression of the cervical spine. Distance moved (Pearson correlation coefficient: r=0.590, Ptapping interval (r=-0.451; P=0.002) were significantly correlated with the Japanese Orthopedic Association score (neurological scale). The quantitative evaluation of finger paralysis was performed by this tapping device. Speed and regularity in repetitive motion of fingers were correlated with the severity of cervical myelopathy.

Cytoplasmic Localization of HTLV-1 HBZ Protein: A Biomarker of HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP).

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Baratella, Marco; Forlani, Greta; Raval, Goutham U; Tedeschi, Alessandra; Gout, Olivier; Gessain, Antoine; Tosi, Giovanna; Accolla, Roberto S

2017-01-01

HTLV-1 is the causative agent of a severe form of adult T cell leukemia/Lymphoma (ATL), and of a chronic progressive neuromyelopathy designated HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). Two important HTLV-1-encoded proteins, Tax-1 and HBZ, play crucial roles in the generation and maintenance of the oncogenic process. Less information is instead available on the molecular and cellular mechanisms leading to HAM/TSP. More importantly, no single specific biomarker has been described that unambiguously define the status of HAM/TSP. Here we report for the first time the finding that HBZ, described until now as an exclusive nuclear protein both in chronically infected and in ATL cells, is instead exclusively localized in the cytoplasm of peripheral blood mononuclear cells (PBMC) from patients suffering of HAM/TSP. Interestingly, at the single cell level, HBZ and Tax-1 proteins are never found co-expressed in the same cell, suggesting the existence of mechanisms of expression uncoupling of these two important HTLV-1 viral products in HAM/TSP patients. Cells expressing cytoplasmic HBZ were almost exclusively found in the CD4+ T cell compartment that was not, at least in a representative HAM/TSP patient, expressing the CD25 marker. Less than 1 percent CD8+ T cells were fond positive for HBZ, while B cells and NK cells were found negative for HBZ in HAM/TSP patients. Our results identify the cytoplasmic localization of HBZ in HAM/TSP patient as a possible biomarker of this rather neglected tropical disease, and raise important hypotheses on the role of HBZ in the pathogenesis of the neuromyelopathy associated to HTLV-1 infection.

Cytoplasmic Localization of HTLV-1 HBZ Protein: A Biomarker of HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP.

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Marco Baratella

2017-01-01

Full Text Available HTLV-1 is the causative agent of a severe form of adult T cell leukemia/Lymphoma (ATL, and of a chronic progressive neuromyelopathy designated HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP. Two important HTLV-1-encoded proteins, Tax-1 and HBZ, play crucial roles in the generation and maintenance of the oncogenic process. Less information is instead available on the molecular and cellular mechanisms leading to HAM/TSP. More importantly, no single specific biomarker has been described that unambiguously define the status of HAM/TSP. Here we report for the first time the finding that HBZ, described until now as an exclusive nuclear protein both in chronically infected and in ATL cells, is instead exclusively localized in the cytoplasm of peripheral blood mononuclear cells (PBMC from patients suffering of HAM/TSP. Interestingly, at the single cell level, HBZ and Tax-1 proteins are never found co-expressed in the same cell, suggesting the existence of mechanisms of expression uncoupling of these two important HTLV-1 viral products in HAM/TSP patients. Cells expressing cytoplasmic HBZ were almost exclusively found in the CD4+ T cell compartment that was not, at least in a representative HAM/TSP patient, expressing the CD25 marker. Less than 1 percent CD8+ T cells were fond positive for HBZ, while B cells and NK cells were found negative for HBZ in HAM/TSP patients. Our results identify the cytoplasmic localization of HBZ in HAM/TSP patient as a possible biomarker of this rather neglected tropical disease, and raise important hypotheses on the role of HBZ in the pathogenesis of the neuromyelopathy associated to HTLV-1 infection.

Thoracic myelopathy with alkaptonuria.

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Akeda, Koji; Kasai, Yuichi; Kawakita, Eiji; Matsumura, Yoshihiro; Kono, Toshibumi; Murata, Tetsuya; Uchida, Atsumasa

2008-01-15

A case of thoracic myelopathy with alkaptonuria (ochronotic spondyloarthropathy) is presented. To present and review the first reported case of an alkaptonuric patient with concomitant thoracic myelopathy. Alkaptonuria, a rare hereditary metabolic disease, is characterized by accumulation of homogentistic acid, ochronosis, and destruction of connective tissue resulting in degenerative spondylosis and arthritis. Despite the high incidence of intervertebral disc diseases among patients with alkaptonuria, neurologic symptoms caused by spinal disease are rare. Thoracic myelopathy in a patient with alkaptonuria has not been previously reported. The clinical course, radiologic features, pathology, and treatment outcome of an alkaptonuria patient with thoracic myelopathy was documented. Myelopathy of the patient was caused by rupture of a thoracic intervertebral disc. The neurologic symptoms of the patient were markedly improved after surgery. We have reported for the first time, that an alkaptonuria patient showed thoracic myelopathy caused by rupture of a thoracic intervertebral disc. Decompression followed by the instrumented fusion of the thoracic spine was effective for improving the neurologic symptoms.

Predicting surgical outcome in cases of cervical myelopathy with magnetic resonance imaging. Critical parameters

International Nuclear Information System (INIS)

Akiyama, Takashi

1997-01-01

In this study, the author attempted to correlate clinical factors significant in cases of cervical myelopathy with postoperative recovery. It is hoped that the results will aid in the preoperative prediction of surgical outcomes. The factors considered were the transverse area of the spinal cord, the cord compression rate, the presence of a high intensity area in T2-weighted MRI, the duration of symptoms before surgery, and age at surgery. Because there are variations in the transverse area of the spinal cord, 100 normal individuals were selected and the standard transverse area was calculated. The transverse area of the spinal cord and the cord constriction rate in the myelopathy cases was then measured and compared to the standard. The data indicated that the constriction rate was most relevant to recovery rate. Clinical thresholds found to correlate with a better than average rate of recovery in cases of cervical spondylotic myelopathy (CSM) were: a cord constriction rate; under 28.7%, cord compression rate; over 0.38, duration of symptoms before surgery; less than 9.2 months, and age at surgery; under 59.2 yrs. In patients with ossification of the longitudinal ligament (OPLL), cord constriction rate; under 36.2%, cord compression rate; over 0.30, duration of symptoms before surgery; less than 14.2 months, and age at surgery; under 57.6 yrs., all correlated with superior recovery, as did cord constriction rate; under 22.3%, and duration of symptoms before surgery; less than 3.7 months with patients suffering from cervical disc herniation (CDH). Furthermore, the absence of a T2-weighted high intensity area in CSM and OPLL patients also correlated with improved recovery. These results suggest that a favorable postoperative recovery rate can be expected in cases of cervical myelopathy that conform to the above criteria. (author)

Radiation myelopathy

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Howell, D A [Derbyshire Royal Infirmary, Derby (UK)

1979-10-01

Following high-dose radiotherapy treatment of tumours, there is a risk of destructive radiation myelopathy developing a few months later as a result of spinal cord irradiation. The emphasis of the present article is on the mechanism of the development of radiation myelopathy. It is suggested that, in the irradiated segments, the normal endothelial cells lining the penetrating arteries and capillaries are replaced by abnormal cells during the latent period. Radiation-induced mutations or chromosomal aberrations are contained in these cells, thus provoking an immunological response. During the attempted rejection of these cells, protein-rich plasma filtrate is leaked into the artery walls and nervous tissue, causing the destructive myelopathy. The signs of paralysis of spinal cord function may be caused either by infarcts or by oedema of the white matter. Since both diagnosis and treatment are difficult, it is necessary to concentrate on prevention by, whenever possible, reducing radiation doses to below tolerance limits during radiotherapy. As regards radiotherapy in children, it is currently believed that there is little or no difference in radiation tolerance between the child and the adult nervous system. Some early benign forms of radiation myelopathy are also briefly discussed.

Radiation myelopathy

International Nuclear Information System (INIS)

Howell, D.A.

1979-01-01

Following high-dose radiotherapy treatment of tumours, there is a risk of destructive radiation myelopathy developing a few months later as a result of spinal cord irradiation. The emphasis of the present article is on the mechanism of the development of radiation myelopathy. It is suggested that, in the irradiated segments, the normal endothelial cells lining the penetrating arteries and capillaries are replaced by abnormal cells during the latent period. Radiation-induced mutations or chromosomal aberrations are contained in these cells, thus provoking an immunological response. During the attempted rejection of these cells, protein-rich plasma filtrate is leaked into the artery walls and nervous tissue, causing the destructive myelopathy. The signs of paralysis of spinal cord function may be caused either by infarcts or by oedema of the white matter. Since both diagnosis and treatment are difficult, it is necessary to concentrate on prevention by, whenever possible, reducing radiation doses to below tolerance limits during radiotherapy. As regards radiotherapy in children, it is currently believed that there is little or no difference in radiation tolerance between the child and the adult nervous system. Some early benign forms of radiation myelopathy are also briefly discussed. (UK)

3 T magnetic resonance diffusion tensor imaging and fibre tracking in cervical myelopathy

International Nuclear Information System (INIS)

Xiangshui, M.; Xiangjun, C.; Xiaoming, Z.; Qingshi, Z.; Yi, C.; Chuanqiang, Q.; Xiangxing, M.; Chuanfu, L.; Jinwen, H.

2010-01-01

Aim: To analyse the characterization of diffusion tensor imaging (DTI) with 3 T magnetic resonance imaging (MRI) in cervical myelopathy. Methods: A total of 21 healthy controls and 84 patients with cervical myelopathy underwent T2-weighted imaging and DTI. The patients were divided into four groups based on the degree of cord compression and MRI signal intensity of the compressed cord as seen on T2-weighted images. The values of apparent diffusion coefficient (ADC), fractional anisotropy (FA), and eigenvalues (λ i ) were analysed, and fibre tracking (FT) was performed. Results: For healthy controls, the mean values from the DTI of the cervical spinal cord were ADC = 0.784 ± 0.083 x 10 -3 mm 2 /s, FA = 0.721 ± 0.027, λ 1 , λ 2 , and λ 3 = 1.509 ± 0.145 x 10 -3 , 0.416 ± 0.094 x 10 -3 , and 0.411 ± 0.102 x 10 -3 mm 2 /s, respectively. Only values for λ 2 and λ 3 differed significantly between the control and A groups (p 2 and λ 3 of group A were 0.516 ± 0.105 x 10 -3 and 0.525 ± 0.129 x 10 -3 mm 2 /s, respectively. ADC, FA, λ 1 , λ 2 and λ 3 differed significantly between the control and B, C, D groups (p i obtained with DTI could assess subtle structural damage and changes of anisotropy in the cord of cervical myelopathy. Fibre tracking was useful in verifying changes in the compressed cord.

Radiation myelopathy

International Nuclear Information System (INIS)

Berlit, P.

1987-01-01

After a review of the world literature, the case histories of 43 patients with radiation myelopathy are analyzed. In 1 patient there was a radiation injury of the medulla oblongata, in 2, cervical, in 28, thoracic, and in 12, lumbosacral. In the medulla oblongata lesion an alternans syndrome resulted. The patients with cervical and thoracic radiation myelopathies presented with a Brown-Sequard syndrome, a spinalis anterior syndrome or a transversal syndrome with pyramidal and spinothalamic tract involvement as the most prominent signs. For this group the term 'pyramidal-spinothalamic radiation myelopathy' is proposed. In lumbosacral radiation lesions a pure anterior horn syndrome may lead to spinothalamic tract involvement and the development of a cauda conus syndrome. The clinical presentation of these cases suggests that the location of the radiation lesion is most likely the region of the conus medullaris. The most frequent initial symptom was dysesthesia; the patients complained of burning pain or a feeling of coldness. Usually the neurological deficits were progressive, in pyramidal-spinothalamic radiation myelopathy over 12 months in average, in lumbosacral radiation lesions up to 10 years. The latent period between the finish of radiation therapy and the first neurological signs was 8 months (median) in cervical and thoracic myelopathy and 33 months in lumbosacral lesions. For the entire group of 43 patients there was an inverse relationship between the radiation dose (ret) and the latent period. A positive relation could be demonstrated between the age of patients at the time of radiation therapy and the latent period. Patients simultaneously receiving cytostatic drugs presented after a longer latent period than the remaining group. (orig./MG)

Relationship between magnetic resonance imaging and clinical results of decompression surgery for cervical myelopathy

International Nuclear Information System (INIS)

Okumura, Hiroshi

1993-01-01

MR imaging was investigated before and after surgery in 60 cases of cervical myelopathy. A preoperative high-signal-intensity area in the spinal cord was thought to be an important indicator of poor prognosis, because the recovery ratio of the preoperative high-signal group was 32±24%, while that of the normal-signal group was 86±15%. But, a high-signal-intensity area had no significant correlation with the morbidity period, preoperative clinical severity and degree of cord compression. There was a significant correlation between postoperative MR imaging and the neurological prognosis. And, atrophy and high-signal-intensity area in the spinal cord were frequently seen in cases of poor neurological postoperative recovery, corresponding to the morbidity period, preoperative clinical severity and degree of cord compression. MR imaging can serve as a useful tool to assess cervical myelopathy and to forecast the postoperative prognosis. (author)

Role of IL-21 in HTLV-1 infections with emphasis on HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

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Rajaei, Taraneh; Farajifard, Hamid; Rafatpanah, Houshang; Bustani, Reza; Valizadeh, Narges; Rajaei, Bahareh; Rezaee, Seyed Abdolrahim

2017-06-01

Interleukin-21 (IL-21) enhances the survival and cytotoxic properties of cytotoxic T cells (CTLs) and exhibits essential roles in controlling chronic viral infections. HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic progressive inflammatory disease of the nervous system. The main determinant of disease progression is efficiency of the CTL response to Human T lymphotropic virus types I (HTLV-1). In this study, the expression of host IL-21 and HTLV-I Tax and proviral load (PVL) was evaluated to understand the role and mechanism of IL-21 in HTLV-1 infections and the subsequent development of HAM/TSP. A cross-sectional study was carried out on 20 HAM/TSP patients, 20 asymptomatic HTLV-1 carriers (ACs) and 20 healthy controls (HCs) to evaluate the expression of IL-21 and Tax and PVL in non-activated and phorbol myristate acetate (PMA)-ionomycin-activated peripheral blood mononuclear cells (PBMCs). The mean mRNA expression of IL-21 in the non-activated and activated PBMCs was higher (by 5-13 times) in the HAM/TSP patients than in ACs and HCs (p Tax and PVL was observed in the HAM/TSP subjects than ACs (p Tax gene expression was positively correlated with PVL (R = 0.595, p = 0.000) and IL-21 gene expression (R = 0.395, p = 0.021) in the HTLV-1-infected subjects. In conclusion, the increase in IL-21 mRNA expression may reflect the attempt of infected T cells to induce an appropriate antiviral response, and the decrease in IL-21 protein expression may reflect the inhibition of IL-21 mRNA translation by viral factors in favour of virus evasion and dissemination.

Neurorestoratology evidence in an animal model with cervical spondylotic myelopathy

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Li X

2017-01-01

Full Text Available Xiang Li,1,2 Guangsheng Li,1,3 Keith Dip-Kei Luk,1 Yong Hu1–3 1Department of Orthopaedics and Traumatology, The University of Hong Kong, Pokfulam, Hong Kong, 2Shenzhen Key Laboratory for Innovative Technology in Orthopaedic Trauma, The University of Hong Kong-Shenzhen Hospital, Shenzhen, 3Spinal Division, Department of Orthopaedics, Affiliated Hospital of Guangdong Medical University, Guangdong, People’s Republic of China Background: Cervical spondylotic myelopathy (CSM is a chronic compression injury of the spinal cord, with potentially reversible conditions after surgical decompression, and a unique model of incomplete spinal cord injury. Several animal studies showed pathological changes of demyelination, axon loss and neuron apoptosis in rats with chronic spinal cord compression. However, there is a limited understanding of the neurological change in the spinal cord after surgical decompression. The aim of this study was to validate the neurorestoratology of myelopathic lesions in the spinal cord in a rat model. Materials and methods: A total of 16 adult Sprague-Dawley rats were divided into four groups: sham control (group 1; CSM model with 4-week chronic compression (group 2, 2 weeks (group 3 and 4 weeks (group 4 after surgical decompression of CSM model. The compression and decompression were verified by magnetic resonance imaging (MRI test. Neurological function was evaluated by Basso, Beattie, and Bresnahan (BBB locomotor rating scale, ladder rung walking test and somatosensory-evoked potentials (SEPs. Neuropathological change was evaluated by histological examinations. Results: MRI confirmed the compression of the cervical spinal cord as well as the reshaping of cord morphology after decompression. After decompression, significant changes of neurological function were observed in BBB scores (p < 0.01, F = 10.52, ladder rung walking test (p < 0.05, F = 14.21 and latencies (p < 0.05, F = 5.76 and amplitudes (p < 0.05, F = 3.8 of

A study on the clinical significance of magnetic resonance imaging (MRI) findings in patients with cervical spondylotic myelopathy

International Nuclear Information System (INIS)

Toyooka, Satoshi

1997-01-01

This study was designed to evaluate magnetic resonance (MR) images of the cervical compressive myelopathy. It was also meant to serve as a review of clinical symptoms and an investigation of the usefulness of MRI. Comparative studies were carried out on 110 cases concerning the shape and signal intensity of the spinal cord, anterior epidural venous plexus MR images and clinical symptoms. The shape of the spinal cord and pre- and post-surgical conditions revealed by MRI correlated with clinical symptoms. As for the signal intensity of the spinal cord, in cases in which both high (T2-weighted image) and low (T1-weighted image) signals detected prior to surgery continued after surgery, as well as cases with high and low signals appearing after surgery, had the lower improvement than average. Low signal intensity on T1-weighted images are assumed to indicate irreversible changes of the spinal cord. High signal intensity on T2-weighted images is assumed to indicate both reversible and irreversible changes of the spinal cord. Epidural venous plexus can also be observed in healthy people and is not directly bound to clinical manifestations. Nevertheless, changes in the shape of the epidural venous plexus and signal intensity can reflect venous plexus compression and circulatory changes caused by compression. In the application of MRI to cervical compressive myelopathies, images of changes in the shape and signal intensity of the spinal cord and anterior epidural venous plexus images were considered important observations linked to clinical symptoms. MRI is an essential non-invasive imaging technique for the diagnosis of cervical compressive myelopathy, estimation of prognosis and postoperative follow-up. More investigations of compressive factors, circulatory dynamics of the spinal cord and high quality image are necessary. (author)

The functional relevance of diffusion tensor imaging in comparison to conventional MRI in patients with cervical compressive myelopathy

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Yang, Young-Mi; Oh, Jae-Keun; Song, Ji-Sun [Hallym University Sacred Heart Hospital, College of Medicine, Hallym University, Spine Center, Anyang-si, Gyeonggi-do (Korea, Republic of); Yoo, Woo-Kyoung [Hallym University Sacred Heart Hospital, College of Medicine, Hallym University, Department of Physical Medicine and Rehabilitation, Anyang-si (Korea, Republic of); Hallym University Sacred Heart Hospital, College of Medicine, Hallym University, Hallym Institute for Translational Genomics and Bioinformatics, Anyang-si (Korea, Republic of); Yoo, Je Hyun; Kwak, Yoon Hae [Hallym University Sacred Heart Hospital, College of Medicine, Hallym University, Department of Orthopaedic surgery, Anyang-si (Korea, Republic of); Kim, Seok Woo [Hallym University Sacred Heart Hospital, College of Medicine, Hallym University, Spine Center, Anyang-si, Gyeonggi-do (Korea, Republic of); Hallym University Sacred Heart Hospital, College of Medicine, Hallym University, Department of Orthopaedic surgery, Anyang-si (Korea, Republic of)

2017-11-15

To determine the functional relevance of diffusion tensor imaging (DTI) metrics and conventional MRI (signal intensity change in T2, compression ratio) by measuring the correlation of these parameters with clinical outcome measured by the modified Japanese Orthopedic Association (mJOA) score. A total of 20 cervical myelopathy (CM) patients participated in this prospective cohort study. The severities of CM were assessed using the mJOA score. Conventional MRIs (T2-weighted images) measuring the signal changes of spinal cords and the degree of compression at the lesion level and DTI metrics [fractional anisotropy (FA), apparent diffusion coefficient (ADC)] at each lesion and below each lesion (C7/T1) level were acquired using a 3-T Achieva MRI. These parameters were correlated with the mJOA scores to determine the functional relevance. Ninety percent of CM patients showed signal changes and 30 % of patients noted a more than 40% canal compression ratio in conventional MRIs at the lesion level; however, these findings were not correlated with the mJOA score (p < 0.05). In contrast, FA values on DTI showed high sensitivity to CM (100%), which was well correlated with the mJOA score (p = 0.034, r = 0.475) below the lesion level (C7/T1). This study showed a meaningful symptomatic correlation between mJOA scores and FA values below the lesion levels in CM patients. It could give us more understanding of the pathological changes in spinal cords matched with various clinical findings in CM patients than the results from conventional MRI. (orig.)

Radiation myelopathy; Myelopathie postradique

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Rafai, M.A.; Boulaajaj, F.Z.; Amriss, O.; El Moutawakil, B.; Slassi, I. [Explorations Fonctionnelles, CHU Ibn Rochd, Service de Neurologie, Quartier des Hopitaux (Morocco); Rafai, M.A. [Faculte de Medecine et de Pharmacie, Lab. des Neurosciences Cliniques, Casablanca (Morocco)

2009-12-15

The post radiation induced myelopathy is a very serious complication, developing months or years after the irradiation of tumors localized near the marrow. It is rare if the radiation dose is inferior to 50 Gy. We report the case of a patients treated for a cavum cancer occurring one year after the radiotherapy with a stable evolution. (N.C.)

Balance, functional mobility, and fall occurrence in patients with human T-cell lymphotropic virus type-1-associated myelopathy/tropical spastic paraparesis: a cross-sectional study.

Science.gov (United States)

Fonseca, Erika Pedreira da; Sá, Katia Nunes; Nunes, Rebeca Freitas Reis; Ribeiro Junior, Antônio Carlos; Lira, Síntia Freitas Bastos; Pinto, Elen Beatriz

2018-01-01

Human T-cell lymphotropic virus type-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) may lead to reduced functional mobility and balance. It is important to establish specific parameters that identify these changes and predict the risk of falls in these patients. The aim was to compare balance, functional mobility, and occurrence of falls among patients with and without HAM/TSP and to suggest values to predict the risk of falls in these patients. A cross-sectional study in patients with and without HAM/TSP involved balance assessments based on the berg balance scale (BBS) and functional mobility evaluation based on the timed up and go (TUG) test. From reports of falls, the sensitivity, specificity, and best cutoff points for the risk of falls assessed by these instruments were established using the receiver-operating characteristic (ROC) curve; 5% alpha was considered. We selected 42 participants: 29 with HAM/TSP and 13 without HAM/TSP. There was a statistically significant difference in the occurrence of falls, balance, and functional mobility between the groups (prisk of falls were defined as 50 points for the BBS and 12.28 seconds for the TUG test. Patients with HAM/TSP present reduced functional mobility and balance in relation to those without HAM/TSP. The risk of falls increased for these patients can be evaluated by the values ​​of 50 points using the BBS and 12.28 seconds using the TUG test.

Venous hypertensive myelopathy associated with cervical spondylosis.

Science.gov (United States)

Okada, Seiji; Chang, Charles; Chang, Geraldine; Yue, James J

2016-11-01

Venous hypertensive myelopathy (VHM) results from spinal vascular malformations of arteriovenous shunting that increases spinal venous pressure, leading to congestive edema and neurologic dysfunction. There has been no report of VHM associated with cervical spondylotic myelopathy (CSM). The aim of this study was to report an extremely rare case of VHM likely due to CSM. This study is a case report and review of the literature. The patient was a 51-year-old man with CSM exhibiting relatively rapid neurologic deterioration with an abnormal expansion of a centromedullary hyperintense lesion on T2-weighted magnetic resonance imaging (MRI) in the absence of traumatic injury. Neurologic examination and radiologic imaging were taken by various means. The patient developed a cervical radiculopathy, followed by gait disturbance and motor weakness. The MRI of the cervical spine demonstrated spinal canal stenosis due to disc bulging and flavum hypertrophy at the C5/C6 and C6/C7 levels as well as hyperintense area over the C5-C7 levels on T2-weighted images. Although decompression surgery was planned, an acute inflammatory process such as transverse myelitis or demyelinating disease other than cord compression was also considered, and the patient received intravenous steroids. His walking improved for several days. However, his symptoms then became significantly worse, and he had difficulty walking. Subsequent MRI demonstrated marked progression of the T2 hyperintense lesion over the C4-T1 vertebral levels. Flow voids were also noted on the dorsal surface of the upper cervical cord on T2-weighted MRI. His lab work, medical history, and the local enhancement on contrast-enhanced MRI indicated low probability of spinal inflammatory diseases. Therefore, the decision was made to perform anterior cervical discectomy and fusion surgery on two levels. Following surgery, his symptoms improved promptly. Our case indicates that VHM could be caused by spondylotic cord compression in the

CERVICAL SPONDYLOTIC MYELOPATHY WITH FUNCTIONAL ...

African Journals Online (AJOL)

CERVICAL SPONDYLOTIC MYELOPATHY WITH FUNCTIONAL DISABILITY. LONG TERM RESULTS CONCERNING 18 PATIENTS OPERATED ON BY ANTERIOR APPROACH IN GABON MYELOPATHIES CERVICARTHROSIQUES INVALIDANTES. RESULTATS A LONG TERME DE 18 PATIENTS OPERES PAR VOIE ...

The efficacy of dynamic MRI in assessing a cervical myelopathy

International Nuclear Information System (INIS)

Morimoto, Tetsuya; Yamada, Tomonori; Okumura, Yoshiya; Hashimoto, Hiroshi; Hiramatsu, Kenichiro; Tsunoda, Shigeru; Sakaki, Toshisuke; Iwasaki, Satoru

1994-01-01

There are problems that are unresolved with regard to the treatment of cases presenting a post-taumatic cervical myelopathy, such as when the surgical indications are not clearly evidence and the proper timing of this surgery. In this regard, the authors have used dynamic MRI to retrospectively analyze the cervical spine of 24 previously treated dynamic MRI cases presenting a subacute myelopathy to determine the efficacy of dynamic MRI as a method of treatment. Dynamic MRI analysis protocol was as follows. For the MR imagings, each patient was placed supine with the neck in the neutral position, after which the neck was set in the extended position. Dynamic changes between the neutral position and extended position images were analyzed by focusing on the following two point: (1) the narrowing of the subarachnoid space in the T2-weighted images and (2) evidence of cord compression in the T1-weighted images. Twelve cases out of 24 were treated conservatively because of a gradual improvement in their myelopathic symptoms. The other 12 cases were treated surgically, because of no improvement in their residual myelopathic symptoms at the time when the dynamic MRI had been performed. In the majority of cases in the surgical group, the narrowing of the subarachnoid space and spinal cord compression were hightened on neck extension, whereas in the conservative group, such findings were minimal. Dynamic MRI also more clearly visualized multiple lesions and the direction of the cord compression. These findings thus provided more detailed information to plan the surgical approach and to estimate the amount of surgical decompression needed. Based on the results of this retrospective study, we thus concluded that surgical treatment appears to benefit subacute cases who show no improvement in their residual myelopathic symptoms and whose dynamic MRI results also demonstrate an increased narrowing of subarachnoid space and a heightened spinal cord compression. (author)

Tropical spastic paraparesis in Northeastern Brazil

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C. M. de Castro Costa

1989-06-01

Full Text Available Ten possible cases of tropical spastic paraparesis (TSP in Northeastern Brazil (Ceará are presented. They show the typical symptoms and signs of TSP consisting of weakness of the lower limbs, spastic gait, hiperreflexia, bladder dysfunction and variable signs of posterior columns impairment. The laboratory examinations excluded other compressive, infective, degenerative or demyelinating lesions of their spinal cord. Our patients age ranged from 21 to 59 years, all were of black origin and all were of lower social class. There was a slight preponderance of females. An etiological implication of a retrovirus (HTLV-I has been shown for TSP, but for lack of technical conditions we could not determine it in our patients, and that stands as our subsequent step in those and further cases.

Increased expression of OX40 is associated with progressive disease in patients with HTLV-1-associated myelopathy/tropical spastic paraparesis.

Science.gov (United States)

Saito, Mineki; Tanaka, Reiko; Arishima, Shiho; Matsuzaki, Toshio; Ishihara, Satoshi; Tokashiki, Takashi; Ohya, Yusuke; Takashima, Hiroshi; Umehara, Fujio; Izumo, Shuji; Tanaka, Yuetsu

2013-05-07

OX40 is a member of the tumor necrosis factor receptor family that is expressed primarily on activated CD4+ T cells and promotes the development of effector and memory T cells. Although OX40 has been reported to be a target gene of human T-cell leukemia virus type-1 (HTLV-1) viral transactivator Tax and is overexpressed in vivo in adult T-cell leukemia (ATL) cells, an association between OX40 and HTLV-1-associated inflammatory disorders, such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP), has not yet been established. Moreover, because abrogation of OX40 signals ameliorates chronic inflammation in animal models of autoimmune disease, novel monoclonal antibodies against OX40 may offer a potential treatment for HTLV-1-associated diseases such as ATL and HAM/TSP. In this study, we showed that OX40 was specifically expressed in CD4+ T cells naturally infected with HTLV-1 that have the potential to produce pro-inflammatory cytokines along with Tax expression. We also showed that OX40 was overexpressed in spinal cord infiltrating mononuclear cells in a clinically progressive HAM/TSP patient with a short duration of illness. The levels of the soluble form of OX40 (sOX40) in the cerebrospinal fluid (CSF) from chronic progressive HAM/TSP patients or from patients with other inflammatory neurological diseases (OINDs) were not different. In contrast, sOX40 levels in the CSF of rapidly progressing HAM/TSP patients were higher than those in the CSF from patients with OINDs, and these patients showed higher sOX40 levels in the CSF than in the plasma. When our newly produced monoclonal antibody against OX40 was added to peripheral blood mononuclear cells in culture, HTLV-1-infected T cells were specifically removed by a mechanism that depends on antibody-dependent cellular cytotoxicity. Our study identified OX40 as a key molecule and biomarker for rapid progression of HAM/TSP. Furthermore, blocking OX40 may have potential in therapeutic intervention for

Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera

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Shuhei Ito

2017-01-01

Full Text Available Extramedullary hematopoiesis (EMH occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera.

Science.gov (United States)

Ito, Shuhei; Fujita, Nobuyuki; Hosogane, Naobumi; Nagoshi, Narihito; Yagi, Mitsuru; Iwanami, Akio; Watanabe, Kota; Tsuji, Takashi; Nakamura, Masaya; Matsumoto, Morio; Ishii, Ken

2017-01-01

Extramedullary hematopoiesis (EMH) occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera

OpenAIRE

Ito, Shuhei; Fujita, Nobuyuki; Hosogane, Naobumi; Nagoshi, Narihito; Yagi, Mitsuru; Iwanami, Akio; Watanabe, Kota; Tsuji, Takashi; Nakamura, Masaya; Matsumoto, Morio; Ishii, Ken

2017-01-01

Extramedullary hematopoiesis (EMH) occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

Degenerative Cervical Myelopathy: A Spectrum of Related Disorders Affecting the Aging Spine.

Science.gov (United States)

Tetreault, Lindsay; Goldstein, Christina L; Arnold, Paul; Harrop, James; Hilibrand, Alan; Nouri, Aria; Fehlings, Michael G

2015-10-01

Cervical spinal cord dysfunction can result from either traumatic or nontraumatic causes, including tumors, infections, and degenerative changes. In this article, we review the range of degenerative spinal disorders resulting in progressive cervical spinal cord compression and propose the adoption of a new term, degenerative cervical myelopathy (DCM). DCM comprises both osteoarthritic changes to the spine, including spondylosis, disk herniation, and facet arthropathy (collectively referred to as cervical spondylotic myelopathy), and ligamentous aberrations such as ossification of the posterior longitudinal ligament and hypertrophy of the ligamentum flavum. This review summarizes current knowledge of the pathophysiology of DCM and describes the cascade of events that occur after compression of the spinal cord, including ischemia, destruction of the blood-spinal cord barrier, demyelination, and neuronal apoptosis. Important features of the diagnosis of DCM are discussed in detail, and relevant clinical and imaging findings are highlighted. Furthermore, this review outlines valuable assessment tools for evaluating functional status and quality of life in these patients and summarizes the advantages and disadvantages of each. Other topics of this review include epidemiology, the prevalence of degenerative changes in the asymptomatic population, the natural history and rates of progression, risk factors of diagnosis (clinical, imaging and genetic), and management strategies.

Sudden onset of cervical spondylotic myelopathy during sleep: a case report.

Science.gov (United States)

Young, Irene A; Burns, Stephen P; Little, James W

2002-03-01

Cervical spondylotic myelopathy is a common cause of compressive spinal cord dysfunction. The typical course involves either a gradual or an episodic increase in symptoms and neurologic deficits, with impairment evolving over a period of months to years. Acute neurologic deterioration in conjunction with cervical spondylosis has been described almost exclusively in traumatic situations such as disk herniation. We report a case of an acute, nontraumatic onset of tetraplegia in association with cervical spondylosis. A 56-year-old man developed tetraplegia during a 1-hour nap, with loss of volitional control of his extremities, impaired sensation below the C3 dermatome, and increased muscle tone. Magnetic resonance imaging of the cervical spine revealed canal stenosis and increased T2 signal within the cord. This case report describes the rehabilitation course for this patient and reviews the clinical spectrum of onset and progression of cervical spondylotic myelopathy. Copyright 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation

Acute progressive paraplegia in heroin-associated myelopathy.

Science.gov (United States)

Mahoney, Kyle W; Romba, Meghan; Gailloud, Philippe; Izbudak, Izlem; Saylor, Deanna

2018-05-01

As the opioid epidemic continues, understanding manifestations of abuse, including heroin-associated myelopathy remains essential. Here we describe a young man with a past medical history significant for polysubstance abuse who developed acute-onset, rapidly progressive myelopathy after resumption of intravenous heroin use. He had significant spinal cord involvement with findings suggestive of heroin-associated myelopathy. The salient features of this case include diffusion imaging of the spine and spinal angiography supporting a possible vasculopathy as the pathophysiologic mechanism underlying heroin-associated myelopathy. Additionally, CSF studies showed the transition from a neutrophilic pleocytosis to a lymphocytic pleocytosis suggesting an inflammatory component. Copyright © 2018 Elsevier Ltd. All rights reserved.

Preoperative and postoperative evaluation of clinical symptoms of cervical myelopathy

International Nuclear Information System (INIS)

Ito, Tomokazu; Oshima, Yoshihiko; Ota, Yoshio

1993-01-01

To evaluate the clinical significance of signal changes on T2-weighted magnetic resonance (MR) images in myelopathy, the relationship between characteristics of clinical symptoms and postoperative recovery was examined. The subjects were a total of 52 patients (37 men and 15 women) with cervical myelopathy (n=44) or ossification of the posterior longitudinal ligament (n=8), who underwent extended surgery of Yamagata University's style. They ranged in age from 33 to 81 years (mean, 60.8). The degree of spinal compression and signal changes in the spinal cord were classified on T1-weighted and T2-weighted sagittal images, respecstively. Hyperintense on T2-weighted images tended to be associated with lower preoperative JOA's scores especially for exercise, legs and bladder or rectum function, as compared with isointense. T2-weighted images seemed to reflect, to some degree, the severity of preoperative clinical symptoms. For the rate of postoperative improvement, however, there was no significant difference between the hyperintense and isointense groups on T2-weighted images. (N.K.)

Intravenous methylprednisolone in HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP Metilprednisolona endovenosa na mielopatia associada ao HTLV-I/Paraparesia Espástica Tropical (MAH/PET

Directory of Open Access Journals (Sweden)

Abelardo Q-C Araújo

1993-09-01

Full Text Available HTLV-I (Human T-lymphotropic virus type I associated myelopathy/tropical spastic paraparesis (HAM/TSP is an immunomediated myelopathy induced by the HTLV-I. Some patients, specially those from Japan, seem to have a good response to steroid treatment. However, this has not been found in other regions of the world. High dose intravenous methylprednisolone has been used with success in patients with relapses of multiple sclerosis (MS, another autoimmune disease of the central nervous system. To test the effectiveness of methylprednisolone in patients with HAM/TSP, we devised an open trial in 23 patients. We found a very limited benefit of this form of treatment in these patients. Only one patient, who had the shortest disease duration (five months in the whole group, showed a sustained benefit. We speculate that those patients with a shorter history, with presumably less demye-lination and more inflammatory lesions, would show a better response to immunossupressive treatments.A mielopatia associada ao protovírus T-linfotrópico humano (HTLV-I, também conhecida como paraparesia espástica tropical associada ao HTLV-I (MAH/PET, constitui enfermidade imunomediada desencadeada pela infecção pelo HTLV-I. Nesta condição tem sido demonstrada, particularmente em pacientes japoneses, boa resposta clínica à terapêutica com corticosteróides. Este efeito benéfico todavia não foi encontrado em todas as regiões do mundo. Pulsoterapia com metilprednisolona endovenosa tem sido utilizada com sucesso em pacientes com esclerose múltipla, outro exemplo de doença auto-imune do sistema nervoso central, especialmente durante as fases de exacerbação da doença. Objetivando testar a eficácia da pulsoterapia com metilprednisolona em pacientes com MAH/PET, conduzimos estudo aberto em 23 doentes. Não constatamos efeito benéfico significativo desta forma de tratamento na maioria dos enfermos estudados. Apenas um dos pacientes, o qual exibia o menor tempo de

Balance, functional mobility, and fall occurrence in patients with human T-cell lymphotropic virus type-1-associated myelopathy/tropical spastic paraparesis: a cross-sectional study

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Erika Pedreira da Fonseca

Full Text Available Abstract INTRODUCTION: Human T-cell lymphotropic virus type-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP may lead to reduced functional mobility and balance. It is important to establish specific parameters that identify these changes and predict the risk of falls in these patients. The aim was to compare balance, functional mobility, and occurrence of falls among patients with and without HAM/TSP and to suggest values to predict the risk of falls in these patients. METHODS: A cross-sectional study in patients with and without HAM/TSP involved balance assessments based on the berg balance scale (BBS and functional mobility evaluation based on the timed up and go (TUG test. From reports of falls, the sensitivity, specificity, and best cutoff points for the risk of falls assessed by these instruments were established using the receiver-operating characteristic (ROC curve; 5% alpha was considered. RESULTS: We selected 42 participants: 29 with HAM/TSP and 13 without HAM/TSP. There was a statistically significant difference in the occurrence of falls, balance, and functional mobility between the groups (p<0.05. Good accuracy was determined for the BBS (77% and TUG test (70% and the cutoff points for the risk of falls were defined as 50 points for the BBS and 12.28 seconds for the TUG test. CONCLUSIONS: Patients with HAM/TSP present reduced functional mobility and balance in relation to those without HAM/TSP. The risk of falls increased for these patients can be evaluated by the values of 50 points using the BBS and 12.28 seconds using the TUG test.

Cardiovascular risk profile in patients with myelopathy associated with HTLV-1.

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Prado, Fabio Luís Silva do; Prado, Renata; Ladeia, Ana Marice Teixeira

HAM/TSP (HTLV-1-associated myelopathy/tropical spastic paraparesis) is a slowly progressive disease, characterized by a chronic spastic paraparesis. It is not known if the disease carries an independent risk for cardiovascular disease. The objective of this study was to evaluate the cardiovascular risk profile related to HAM/TSP and compare it with the general population. This was a cross-sectional study, with a control group. HAM/TSP patients were evaluated using cardiovascular risk scores (ASCVD RISK, SCORE and Framingham) and inflammatory markers (ultrasensitive CRP and IL-6), and compared with a control group of healthy individuals. We also evaluated the correlation between cardiovascular risk and the functional status of patients with HAM/TSP evaluated by the FIM scale. Eighty percent of patients in this study were females, mean age of 51 years (11.3). The control group showed an increased cardiovascular event risk in 10 years when ASCVD was analyzed (cardiovascular risk ≥7.5% in 10 years seen in 43% of patients in the control group vs. 23% of patients with HAM/TSP; p=0.037). There was no difference in ultrasensitive CRP or IL-6 values between the groups, even when groups were stratified into low and high risk. There was no correlation between the functional status of HAM/TSP patients and the cardiovascular risk. In this study, the cardiovascular risk profile of patients with HAM/TSP was better than the risk of the control group. Copyright © 2017 Sociedade Brasileira de Infectologia. Published by Elsevier Editora Ltda. All rights reserved.

[Spasticity and dynamic plantar pressure distribution measurements in hemiplegic spastic children].

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Femery, V; Moretto, P; Renaut, H; Thévenon, A

2001-02-01

The aim of this study was to analyse the plantar pressure distribution in nine hemiplegic spastic children to illustrate the dynamic alteration during stance phase linked spasticity grade. The graduation of the lower limbs muscle tone related to the Aschworth spasticity scale enabled us to identify two groups of hemiplegics subjects. The groups Asch 1 and Asch 3 have respectively presented a low and a strong spasticity. The peak pressures during consecutive gait cycles were determined under the feet of 30 healthy subjects and two cerebral palsy groups using a wearable footprint analysis system. A statistical study showed a similarity between the two disabled groups. Peak pressures under the midfoot were significantly higher compared to the control group. While the plantar pressure distribution profile was specific for each group under all other anatomical structures. The significant alterations were observed under the forefoot and hallux. Spasticity modifies the foot contact to ground and leads to a specific plantar pressure distribution profile linked to the spasticity grade. The equinovarus with clawed toes deformity due to higher spasticity seems to be an important factor in terminal stance phase perturbations. However spastic hemiplegic subjects seem to adopt a gait pattern in agreement with stability optimization criteria.

Tract-Specific Diffusion Tensor Imaging Reveals Laterality of Neurological Symptoms in Patients with Cervical Compression Myelopathy.

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Maki, Satoshi; Koda, Masao; Saito, Junya; Takahashi, Sho; Inada, Taigo; Kamiya, Koshiro; Ota, Mitsutoshi; Iijima, Yasushi; Masuda, Yoshitada; Matsumoto, Koji; Kojima, Masatoshi; Takahashi, Kazuhisa; Obata, Takayuki; Yamazaki, Masashi; Furuya, Takeo

2016-12-01

Patients with cervical compression myelopathy (CCM) generally present bilateral neurological symptoms in their extremities. However, a substantial portion of patients with CCM exhibit laterality of neurological symptoms. The aim of this study was to assess the correlation between intrinsic structural damage and laterality of symptoms using spinal cord diffusion tensor imaging (DTI) of the corticospinal tract. We enrolled 10 healthy volunteers and 40 patients with CCM in this study. We evaluated motor function using the American Spinal Injury Association (ASIA) motor score for left and right extremities. For DTI acquisitions, a 3.0-T magnetic resonance imaging system with diffusion-weighted spin-echo sequence was used. Regions-of-interest in the lateral column tracts were determined. We determined the correlations between fractional anisotropy (FA) and ASIA motor scores. An FA asymmetry index was calculated using left and right regions-of-interest. Four patients exhibited laterality of symptoms in their extremities, for which left and right ASIA scores correlated moderately with FA in the left and right lateral columns, respectively (left: ρ = 0.64, P laterality of symptoms. Using tract-specific DTI, we demonstrated that microstructural damages in the left and right corticospinal tracts correlated with corresponding neurological symptoms in the ipsilateral side and the FA asymmetry index could indicate laterality in neurological symptoms of patients with CCM. Copyright © 2016 Elsevier Inc. All rights reserved.

The efficacy of dynamic MRI in assessing a cervical myelopathy; A retrospective study

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Morimoto, Tetsuya; Yamada, Tomonori; Okumura, Yoshiya; Hashimoto, Hiroshi; Hiramatsu, Kenichiro; Tsunoda, Shigeru; Sakaki, Toshisuke; Iwasaki, Satoru (Nara Medical Univ., Kashihara (Japan))

1994-01-01

There are problems that are unresolved with regard to the treatment of cases presenting a post-taumatic cervical myelopathy, such as when the surgical indications are not clearly evidence and the proper timing of this surgery. In this regard, the authors have used dynamic MRI to retrospectively analyze the cervical spine of 24 previously treated dynamic MRI cases presenting a subacute myelopathy to determine the efficacy of dynamic MRI as a method of treatment. Dynamic MRI analysis protocol was as follows. For the MR imagings, each patient was placed supine with the neck in the neutral position, after which the neck was set in the extended position. Dynamic changes between the neutral position and extended position images were analyzed by focusing on the following two point: (1) the narrowing of the subarachnoid space in the T2-weighted images and (2) evidence of cord compression in the T1-weighted images. Twelve cases out of 24 were treated conservatively because of a gradual improvement in their myelopathic symptoms. The other 12 cases were treated surgically, because of no improvement in their residual myelopathic symptoms at the time when the dynamic MRI had been performed. In the majority of cases in the surgical group, the narrowing of the subarachnoid space and spinal cord compression were hightened on neck extension, whereas in the conservative group, such findings were minimal. Dynamic MRI also more clearly visualized multiple lesions and the direction of the cord compression. These findings thus provided more detailed information to plan the surgical approach and to estimate the amount of surgical decompression needed. Based on the results of this retrospective study, we thus concluded that surgical treatment appears to benefit subacute cases who show no improvement in their residual myelopathic symptoms and whose dynamic MRI results also demonstrate an increased narrowing of subarachnoid space and a heightened spinal cord compression. (author).

HTLV-1 antibodies in serum and cerebrospinal fluid in tropical spastic paraparesis in Brazil

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A. Spina-França

1990-12-01

Full Text Available HTLV-l antibodies were investigated in serum and in CSF of 150 patients with neurologic disorders mainly myelopathies. The patients were considered into three groups according to the possible relationship of their disease to the presence of HTLV-l antibodies: no relationship risk (control group, occasional risk group, and possible risk group. In this latter are 56 patients with crural spastic paraparesis or paraplegia of unknown etiology (SP. HTLV-l antibodies were tested by the passive particle-agglutination method for anti-ATLA antibody detection. The search was negative in all patients of the control group, and positive (serum and/or CSF in 16.5% of the patients from the second group and in 55.4% of the SP patients group. Clinical patterns in SP cases with HTLV-l antibodies were those of tropical spastic paraparesis (TSP. CSF patterns considered (cytology, protein content and gamma-globulins rate were different between TSP group with HTLV-l antibodies in CSF and SP group with no HTLV-l antibodies detection either in serum or in CSF. The difference was significant. Results of this investigation confirm the high incidence of TSP in Brazil, and bring additional indication for searching HTLV-l antibodies in the CSF.

Case of chronic progressive radiation myelopathy with a CT myelogram simulating intramedullary tumor

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Kanemaru, Kazutomi; Kamo, Hisaki; Yamao, Satoshi; Akiguchi, Ichiro; Kameyama, Masakuni

1985-05-01

A 58-year-old man underwent a right middle lobectomy in June, 1975, for poorly differentiated adenocarcinoma of the lung. Postoperative irradiation was given to the hilus (6100 rads), and to the right supraclavicular area (6000 rads). In 1980, 60 months after completion of irradiation, the patient noticed weakness of his legs particularly on the left side. In 1982, he noticed the girdle sensation in the upper thoracic region, and paresthesia in the lateral side of the right thigh. In Dec 1983, micturition disturbance appeared, and gait disturbance progressed, he was admitted to the Kyoto University Hospital. Neurological examination revealed an incomplete left Brown-Sequard syndrome with diminution of pain and thermal sensation on the right lower limb, and weakness and spasticity particularly on the left lower limb. Conventional myelogram with CT myelogram showed spinal cord swelling from T-2 through T-5. No extramedullary lesion was found. Laminectomy was performed through T-1 to T-6. When the dura was opened, the cord was swollen and necrotic with a cyst formation. Microscopic examination of the thickened part of the cord showed necrosis and gliosis. The lesion was correspond to the cord segments exposed to the radiation, and a diagnosis of radiation myelopathy was made. Several cases of radiation myelopathy with definite swelling of the cord at myelography were reported, but myelography in these cases was performed at most within 11 months after the onset. In this case, myelography was performed three years after the onset, and revealed difinite swelling of the cord due to a cyst formation. (author).

Correlação clinica entre a mielopatia cervical e o índice de Torg Correlation between the clinic and the index of cervical myelopathy Torg

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Agnaldo Rogério Lozorio

2012-01-01

Full Text Available OBJETIVO: A mielopatia cervical é uma disfunção da medula espinhal relacionada a degeneração típica do envelhecimento, cuja patologia se relaciona com a isquemia e compressão da medula. Muitos são os problemas clínicos apresentados por portadores de mielopatia, nos casos mais graves este acometimento pode levar a para ou tetraplegia quando não tratado. Devido a patologia primária desta doença ser causada por compressão gerando isquemia medular, julgamos poder existir uma correlação entre o grau de compressão e clínica dos pacientes portadores de mielopatia cervical, porém não encontramos nenhum estudo na literatura que realizou esta correlação, por existir esta dúvida na literatura é que objetivamos em nosso estudo analisar a correlação entre o grau do comprometimento clínico dos pacientes com mielopatia cervical e o índice de Torg. MÉTODOS: Estudo prospectivo, de caráter descritivo, avaliados 46 pacientes, realizado mensuração radiográfica do índice de Torg e análise clínica através da escala de JOA e Nurick. RESULTADOS: Dos 46 pacientes, 100% apresentaram Torg OBJECTIVE: Cervical myelopathy is a spinal cord dysfunction related to degeneration typical of aging. Its primary pathology is related to ischemia and spinal cord compression. Patients with myelopathy present many clinical problems; more severe cases may lead to quadriplegia if not treated in a timely manner. Because the primary pathology of this disease is caused by compression, thus generating spinal cord ischemia, we believed there must be a correlation between the degree of compression and the clinical assessment of patients with cervical myelopathy, but we did not find any study in the literature that made this correlation. Because there is doubt the literature we aimed, in our study, to analyze the correlation between the degree of clinical impairment of patients with cervical myelopathy and the Torg index. METHODS: A prospective, descriptive

A case of chronic progressive radiation myelopathy with a CT myelogram simulating intramedullary tumor

International Nuclear Information System (INIS)

Kanemaru, Kazutomi; Kamo, Hisaki; Yamao, Satoshi; Akiguchi, Ichiro; Kameyama, Masakuni

1985-01-01

A 58-year-old man underwent a right middle lobectomy in June, 1975, for poorly differentiated adenocarcinoma of the lung. Postoperative irradiation was given to the hilus (6100 rads), and to the right supraclavicular area (6000 rads). In 1980, 60 months after completion of irradiation, the patient noticed weakness of his legs particularly on the left side. In 1982, he noticed the girdle sensation in the upper thoracic region, and paresthesia in the lateral side of the right thigh. In Dec 1983, micturition disturbance appeared, and gait disturbance progressed, he was admitted to the Kyoto University Hospital. Neurological examination revealed an incomplete left Brown-Sequard syndrome with diminution of pain and thermal sensation on the right lower limb, and weakness and spasticity particularly on the left lower limb. Conventional myelogram with CT myelogram showed spinal cord swelling from T-2 through T-5. No extramedullary lesion was found. Laminectomy was performed through T-1 to T-6. When the dura was opened, the cord was swollen and necrotic with a cyst formation. Microscopic examination of the thickened part of the cord showed necrosis and gliosis. The lesion was correspond to the cord segments exposed to the radiation, and a diagnosis of radiation myelopathy was made. Several cases of radiation myelopathy with definite swelling of the cord at myelography were reported, but myelography in these cases was performed at most within 11 months after the onset. In this case, myelography was performed three years after the onset, and revealed difinite swelling of the cord due to a cyst formation. (author)

Mielopatia associada ao HTLV-I / paraparesia espástica tropical: relato dos primeiros casos em Sergipe HTLV-I associated myelopathy, tropical spastic paraparesis: report of the first cases in Sergipe-Brazil

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HÉLIO ARAUJO OLIVEIRA

1998-03-01

Full Text Available Mielopatia associada ao HTLV-I / paraparesia espástica tropical (MAH/PET, tem sido descrita em quase todas as regiões do Brasil.Os autores apresentam oito casos clinicamente definidos como MAH/PET, os primeiros relatados no Estado de Sergipe .Todos foram positivos para HTLV-I, através do método ELISA, realizado duas vezes; em apenas dois casos foi possível a confirmação por Western Blot. De acordo com protocolo de investigação clínico-laboratorial, todos os pacientes apresentaram acometimento do tracto piramidal, com mínimo comprometimeto da sensibilidade e alterações esfincterianas. Os autores chamam a atenção para a endemicidade do HTLV-I no Estado, cuja prevalência entre doadores de sangue é significativa (0,43%.HTLV-I associated myelopathy/ tropical spastic paraparesis (HAM/TSP has been decribed in practically all regions of Brazil. The authors present eight clinically defined cases of HAM/TSP, as being the first reported in Sergipe (Northeastern Brazil. All of them were confirmed through ELISA in two examinations, although only two were confirmed by Western Blot. According to clinical/laboratorial investigation protocol, all patients presented involvement of the pyramidal tract with minimal sensory loss and sphincter alteration. The authors call the attention for the endemicity of HTLV-I in the region, whose prevalence amongst blood donors is significant (0.43%.

Association of myelopathy scores with cervical sagittal balance and normalized spinal cord volume: analysis of 56 preoperative cases from the AOSpine North America Myelopathy study.

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Smith, Justin S; Lafage, Virginie; Ryan, Devon J; Shaffrey, Christopher I; Schwab, Frank J; Patel, Alpesh A; Brodke, Darrel S; Arnold, Paul M; Riew, K Daniel; Traynelis, Vincent C; Radcliff, Kris; Vaccaro, Alexander R; Fehlings, Michael G; Ames, Christopher P

2013-10-15

Post hoc analysis of prospectively collected data. Development of methods to determine in vivo spinal cord dimensions and application to correlate preoperative alignment, myelopathy, and health-related quality-of-life scores in patients with cervical spondylotic myelopathy (CSM). CSM is the leading cause of spinal cord dysfunction. The association between cervical alignment, sagittal balance, and myelopathy has not been well characterized. This was a post hoc analysis of the prospective, multicenter AOSpine North America CSM study. Inclusion criteria for this study required preoperative cervical magnetic resonance imaging (MRI) and neutral sagittal cervical radiography. Techniques for MRI assessment of spinal cord dimensions were developed. Correlations between imaging and health-related quality-of-life scores were assessed. Fifty-six patients met inclusion criteria (mean age = 55.4 yr). The modified Japanese Orthopedic Association (mJOA) scores correlated with C2-C7 sagittal vertical axis (SVA) (r = -0.282, P = 0.035). Spinal cord volume correlated with cord length (r = 0.472, P balance (C2-C7 SVA) to myelopathy severity. We found a moderate negative correlation in kyphotic patients of cord volume and cross-sectional area to mJOA scores. The opposite (positive correlation) was found for lordotic patients, suggesting a relationship of cord volume to myelopathy that differs on the basis of sagittal alignment. It is interesting to note that sagittal balance but not kyphosis is tied to myelopathy score. Future work will correlate alignment changes to cord morphology changes and myelopathy outcomes. SUMMARY STATEMENTS: This is the first study to correlate sagittal balance (C2-C7 SVA) to myelopathy severity. We found a moderate negative correlation in kyphotic patients of cord volume and cross-sectional area to mJOA scores. The opposite (positive correlation) was found for lordotic patients, suggesting a relationship of cord volume to myelopathy that differs on the

The relationship between spasticity and gross motor capability in nonambulatory children with spastic cerebral palsy.

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Katusic, Ana; Alimovic, Sonja

2013-09-01

Spasticity has been considered as a major impairment in cerebral palsy (CP), but the relationship between this impairment and motor functions is still unclear, especially in the same group of patients with CP. The aim of this investigation is to determine the relationship between spasticity and gross motor capability in nonambulatory children with spastic CP. Seventy-one children (30 boys, 41 girls) with bilateral spastic cerebral palsy and with Gross Motor Function Classification System (GMFCS) levels IV (n=34) and V (n=37) were included in the study. The spasticity level in lower limbs was evaluated using the Modified Modified Ashworth Scale and the gross motor function with the Gross Motor Function Measure (GMFM-88). Spearman's correlation analysis was used to determine the nature and the strength of the relationship. The results showed a moderate correlation between spasticity and gross motor skills (ρ=0.52 for the GMFCS level; ρ=0.57 for the GMFM-88), accounting for less than 30% of the explained variance. It seems that spasticity is just one factor among many others that could interfere with gross motor skills, even in children with severe forms of spastic CP. Knowledge of the impact of spasticity on motor skills may be useful in the setting of adequate rehabilitation strategies for nonambulatory children with spastic CP.

Radiation-induced myelopathy

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Gaenshirt, H [Heidelberg Univ. (F.R. Germany). Neurologische Klinik

1975-10-01

12 cases of radiation-induced myelopathy after /sup 60/Co teletherapy are reported on. Among these were 10 thoracal lesions, one cerviothoracal lesion, and one lesion of the medulla oblongata. In 9 cases, Hodgkin's disease had been the primary disease, tow patients had been irradiated because of suspected vertebral metastases of cancer of the breast, and one patient had suffered from a glomus tumour of the petrous bone. The spinal doses had exceeded the tolerance doses recommended in the relevant literature. There was no close correlation between the radiation dose and the course of the disease. The latency periods between the end of the radiotherapy and the onset of the neurological symptons varied from 6 to 16 mouths and were very constant in 7 cases with 6 to 9 months. The segmental height of the lesion corresponded to the level of irradiation. The presenting symptons of radiation-induced myelopathy are buruing dysaesthesias and Brown-Sequard's paralysis which may develop into transverse lesion of the cord with paraplegia still accompanied by dissociated perception disorders. The disease developed intermittently. Disturbances of the bladder function are frequent. The fluid is normal in most cases. Myelographic examinations were made in 8 cases. 3 cases developed into stationary cases exhibiting. Brown-Sequard syndrome, while 9 patients developed transverse lesion of the cord with paraplegia. 3 patients have died; antopsy findings are given for two of these. In the pathogenesis of radiation-induced myelopathy, the vascular factor is assumed to be of decisive importance.

Approach to a case of myeloneuropathy

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Ravindra Kumar Garg

2016-01-01

Full Text Available Myeloneuropathy is a frequently encountered condition and often poses a diagnostic challenge. A variety of nutritional, toxic, metabolic, infective, inflammatory, and paraneoplastic disorders can present with myeloneuropathy. Deficiencies of vitamin B12, folic acid, copper, and vitamin E may lead to myeloneuropathy with a clinical picture of subacute combined degeneration of the spinal cord. Among infective causes, chikungunya virus has been shown to produce a syndrome similar to myeloneuropathy. Vacuolar myelopathy seen in human immunodeficiency virus (HIV infection is clinically very similar to subacute combined degeneration. A paraneoplastic myeloneuropathy, an immune-mediated disorder associated with an underlying malignancy, may rarely be seen with breast cancer. Tropical myeloneuropathies are classified into two overlapping clinical entities — tropical ataxic neuropathy and tropical spastic paraparesis. Tropical spastic paraparesis, a chronic noncompressive myelopathy, has frequently been reported from South India. Establishing the correct diagnosis of myeloneuropathy is important because compressive myelopathies may pose diagnostic confusion. Magnetic resonance imaging (MRI in subacute combined degeneration of the spinal cord typically reveals characteristic signal changes on T2-weighted images of the cervical spinal cord. Once the presence of myeloneuropathy is established, all these patients should be subjected to a battery of tests. Blood levels of vitamin B12, folic acid, vitamins A, D, E, and K, along with levels of iron, methylmalonic acid, homocysteine, and calcium should be assessed. The pattern of neurologic involvement and results obtained from a battery of biochemical tests often help in establishing the correct diagnosis.

Diffusion Tensor Imaging Correlates with Short-Term Myelopathy Outcome in Patients with Cervical Spondylotic Myelopathy.

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Vedantam, Aditya; Rao, Avinash; Kurpad, Shekar N; Jirjis, Michael B; Eckardt, Gerald; Schmit, Brian D; Wang, Marjorie C

2017-01-01

To determine if spinal cord diffusion tensor imaging indexes correlate with short-term clinical outcome in patients undergoing elective cervical spine surgery for cervical spondylotic myelopathy (CSM). A prospective consecutive cohort study was performed in patients undergoing elective cervical spine surgery for CSM. After obtaining informed consent, patients with CSM underwent preoperative T2-weighted magnetic resonance imaging and diffusion tensor imaging of the cervical spine. Fractional anisotropy (FA) values at the level of maximum cord compression and at the noncompressed C1-2 level were calculated on axial images. We recorded the modified Japanese Orthopaedic Association (mJOA) scale, Neck Disability Index, and Short Form-36 physical functioning subscale scores for all patients preoperatively and 3 months postoperatively. Statistical analysis was performed to identify correlations between FA and clinical outcome scores. The study included 27 patients (mean age 54.5 years ± 1.9, 12 men). The mean postoperative changes in mJOA scale, Neck Disability Index, and Short Form-36 physical functioning subscale scores were 0.9 ± 0.3, -6.0 ± 1.9, and 3.4 ± 1.9. The mean FA at the level of maximum compression was significantly lower than the mean FA at the C1-2 level (0.5 vs. 0.55, P = 0.01). FA was significantly correlated with change in mJOA scale score (Pearson r = -0.42, P = 0.02). FA was significantly correlated with the preoperative mJOA scale score (Pearson r = 0.65, P < 0.001). Preoperative FA at the level of maximum cord compression significantly correlates with the 3-month change in mJOA scale score among patients with CSM. FA was also significantly associated with preoperative mJOA scale score and is a potential biomarker for spinal cord dysfunction in CSM. Published by Elsevier Inc.

Development of a self-administered questionnaire to screen patients for cervical myelopathy

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Sekiguchi Yasufumi

2010-11-01

Full Text Available Abstract Background In primary care, it is often difficult to diagnose cervical myelopathy. However, a delay in treatment could cause irreversible aftereffects. With a brief and effective self-administered questionnaire for cervical myelopathy, cervical myelopathy may be screened more easily and oversight may be avoided. As there is presently no screening tool for cervical myelopathy, the aim of this study was to develop a self-administered questionnaire for the screening of cervical myelopathy. Methods A case-control study was performed with the following two groups at our university hospital from February 2006 to September 2008. Sixty-two patients (48 men, 14 women with cervical myelopathy who underwent operative treatment were included in the myelopathy group. In the control group, 49 patients (20 men, 29 women with symptoms that could be distinguished from those of cervical myelopathy, such as numbness, pain in the upper extremities, and manual clumsiness, were included. The underlying conditions were diagnosed as carpal tunnel syndrome, cubital tunnel syndrome, thoracic outlet syndrome, tarsal tunnel syndrome, diabetes mellitus neuropathy, cervical radiculopathy, and neuralgic amyotrophy. Twenty items for a questionnaire in this study were chosen from the Japanese Orthopaedic Association Cervical Myelopathy Evaluation Questionnaire, which is a new self-administered questionnaire, as an outcome measure for patients with cervical myelopathy. Data were analyzed by univariate analysis using the chi-square test and by multiple logistic regression analysis. According to the resulting odds ratio, β-coefficients, and p value, items were chosen and assigned a score. Results Eight items were chosen by univariate and multiple logistic regression analyses and assigned a score. The Hosmer-Lemeshow statistic showed p = 0.805. The area under the receiver operation characteristic curve was 0.86. The developed questionnaire had a sensitivity of 93.5% and a

Clinical significance of MRI/18F-FDG PET fusion imaging of the spinal cord in patients with cervical compressive myelopathy

International Nuclear Information System (INIS)

Uchida, Kenzo; Nakajima, Hideaki; Watanabe, Shuji; Yoshida, Ai; Baba, Hisatoshi; Okazawa, Hidehiko; Kimura, Hirohiko; Kudo, Takashi

2012-01-01

18 F-FDG PET is used to investigate the metabolic activity of neural tissue. MRI is used to visualize morphological changes, but the relationship between intramedullary signal changes and clinical outcome remains controversial. The present study was designed to evaluate the use of 3-D MRI/ 18 F-FDG PET fusion imaging for defining intramedullary signal changes on MRI scans and local glucose metabolic rate measured on 18 F-FDG PET scans in relation to clinical outcome and prognosis. We studied 24 patients undergoing decompressive surgery for cervical compressive myelopathy. All patients underwent 3-D MRI and 18 F-FDG PET before surgery. Quantitative analysis of intramedullary signal changes on MRI scans included calculation of the signal intensity ratio (SIR) as the ratio between the increased lesional signal intensity and the signal intensity at the level of the C7/T1 disc. Using an Advantage workstation, the same slices of cervical 3-D MRI and 18 F-FDG PET images were fused. On the fused images, the maximal count of the lesion was adopted as the standardized uptake value (SUV max ). In a similar manner to SIR, the SUV ratio (SUVR) was also calculated. Neurological assessment was conducted using the Japanese Orthopedic Association (JOA) scoring system for cervical myelopathy. The SIR on T1-weighted (T1-W) images, but not SIR on T2-W images, was significantly correlated with preoperative JOA score and postoperative neurological improvement. Lesion SUV max was significantly correlated with SIR on T1-W images, but not with SIR on T2-W images, and also with postoperative neurological outcome. The SUVR correlated better than SIR on T1-W images and lesion SUV max with neurological improvement. Longer symptom duration was correlated negatively with SIR on T1-W images, positively with SIR on T2-W images, and negatively with SUV max . Our results suggest that low-intensity signal on T1-W images, but not on T2-W images, is correlated with a poor postoperative neurological

Myelopathy: Retrospective Evaluation of Twenty-Eight Cases

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Yüksel Erdal

2017-12-01

Full Text Available Objective: Myelopathy is used to describe any neurologic deficit related to the spinal cord. Determining the etiology is important for detecting emergency situations and treating the cause. Materials and Methods: We evaluated 28 patients who were diagnosed as having and treated for myelopathy between January 2014 and January 2015 in our hospital. The clinical and laboratory findings of the patients and their response to treatment were assessed. Results: Of the 28 patients, 16 were male and 12 were female, the mean age was 48 years (range, 22-77 years. The most common initial symptoms were sensorial deficits and motor weakness. Demyelinating diseases were the most common diseases and multiple sclerosis was the most frequent etiology among demiyelinating diseases. Cervical spinal cord was the most frequent region involved in myelopathy episodes which was detected by magnetic resonance imaging on T2W images. We observed clinical improvement in 15 of the 20 patients who were treated with corticosteroids. In addition, vitamin B12 treatment led to clinical improvement in two patients who were diagnosed as having subacute combined degeneration. Conclusion: The diagnosis and etiology of myelopathy and identification of rare conditions that require emergency surgery or interventional treatments are of utmost importance

Imaging Modalities for Cervical Spondylotic Stenosis and Myelopathy

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C. Green

2012-01-01

Full Text Available Cervical spondylosis is a spectrum of pathology presenting as neck pain, radiculopathy, and myelopathy or all in combination. Diagnostic imaging is essential to diagnosis and preoperative planning. We discuss the modalities of imaging in common practice. We examine the use of imaging to differentiate among central, subarticular, and lateral stenosis and in the assessment of myelopathy.

Research progress of HIV-associated myelopathy

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Kun HONG

2016-08-01

Full Text Available The wide usage of highly active antiretroviral therapy (HAART leads to reduction of the occurence rate of focal or diffuse neurological damage caused by human immunodeficiency virus (HIV infection, which prominently improves the living quality of HIV-infected patients. Despite this progress, about 70% of HIV-infected patients develop neurological complications. Although neurological disease typically occurs in the advanced stage of the disease or after severe damage of immune functions, it may also occur during early stage of the infection. HIV-associated myelopathy is a common complication of immunodeficiency syndrome and its typical pathological appearence is vacuolar degeneration. In many patients the clinical manifestations of vacuolar myelopathy are in fact limited to non-specific sphincter or sexual dysfunction, and may remain completely asymptomatic. Even when motor and sensory symptoms become evident, the diagnosis is often complicated by a concomitant peripheral neuropathy. The purpose of this study is to summarize pathogenesis, clinical manifestations, pathological features, diagnosis and treatment of HIV-associated myelopathy. DOI: 10.3969/j.issn.1672-6731.2016.08.004

Imaging of Spinal Cord Injury: Acute Cervical Spinal Cord Injury, Cervical Spondylotic Myelopathy, and Cord Herniation.

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Talekar, Kiran; Poplawski, Michael; Hegde, Rahul; Cox, Mougnyan; Flanders, Adam

2016-10-01

We review the pathophysiology and imaging findings of acute traumatic spinal cord injury (SCI), cervical spondylotic myelopathy, and briefly review the much less common cord herniation as a unique cause of myelopathy. Acute traumatic SCI is devastating to the patient and the costs to society are staggering. There are currently no "cures" for SCI and the only accepted pharmacologic treatment regimen for traumatic SCI is currently being questioned. Evaluation and prognostication of SCI is a demanding area with significant deficiencies, including lack of biomarkers. Accurate classification of SCI is heavily dependent on a good clinical examination, the results of which can vary substantially based upon the patient׳s condition or comorbidities and the skills of the examiner. Moreover, the full extent of a patients׳ neurologic injury may not become apparent for days after injury; by then, therapeutic response may be limited. Although magnetic resonance imaging (MRI) is the best imaging modality for the evaluation of spinal cord parenchyma, conventional MR techniques do not appear to differentiate edema from axonal injury. Recently, it is proposed that in addition to characterizing the anatomic extent of injury, metrics derived from conventional MRI and diffusion tensor imaging, in conjunction with the neurological examination, can serve as a reliable objective biomarker for determination of the extent of neurologic injury and early identification of patients who would benefit from treatment. Cervical spondylosis is a common disorder affecting predominantly the elderly with a potential to narrow the spinal canal and thereby impinge or compress upon the neural elements leading to cervical spondylotic myelopathy and radiculopathy. It is the commonest nontraumatic cause of spinal cord disorder in adults. Imaging plays an important role in grading the severity of spondylosis and detecting cord abnormalities suggesting myelopathy. Copyright © 2016 Elsevier Inc. All rights

Dose effect relationships in cervical and thoracic radiation myelopathies

International Nuclear Information System (INIS)

Holdorff, B.

1980-01-01

The course and prognosis of radiation myelopathies are determined by 3 factors: the segmental (vertical) location of the lesion, the extent of the transverse syndrome (complete or incomplete) and the radiation dose. The median spinal dose in cervical radiation myelopathies with fatal outcome was higher than in survivals with an incomplete transverse syndrome. In thoracic radiation myelopathies a dose difference between complete and incomplete transverse syndromes could be found as well. Incomplete transverse syndromes as submaximum radiation injuries are more suitable for the determination of the spinal tolerance dose than complete transverse syndromes. The lowest threshold could be stated for cases following high-volume irradiation of the lymphatic system. (Auth.)

Tax secretion from peripheral blood mononuclear cells and Tax detection in plasma of patients with human T-lymphotropic virus-type 1-associated myelopathy/tropical spastic paraparesis and asymptomatic carriers.

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Medina, Fernando; Quintremil, Sebastián; Alberti, Carolina; Godoy, Fabián; Pando, María E; Bustamante, Andrés; Barriga, Andrés; Cartier, Luis; Puente, Javier; Tanaka, Yuetsu; Valenzuela, María A; Ramírez, Eugenio

2016-03-01

Human T-lymphotropic virus-type 1 (HTLV-1) is the etiologic agent of the neurologic disease HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Tax viral protein plays a critical role in viral pathogenesis. Previous studies suggested that extracellular Tax might involve cytokine-like extracellular effects. We evaluated Tax secretion in 18 h-ex vivo peripheral blood mononuclear cells (PBMCs) cultures from 15 HAM/TSP patients and 15 asymptomatic carriers. Futhermore, Tax plasma level was evaluated from other 12 HAM/TSP patients and 10 asymptomatic carriers. Proviral load and mRNA encoding Tax were quantified by PCR and real-time RT-PCR, respectively. Intracellular Tax in CD4(+)CD25(+) cells occurred in 100% and 86.7% of HAM/TSP patients and asymptomatic carriers, respectively. Percentage of CD4(+)CD25(+) Tax+, proviral load and mRNA encoding Tax were significantly higher in HAM/TSP patients. Western blot analyses showed higher secretion levels of ubiquitinated Tax in HAM/TSP patients than in asymptomatic carriers. In HTLV-1-infected subjects, Western blot of plasma Tax showed higher levels in HAM/TSP patients than in asymptomatic carriers, whereas no Tax was found in non-infected subjects. Immunoprecipitated plasma Tax resolved on SDS-PAGE gave two major bands of 57 and 48 kDa allowing identification of Tax and Ubiquitin peptides by mass spectrometry. Relative percentage of either CD4(+)CD25(+) Tax+ cells, or Tax protein released from PBMCs, or plasma Tax, correlates neither with tax mRNA nor with proviral load. This fact could be explained by a complex regulation of Tax expression. Tax secreted from PBMCs or present in plasma could potentially become a biomarker to distinguish between HAM/TSP patients and asymptomatic carriers. © 2015 Wiley Periodicals, Inc.

Identification of Human T-lymphotropic Virus Type I (HTLV-I Subtypes Using Restrited Fragment Length Polymorphism in a Cohort of Asymptomatic Carriers and Patients with HTLV-I-associated Myelopathy/tropical Spastic Paraparesis from São Paulo, Brazil

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Segurado Aluisio AC

2002-01-01

Full Text Available Although human T-lymphotropic virus type I (HTLV-I exhibits high genetic stability, as compared to other RNA viruses and particularly to human immunodeficiency virus (HIV, genotypic subtypes of this human retrovirus have been characterized in isolates from diverse geographical areas. These are currently believed not to be associated with different pathogenetic outcomes of infection. The present study aimed at characterizing genotypic subtypes of viral isolates from 70 HTLV-I-infected individuals from São Paulo, Brazil, including 42 asymptomatic carriers and 28 patients with HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP, using restricted fragment length polymorphism (RFLP analysis of long terminal repeat (LTR HTLV-I proviral DNA sequences. Peripheral blood mononuclear cell lysates were amplified by nested polymerase chain reaction (PCR and amplicons submitted to enzymatic digestion using a panel of endonucleases. Among HTLV-I asymptomatic carriers, viral cosmopolitan subtypes A, B, C and E were identified in 73.8%, 7.1%, 7.1% and 12% of tested samples, respectively, whereas among HAM/TSP patients, cosmopolitan A (89.3%, cosmopolitan C (7.1% and cosmopolitan E (3.6% subtypes were detected. HTLV-I subtypes were not statistically significant associated with patients' clinical status. We also conclude that RFLP analysis is a suitable tool for descriptive studies on the molecular epidemiology of HTLV-I infections in our environment.

Tax posttranslational modifications and interaction with calreticulin in MT-2 cells and human peripheral blood mononuclear cells of human T cell lymphotropic virus type-I-associated myelopathy/tropical spastic paraparesis patients.

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Medina, Fernando; Quintremil, Sebastian; Alberti, Carolina; Barriga, Andres; Cartier, Luis; Puente, Javier; Ramírez, Eugenio; Ferreira, Arturo; Tanaka, Yuetsu; Valenzuela, Maria Antonieta

2014-04-01

The human retrovirus human T cell lymphotropic virus type-I (HTLV-1) is the etiologic agent of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Axonal degeneration in HAM/TSP patients occurs without neuron infection, with the secreted viral Tax protein proposed to be involved. We previously found that Tax secreted into the culture medium of MT-2 cells (HTLV-1-infected cell line) produced neurite retraction in neuroblastoma cells differentiated to neuronal type. To assess the relevance of Tax posttranslational modifications on this effect, we addressed the question of whether Tax secreted by MT-2 cells and peripheral blood mononuclear cells (PBMCs) of HTLV-1-infected subjects is modified. The interaction of Tax with calreticulin (CRT) that modulates intracellular Tax localization and secretion has been described. We studied Tax localization and modifications in MT-2 cells and its interaction with CRT. Intracellular Tax in MT-2 cells was assessed by flow cytometry, corresponding mainly to a 71-kDa protein followed by western blot. This protein reported as a chimera with gp21 viral protein-confirmed by mass spectrometry-showed no ubiquitination or SUMOylation. The Tax-CRT interaction was determined by confocal microscopy and coimmunoprecipitation. Extracellular Tax from HAM/TSP PBMCs is ubiquitinated according to western blot, and its interaction with CRT was shown by coimmunoprecipitation. A positive correlation between Tax and CRT secretion was observed in HAM/TSP PBMCs and asymptomatic carriers. For both proteins inhibitors and activators of secretion showed secretion through the endoplasmic reticulum-Golgi complex. Tax, present in PBMC culture medium, produced neurite retraction in differentiated neuroblastoma cells. These results suggest that Tax, whether ubiquitinated or not, is active for neurite retraction.

Understanding spasticity from patients' perspectives over time.

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Bhimani, Rozina H; McAlpine, Cynthia Peden; Henly, Susan J

2012-11-01

  The purpose of this paper was to report patients' understanding and perceptions of personal spasticity experiences over time.   Spasticity is an unpleasant and poorly understood experience associated with upper motor neuron disease.   An original qualitative study was conducted in 2008-2009.   Content analysis was used to extract meaning from the responses of 23 patients to semi-structured interviews during 7 days of acute rehabilitation for neurological diseases associated with spasticity. Findings.  Patients used words reflecting muscle tone and spasms to describe spasticity. Themes reflecting the spasticity experience over time were Ambiguous Experiences, Navigating Symptom Experience, Wounded Self, and Unending Journey.   Spasticity as experienced is complex, involving a wide range of unusual sensations sensitive to stressors in everyday life. Clinical evaluation of spasticity should include patient reports. Knowledge about patient word choice used to describe spasticity can enhance communication with healthcare providers. © 2012 Blackwell Publishing Ltd.

[Evaluation of pendulum testing of spasticity].

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Le Cavorzin, P; Hernot, X; Bartier, O; Carrault, G; Chagneau, F; Gallien, P; Allain, H; Rochcongar, P

2002-11-01

To identify valid measurements of spasticity derived from the pendulum test of the leg in a representative population of spastic patients. Pendulum testing was performed in 15 spastic and 10 matched healthy subjects. The reflex-mediated torque evoked in quadriceps femoris, as well as muscle mechanical parameters (viscosity and elasticity), were calculated using mathematical modelling. Correlation with the two main measures derived from the pendulum test reported in the literature (the Relaxation Index and the area under the curve) was calculated in order to select the most valid. Among mechanical parameters, only viscosity was found to be significantly higher in the spastic group. As expected, the computed integral of the reflex-mediated torque was found to be larger in spastics than in healthy subjects. A significant non-linear (logarithmic) correlation was found between the clinically-assessed muscle spasticity (Ashworth grading) and the computed reflex-mediated torque, emphasising the non-linear behaviour of this scale. Among measurements derived from the pendulum test which are proposed in the literature for routine estimation of spasticity, the Relaxation Index exhibited an unsuitable U-shaped pattern of variation with increasing reflex-mediated torque. On the opposite, the area under the curve revealed a linear regression, which is more convenient for routine estimation of spasticity. The pendulum test of the leg is a simple technique for the assessment of spastic hypertonia. However, the measurement generally used in the literature (the Relaxation Index) exhibits serious limitations, and would benefit to be replaced by more valid measures, such as the area under the goniometric curve, especially for the assessment of therapeutics.

Spasticity

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... Easter Seals March of Dimes National Multiple Sclerosis Society United Cerebral Palsy (UCP) See all related organizations Publications Order NINDS Publications Definition Spasticity is a condition in which there is ...

Surgical Management in Cervical Spondylotic Myelopathy Due to Alkaptonuria.

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Karadag, Ali; Sandal, Evren; Middlebrooks, Erik H; Senoglu, Mehmet

2018-05-01

Ochronotic arthropathy related to alkaptonuria is a rare condition. Cervical spine involvement with myelopathic features has been even more rarely described, particularly related to atlantoaxial instability. As such, little is known about the optimal surgical management in these patients. We described the first case, to our knowledge, of a patient with alkaptonuria and related cervical spondylotic myelopathy from pannus formation at the atlantoaxial joint. We describe our choices in surgical management of this rare condition in a patient with an excellent outcome. Ochronotic cervical spondylotic myelopathy is a rare condition and may require additional considerations in surgical treatment compared to more common causes of cervical spondylotic myelopathy. In our case, we elected for decompression with posterior occipitocervical screw fixation and were able to achieve neurologic recovery with no complications, currently at 2-year follow-up. Copyright © 2018 Elsevier Inc. All rights reserved.

Clinical significance of MRI/{sup 18}F-FDG PET fusion imaging of the spinal cord in patients with cervical compressive myelopathy

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Uchida, Kenzo; Nakajima, Hideaki; Watanabe, Shuji; Yoshida, Ai; Baba, Hisatoshi [University of Fukui, Department of Orthopaedics and Rehabilitation Medicine, Faculty of Medical Sciences, Eiheiji, Fukui (Japan); Okazawa, Hidehiko [University of Fukui, Department of Biomedical Imaging Research Center, Eiheiji, Fukui (Japan); Kimura, Hirohiko [University of Fukui, Departments of Radiology, Faculty of Medical Sciences, Eiheiji, Fukui (Japan); Kudo, Takashi [Nagasaki University, Department of Radioisotope Medicine, Atomic Bomb Disease and Hibakusha Medicine Unit, Atomic Bomb Disease Institute, Nagasaki (Japan)

2012-10-15

{sup 18}F-FDG PET is used to investigate the metabolic activity of neural tissue. MRI is used to visualize morphological changes, but the relationship between intramedullary signal changes and clinical outcome remains controversial. The present study was designed to evaluate the use of 3-D MRI/{sup 18}F-FDG PET fusion imaging for defining intramedullary signal changes on MRI scans and local glucose metabolic rate measured on {sup 18}F-FDG PET scans in relation to clinical outcome and prognosis. We studied 24 patients undergoing decompressive surgery for cervical compressive myelopathy. All patients underwent 3-D MRI and {sup 18}F-FDG PET before surgery. Quantitative analysis of intramedullary signal changes on MRI scans included calculation of the signal intensity ratio (SIR) as the ratio between the increased lesional signal intensity and the signal intensity at the level of the C7/T1 disc. Using an Advantage workstation, the same slices of cervical 3-D MRI and {sup 18}F-FDG PET images were fused. On the fused images, the maximal count of the lesion was adopted as the standardized uptake value (SUV{sub max}). In a similar manner to SIR, the SUV ratio (SUVR) was also calculated. Neurological assessment was conducted using the Japanese Orthopedic Association (JOA) scoring system for cervical myelopathy. The SIR on T1-weighted (T1-W) images, but not SIR on T2-W images, was significantly correlated with preoperative JOA score and postoperative neurological improvement. Lesion SUV{sub max} was significantly correlated with SIR on T1-W images, but not with SIR on T2-W images, and also with postoperative neurological outcome. The SUVR correlated better than SIR on T1-W images and lesion SUV{sub max} with neurological improvement. Longer symptom duration was correlated negatively with SIR on T1-W images, positively with SIR on T2-W images, and negatively with SUV{sub max}. Our results suggest that low-intensity signal on T1-W images, but not on T2-W images, is correlated

CT Chest and pulmonary functional changes in patients with HTLV-associated myelopathy in the Eastern Brazilian Amazon.

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Luiz Fábio Magno Falcão

Full Text Available The aim of this study was to compare computed tomography (CT scans of chest and lung function among patients with Human T-Lymphotropic Virus Type 1 (HTLV with and without HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP. In this cross-sectional study performed between January 2013 and June 2016, we included 48 patients with HAM/TSP (19 women and 11 men and without HAM/TSP (12 women and 6 men. We compared CT findings and lung functions of these groups. Patients who had HAM/TSP had abnormal CT findings (P = 0.000, including more frequent bronchiectasis (P = 0.049, parenchymal bands (P = 0.007, interlobular septal thickening (P = 0.035, and pleural thickening (P = 0.009. In addition, neither patients with HAM/TSP (9/30; 30% nor the controls (0/18; 0% had obstructive or restrictive lung disease (P = 0.009. HTLV diagnosis should be considered in all patients with abnormal CT findings in whom no other cause is apparent. It is important to remember that lung disease increases the rates of morbidity and mortality in developing countries.

Effects of a Nintendo Wii exercise program on spasticity and static standing balance in spastic cerebral palsy.

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Gatica-Rojas, Valeska; Cartes-Velásquez, Ricardo; Méndez-Rebolledo, Guillermo; Guzman-Muñoz, Eduardo; Lizama, L Eduardo Cofré

2017-08-01

This study sought to evaluate the effects of a Nintendo Wii Balance Board (NWBB) intervention on ankle spasticity and static standing balance in young people with spastic cerebral palsy (SCP). Ten children and adolescents (aged 72-204 months) with SCP participated in an exercise program with NWBB. The intervention lasted 6 weeks, 3 sessions per week, 25 minutes for each session. Ankle spasticity was assessed using the Modified Modified Ashworth Scale (MMAS), and static standing balance was quantified using posturographic measures (center-of-pressure [CoP] measures). Pre- and post-intervention measures were compared. Significant decreases of spasticity in the ankle plantar flexor muscles (p balance in young people with SCP.

Spasticity-assessment: a review

DEFF Research Database (Denmark)

Biering-Sørensen, F.; Nielsen, Jens Bo; Klinge, Klaus Peter

2006-01-01

Study design:Review of the literature on the validity and reliability of assessment of spasticity and spasms.Objectives:Evaluate the most frequently used methods for assessment of spasticity and spasms, with particular focus on individuals with spinal cord lesions.Setting:Clinic for Spinal Cord...... Injuries, Rigshospitalet, University Hospital of Copenhagen, and Department of Medical Physiology, University of Copenhagen, Denmark.Methods:The assessment methods are grouped into clinical, biomechanical and electrophysiological, and the correlation between these is evaluated.Results:Clinical methods......: For assessment of spasticity, the Ashworth and the modified Ashworth scales are commonly used. They provide a semiquantitative measure of the resistance to passive movement, but have limited interrater reliability. Guidelines for the testing procedures should be adhered to. Spasm frequency scales seem...

Clinical utility of MR imaging in chronic progressive radiation myelopathy

International Nuclear Information System (INIS)

Melki, P.S.; Halimi, P.; Wibault, P.; Doyon, D.

1990-01-01

This paper defines the diagnostic and prognostic value of MR imaging in chronic progressive radiation myelopathy 9CPRM). In this series, MR imaging showed excellent sensitivity (199%) for the demonstration of radiation-induced lesions of the spinal cord. Fifty percent of the cases showed spinal cord hypertrophy (pseudotumoral, 33%; cystic, 17%) occurring within 8 months of the clinical onset of myelopathy. The remaining 50% showed spinal cord atrophy, which occurred more than 8 months following the onset of myelopathy. These medullary lesions were located at least partially in the radiation field but extended beyond its boundaries in 73% of the cases. MR imaging helped to establish disease prognosis: spinal cord hypertrophy was usually associated with neurologic deterioration and fatal outcome within a mean of 11.5 months; in spinal atrophy, neurologic deficit was often static and survival rates were better

Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India

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Ashok Kumar Kayal

2017-01-01

Full Text Available Background: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21st century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic. Objective: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India. Materials and Methods: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Patients of noncompressive myelopathies who underwent magnetic resonance imaging (MRI of the spine were segregated into two categories: acute-to-subacute myelopathy (ASM and chronic myelopathy (CM. In addition to routine blood tests, chest X-ray, urinalysis, and visual evoked potentials, investigations included MRI of the brain, cerebrospinal fluid analysis, and immunological, infectious, and metabolic profile based on the pattern of involvement. Results: The study had 151 patients (96 ASM and 55 CM with a median age of 35 years and male: female ratio 1.4:1. The causes of ASM were neuromyelitis optica spectrum disorder (23, multiple sclerosis (MS (8, systemic lupus erythematosus (1, Hashimoto's disease (1, postinfectious acute disseminated encephalomyelitis (6, postinfectious myelitis (8, infections (9, spinal cord infarct (5, and electrocution (1. The causes of CM were MS (1, probable or possible sarcoidosis (7, mixed connective tissue disease (1, Hashimoto's disease (2, infections (9, Vitamin B12deficiency (4, folate deficiency (2, hepatic myelopathy (2, radiation (11, and paraneoplastic (1. No etiology could be found in 48 (31.8% patients (34 ASM and 14 CM. In 21/96 (21.9% patients of ASM, acute transverse myelitis was idiopathic based on current diagnostic criteria. Conclusion: Underlying etiology (demyelinating, autoimmune, infectious, vascular, metabolic disorder, or physical agent was found in 68% patients of noncompressive

Physiotherapy for human T-lymphotropic virus 1-associated myelopathy: review of the literature and future perspectives.

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Sá, Katia N; Macêdo, Maíra C; Andrade, Rosana P; Mendes, Selena D; Martins, José V; Baptista, Abrahão F

2015-01-01

Human T-lymphotropic virus 1 (HTLV-1) infection may be associated with damage to the spinal cord - HTLV-associated myelopathy/tropical spastic paraparesis - and other neurological symptoms that compromise everyday life activities. There is no cure for this disease, but recent evidence suggests that physiotherapy may help individuals with the infection, although, as far as we are aware, no systematic review has approached this topic. Therefore, the objective of this review is to address the core problems associated with HTLV-1 infection that can be detected and treated by physiotherapy, present the results of clinical trials, and discuss perspectives on the development of knowledge in this area. Major problems for individuals with HTLV-1 are pain, sensory-motor dysfunction, and urinary symptoms. All of these have high impact on quality of life, and recent clinical trials involving exercises, electrotherapeutic modalities, and massage have shown promising effects. Although not influencing the basic pathologic disturbances, a physiotherapeutic approach seems to be useful to detect specific problems related to body structures, activity, and participation related to movement in HTLV-1 infection, as well as to treat these conditions.

Using Telemedicine to Improve Spasticity Diagnosis Rates

Science.gov (United States)

2018-02-20

Spasticity, Muscle; Muscular Diseases; Musculoskeletal Disease; Muscle Hypertonia; Muscle Spasticity; Neuromuscular Manifestations; Signs and Symptoms; Nervous System Diseases; Neurologic Manifestations

Trends analysis of surgical procedures for cervical degenerative disc disease and myelopathy in patients with tobacco use disorder.

Science.gov (United States)

Grisdela, Phillip; Buser, Zorica; D'Oro, Anthony; Paholpak, Permsak; Liu, John C; Wang, Jeffrey C

2017-09-01

This study defined the incidence and trends of surgeries performed for patients with cervical disc degeneration with and without tobacco use disorder (TUD). This study utilized the Humana Inc. database between 2007 and 2013 to identify patients with cervical disc degeneration with or without myelopathy. International Classification of Diseases, ninth revision (ICD-9) and Current Procedural Terminology (CPT) codes determined the initial diagnosis of disc degeneration, myelopathy status and TUD, whether patients received surgery, and TUD status at surgery. The prevalence of disc degeneration with myelopathy increased by 32.8% between 2007 and 2013, while disc disease with myelopathy and TUD increased by 91.6%. For patients without myelopathy, the prevalence of disc degeneration alone increased by 65.4%, and disc degeneration with myelopathy increased by 148.7%. Of myelopathy patients, 1717 (6.4%) had TUD and 1024 (59.6%) received surgery, compared to 6508 patients without TUD (26.1%). For patients without myelopathy, 11,337 (3.5%) had TUD and 787 (6.9%) underwent surgery, compared to 9716 patients (3%) without TUD. Of surgical patients, 781 (76.3%) with myelopathy and TUD still had a TUD diagnosis at surgery, and 542 (68.9%) of patients without myelopathy still had a TUD diagnosis at surgery. The prevalence of degenerative disc disease and TUD has increased more than disc disease alone. Patients with TUD were more likely to get surgery, and to have surgeries earlier than patients without TUD. Patients with TUD at the time of the diagnosis of their disc degeneration likely still had a TUD diagnosis at the time of surgery.

New insights into the pathophysiology of post-stroke spasticity

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Sheng eLi

2015-04-01

Full Text Available Spasticity is one of many consequences after stroke. It is characterized by a velocity-dependent increase in resistance during passive stretch, resulting from hyperexcitability of the stretch reflex. The underlying mechanism of the hyperexcitable stretch reflex, however, remains poorly understood. Accumulated experimental evidence has supported supraspinal origins of spasticity, likely from an imbalance between descending inhibitory and facilitatory regulation of spinal stretch reflexes secondary to cortical disinhibition after stroke. The excitability of reticulospinal and vestibulospinal tracts has been assessed in stroke survivors with spasticity using non-invasive indirect measures. There are strong experimental findings that support the reticulospinal hyperexcitability as a prominent underlying mechanism of post-stroke spasticity. This mechanism can at least partly account for clinical features associated with spasticity and provide insightful guidance for clinical assessment and management of spasticity. However, the possible role of VST hyperexcitability can not be ruled out from indirect measures. In vivo measure of individual brainstem nuclei in stroke survivors with spasticity using advanced fMRI techniques in the future is probably able to provide direct evidence of pathogenesis of post-stroke spasticity.

New insights into the pathophysiology of post-stroke spasticity.

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Li, Sheng; Francisco, Gerard E

2015-01-01

Spasticity is one of many consequences after stroke. It is characterized by a velocity-dependent increase in resistance during passive stretch, resulting from hyperexcitability of the stretch reflex. The underlying mechanism of the hyperexcitable stretch reflex, however, remains poorly understood. Accumulated experimental evidence has supported supraspinal origins of spasticity, likely from an imbalance between descending inhibitory and facilitatory regulation of spinal stretch reflexes secondary to cortical disinhibition after stroke. The excitability of reticulospinal (RST) and vestibulospinal tracts (VSTs) has been assessed in stroke survivors with spasticity using non-invasive indirect measures. There are strong experimental findings that support the RST hyperexcitability as a prominent underlying mechanism of post-stroke spasticity. This mechanism can at least partly account for clinical features associated with spasticity and provide insightful guidance for clinical assessment and management of spasticity. However, the possible role of VST hyperexcitability cannot be ruled out from indirect measures. In vivo measure of individual brainstem nuclei in stroke survivors with spasticity using advanced fMRI techniques in the future is probably able to provide direct evidence of pathogenesis of post-stroke spasticity.

Clinical evidence for cervical myelopathy due to Chiari malformation and spinal stenosis in a non-randomized group of patients with the diagnosis of fibromyalgia.

Science.gov (United States)

Heffez, Dan S; Ross, Ruth E; Shade-Zeldow, Yvonne; Kostas, Konstantinos; Shah, Sagar; Gottschalk, Robert; Elias, Dean A; Shepard, Alan; Leurgans, Sue E; Moore, Charity G

2004-10-01

. CONCLUSION. Our findings indicate that some patients who carry the diagnosis of fibromyalgia have both signs and symptoms consistent with cervical myelopathy, most likely resulting from spinal cord compression. We recommend detailed neurological evaluation of patients with fibromyalgia in order to exclude cervical myelopathy, a potentially treatable condition.

HTLV-I associated myelopathy with multiple spotty areas in cerebral white matter and brain stem by MRI

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Hara, Yasuo; Takahashi, Mitsuo; Yoshikawa, Hiroo; Yorifuji, Shirou; Tarui, Seiichiro

1988-01-01

A 48-year-old woman was admitted with complaints of urinary incontinence and gait disturbance, both of which had progressed slowly without any sign of remission. Family history was not contributory. Neurologically, extreme spasticity was recoginized in the lower limbs. Babinski sign was positive bilaterally. Flower-like atypical lymphocytes were seen in blood. Positive anti-HTLV-I antibody was confirmed in serum and spinal fluid by western blot. She was diagnosed as having HTLV-I associated myelopathy (HAM). CT reveald calcification in bilateral globus pallidus, and MRI revealed multiple spotty areas in cerebral white matter and brain stem, but no spinal cord lesion was detectable. Electrophysiologically, brain stem auditory evoked potential (BAEP) suggested the presence of bilateral brain stem lesions. Neither median nor posterior tibial nerve somatosensory evoked potentials were evoked, a finding suggesting the existence of spinal cord lesion. In this case, the lesion was not confined to spinal cord, it was also observed in brain stem and cerebral white matter. Such distinct lesions in cerebral white matter and brain stem have not been reported in patients with HAM. It is suggested that HTLV-I is probably associated with cerebral white matter and brain stem.

Treatment of Portosystemic Shunt Myelopathy with a Stent Graft Deployed through a Transjugular Intrahepatic Route

International Nuclear Information System (INIS)

Jain, Deepak; Arora, Ankur; Deka, Pranjal; Mukund, Amar; Bhatnagar, Shorav; Jindal, Deepti; Kumar, Niteen; Pamecha, Viniyendra

2013-01-01

A case of surgically created splenorenal shunt complicated with shunt myelopathy was successfully managed by placement of a stent graft within the splenic vein to close the portosystemic shunt and alleviate myelopathy. To our knowledge, this is the first report of a case of shunt myelopathy in a patient with noncirrhotic portal fibrosis without cirrhosis treated by a novel technique wherein a transjugular intrahepatic route was adopted to deploy the stent graft

Does calf muscle spasticity contribute to postural imbalance? A study in persons with pure hereditary spastic paraparesis

NARCIS (Netherlands)

Niet, M. de; Weerdesteyn, V.G.M.; Bot, S.T. de; Warrenburg, B.P.C. van de; Geurts, A.C.H.

2013-01-01

OBJECTIVES: The contribution of spasticity to postural imbalance in patients with upper motor neuron syndrome is still unclear. This study aimed to evaluate the responses to support-surface perturbations in patients with hereditary spastic paraparesis (HSP). These patients typically suffer from

Radiation myelopathy following transplantation and radiotherapy for non-Hodgkin's lymphoma

International Nuclear Information System (INIS)

Chao, Michael W.T.; Wirth, Andrew; Ryan, Gail; MacManus, Michael; Liew, K.H.

1998-01-01

Background: Combined modality therapy with chemotherapy and radiotherapy has become increasingly popular in the management of solid malignancies. However, unexpected toxicities may arise from their interactions. Methods and Materials: We report the case of a young woman with a large mediastinal non-Hodgkin's lymphoma who underwent high-dose chemotherapy with autologous bone marrow transplantation and involved field radiotherapy, and who developed radiation myelopathy after a latent period of only 3 months. The spinal cord dose did not exceed 40.3 Gy in 22 fractions over 4.5 weeks, which is well within accepted tolerance limits. She had no other identifiable risk factors for radiation myelopathy, suggesting an adverse drug-radiation interaction as the most likely cause of her injury. Results and Conclusions: This represents the first report of radiation myelopathy at accepted safe radiation doses following high-dose chemotherapy with autologous bone marrow transplantation, and we recommend caution in the choice of radiotherapeutic dose in this setting

Spasticity: its physiology and management. Part IV. Current and projected treatment procedures for spasticity.

Science.gov (United States)

Bishop, B

1977-04-01

Today's prescriptions for treating spasticity may include pharmacological, surgical, or physical procedures. All derive their rationale from the classical concepts of decerebrate rigidity and of brain organization as discussed in Part I. This paper describes the advantages and disadvantages of these current treatment procedures and proposes that recent discoveries about the "recovery" capabilities of the central nervous system may influence the means for managing spasticity in the future.

Approaches to radiotherapy in metastatic spinal cord compression.

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Suppl, Morten Hiul

2018-04-01

Metastatic spinal cord compression is caused by the progression of metastatic lesions within the vicinity of the spinal cord. The consequences are very severe with loss of neurological function and severe pain. The standard treatment is surgical intervention followed by radiotherapy or radiotherapy alone. However, the majority of patients are treated with radiotherapy only due to contraindications to surgery and technical inoperability. Stereotactic body radiotherapy is a technology to deliver higher radiation dose to the radiotherapy target with the use of spatial coordinates. This modality has shown positive results in treating lesions in brain and lungs. Hence, it could prove beneficial in metastatic spinal cord compression. We designed and planned a trial to investigate this method in patients with metastatic spinal cord compression. The method was usable but the trial was stopped prematurely due to low accrual that made comparison with surgery impossible. Low accrual is a known problem for trials evaluating new approaches in radiotherapy. Target definition in radiotherapy of metastatic spinal cord compression is defined by patient history, examination and imaging. Functional imaging could provide information to guide target definition with the sparring of normal tissue e.g. spinal cord and hematopoietic tissue of the bone marrow. In future trials this may be used for dose escalation of spinal metastases. The trial showed that PET/MRI was feasible in this group of patients but did not change the radiotherapy target in the included patients. Neurological outcome is similar irrespective of course length and therefore single fraction radiotherapy is recommended for the majority of patients. In-field recurrence is a risk factor of both short and long fractionation schemes and re-irradiation have the potential risk of radiation-induced myelopathy. In a retrospective study of re-irradiation, we investigated the incidence of radiation-induced myelopathy. In our study

Morphology of the cervical spinal cord with myelopathy on computed myelography

International Nuclear Information System (INIS)

Iwasaki, Hiroaki; Asano, Masafumi; Yokota, Hidemaro

1984-01-01

The relationship between morphological changes in the spinal cord shown on computer-assisted myelography and symptoms was investigated in 73 patients with cervical spondylotic myelopathy. Flatness of the spinal cord was seen in many of the patients. Symptoms were likely to be severer with increasing the degree of flatness of the spinal cord. The length of the flat spinal cord will help to select the operative method for cervical spondylotic myelopathy. (Namekawa, K.)

Quantifying spasticity in individual muscles using shear wave elastography

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Sarah F. Eby, BS

2017-06-01

Full Text Available Spasticity is common following stroke; however, high subject variability and unreliable measurement techniques limit research and treatment advances. Our objective was to investigate the use of shear wave elastography (SWE to characterize the spastic reflex in the biceps brachii during passive elbow extension in an individual with spasticity. The patient was a 42-year-old right-hand-dominant male with history of right middle cerebral artery-distribution ischemic infarction causing spastic left hemiparesis. We compared Fugl-Meyer scores (numerical evaluation of motor function, sensation, motion, and pain, Modified Ashworth scores (most commonly used clinical assessment of spasticity, and SWE measures of bilateral biceps brachii during passive elbow extension. We detected a catch that featured markedly increased stiffness of the brachialis muscle during several trials of the contralateral limb, especially at higher extension velocities. SWE was able to detect velocity-related increases in stiffness with extension of the contralateral limb, likely indicative of the spastic reflex. This study offers optimism that SWE can provide a rapid, real-time, quantitative technique that is readily accessible to clinicians for evaluating spasticity.

Asymptomatic HIV positive patient presenting with myelopathy

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Jatin Agrawal

2016-01-01

Full Text Available A wide variety of disorders of diverse pathogenic mechanisms can trigger spinal cord dysfunction in HIV-1-infected patients. The most common such condition is HIV-1-associated myelopathy (HAM which characteristically seen during advanced HIV infection in patients with low CD4 cell counts and previous AIDS-defining diagnoses. Histologically seen in approximately 30% of AIDS patients, but only 10% have clinical symptoms related to the disease. We describe an unusual case of HAM in previously asymptomatic patient with relatively low CD4 cell count (78 cells/mm3. The patient unaware of her seropositive status presented with a clinically slowly progressive myelopathy with difficulty in walking without assistance. We discharged a patient on antiretroviral therapy. We also review the disorders reported to derange spinal cord function in previously asymptomatic HIV-1 infected patients with preserved counts.

Operative Outcomes for Cervical Myelopathy and Radiculopathy

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J. G. Galbraith

2012-01-01

Full Text Available Cervical spondylotic myelopathy and radiculopathy are common disorders which can lead to significant clinical morbidity. Conservative management, such as physical therapy, cervical immobilisation, or anti-inflammatory medications, is the preferred and often only required intervention. Surgical intervention is reserved for those patients who have intractable pain or progressive neurological symptoms. The goals of surgical treatment are decompression of the spinal cord and nerve roots and deformity prevention by maintaining or supplementing spinal stability and alleviating pain. Numerous surgical techniques exist to alleviate symptoms, which are achieved through anterior, posterior, or circumferential approaches. Under most circumstances, one approach will produce optimal results. It is important that the surgical plan is tailored to address each individual's unique clinical circumstance. The objective of this paper is to analyse the major surgical treatment options for cervical myelopathy and radiculopathy focusing on outcomes and complications.

Reproducibility, temporal stability, and functional correlation of diffusion MR measurements within the spinal cord in patients with asymptomatic cervical stenosis or cervical myelopathy.

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Ellingson, Benjamin M; Salamon, Noriko; Woodworth, Davis C; Yokota, Hajime; Holly, Langston T

2018-05-01

OBJECTIVE The purpose of this study was to quantify the reproducibility, temporal stability, and functional correlation of diffusion MR characteristics in the spinal cord in patients with cervical stenosis with or without myelopathy. The association between longitudinal diffusion tensor imaging (DTI) measurements and serial neurological function assessment was explored at both the group and individual level. METHODS Sixty-six nonoperatively treated patients with cervical stenosis were prospectively followed (3 months to > 5 years) using synchronous serial MRI and functional outcome assessment. A total of 183 separate MRI examinations were performed, separated by at least 3 months, and each patient had a minimum of 2 MRI scans (range 2-5 scans). Anatomical and DTI measurements were performed within the spinal cord at the C1-2 region as well as at the area of highest compression. Coefficients of variance (COVs) were compared across measurements in both reference tissue and areas of compression for anatomical measurements, fractional anisotropy (FA), and mean diffusivity (MD). The correlation between diffusion MR measures at the site of compression and evaluations of neurological function assessed using the modified Japanese Orthopaedic Association (mJOA) scale at multiple time points was evaluated. RESULTS The COVs for anatomical measurements (Torg ratio and canal diameter) were between 7% and 10%. The median COV for FA measurements at the site of compression was 9%, and for reference tissue at C1-2 it was 6%. The median COV for MD at the site of compression was approximately 12%, and for reference tissue at C1-2 it was 10%. The FA and MD measurements of C1-2 averaged 0.61 and 0.91 μm 2 /msec, respectively, whereas the FA and MD measurements at the site of compression averaged 0.51 and 1.26 μm 2 /msec, respectively. Both FA (slope = 0.037; R 2 = 0.3281, p slope = -0.074; R 2 = 0.1101, p = 0.0084) were significantly correlated with the mJOA score. The FA decreased

Quadriplegia secondary to cervical spondylotic myelopathy-a rare complication of head and neck surgery.

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Chen, Wei-Fan; Kang, Chung-Jan; Lee, Sai-Cheung; Tsao, Chung-Kan

2013-02-01

Free tissue reconstruction after ablation of head and neck malignancy often requires extensive cervical manipulation, which may exacerbate preexisting cervical spondylosis and result in progression to cervical myelopathy. We present a rare case of postoperative quadriplegia caused by cervical spondylotic myelopathy after head and neck reconstruction. A 63-year-old man without a history of cervical spondylosis underwent resection of a gingivo-buccal squamous cell carcinoma with immediate reconstruction with free fibula osteocutaneous flap. On postoperative day 4, the patient was found to have quadriplegia. MRI demonstrated severe cervical myelopathy. Decompressive laminectomy was performed. The patient underwent an extensive rehabilitation program but only realized moderate improvement. Cervical spondylotic myelopathy is a rare but disastrous complication of head and neck surgery. We hypothesize that it is potentially avoidable with heightened awareness of this disease entity, preoperative identification of patients at risk, and prophylactic interventions Copyright © 2011 Wiley Periodicals, Inc.

The correlation between evoked spinal cord potentials and magnetic resonance imaging before Surgery in cervical spondylotic myelopathy

International Nuclear Information System (INIS)

Akashi, Kosuke; Kanchiku, Tsukasa; Taguchi, Toshihiko; Kato, Yoshihiko; Imajo, Yasuaki; Suzuki, Hidenori

2010-01-01

The purpose of this study is to clarify the correlation between electrophysiological examination and MRI diagnosis. Twenty-four patients with cervical spondylotic myelopathy were examined with magnetic resonance imaging and evoked spinal cord potentials (ESCPs) before surgery. In all the patients, only the intervertebral level was symptomatic, as shown by ESCPs. ESCPs following median nerve stimulation (MN-ESCPs), transcranial electric stimulation (TCE-ESCPs), and spinal cord stimulation (Spinal-ECSPs) were recorded. The patients were grouped into two groups as follows: group A, all ESCPs were abnormal; group B, normal spinal cord stimulation. Spinal cord transverse area and compression ratio (central and 1/4-lateral anteroposterior diameter divided by transverse diameter) were measured on T1-weighted axial imaging, with abnormal ESCPs as indicators of spinal cord morphology. Central and 1/4-lateral compression ratio was significantly lower in group A. Spinal cord morphology of magnetic resonance imaging is useful for functional diagnosis. (author)

Pharmacological management of spasticity in multiple sclerosis

DEFF Research Database (Denmark)

Otero-Romero, Susana; Sastre-Garriga, Jaume; Comi, Giancarlo

2016-01-01

Background and objectives: Treatment of spasticity poses a major challenge given the complex clinical presentation and variable efficacy and safety profiles of available drugs. We present a systematic review of the pharmacological treatment of spasticity in multiple sclerosis (MS) patients. Methods...... improvement is seen with the previous drugs. Nabiximols has a positive effect when used as add-on therapy in patients with poor response and/or tolerance to first-line oral treatments. Despite limited evidence, intrathecal baclofen and intrathecal phenol show a positive effect in severe spasticity...... and suboptimal response to oral drugs. Conclusion: The available studies on spasticity treatment offer some insight to guide clinical practice but are of variable methodological quality. Large, well-designed trials are needed to confirm the effectiveness of antispasticity agents and to produce evidence...

Body-Sensor-Network-Based Spasticity Detection.

Science.gov (United States)

Misgeld, Berno J E; Luken, Markus; Heitzmann, Daniel; Wolf, Sebastian I; Leonhardt, Steffen

2016-05-01

Spasticity is a common disorder of the skeletal muscle with a high incidence in industrialised countries. A quantitative measure of spasticity using body-worn sensors is important in order to assess rehabilitative motor training and to adjust the rehabilitative therapy accordingly. We present a new approach to spasticity detection using the Integrated Posture and Activity Network by Medit Aachen body sensor network (BSN). For this, a new electromyography (EMG) sensor node was developed and employed in human locomotion. Following an analysis of the clinical gait data of patients with unilateral cerebral palsy, a novel algorithm was developed based on the idea to detect coactivation of antagonistic muscle groups as observed in the exaggerated stretch reflex with associated joint rigidity. The algorithm applies a cross-correlation function to the EMG signals of two antagonistically working muscles and subsequent weighting using a Blackman window. The result is a coactivation index which is also weighted by the signal equivalent energy to exclude positive detection of inactive muscles. Our experimental study indicates good performance in the detection of coactive muscles associated with spasticity from clinical data as well as measurements from a BSN in qualitative comparison with the Modified Ashworth Scale as classified by clinical experts. Possible applications of the new algorithm include (but are not limited to) use in robotic sensorimotor therapy to reduce the effect of spasticity.

Two cases of radiation myelopathy with best visualization in MRI

International Nuclear Information System (INIS)

Toribatake, Yasumitsu; Yamada, Yoshio; Chujo, Masahiro; Kadoya, Masumi.

1990-01-01

Radiation myelopathy is considered difficult to diagnose. Two patients underwent magnetic resonance imaging (MRI) for clinically suspected radiation myelopathy. Both of them had previously received intraoperative irradiation of 30 Gy for Borrmann IV type gastric cancer to the lymph nodes surrounding the celiac artery. Several years later, they presented with neurologic symptoms associated with disturbance of the bladder and rectum. T1-weighted imaging revealed high signal intensities in verbetrae at the levels of Th 10 -L 1 for one patient and at the levels of Th 11 -L 1 for the other patient, respectively, which corresponded to the irradiation field. These MRI signs were considered to be fatty degeneration of the bone marrow resulting from irradiation. In one patient, there are ellipsoid areas with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images in the spinal cord, which seemed to be edematous degeneration of the spinal cord attributable to irradiation. MRI appeared promising for the diagnosis of radiation myelopathy. (N.K.)

Surgical results and MRI findings of cervical myelopathy treated with anterior decompression and fusion

International Nuclear Information System (INIS)

Miyazato, Takenari; Teruya, Yoshimitsu; Kinjo, Yukio

1995-01-01

We reviewed 19 patients with cervical myelopathy treated with anterior decompression and fusion. Etiology of cervical myelopathy was cervical disc herniation (CDH) in 13 patients and cervical spondylosis (CSM) in 6. Clinical recovery rate (%) was calculated from preoperative cervical myelopathy score (JOA) and the score at follow-up. Correlation between the clinical recovery rate and MRI findings (area and flatness at the narrowest part of the spinal cord), age at surgery, duration of myelopathy and pre-operative clinical score were analyzed separately in the CDH and CSM groups. Clinical recovery rate averaged 69% in the CDH group and 75% in the CSM group. In the CDH group, average clinical recovery rate in patients younger than 60 years was 80 and in patients over 60 years was 60. There was a significant negative correlation between the clinical recovery rate and age at surgery (p<0.05). No significant correlation was found between the clinical recovery rate and other factors investigated. (author)

Diagnostic Approach to Myelopathies

International Nuclear Information System (INIS)

Granados Sanchez, Ana Maria; Garcia Posada, Lina Maria; Ortega Toscano, Cesar Andres; Lopez Lopez, Alejandra

2011-01-01

Myelopathy is a broad term that refers to spinal cord involvement of multiple etiologies. Spinal cord diseases often have devastating consequences, ranging from quadriplegia and paraplegia to severe sensory deficits due to its confinement in a very small area. Many of these diseases are potentially reversible if they are recognized on time, hence the importance of recognizing the significance of magnetic resonance imaging when approaching a multifactorial disease considered as one of the most critical neurological emergencies, where prognosis depends on an early and accurate diagnosis.

MRI findings in acute idiopathic transverse myelopathy in children

International Nuclear Information System (INIS)

Andronikou, Savvas; Albuquerque-Jonathan, Glenda; Hewlett, Richard; Wilmshurst, Jo

2003-01-01

To describe the clinical and MRI findings in three children with acute idiopathic myelopathy (AIM). Retrospective review of the clinical presentation, MRI findings and outcome of three patients diagnosed with acute idiopathic transverse myelitis. Of note was the swift onset of symptoms in all patients, without any preceding illness or history of vaccination in two of the patients, and the rapid resolution of symptoms on steroid therapy in all the patients. MRI showed T2-weighted hyperintensity and patchy enhancement with gadolinium, but the extensive cord involvement did not correlate with the severity of presentation or outcome. Our findings do not support that MRI evidence alone of diffuse myelopathy is a predictor of poor outcome in childhood AIM. (orig.)

Radiation myelopathy (case presentation)

International Nuclear Information System (INIS)

Sangheli, M.; Lisnic, V.; Plesca, S.; Odainic, O.; Chetrari, L.

2009-01-01

The radiotherapy is one of the most widely spread and commonly used method in treating tumors of different localization. A detailed analysis of benefits and possible side effects along with evaluation of the risk factors allows preventing one of the most difficult complication, and namely the radiation myelopathy. The delayed form of such a disease is distinguished by a pronounced unfavorable prognosis. The presented case provoked the discussion of difficulties in performing differential diagnosis, as well as the importance of determining the localization of vulnerable tissues as regards the target organ exposed to radiotherapy. (authors)

Myelopathy and peripheral neuropathy after X-ray therapy

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Berstad, J.

1986-03-01

Three patients with injury to the spinal cord after X-ray therapy are reported. One patient suffered from a chronic progressive myelopathy, whereas two others were considered to have a peripheral motor neuropathy due to selective damage to the motoneurons. The prognosis of patients with peripheral motor neuropathy is good, in contrast to chronic progressive myelopathy which most often leads to severe disability and death. Characteristically there is a latent interval from months to years between completed radiation therapy and the appearance of neurological symptoms. The mechanism for delayed radiation injury to the cord is at present unknown, but the possibilities of fibrosis, injury to the microcirculation, or direct injury to the nervous tissue are discussed. The importance of a correct diagnosis before further treatment is decided upon is stressed. The most difficult differential diagnosis is intraspinal metastases.

Myelopathy and peripheral neuropathy after X-ray therapy

International Nuclear Information System (INIS)

Berstad, J.

1986-01-01

Three patients with injury to the spinal cord after X-ray therapy are reported. One patient suffered from a chronic progressive myelopathy, whereas two others were considered to have a peripheral motor neuropathy due to selective damage to the motoneurons. The prognosis of patients with peripheral motor neuropathy is good, in contrast to chronic progressive myelopathy which most often leads to severe disability and death. Characteristically there is a latent interval from months to years between completed radiation therapy and the appearance of neurological symptoms. The mechanism for delayed radiation injury to the cord is at present unknown, but the possibilities of fibrosis, injury to the microcirculation, or direct injury to the nervous tissue are discussed. The importance of a correct diagnosis before further treatment is decided upon is stressed. The most difficult differential diagnosis is intraspinal metastases

Surgical Treatment of Cervical Spondylotic Myelopathy Associated Hypertension--A Retrospective Study of 309 Patients.

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Ze-qun Li

Full Text Available Hypertension is the most prevalent cardiovascular disease, and various risk factors are known to be involved in it. Cervical spondylotic myelopathy (CSM is the most common non-traumatic cause of myelopathy, which displays neurological symptoms and may induce systemic symptoms. To date, it is still unknown whether CSM is associated with hypertension, and if so, whether the decompression operations can attenuate CSM associated hypertension. Here, a total of 309 patients with CSM who received anterior or posterior decompression surgery were enrolled as subjects. Blood pressure measurements were performed before and within one week after the surgery. Among the 309 subjects, 144 (46.6% of them exhibited hypertension before surgery, a significantly higher ratio than that of the whole population. One week after surgery, blood pressure of 106 (73.6% patients turned back to normal. Blood pressure of another 37(25.7% patients decreased with different degrees, although still higher than normal. Moreover, it appears that both approaches were effective in improving blood pressure, while the posterior approach was more effective in decreasing systolic blood pressure. We speculate this type of hypertension might result from hyperactivity of sympathetic nervous system as the heart rate of these patients decreased after surgery as well. Collectively, compression of spinal cord in CSM patients might be associated with hypertension, and decompression surgery largely attenuated this type of hypertension. These findings prove CSM to be a potential associated factor of high blood pressure and may shed light on therapies of hypertension in clinics.

Full Body Gait Analysis May Improve Diagnostic Discrimination Between Hereditary Spastic Paraplegia and Spastic Diplegia: A Preliminary Study

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Bonnefoy-Mazure, A.; Turcot, K.; Kaelin, A.; De Coulon, G.; Armand, S.

2013-01-01

Hereditary spastic paraplegia (HSP) and spastic diplegia (SD) patients share a strong clinical resemblance. Thus, HSP patients are frequently misdiagnosed with a mild form of SD. Clinical gait analysis (CGA) has been highlighted as a possible tool to support the differential diagnosis of HSP and SD. Previous analysis has focused on the lower-body…

Short communication an interferon-γ ELISPOT assay with two cytotoxic T cell epitopes derived from HTLV-1 tax region 161-233 discriminates HTLV-1-associated myelopathy/tropical spastic paraparesis patients from asymptomatic HTLV-1 carriers in a Peruvian population.

Science.gov (United States)

Best, Ivan; López, Giovanni; Talledo, Michael; MacNamara, Aidan; Verdonck, Kristien; González, Elsa; Tipismana, Martín; Asquith, Becca; Gotuzzo, Eduardo; Vanham, Guido; Clark, Daniel

2011-11-01

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic and progressive disorder caused by the human T-lymphotropic virus type 1 (HTLV-1). In HTLV-1 infection, a strong cytotoxic T cell (CTL) response is mounted against the immunodominant protein Tax. Previous studies carried out by our group reported that increased IFN-γ enzyme-linked immunospot (ELISPOT) responses against the region spanning amino acids 161 to 233 of the Tax protein were associated with HAM/TSP and increased HTLV-1 proviral load (PVL). An exploratory study was conducted on 16 subjects with HAM/TSP, 13 asymptomatic carriers (AC), and 10 HTLV-1-seronegative controls (SC) to map the HAM/TSP-associated CTL epitopes within Tax region 161-233. The PVL of the infected subjects was determined and the specific CTL response was evaluated with a 6-h incubation IFN-γ ELISPOT assay using peripheral blood mononuclear cells (PBMCs) stimulated with 16 individual overlapping peptides covering the Tax region 161-233. Other proinflammatory and Th1/Th2 cytokines were also quantified in the supernatants by a flow cytometry multiplex assay. In addition, a set of human leukocyte antigen (HLA) class I alleles that bind with high affinity to the CTL epitopes of interest was determined using computational tools. Univariate analyses identified an association between ELISPOT responses to two new CTL epitopes, Tax 173-185 and Tax 181-193, and the presence of HAM/TSP as well as an increased PVL. The HLA-A*6801 allele, which is predicted to bind to the Tax 181-193 peptide, was overpresented in the HAM/TSP patients tested.

Lessons From Recruitment to an Internet-Based Survey for Degenerative Cervical Myelopathy: Comparison of Free and Fee-Based Methods

Science.gov (United States)

2018-01-01

Background Degenerative Cervical Myelopathy (DCM) is a syndrome of subacute cervical spinal cord compression due to spinal degeneration. Although DCM is thought to be common, many fundamental questions such as the natural history and epidemiology of DCM remain unknown. In order to answer these, access to a large cohort of patients with DCM is required. With its unrivalled and efficient reach, the Internet has become an attractive tool for medical research and may overcome these limitations in DCM. The most effective recruitment strategy, however, is unknown. Objective To compare the efficacy of fee-based advertisement with alternative free recruitment strategies to a DCM Internet health survey. Methods An Internet health survey (SurveyMonkey) accessed by a new DCM Internet platform (myelopathy.org) was created. Using multiple survey collectors and the website’s Google Analytics, the efficacy of fee-based recruitment strategies (Google AdWords) and free alternatives (including Facebook, Twitter, and myelopathy.org) were compared. Results Overall, 760 surveys (513 [68%] fully completed) were accessed, 305 (40%) from fee-based strategies and 455 (60%) from free alternatives. Accounting for researcher time, fee-based strategies were more expensive ($7.8 per response compared to $3.8 per response for free alternatives) and identified a less motivated audience (Click-Through-Rate of 5% compared to 57% using free alternatives) but were more time efficient for the researcher (2 minutes per response compared to 16 minutes per response for free methods). Facebook was the most effective free strategy, providing 239 (31%) responses, where a single message to 4 existing communities yielded 133 (18%) responses within 7 days. Conclusions The Internet can efficiently reach large numbers of patients. Free and fee-based recruitment strategies both have merits. Facebook communities are a rich resource for Internet researchers. PMID:29402760

Lessons From Recruitment to an Internet-Based Survey for Degenerative Cervical Myelopathy: Comparison of Free and Fee-Based Methods.

Science.gov (United States)

Davies, Benjamin; Kotter, Mark

2018-02-05

Degenerative Cervical Myelopathy (DCM) is a syndrome of subacute cervical spinal cord compression due to spinal degeneration. Although DCM is thought to be common, many fundamental questions such as the natural history and epidemiology of DCM remain unknown. In order to answer these, access to a large cohort of patients with DCM is required. With its unrivalled and efficient reach, the Internet has become an attractive tool for medical research and may overcome these limitations in DCM. The most effective recruitment strategy, however, is unknown. To compare the efficacy of fee-based advertisement with alternative free recruitment strategies to a DCM Internet health survey. An Internet health survey (SurveyMonkey) accessed by a new DCM Internet platform (myelopathy.org) was created. Using multiple survey collectors and the website's Google Analytics, the efficacy of fee-based recruitment strategies (Google AdWords) and free alternatives (including Facebook, Twitter, and myelopathy.org) were compared. Overall, 760 surveys (513 [68%] fully completed) were accessed, 305 (40%) from fee-based strategies and 455 (60%) from free alternatives. Accounting for researcher time, fee-based strategies were more expensive ($7.8 per response compared to $3.8 per response for free alternatives) and identified a less motivated audience (Click-Through-Rate of 5% compared to 57% using free alternatives) but were more time efficient for the researcher (2 minutes per response compared to 16 minutes per response for free methods). Facebook was the most effective free strategy, providing 239 (31%) responses, where a single message to 4 existing communities yielded 133 (18%) responses within 7 days. The Internet can efficiently reach large numbers of patients. Free and fee-based recruitment strategies both have merits. Facebook communities are a rich resource for Internet researchers. ©Benjamin Davies, Mark Kotter. Originally published in JMIR Research Protocols (http

Intrathecal Baclofen Pump for Spasticity

Science.gov (United States)

2005-01-01

Executive Summary Objective To conduct an evidence-based analysis of the effectiveness and cost-effectiveness of intrathecal baclofen for spasticity. The Technology Spasticity is a motor disorder characterized by tight or stiff muscles that may interfere with voluntary muscle movements and is a problem for many patients with multiple sclerosis (MS), spinal cord injury (SCI), cerebral palsy (CP), and acquired brain injury (ABI).(1). Increased tone and spasm reduces mobility and independence, and interferes with activities of daily living, continence and sleep patterns. Spasticity may also be associated with significant pain or discomfort (e.g., due to poor fit in braces, footwear, or wheelchairs), skin breakdown, contractures, sleep disorders and difficulty in transfer. Goals of treatment are to decrease spasticity in order to improve range of motion, facilitate movement, reduce energy expenditure and reduce risk of contractures. Existing treatments include physical therapy, oral medications, injections of phenol or botulinum toxin, or surgical intervention. Baclofen is the oral drug most frequently prescribed for spasticity in cases of SCI and MS.(1) Baclofen is a muscle relaxant and antispasticity drug. In the brain, baclofen delivered orally has some supraspinal activity that may contribute to clinical side effects. The main adverse effects of oral baclofen include sedation, excessive weakness, dizziness, mental confusion, and somnolence.(2) The incidence of adverse effects is reported to range from 10% to 75%.(2) Ochs et al. estimated that approximately 25-30% of SCI and MS patients fail to respond to oral baclofen.(3;4) Adverse effects appear to be dose-related and may be minimized by initiating treatment at a low dose and gradually titrating upwards.(2) Adverse effects usually appear at doses >60 mg/day.(2) The rate of treatment discontinuation due to intolerable adverse effects has generally been reported to range from 4% to 27%.(2) When baclofen is

Four-fold increase in direct costs of stroke survivors with spasticity compared with stroke survivors without spasticity: the first year after the event.

Science.gov (United States)

Lundström, Erik; Smits, Anja; Borg, Jörgen; Terént, Andreas

2010-02-01

The prevalence of spasticity after first-ever stroke is approximately 20%, but there are no health economic studies on costs associated with spasticity after stroke. The objective of our study was to estimate direct costs of stroke with spasticity for patients surviving up to 1 year after the stroke event in comparison to costs of stroke without spasticity. A representative sample of patients with first-ever stroke hospitalized at Uppsala University Hospital was eligible for our cross-sectional survey. All direct costs during 1 year were identified for each patient, including costs for hospitalization (acute and rehabilitation), primary health care, medication, and costs for municipality services. Swedish currency was converted to Purchasing Power Parities US dollar (PPP$). Median age (interquartile range) was 73 years (18), and the proportion of women was 48%. The majority of the direct costs (78%) was associated with hospitalization, whereas 20% was associated with municipality services during 1 year after a first-ever stroke. Only 1% of all direct costs were related to primary health care and 1% to medication. The level of costs for patients with stroke was correlated with the presence of spasticity as measured with the modified Ashworth scale (r(s)=0.524) and with the degree of disability as measured with modified Rankin Scale (r(s)=0.624). The mean (median, interquartile range) direct cost for stroke patients with spasticity was PPP$ 84,195 (72,116, 53,707) compared with PPP$ 21,842 (12,385, 17,484) for patients with stroke without spasticity (Pstroke survivors are 4 times higher than direct costs for patients with stroke without spasticity during the first year after the event.

Cellular Pathways of Hereditary Spastic Paraplegia*

OpenAIRE

Blackstone, Craig

2012-01-01

Human voluntary movement is controlled by the pyramidal motor system, a long CNS pathway comprising corticospinal and lower motor neurons. Hereditary spastic paraplegias (HSPs) are a large, genetically diverse group of inherited neurologic disorders characterized by a length-dependent distal axonopathy of the corticospinal tracts, resulting in lower limb spasticity and weakness. A range of studies are converging on alterations in the shaping of organelles, particularly the endoplasmic reticul...

Radiation myelopathy. Analysis of the clinical picture. Die Strahlenmyelopathie. Klinische Analyse des Krankheitsbildes

Energy Technology Data Exchange (ETDEWEB)

Berlit, P

1987-01-01

After a review of the world literature, the case histories of 43 patients with radiation myelopathy are analyzed. In 1 patient there was a radiation injury of the medulla oblongata, in 2, cervical, in 28, thoracic, and in 12, lumbosacral. In the medulla oblongata lesion an alternans syndrome resulted. The patients with cervical and thoracic radiation myelopathies presented with a Brown-Sequard syndrome, a spinalis anterior syndrome or a transversal syndrome with pyramidal and spinothalamic tract involvement as the most prominent signs. For this group the term 'pyramidal-spinothalamic radiation myelopathy' is proposed. In lumbosacral radiation lesions a pure anterior horn syndrome may lead to spinothalamic tract involvement and the development of a cauda conus syndrome. The clinical presentation of these cases suggests that the location of the radiation lesion is most likely the region of the conus medullaris. The most frequent initial symptom was dysesthesia; the patients complained of burning pain or a feeling of coldness. Usually the neurological deficits were progressive, in pyramidal-spinothalamic radiation myelopathy over 12 months in average, in lumbosacral radiation lesions up to 10 years. The latent period between the finish of radiation therapy and the first neurological signs was 8 months (median) in cervical and thoracic myelopathy and 33 months in lumbosacral lesions. For the entire group of 43 patients there was an inverse relationship between the radiation dose (ret) and the latent period. A positive relation could be demonstrated between the age of patients at the time of radiation therapy and the latent period. Patients simultaneously receiving cytostatic drugs presented after a longer latent period than the remaining group. With 17 figs.

Balneotherapy in treatment of spastic upper limb after stroke.

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Erceg-Rukavina, Tatjana; Stefanovski, Mihajlo

2015-02-01

After stroke, spasticity is often the main problem that prevents functional recovery. Pain occurs in up to 70% of patients during the first year post-stroke. A total of 70 patients (30 female and 45 male) mean age (65.67) participated in prospective, controlled study. ischaemic stroke, developed spasticity of upper limb, post-stroke interval balneotherapy and inability to follow commands. Experimental group (Ex) (n=35) was treated with sulphurous baths (31°-33°C) and controlled group (Co) with taped water baths, during 21 days. All patients were additionally treated with kinesitherapy and cryotherapy. The outcome was evaluated using Modified Ashworth scale for spasticity and VAS scale for pain. The significance value was sat at pbalneotherapy with sulphurous bath on spasticity and pain in affected upper limb. Reduction in tone of affected upper limb muscles was significant in Ex group (pbalneotherapy with sulphurous water reduces spasticity and pain significantly and can help in treatment of post-stroke patients.

Reevaluation of the Pavlov ratio in patients with cervical myelopathy.

Science.gov (United States)

Suk, Kyung-Soo; Kim, Ki-Tack; Lee, Jung-Hee; Lee, Sang-Hun; Kim, Jin-Soo; Kim, Jin-Young

2009-03-01

This study was designed to reevaluate the effectiveness of the Pavlov ratio in patients with cervical myelopathy. We studied 107 patients who underwent open door laminoplasty for the treatment of cervical myelopathy between the C3 to C7 levels. We determined the Pavlov ratio on preoperative and postoperative cervical spine lateral radiographs, the vertebral body-to-canal ratio on sagittal reconstruction CT scans, and the vertebral body-to-cerebrospinal fluid (CSF) column ratio on T2-weighted sagittal MR images from C3 to C6. The severity of myelopathy was determined using the JOA score on both preoperative and postoperative images. The recovery rate was also calculated. The Pavlov ratio in plain radiographs from patients with myelopathy was compared with the ratio of the vertebral body to the spinal canal on CT and MRI. The average Pavlov ratio between C3 and C6 ranged from 0.71 to 0.76. On CT scan, the average vertebral body-to-canal ratio between C3 and C6 ranged from 0.62 to 0.66. On MRI, the vertebral body-to-CSF column ratio between C3 and C6 ranged between 0.53 and 0.57. A positive correlation was noted between the Pavlov ratio and the vertebral body-to-canal ratio on sagittal-reconstruction CT (correlation coefficient = 0.497-0.627, p = 0.000) and between the Pavlov ratio and the vertebral body-to-CSF column ratio on MRI (correlation coefficient = 0.511-0.649, p = 0.000). We demonstrated a good correlation between the Pavlov ratio and both the vertebral body-to-canal ratio on CT and the vertebral body-to-CSF column ratio on MRI. Therefore, the Pavlov ratio can be relied upon to predict narrowing of the cervical spinal canal in the sagittal plane.

Comparison of Anterior and Posterior Surgery for Degenerative Cervical Myelopathy: An MRI-Based Propensity-Score-Matched Analysis Using Data from the Prospective Multicenter AOSpine CSM North America and International Studies.

Science.gov (United States)

Kato, So; Nouri, Aria; Wu, Dongjin; Nori, Satoshi; Tetreault, Lindsay; Fehlings, Michael G

2017-06-21

Surgeons often choose between 2 different approaches (anterior and posterior) for surgical treatment of degenerative cervical myelopathy on the basis of imaging features of spinal cord compression, the number of levels affected, and the spinal alignment. However, there is a lack of consensus on which approach is preferable. The objective of the present study was to use magnetic resonance imaging (MRI)-based propensity-score-matched analysis to compare postoperative outcomes between the anterior and posterior surgical approaches for degenerative cervical myelopathy. A total of 757 patients were enrolled in 2 prospective multicenter AOSpine studies, which involved 26 international sites. Preoperative MRIs were reviewed to characterize the causes of the cord compression, including single-level disc disease, multilevel disc disease, ossification of the posterior longitudinal ligament, enlargement of the ligamentum flavum, vertebral subluxation/spondylolisthesis, congenital fusion, number of compressed levels, or kyphosis. The propensity to choose anterior decompression was calculated using demographic data, preoperative MRI findings, and the modified Japanese Orthopaedic Association (mJOA) scores in a logistic regression model. We then performed 1-to-1 matching of patients who had received anterior decompression with those who had the same propensity score but had received posterior decompression to compare 2-year postoperative outcomes and 30-day perioperative complication rates between the 2 groups after adjustment for background characteristics. A total of 435 cases were included in the propensity score calculation, and 1-to-1 matching resulted in 80 pairs of anterior and posterior surgical cases; 99% of these matched patients had multilevel compression. The anterior and posterior groups did not differ significantly in terms of the postoperative mJOA score (15.1 versus 15.3, p = 0.53), Neck Disability Index (20.5 versus 24.1, p = 0.44), or Short Form-36 (SF-36

[Cervical myelopathy from an aspect of a neurological surgeon].

Science.gov (United States)

Koyama, T

1994-12-01

More than 65 per cent of intramedurally tumors and intradural extramedurally tumors were located in cervical region. They enact a cause of cervical myelopathy. A special attention should be paid to solitaly intramedurally vascular malformations, when the patients show an acute progressive myelopathy. In very rare cases of syringomyelia, intramedurally tumors such as hemangioblastoma or ependymoma could be found, so that enhanced MRI or angiography should be done if necessary. In cases of a herniated disc, cervical spondylosis and OPLL, the distance between articulo-pillar line and spinolaminal line must be measured. If the distance of both lines is near or the same, that means narrow cervical canal. In rare cases the form of the vertebral body is round. This is a rare cause of a type of narrow canal.

Spasticity and Its Contribution to Hypertonia in Cerebral Palsy

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Lynn Bar-On

2015-01-01

Full Text Available Spasticity is considered an important neural contributor to muscle hypertonia in children with cerebral palsy (CP. It is most often treated with antispasticity medication, such as Botulinum Toxin-A. However, treatment response is highly variable. Part of this variability may be due to the inability of clinical tests to differentiate between the neural (e.g., spasticity and nonneural (e.g., soft tissue properties contributions to hypertonia, leading to the terms “spasticity” and “hypertonia” often being used interchangeably. Recent advancements in instrumented spasticity assessments offer objective measurement methods for distinction and quantification of hypertonia components. These methods can be applied in clinical settings and their results used to fine-tune and improve treatment. We reviewed current advancements and new insights with respect to quantifying spasticity and its contribution to muscle hypertonia in children with CP. First, we revisit what is known about spasticity in children with CP, including the various definitions and its pathophysiology. Second, we summarize the state of the art on instrumented spasticity assessment in CP and review the parameters developed to quantify the neural and nonneural components of hypertonia. Lastly, the impact these quantitative parameters have on clinical decision-making is considered and recommendations for future clinical and research investigations are discussed.

Radiation myelopathy after irradiation of a larynx carcinoma

International Nuclear Information System (INIS)

Buchholz, A.; Daehn, I.

1980-01-01

The morphological characteristics and clinical phenomena of radiation myelopathy after X-irradiation of larynx carcinoma are demonstrated. In spite of constant improvement of radiotherapy the occurrence of a radiation damage of the central nervous system must be expected and included into considerations concerning differential diagnosis

Mielopatia torácica por calcificação do ligamento amarelo cursando com hiperproteinorraquia e resposta à corticoterapia: relato de caso Thoracic myelopathy due to calcification of the ligamentum flavum with hyperproteinorachia and responsive to steroid therapy: case report

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Flávia Yuri Shiguematsu

2012-06-01

Full Text Available Calcificação e ossificação do ligamento amarelo ou do ligamento longitudinal posterior são causas de mielopatia compressiva, mais frequentes nos níveis torácicos inferiores e bastante raras em populações ocidentais. A descompressão cirúrgica é a única terapia proposta, mas a doença costuma ser progressiva e sua recorrência após a cirurgia não é incomum. Mediadores inflamatórios podem ter algum papel na progressão da mielopatia compressiva, mas não se tem notícia de qualquer proposta de abordagem terapêutica envolvendo agentes anti-inflamatórios. Neste contexto, relatamos um caso de mielopatia compressiva por calcificação do ligamento amarelo em que se observou hiperproteinorraquia e resposta à corticoterapia. Tais informações são inéditas e podem fornecer novas ideias para a compreensão da doença.Calcification and ossification of the ligamentum flavum or of the posterior longitudinal ligament are causes of compressive myelopathy, more frequent in the lower thoracic levels, and extremely rare in Western populations. Surgical decompression is the only therapy, but the disease is usually progressive, and its recurrence after surgery is common. Inflammatory mediators might play a role in the progression of compressive myelopathy, but, to our knowledge, the therapeutic approach involving anti-inflammatory agents has never been tried before. We report a case of compressive myelopathy due to calcification of the ligamentum flavum, in which hyperproteinorachia and response to steroid therapy have been observed. Those data have not been published before and might provide new ideas for the disease understanding.

MR findings of subacute necrotizing myelopathy: case report

International Nuclear Information System (INIS)

Na, Dong Gyu; Chang, Kee Hyun; Han, Moon Hee; Kim, Hyun Jip; Kim, Chong Jai; Chi, Je G.

1994-01-01

Subacute necrotizing myelopathy(SNM) is a rare non-tumorous disease of spinal cord characterized by subacute clinical course of progressive neurological deterioration. We report MR findings of a patient with pathologically proved SNM. 1 case of pathologically proved subacute necrotizing myelopathy. The patients was a 56-year-old man with progressive motor weakness and sensory loss of the lower extremities, and urinary and fecal incontinence for 11 months. Spine MRI revealed diffuse enlargement of the thoracic spinal cord from T2 to T7 level. Signal intensity of the expanded spinal cord was isointense relative to normal cord on T1-weighted image and hyperintense on proton-density and T2-weighted images. On contrast enhanced T1-weighted image, there was diffuse homogeneous enhancement in the expanded cord lesion. MR demonstration of stable persistence of spinal cord lesion or atrophy over months or years with clinical findings of gradual progressive neurologic deterioration may be helpful in the diagnosis of SNM

Osteochondromatosis of the cervical spine causing compressive myelopathy in a dog

International Nuclear Information System (INIS)

Caporn, T.M.; Read, R.A.

1996-01-01

A 10-month-old Alaskan malamute presented with cervical pain and hindlimb proprioceptive deficits. Plain and myelographic radiographic studies of the cervical spine demonstrated extradural compression of the spinal cord at the level of C7 and C5. Computed tomography assisted presurgical characterisation of the lesions as osteochondromatosis. Laminectomy permitted successful removal of the lesions

Procedure-Oriented Torsional Anatomy of the Hand for Spasticity Injection.

Science.gov (United States)

John, Joslyn; Cianca, John; Chiou-Tan, Faye; Pandit, Sindhu; Furr-Stimming, Erin; Taber, Katherine H

To provide musculoskeletal ultrasound (MSKUS) images of hand anatomy in the position of hemiparetic flexion as a reference for spasticity injections. After a stroke, spasticity can result in anatomic distortion of the hand. Spasticity may require treatment with botulinum toxin or phenol injections. Anatomic distortion may decrease the accuracy of injections. Standard anatomic references are of limited utility because they are not in this spastic hemiparetic position. There presently is no anatomic reference in the literature for these spastic postures. This study is part three of a series examining torsional anatomy of the body. Ultrasound (US) images were obtained in a healthy subject. The muscles examined included the lumbricals and the flexor pollicis brevis. A marker dot was placed at each dorsal and palmar anatomic injection site for these muscles. The US probe was placed on these dots to obtain a cross-sectional view. A pair of US images was recorded with and without power Doppler imaging: the first in anatomic neutral and second in hemiparetic spastic positions. In addition, a video recording of the movement of the muscles during this rotation was made at each site. On the palmar view, the lumbricals rotated medially. On dorsal view, the lumbricals can be seen deep to the dorsal interossei muscles, with spastic position, and they become difficult to identify. The flexor pollicis brevis (FPB) muscle contracts with torsion, making abductor pollicis brevis (APB) predominately in view. The anatomic location of the lumbrical muscles makes them difficult to inject even with ultrasound guidance. However, recognizing the nearby digital vasculature allows for improved identification of the musculature for injection purposes. The FPB muscle also can be identified by its adjacent radial artery lateral to the flexor pollicus longus tendon. Normal anatomy of hand can become distorted in spastic hemiparesis. Diagnostic ultrasound is able to discern these anatomic

Case of sensory ataxic ganglionopathy-myelopathy in copper deficiency.

Science.gov (United States)

Zara, Gabriella; Grassivaro, Francesca; Brocadello, Filippo; Manara, Renzo; Pesenti, Francesco Francini

2009-02-15

Spinal cord involvement associated with severe copper deficiency has been reported in the last 8 years. Copper deficiency may produce an ataxic myelopathy. Clinical and neuroimaging findings are similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. Macrocytic, normocytic and microcytic anemia, leukopenia and, in severe cases, pancytopenia are well known hematologic manifestations. The most patients with copper deficiency myelopathy had unrecognized carency. Some authors suggested that early recognition and copper supplementation may prevent neurologic deterioration but clinical findings do not improve. We present a patient with copper deficiency, dorsal root ganglions and cervical dorsal columns involvement. Clinical status and neuroimaging improved after copper replacement therapy. Sensory neurons of dorsal root ganglia may be the most sensitive nervous pathway. In this case the early copper treatment allowed to improve neurologic lesions and to prevent further involvements.

Repetitive Transcranial Magnetic Stimulation in Patients with Hereditary Spastic Paraplegia

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Mehmet Ağırman

2011-06-01

Full Text Available Hereditary spastic paraplegia (HSPP is a heterogeneous genetic disease characterized by progressive spasticity of lower extremities. Spasticity is a major cause of long-term disability in HSPP and significantly affects the functional life of patients. Repetitive transcranial magnetic stimulation (rTMS is widely used in diagnosis and treatment of many neurological and psychiatric diseases. Although the positive impacts of rTMS for spasticity have been reported, no study has been found on HSPP. We present two HSPP patients treated with low frequency rTMS (20 minutes at a frequency of 1 Hz (1200 pulses, for a period of 10 treatment sessions.

Repetitive Transcranial Magnetic Stimulation in Patients with Hereditary Spastic Paraplegia

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Mehmet Ağırman

2011-06-01

Full Text Available Hereditary spastic paraplegia (HSPP is a heterogeneous genetic disease characterized by progressive spasticity of lower extremities. Spasticity is a major cause of long-term disability in HSPP and significantly affects the functional life of patients. Repetitive transcranial magnetic stimulation (rTMS is widely used in diagnosis and treatment of many neurological and psychiatric diseases. Although the positive impacts of rTMS for spasticity have been reported, no study has been found on HSPP. We present two HSPP patients treated with low frequency rTMS (20 minutes at a frequency of 1 Hz (1200 pulses, for a period of 10 treatment sessions

Hereditary spastic paraplegia.

Science.gov (United States)

Blackstone, Craig

2018-01-01

The hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurologic disorders with the common feature of prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. The HSPs exist not only in "pure" forms but also in "complex" forms that are associated with additional neurologic and extraneurologic features. The HSPs are among the most genetically diverse neurologic disorders, with well over 70 distinct genetic loci, for which about 60 mutated genes have already been identified. Numerous studies elucidating the molecular pathogenesis underlying HSPs have highlighted the importance of basic cellular functions - especially membrane trafficking, mitochondrial function, organelle shaping and biogenesis, axon transport, and lipid/cholesterol metabolism - in axon development and maintenance. An encouragingly small number of converging cellular pathogenic themes have been identified for the most common HSPs, and some of these pathways present compelling targets for future therapies. Copyright © 2018 Elsevier B.V. All rights reserved.

Maple Syrup Urine Disease Complicated with Kyphoscoliosis and Myelopathy

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Jia-Woei Hou

2016-10-01

Full Text Available Maple syrup urine disease (MSUD is an autosomal recessive aminoacidopathy secondary to an enzyme defect in the catabolic pathway of the branched-chain amino acids (BCAAs: leucine, isoleucine, and valine. Accumulation of their corresponding keto-acids leads to encephalopathy if not treated in time. A newborn male patient was suspected to have MSUD after tandem mass study when he presented symptoms and signs suggestive neonatal sepsis, anemia, and diarrhea. Food restriction of BCAAs was started; however, acrodermatitis enteropathica-like skin eruptions occurred at age 2 months. The skin rashes resolved after adding BCAAs and adjusting the infant formula. At age 7 months, he suffered from recurrent skin lesions, zinc deficiency, osteoporosis, and kyphosis of the thoracic spine with acute angulation over the T11-T12 level associated with spinal compression and myelopathy. After supplementation of zinc products and pamidronate, skin lesions and osteopenia improved gradually. Direct sequencing of the DBT gene showed a compound heterozygous mutation [4.7 kb deletion and c.650-651insT (L217F or L217fsX223]. It is unusual that neurodegeneration still developed in this patient despite diet restriction. Additionally, brain and spinal magnetic resonance imaging, bone mineral density study, and monitoring of zinc status are suggested in MSUD patients.

Radiation myelopathy in nasopharyngeal carcinoma

International Nuclear Information System (INIS)

Enomoto, Hiroyuki; Tsukuda, Mamoru; Kono, Hidehiro; Omata, Toshiyuki; Mochimachi, Izumi; Hasegawa, Osamu

1995-01-01

A 36-year-old woman with nasopharyngeal carcinoma developed radiation myelopathy. She was treated with radiotherapy, a total dose of about 120 Gy to the cervical spinal cord at the C1 level, chemotherapy and immunotherapy. Nine months after the final radiation therapy, she complained of a paresthesia in the distal area of the right leg. Neurological examination revealed incomplete left Brown-Sequard syndrome below the level of C5. Neurological symptoms had been progressive. The spinal MRI showed an abnormal intramedullary high intensity area enhanced by Gd-DTPA. (author)

Radiation myelopathy in nasopharyngeal carcinoma

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Enomoto, Hiroyuki; Tsukuda, Mamoru; Kono, Hidehiro; Omata, Toshiyuki; Mochimachi, Izumi; Hasegawa, Osamu [Yokohama City Univ. (Japan). School of Medicine

1995-08-01

A 36-year-old woman with nasopharyngeal carcinoma developed radiation myelopathy. She was treated with radiotherapy, a total dose of about 120 Gy to the cervical spinal cord at the C1 level, chemotherapy and immunotherapy. Nine months after the final radiation therapy, she complained of a paresthesia in the distal area of the right leg. Neurological examination revealed incomplete left Brown-Sequard syndrome below the level of C5. Neurological symptoms had been progressive. The spinal MRI showed an abnormal intramedullary high intensity area enhanced by Gd-DTPA. (author).

CERVICAL SPONDYLOTIC MYELOPATHY WITH FUNCTIONAL ...

African Journals Online (AJOL)

Thirteen of them complained about neck pain-8, brachialgia-3 or paresthesia-2. All patients presented various progressive spinal cord lesions (tetraparesis: 13, paraparesis: 4, tetraplegia: 1). They had also spasticity. Anterior approach were performed in the 18 cases. The average follow-up was 6.9 years. Results: All ...

PROMIS Physical Function Correlation With NDI and mJOA in the Surgical Cervical Myelopathy Patient Population.

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Owen, Robert J; Zebala, Lukas P; Peters, Colleen; McAnany, Steven

2018-04-15

Retrospective review. To determine the correlation of Patient-Reported Outcomes Measurement Information System (PROMIS) physical function with Neck Disability Index (NDI) and Modified Japanese Orthopedic Association (mJOA) scores in the surgical cervical myelopathy patient population. Outcome measures such as NDI and mJOA are essential for analyzing treatments for cervical myelopathy. Administrative burdens impose limits on completion of these measures. The PROMIS group developed an outcome measure to improve reporting of patient symptoms and function and to reduce administrative burden. Despite early success, NDI and mJOA have not been compared with PROMIS in patients with cervical myelopathy. This study determines the correlation of NDI and mJOA with PROMIS in surgical patients with cervical myelopathy. A total of 60 patients with cervical myelopathy undergoing surgery were included. PROMIS, NDI, and mJOA were collected preoperatively, and in the first 6 months postoperatively. Correlations between NDI, mJOA, and PROMIS were quantified using Pearson correlation coefficients. Students t tests were used to test significance. All 60 (100%) of patients completed preoperative questionnaires. Fifty-five (92%) of patients completed initial follow-up questionnaires within the first 6 months. PROMIS physical function and NDI demonstrated a strong negative correlation at baseline and in initial follow-up (R = -0.69, -0.76). PROMIS and mJOA demonstrated a strong positive correlation at baseline and in initial follow-up (R = 0.61, 0.72). PROMIS physical function has a strong negative correlation with NDI and a strong positive correlation with mJOA at baseline and in the early postoperative course in patients undergoing surgery for cervical myelopathy. Surgeons may factor these outcomes into the delivery and interpretation of patient-reported outcome measures in this population. Use of PROMIS may improve completion of outcome measures in the office and reduce

Cytokine expression of macrophages in HIV-1-associated vacuolar myelopathy.

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Tyor, W R; Glass, J D; Baumrind, N; McArthur, J C; Griffin, J W; Becker, P S; Griffin, D E

1993-05-01

Macrophages are frequently present within the periaxonal and intramyelinic vacuoles that are located primarily in the posterior and lateral funiculi of the thoracic spinal cord in HIV-associated vacuolar myelopathy. But the role of these macrophages in the formation of the vacuoles is unclear. One hypothesis is that cytokines, such as interleukin-1 (IL-1) and tumor necrosis factor (TNF)-alpha, are produced locally by macrophages and have toxic effects on myelin or oligodendrocytes. The resulting myelin damage eventually culminates in the removal of myelin by macrophages and vacuole formation. We studied thoracic spinal cord specimens taken at autopsy from HIV-positive (+) and HIV-negative individuals. The predominant mononuclear cells present in HIV+ spinal cords are macrophages. They are located primarily in the posterior and lateral funiculi regardless of the presence or absence of vacuolar myelopathy. Macrophages and microglia are more frequent in HIV+ than HIV-negative individuals and these cells frequently stain for class I and class II antigens, IL-1, and TNF-alpha. Activated macrophages positive for IL-1 and TNF-alpha are great increased in the posterior and lateral funiculi of HIV+ individuals with and without vacuolar myelopathy, suggesting they are present prior to the development of vacuoles. Cytokines, such as TNF-alpha, may be toxic for myelin or oligodendrocytes, leading to myelin damage and removal by macrophages and vacuole formation.

Management of spastic hand by selective peripheral neurotomies ...

African Journals Online (AJOL)

Introduction: Selective peripheral neurotomies (SPN) are proposed when spasticity is focalized on muscles that are under the control of a single or few peripheral nerves. Objective: This study was done to evaluate the functional results of SPN of median and ulnar nerves in 10 patients who had spastic hyperflexion of the ...

Recurrent acute transverse myelopathy: association with antiphospholipid antibody syndrome.

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Shaharao, Vijaya; Bartakke, Sandip; Muranjan, Mamta N; Bavdekar, Manisha S; Bavdekar, Sandeep B; Udani, Vrajesh P

2004-06-01

A seven-year-old boy presented with a second episode of acute transverse myelopathy. The first episode had responded dramatically to methylprednisolone. The manifestations of the second episode did not respond to methylprednisolone or IVIG. He showed persistently raised levels of antiphospholipid antibodies in the serum. Primary conditions like collagen vascular diseases, malignancy, exposure to drugs and HIV infection, which are known to be associated with the raised titers of these antibodies were ruled out clinically and by investigations. Recurrent transverse myelopathy is a rare event in childhood and reports of its association with Antiphospholipid Antibody Syndrome (APLAS) are scanty. The etiological role for these antibodies remains to be established. However, once the diagnosis is established, it may be prudent to treat the condition with agents and procedures to bring about a decrease in their titers. Long-term therapy to prevent thromboembolic complications of APLAS may also be instituted.

Association between spasticity and the level of motor function with quality of life in community dwelling Iranian young adults with spastic cerebral palsy

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Nasrin Salehi Dehno

2012-11-01

Full Text Available  Abstract Background: Consequences of cerebral palsy in adulthood can affect physical, psychological capabilities and quality of life. The purpose of this study was to investigate the relationship between quality of life with spasticity and level of motor function in Iranian young adults with spastic cerebral palsy who were community dweller. Methods: In an analytical cross sectional study, 77 participants with spastic cerebral palsy (44 women، 33 men with age range of 20 to 40 years; (mean age 26.19±5 yr took part in this study. They were enrolled from three Raad Rehabilitation Goodwill complexes in Tehran and Karaj cities. All subjects were recruited through convenient sampling. Severity of Spasticity for knee flexors was measured with Modified Tardieu Scale. In addition, the level of motor function, and quality of life were assessed respectively through Gross Motor Function Classification System and World Health Organization Quality of life questionnaire (WHOQOL- BREF. To analyze data, Pearson and spearman correlation coefficient was used. Results: No correlation found between quality of life with knee flexor muscles spasticity and level of motor function (p> 0.05. Conclusion: Quality of life as a multi dimensional concept has been impacted by many factors such as physical status, environmental issues and culture. Possibly, severity of spasticity and level of function have a less pronounced effect on quality of life in community dwelling adults with cerebral palsy. 

Laminoplasty and laminectomy for cervical sponydylotic myelopathy: a systematic review

DEFF Research Database (Denmark)

Bartels, R.H.M.A.; van Tulder, M.W.; Moojen, W.A.

2015-01-01

BACKGROUND: Cervical spondylotic myelopathy is frequently encountered in neurosurgical practice. The posterior surgical approach includes laminectomy and laminoplasty.OBJECTIVE: To perform a systematic review evaluating the effectiveness of posterior laminectomy compared with posterior laminoplasty...

Impact of Spasticity on Balance Control during Quiet Standing in Persons after Stroke

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Reza Rahimzadeh Khiabani

2017-01-01

Full Text Available Background. Balance impairments, falls, and spasticity are common after stroke, but the effect of spasticity on balance control after stroke is not well understood. Methods. In this cross-sectional study, twenty-seven participants with stroke were divided into two groups, based on ankle plantar flexor spasticity level. Fifteen individuals with high spasticity (Modified Ashworth Scale (MAS score of ≥2 and 12 individuals with low spasticity (MAS score <2 completed quiet standing trials with eyes open and closed conditions. Balance control measures included centre of pressure (COP root mean square (RMS, COP velocity, and COP mean power frequency (MPF in anterior-posterior and mediolateral (ML directions. Trunk sway was estimated using a wearable inertial measurement unit to measure trunk angle, trunk velocity, and trunk velocity frequency amplitude in pitch and roll directions. Results. The high spasticity group demonstrated greater ML COP velocity, trunk roll velocity, trunk roll velocity frequency amplitude at 3.7 Hz, and trunk roll velocity frequency amplitude at 4.9 Hz, particularly in the eyes closed condition (spasticity by vision interaction. ML COP MPF was greater in the high spasticity group. Conclusion. Individuals with high spasticity after stroke demonstrated greater impairment of balance control in the frontal plane, which was exacerbated when vision was removed.

Radiation-induced myelopathy in long-term surviving metastatic spinal cord compression patients after hypofractionated radiotherapy: a clinical and magnetic resonance imaging analysis

International Nuclear Information System (INIS)

Maranzano, Ernesto; Bellavita, Rita; Floridi, Piero; Celani, Grazia; Righetti, Enrico; Lupattelli, Marco; Panizza, Bianca Moira; Frattegiani, Alessandro; Pelliccioli, Gian Piero; Latini, Paolo

2001-01-01

Background and purpose: Hypofractionated radiotherapy is often administered in metastatic spinal cord compression (MSCC), but no studies have been published on the incidence of radiation-induced myelopathy (RIM) in long-term surviving patients. Our report addresses this topic. Patients and methods: Of 465 consecutive MSCC patients submitted to radiotherapy between 1988 and 1997, 13 live patients (seven females, six males, median age 69 years, median follow-up 69 months) surviving for 2 years or more were retrospectively reviewed to evaluate RIM. All patients underwent radiotherapy. Eight patients underwent a short-course regimen of 8 Gy, with 7 days rest, and then another 8 Gy. Five patients underwent a split-course regimen of 5 Gy x3, 4 days rest, and then 3 Gy x5. Only one patient also underwent laminectomy. Full neurological examination and magnetic resonance imaging (MRI) were performed. Results: Of 12 patients submitted to radiotherapy alone, 11 were ambulant (eight without support and three with support) with good bladder function. In nine of these 11 patients, MRI was negative; in one case MRI evidenced an in-field relapse 30 months after the end of radiotherapy, and in the other, two new MSCC foci outside the irradiated spine. In the remaining patient RIM was suspected at 18 months after radiotherapy when the patient became paraplegic and cystoplegic, and magnetic resonance images evidenced an ischemic injury in the irradiated area. The only patient treated with surgery plus postoperative radiotherapy worsened and remained paraparetic. Magnetic resonance images showed cord atrophy at the surgical level, explained as an ischemic necrosis due to surgery injury. Conclusions: On the grounds of our data regarding RIM in long-term surviving MSCC patients, we believe that a hypofractionated radiotherapy regimen can be used for the majority of patients. For a minority of patients, more protracted radiation regimens could be considered

Mania associated with complicated hereditary spastic paraparesis

OpenAIRE

Raghavendra B Nayak; Govind S Bhogale; Nanasaheb M Patil; Aditya A Pandurangi

2011-01-01

Hereditary spastic paraparesis (HSP) is an inherited group of neurological disorders with progressive lower limb spasticity. HSP can be clinically grouped into pure and complicated forms. Pure HSP is one without any associated neurological/psychiatric comorbidity. Depression is the most common psychiatric comorbidity. Presence of mania or bipolar affective illness with HSP is a rare phenomenon. We report a case of a 17-year-old boy who presented with classical features of HSP with complaints ...

The prevalence of depression in hereditary spastic paraplegia.

Science.gov (United States)

Vahter, L; Braschinsky, M; Haldre, S; Gross-Paju, K

2009-09-01

To evaluate the prevalence of depression and sensitivity and specificity of the single-item interview 'Are you depressed?' for people with hereditary spastic paraplegia in Estonia. Single-item interview 'Are you depressed?' was used as a screening question for depression; all participants then completed the Beck Depression Inventory. People with hereditary spastic paraplegia identified from the epidemiological database who agreed to participate in the study. Beck Depression Inventory, clinical interview. The epidemiological database consisted of 59 patients with clinically confirmed diagnosis of hereditary spastic paraplegia. Forty-eight of these consented to participate in the study. The Beck Depression Inventory score was higher than cut-off point in 58% (28/48) and lower in 42% (20/48). Of the study group, 44% (21/48) had mild, 13% (6/48) moderate and one person revealed severe depression. There was a statistically significant correlation between Beck Depression Inventory score and level of mobility; no other significant correlations with other measures were detected. Of the participants, 54% (26/48) had subjective complaints about depression and answered 'Yes' to the single-item interview 'Are you depressed?'. The sensitivity of the one-item interview in the hereditary spastic paraplegia group was 75% and specificity 75%. Our results show that mild depression is prevalent among people with hereditary spastic paraplegia. Although the single question may be helpful, it cannot be relied upon entirely when assessing a person for depression.

Genetic and phenotypic characterization of complex hereditary spastic paraplegia

Science.gov (United States)

Kara, Eleanna; Tucci, Arianna; Manzoni, Claudia; Lynch, David S.; Elpidorou, Marilena; Bettencourt, Conceicao; Chelban, Viorica; Manole, Andreea; Hamed, Sherifa A.; Haridy, Nourelhoda A.; Federoff, Monica; Preza, Elisavet; Hughes, Deborah; Pittman, Alan; Jaunmuktane, Zane; Brandner, Sebastian; Xiromerisiou, Georgia; Wiethoff, Sarah; Schottlaender, Lucia; Proukakis, Christos; Morris, Huw; Warner, Tom; Bhatia, Kailash P.; Korlipara, L.V. Prasad; Singleton, Andrew B.; Hardy, John; Wood, Nicholas W.; Lewis, Patrick A.

2016-01-01

Abstract The hereditary spastic paraplegias are a heterogeneous group of degenerative disorders that are clinically classified as either pure with predominant lower limb spasticity, or complex where spastic paraplegia is complicated with additional neurological features, and are inherited in autosomal dominant, autosomal recessive or X-linked patterns. Genetic defects have been identified in over 40 different genes, with more than 70 loci in total. Complex recessive spastic paraplegias have in the past been frequently associated with mutations in SPG11 (spatacsin), ZFYVE26/SPG15 , SPG7 (paraplegin) and a handful of other rare genes, but many cases remain genetically undefined. The overlap with other neurodegenerative disorders has been implied in a small number of reports, but not in larger disease series. This deficiency has been largely due to the lack of suitable high throughput techniques to investigate the genetic basis of disease, but the recent availability of next generation sequencing can facilitate the identification of disease-causing mutations even in extremely heterogeneous disorders. We investigated a series of 97 index cases with complex spastic paraplegia referred to a tertiary referral neurology centre in London for diagnosis or management. The mean age of onset was 16 years (range 3 to 39). The SPG11 gene was first analysed, revealing homozygous or compound heterozygous mutations in 30/97 (30.9%) of probands, the largest SPG11 series reported to date, and by far the most common cause of complex spastic paraplegia in the UK, with severe and progressive clinical features and other neurological manifestations, linked with magnetic resonance imaging defects. Given the high frequency of SPG11 mutations, we studied the autophagic response to starvation in eight affected SPG11 cases and control fibroblast cell lines, but in our restricted study we did not observe correlations between disease status and autophagic or lysosomal markers. In the remaining

Ancillary outcome measures for assessment of individuals with cervical spondylotic myelopathy.

Science.gov (United States)

Kalsi-Ryan, Sukhvinder; Singh, Anoushka; Massicotte, Eric M; Arnold, Paul M; Brodke, Darrel S; Norvell, Daniel C; Hermsmeyer, Jeffrey T; Fehlings, Michael G

2013-10-15

Narrative review. To identify suitable outcome measures that can be used to quantify neurological and functional impairment in the management of cervical spondylotic myelopathy (CSM). CSM is the leading cause of acquired spinal cord disability, causing varying degrees of neurological impairment which impact on independence and quality of life. Because this impairment can have a heterogeneous presentation, a single outcome measure cannot define the broad range of deficits seen in this population. Therefore, it is necessary to define outcome measures that characterize the deficits with greater validity and sensitivity. This review was conducted in 3 stages. Stage I: To evaluate the current use of outcome measures in CSM, PubMed was searched using the name of the outcome measure and the common abbreviation combined with "CSM" or "myelopathy." Stage II: Having identified a lack of appropriate outcome measures, we constructed criteria by which measures appropriate for assessing the various aspects of CSM could be identified. Stage III: A second literature search was then conducted looking at specified outcomes that met these criteria. All literature was reviewed to determine specificity and psychometric properties of outcomes for CSM. Nurick grade, modified Japanese Orthopaedic Association Scale, visual analogue scale (VAS) for pain, Short Form (36) Health Survey (SF-36), and Neck Disability Index were the most commonly cited measures. The Short-Form 36 Health Survey and Myelopathy Disability Index have been validated in the CSM population with multiple studies, whereas the modified Japanese Orthopaedic Association Scale score, Nurick grade, and European Myelopathy Scale each had only one study assessing psychometric characteristics. No validity, reliability, or responsiveness studies were found for the VAS or Neck Disability Index in the CSM population. We recommend that the modified Japanese Orthopaedic Association Scale, Nurick grade, Myelopathy Disability Index

Preoperative cervical sagittal alignment parameters and their impacts on myelopathy in patients with cervical spondylotic myelopathy: a retrospective study

Directory of Open Access Journals (Sweden)

Wei Yuan

2017-11-01

Full Text Available Background Cervical sagittal alignment plays an important role in the pathogenesis of cervical spondylotic myelopathy (CSM, but there are limited studies on the cervical sagittal parameters in CSM patients and their correlations with myelopathy. The aim of this study is to investigate the correlations among the preoperative cervical sagittal alignment parameters and their correlations with the development of myelopathy in patients with CSM. Methods We retrospectively collected 212 patients with CSM who underwent surgical interventions. Gender, age, modified Japanese Orthopedic Association score (mJOA, cervical lordosis (CL, C2–C7 sagittal vertical axis (C2–C7 SVA, T1 slope (T1S, neck tilt (NT and thoracic inlet angle (TIA were collected before operation. Interobserver and intraobserver reliability were calculated for all measurements (intraclass correlation coefficient, ICC. Data were analyzed with Pearson and Spearman correlation tests and multiple linear regression analysis. Results A total of 212 patients with CSM were included in this study (male: 136, female: 76 with an average age of 54.5 ± 10.1 years old. Intraobserver and interobserver reliability for all included radiographic parameters presented good to excellent agreement (ICC > 0.7. No significant differences in demographic and radiological parameters have been observed between males and females (P > 0.05. We found statistically significant correlations among the following parameters: age with CL (r = 0.135, P = 0.049, age with T1S (r = 0.222, P = 0.001, CL with T1S (r = 0.291, P < 0.001, CL with C2-C7 SVA (r =  − 0.395, P < 0.001, mJOA with age (r =  − 0.274, P < 0.001, mJOA with C2–C7 SVA (r =  − 0.219, P < 0.001 and mJOA with T1S(r =  − 0.171, p = 0.013. Linear regression analysis showed that C2–C7 SVA was the predictor of CL (adjusted R2 = 0.152, P < 0.001 and multiple linear regression showed that age combined with C

Surfer's myelopathy: a rare presentation in a non-surfing setting and review of the literature.

Science.gov (United States)

Maharaj, Monish M; Phan, Kevin; Hariswamy, Soumya; Rao, Prashanth J

2016-09-01

Surfers myelopathy can be a rapidly devastating disease and little is known surrounding the pathophysiology of the condition. Although the classical pattern of illness has been well reported, it has never been observed in a non-surfing setting. A 51-year-old demolition worker presented with acute non-traumatic myelopathy. Clinical examination revealed sensory loss to the level of L2. T2-MRI and MRI-DWI revealed a hyperintense signal suggestive of an ischaemic event. A diagnosis of surfer's myelopathy was made and he was commenced on steroid therapy. Following steroid therapy and fluid management the patient was discharged after 6 days with minor anaethesia but significant overall neurological improvement. Diagnosis of SM requires a thorough history, clinical examination and imaging (MRI, MRI-DWI). The patient should be admitted early and investigated. The use of rehabilitation services may be useful if available.

Technologically-advanced assessment of upper-limb spasticity

DEFF Research Database (Denmark)

Posteraro, Federico; Crea, Simona; Mazzoleni, Stefano

2018-01-01

post stroke patients. METHODS: A new robotic device able to automatically assess upper-limb spasticity during passive and active mobilization has been developed. The elbow spasticity of five post stroke patients has been assessed by using the new device and by means of the Modified Ashworth Scale (MAS......). After the first assessment, subjects were treated with botulin toxin injections, and then underwent 10 sessions of robotic treatments. After the treatment, subjects spasticity was assessed by using the robotic device and the MAS score. RESULTS: In four out of five patients, the botulin toxin injection...... and robotic treatment resulted in the improvement of the MAS score; in three patients the robotic measures were able to detect the MAS changes. In one subject botulin toxin was not effective and the robotic device was able to detect the lack of effectiveness. CONCLUSIONS: By using the robotic device some...

Imaging spinal cord atrophy in progressive myelopathies: HTLV-I-associated neurological disease (HAM/TSP) and multiple sclerosis (MS).

Science.gov (United States)

Azodi, Shila; Nair, Govind; Enose-Akahata, Yoshimi; Charlip, Emily; Vellucci, Ashley; Cortese, Irene; Dwyer, Jenifer; Billioux, B Jeanne; Thomas, Chevaz; Ohayon, Joan; Reich, Daniel S; Jacobson, Steven

2017-11-01

Previous work measures spinal cord thinning in chronic progressive myelopathies, including human T-lymphotropic virus 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and multiple sclerosis (MS). Quantitative measurements of spinal cord atrophy are important in fully characterizing these and other spinal cord diseases. We aimed to investigate patterns of spinal cord atrophy and correlations with clinical markers. Spinal cord cross-sectional area was measured in individuals (24 healthy controls [HCs], 17 asymptomatic carriers of HTLV-1 (AC), 47 HAM/TSP, 74 relapsing-remitting MS [RRMS], 17 secondary progressive MS [SPMS], and 40 primary progressive MS [PPMS]) from C1 to T10. Clinical disability scores, viral markers, and immunological parameters were obtained for patients and correlated with representative spinal cord cross-sectional area regions at the C2 to C3, C4 to C5, and T4 to T9 levels. In 2 HAM/TSP patients, spinal cord cross-sectional area was measured over 3 years. All spinal cord regions are thinner in HAM/TSP (56 mm 2 [standard deviation, 10], 59 [10], 23 [5]) than in HC (76 [7], 83 [8], 38 [4]) and AC (71 [7], 78 [9], 36 [7]). SPMS (62 [9], 66 [9], 32 [6]) and PPMS (65 [11], 68 [10], 35 [7]) have thinner cervical cords than HC and RRMS (73 [9], 77 [10], 37 [6]). Clinical disability scores (Expanded Disability Status Scale [p = 0.009] and Instituto de Pesquisas de Cananeia [p = 0.03]) and CD8 + T-cell frequency (p = 0.04) correlate with T4 to T9 spinal cord cross-sectional area in HAM/TSP. Higher cerebrospinal fluid HTLV-1 proviral load (p = 0.01) was associated with thinner spinal cord cross-sectional area. Both HAM/TSP patients followed longitudinally showed thoracic thinning followed by cervical thinning. Group average spinal cord cross-sectional area in HAM/TSP and progressive MS show spinal cord atrophy. We further hypothesize in HAM/TSP that is possible that neuroglial loss from a thoracic inflammatory

The Efficacy of Botulinum Toxin A Intramuscular Injections in After-Stroke Spasticity

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Melek Karaçam

2010-09-01

Full Text Available OBJECTIVE: Spasticity is a common dysfunction in stroke patients. It hinders the performance of everyday living activities and lowers the quality of life. In this study, it was aimed to investigate the effects of botulinum toxin A therapy on various aspects, such as muscle tone, pain, daily living activities and disability. METHODS: Fifteen patients with stroke presenting with focal spasticity in the botulinum toxin outpatient unit were evaluated. Results before and after treatment were evaluated by applying different scales. Modified Ashworth Scale was applied for the severity of spasticity. The Medical Council Research Scale was used to test muscle power, and the disability scoring scale, Visual Analogue Pain Scale and Barthel index were the other measures tested. RESULTS: It was found that therapy with botulinum toxin A was effective in spasticity. The increased muscle tone and the disability scores decreased prominently after the treatment (p< 0.05. Lower values in pain scores (p< 0.05 also contributed to better functional outcome (p< 0.01. Along with the significantly good outcome according to the scales, the higher scores in quality of life, feeling of well-being, good performance during the physiotherapy sessions, and less medications needed for spasticity were also indications in commencing the therapy of botulinum toxin A in spasticity. CONCLUSION: Spasticity is a complicated condition causing serious disability. Botulinum toxin A is a preferred therapy when there is an increased motor activity. The effects of the agent are reversible and reliable. The duration of the treatment is long-lasting. Since botulinum toxin A is easily applied and the outcome in focal spasticity is favorable, it is recommended as the first-line choice in the treatment of focal spasticity

An analysis of cervical myelopathy due to cervical spondylosis or ossification of posterior longitudinal ligament by CT myelography

International Nuclear Information System (INIS)

Fujiwara, Keiju; Yonenobe, Sakuo; Ebara, Sohei; Yamashita, Kazuo; Ono, Keiro

1988-01-01

CT-myelographic (CTM) findings of 20 patients with ossification of posterior longitudinal ligament (OPLL) and 24 patients with cervical spondylotic myelopathy (CSM) were reviewed for the evaluation of (1) contributing factors to preoperative neurologic symptoms and therapeutic prognosis in OPLL, and (2) differences in pathology between OPLL and CSM. In OPLL, the severity of preoperative neurologic symptoms was not related to the degree of deformed spinal cord - as expressed by the transverse area of the spinal cord and the rate of flatness - nor the degree of ossification - as expressed by the rate of stricture, and the transverse areas of the effective spinal canal and ossification. The transverse areas of the spinal cord and effective spinal canal were correlated with both postoperative scores for neurologic symptoms and the recovery rate. Osseous compression to the spinal cord was severer in OPLL than OSM. Regarding other factors, such as size and shape of the spinal cord and therapeutic prognosis, there was no difference between the two diseases. This implied the association of dynamic compression to the spinal cord that resulted from the unstable cervical spine in the case of CSM. (Namekawa, K.)

Spinal Cord Stimulation for Spasticity: Historical Approaches, Current Status, and Future Directions.

Science.gov (United States)

Nagel, Sean J; Wilson, Saul; Johnson, Michael D; Machado, Andre; Frizon, Leonardo; Chardon, Matthieu K; Reddy, Chandan G; Gillies, George T; Howard, Matthew A

2017-06-01

Millions of people worldwide suffer with spasticity related to irreversible damage to the brain or spinal cord. Typical antecedent events include stroke, traumatic brain injury, and spinal cord injury, although insidious onset is also common. Regardless of the cause, the resulting spasticity leads to years of disability and reduced quality of life. Many treatments are available to manage spasticity; yet each is fraught with drawbacks including incomplete response, high cost, limited duration, dose-limiting side effects, and periodic maintenance. Spinal cord stimulation (SCS), a once promising therapy for spasticity, has largely been relegated to permanent experimental status. In this review, our goal is to document and critique the history and assess the development of SCS as a treatment of lower limb spasticity. By incorporating recent discoveries with the insights gained from the early pioneers in this field, we intend to lay the groundwork needed to propose testable hypotheses for future studies. SCS has been tested in over 25 different conditions since a potentially beneficial effect was first reported in 1973. However, the lack of a fully formed understanding of the pathophysiology of spasticity, archaic study methodology, and the early technological limitations of implantable hardware limit the validity of many studies. SCS offers a measure of control for spasticity that cannot be duplicated with other interventions. With improved energy-source miniaturization, tailored control algorithms, novel implant design, and a clearer picture of the pathophysiology of spasticity, we are poised to reintroduce and test SCS in this population. © 2017 International Neuromodulation Society.

Effect of whole-body vibration on muscle strength, spasticity, and ...

African Journals Online (AJOL)

CP) and is characterized by spasticity and muscle weakness of both lower limbs resulting in decreased walking ability. The purpose of this study was to evaluate the effect of whole body vibration (WBV) training on muscle strength, spasticity, and ...

Vascular myelopathy: causes and mechanisms, possibilities of diagnosis and treatment

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G. V. Ponomarev

2018-01-01

Full Text Available Vascular myelopathy is a rare severe disease caused by a broad spectrum of causes, among which pathology of the aorta and its branches, aortic surgery, spinal diseases, and spinal trauma occupy the main place. The processes of neuroinflammation and glutamate neurotoxicity play a leading role in the pathogenesis of myeloischemia. The clinical picture of the disease is nonspecific and depends on the location and volume of an ischemic focus. Magnetic resonance imaging is a gold standard for diagnosis. However, this method remains insensitive in the acute period and fails to detect spinal cord ischemia at preclinical stages. The investigation and introduction of specific biochemical markers (glutamate receptors and their antibodies for neurotoxicity, which can identify ischemia in the advanced stage and predetermine its development, are promising. The treatment of vascular myelopathy has not currently been standardized and it is mainly pathogenetic and symptomatic.

Myelopathy and sciatica induced by an extradural S1 root haemangioblastoma

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Hermier, M.; Cotton, F.; Froment, J.C. [Department of Radiology, Hopital Neurologique et Neurochirurgical, Lyon (France); Saint-Pierre, G.; Jouvet, A. [Department of Neuropathology, Hopital Neurologique et Neurochirurgical, Lyon (France); Ongolo-Zogo, P. [Department of Radiology, Hopital Neurologique et Neurochirurgical, Lyon (France); Department of Radiology, Hopital Central, Yaounde (Cameroon); Fischer, G. [Department of Neurosurgery, Hopital Neurologique et Neurochirurgical, Lyon (France)

2002-06-01

Haemangioblastomas are vascular tumours which mainly involve the central nervous system and retina, often in the setting of von Hippel-Lindau disease. Haemangioblastomas occurring outside the central nervous system are uncommon. Wherever it is, recognising this tumour prior to surgery is desirable, as preoperative embolisation may be considered. We report the clinical, imaging and pathological features of a sporadic sacral root haemangioblastoma in a 58-year-old man with chronic sciatica and myelopathy. The diagnosis was questioned preoperatively because an enlarged sacral foramen, seen to be filled by a highly vascular, enhancing mass and dilated vessels. Myelopathy was attributed to the presumed high venous pressure resulting from increased flow in veins draining the vascular tumour. Microneurosurgical excision was performed after endovascular embolisation and led to persistent clinical improvement. (orig.)

Myelopathy and sciatica induced by an extradural S1 root haemangioblastoma

International Nuclear Information System (INIS)

Hermier, M.; Cotton, F.; Froment, J.C.; Saint-Pierre, G.; Jouvet, A.; Ongolo-Zogo, P.; Fischer, G.

2002-01-01

Haemangioblastomas are vascular tumours which mainly involve the central nervous system and retina, often in the setting of von Hippel-Lindau disease. Haemangioblastomas occurring outside the central nervous system are uncommon. Wherever it is, recognising this tumour prior to surgery is desirable, as preoperative embolisation may be considered. We report the clinical, imaging and pathological features of a sporadic sacral root haemangioblastoma in a 58-year-old man with chronic sciatica and myelopathy. The diagnosis was questioned preoperatively because an enlarged sacral foramen, seen to be filled by a highly vascular, enhancing mass and dilated vessels. Myelopathy was attributed to the presumed high venous pressure resulting from increased flow in veins draining the vascular tumour. Microneurosurgical excision was performed after endovascular embolisation and led to persistent clinical improvement. (orig.)

Advances in the management of multiple sclerosis symptoms: pathophysiology and assessment of spasticity in multiple sclerosis.

Science.gov (United States)

Tintoré, Mar

2015-01-01

Spasticity is a prevalent and troublesome symptom for people with multiple sclerosis (MS). Common instruments to measure MS spasticity include the clinician-rated (modified) Ashworth scale and the patient-rated 0-10 spasticity Numerical Rating Scale (NRS). Current opinion is that measurement of MS spasticity should incorporate the patient's perspective. Other instruments to assess spasticity-associated symptoms such as the Penn spasms frequency scale, sleep quality NRS and pain NRS can assist in tracking MS spasticity evolution and inform management choices. Worsening spasticity reduces patient autonomy, impacts negatively on quality of life and increases health resource utilization and costs. Despite the wide range of issues associated with MS spasticity, undertreatment is common and standard treatment options (physiotherapy and classical oral therapies) often fail to provide adequate symptomatic control.

Characterization of Spasticity in Cerebral Palsy: Dependence of Catch Angle on Velocity

Science.gov (United States)

Wu, Yi-Ning; Ren, Yupeng; Goldsmith, Ashlee; Gaebler, Deborah; Liu, Shu Q.; Zhang, Li-Qun

2010-01-01

Aim: To evaluate spasticity under controlled velocities and torques in children with cerebral palsy (CP) using a manual spasticity evaluator. Method: The study involved 10 children with spastic CP (six males, four females; mean age 10y 1mo, SD 2y 9mo, range 7-16y; one with quadriplegia, six with right hemiplegia, three with left hemiplegia; Gross…

Spasticity, an impairment that is poorly defined and poorly measured

NARCIS (Netherlands)

Malhotra, S.; Malhotra, S.; Pandyan, A.D.; Day, C.R.; Jones, Valerie M.; Hermens, Hermanus J.

Objective: To explore, following a literature review, whether there is a consistent definition and a unified assessment framework for the term 'spasticity'. The congruence between the definitions of spasticity and the corresponding methods of measurement were also explored. Data sources: The search

HTLV-I/II prevalence in different geographic locations

NARCIS (Netherlands)

Vrielink, Hans; Reesink, Henk W.

2004-01-01

Human T-cell lymphotropic virus (HTLV) type I (HTLV-I) is the etiological agent of adult T-cell leukemia and HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). HTLV-II is a closely related virus, and this infection is not clearly associated with clinical disease, although

Molecular epidemiology of endemic human t-lymphotropic virus type 1 in a rural community in guinea-bissau

NARCIS (Netherlands)

C. van Tienen (Carla); T.I. de Silva (Thushan); L.C.J. Alcantara (Luiz); C. Onyango (Clayton); S. Jarju (Sheikh); N. Gonçalves (Nato); T. Vincent (Tim); P. Aaby; H. Whittle (Hilton); M. Schim van der Loeff (Maarten); M. Cotten (Matthew)

2012-01-01

textabstractBackground: Human T-Lymphotropic Virus Type 1 (HTLV-1) infection causes lethal adult T-cell leukemia (ATL) and severely debilitating HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in up to 5% of infected adults. HTLV-1 is endemic in parts of Africa and the highest

Magnetic resonance imaging in cervical spinal cord compression

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Giovanni Giammona

1993-09-01

Full Text Available In patients with cervical spondylotic myelopathy MRI sometimes shows increased signal intensity zones on the T2-weighted images. It has been suggested that these findings carry prognostic significance. We studied 56 subjects with cervical spinal cord compression. Twelve patients showed an increased signal intensity (21.4% and a prevalence of narrowing of the AP-diameter (62% vs 24%. Furthemore, in this group, there was evidence of a longer mean duration of the symptoms and, in most of the patients, of more serious clinical conditions. The importance of these predisposing factors remains, however, to be clarified since they are also present in some patients without the increased signal intensity.

Etiologic profile of spastic quadriplegia in children.

Science.gov (United States)

Venkateswaran, Sunita; Shevell, Michael I

2007-09-01

The etiologic profile and possible predictors of etiology in children with spastic quadriplegia were assessed in a consecutive cohort of children with this motor impairment. Medical records from a single pediatric neurology practice over a 14-year interval were retrospectively and systematically reviewed. Variables comprised possible demographic, prenatal, perinatal, and postnatal risk factors. Of the 99 patients included in the study, 39 were premature (quadriplegia was 83%, with differing underlying etiologies depending on gestational age. These results should help guide physicians in investigating possible underlying etiologies in patients with spastic quadriplegia.

The meaning of spasticity to people with multiple sclerosis: what can health professionals learn?

Science.gov (United States)

Morley, Alex; Tod, Angela; Cramp, Mary; Mawson, Sue

2013-07-01

Multiple sclerosis (MS) is the most common disabling neurological condition affecting young adults. One third of people on an American registry of people with MS (PWMS) reported having activities affected by spasticity. The psychosocial effects of spasticity in people with MS have been shown to be distressing and detrimental to emotional and social relationships when investigated from a psychology perspective. This paper investigates the impact of spasticity on the lives of people living with MS from a physiotherapeutic perspective. This study involved 12 semi-structured interviews with individuals experiencing MS-related spasticity. Ten sets of data were analyzed following framework analysis principles. Results suggest spasticity effects life experience of these PWMS in diverse and complex ways. Physical, psychological and social consequences of spasticity are closely linked and can be far reaching. Therapists need to be aware of links between specific physical symptoms and their psychosocial consequences if they want to improve peoples' quality of life. This paper provides in depth qualitative research evidence for the complexity of the spasticity experience for each individual, strengthening the argument for a patient-centred approach to treatment. These results also support the case for targeted interventions with effectiveness recorded in a patient-centred way. • Spasticity is suggested here to affect the lives of individuals with multiple sclerosis in diverse and far reaching ways. Therapists need to investigate this fully in subjective assessment to impact on people's quality of life. • Direct links were identified between treatable physical symptoms and far reaching consequences of spasticity. • Knowledge about the complexity of the spasticity experience for each individual will allow therapists to target interventions appropriately and accurately record effectiveness in a patient-centred way.

The Adverse Effect of Spasticity on 3-Month Poststroke Outcome Using a Population-Based Model

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S. R. Belagaje

2014-01-01

Full Text Available Several devices and medications have been used to address poststroke spasticity. Yet, spasticity’s impact on outcomes remains controversial. Using data from a cohort of 460 ischemic stroke patients, we previously published a validated multivariable regression model for predicting 3-month modified Rankin Score (mRS as an indicator of functional outcome. Here, we tested whether including spasticity improved model fit and estimated the effect spasticity had on the outcome. Spasticity was defined by a positive response to the question “Did you have spasticity following your stroke?” on direct interview at 3 months from stroke onset. Patients who had expired by 90 days (n=30 or did not have spasticity data available (n=102 were excluded. Spasticity affected the 3-month functional status (β=0.420, 95 CI=0.194 to 0.645 after accounting for age, diabetes, leukoaraiosis, and retrospective NIHSS. Using spasticity as a covariable, the model’s R2 changed from 0.599 to 0.622. In our model, the presence of spasticity in the cohort was associated with a worsened 3-month mRS by an average of 0.4 after adjusting for known covariables. This significant adverse effect on functional outcomes adds predictive value beyond previously established factors.

Surfer’s myelopathy: a rare presentation in a non-surfing setting and review of the literature

Science.gov (United States)

Phan, Kevin; Hariswamy, Soumya; Rao, Prashanth J.

2016-01-01

Background Surfers myelopathy can be a rapidly devastating disease and little is known surrounding the pathophysiology of the condition. Although the classical pattern of illness has been well reported, it has never been observed in a non-surfing setting. Methods A 51-year-old demolition worker presented with acute non-traumatic myelopathy. Clinical examination revealed sensory loss to the level of L2. T2-MRI and MRI-DWI revealed a hyperintense signal suggestive of an ischaemic event. A diagnosis of surfer’s myelopathy was made and he was commenced on steroid therapy. Results Following steroid therapy and fluid management the patient was discharged after 6 days with minor anaethesia but significant overall neurological improvement. Conclusions Diagnosis of SM requires a thorough history, clinical examination and imaging (MRI, MRI-DWI). The patient should be admitted early and investigated. The use of rehabilitation services may be useful if available. PMID:27757436

Molecular Epidemiology of Endemic Human T-Lymphotropic Virus Type 1 in a Rural Community in Guinea-Bissau

NARCIS (Netherlands)

van Tienen, Carla; de Silva, Thushan I.; Alcantara, Luiz Carlos Junior; Onyango, Clayton O.; Jarju, Sheikh; Gonçalves, Nato; Vincent, Tim; Aaby, Peter; Whittle, Hilton; Schim van der Loeff, Maarten; Cotten, Matthew

2012-01-01

Background: Human T-Lymphotropic Virus Type 1 (HTLV-1) infection causes lethal adult T-cell leukemia (ATL) and severely debilitating HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in up to 5% of infected adults. HTLV-1 is endemic in parts of Africa and the highest prevalence in

Validity of Modified Ashworth Scale as a Measure of Wrist Spasticity in Stroke Patients

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Mohammad Heidari

2011-04-01

Full Text Available Objectives: There are some controversies about the value of modified Ashworth Scale (MAS for assessing spasticity. The goal of this study was to investigate if there is any correlation between scores obtained from MAS for wrist spasticity and electrophysiological recordings as the objective measure of spasticity. Methods: In this cross-sectional study, 34 stroke patients were employed. Wrist spasticity was clinically measured by means of MAS. Also, an electromyogram (EMG machine was used to elicit Hmax and Mmax from the flexor carpi radialis muscle. Spearman’s correlation coefficient test was used to investigate potential correlation between clinically and electrophysiologically measures of spasticity. Results: The observed relation between MAS and EMG recordings was not statistically significant (rho=0.183, P>0.05. Discussion: Our findings suggest that MAS may be a useful tool for grading hypertonia, but it is not a valid measure of spasticity in selected patients.

Experimental Study on radiation myelopathy

International Nuclear Information System (INIS)

Kaneko, Itsuo; Matsushima, Hideno; Yamada, Teruyo

1979-01-01

Experimental radiation myelopathy was carried out useing rats. This studies were done refering the effect to skin, the body weight, the status of the paralysis and the capillary densities of the cervical cords. The quadriplegia was seen on the animals which were irradiated over 4000 rad. The vacuoal degeneration was observed on the cervical cords which were irradiated over 4000 rad. The capillary densities of gray matter and white matter decreased finally in proportions to the irradiation dose. The vacuoal degeneration was recognized on the cervical cord in which the capillary density decreased to under 70 per cent of normal density. Decrease of the capillary density is seemed to be the one of the cause of the paralysis. (author)

A case of radiation myelopathy

International Nuclear Information System (INIS)

Hatano, Noriyoshi; Iwamoto, Toshihiko; Hagiwara, Akiyoshi; Sato, Yoshihito; Shimizu, Nobuya; Takagi, Yasushi

1988-01-01

A 72-year-old woman presented with right hemiparesthesia and left hemiparalysis. At the age of 63, the patient was treated with pre- and post-operative radiation with a total dose of 100 Gy for maxillary squamous cell carcinoma. Five years later, a dose of 50 Gy was delivered again to the head and neck because of the suspicion of recurrence. Magnetic resonance imaging showed flat and thinned cervical spine on the transverse section and markedly atrophied cervical spine at the C1 to C3 levels on the sagittal section. She was diangosed as having chronic progressive radiation myelopathy in view of a history of large doses of radiation to the head and neck. (Namekawa, K.)

Surfer's myelopathy: case report and review.

Science.gov (United States)

Karabegovic, Amna; Strachan-Jackman, Shirley; Carr, David

2011-09-01

Nontraumatic spinal cord injury from surfing is a new entity first described in 2004 and likely of ischemic etiology. We report the case of a 25-year-old man who presented to the emergency department with a 2-week history of lower extremity weakness after surfing in Indonesia. The patient reported developing low back pain, lower extremity weakness, sensory changes, and urinary retention shortly after his first surfing lesson. The patient was subsequently diagnosed with surfer's myelopathy. The purpose of this report is to review the clinical presentation, etiology, risk factors, and management of this increasingly described entity.

Validity and Inter-Rater Reliability of a Novel Bedside Referral Tool for Spasticity

Science.gov (United States)

2018-02-20

Spasticity, Muscle; Muscular Diseases; Musculoskeletal Disease; Muscle Hypertonia; Muscle Spasticity; Neuromuscular Manifestations; Signs and Symptoms; Nervous System Diseases; Neurologic Manifestations

Clinical features and management of hereditary spastic paraplegia

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Ingrid Faber

2014-03-01

Full Text Available Hereditary spastic paraplegia (HSP is a group of genetically-determined disorders characterized by progressive spasticity and weakness of lower limbs. An apparently sporadic case of adult-onset spastic paraplegia is a frequent clinical problem and a significant proportion of cases are likely to be of genetic origin. HSP is clinically divided into pure and complicated forms. The later present with a wide range of additional neurological and systemic features. To date, there are up to 60 genetic subtypes described. All modes of monogenic inheritance have been described: autosomal dominant, autosomal recessive, X-linked and mitochondrial traits. Recent advances point to abnormal axonal transport as a key mechanism leading to the degeneration of the long motor neuron axons in the central nervous system in HSP. In this review we aim to address recent advances in the field, placing emphasis on key diagnostic features that will help practicing neurologists to identify and manage these conditions.

Hereditary spastic paraplegia with cerebellar ataxia

DEFF Research Database (Denmark)

Nielsen, J E; Johnsen, B; Koefoed, P

2004-01-01

Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria......, unipolar depression, epilepsy, migraine, and cognitive impairment was investigated. Genetic linkage analysis and sequencing of the SPG4 gene was performed and electrophysiologic investigations were carried out in six individuals and positron emission tomography (PET) in one patient. The disease was linked...... in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance...

Quality of life in symptomatic cervical myelopathy after open-door laminoplasty

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Rodrigo Yuiti Nakao

2015-06-01

Full Text Available OBJECTIVE: To analyze the results obtained by open-door laminoplasty using the Newbridge(r/Blackstone plate, in cases of myelopathy associated with cervical lordosis.METHODS: From December 2010 to October 2012, eight patients between the ages of 49 and 68 underwent open-door laminoplasty with the use of the Newbridge(r fixation system for maintenance and stabilization of the cervical laminoplasty. Minimum follow-up was four months. For the evaluation of quality of life the questionnaire SF-36 was applied at the following times: preoperative, one month and three months after surgery associated with the subjective assessment of the patient regarding satisfaction with the procedure and with the Nurick neurological scale applied prior to surgery and three months later.RESULTS: According to the SF-36, there was significant improvement in the domains functional ability, general health perceptions and emotional aspects over time; regarding physical limitations and social aspects there was no improvement in the first postoperative month, only in the third month. There were no statistically significant changes observed during the period covered by this study related to pain, vitality and mental health. According to Nurick scale, there was evidence of improvement in symptoms of cervical myelopathy. Based on the subjective evaluation of the patients, surprisingly, all patients were satisfied with the surgical procedure and the results.CONCLUSION: The open-door laminoplasty technique with rigid fixation to maintain the opening is useful in improving the symptoms of cervical myelopathy associated with lordosis, leading to improved quality of life and with a high degree of patient satisfaction and fewer complications.

[Management of symptoms associated with spasticity in patients with multiple sclerosis].

Science.gov (United States)

Fernández-Pablos, María Asunción; Costa-Frossard, Lucienne; García-Hernández, Carlos; García-Montes, Inmaculada; Escutia-Roig, Matilde

To describe the role of nurses in the management of symptoms related to spasticity in patients with multiple sclerosis (MS). A descriptive study was developed based on a questionnaire on spasticity in MS patients. The questionnarie was completed through an anonymous tele-voting system at a national meeting with nurses involved in the management of these patients. Apart from fatigue, according to the opinion of the participants, the spasticity symptom associated with MS most notified by patients was difficulty in walking, followed by spasms and pain. Participants thought that it is important that nursing takes: 1) a role in identifying these symptoms, 2) should focus on the detection of the triggering or aggravating factors, and 3) on providing support in the assessment of the level of spasticity. It is important to inform about the correct use of anti-spasticity drugs, how to adjust the dosage and side effects of treatments, including cannabinoids via an oromucosal spray, titrating its doses according to each patient, and monitoring its tolerability, efficacy and adherence. Although there are usually resources to follow up these patients, there are still important gaps, including the lack of a specific follow-up protocol. Although all the participants are experts in the management of patients with MS, there is still diversity in the functions they perform, and the available resources they have in their hospitals. Nurses act as a key element in the process of identification of symptoms, training and monitoring of these patients with spasticity in EM. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

Functional Brain Correlates of Upper Limb Spasticity and Its Mitigation following Rehabilitation in Chronic Stroke Survivors

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Svetlana Pundik

2014-01-01

Full Text Available Background. Arm spasticity is a challenge in the care of chronic stroke survivors with motor deficits. In order to advance spasticity treatments, a better understanding of the mechanism of spasticity-related neuroplasticity is needed. Objective. To investigate brain function correlates of spasticity in chronic stroke and to identify specific regional functional brain changes related to rehabilitation-induced mitigation of spasticity. Methods. 23 stroke survivors (>6 months were treated with an arm motor learning and spasticity therapy (5 d/wk for 12 weeks. Outcome measures included Modified Ashworth scale, sensory tests, and functional magnetic resonance imaging (fMRI for wrist and hand movement. Results. First, at baseline, greater spasticity correlated with poorer motor function (P=0.001 and greater sensory deficits (P=0.003. Second, rehabilitation produced improvement in upper limb spasticity and motor function (P<0.0001. Third, at baseline, greater spasticity correlated with higher fMRI activation in the ipsilesional thalamus (rho=0.49, P=0.03. Fourth, following rehabilitation, greater mitigation of spasticity correlated with enhanced fMRI activation in the contralesional primary motor (r=-0.755, P=0.003, premotor (r=−0.565, P=0.04, primary sensory (r=−0.614, P=0.03, and associative sensory (r=−0.597, P=0.03 regions while controlling for changes in motor function. Conclusions. Contralesional motor regions may contribute to restoring control of muscle tone in chronic stroke.

Percutaneous epidural neurostimulation in modulation of paraplegic spasticity. Six case reports.

Science.gov (United States)

Richardson, R R; Cerullo, L J; McLone, D G; Gutierrez, F A; Lewis, V

1979-01-01

Six cases of paraplegic, post-traumatic spasticity, alleviated by percutaneous epidural neurostimulation with temporary or permanent implanted neuroelectrodes from the L1 to L4 intervertebral levels are presented. Modulation of this spasticity and secondary beneficial physiological effects were achieved, including regulation of bowel regimens, production of sweating and piloerection below the level of the lesion, and morning erections. The main advantages of percutaneous epidural neurostimulation in modulating spasticity are the avoidance of destructive neurosurgical procedures, the regulation of secondary physiological and autonomic responses, the avoidance of antispasticity medications, and the reversibility of the neurostimulation procedure.

Hereditary spastic paraplegias: membrane traffic and the motor pathway

OpenAIRE

Blackstone, Craig; O’Kane, Cahir J.; Reid, Evan

2011-01-01

Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent wo...

Converging cellular themes for the hereditary spastic paraplegias.

Science.gov (United States)

Blackstone, Craig

2018-05-10

Hereditary spastic paraplegias (HSPs) are neurologic disorders characterized by prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. They are among the most genetically-diverse neurologic disorders, with >80 distinct genetic loci and over 60 identified genes. Studies investigating the molecular pathogenesis underlying HSPs have emphasized the importance of converging cellular pathogenic themes in the most common forms of HSP, providing compelling targets for therapy. Most notably, these include organelle shaping and biogenesis as well as membrane and cargo trafficking. Published by Elsevier Ltd.

A study of measurement of the spinal cord of cervical myelopathy with CT-myelography and forecast of operative result from the size of the spinal cord

International Nuclear Information System (INIS)

Oosawa, Yoshimitsu

1985-01-01

The antero-posterior (AP) and transverse (T) diameter and the T area of the spinal canal, dural canal, and spinal cord were measured using CT-myelography (CT-M) in 44 patients with cervical myelopathy (CM) and 20 control subjects. The AP diameter of these canals and cord and the T diameter of the spinal canal were smaller in the CM group than in the control group. Postoperative CT-M showed that the dural canal and spinal cord had an increase in the AP diameter and T area and a decrease in the T diameter. Preoperative symptoms were well correlated with the AP diameter and the T area of the spinal canal, dural canal, and spinal cord, and spinal cord compression. The symptoms tended to be milder with larger AT diameter and T area of the spinal canal, dural canal, and spinal cord and with smaller spinal cord compression and deformity. Functional damage was reversible in patients with slight spinal cord compression. Favorable operative outcome tended to be achieved when the preoperative AP diameter and T area of the spinal cord were ≥ 5 mm and ≥ 50 mm 2 , respectively. (Namekawa, K.)

A Systematic Review of Non-Traumatic Spinal Cord Injuries in Sub-Saharan Africa and a Proposed Diagnostic Algorithm for Resource-Limited Settings

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Abdu Kisekka Musubire

2017-12-01

Full Text Available BackgroundNon-traumatic myelopathy is common in Africa and there are geographic differences in etiology. Clinical management is challenging due to the broad differential diagnosis and the lack of diagnostics. The objective of this systematic review is to determine the most common etiologies of non-traumatic myelopathy in sub-Saharan Africa to inform a regionally appropriate diagnostic algorithm.MethodsWe conducted a systemic review searching Medline and Embase databases using the following search terms: “Non traumatic spinal cord injury” or “myelopathy” with limitations to epidemiology or etiologies and Sub-Saharan Africa. We described the frequencies of the different etiologies and proposed a diagnostic algorithm based on the most common diagnoses.ResultsWe identified 19 studies all performed at tertiary institutions; 15 were retrospective and 13 were published in the era of the HIV epidemic. Compressive bone lesions accounted for more than 48% of the cases; a majority were Pott’s disease and metastatic disease. No diagnosis was identified in up to 30% of cases in most studies; in particular, definitive diagnoses of non-compressive lesions were rare and a majority were clinical diagnoses of transverse myelitis and HIV myelopathy. Age and HIV were major determinants of etiology.ConclusionCompressive myelopathies represent a majority of non-traumatic myelopathies in sub-Saharan Africa, and most were due to Pott’s disease. Non-compressive myelopathies have not been well defined and need further research in Africa. We recommend a standardized approach to management of non-traumatic myelopathy focused on identifying treatable conditions with tests widely available in low-resource settings.

Botulinum therapy for poststroke spasticity of the lower extremity (clinical cases

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L. V. Krylova

2014-01-01

Full Text Available The paper deals with the topical problem – the medical rehabilitation of patients with poststroke spasticity. It describes clinical cases of patients with poststroke spasticity of the upper and lower extremities who have received combined therapy using botulinum toxin type A (Botox injections.

Adult T-cell leukemia-lymphoma in a patient with HTLV-I/II associated myelopathy Leucemia - linfoma de células T do adulto em um paciente com mielopatia associada a HTLV-I/II

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Virgínia Freitas

1997-06-01

Full Text Available Chronic myelopathy associated with T-lymphotropic virus type I (HAM has been described as an endemic disease in several areas of the world, meanwhile there are few papers describing the association between HAM and adult T cell leukemia-lymphoma. We report the case of a man that, after four years of progressive spastic paraparesis and neurogenic bladder, developed a clinical picture of a lymphoproliferative disorder characterized by dermal and systemic envolvement, mimicking mycosis fungoides/Sézary syndrome.Apesar da infecção pelo HTLV-I ser endêmica em várias regiões do mundo, poucos são os relatos da associação entre leucemia-linfoma de células T do adulto (ATLL e encefalomieloneuropatia pelo HTLV-I. No presente artigo é descrito um paciente que no curso do comprometimento neurológico pelo HTLV-I desenvolveu quadro de leucemia com infiltração de tecido dérmico semelhante ao encontrado na micose fungóide/síndrome de Sézary.

MRI of the cranio-cervical region in rheumatoid arthritis

International Nuclear Information System (INIS)

Algra, P.R.; Breedveld, F.C.; Vielvoye, G.J.; Doornbos, J.; Roos, A. de

1987-01-01

16 patients suffering from rheumatoid arthritis and suspected compressive cervical myelopathy were studied with a 0.5 T superconducting magnetic resonance imaging (MRI) scanner. MRI findings were compared with those of plain radiography and myelography. Subluxation was detected equally well by MRI and radiography. MRI was able to detect the presence, level and origin of cord compression. MRI was superior in diagnosing the extent of cord compression. Cord distortion detected by MRI correlated better with clinical evidence of myelopathy than did the radiographically established extent of the subluxation. MRI correctly diagnosed the level and cause of cord compression in six patients who were subsequently operated upon because of progressive neurological signs. These findings suggest that MRI has potential in the diagnosis and management of patients with rheumatoid arthritis suspected of compressive cervical myelopathy. 20 refs.; 4 figs.; 1 table

Prevalence and risk factors for epilepsy in children with spastic cerebral palsy

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Dedy Rahmat

2010-03-01

Conclusions The prevalence of epilepsy in spastic CP is 39%. The risk factors for epilepsy in spastic CP are post central nervous system infection, and ocurrence of seizure in the first year of life. [Paediatr Indones. 2010;50:11-7].

Effects of repetitive peripheral magnetic stimulation on upper-limb spasticity and impairment in patients with spastic hemiparesis: a randomized, double-blind, sham-controlled study.

Science.gov (United States)

Krewer, Carmen; Hartl, Sandra; Müller, Friedemann; Koenig, Eberhard

2014-06-01

To investigate short-term and long-term effects of repetitive peripheral magnetic stimulation (rpMS) on spasticity and motor function. Monocentric, randomized, double-blind, sham-controlled trial. Neurologic rehabilitation hospital. Patients (N=66) with severe hemiparesis and mild to moderate spasticity resulting from a stroke or a traumatic brain injury. The average time ± SD since injury for the intervention groups was 26 ± 71 weeks or 37 ± 82 weeks. rpMS for 20 minutes or sham stimulation with subsequent occupational therapy for 20 minutes, 2 times a day, over a 2-week period. Modified Tardieu Scale and Fugl-Meyer Assessment (arm score), assessed before therapy, at the end of the 2-week treatment period, and 2 weeks after study treatment. Additionally, the Tardieu Scale was assessed after the first and before the third therapy session to determine any short-term effects. Spasticity (Tardieu >0) was present in 83% of wrist flexors, 62% of elbow flexors, 44% of elbow extensors, and 10% of wrist extensors. Compared with the sham stimulation group, the rpMS group showed short-term effects on spasticity for wrist flexors (P=.048), and long-term effects for elbow extensors (P<.045). Arm motor function (rpMS group: median 5 [4-27]; sham group: median 4 [4-9]) did not significantly change over the study period in either group, whereas rpMS had a positive effect on sensory function. Therapy with rpMS increases sensory function in patients with severe limb paresis. The magnetic stimulation, however, has limited effect on spasticity and no effect on motor function. Copyright © 2014 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Reliability of three-dimensional gait analysis in cervical spondylotic myelopathy.

LENUS (Irish Health Repository)

McDermott, Ailish

2010-10-01

Gait impairment is one of the primary symptoms of cervical spondylotic myelopathy (CSM). Detailed assessment is possible using three-dimensional gait analysis (3DGA), however the reliability of 3DGA for this population has not been established. The aim of this study was to evaluate the test-retest reliability of temporal-spatial, kinematic and kinetic parameters in a CSM population.

The effect of Sativex in neuropathic pain and spasticity in spinal cord injury

DEFF Research Database (Denmark)

Andresen, Sven Robert; Hansen, Rikke Bod Middelhede; Johansen, Inger Lauge

2014-01-01

Introduction: Neuropathic pain and spasticity after spinal cord injury represent significant but still unresolved problems, which cause considerable suffering and reduced quality of life for patients with spinal cord injury. Treatment of neuropathic pain and spasticity is complicated and patients...... often receive incomplete relief from present available and recommended treatment. Cannabinoids has shown efficacy on both neuropathic pain and spasticity in patients with spinal cord injury, but the studies one the topic has been too small to make a general conclusion for patients with spinal cord...... injury. Aims: To investigate the effect of Sativex (cannabinoid agonist given as an oral mucosal spray), on neuropathic pain and spasticity in patients with spinal cord injury. Methods: A randomized, double-blind, placebo-controlled crossover study. We will include 30 patients with neuropathic pain...

Decreased contribution from afferent feedback to the soleus muscle during walking in patients with spastic stroke

DEFF Research Database (Denmark)

Mazzaro, Nazarena; Nielsen, Jørgen Feldbæk; Grey, Michael James

2007-01-01

We investigated the contribution of afferent feedback to the soleus (SOL) muscle activity during the stance phase of walking in patients with spastic stroke. A total of 24 patients with hemiparetic spastic stroke and age-matched healthy volunteers participated in the study. A robotic actuator...... by the Ashworth score. These results indicate that although the stretch reflex response is facilitated during spastic gait, the contribution of afferent feedback to the ongoing locomotor SOL activity is depressed in patients with spastic stroke....

Hereditary spastic paraplegia and amyotrophy associated with a novel locus on chromosome 19

Science.gov (United States)

Meilleur, K.G.; Traoré, M.; Sangaré, M.; Britton, A.; Landouré, G.; Coulibaly, S.; Niaré, B.; Mochel, F.; La Pean, A.; Rafferty, I.; Watts, C.; Littleton-Kearney, M. T.; Blackstone, C.; Singleton, A.; Fischbeck, K.H.

2010-01-01

We identified a family in Mali with two sisters affected by spastic paraplegia. In addition to spasticity and weakness of the lower limbs, the patients had marked atrophy of the distal upper extremities. Homozygosity mapping using single nucleotide polymorphism arrays showed that the sisters shared a region of extended homozygosity at chromosome 19p13.11-q12 that was not shared by controls. These findings indicate a clinically and genetically distinct form of hereditary spastic paraplegia with amyotrophy, designated SPG43. PMID:20039086

Intrathecal baclofen pump for spasticity: an evidence-based analysis.

Science.gov (United States)

2005-01-01

To conduct an evidence-based analysis of the effectiveness and cost-effectiveness of intrathecal baclofen for spasticity. Spasticity is a motor disorder characterized by tight or stiff muscles that may interfere with voluntary muscle movements and is a problem for many patients with multiple sclerosis (MS), spinal cord injury (SCI), cerebral palsy (CP), and acquired brain injury (ABI).(1). Increased tone and spasm reduces mobility and independence, and interferes with activities of daily living, continence and sleep patterns. Spasticity may also be associated with significant pain or discomfort (e.g., due to poor fit in braces, footwear, or wheelchairs), skin breakdown, contractures, sleep disorders and difficulty in transfer. Goals of treatment are to decrease spasticity in order to improve range of motion, facilitate movement, reduce energy expenditure and reduce risk of contractures. Existing treatments include physical therapy, oral medications, injections of phenol or botulinum toxin, or surgical intervention. Baclofen is the oral drug most frequently prescribed for spasticity in cases of SCI and MS.(1) Baclofen is a muscle relaxant and antispasticity drug. In the brain, baclofen delivered orally has some supraspinal activity that may contribute to clinical side effects. The main adverse effects of oral baclofen include sedation, excessive weakness, dizziness, mental confusion, and somnolence.(2) The incidence of adverse effects is reported to range from 10% to 75%.(2) Ochs et al. estimated that approximately 25-30% of SCI and MS patients fail to respond to oral baclofen.(3;4) Adverse effects appear to be dose-related and may be minimized by initiating treatment at a low dose and gradually titrating upwards.(2) Adverse effects usually appear at doses >60 mg/day.(2) The rate of treatment discontinuation due to intolerable adverse effects has generally been reported to range from 4% to 27%.(2) When baclofen is administered orally, only a small portion of the

Physical therapy in patients with disorders of consciousness: Impact on spasticity and muscle contracture.

Science.gov (United States)

Thibaut, A; Wannez, S; Deltombe, T; Martens, G; Laureys, S; Chatelle, C

2018-01-01

Spasticity is a frequent complication after severe brain injury, which may prevent the rehabilitation process and worsen the patients' quality of life. In this study, we investigated the correlation between spasticity, muscle contracture, and the frequency of physical therapy (PT) in subacute and chronic patients with disorders of consciousness (DOC). 109 patients with subacute and chronic disorders of consciousness (Vegetative state/Unresponsive wakefulness syndrome - VS/UWS; minimally conscious state - MCS and patients who emerged from MCS - EMCS) were included in the study (39 female; mean age: 40±13.5y; 60 with traumatic etiology; 35 VS/UWS, 68 MCS, 6 EMCS; time since insult: 38±42months). The number of PT sessions (i.e., 20 to 30 minutes of conventional stretching of the four limbs) was collected based on patients' medical record and varied between 0 to 6 times per week (low PT = 0-3 and high PT = 4-6 sessions per week). Spasticity was measured with the Modified Ashworth Scale (MAS) on every segment for both upper (UL) and lower limbs (LL). The presence of muscle contracture was assessed in every joint. We tested the relationship between spasticity and muscle contracture with the frequency of PT as well as other potential confounders such as time since injury or anti-spastic medication intake. We identified a negative correlation between the frequency of PT and MAS scores as well as the presence of muscle contracture. We also identified that patients who received less than four sessions per week were more likely to be spastic and suffer from muscle contracture than patients receiving 4 sessions or more. When separating subacute (3 to 12 months post-insult) and chronic (>12months post-insult) patients, these negative correlations were only observed in chronic patients. A logit regression model showed that frequency of PT influenced spasticity, whereas neither time since insult nor medication had a significant impact on the presence of spasticity. On

Muscle specific changes in length-force characteristics of the calf muscles in the spastic Han-Wistar rat

DEFF Research Database (Denmark)

Olesen, Annesofie Thorup; Jensen, Bente Rona; Uhlendorf, Toni L

2014-01-01

length, passive stiffness and passive force of spastic GA were decreased whereas those of spastic SO were increased. No mechanical interaction between the calf muscles and TA was found. As GA was lengthened, force from SO and PL declined despite a constant muscle-tendon unit length of SO and PL. However......, the extent of this interaction was not different in the spastic rats. In conclusion, the effects of spasticity on length-force characteristics were muscle specific. The changes seen for GA and PL muscles are consistent with the changes in limb mechanics reported for human patients. Our results indicate......The purpose of the present study was to investigate muscle mechanical properties and mechanical interaction between muscles in the lower hindlimb of the spastic mutant rat. Length-force characteristics of gastrocnemius (GA), soleus (SO) and plantaris (PL) were assessed in anesthetized spastic...

TREATMENT OF THE SPASTICITY IN CHILDREN WITH CEREBRAL PALSY

Science.gov (United States)

Meholjić-Fetahović, Ajša

2007-01-01

Botulinum toxin is a natural purified protein and one of the strongest biological poisons - neurotoxin. It is produced by the bacterium Clostridium botulinum. Its medical usage started in USA in 1981 and in Europe in 1992. There are seven different immune types of the toxin: A, B, C1, D, E, F and G. Toxin types A and B are used to decrease muscular spasticity. Botulinum toxin prevents the formation of acetylcholine from cholinergic nerve tissues in muscles, which in the end irreversibly destroys neuromuscular synapses. It is called temporary local chemodenervation. It does not affect the synthesis of acetylcholine. As it affects neuromuscular bond it also affects one of the symptoms of cerebral palsy - spasticity Decreasing the spasticity of children with cerebral palsy leads to the improvement of conscious movements, muscles are less toned, passive mobility is improved, orthosis tolerance is also improved, and the child is enabled to perform easier and better motor functions such as crawling, standing and walking. Since the action of Botulinum toxin is limited to 2-6 months, new neural collaterals are formed and neuromuscular conductivity is reestablished which in the end once again develops a muscular spasm. This leads to a conclusion that botulinum toxin should again be applied into spastic muscles. It is very important for good effect of Botulinum toxin to set the goals of the therapy in advance. The goals include improvement of a function, prevention of contractions and deformities, ease of care and decrease of pain for children with cerebral palsy. After application of botulinum toxin, it is necessary to perform adequate and intensive physical treatment with regular monitoring of effects. This work shows a case of a boy with spastic form of cerebral palsy. After being habilitated using Vojta therapy and Bobath concept and the conduct of certain physical procedures, botulinum toxin is administered into his lower limbs’ muscles and kinezitherapy intensified

Treatment of the Spasticity in Children with Cerebral Palsy

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Ajša Meholjić-Fetahović

2008-11-01

Full Text Available Botulinum toxin is a natural purified protein and one of the strongest biological poisons - neurotoxin. It is produced by the bacterium Clostridium botulinum. Its medical usage started in USA in 1981 and in Europe in 1992. There are seven different immune types of the toxin: A, B, C1, D, E, F and G. Toxin types A and B are used to decrease muscular spasticity. Botulinum toxin prevents the formation of acetylcholine from cholinergic nerve tissues in muscles, which in the end irreversibly destroys neuromuscular synapses. It is called temporary local chemodenervation. It does not affect the synthesis of acetylcholine.As it affects neuromuscular bond it also affects one of the symptoms of cerebral palsy - spasticity Decreasing the spasticity of children with cerebral palsy leads to the improvement of conscious movements, muscles are less toned, passive mobility is improved, orthosis tolerance is also improved, and the child is enabled to perform easier and better motor functions such as crawling, standing and walking. Since the action of Botulinum toxin is limited to 2-6 months, new neural collaterals are formed and neuromuscular conductivity is reestablished which in the end once again develops a muscular spasm. This leads to a conclusion that botulinum toxin should again be applied into spastic muscles.It is very important for good effect of Botulinum toxin to set the goals of the therapy in advance. The goals include improvement of a function, prevention of contractions and deformities, ease of care and decrease of pain for children with cerebral palsy. After application of botulinum toxin, it is necessary to perform adequate and intensive physical treatment with regular monitoring of effects.This work shows a case of a boy with spastic form of cerebral palsy. After being habilitated using Vojta therapy and Bobath concept and the conduct of certain physical procedures, botulinum toxin is administered into his lower limbs’ muscles and

Neuro-musculoskeletal simulation of instrumented contracture and spasticity assessment in children with cerebral palsy.

Science.gov (United States)

van der Krogt, Marjolein Margaretha; Bar-On, Lynn; Kindt, Thalia; Desloovere, Kaat; Harlaar, Jaap

2016-07-16

Increased resistance in muscles and joints is an important phenomenon in patients with cerebral palsy (CP), and is caused by a combination of neural (e.g. spasticity) and non-neural (e.g. contracture) components. The aim of this study was to simulate instrumented, clinical assessment of the hamstring muscles in CP using a conceptual model of contracture and spasticity, and to determine to what extent contracture can be explained by altered passive muscle stiffness, and spasticity by (purely) velocity-dependent stretch reflex. Instrumented hamstrings spasticity assessment was performed on 11 children with CP and 9 typically developing children. In this test, the knee was passively stretched at slow and fast speed, and knee angle, applied forces and EMG were measured. A dedicated OpenSim model was created with motion and muscles around the knee only. Contracture was modeled by optimizing the passive muscle stiffness parameters of vasti and hamstrings, based on slow stretch data. Spasticity was modeled using a velocity-dependent feedback controller, with threshold values derived from experimental data and gain values optimized for individual subjects. Forward dynamic simulations were performed to predict muscle behavior during slow and fast passive stretches. Both slow and fast stretch data could be successfully simulated by including subject-specific levels of contracture and, for CP fast stretches, spasticity. The RMS errors of predicted knee motion in CP were 1.1 ± 0.9° for slow and 5.9 ± 2.1° for fast stretches. CP hamstrings were found to be stiffer compared with TD, and both hamstrings and vasti were more compliant than the original generic model, except for the CP hamstrings. The purely velocity-dependent spasticity model could predict response during fast passive stretch in terms of predicted knee angle, muscle activity, and fiber length and velocity. Only sustained muscle activity, independent of velocity, was not predicted by our model. The

Genetics Home Reference: spastic paraplegia type 7

Science.gov (United States)

... in the arms; speech difficulties (dysarthria); difficulty swallowing (dysphagia); involuntary movements of the eyes (nystagmus); mild hearing ... AH, Warner TT. A clinical, genetic and biochemical study of SPG7 mutations in hereditary spastic paraplegia. Brain. ...

Two Babinski signs in seropositive (HAM and seronegative tropical spastic paraparesis Dos signos de Babinski en pacientes con paraparesia espástica tropical seropositiva (HAM y seronegativa

Directory of Open Access Journals (Sweden)

Fidias E. Leon-Sarmiento

2008-01-01

Full Text Available Tropical spastic paraparesis (TSP may or may not be associated to HTLV-I antibodies and is usually characterized by clinical and pathological spinal cord abnormalities at thoracic levels. We present here five Brazilian patients who had typical chronic idiopatic spastic paraparesis; two of them were HTLV-I seropositive (HAM and three HTLV-I seronegative (TSP - associated-myelopathy. Three out of these five patients also displayed clinical supraspinal involvement, indeed, platysma muscle hypotrophy or atrophy (the Babinski plus sign. These findings support the view that clinical involvement in HAM and TSP is wider than the spinal cord abnormalities usually considered. Possible non-infectious co-factors (e.g., mycotoxins may be involved in disease pathogenesis in a multistep process of viruses, toxins and environment which may account for serological differences found in this group of patients.La paraparesia espástica tropical (PET, puede o no estar asociada con anticuerpos contra el HTLV-I y se caracteriza, usualmente, por alteraciones clínicas y patológicas a nivel de region dorso-lumbar de la medula espinal. Presentamos cinco pacientes brasileros, quienes tuvieron hallazgos típicos de paraparesia espástica crónica idiopática; dos de ellos tuvieron (HAM y tres no tuvieron (TSP anticuerpos, en el suero, contra el HTLV-I. En tres pacientes se encontró hipotrofia o atrofia del músculo platisma (signo de Babinski plus, demostrando que el compromiso clínico en pacientes con HAM y TSP se extiende más allá de la médula espinal torácica. Cofactores (por ejemplo, micotoxinas podrían estar involucrados en la patogénesis de esta enfermedad, en una interacción compleja de virus, toxinas y medio ambiente, lo cual explicaría las diferencias serológicas encontradas en este grupo de pacientes.

Cervical Fusion for Absent Pedicle Syndrome Manifesting with Myelopathy.

Science.gov (United States)

Goodwin, C Rory; Desai, Atman; Khattab, Mohamed H; Elder, Benjamin D; Bydon, Ali; Wolinsky, Jean-Paul

2016-02-01

Absent congenital pedicle syndrome is a posterior arch defect characterized by numerous congenital and mechanical abnormalities that result from disconnection of the anterior and posterior columns of the spinal canal. Absent congenital pedicle syndrome is a rare anomaly that is most commonly diagnosed incidentally, after evaluation of minor trauma, or after complaints of chronic neck pain. We report a case of absent congenital pedicle syndrome in a patient who presented with myelopathy and lower extremity weakness and review the literature on the surgical management of this entity. A 32-year-old woman with a history of systemic lupus erythematosus presented to the Neurosurgery Service with progressive weakness in her upper and lower extremities, clonus, and hyperreflexia. Magnetic resonance imaging revealed congenital absence of the pedicles of C2, C3, C4, C5, and C6 with a congenitally narrow canal at C4-5. The patient underwent a staged anterior and posterior cervical decompression and fusion. She was placed in a halo after surgery; at the 1-year follow-up, she was ambulatory with demonstrated improvement in weakness and fusion of her cervical spine. Absent congenital pedicle syndrome is rare, and most reported cases were treated conservatively. Surgical management is reserved for patients with myelopathy or instability. Copyright © 2016 Elsevier Inc. All rights reserved.

Hindlimb spasticity after unilateral motor cortex lesion in rats is reduced by contralateral nerve root transfer.

Science.gov (United States)

Zong, Haiyang; Ma, Fenfen; Zhang, Laiyin; Lu, Huiping; Gong, Jingru; Cai, Min; Lin, Haodong; Zhu, Yizhun; Hou, Chunlin

2016-12-01

Lower extremity spasticity is a common sequela among patients with acquired brain injury. The optimum treatment remains controversial. The aim of our study was to test the feasibility and effectiveness of contralateral nerve root transfer in reducing post stroke spasticity of the affected hindlimb muscles in rats. In our study, we for the first time created a novel animal hindlimb spastic hemiplegia model in rats with photothrombotic lesion of unilateral motor cortex and we established a novel surgical procedure in reducing motor cortex lesion-induced hindlimb spastic hemiplegia in rats. Thirty six rats were randomized into three groups. In group A, rats received sham operation. In group B, rats underwent unilateral hindlimb motor cortex lesion. In group C, rats underwent unilateral hindlimb cortex lesion followed by contralateral L4 ventral root transfer to L5 ventral root of the affected side. Footprint analysis, Hoffmann reflex (H-reflex), cholera toxin subunit B (CTB) retrograde tracing of gastrocnemius muscle (GM) motoneurons and immunofluorescent staining of vesicle glutamate transporter 1 (VGLUT1) on CTB-labelled motoneurons were used to assess spasticity of the affected hindlimb. Sixteen weeks postoperatively, toe spread and stride length recovered significantly in group C compared with group B (Pmotor cortex lesion-induced hindlimb spasticity in rats. Our data indicated that this could be an alternative treatment for unilateral lower extremity spasticity after brain injury. Therefore, contralateral neurotization may exert a potential therapeutic candidate to improve the function of lower extremity in patients with spastic hemiplegia. © 2016 The Author(s).

Whole plant cannabis extracts in the treatment of spasticity in multiple sclerosis: a systematic review

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Rowland Marie

2009-12-01

Full Text Available Abstract Background Cannabis therapy has been considered an effective treatment for spasticity, although clinical reports of symptom reduction in multiple sclerosis (MS describe mixed outcomes. Recently introduced therapies of combined Δ9-tetrahydrocannabinol (THC and cannabidiol (CBD extracts have potential for symptom relief with the possibility of reducing intoxication and other side effects. Although several past reviews have suggested that cannabinoid therapy provides a therapeutic benefit for symptoms of MS, none have presented a methodical investigation of newer cannabinoid treatments in MS-related spasticity. The purpose of the present review was to systematically evaluate the effectiveness of combined THC and CBD extracts on MS-related spasticity in order to increase understanding of the treatment's potential effectiveness, safety and limitations. Methods We reviewed MEDLINE/PubMed, Ovid, and CENTRAL electronic databases for relevant studies using randomized controlled trials. Studies were included only if a combination of THC and CBD extracts was used, and if pre- and post-treatment assessments of spasticity were reported. Results Six studies were systematically reviewed for treatment dosage and duration, objective and subjective measures of spasticity, and reports of adverse events. Although there was variation in the outcome measures reported in these studies, a trend of reduced spasticity in treated patients was noted. Adverse events were reported in each study, however combined TCH and CBD extracts were generally considered to be well-tolerated. Conclusion We found evidence that combined THC and CBD extracts may provide therapeutic benefit for MS spasticity symptoms. Although some objective measures of spasticity noted improvement trends, there were no changes found to be significant in post-treatment assessments. However, subjective assessment of symptom relief did often show significant improvement post-treatment. Differences in

Reduction in spasticity in stroke patient with paraffin therapy.

Science.gov (United States)

Wang, Jing; Yu, Peng; Zeng, Ming; Gu, Xudong; Liu, Yan; Xiao, Mingyue

2017-01-01

The aim of the study was to confirm whether paraffin therapy offer clinical value in the treatment of spasticity due to stroke. Fifty-two patients with spasticity in the upper limb were included. The patients were randomized into the experimental group with paraffin therapy (n = 27) and the control group with placebo therapy (n = 25). The outcome measures besides temperature examination were undertaken at time points of 0 (T0), 2 (T1) and 4 weeks (T2) following therapy treatment. The extent of spasticity was measured using Modified Ashworth Score (MAS) during passive movement at the shoulder, elbow, wrist and finger joints. Visual analogue scale (VAS) was used to evaluate the hemiplegic upper limb pain and functional activity of the upper limb motor function was evaluated by Brunnstrom recovery stage. All adverse events were recorded. MAS decreased significantly in Exp group compared with Con group, at the time points of T1 and T2, both before and immediately after paraffin therapy. Paraffin treatment failed to show remarkable improvement in pain compared with placebo-treated patient at movement at any time point. But VAS in Exp exhibited a tendency to decrease over time in shoulder, elbow, wrist and hand. With regard to the Brunnstrom score, patients in Exp showed significant improvement at the end of trial compared to the beginning. The values of temperature showed significant increment immediately after paraffin therapy at each time point in Exp group. Paraffin therapy may be a kind of noninvasive, promising method to reduce spasticity of stroke patients.

Radiation-induced osteochondroma of the T4 vertebra causing spinal cord compression

Energy Technology Data Exchange (ETDEWEB)

Gorospe, Luis; Madrid-Muniz, Carmen; Royo, Aranzazu; Garcia-Raya, Pilar [Department of Radiology, La Paz University Hospital, Madrid (Spain); Alvarez-Ruiz, Fernando [Department of Neurosurgery, La Paz University Hospital, Madrid (Spain); Lopez-Barea, Fernando [Department of Pathology, La Paz University Hospital, Madrid (Spain)

2002-04-01

A case of a radiation-induced osteochondroma arising from the vertebral body of T4 in an 18-year-old man is reported. The patient presented with a history of progressive left lower extremity weakness. At 7 years of age, he had undergone resection of a cerebellar medulloblastoma and received adjunctive craniospinal irradiation and systemic chemotherapy. Both CT and MR imaging revealed an extradural mass contiguous with the posteroinferior endplate of the T4 vertebral body. This case indicates that radiation-induced osteochondroma should be considered in the differential diagnosis of patients with symptoms of myelopathy or nerve root compression and a history of radiation therapy involving the spine in childhood. (orig.)

Influence of post-stroke spasticity on EMG-force coupling and force steadiness in biceps brachii.

Science.gov (United States)

Carlyle, Jennilee K; Mochizuki, George

2018-02-01

Individuals with spasticity after stroke experience a decrease in force steadiness which can impact function. Alterations in the strength of EMG-force coupling may contribute to the reduction in force steadiness observed in spasticity. The aim was to determine the extent to which force steadiness and EMG-force coupling is affected by post-stroke spasticity. This cross-sectional study involved individuals with upper limb spasticity after stroke. Participants were required to generate and maintain isometric contractions of the elbow flexors at varying force levels. Coefficient of variation of force, absolute force, EMG-force cross-correlation function peak and peak latency was measured from both limbs with surface electromyography and isometric dynamometry. Statistically significant differences were observed between the affected and less affected limbs for all outcome measures. Significant main effects of force level were also observed. Force steadiness was not statistically significantly correlated with EMG-force coupling; however, both force steadiness and absolute force were associated with the level of impairment as measured by the Chedoke McMaster Stroke Assessment Scale. Spasticity after stroke uncouples the relationship between EMG and force and is associated with reduced force steadiness during isometric contractions; however, these features of control are not associated in individuals with spasticity. Copyright © 2017 Elsevier Ltd. All rights reserved.

NIPA1 mutation in complex hereditary spastic paraplegia with epilepsy

DEFF Research Database (Denmark)

Svenstrup, K; Møller, R S; Christensen, J

2011-01-01

or signs are found. Mutations in the NIPA1 gene have been reported to cause spastic paraplegia type 6 (SPG6) in 10 families. SPG6 is a rare form of autosomal dominantly inherited HSP associated with a pure phenotype; however, in one complex SPG6 family, idiopathic generalized epilepsy (IGE) has been...... described and in addition, recurrent microdeletions at 15q11.2 including NIPA1 have been identified in patients with IGE. The purpose was to identify NIPA1 mutations in patients with pure and complex HSP. Methods: Fifty-two patients with HSP were screened for mutations in NIPA1. Results: One previously...... reported missense mutation c.316G>A, p.Gly106Arg, was identified in a complex HSP patient with spastic dysarthria, facial dystonia, atrophy of the small hand muscles, upper limb spasticity, and presumably IGE. The epilepsy co-segregated with HSP in the family. Conclusion: NIPA1 mutations were rare in our...

A literature review on the efficacy and safety of botulinum toxin: An injection in post-stroke spasticity

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Majid Ghasemi

2013-01-01

Full Text Available Background: A variety of techniques for the management of spasticity have been suggested, including positioning, cryotherapy, splinting and casting, biofeedback, electrical stimulation, and medical management by pharmacological agents, Botulinum toxin A (BTA is now the pharmacological treatment of choice in focal spasticity. BTA by blocking acetylcholine release at neuromuscular junctions accounts for its therapeutic action to relieve spasticity. Methods: A computerized search of Pub Med was carried out to find the latest result about efficacy of BTA in management of post stroke spasticity. Result: Among 84 articles were found, frothy of them included in this review and divided to lower and upper extremity. Conclusions: BTA is a treatment choice in reducing tone and managing post stroke spasticity .

Clinical and neuroimaging profile of congenital brain malformations in children with spastic cerebral palsy

International Nuclear Information System (INIS)

Kulak, W.; Okurowska-Zawada, B.; Sobaniec, W.; Goscik, M.; Olenski, J.

2008-01-01

Purpose: Analysis of the incidence of congenital brain malformations in children with spastic cerebral palsy (CP) in a hospital based study. Material and Methods: The present study included 74 boys and 56 girls with spastic tetraplegia, diplegia, and hemiplegia CP. Magnetic resonance imaging MRI findings were analyzed in children with CP. Results: Significant abnormalities relevant to the CP were evident on MRI in 124 (95.3%) subjects. Periventicular leukomalacia (PVL) was detected more frequently in children with spastic diplegia than in patients with tetraplegia or hemiplegia. Cerebral atrophy was found more often in the tetraplegic group compared to the diplegic patients. Porencephalic cysts were detected more often in children with spastic hemiplegia. Congenital brain anomalies were evident in 15 (10.7%) children with spastic CP. Brain malformations included: schizencephaly (5), agenesis corpus callosum (4), polymicrogyria (2), holoprosencephaly (2) and lissencephaly (2). Intractable epilepsy and mental retardation were observed more often in children with brain anomalies. Twelve patients with congenital brain malformations were born at term and three born at preterm. Conclusions: Neuroimaging results in children with CP may help determine the etiology and make better prognosis of CP. (authors)

Mania associated with complicated hereditary spastic paraparesis

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Raghavendra B Nayak

2011-01-01

Full Text Available Hereditary spastic paraparesis (HSP is an inherited group of neurological disorders with progressive lower limb spasticity. HSP can be clinically grouped into pure and complicated forms. Pure HSP is one without any associated neurological/psychiatric comorbidity. Depression is the most common psychiatric comorbidity. Presence of mania or bipolar affective illness with HSP is a rare phenomenon. We report a case of a 17-year-old boy who presented with classical features of HSP with complaints of excessive happiness, irritability, increased self-esteem and decreased sleep since 1 month. The patient also had complex partial seizure ever since he had features of HSP. The patient′s father and younger sister suffer from pure HSP. The patient was diagnosed to have first episode mania with complicated HSP. The details of treatment and possible neurobiology are discussed in this case report.

Mania associated with complicated hereditary spastic paraparesis.

Science.gov (United States)

Nayak, Raghavendra B; Bhogale, Govind S; Patil, Nanasaheb M; Pandurangi, Aditya A

2011-07-01

Hereditary spastic paraparesis (HSP) is an inherited group of neurological disorders with progressive lower limb spasticity. HSP can be clinically grouped into pure and complicated forms. Pure HSP is one without any associated neurological/psychiatric comorbidity. Depression is the most common psychiatric comorbidity. Presence of mania or bipolar affective illness with HSP is a rare phenomenon. We report a case of a 17-year-old boy who presented with classical features of HSP with complaints of excessive happiness, irritability, increased self-esteem and decreased sleep since 1 month. The patient also had complex partial seizure ever since he had features of HSP. The patient's father and younger sister suffer from pure HSP. The patient was diagnosed to have first episode mania with complicated HSP. The details of treatment and possible neurobiology are discussed in this case report.

SPG20 mutation in three siblings with familial hereditary spastic paraplegia.

Science.gov (United States)

Dardour, Leila; Roelens, Filip; Race, Valerie; Souche, Erika; Holvoet, Maureen; Devriendt, Koen

2017-07-01

Troyer syndrome (MIM#275900) is an autosomal recessive form of complicated hereditary spastic paraplegia. It is characterized by progressive lower extremity spasticity and weakness, dysarthria, distal amyotrophy, developmental delay, short stature, and subtle skeletal abnormalities. It is caused by deleterious mutations in the SPG20 gene, encoding spartin, on Chromosome 13q13. Until now, six unrelated families with a genetically confirmed diagnosis have been reported. Here we report the clinical findings in three brothers of a consanguineous Moroccan family, aged 24, 17, and 7 yr old, with spastic paraplegia, short stature, motor and cognitive delay, and severe intellectual disability. Targeted exon capture and sequencing showed a homozygous nonsense mutation in the SPG20 gene, c.1369C>T (p.Arg457*), in the three affected boys. © 2017 Dardour et al.; Published by Cold Spring Harbor Laboratory Press.

Flexion synergy overshadows flexor spasticity during reaching in chronic moderate to severe hemiparetic stroke.

Science.gov (United States)

Ellis, Michael D; Schut, Ingrid; Dewald, Julius P A

2017-07-01

Pharmaceutical intervention targets arm flexor spasticity with an often-unsuccessful goal of improving function. Flexion synergy is a related motor impairment that may be inadvertently neglected. Here, flexor spasticity and flexion synergy are disentangled to determine their contributions to reaching dysfunction. Twenty-six individuals participated. A robotic device systematically modulated shoulder abduction loading during ballistic reaching. Elbow muscle electromyography data were partitioned into windows delineated by elbow joint velocity allowing for the separation of synergy- and spasticity-related activation. Reaching velocity decreased with abduction loading (psynergy increased with abduction loading (psynergy is the predominant contributor to reaching dysfunction while flexor spasticity appears only relevant during unnaturally occurring passively supported movement. Interventions targeting flexion synergy should be leveraged in future stroke recovery trials. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Outcomes in spasticity after repetitive transcranial magnetic and transcranial direct current stimulations

OpenAIRE

Gunduz, Aysegul; Kumru, Hatice; Pascual-Leone, Alvaro

2014-01-01

Non-invasive brain stimulations mainly consist of repetitive transcranial magnetic stimulation and transcranial direct current stimulation. Repetitive transcranial magnetic stimulation exhibits satisfactory outcomes in improving multiple sclerosis, stroke, spinal cord injury and cerebral palsy-induced spasticity. By contrast, transcranial direct current stimulation has only been studied in post-stroke spasticity. To better validate the efficacy of non-invasive brain stimulations in improving ...

Post-activation depression of soleus stretch reflexes in healthy and spastic humans

DEFF Research Database (Denmark)

Grey, Michael James; Klinge, Klaus; Crone, Clarissa

2007-01-01

Reduced depression of transmitter release from Ia afferents following previous activation (post-activation depression) has been suggested to be involved in the pathophysiology of spasticity. However, the effect of this mechanism on the myotatic reflex and its possible contribution to increased...... reflex excitability in spastic participants has not been tested. To investigate these effects, we examined post-activation depression in Soleus H-reflex responses and in mechanically evoked Soleus stretch reflex responses. Stretch reflex responses were evoked with consecutive dorsiflexion perturbations...... of the soleus stretch reflex and H-reflex decreased as the interval between the stimulus/perturbation was decreased. Similarly, the stretch-evoked torque decreased. In the spastic participants, the post-activation depression of both reflexes and the stretch-evoked torque was significantly smaller than...

Lipid myopathy associated with renal tubular acidosis and spastic diplegia in two brothers.

Science.gov (United States)

Tung, Y C; Tsau, Y K; Chu, L W; Young, C; Shen, Y Z

2001-07-01

Lipid myopathy is a group of disorders involving mitochondrial fatty acid oxidation. We describe two brothers, 3 years 8 months old and 2 years 9 months old, respectively, with progressive spastic diplegia, developmental delay, failure to thrive, and chronic metabolic acidosis who had lipid myopathy and renal tubular acidosis. Brain magnetic resonance imaging revealed demyelinating changes in the periventricular white matter, which was compatible with spastic diplegia. These symptoms may be related to errors in fatty acid metabolism. Cerebral palsy had been misdiagnosed in both of these patients at another hospital. Therefore, for patients with late-onset and progressive spastic diplegia, detailed investigations for underlying diseases are warranted.

SACS gene-related autosomal recessive spastic ataxia of Charlevoix-Saguenay from South India

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M Suraj Menon

2016-01-01

Full Text Available Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS is a neurodegenerative disorder characterized by late infantile onset spastic ataxia and other neurological features. Initially described in the Charlevoix-Saguenay region of Quebec, Canada, it is being increasingly reported from many other countries. Here, we present the case of a 20-year-old male from South India, who presented with progressive ataxia, spasticity, and peripheral neuropathy with imaging features and genetic testing suggestive of SACS gene-related ARSACS. The phenotypic variability from other cases and occurrence in a geographically distinct region is stressed upon to alert the clinicians to consider ARSACS in progressive ataxias.

Physiotherapy for human T-lymphotropic virus 1-associated myelopathy: review of the literature and future perspectives

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Sá KN

2015-02-01

Full Text Available Katia N Sá,1 Maíra C Macêdo,1 Rosana P Andrade,2 Selena D Mendes,1 José V Martins,3 Abrahão F Baptista1,4 1Neuromusculoskeletal Research Group, Bahian School of Medicine and Human Health, Salvador, Brazil; 2Edgard Santos University Hospital, Federal University of Bahia, Salvador, 3Deolindo Couto Institute of Neurology, Federal University of Rio de Janeiro, Rio de Janeiro, 4Biomorphology Department, Health Sciences Institute, Federal University of Bahia, Salvador, Brazil Abstract: Human T-lymphotropic virus 1 (HTLV-1 infection may be associated with damage to the spinal cord – HTLV-associated myelopathy/tropical spastic paraparesis – and other neurological symptoms that compromise everyday life activities. There is no cure for this disease, but recent evidence suggests that physiotherapy may help individuals with the infection, although, as far as we are aware, no systematic review has approached this topic. Therefore, the objective of this review is to address the core problems associated with HTLV-1 infection that can be detected and treated by physiotherapy, present the results of clinical trials, and discuss perspectives on the development of knowledge in this area. Major problems for individuals with HTLV-1 are pain, sensory-motor dysfunction, and urinary symptoms. All of these have high impact on quality of life, and recent clinical trials involving exercises, electrotherapeutic modalities, and massage have shown promising effects. Although not influencing the basic pathologic disturbances, a physiotherapeutic approach seems to be useful to detect specific problems related to body structures, activity, and participation related to movement in HTLV-1 infection, as well as to treat these conditions. Keywords: HTLV-1, HAM/TSP, physical therapy modalities, quality of life, pain, sensory-motor dysfunction, urinary symptoms

Cautious Use of Intrathecal Baclofen in Walking Spastic Patients: Results on Long-term Follow-up.

Science.gov (United States)

Dones, Ivano; Nazzi, Vittoria; Tringali, Giovanni; Broggi, Giovanni

2006-04-01

Intrathecal baclofen is presently the most effective treatment for diffuse spasticity whatever the cause. The fact that both spasticity is always accompanied by a degree of muscle weakness and that any antispastic treatment causes a decrease in muscle strength indicate that major attention must be paid in treating spasticity in ambulant patients. Methods.  We present here a retrospective study, approved by the insitutional ethics committee, of 22 ambulant spastic patients, selected as homogeneous for disease and disease duration, who were treated with intrathecal baclofen at the Istituto Nazionale Neurologico "C.Besta" in Milan. These patients were followed-up for to 15 years of treatment and their clinical assessment was enriched by the evaluation of their functional independence measurement (FIM) before and during treatment. Results.  There was improvement in quality of life as measured by the FIM scale; however, an increase in the patient's motor performance could not be detected. Conclusion.  Although we did not show any improvement in muscle performance, intrathecal baclofen did improve daily quality of life, even in spastic patients who were able to walk.

Intracranial dural arterio-venous fistula presenting with progressive myelopathy.

LENUS (Irish Health Repository)

Ogbonnaya, Ebere Sunny

2011-01-01

Spinal dural arterio-venous fistula (DAVF) is rare and usually involves the thoracic segments. The classical presentation is a slowly progressive ataxia. Clinical presentation of intracranial DAVF depends on the site of the DAVF, as well as the vessels involved. Patients may present with pulsatile tinnitus, occipital bruit, headache, dementia, visual impairment as well as neurological deterioration distant from the DAVF as a result of venous hypertension and cortical haemorrhage. The authors present a rare case of progressive myelopathy secondary to an intracranial DAVF.

Computed tomographic findings in children with spastic diplegia

International Nuclear Information System (INIS)

Yokochi, Kenji; Horie, Masayo; Inukai, Kazuhisa; Kito, Hideyuki; Shimabukuro, Satoshi; Kodama, Kazuo.

1989-01-01

Computed tomographic findings of 46 children with spastic diplegia examined at nine months to three years of age corrected for preterm births were analyzed. Both the size of the lateral ventricles measured by the width of the anterior horns, and the volume of the extracerebral low-density areas were enlarged in some patiens. Both enlargements did not, however, correlate to the severity of the motor abnormality in the patients. The low-density areas of the periventricular white matter, especially adjacent to the trigone, were reduced in many children, probably due to the atrophy of the cerebral white matter having periventricular leukomalacia. The anterior expansion of the white matter reduction from the trigone corresponded to the severer motor abnormality in the children with spastic diplegia. (author)

Operative techniques for cervical radiculopathy and myelopathy.

LENUS (Irish Health Repository)

Moran, C

2012-02-01

The surgical treatment of cervical spondylosis and resulting cervical radiculopathy or myelopathy has evolved over the past century. Surgical options for dorsal decompression of the cervical spine includes the traditional laminectomy and laminoplasty, first described in Asia in the 1970\\'s. More recently the dorsal approch has been explored in terms of minimally invasive options including foraminotomies for nerve root descompression. Ventral decompression and fusion techniques are also described in the article, including traditional anterior cervical discectomy and fusion, strut grafting and cervical disc arthroplasty. Overall, the outcome from surgery is determined by choosing the correct surgery for the correct patient and pathology and this is what we hope to explain in this brief review.

Hereditary spastic paraplegias: membrane traffic and the motor pathway.

Science.gov (United States)

Blackstone, Craig; O'Kane, Cahir J; Reid, Evan

2011-01-01

Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent work aimed at elucidating the molecular cell biology underlying the HSPs has revealed the importance of basic cellular processes — especially membrane trafficking and organelle morphogenesis and distribution— in axonal maintenance and degeneration.

Intrathecal baclofen therapy in children with intractable spastic cerebral palsy: a cost-effectiveness analysis

NARCIS (Netherlands)

Hoving, M.A.; Evers, S.M.A.A.; Ament, A.J.H.A.; van Raak, E.P.M.; Becher, J.G.; Rotteveel, J.; Vles, J.S.H.

2008-01-01

In a Dutch national study, we recently established the effectiveness and safety of continuous intrathecal baclofen infusion (CITB) in children with intractable spastic cerebral palsy (CP). Because prospective studies on the cost-effectiveness of CITB in children with spastic CP are lacking, we

Intrathecal baclofen therapy in children with intractable spastic cerebral palsy: a cost-effectiveness analysis.

NARCIS (Netherlands)

Hoving, M.A.; Evers, S.M.; Ament, A.J.; Raak, E.P. van; Vles, J.S.; Becher, J.G.; Vermeulen, R.; Brouwer, O.F.; Maathuis, C.G.B.; Catsman-Berrevoets, C.E.; Gerritsen, J.; Geerts, M.J.; Jongerius, P.H.; Nieuwenhuizen, O.F.; Rotteveel, J.J.; Speth, L.A.; Stroink, H.; Ziel, E.G. van der

2008-01-01

In a Dutch national study, we recently established the effectiveness and safety of continuous intrathecal baclofen infusion (CITB) in children with intractable spastic cerebral palsy (CP). Because prospective studies on the cost-effectiveness of CITB in children with spastic CP are lacking, we

Association of Early-Onset Spasticity and Risk for Cognitive Impairment With Mutations at Amino Acid 499 in SPAST.

Science.gov (United States)

Gillespie, Meredith K; Humphreys, Peter; McMillan, Hugh J; Boycott, Kym M

2018-04-01

Hereditary spastic paraplegia is a phenotypically and genetically heterogeneous group of neurodegenerative disorders characterized by lower extremity weakness and spasticity. Spastic paraplegia 4 (SPG4), caused by heterozygous mutations in the gene SPAST, typically causes a late-onset, uncomplicated form of hereditary spastic paraplegia in affected individuals. Additional clinical features in SPG4 have been reported on occasion, but no genotype-phenotype correlation has been established. Through targeted clinical testing, we identified 2 unrelated female patients with the same de novo p.Arg499His mutation in SPAST. Both patients presented with early-onset spasticity resulting in delayed motor milestones, which led to a diagnosis of cerebral palsy in one child and tethered cord in the other. Review of the literature identified several patients with mutations at amino acid 499 and early-onset symptoms associated with a risk of cognitive impairment. Early and accurate diagnosis of children with early-onset spasticity is important for informed prognosis and genetic counselling.

HTLV-I Associated uveitis, myelopathy, rheumatoid arthritis and Sjögren's syndrome Uveite, mielopatia, artrite reumatóide e sindrome de Sjogren associadas ao HTLV-I

Directory of Open Access Journals (Sweden)

Sônia Regina A. A. Pinheiro

1995-12-01

Full Text Available A 62 year-old white female presented with a 10-year-history of slowly progressive spastic paraparesis, pain and dysesthesia in the lower limbs and sphincter disturbance. A few years after the onset of the neurologic symptoms she developped migratory arthritis with swelling of the knees and pain on palpation of knees and fingers, dry eyes, mouth and skin. Two months before admission she presented bilateral nongranulomatous anterior uveitis. Examination revealed spastic paraparesis with bilateral Babinski sign, a decreased sensation level below L3, decreased vibration sense in the lower extremities, and a postural tremor of the upper limbs. Laboratory work-up disclosed HTLV-I positive tests in the blood and cerebrospinal fluid (CSF, and a mild pleocytosis in the CSF with a normal protein content. Nerve conduction velocity studies were normal. The present case shows the association of uveitis, arthritis and Sjögren's syndrome in a patient with tropical spastic paraparesis / human T-cell lymphotropic virus type I (HTLV-I associated myelopathy (TSP/HAM, and illustrates the wide spectrum of clinical manifestations which may accompany this infection with this virus.Uma mulher branca de 62 anos foi internada apresentando história de paraparesia lentamente progressiva durante 10 anos. Dois meses antes da internação ela apresentou uveíte anterior não granulomatosa bilateral. Poucos anos após o início dos sintomas neurológicos, ela desenvolveu artrite migratória com edema dos joelhos e dor a palpação dos joelhos e dedos dos pés, boca, pele e olhos secos. Ao exame físico foi observado paraparesia espástica com sinal de Babinski positivo, sensibilidade diminuída abaixo de L3, diminuição da sensação de vibração nas extremidades inferiores, e tremor postural dos membros superiores. Apresentou testes positivos para o HTLV-I no sangue. O estudo do líquido cefalorraquidiano mostrou discreta pleocitose, proteínas normais e ELISA e Western

Effects of Prolonged Standing on Gait in Children with Spastic Cerebral Palsy

Science.gov (United States)

Salem, Yasser; Lovelace-Chandler, Venita; Zabel, Reta J.; McMillan, Amy Gross

2010-01-01

The purpose of this study was to determine the effects of prolonged standing on gait characteristics in children with spastic cerebral palsy. Six children with spastic cerebral palsy participated in this study with an average age of 6.5 years (SD = 2.5, range = 4.0-9.8 years). A reverse baseline design (A-B-A) was used over a 9-week period. During…

Treatment diary for botulinum toxin spasticity treatment

DEFF Research Database (Denmark)

Biering-Sørensen, Bo; Iversen, Helle K; Frederiksen, Inge M S

2017-01-01

The aim of this study is to develop a treatment diary for patients receiving spasticity treatment including botulinum toxin injection and physiotherapy and/or occupational therapy. The diary focuses on problems triggered by skeletal muscle overactivity; agreed goals for treatment and the patient...

Influence of Magnetic Resonance Imaging Features on Surgical Decision-Making in Degenerative Cervical Myelopathy: Results from a Global Survey of AOSpine International Members.

Science.gov (United States)

Nouri, Aria; Martin, Allan R; Nater, Anick; Witiw, Christopher D; Kato, So; Tetreault, Lindsay; Reihani-Kermani, Hamed; Santaguida, Carlo; Fehlings, Michael G

2017-09-01

We conducted a survey to understand how specific pathologic features on magnetic resonance imaging (MRI) influence surgeons toward an anterior or posterior surgical approach in degenerative cervical myelopathy (DCM). A questionnaire was sent out to 6179 AOSpine International members via e-mail. This included 18 questions on a 7-point Likert scale regarding how MRI features influence the respondent's decision to perform an anterior or posterior surgical approach. Influence was classified based on the mean and mode. Variations in responses were assessed by region and training. Of 513 respondents, 51.7% were orthopedic surgeons, 36.8% were neurosurgeons, and the remainder were fellows, residents, or other. In ascending order, multilevel bulging disks, cervical kyphosis, and a high degree of anterior cord compression had a moderate to strong influence toward an anterior approach. A high degree of posterior cord compression had a moderate to strong influence, whereas multilevel compression, ossification of the posterior longitudinal ligament, ligamentum flavum enlargement, and congenital stenosis had a moderate influence toward a posterior approach. Neurosurgeons chose anterior approaches more and posterior approaches less in comparison with orthopedic surgeons (P influences the choice for anterior or posterior surgical approach. These data highlight factors based on surgeon experience, training, and region of practice. They will be helpful in defining future areas of investigation in an effort to provide individualized surgical strategies and optimize patient outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

Fibrocartilaginous embolic myelopathy: demographics, clinical presentation, and functional outcomes.

Science.gov (United States)

Moore, Brittany J; Batterson, Anna M; Luetmer, Marianne T; Reeves, Ronald K

2018-05-25

Retrospective cohort study. To describe the demographics, clinical presentation, and functional outcomes of fibrocartilaginous embolic myelopathy (FCEM). Academic inpatient rehabilitation unit in the midwestern United States. We retrospectively searched our database to identify patients admitted between January 1, 1995 and March 31, 2016, with a high probability of FCEM. Demographic, clinical, and functional outcome measures, including Functional Independence Measure (FIM) information was obtained by chart review. We identified 31 patients with findings suggestive of FCEM (52% male), which was 2% of the nontraumatic spinal cord injury population admitted to inpatient rehabilitation. The age distribution was bimodal, with peaks in the second and sixth-to-seventh decades. The most common clinical presentation was acute pain and rapid progression of neurologic deficits consistent with a vascular myelopathy. Only three patients (10%) had FCEM documented as a diagnostic possibility. Most patients had paraplegia and neurologically incomplete injuries and were discharged to home. Nearly half of the patients required no assistive device for bladder management at discharge, but most were discharged with medications for bowel management. Median FIM walking locomotion score for all patients was 5, but most patients were discharged using a wheelchair for primary mobility. Median motor FIM subscale score was 36 at admission and 69 at discharge, with a median motor efficiency of 1.41. FCEM may be underdiagnosed and should be considered in those with the appropriate clinical presentation, because their functional outcomes may be more favorable than those with other causes of spinal cord infarction.

[H reflex in patients with spastic quadriplegia].

Science.gov (United States)

Miyama, Sahoko; Arimoto, Kiyoshi; Kimiya, Satoshi

2009-01-01

Hoffmann reflex (H reflex) is an electrically elicited spinal monosynaptic reflex. H reflex was examined in 18 patients with spastic quadriplegia who had perinatal or postnatal problems. H reflex was elicitable in 11 patients for the abductor pollicis brevis (61.1%), 10 for the abductor digiti minimi (55.6%) and 16 for the abductor hallucis (88.9%). Because the abductor pollicis brevis and the abductor digiti minimi do not exhibit H reflex in normal subjects, it was suggested that the excitability of alpha motor neurons innervating these muscles was increased. H reflex was not detected for the extensor digitorum brevis in any patients, indicating the difference in the excitability among alpha motor neurons. In some patients, H reflex did not disappear under supramaximal stimuli. We conclude that the mechanism of evolution of H reflex in patients with spastic quadriplegia is different from that in normal subjects.

Cannabinoids in the management of spasticity associated with multiple sclerosis

Directory of Open Access Journals (Sweden)

Anna Maria Malfitano

2008-08-01

Full Text Available Anna Maria Malfitano, Maria Chiara Proto, Maurizio BifulcoDipartimento di Scienze Farmaceutiche, Università degli Studi di SalernoAbstract: The endocannabinoid system and cannabinoid-based treatments have been involved in a wide number of diseases. In particular, several studies suggest that cannabinoids and endocannabinoids may have a key role in the pathogenesis and therapy of multiple sclerosis (MS. In this study we highlight the main findings reported in literature about the relevance of cannabinoid drugs in the management and treatment of MS. An increasing body of evidence suggests that cannabinoids have beneficial effects on the symptoms of MS, including spasticity and pain. In this report we focus on the effects of cannabinoids in the relief of spasticity describing the main findings in vivo, in the mouse experimental allergic encephalomyelitis model of MS. We report on the current treatments used to control MS symptoms and the most recent clinical studies based on cannabinoid treatments, although long-term studies are required to establish whether cannabinoids may have a role beyond symptom amelioration in MS.Keywords: cannabinoids, multiple sclerosis, spasticity

Anterolisthesis and retrolisthesis of the cervical spine in cervical spondylotic myelopathy in the elderly

International Nuclear Information System (INIS)

Kawasaki, Motohiro; Tani, Toshikazu; Ushida, Takahiro; Ishida, Kenji

2007-01-01

Degenerative spondylolisthesis of the cervical spine has received insufficient attention in contrast to that of the lumbar spine. The authors analyzed the functional significance of anterior and posterior degenerative spondylolisthesis (anterolisthesis and retrolisthesis) of the cervical spine to elucidate its role in the development of cervical spondylotic myelopathy (CSM) in the elderly. A total of 79 patients aged 65 or older who eventually had surgical treatment for CSM were evaluated radiographically. Altogether, 24 patients (30%) had displacement of 3.5 mm or more (severe spondylolisthesis group), 31 had displacement of 2.0-3.4 mm (moderate spondylolisthesis group), and 24 had less than 2.0 mm displacement (mild spondylolisthesis group). The severe spondylolisthesis group consisted of 14 patients with anterolisthesis (anterolisthesis group) and 10 patients with retrolisthesis (retrolisthesis group). Patients with severe spondylolisthesis had a high incidence (93%) of degenerative spondylolisthesis at C3/4 or C4/5 and significantly greater cervical mobility than those with mild spondylolisthesis. The anterolisthesis group, but not the retrolisthesis group, had a significantly wider spinal canal than the mild spondylolisthesis group, although the degree of horizontal displacement and cervical mobility did not differ significantly between the anterolisthesis and retrolisthesis groups. Severe cord compression seen on T1-weighted magnetic resonance imaging (MRI) scans and high-intensity spinal cord signals seen on T2-weighted MRI scans corresponded significantly to the levels of the spondylolisthesis. Degenerative spondylolisthesis is not a rare radiographic finding in elderly patients with CSM, which tends to cause intense cord compression that is seen on MRI scans. Greater mobility of the upper cervical segments may be a compensatory reaction for advanced disc degeneration of the lower cervical segments, leading to the development of degenerative

Gait Trainer for Children with Spastic Cerebral Palsy

National Research Council Canada - National Science Library

Urhan, Oguzhan

2001-01-01

A device is developed to improve the walking ability of children with Spastic Cerebral Palsy, who have damages to the area of their brain which controls the muscle tone and that causes trouble walking...

Co-segregation of Huntington disease and hereditary spastic paraplegia in 4 generations.

Science.gov (United States)

Panas, Marios; Karadima, Georgia; Kalfakis, Nikolaos; Vassilopoulos, Dimitris

2011-07-01

Huntington disease (HD) is an autosomal dominant neurodegenerative disease characterized by choreic hyperkinesias, cognitive decline, and psychiatric manifestations, caused by an increased number of CAG repeats in the IT15 gene on chromosome 4p16.3. Silver syndrome is a rare autosomal dominant form of complicated hereditary spastic paraplegia, characterized by lower limb spasticity in addition to amyotrophy of the small muscles of the hands. In addition to the previously identified locus SPG17 on chromosome 11q12-q14, a new locus (SPG38) on chromosome 4p16-p15 has been recently identified, a region that includes the HD gene. We present a Greek family with 5 members diagnosed with HD in 4 generations. All affected members also presented with clinical features of Silver syndrome showing severe spastic paraplegia and prominent atrophy of all small hand muscles bilaterally. None of the other family members showed features of either HD or spastic paraplegia. The reported coexistence of Silver syndrome with HD in 4 generations is not fortuitous, suggesting that these 2 distinct genetic disorders are in linkage disequilibrium. Although rare, it is reasonable to expect additional similar cases. Clinical neurologists should perhaps investigate this possibility in cases combining features of HD and involvement of the upper and lower motor neurons.

Vertebral Arteriovenous Fistula Presenting as Cervical Myelopathy: A Rapid Recovery with Balloon Embolization

International Nuclear Information System (INIS)

Modi, Manish; Bapuraj, J. Rajiv; Lal, Anupam; Prabhakar, S.; Khandelwal, N.

2010-01-01

A 24-year-old male presented with progressive cervical myelopathy of 2 months' duration. Magnetic resonance imaging of the cervical spine and angiography revealed a large arteriovenous fistula arising from the left vertebral artery. The present case highlights the clinical features and dramatic recovery following endovascular balloon occlusion of a giant cervical arteriovenous fistula.

Transcranial and spinal cord magnetic stimulation in treatment of spasticity: a literature review and meta-analysis.

Science.gov (United States)

Korzhova, Julia; Sinitsyn, Dmitry; Chervyakov, Alexander; Poydasheva, Alexandra; Zakharova, Maria; Suponeva, Natalia; Chernikova, Lyudmila; Piradov, Michael

2018-02-01

Spasticity is associated with various diseases of the nervous system. Current treatments such as drug therapy, botulinum toxin injections, kinesitherapy, and physiotherapy are not sufficiently effective in a large number of patients. Transcranial magnetic stimulation (TMS) can be considered as an alternative method of treatment. The purpose of this article was to conduct a systematic review and meta-analysis of all available publications assessing the efficacy of repetitive TMS in treatment of spasticity. Search for articles was conducted in databases PubMed, Willey, and Google. Keywords included "TMS", "spasticity", "TMS and spasticity", "non-invasive brain stimulation", and "non-invasive spinal cord stimulation". The difference in scores according to the Modified Ashworth Scale (MAS) for one joint before and after treatment was taken as the effect size. We found 26 articles that examined the TMS efficacy in treatment of spasticity. Meta-analysis included 6 trials comprising 149 patients who underwent real stimulation or simulation. No statistically significant difference in the effect of real and simulated stimulation was found in stroke patients. In patients with spinal cord injury and spasticity, the mean effect size value and the 95% confidence interval were -0.80 and (-1.12, -0.49), respectively, in a group of real stimulation; in the case of simulated stimulation, these parameters were 0.15 and (-0.30, -0.00), respectively. Statistically significant differences between groups of real stimulation and simulation were demonstrated for using high-frequency repetitive TMS or iTBS mode for the M1 area of the spastic leg (P=0.0002). According to the meta-analysis, the statistically significant effect of TMS in the form of reduced spasticity was demonstrated only for the developed due to lesions at the brain stem and spinal cord level. To clarify the amount of the antispasmodic effect of repetitive TMS at other lesion levels, in particular in patients with

Neural tension technique is no different from random passive movements in reducing spasticity in patients with traumatic brain injury

DEFF Research Database (Denmark)

Lorentzen, Jakob; Nielsen, Dorthe; Holm, Karl

2012-01-01

Purpose: Neural tension technique (NTT) is a therapy believed to reduce spasticity and to increase range of motion (ROM). This study compared the ability of NTT and random passive movements (RPMs) to reduce spasticity in the knee flexors in 10 spastic patients with brain injury. Methods: An RCT...

Incidence estimate and guideline-oriented treatment for post-stroke spasticity: an analysis based on German statutory health insurance data

Directory of Open Access Journals (Sweden)

Egen-Lappe V

2013-03-01

Full Text Available Veronika Egen-Lappe, Ingrid Köster, Ingrid SchubertPMV Research Group, Department of Child and Adolescence Psychiatry and Psychotherapy, University of Cologne, Cologne, GermanyBackground: Spasticity after stroke has been internationally recognized as an important health problem causing impairment of mobility, deformity, and pain. The aim of this study was to assess the frequency of first-ever and recurrent stroke and of subsequent spastic and flaccid paresis. Factors influencing the development of spasticity were analyzed. A further major aim was to provide a "real-life" assessment of the treatment of spasticity in Germany and to discuss this in view of the treatment recommended by German and international clinical guidelines.Methods: The database used in this study comprised a cohort of 242,090 insurants from a large statutory health insurance fund in the federal state of Hesse, Germany. A first hospital discharge diagnosis in 2009 with any of the International Classification of Diseases, Tenth Revision (ICD-10 codes I60–I64 was used to identify patients with acute stroke (hemorrhage and ischemic. These patients were followed up six months after stroke to monitor whether they developed spastic or flaccid paresis (hospital or ambulatory care diagnoses ICD-10 code G81–G83 [excluding G82.6/G83.4/G83.8]. For patients with spastic paresis after stroke the spasticity treatment was analyzed for a six-month period (physiotherapy, oral muscle relaxants, intrathecal baclofen, and botulinum toxin.Results: Standardized to the population of Germany, 3.7 per 1000 persons suffered a stroke in 2009 (raw 5.2/1000. Of all surviving patients, 10.2% developed spasticity within 6 months. Cox regression revealed no significant influence of patient age, gender, morbidity (diabetes, hypertensive diseases, ischemic heart diseases or type of stroke on development of spasticity. 97% of surviving patients with spasticity received physiotherapy (inpatient care 89

Asphyxia-related risk factors and their timing in spastic cerebral palsy

DEFF Research Database (Denmark)

Nielsen, Lene F.; Schendel, Diana; Grove, Jakob

2008-01-01

Objective To investigate the association of asphyxia-related conditions (reducing blood flow or blood oxygen levels in the fetus) with spastic cerebral palsy (CP) considering different gestational age groups and the timing of risk. Design Population-based case-control study. Setting Danish Cerebral...... Palsy Register in eastern Denmark and Danish Medical Birth Register. Population or Sample 271 singletons with spastic CP and 217 singleton controls, frequency matched by gestational age group, born 1982-1990 in eastern Denmark. Methods Data were abstracted from medical records, and a priori asphyxia...

Clinical experiences with cannabinoids in spasticity management in multiple sclerosis.

Science.gov (United States)

Lorente Fernández, L; Monte Boquet, E; Pérez-Miralles, F; Gil Gómez, I; Escutia Roig, M; Boscá Blasco, I; Poveda Andrés, J L; Casanova-Estruch, B

2014-06-01

Spasticity is a common symptom among patients with multiple sclerosis (MS). This study aims to assess the effectiveness and safety of the combination of delta-9-tetrahydrocannabinol (THC) and cannabidiol (CBD) in clinical practice for the treatment of spasticity in MS. Retrospective observational study with patients treated with inhaled THC/CBD between April 2008 and March 2012. Descriptive patient and treatment variables were collected. Therapeutic response was evaluated based on the doctor's analysis and overall impression. Of the 56 patients who started treatment with THC/CBD, 6 were excluded because of missing data. We evaluated 50 patients (42% male) with a median age 47.8 years (25.6-76.8); 38% were diagnosed with primary progressive MS, 44% with secondary progressive MS, and 18% with relapsing-remitting MS. The reason for prescribing the drug was spasticity (44%), pain (10%), or both (46%). Treatment was discontinued in 16 patients because of ineffectiveness (7 patients), withdrawal (4), and adverse effects (5). The median exposure time in patients whose treatment was discontinued was 30 days vs 174 days in those whose treatment continued at the end of the study. THC/CBD was effective in 80% of patients at a median dose of 5 (2-10) inhalations/day. The adverse event profile consisted of dizziness (11 patients), somnolence (6), muscle weakness (7), oral discomfort (2), diarrhoea (3), dry mouth (2), blurred vision (2), agitation (1), nausea (1), and paranoid ideation (1). THC/CBD appears to be a good alternative to standard treatment as it improves refractory spasticity in MS and has an acceptable toxicity profile. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

Ocular disorders in children with spastic subtype of cerebral palsy.

Science.gov (United States)

Ozturk, A Taylan; Berk, A Tulin; Yaman, Aylin

2013-01-01

To document common ocular abnormalities in children with spastic subtype of cerebral palsy (CP) and to find out whether any correlation exists between their occurance and etiologic factors. Totally 194 patients with the diagnosis of spastic type CP were enrolled in this retrospective study. Detailed ophthalmic examinations were performed. Demographic data and neuroradiological findings were documented. Kruskal-Wallis, Mann Whitney U, Pearson Chi-square tests and Student's t tests were used in the statistical analysis. The mean age was 64.7±44.2 months on the first ophthalmic examination. Prevalences of diplegia (47.4%) and tetraplegia (36.1%) were found to be higher than the frequency of hemiplegia (16.5%) in our study population. Etiologic factor was asphyxia in 60.8% of the patients. Abnormal ocular findings were present in 78.9% of the patients. Statistically significant poor vision was detected in tetraplegia group among all the spastic ubtypes of CP (P=0.000). Anisometropia and significant refractive error were found in 14.4% and 70.1% of the patients, respectively. Thirty-six children (18.6%) had nystagmus and 107 children (55.2%) had strabismus. Lower gestational age and birth weight were statistically higher in patients with esotropia than exotropia (P=0.009 and P=0.024, respectively). Abnormal morphology of the optic disc was present in 152 eyes (39.2%). Severe periventricular leukomalacia (PVL) was found in 48 patients and statistically significant poor vision was detected in the presence of PVL (P=0.000). Spastic diplegic or tetraplegic CP patients with positive neuroradiological symptoms, younger gestational age and lower birth weight ought to have detailed ophthalmic examinations as early as possible to provide best visual rehabilitation.

Clinical assessment of spasticity in children with cerebral palsy: a critical review of available instruments

NARCIS (Netherlands)

Scholtes, Vanessa A. B.; Becher, Jules G.; Beelen, Anita; Lankhorst, Gustaaf J.

2006-01-01

This study reviews the instruments used for the clinical assessment of spasticity in children with cerebral palsy, and evaluates their compliance with the concept of spasticity, defined as a velocity-dependent increase in muscle tone to passive stretch. Searches were performed in Medline, Embase,

Skeletal Maturation and Mineralisation of Children with Moderate to Severe Spastic Quadriplegia

Science.gov (United States)

Sitaraman, Sadasivan

2016-01-01

Introduction Diminished bone mineral density and delayed skeletal maturation are common in children with spastic quadriplegia. Aim The purpose of our study was to evaluate the Bone Mineral Density (BMD) of children with moderate to severe spastic quadriplegia and its relationship with other variables like nutrition and growth. Materials and Methods This was a hospital based, cross- sectional, case-control study. Forty-two (28 males, 14 females) children with spastic quadriplegia and 42 (24 males, 18 females) healthy children were included in the study. BMD of cases and control were measured by Dual Energy X-ray Absorptiometry (DEXA). Radiographs of left hand and wrist of cases and controls were taken and bone age was determined. Results BMD values of upper extremity, lower extremity, thoraco-lumbar spine and pelvis in cases were lower than those of controls (p quadriplegia and nutritional status is an important contributing factor. PMID:27504366

Modified Ashworth Scale (MAS) Model based on Clinical Data Measurement towards Quantitative Evaluation of Upper Limb Spasticity

Science.gov (United States)

Puzi, A. Ahmad; Sidek, S. N.; Mat Rosly, H.; Daud, N.; Yusof, H. Md

2017-11-01

Spasticity is common symptom presented amongst people with sensorimotor disabilities. Imbalanced signals from the central nervous systems (CNS) which are composed of the brain and spinal cord to the muscles ultimately leading to the injury and death of motor neurons. In clinical practice, the therapist assesses muscle spasticity using a standard assessment tool like Modified Ashworth Scale (MAS), Modified Tardiue Scale (MTS) or Fugl-Meyer Assessment (FMA). This is done subjectively based on the experience and perception of the therapist subjected to the patient fatigue level and body posture. However, the inconsistency in the assessment is prevalent and could affect the efficacy of the rehabilitation process. Thus, the aim of this paper is to describe the methodology of data collection and the quantitative model of MAS developed to satisfy its description. Two subjects with MAS of 2 and 3 spasticity levels were involved in the clinical data measurement. Their level of spasticity was verified by expert therapist using current practice. Data collection was established using mechanical system equipped with data acquisition system and LABVIEW software. The procedure engaged repeated series of flexion of the affected arm that was moved against the platform using a lever mechanism and performed by the therapist. The data was then analyzed to investigate the characteristics of spasticity signal in correspondence to the MAS description. Experimental results revealed that the methodology used to quantify spasticity satisfied the MAS tool requirement according to the description. Therefore, the result is crucial and useful towards the development of formal spasticity quantification model.

Tilt table standing for reducing spasticity after spinal cord injury.

Science.gov (United States)

Bohannon, R W

1993-10-01

A patient with a T12 spinal cord injury and intractable extensor spasms of the lower extremities participated in tilt table standing trial on 5 nonconsecutive days to determine if the intervention would affect his spasticity and spasms. Each day's standing trial was followed by an immediate reduction in lower extremity spasticity (measured using the modified Ashworth scale and pendulum testing). Standing was also accompanied by a reduction in spasms that lasted until the following morning. The reduction of spasms was particularly advantageous to the performance of car transfers. Tilt table standing merits further examination as a physical treatment of spasms that accompany central nervous system lesions.

Outcomes of conservative treatment for cervical myelopathy caused by soft disc herniation

International Nuclear Information System (INIS)

Matsumoto, Morio; Chiba, Kazuhiro; Ishii, Ken; Ogawa, Yuto; Takaishi, Hironari; Nakamura, Masaya; Toyama, Yoshiaki

2006-01-01

The purposes of this study are to delineate the clinical course and MRI findings of patients with disc hernias which regressed spontaneously and to determine who is eligible for conservative treatment. Twenty-three patients with mild cervical myelopathy (initial Japanese Orthopedic Association (JOA) scores >10) caused by soft disc herniation were treated conservatively for more than two years (13 males, 10 females, mean age 50, mean follow-up 3.6 years). The investigated items included JOA scores and MRI findings (morphology of disc herniation). Morphology of disc herniation was classified into focal type (herniated mass persisting at the intervertebral level) and diffuse type (herniated mass migrating rostrally or caudally) in the sagittal plane and median type or paramedian type in the axial plane. The mean JOA scores were 13.4±1.5 before treatment, and 16.0±1.0 at follow-up. Regression of herniation was observed in 14 patients (Group A), while no regression was seen in 9 patients (Group B). The JOA scores were 13.7±1.5 (Group A) and 14.0±1.6 (Group B) before treatment, and 16.3±1.6 and 15.7±1.2 at follow-up, respectively. On sagittal MR images, diffuse-type herniation was observed in 9 patients (64%) and focal-type in 5 (36%) in Group A, and 3 (33%) and 6 (67%) in Group B, respectively. On axial images, median-type herniation was observed in 10 patients (71%), and paramedian-type in 4 (29%) in Group A, and 6 (67%) and 3 (33%) in Group B, respectively. Spontaneous soft disc regression in patients with cervical myelopathy was observed in more than half of those treated conservatively for longer than 2 years. Conservative treatment can be recommended for patients with mild cervical myelopathy, especially when caused by median-and/or diffuse-type disc hernia, although close observation is mandatory. (author)

Fitzsimmons Syndrome: Spastic Paraplegia, Brachydactyly, and Cognitive Impairment

NARCIS (Netherlands)

Armour, Christine M.; Humphreys, Peter; Hennekam, Raoul C. M.; Boycott, Kym M.

2009-01-01

Fitzsimmons syndrome is an infrequently described entity comprising slowly progressive spastic paraplegia, brachydactyly, and cone-shaped epiphyses, dysarthria, and low-normal intelligence. Five patients with this syndrome have been reported. The cause remains unknown. Here we describe a 16-year-old

The use of transcutaneous electrical nerve stimulation (tens in the treatment of the spasticity - a review

Directory of Open Access Journals (Sweden)

Dahyan Wagner da Silva Silveira

2008-01-01

Full Text Available This study it has as objective to argue the job of TENS in the spasticity, observing the main parameters, form of application and the mechanism for which TENS it acts in the spasticity. One is about a bibliographical revision based in the literature specialized selected scientific articles through search in the data base of scielo and of bireme, from the sources Medline and Lilacs. The studies found on the job of TENS in the spasticity, had pointed mainly that this chain reduces the spasticity significantly, in lower degrees. The stimulation electrical parameters had disclosed that TENS it (about 100Hz of raised frequency provides one better effect in the reduction of the spasticity. The types of TENS more used had been the conventional and the soon-intense one, however some studies had not presented the used duration of pulse, limit the determination of one better modality of TENS. Few studies had explained the mechanism of performance of the current related one. The ones that had made it, had pointed the release of opioid endogenous (Dynorphins for the central nervous system as main mechanism of performance, however this contrasts with the neurophysiologic bases of the high-frequency stimulation, that demonstrated better resulted in the joined studies. Still it is necessary more studies on the job of this modality of stimulation electrical in the spasticity, since important parameters as duration of pulse, time of application, numbers of attendance and performance mechanism remains without scientific evidence.

Effects of interactive games on motor performance in children with spastic cerebral palsy

OpenAIRE

AlSaif, Amer A.; Alsenany, Samira

2015-01-01

[Purpose] Motor control and muscle strength impairments are the prime reasons for motor behavior disorders in children with spastic cerebral palsy. These impairments lead to histological changes in muscle growth and the learning of motor skills. Therefore, such children experience reduced muscle force generation and decreased muscle flexibility. We investigated the effect of training with Nintendo Wii Fit games on motor performance in children with spastic cerebral palsy. [Subjects and Method...

Magnetic resonance imaging in patients with progressive myelopathy following spinal surgery.

OpenAIRE

Avrahami, E; Tadmor, R; Cohn, D F

1989-01-01

Thirty one patients with insidious progressive myelopathy 2 to 8 years following surgery of the cervical spine were subjected to magnetic resonance imaging (MRI). In 15 patients operated on for vascular malformations or intramedullary tumours, syringomyelia and cystic lesions of the spinal cord were shown. Seven of these patients also showed a combination of a recurrent tumour and spinal atrophy. Out of 16 patients who had surgery for herniated disc or spinal stenosis of the cervical spine, f...

Diagnosis of cervical spinal cord disorders with MRI

International Nuclear Information System (INIS)

Suyama, Naohito; Iizuka, Tadashi

1991-01-01

From September 1987 through May 1989, magnetic resonance imaging (MRI) has been performed in 58 patients with myelopathy and 9 patients with spinal cord injuries. This study was designed to determine the rate of spinal cord stricture and changes of signal intensities. Increased signal intensity on T2-weighted images was more frequently observed than decreased intensity on T1-weighted images in the group of myelopathy (19/58 vs 10/58). In the group of spinal cord injuries, however, there was no significant difference in the incidence between increased intensity on T2-weighted images (4/9) and decreased intensity on T1-weighted images (7/9). Twelve patients with chronic compressive spinal myelopathy tended to have an increased intensity on T2-weighted images. In such cases, although JOA scores were low before surgery, signal intensity returned to that without marked signal changes. In chronic compressive cervical myelopathy, the degree of preoperative compression was the same as the postoperative JOA scores. Regarding cervical spinal injury, there was a good correlation between the size of low signal area and the degree of paralysis. (N.K.)

Treatment of os odontoideum in a patient with spastic quadriplegic cerebral palsy.

Science.gov (United States)

Akpolat, Yusuf T; Fegale, Ben; Cheng, Wayne K

2015-08-01

Severe atlantoaxial instability due to os odontoideum in a patient with spastic cerebral palsy has not been well described. There is no consensus on treatment, particularly with regard to conservative or surgical options. Our patient was a 9-year-old girl with spastic cerebral palsy and unstable os odontoideum as an incidental finding. During the waiting period for elective surgical treatment, the patient developed respiratory compromise. Surgery was performed to reduce the subluxation and for C1-C2 arthrodesis and the girl regained baseline respiratory function. A CT scan was obtained 1 year after the initial surgery and revealed adequate maintenance of reduction and patency of the spinal canal. This patient highlights the fact that unstable os odontoideum can cause mortality due to respiratory distress in patients with spastic cerebral palsy. This is an important factor in deciding treatment options for cerebral palsy patients with low functional demand. We review the relevant literature. Copyright © 2015 Elsevier Ltd. All rights reserved.

Botulinum toxin therapy for treatment of spasticity in multiple sclerosis: review and recommendations of the IAB-Interdisciplinary Working Group for Movement Disorders task force.

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Dressler, Dirk; Bhidayasiri, Roongroj; Bohlega, Saeed; Chahidi, Abderrahmane; Chung, Tae Mo; Ebke, Markus; Jacinto, L Jorge; Kaji, Ryuji; Koçer, Serdar; Kanovsky, Petr; Micheli, Federico; Orlova, Olga; Paus, Sebastian; Pirtosek, Zvezdan; Relja, Maja; Rosales, Raymond L; Sagástegui-Rodríguez, José Alberto; Schoenle, Paul W; Shahidi, Gholam Ali; Timerbaeva, Sofia; Walter, Uwe; Saberi, Fereshte Adib

2017-01-01

Botulinum toxin (BT) therapy is an established treatment of spasticity due to stroke. For multiple sclerosis (MS) spasticity this is not the case. IAB-Interdisciplinary Working Group for Movement Disorders formed a task force to explore the use of BT therapy for treatment of MS spasticity. A formalised PubMed literature search produced 55 publications (3 randomised controlled trials, 3 interventional studies, 11 observational studies, 2 case studies, 35 reviews, 1 guideline) all unanimously favouring the use of BT therapy for MS spasticity. There is no reason to believe that BT should be less effective and safe in MS spasticity than it is in stroke spasticity. Recommendations include an update of the current prevalence of MS spasticity and its clinical features according to classifications used in movement disorders. Immunological data on MS patients already treated should be analysed with respect to frequencies of MS relapses and BT antibody formation. Registration authorities should expand registration of BT therapy for spasticity regardless of its aetiology. MS specialists should consider BT therapy for symptomatic treatment of spasticity.

Somatosensory inputs by application of KinesioTaping: Effects on spasticity, balance, and gait in chronic spinal cord injury

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Federica eTamburella

2014-05-01

Full Text Available Introduction: Leg paralysis, spasticity, reduced inter limb coordination and impaired balance are considered the chief limitations to overground ambulation in subjects with incomplete spinal cord injury (SCI. In the last years KinesioTaping (KT application has been proposed for enhancing sensory inputs, decreasing spasticity via proprioception feedback and relieving abnormal muscle tension. No studies addressed KT technique on SCI subjects: our goal was to analyze effects of ankle joint KT application on spasticity, balance and gait. Material and Methods: A randomized cross-over case control design was used to compare KT and conventional non-elastic silk tape (ST application’s effects in 11 chronic SCI subjects, AIS level D, with soleus/gastrocnemius (S/G muscles’ spasticity , balance and gait impairments. Treatment: 48 hours of either KT or ST treatment was followed after 1 week interval by a reverse protocol. Patient treated with KT were subjected to 48 hours of ST treatment and viceversa. Single Y-stripe of Cure©tape (KT and ST were applied to S and G with 0% stretch. Before and after 48 hours of KT and ST application, clinical data of range of motion (ROM, spasticity, clonus, pain, balance and gait were collected. Stabilometric platform assessment of Centre of Pressure (COP movements, bi-dimensional gait analysis and electromyograpich (EMG activity of S, G, Tibialis Anterior and Extensor Hallicus Lungus muscles were also collected. Results: Only After KT treatment significant effects on spasticity, clonus and COP movements, kinematic gait parameters and EMG activities were recorded. Comparison between KT and ST improvements pointed out significant differences for ROM, spasticity, clonus, pain, COP parameters and most of all kinematic gait data. Discussion: KT short term application reduces spasticity and pain and improves balance and gait performances in chronic incomplete SCI subjects.

Ocular disorders in children with spastic subtype of cerebral palsy

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Aylin Yaman

2013-04-01

Full Text Available AIM: To document common ocular abnormalities in children with spastic subtype of cerebral palsy (CP and to find out whether any correlation exists between their occurance and etiologic factors. METHODS: Totally 194 patients with the diagnosis of spastic type CP were enrolled in this retrospective study. Detailed ophthalmic examinations were performed. Demographic data and neuroradiological findings were documented. Kruskal-Wallis, Mann Whitney U, Pearson Chi-square tests and Student’s t tests were used in the statistical analysis. RESULTS: The mean age was 64.7±44.2 months on the first ophthalmic examination. Prevalences of diplegia (47.4% and tetraplegia (36.1% were found to be higher than the frequency of hemiplegia (16.5% in our study population. Etiologic factor was asphyxia in 60.8% of the patients. Abnormal ocular findings were present in 78.9% of the patients. Statistically significant poor vision was detected in tetraplegia group among all the spastic ubtypes of CP (P=0.000. Anisometropia and significant refractive error were found in 14.4% and 70.1% of the patients, respectively. Thirty-six children (18.6% had nystagmus and 107 children (55.2% had strabismus. Lower gestational age and birth weight were statistically higher in patients with esotropia than exotropia (P=0.009 and P=0.024, respectively. Abnormal morphology of the optic disc was present in 152 eyes (39.2%. Severe periventricular leukomalacia (PVL was found in 48 patients and statistically significant poor vision was detected in the presence of PVL (P=0.000. CONCLUSION: Spastic diplegic or tetraplegic CP patients with positive neuroradiological symptoms, younger gestational age and lower birth weight ought to have detailed ophthalmic examinations as early as possible to provide best visual rehabilitation.

Biomechanical effects of spinal cord compression due to ossification of posterior longitudinal ligament and ligamentum flavum: a finite element analysis.

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Kim, Yoon Hyuk; Khuyagbaatar, Batbayar; Kim, Kyungsoo

2013-09-01

Ossification of the posterior longitudinal ligament (OPLL) and ossification of the ligamentum flavum (OLF) have been recognized as causes of myelopathy due to thickening of the ligaments resulting in narrowing of the spinal canal and compression of the spinal cord. However, few studies have focused on predicting stress distribution under conditions of OPLL and OLF based on clinical aspects such as the relationship between level of stress and severity of neurologic symptoms because direct in vivo measurement of stress is very restrictive. In this study, a three-dimensional finite element model of the spinal cord in T12-L1 was developed based on MR images. The von-Mises stresses in the cord and the cross-sectional area of the cord were investigated for various grades and shapes of spinal cord compression in OPLL and OLF. Substantial increases in maximum stresses resulting in the manifestation of spinal cord symptoms occurred when the cross-sectional area was reduced by 30-40% at 60% compression of the antero-posterior diameter of the cord in OPLL and at 4mm compression in OLF. These results indicate that compression greater than these thresholds may induce spinal symptoms, which is consistent with clinical observations. Copyright © 2013 IPEM. Published by Elsevier Ltd. All rights reserved.

Supplemental Stimulation Improves Swing Phase Kinematics During Exoskeleton Assisted Gait of SCI Subjects With Severe Muscle Spasticity

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Ekelem, Andrew; Goldfarb, Michael

2018-01-01

Spasticity is a common comorbidity associated with spinal cord injury (SCI). Robotic exoskeletons have recently emerged to facilitate legged mobility in people with motor complete SCI. Involuntary muscle activity attributed to spasticity, however, can prevent such individuals from using an exoskeleton. Specifically, although most exoskeleton technologies can accommodate low to moderate spasticity, the presence of moderate to severe spasticity can significantly impair gait kinematics when using an exoskeleton. In an effort to potentially enable individuals with moderate to severe spasticity to use exoskeletons more effectively, this study investigates the use of common peroneal stimulation in conjunction with exoskeleton gait assistance. The electrical stimulation is timed with the exoskeleton swing phase, and is intended to acutely suppress extensor spasticity through recruitment of the flexion withdrawal reflex (i.e., while the stimulation is activated) to enable improved exoskeletal walking. In order to examine the potential efficacy of this approach, two SCI subjects with severe extensor spasticity (i.e., modified Ashworth ratings of three to four) walked in an exoskeleton with and without supplemental stimulation while knee and hip motion was measured during swing phase. Stimulation was alternated on and off every ten steps to eliminate transient therapeutic effects, enabling the acute effects of stimulation to be isolated. These experiments indicated that common peroneal stimulation on average increased peak hip flexion during the swing phase of walking by 21.1° (236%) and peak knee flexion by 14.4° (56%). Additionally, use of the stimulation decreased the swing phase RMS motor current by 228 mA (15%) at the hip motors and 734 mA (38%) at the knee motors, indicating improved kinematics were achieved with reduced effort from the exoskeleton. Walking with the exoskeleton did not have a significant effect on modified Ashworth scores, indicating the common

Supplemental Stimulation Improves Swing Phase Kinematics During Exoskeleton Assisted Gait of SCI Subjects With Severe Muscle Spasticity.

Science.gov (United States)

Ekelem, Andrew; Goldfarb, Michael

2018-01-01

Spasticity is a common comorbidity associated with spinal cord injury (SCI). Robotic exoskeletons have recently emerged to facilitate legged mobility in people with motor complete SCI. Involuntary muscle activity attributed to spasticity, however, can prevent such individuals from using an exoskeleton. Specifically, although most exoskeleton technologies can accommodate low to moderate spasticity, the presence of moderate to severe spasticity can significantly impair gait kinematics when using an exoskeleton. In an effort to potentially enable individuals with moderate to severe spasticity to use exoskeletons more effectively, this study investigates the use of common peroneal stimulation in conjunction with exoskeleton gait assistance. The electrical stimulation is timed with the exoskeleton swing phase, and is intended to acutely suppress extensor spasticity through recruitment of the flexion withdrawal reflex (i.e., while the stimulation is activated) to enable improved exoskeletal walking. In order to examine the potential efficacy of this approach, two SCI subjects with severe extensor spasticity (i.e., modified Ashworth ratings of three to four) walked in an exoskeleton with and without supplemental stimulation while knee and hip motion was measured during swing phase. Stimulation was alternated on and off every ten steps to eliminate transient therapeutic effects, enabling the acute effects of stimulation to be isolated. These experiments indicated that common peroneal stimulation on average increased peak hip flexion during the swing phase of walking by 21.1° (236%) and peak knee flexion by 14.4° (56%). Additionally, use of the stimulation decreased the swing phase RMS motor current by 228 mA (15%) at the hip motors and 734 mA (38%) at the knee motors, indicating improved kinematics were achieved with reduced effort from the exoskeleton. Walking with the exoskeleton did not have a significant effect on modified Ashworth scores, indicating the common

Supplemental Stimulation Improves Swing Phase Kinematics During Exoskeleton Assisted Gait of SCI Subjects With Severe Muscle Spasticity

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Andrew Ekelem

2018-06-01

Full Text Available Spasticity is a common comorbidity associated with spinal cord injury (SCI. Robotic exoskeletons have recently emerged to facilitate legged mobility in people with motor complete SCI. Involuntary muscle activity attributed to spasticity, however, can prevent such individuals from using an exoskeleton. Specifically, although most exoskeleton technologies can accommodate low to moderate spasticity, the presence of moderate to severe spasticity can significantly impair gait kinematics when using an exoskeleton. In an effort to potentially enable individuals with moderate to severe spasticity to use exoskeletons more effectively, this study investigates the use of common peroneal stimulation in conjunction with exoskeleton gait assistance. The electrical stimulation is timed with the exoskeleton swing phase, and is intended to acutely suppress extensor spasticity through recruitment of the flexion withdrawal reflex (i.e., while the stimulation is activated to enable improved exoskeletal walking. In order to examine the potential efficacy of this approach, two SCI subjects with severe extensor spasticity (i.e., modified Ashworth ratings of three to four walked in an exoskeleton with and without supplemental stimulation while knee and hip motion was measured during swing phase. Stimulation was alternated on and off every ten steps to eliminate transient therapeutic effects, enabling the acute effects of stimulation to be isolated. These experiments indicated that common peroneal stimulation on average increased peak hip flexion during the swing phase of walking by 21.1° (236% and peak knee flexion by 14.4° (56%. Additionally, use of the stimulation decreased the swing phase RMS motor current by 228 mA (15% at the hip motors and 734 mA (38% at the knee motors, indicating improved kinematics were achieved with reduced effort from the exoskeleton. Walking with the exoskeleton did not have a significant effect on modified Ashworth scores, indicating the

Watsu approach for improving spasticity and ambulatory function in hemiparetic patients with stroke.

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Chon, Seung Chul; Oh, Duck Won; Shim, Jae Hun

2009-06-01

This study reports the effect of Watsu as rehabilitation method for hemiparetic patients with stroke. Watsu consisted of 40 treatment sessions for 8 weeks, delivered underwater or at water surface level, it applied in three patients. Outcome measures included tools for assessing spasticity and ambulatory function. All patients showed decreased scores in the TAS and RVGA after Watsu application. Watsu was helpful in controlling spasticity and improving ambulatory function of the patients with hemiparesis.

[The modified method registration of kinesthetic evoked potentials and its application for research of proprioceptive sensitivity disorders at spondylogenic cervical myelopathy].

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Gordeev, S A; Voronin, S G

2016-01-01

To analyze the efficacy of modified (passive radiocarpal articulation flexion/extension) and «standard» (passive radiocarpal articulation flexion) methods of kinesthetic evoked potentials for proprioceptive sensitivity assessment in healthy subjects and patients with spondylotic cervical myelopathy. The study included 14 healthy subjects (4 women and 10 men, mean age 54.1±10.5 years) and 8 patients (2 women and 6 men, mean age 55.8±10.9 years) with spondylotic cervical myelopathy. Muscle-joint sensation was examined during the clinical study. A modified method of kinesthetic evoked potentials was developed. This method differed from the "standard" one by the organization of a cycle including several passive movements,where each new movement differed from the preceding one by the direction. The modified method of kinesthetic evoked potentials ensures more reliable kinesthetic sensitivity assessment due to movement variability. Asignificant increaseof the latent periods of the early components of the response was found in patients compared to healthy subjects. The modified method of kinesthetic evoked potentials can be used for objective diagnosis of proprioceptive sensitivity disorders in patients with spondylotic cervical myelopathy.

EFFICACY OF CAGE PLACEMENT WITHOUT PLATE IN PATIENTS WITH CERVICAL MYELOPATHY WITH SINGLE-LEVEL AFFECTION

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CARLOS ALBERTO ZUÑIGA-MAZÓN

Full Text Available ABSTRACT Objective: To determine the efficacy of PEEK (Poly-ether-ether-ketone cage without plate for the treatment of single-level cervical spondylosis. Methods: Ten patients with cervical myelopathy data, with a single-level root condition, seen at the outpatient clinic of the Neurosurgery Service, operated in 2016, mean age 53 years, 6 (60% female, 4 (40% obese, 3 (30% smokers. The Cloward technique was used by anterior approach, discectomy, and PEEK cage placement. Results: At six months of surgery, 100% of the patients had increased intervertebral space, with a 100% reduction in osteophytes; only one patient had dysphagia, no patient had lesion of the adjacent segment and 10% had persistent root pathology. Cervical lordosis was observed in 90% of the patients and arthrodesis in 100% of the cases. Conclusions: Anterior approach arthrodesis using PEEK cage without cervical plate is effective as a treatment of cervical myelopathy in a single level.

Sensory Barrage Stimulation in the Treatment of Elbow Spasticity: A Crossover Double Blind Randomized Pilot Trial.

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Slovak, Martin; Chindo, Joseph; Nair, Krishnan Padmakumari Sivaraman; Reeves, Mark L; Heller, Ben; Barker, Anthony T

2016-02-01

To assess the feasibility of using a novel form of multichannel electrical stimulation, termed Sensory Barrage Stimulation (SBS) for the treatment of spasticity affecting the elbow flexor muscles and to compare this with conventional single-channel TENS stimulation. Altogether ten participants with spasticity of the flexor muscles of the elbow of Grade 2 or above on the Modified Ashworth Scale (MAS) were recruited to this crossover double blind randomized trial. The participants received two intervention sessions (SBS and TENS), one week apart in a randomized order. Both interventions were applied over the triceps brachii on the affected arm for a duration of 60 minutes. Spasticity was measured using the MAS. Secondary outcome measures were self-reported change in spasticity, measured on a visual analog scale (VAS, 0-100), and therapist-rated strength of elbow extension and strength of elbow flexion. Measurements were taken immediately before each intervention was applied, immediately after the intervention, and one hour after the intervention. Immediately after stimulation spasticity showed a significant reduction for both TENS and SBS groups assessed by MAS -0.9 ± 0.2 vs. -1.1 ± 0.2 and by VAS -15 ± 3 vs. -31 ± 8. For SBS this improvement in MAS was still present at one hour after the stimulation, but not for TENS. Altogether seven SBS responders and four TENS responders were identified. This study demonstrates the feasibility and practicality of applying the new concept of SBS. Promising results indicate it causes a reduction in spasticity. © 2015 International Neuromodulation Society.

Development of a clinical spasticity scale for evaluation of dogs with chronic thoracolumbar spinal cord injury.

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Lewis, Melissa J; Olby, Natasha J

2017-07-01

OBJECTIVE To develop a spasticity scale for dogs with chronic deficits following severe spinal cord injury (SCI) for use in clinical assessment and outcome measurement in clinical trials. ANIMALS 20 chronically paralyzed dogs with a persistent lack of hind limb pain perception caused by an acute SCI at least 3 months previously. PROCEDURES Spasticity was assessed in both hind limbs via tests of muscle tone, clonus, and flexor and extensor spasms adapted from human scales. Measurement of patellar clonus duration and flexor spasm duration and degree was feasible. These components were used to create a canine spasticity scale (CSS; overall score range, 0 to 18). Temporal variation for individual dogs and interrater reliability were evaluated. Gait was quantified with published gait scales, and CSS scores were compared with gait scores and clinical variables. Owners were questioned regarding spasticity observed at home. RESULTS 20 dogs were enrolled: 18 with no apparent hind limb pain perception and 2 with blunted responses; 5 were ambulatory. Testing was well tolerated, and scores were repeatable between raters. Median overall CSS score was 7 (range, 3 to 11), and flexor spasms were the most prominent finding. Overall CSS score was not associated with age, SCI duration, lesion location, or owner-reported spasticity. Overall CSS score and flexor spasm duration were associated with gait scores. CONCLUSIONS AND CLINICAL RELEVANCE The CSS could be used to quantify hind limb spasticity in dogs with chronic thoracolumbar SCI and might be a useful outcome measure. Flexor spasms may represent an integral part of stepping in dogs with severe SCI.

Modeling neurodevelopment and cortical dysfunction in SPG11-linked hereditary spastic paraplegia using human induced pluripotent stem cells

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Mishra, Himanshu Kumar

2016-01-01

Hereditary spastic paraplegias (HSPs) are a heterogeneous group of inherited motor neuron diseases characterized by progressive spasticity and weakness of the lower limbs. Mutations in the Spastic Paraplegia Gene11 (SPG11), encoding spatacsin, cause the most frequent form of autosomal recessive HSP. SPG11 patients are clinically distinguishable from most other HSPs, by severe cortical atrophy and presence of a thin corpus callosum (TCC), associated with cognitive deficits. Partly due to l...

Effects of intermittent theta burst stimulation on spasticity after stroke.

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Kim, Dae Hyun; Shin, Ji Cheol; Jung, Seungsoo; Jung, Tae-Min; Kim, Deog Young

2015-07-08

Spasticity is a common cause of long-term disability in poststroke hemiplegic patients. We investigated whether intermittent theta burst stimulation (iTBS) could reduce upper-limb spasticity after a stroke. Fifteen hemiplegic stroke patients were recruited for a double-blind sham-controlled cross-over design study. A single session of iTBS or sham stimulation was delivered on the motor hotspot of the affected flexor carpi radialis muscle in a random and counterbalanced order with a 1-week interval. Modified Ashworth scale (MAS), modified Tardieu scale (MTS), H-wave/M-wave amplitude ratio, peak torque (PT), peak torque angle (PTA), work of affected wrist flexor, and rectified integrated electromyographic activity of the flexor carpi radialis muscle were measured before, immediately after, 30 min after, and 1 week after iTBS or sham stimulation. Repeated-measures analysis of variance showed a significant interaction between time and intervention for the MAS, MTS, PT, PTA, and rectified integrated electromyographic activity (PiTBS compared with sham stimulation. However, the H-wave/M-wave amplitude ratio and work were not affected. MAS and MTS significantly improved for at least 30 min after iTBS, but the other parameters only improved immediately after iTBS (PiTBS on the affected hemisphere may help to reduce poststroke spasticity transiently.

Biological prevention and/or treatment strategies for radiation myelopathy. Discussion of a new perspective

International Nuclear Information System (INIS)

Nieder, C.; Ataman, F.; Price, R.E.; Kian Ang, K.

1999-01-01

Background: Radiosensitivity of the spinal cord makes both curative first-line treatment of numerous malignancies and re-irradiation of recurrent or second tumors more difficult. This review discusses recent advances in basic research that alter the view on the pathogenesis of radiation myelopathy, possibly offering strategies for prevention and/or therapy. Results: Available data of developmental neurobiology and preclinical studies of demyelinating diseases revealed interesting insights into oligodendrocyte development, intercellular signaling pathways, and myelination processes. Current findings suggest that administration of cytokines could increase proliferation of oligodendrocyte progenitor cells, enhance their differentiation, upregulate synthesis of myelin constituents, and promote myelin regeneration in the adult central nervous system. Other compounds might also be able to modulate progression of pathogenic processes that eventually lead to radiation myelopathy. This offers several possible biological prevention and/or treatment strategies, which currently are being investigated in animal studies. Conclusions: Technical options as well as optimization of fractionation parameters should be given priority in the attempt to reduce iatrogenic neurotoxicity. However, rational biological strategies could offer a new perspective for many patients. (orig.) [de

PRESENT SCENARIO OF NON TRAUMATIC QUADRIPARESIS IN A TEACHING HOSPITAL

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Radha Krishnan

2015-04-01

Full Text Available AIMS & OBJECTIVES: Patients presenting with acute quadriparesis may pose therapeutic challenge to the treating physician especially the development of bulbar palsy and respiratory paralysis and require intensive monitoring and treatment in acute clinical and respiratory care units. So this study was conducted to know the etiology of cases of non - traumatic Quadriparesis and its outcome. MATERIALS AND METHODS: 50 adult patients admitted in medical and neurology wards with non - traumatic quadriparesis were prospectively studied b etween October ’2012 to September ’2014at Government General Hospital, Kakinada, a teaching hospital with rural referrals. OBSERVATIONS AND RESULTS: In the study cohort of 50 cases the age of patients ranged from 13 to 80 years with more number of male patients. 29 patients (58% presented with flaccid and 21 cases (42% with spastic quadriparesis. Guillian barre syndrome with 18 (36% cases was the most common cause of quadriparesis followed by Spondylotic myelopathy 11 cases ( 22% and Hypokalemic perio dic paralysis in 8 cases (16%. Transverse Myelitis. Caries spine. Secondaries cervical spine, spinal epidural abscess were in other cases.7 (14% patients had cranial nerve dysfunction. 4(8% patients had facial nerve palsy . CONCLUSION: Guillian barre syn drome constituted the most common cause of nontraumatic quadriparesis, followed by Spondylotic myelopathy, Transverse Myelitis. Caries spine. S econdaries cervical spine, spinal epidural abscess . AIDP and Hypokalemic periodic paralysis were the most frequen t causes of flaccid quadriparesis while Spondylotic myelopathy was the most common cause of spastic quadriparesis . M.R.I was the most useful and appropriate investigation . Severity of paralysis and need for ventilator support were associated with poor prog nosis in patients with acute flaccid quadriparesis . Decompressive surgery in spondylotic myelopathy had good recovery after surgery. Patient recovery was

Symptomatic thoracic spinal cord herniation: case series and technical report.

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Hawasli, Ammar H; Ray, Wilson Z; Wright, Neill M

2014-09-01

Idiopathic spinal cord herniation (ISCH) is an uncommon condition located predominantly in the thoracic spine and often associated with a remote history of a major traumatic injury. ISCH has an incompletely described presentation and unknown etiology. There is no consensus on the treatment algorithm and surgical technique, and there are few data on clinical outcomes. In this case series and technical report, we describe the atypical myelopathy presentation, remote history of traumatic injury, radiographic progression, treatment, and outcomes of 5 patients treated at Washington University for symptomatic ISCH. A video showing surgical repair is presented. In contrast to classic compressive myelopathy symptomatology, ISCH patients presented with an atypical myelopathy, characterized by asymmetric motor and sensory deficits and early-onset urinary incontinence. Clinical deterioration correlated with progressive spinal cord displacement and herniation observed on yearly spinal imaging in a patient imaged serially because of multiple sclerosis. Finally, compared with compressive myelopathy in the thoracic spine, surgical treatment of ISCH led to rapid improvement despite a long duration of symptoms. Symptomatic ISCH presents with atypical myelopathy and slow temporal progression and can be successfully managed with surgical repair.

A retrospective neurocognitive study in children with spastic diplegia

NARCIS (Netherlands)

Pirila, S; van der Meere, J; Korhonen, P; Ruusu-Niemi, P; Kyntaja, M; Nieminen, P; Korpela, R

2004-01-01

The study presents the results on neonatal cranial ultrasonography (US) and later intelligence (Wechsler Intelligence Scale-Third Edition and Wechsler Preschool and Primary Scale of Intelligence-Revised) and Neuropsychological assessments of 15 children with spastic diplegia. The assessments were

CT and MRI findings in HAM. A report on five cases

Energy Technology Data Exchange (ETDEWEB)

Fukaura, Hikoaki; Tashiro, Kunio; Maruo, Yasunori; Moriwaka, Fumio; Itoh, Kazunori; Matsuura, Tooru; Togashi, Takehiro (Hokkaido Univ., Sapporo (Japan). School of Medicine); Hamada, Takeshi; Matsumoto, Akihisa

1989-02-01

The findings of the spinal MR imagings and the CT myelography of 5 cases with HTLV-I associated myelopathy (HAM) were presented. One case who showed transverse myelopathy with a multiple sclerosis-like abrupt onset showed spinal-cord swelling at the early stage on CT myelography; its disappearance was documented 4 months after corticosteroid therapy. The other 4 cases, all with slowly progressive myelopathy, clinically revealed spinal-cord atrophy, especially at the thoracic level, on MR imagings. The degree of spinal-cord atrophy seemed not have any correlation with the clinical and laboratory data or with the corticosteroid therapy. The clinical entity of HAM has not yet been determined, and its relationship with tropical spastic paraparesis (TSP) as well as that with some form of multiple sclerosis is controversial. The neuroradiological findings of MR imaging and CT myelography, including spinal-cord swelling at the early stage, in a case diagnosed as HAM with atypical clinical features or as spinal-cord atrophy in typical chronic cases might contribute to its resolution. (author).

Somatosensory inputs by application of KinesioTaping: effects on spasticity, balance, and gait in chronic spinal cord injury.

Science.gov (United States)

Tamburella, Federica; Scivoletto, Giorgio; Molinari, Marco

2014-01-01

Leg paralysis, spasticity, reduced interlimb coordination, and impaired balance are the chief limitations to overground ambulation in subjects with incomplete spinal cord injury (SCI). In recent years, the application of KinesioTaping (KT) has been proposed to enhance sensory inputs, decreasing spasticity by proprioception feedback and relieving abnormal muscle tension. Because no studies have examined KT-based techniques in SCI subjects, our goal was to analyze the effects of ankle joint KT on spasticity, balance, and gait. A randomized crossover case control design was used to compare the effects of KT and conventional nonelastic silk tape (ST) in 11 chronic SCI subjects, AIS level D, with soleus/gastrocnemius (S/G) muscle spasticity and balance and gait impairments. 48 h of treatment with KT or ST was followed by 48 h with the other technique after 1 week. A single Y-strip of Cure(©) tape (KT) and ST was to the S and G muscles with 0% stretch. Before and 48 h after of application of KT and ST, clinical data on the range of motion (ROM), spasticity, clonus, pain, balance, and gait were collected. Stabilometric platform assessment of center of pressure (COP) movements; bidimensional gait analysis; and recording of electromyographic (EMG) activity of the S, G, and tibialis anterior and extensor hallucis lungus muscles were also performed. Only KT had significant effects on spasticity (p < 0.05), clonus (p < 0.001) and COP movements (p < 0.05), kinematic gait parameters (p < 0.001), and EMG activity (p < 0.001). Comparison between ST and KT improvements pointed out significant differences as concerns ROM (p < 0.001), spasticity (p < 0.001), clonus (p < 0.001), pain (p < 0.001), COP parameters (p < 0.05), and most kinematic gait data (p < 0.05). Short-term application of KT reduces spasticity and pain and improves balance and gait in chronic SCI subjects. Although these data are promising, they require confirmation in a larger cohort of patients.

Influence of Botulinum Toxin Therapy on Postural Control and Lower Limb Intersegmental Coordination in Children with Spastic Cerebral Palsy

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Bernard Dan

2013-01-01

Full Text Available Botulinum toxin injections may significantly improve lower limb kinematics in gait of children with spastic forms of cerebral palsy. Here we aimed to analyze the effect of lower limb botulinum toxin injections on trunk postural control and lower limb intralimb (intersegmental coordination in children with spastic diplegia or spastic hemiplegia (GMFCS I or II. We recorded tridimensional trunk kinematics and thigh, shank and foot elevation angles in fourteen 3–12 year-old children with spastic diplegia and 14 with spastic hemiplegia while walking either barefoot or with ankle-foot orthoses (AFO before and after botulinum toxin infiltration according to a management protocol. We found significantly greater trunk excursions in the transverse plane (barefoot condition and in the frontal plane (AFO condition. Intralimb coordination showed significant differences only in the barefoot condition, suggesting that reducing the degrees of freedom may limit the emergence of selective coordination. Minimal relative phase analysis showed differences between the groups (diplegia and hemiplegia but there were no significant alterations unless the children wore AFO. We conclude that botulinum toxin injection in lower limb spastic muscles leads to changes in motor planning, including through interference with trunk stability, but a combination of therapies (orthoses and physical therapy is needed in order to learn new motor strategies.

Identification of the neural component of torque during manually-applied spasticity assessments in children with cerebral palsy

NARCIS (Netherlands)

Bar-On, L.; Desloovere, K.; Molenaers, G.; Harlaar, J.; Kindt, T.; Aertbelien, E.

2014-01-01

Clinical assessment of spasticity is compromised by the difficulty to distinguish neural from non-neural components of increased joint torque. Quantifying the contributions of each of these components is crucial to optimize the selection of anti-spasticity treatments such as botulinum toxin (BTX).

Cervical spondylotic myelopathy: the clinical phenomenon and the current pathobiology of an increasingly prevalent and devastating disorder.

Science.gov (United States)

Kalsi-Ryan, Sukhvinder; Karadimas, Spyridon K; Fehlings, Michael G

2013-08-01

Cervical spondylotic myelopathy (CSM) is a common disorder involving chronic progressive compression of the cervical spinal cord due to degenerative disc disease, spondylosis, or other degenerative pathology. CSM is the most common form of spinal cord impairment and causes functional decline leading to reduced independence and quality of life. Despite a sound understanding of the disease process, clinical presentation and management, a universal definition of CSM and a standardized index of severity are not currently used universally. Work is required to develop a definition and establish clinical predictors of progression to improve management of CSM. Despite advances in decompressive and reconstructive surgery, patients are often left with residual disability. Gaps in knowledge of the pathobiology of CSM have limited therapeutic advances to complement surgery. Although the histopathologic and pathophysiologic similarities between CSM and traumatic spinal cord injury have long been acknowledged, the unique pathomechanisms of CSM remain unexplored. Increased efforts to elucidate CSM pathobiology could lead to the discovery of novel therapeutic targets for human CSM and other spinal cord diseases. Here, the natural history of CSM, epidemiology, clinical presentation, and current methods of clinical management are reported, along with the current state of basic scientific research in the field.

Restoration of Upper Limb Function in an Individual with Cervical Spondylotic Myelopathy using Functional Electrical Stimulation Therapy: A Case Study.

Science.gov (United States)

Popovic, Milos R; Zivanovic, Vera; Valiante, Taufik A

2016-01-01

Non-traumatic spinal cord pathology is responsible for 25-52% of all spinal cord lesions. Studies have revealed that spinal stenosis accounts for 16-21% of spinal cord injury (SCI) admissions. Impaired grips as well as slow unskilled hand and finger movements are the most common complaints in patients with spinal cord disorders, such as myelopathy secondary to cervical spondylosis. In the past, our team carried out couple of successful clinical trials, including two randomized control trials, showing that functional electrical stimulation therapy (FEST) can restore voluntary reaching and/or grasping function, in people with stroke and traumatic SCI. Motivated by this success, we decided to examine changes in the upper limb function following FEST in a patient who suffered loss of hand function due to myelopathy secondary to cervical spondylosis. The participant was a 61-year-old male who had C3-C7 posterior laminectomy and instrumented fusion for cervical myelopathy. The participant presented with progressive right hand weakness that resulted in his inability to voluntarily open and close the hand and to manipulate objects unilaterally with his right hand. The participant was enrolled in the study ~22 months following initial surgical intervention. Participant was assessed using Toronto Rehabilitation Institute's Hand Function Test (TRI-HFT), Action Research Arm Test (ARAT), Functional Independence Measure (FIM), and Spinal Cord Independence Measure (SCIM). The pre-post differences in scores on all measures clearly demonstrated improvement in voluntary hand function following 15 1-h FEST sessions. The changes observed were meaningful and have resulted in substantial improvement in performance of activities of daily living. These results provide preliminary evidence that FEST has a potential to improve upper limb function in patients with non-traumatic SCI, such as myelopathy secondary to cervical spondylosis.

Hereditary spastic paraplegia: More than an upper motor neuron disease.

Science.gov (United States)

Parodi, L; Fenu, S; Stevanin, G; Durr, A

2017-05-01

Hereditary spastic paraplegias (HSPs) are a group of rare inherited neurological diseases characterized by extreme heterogeneity in both their clinical manifestations and genetic backgrounds. Based on symptoms, HSPs can be divided into pure forms, presenting with pyramidal signs leading to lower-limb spasticity, and complex forms, when additional neurological or extraneurological symptoms are detected. The clinical diversity of HSPs partially reflects their underlying genetic backgrounds. To date, 76 loci and 58 corresponding genes [spastic paraplegia genes (SPGs)] have been linked to HSPs. The genetic diagnosis is further complicated by the fact that causative mutations of HSP can be inherited through all possible modes of transmission (autosomal-dominant and -recessive, X-linked, maternal), with some genes showing multiple inheritance patterns. The pathogenic mutations of SPGs primarily lead to progressive degeneration of the upper motor neurons (UMNs) comprising corticospinal tracts. However, it is possible to observe lower-limb muscle atrophy and fasciculations on clinical examination that are clear signs of lower motor neuron (LMN) involvement. The purpose of this review is to classify HSPs based on their degree of motor neuron involvement, distinguishing forms in which only UMNs are affected from those involving both UMN and LMN degeneration, and to describe their differential diagnosis from diseases such as amyotrophic lateral sclerosis. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Gait impairment in cervical spondylotic myelopathy: comparison with age- and gender-matched healthy controls.

LENUS (Irish Health Repository)

Malone, Ailish

2012-12-01

Gait impairment is a primary symptom of cervical spondylotic myelopathy (CSM); however, little is known about specific kinetic and kinematic gait parameters. The objectives of the study were: (1) to compare gait patterns of people with untreated CSM to those of age- and gender-matched healthy controls; (2) to examine the effect of gait speed on kinematic and kinetic parameters.

[Clinical study on spinal cord decompression combined with traditional Chinese medicine for the treatment of cervical spondylotic myelopathy].

Science.gov (United States)

Yang, Feng; Tan, Ming-Sheng; Yi, Ping; Tang, Xiang-Sheng; Hao, Qing-Ying; Qi, Ying-Na

2018-01-25

To compare the clinical effect between spinal card decompression combined with traditional Chinese medicine and simple spinal card decompression for cervical spondylotic myelopathy. From June 2012 to June 2015, 73 patients with cervical spondylotic myelopathy were treated, including 42 males and 31 females, aged from 29 to 73 years old with a mean of 50.9 years old. The patients were divided into the simple operation group (34 cases) and the operation combined with traditional Chinese medicine group(39 cases) according to the idea of themselves. The anterior discectomy or subtotal corpectomy with internal fixation or posterior simple open-door decompression with lateral mass screw fixation were performed in the patients. Among them, 39 cases were treated with traditional Chinese medicine after surgery. The Japanese orthopedic association (JOA) score of spinal cord function, the improvement rate of neural function, the neck dysfunction index (NDI) score and the governor vessel stasis syndrome score were compared between two groups preoperative and postoperative 1 week, 1 month and the final follow-up respectively. The internal fixation and the condition of spinal cord decompression were observed by CT, MRI and X-rays before and after operation. All the operations were successful, no injuries such as dura mater, spinal cord and nerve root were found. All the wounds were healed without infection except one patient had a superficial infection. It was solved after intermittent debridement and anti-infective therapy. Hematoma occurred in 1 case, complicated with spinal cord compression, caused incomplete paralysis, and promptly performed the re-operation to remove the hematoma without any obvious sequelae. All the patients were followed up from 12 to 24 months, (14.6±0.8) months for simple operation group and (13.5±0.7) months for operation combined with traditional Chinese medicine group, and there was no significant difference( P >0.05). The scores of JOA, NDI and

What's new in multiple sclerosis spasticity research? Poster session highlights.

Science.gov (United States)

Linker, Ralf

2017-11-01

Each year at the Multiple Sclerosis Experts Summit, relevant research in the field of multiple sclerosis spasticity is featured in poster sessions. The main studies presented at this year's meeting are summarized herein.

Motor activation in SPG4-linked hereditary spastic paraplegia

DEFF Research Database (Denmark)

Scheuer, KH; Nielsen, JE; Krabbe, Katja

2006-01-01

OBJECTIVE: The aim of this study was to investigate the extent of motor cortical functional reorganisation in patients with SPG4-linked hereditary spastic paraplegia by exploring cortical motor activation related to movements of clinically affected (lower) and unaffected (upper) limbs. METHODS: T...

Simultaneous characterizations of reflex and nonreflex dynamic and static changes in spastic hemiparesis

Science.gov (United States)

Chung, Sun G.; Ren, Yupeng; Liu, Lin; Roth, Elliot J.; Rymer, W. Zev

2013-01-01

This study characterizes tonic and phasic stretch reflex and stiffness and viscosity changes associated with spastic hemiparesis. Perturbations were applied to the ankle of 27 hemiparetic and 36 healthy subjects under relaxed or active contracting conditions. A nonlinear delay differential equation model characterized phasic and tonic stretch reflex gains, elastic stiffness, and viscous damping. Tendon reflex was characterized with reflex gain and threshold. Reflexively, tonic reflex gain was increased in spastic ankles at rest (P hemiparesis may help to evaluate and treat them more effectively. PMID:23636726

Increased lower limb muscle coactivation reduces gait performance and increases metabolic cost in patients with hereditary spastic paraparesis.

Science.gov (United States)

Rinaldi, Martina; Ranavolo, Alberto; Conforto, Silvia; Martino, Giovanni; Draicchio, Francesco; Conte, Carmela; Varrecchia, Tiwana; Bini, Fabiano; Casali, Carlo; Pierelli, Francesco; Serrao, Mariano

2017-10-01

The aim of this study was to investigate the lower limb muscle coactivation and its relationship with muscles spasticity, gait performance, and metabolic cost in patients with hereditary spastic paraparesis. Kinematic, kinetic, electromyographic and energetic parameters of 23 patients and 23 controls were evaluated by computerized gait analysis system. We computed ankle and knee antagonist muscle coactivation indexes throughout the gait cycle and during the subphases of gait. Energy consumption and energy recovery were measured as well. In addition to the correlation analysis between coactivation indexes and clinical variables, correlations between coactivation indexes and time-distance, kinematic, kinetic, and energetic parameters were estimated. Increased coactivity indexes of both knee and ankle muscles throughout the gait cycle and during the subphases of gait were observed in patients compared with controls. Energetic parameters were significantly higher in patients than in controls. Both knee and ankle muscle coactivation indexes were positively correlated with knee and ankle spasticity (Ashworth score), respectively. Knee and ankle muscle coactivation indexes were both positively correlated with energy consumption and both negatively correlated with energy recovery. Positive correlations between the Ashworth score and lower limb muscle coactivation suggest that abnormal lower limb muscle coactivation in patients with hereditary spastic paraparesis reflects a primary deficit linked to lower limb spasticity. Furthermore, these abnormalities influence the energetic mechanisms during walking. Identifying excessive muscle coactivation may be helpful in individuating the rehabilitative treatments and designing specific orthosis to restrain spasticity. Copyright © 2017 Elsevier Ltd. All rights reserved.

A single trial of transcutaneous electrical nerve stimulation (TENS) improves spasticity and balance in patients with chronic stroke.

Science.gov (United States)

Cho, Hwi-young; In, Tae Sung; Cho, Ki Hun; Song, Chang Ho

2013-03-01

Spasticity management is pivotal for achieving functional recovery of stroke patients. The purpose of this study was to investigate the effects of a single trial of transcutaneous electrical nerve stimulation (TENS) on spasticity and balance in chronic stroke patients. Forty-two chronic stroke patients were randomly allocated into the TENS (n = 22) or the placebo-TENS (n = 20) group. TENS stimulation was applied to the gastrocnemius for 60 min at 100 Hz, 200 µs with 2 to 3 times the sensory threshold (the minimal threshold in detecting electrical stimulation for subjects) after received physical therapy for 30 min. In the placebo-TENS group, electrodes were placed but no electrical stimulation was administered. For measuring spasticity, the resistance encountered during passive muscle stretching of ankle joint was assessed using the Modified Ashworth Scale, and the Hand held dynamometer was used to assess the resistive force caused by spasticity. Balance ability was measured using a force platform that measures postural sway generated by postural imbalance. The TENS group showed a significantly greater reduction in spasticity of the gastrocnemius, compared to the placebo-TENS group (p TENS resulted in greater balance ability improvements, especially during the eyes closed condition (p TENS provides an immediately effective means of reducing spasticity and of improving balance in chronic stroke patients. The present data may be useful to establish the standard parameters for TENS application in the clinical setting of stroke.

Muscle releases to improve passive motion and relieve pain in patients with spastic hemiplegia and elbow flexion contractures.

Science.gov (United States)

Namdari, Surena; Horneff, J Gabe; Baldwin, Keith; Keenan, Mary Ann

2012-10-01

Patients with spastic hemiplegia after upper motor neuron (UMN) injury can develop elbow contractures. This study evaluated outcomes of elbow releases in treating spastic elbow flexion contractures in hemiplegic patients. Adults with spastic hemiplegia due to UMN injury who underwent elbow releases (brachialis, brachioradialis, and biceps muscles) were included. Nonoperative treatment was unsuccessful in all patients. Patients complained of difficulty with passive functions. Passive range of motion (ROM), pain relief, Modified Ashworth spasticity score, and complications were evaluated preoperatively and postoperatively. There were 8 men and 21 women with an average age of 52.4 years (range, 24.1-81.4 years). Seventeen patients had pain preoperatively. Postoperative follow-up was a mean of 1.7 years (range, 1-4.5 years). Preoperatively, patients lacked a mean of 78° of passive elbow extension compared with 17° postoperatively (P .05). Releases of the brachialis, brachioradialis, and biceps muscles can be an effective means of pain relief, improved passive ROM, and decreased spasticity in patients with elbow flexion deformity after UMN injury. Copyright © 2012 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Mosby, Inc. All rights reserved.

Dynamic changes in the dural space and spinal cord cross-sectional area during flexion and extension in patients with cervical spondylotic myelopathy

International Nuclear Information System (INIS)

Machino, Masaaki; Yukawa, Yasutsugu; Ito, Keigo; Nakashima, Hiroaki; Kato, Fumihiko

2009-01-01

The number of patients with cervical spondylotic myelopathy (CSM) is increasing with the aging of the population. The patients' during spinal cord tends to be compressed neck extension, because the yellow ligaments and intervertebral discs protrude into the spinal canal during neck extension (pincer mechanism). A total of 100 patients with CSM were prospectively enrolled in this study. After preoperative myelography, multi-detector-row CT (MDCT) scans were acquired in flexion and extension, and the dural space and spinal cord cross-sectional area at each disc level from C2/3 to C7/Th1 were measured by using Scion imaging software. The average dural space and average spinal cord cross-sectional area were smaller in extension than in flexion from the C3/4 to C7/Th1 disc level, and the greatest dynamic changes were seen at the C5/6 level. MDCT demonstrated dynamic factors in patients with CSM. The spinal cord cross-sectional area became narrower during extension in patients with CSM. (author)

Disturbances of motor unit rate modulation are prevalent in muscles of spastic-paretic stroke survivors

Science.gov (United States)

Heckman, C. J.; Powers, R. K.; Rymer, W. Z.; Suresh, N. L.

2014-01-01

Stroke survivors often exhibit abnormally low motor unit firing rates during voluntary muscle activation. Our purpose was to assess the prevalence of saturation in motor unit firing rates in the spastic-paretic biceps brachii muscle of stroke survivors. To achieve this objective, we recorded the incidence and duration of impaired lower- and higher-threshold motor unit firing rate modulation in spastic-paretic, contralateral, and healthy control muscle during increases in isometric force generated by the elbow flexor muscles. Impaired firing was considered to have occurred when firing rate became constant (i.e., saturated), despite increasing force. The duration of impaired firing rate modulation in the lower-threshold unit was longer for spastic-paretic (3.9 ± 2.2 s) than for contralateral (1.4 ± 0.9 s; P unit was also longer for the spastic-paretic (1.7 ± 1.6 s) than contralateral (0.3 ± 0.3 s; P = 0.007) and control (0.1 ± 0.2 s; P = 0.009) muscles. This impaired firing rate of the lower-threshold unit arose, despite an increase in the overall descending command, as shown by the recruitment of the higher-threshold unit during the time that the lower-threshold unit was saturating, and by the continuous increase in averages of the rectified EMG of the biceps brachii muscle throughout the rising phase of the contraction. These results suggest that impairments in firing rate modulation are prevalent in motor units of spastic-paretic muscle, even when the overall descending command to the muscle is increasing. PMID:24572092

Surgical correction of scoliosis in children with spastic quadriplegia: benefits, adverse effects, and patient selection.

Science.gov (United States)

Legg, Julian; Davies, Evan; Raich, Annie L; Dettori, Joseph R; Sherry, Ned

2014-04-01

Cerebral palsy (CP) is a group of nonprogressive syndromes of posture and motor impairment associated with lesions of the immature brain. Spastic quadriplegia is the most severe form with a high incidence of scoliosis, back pain, respiratory compromise, pelvic obliquity, and poor sitting balance. Surgical stabilization of the spine is an effective technique for correcting deformity and restoring sitting posture. The decision to operate in this group of patients is challenging. The aim of this study is to determine the benefits of surgical correction of scoliosis in children with spastic quadriplegia, the adverse effects of this treatment, and what preoperative factors affect patient outcome after surgical correction. A systematic review was undertaken to identify studies describing benefits and adverse effects of surgery in spastic quadriplegia. Factors affecting patient outcome following surgical correction of scoliosis were assessed. Studies involving adults and nonspastic quadriplegia were excluded. A total of 10 case series and 1 prospective and 3 retrospective cohort studies met inclusion criteria. There was significant variation in the overall risk of complications (range, 10.9-70.9%), mortality (range, 2.8-19%), respiratory/pulmonary complications (range, 26.9-57.1%), and infection (range, 2.5-56.8%). Factors associated with a worse outcome were a significant degree of thoracic kyphosis, days in the intensive care unit, and poor nutritional status. Caregivers report a high degree of satisfaction with scoliosis surgery for children with spastic quadriplegia. There is limited evidence of preoperative factors that can predict patient outcome after scoliosis. There is a need for well-designed prospective studies of scoliosis surgery in spastic quadriplegia.

Caregiver burden, productivity loss, and indirect costs associated with caring for patients with poststroke spasticity

Directory of Open Access Journals (Sweden)

Ganapathy V

2015-11-01

Full Text Available Vaidyanathan Ganapathy,1 Glenn D Graham,2 Marco D DiBonaventura,3 Patrick J Gillard,1 Amir Goren,3 Richard D Zorowitz41Allergan, Irvine, CA, USA; 2Department of Veterans Affairs, San Francisco, CA, USA; 3Health Outcomes Practice, Kantar Health, New York, NY, USA; 4Johns Hopkins Bayview Medical Center, Baltimore, MD, USAObjective: Many stroke survivors experience poststroke spasticity and the related inability to perform basic activities, which necessitates patient management and treatment, and exerts a considerable burden on the informal caregiver. The current study aims to estimate burden, productivity loss, and indirect costs for caregivers of stroke survivors with spasticity.Methods: Internet survey data were collected from 153 caregivers of stroke survivors with spasticity including caregiving time and difficulty (Oberst Caregiver Burden Scale, Work Productivity and Activity Impairment measures, and caregiver and patient characteristics. Fractional logit models examined predictors of work-related restriction, and work losses were monetized (2012 median US wages.Results: Mean Oberst Caregiver Burden Scale time and difficulty scores were 46.1 and 32.4, respectively. Employed caregivers (n=71 had overall work restriction (32%, absenteeism (9%, and presenteeism (27%. Caregiver characteristics, lack of nursing home coverage, and stroke survivors’ disability predicted all work restriction outcomes. The mean total lost-productivity cost per employed caregiver was US$835 per month (>$10,000 per year; 72% attributable to presenteeism.Conclusion: These findings demonstrate the substantial burden of caring for stroke survivors with spasticity illustrating the societal and economic impact of stroke that extends beyond the stroke survivor.Keywords: burden, caregiver, productivity, spasticity, stroke

Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders

Directory of Open Access Journals (Sweden)

Ingrid Faber

Full Text Available ABSTRACT Hereditary spastic paraplegia (HSP is a diverse group of single-gene disorders that share the predominant clinical feature of progressive lower limb spasticity and weakness. More than 70 different genetic subtypes have been described and all modes of inheritance are possible. Intellectual dysfunction in HSP is frequent in recessive forms but rare in dominant families. It may manifest by either mental retardation and/or cognitive decline. The latter may be subtle, restricted to executive dysfunction or may evolve to severe dementia. The cognitive profile is thought to depend largely on the genetic subtype of HSP, although wide phenotypic variability within the same genetic subtype and also within the same family can be found.

Surgical management of spasticity | Enslin | South African Medical ...

African Journals Online (AJOL)

The management of patients with cerebral palsy and other causes of spasticity is a challenge to an entire rehabilitation team and to caregivers. In South Africa, neurosurgeons have had limited involvement in this field owing to a perceived lack of options, leaving the care of these patients largely in the hands of paediatric ...

Lower limb spasticity assessment using an inertial sensor: a reliability study

International Nuclear Information System (INIS)

Sterpi, I; Colombo, R; Caroli, A; Meazza, E; Maggioni, G; Pistarini, C

2013-01-01

Spasticity is a common motor impairment in patients with neurological disorders that can prevent functional recovery after rehabilitation. In the clinical setting, its assessment is carried out using standardized clinical scales. The aim of this study was to verify the applicability of inertial sensors for an objective measurement of quadriceps spasticity and evaluate its test–retest and inter-rater reliability during the implementation of the Wartenberg pendulum test. Ten healthy subjects and 11 patients in vegetative state with severe brain damage were enrolled in this study. Subjects were evaluated three times on three consecutive days. The test–retest reliability of measurement was assessed in the first two days. The third day was devoted to inter-rater reliability assessment. In addition, the lower limb muscle tone was bilaterally evaluated at the knee joint by the modified Ashworth scale. The factorial ANOVA analysis showed that the implemented method allowed us to discriminate between healthy and pathological conditions. The fairly low SEM and high ICC values obtained for the pendulum parameters indicated a good test–retest and inter-rater reliability of measurement. This study shows that an inertial sensor can be reliably used to characterize leg kinematics during the Wartenberg pendulum test and provide quantitative evaluation of quadriceps spasticity. (paper)

Serial casting for neuromuscular flatfoot and vertical talus in an adolescent with hereditary spastic paraplegia.

Science.gov (United States)

Sweet, Laurene A; OʼNeill, Lindsey M; Dobbs, Matthew B

2014-01-01

The purpose of this report is to explore assessment and serial casting intervention for painful rigid flatfoot deformities with vertical talus in an adolescent girl with hereditary spastic paraplegia who was nonambulatory. The participant's right foot underwent 2 phases of casting with correction first toward hindfoot inversion and then dorsiflexion. Because of a vertical talus, her left foot required an intermediate casting toward plantar flexion, inversion, and forefoot adduction prior to casting toward dorsiflexion. The patient improved despite the underlying progressive neuromuscular disorder. Pain ameliorated and she returned to supported standing and transfers. Spasticity decreased bilaterally and the flexibility of her foot deformities improved to allow orthotic fabrication in subtalar neutral. Results were maintained at 12 and 16 months. Individualized multiphase serial casting requires further investigation with patients such as those with hereditary spastic paraplegia.

Intramuscular Connective Tissue Differences in Spastic and Control Muscle: A Mechanical and Histological Study

Science.gov (United States)

de Bruin, Marije; Smeulders, Mark J.; Kreulen, Michiel; Huijing, Peter A.; Jaspers, Richard T

2014-01-01

Cerebral palsy (CP) of the spastic type is a neurological disorder characterized by a velocity-dependent increase in tonic stretch reflexes with exaggerated tendon jerks. Secondary to the spasticity, muscle adaptation is presumed to contribute to limitations in the passive range of joint motion. However, the mechanisms underlying these limitations are unknown. Using biopsies, we compared mechanical as well as histological properties of flexor carpi ulnaris muscle (FCU) from CP patients (n = 29) and healthy controls (n = 10). The sarcomere slack length (mean 2.5 µm, SEM 0.05) and slope of the normalized sarcomere length-tension characteristics of spastic fascicle segments and single myofibre segments were not different from those of control muscle. Fibre type distribution also showed no significant differences. Fibre size was significantly smaller (1933 µm2, SEM 190) in spastic muscle than in controls (2572 µm2, SEM 322). However, our statistical analyses indicate that the latter difference is likely to be explained by age, rather than by the affliction. Quantities of endomysial and perimysial networks within biopsies of control and spastic muscle were unchanged with one exception: a significant thickening of the tertiary perimysium (3-fold), i.e. the connective tissue reinforcement of neurovascular tissues penetrating the muscle. Note that this thickening in tertiary perimysium was shown in the majority of CP patients, however a small number of patients (n = 4 out of 23) did not have this feature. These results are taken as indications that enhanced myofascial loads on FCU is one among several factors contributing in a major way to the aetiology of limitation of movement at the wrist in CP and the characteristic wrist position of such patients. PMID:24977410

Analysis of Patients with Myelopathy due to Benign Intradural Spinal Tumors with Concomitant Lumbar Degenerative Diseases Misdiagnosed and Erroneously Treated with Lumbar Surgery.

Science.gov (United States)

Lu, Kang; Wang, Hao-Kuang; Liliang, Po-Chou; Yang, Chih-Hui; Yen, Cheng-Yo; Tsai, Yu-Duan; Chen, Po-Yuan; Chye, Cien-Leong; Wang, Kuo-Wei; Liang, Cheng-Loong; Chen, Han-Jung

2017-09-01

When a cervical or thoracic benign intradural spinal tumor (BIST) coexists with lumbar degenerative diseases (LDD), diagnosis can be difficult. Symptoms of BIST-myelopathy can be mistaken as being related to LDD. Worse, an unnecessary lumbar surgery could be performed. This study was conducted to analyze cases in which an erroneous lumbar surgery was undertaken in the wake of failure to identify BIST-associated myelopathy. Cases were found in a hospital database. Patients who underwent surgery for LDD first and then another surgery for BIST removal within a short interval were studied. Issues investigated included why the BISTs were missed, how they were found later, and how the patients reacted to the unnecessary lumbar procedures. Over 10 years, 167 patients received both surgeries for LDD and a cervical or thoracic BIST. In 7 patients, lumbar surgery preceded tumor removal by a short interval. Mistakes shared by the physicians included failure to detect myelopathy and a BIST, and a hasty decision for lumbar surgery, which soon turned out to be futile. Although the BISTs were subsequently found and removed, 5 patients believed that the lumbar surgery was unnecessary, with 4 patients expressing regrets and 1 patient threatening to take legal action against the initial surgeon. Concomitant symptomatic LDD and BIST-associated myelopathy pose a diagnostic challenge. Spine specialists should refrain from reflexively linking leg symptoms and impaired ability to walk to LDD. Comprehensive patient evaluation is fundamental to avoid misdiagnosis and wrong lumbar surgery. Copyright © 2017 Elsevier Inc. All rights reserved.

Botulinum toxin type A chemodenervation treatment in spastic forms of cerebral palsy

Directory of Open Access Journals (Sweden)

A. L. Kurenkov

2013-01-01

Full Text Available Cerebral palsy (CP is one of the most serious outcomes of the perinatal lesion of central nervous system and the most common reason for neurological disability in children. Being the key cause of pathological dynamic stereotypes that frequently result in pathological posture and contractures, spasticity is critically important for CP. The use of botulinum toxin type A (BTA in complex treatment 2-6 years old CP patients allows significantly to improve motor abilities, help to change the surgical procedure, delay or even avoid some types of surgery. For elder children the use of BTA allows to improve local motor impairment. The treatment of spasticity in CP with BTA is safe (evidence level A and highly effective (evidence level A. It leads to the positive change of pathological dynamic stereotype, significantly improves gait, decreases muscle tone with Ashworth and Tardeu scales and rises the gross motor function score. Our own experience of onabotulinumtoxinA treatment as a part of complex therapy in 68 patients with spastic forms of CP demonstrates the significant improvement of motor function, most noticeable in younger patients(early pre-school age with GMFS I-III.

Modulation of back geometry in children with spastic diplegic ...

African Journals Online (AJOL)

Gehan H. El-Meniawy

2011-11-29

Nov 29, 2011 ... disability in childhood and may affect the child on several ... Children with spastic diplegia have significant weakness in ... The Formetric instrument system represents a reliable meth- .... The child was almost always feeling some degree of gentle ..... Growth and Developmental Disorder in Children and its.

Interaction of HTLV-1 Tax protein with the calreticulin: Implications for Tax nuclear export and secretion

OpenAIRE

Alefantis, Timothy; Flaig, Katherine E.; Wigdahl, Brian; Jain, Pooja

2007-01-01

Human T cell leukemia virus type 1 (HTLV-1) is the etiologic agent of adult T cell leukemia (ATL) and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). The HTLV-1 transcriptional transactivator protein Tax plays an integral role in virus replication and disease progression. Traditionally, Tax is described as a nuclear protein where it performs its primary role as a transcriptional transactivator. However, recent studies have clearly shown that Tax can also be localized to t...

Caregiver burden, productivity loss, and indirect costs associated with caring for patients with poststroke spasticity

Science.gov (United States)

Ganapathy, Vaidyanathan; Graham, Glenn D; DiBonaventura, Marco D; Gillard, Patrick J; Goren, Amir; Zorowitz, Richard D

2015-01-01

Objective Many stroke survivors experience poststroke spasticity and the related inability to perform basic activities, which necessitates patient management and treatment, and exerts a considerable burden on the informal caregiver. The current study aims to estimate burden, productivity loss, and indirect costs for caregivers of stroke survivors with spasticity. Methods Internet survey data were collected from 153 caregivers of stroke survivors with spasticity including caregiving time and difficulty (Oberst Caregiver Burden Scale), Work Productivity and Activity Impairment measures, and caregiver and patient characteristics. Fractional logit models examined predictors of work-related restriction, and work losses were monetized (2012 median US wages). Results Mean Oberst Caregiver Burden Scale time and difficulty scores were 46.1 and 32.4, respectively. Employed caregivers (n=71) had overall work restriction (32%), absenteeism (9%), and presenteeism (27%). Caregiver characteristics, lack of nursing home coverage, and stroke survivors’ disability predicted all work restriction outcomes. The mean total lost-productivity cost per employed caregiver was US$835 per month (>$10,000 per year; 72% attributable to presenteeism). Conclusion These findings demonstrate the substantial burden of caring for stroke survivors with spasticity illustrating the societal and economic impact of stroke that extends beyond the stroke survivor. PMID:26609225

Mechanistic basis of an epistatic interaction reducing age at onset in hereditary spastic paraplegia.

Science.gov (United States)

Newton, Timothy; Allison, Rachel; Edgar, James R; Lumb, Jennifer H; Rodger, Catherine E; Manna, Paul T; Rizo, Tania; Kohl, Zacharias; Nygren, Anders O H; Arning, Larissa; Schüle, Rebecca; Depienne, Christel; Goldberg, Lisa; Frahm, Christiane; Stevanin, Giovanni; Durr, Alexandra; Schöls, Ludger; Winner, Beate; Beetz, Christian; Reid, Evan

2018-05-01

Many genetic neurological disorders exhibit variable expression within affected families, often exemplified by variations in disease age at onset. Epistatic effects (i.e. effects of modifier genes on the disease gene) may underlie this variation, but the mechanistic basis for such epistatic interactions is rarely understood. Here we report a novel epistatic interaction between SPAST and the contiguous gene DPY30, which modifies age at onset in hereditary spastic paraplegia, a genetic axonopathy. We found that patients with hereditary spastic paraplegia caused by genomic deletions of SPAST that extended into DPY30 had a significantly younger age at onset. We show that, like spastin, the protein encoded by SPAST, the DPY30 protein controls endosomal tubule fission, traffic of mannose 6-phosphate receptors from endosomes to the Golgi, and lysosomal ultrastructural morphology. We propose that additive effects on this pathway explain the reduced age at onset of hereditary spastic paraplegia in patients who are haploinsufficient for both genes.

The benefits of a comprehensive rehabilitation program in patients diagnosed with spastic quadriplegia.

Science.gov (United States)

Rogoveanu, O C; Tuțescu, N C; Kamal, D; Alexandru, D O; Kamal, C; Streba, C T; Trăistaru, M R

2016-01-01

Spastic quadriplegia has as an etiopathogenic substrate, a non-progressive brain lesion; however, the clinical manifestations of the disease evolve over time. Children diagnosed with spastic quadriplegia show a variety of symptoms in different areas: sensorimotor, emotional, cognitive, and social. The purpose of this study was to assess the functional status in patients diagnosed with spastic quadriplegia, who followed a complex medical rehabilitation program, during a year, and highlight the importance of using physical and kinetic techniques in improving their status. A total of 10 children diagnosed with spastic quadriplegia were included in the study and the Gross Motor Function Classification System (GMFCS) and manual ability classification system (MACS) were used to evaluate the functionality status of each patient. Every patient was evaluated initially (T1), after six months of program (T2), and after they completed the study. All the children were originally monitored daily, for 5 days per week for a period of one month, then two times a week for a year. A statistically significant difference regarding the modification of the GMFCS and MACS stage was found, which occurred between the first and the third evaluation. The inverse correlation of the statistical significance between the ages of patients and the decrease in GMFCS or MACS stage was highlighted; the younger the patient, the more the scale decreased. A direct link between the gross motor function and the manual ability was noticed. Applying a complex rehabilitation program has proven efficient by improving both the gross motor functionality and the manual ability.

Health Economics and the Management of Degenerative Cervical Myelopathy.

Science.gov (United States)

Witiw, Christopher D; Smieliauskas, Fabrice; Fehlings, Michael G

2018-01-01

Degenerative cervical myelopathy (DCM) is the leading cause of spinal cord impairment worldwide. Surgical intervention has been demonstrated to be effective and is becoming standard of care. Spine surgery, however, is costly and value needs to be demonstrated. This review serves to summarize the key health economic concepts as they relate to the assessment of the value of surgery for DCM. This is followed by a discussion of current health economic research on DCM, which suggests that surgery is likely to be cost effective. The review concludes with a summary of future questions that remain unanswered, such as which patient subgroups derive the most value from surgery and which surgical approaches are the most cost effective. Copyright © 2017 Elsevier Inc. All rights reserved.

Altered sense of agency in children with spastic cerebral palsy

DEFF Research Database (Denmark)

Ritterband-Rosenbaum, Anina; Christensen, Mark S; Kliim-Due, Mette

2011-01-01

ABSTRACT: Background Children diagnosed with spastic Cerebral Palsy (CP) often show perceptual and cognitive problems, which may contribute to their functional deficit. Here we investigated if altered ability to determine whether an observed movement is performed by themselves (sense of agency...

Thoracic Synovial Cyst at the Th2-3 Level Causing Myelopathy

DEFF Research Database (Denmark)

Sundskarð, Martin M; Gaini, Shahin

2017-01-01

Intraspinal synovial cyst is a rare cause of myelopathy. These cysts present most often in the lumbar and cervical parts of the spine but are more infrequent in the thoracic spine. We present a case of a 73-year-old man with an intraspinal, extradural synovial cyst at the Th2-3 level causing...... paraesthesia and weakness in the legs. A laminectomy and excision of the cyst were performed and the patient recovered fully. In the thoracic spine, synovial cysts are almost exclusively found in the lower part. Laminectomy, with excision, is the treatment of choice, although steroid injections have been...

KAPS (kinematic assessment of passive stretch): a tool to assess elbow flexor and extensor spasticity after stroke using a robotic exoskeleton.

Science.gov (United States)

Centen, Andrew; Lowrey, Catherine R; Scott, Stephen H; Yeh, Ting-Ting; Mochizuki, George

2017-06-19

Spasticity is a common sequela of stroke. Traditional assessment methods include relatively coarse scales that may not capture all characteristics of elevated muscle tone. Thus, the aim of this study was to develop a tool to quantitatively assess post-stroke spasticity in the upper extremity. Ninety-six healthy individuals and 46 individuals with stroke participated in this study. The kinematic assessment of passive stretch (KAPS) protocol consisted of passive elbow stretch in flexion and extension across an 80° range in 5 movement durations. Seven parameters were identified and assessed to characterize spasticity (peak velocity, final angle, creep (or release), between-arm peak velocity difference, between-arm final angle, between-arm creep, and between-arm catch angle). The fastest movement duration (600 ms) was most effective at identifying impairment in each parameter associated with spasticity. A decrease in peak velocity during passive stretch between the affected and unaffected limb was most effective at identifying individuals as impaired. Spasticity was also associated with a decreased passive range (final angle) and a classic 'catch and release' as seen through between-arm catch and creep metrics. The KAPS protocol and robotic technology can provide a sensitive and quantitative assessment of post-stroke elbow spasticity not currently attainable through traditional measures.

Prediction of the recovery rate after surgery for cervical myelopathy from the view of CT-myelography

International Nuclear Information System (INIS)

Koyanagi, Takahiro; Satomi, Kazuhiko; Asazuma, Takahito; Toyama, Yoshiaki; Fujimura, Shoichi; Hirabayashi, Kiyoshi; Hamano, Yasuyuki; Shiraishi, Takeshi.

1991-01-01

This study was designed to prepare a formula for predicting postoperative recovery in cervical myelopathy. Preoperative CT-myelography (CT-M) was performed in a total of 103 patients, consisting of 44 with cervical spinal myelopathy (CSM), 39 with ossification of the posterior longitudinal ligament (OPLL), and 20 with cervical disk herniation (CDH). Multivariate analyses were used to obtain correlations between CT-M findings (spinal cord area and the rate of spinal cord flatness) and clinical items (age, disease duration, preoperative JOA score, and postoperative recovery rate). There was a strong positive correlation between spinal cord area and postoperative recovery rate. Because both spinal cord area and disease duration for the CSM and OPLL groups had a strong positive correlation with the recovery rate, they were found to predict postoperative recovery. In the CDH group, there was no predictive index. Spinal cord area was more potential index than preoperative severity. Disease duration may also serve as an index complementing spinal cord area in the evaluation of postoperative recovery. (N.K.)

Neuropsychological improvement in patients with cervical spondylotic myelopathy after posterior decompression surgery

International Nuclear Information System (INIS)

Hoshimaru, Minoru

2010-01-01

Patients with cervical spondylotic myelopathy sometimes complain of cognitive dysfunction, which may be coincidence. However, cognitive dysfunction may be related to disorders of the cervical spine and/or spinal cord. This study investigated cognitive dysfunction in patients with cervical spinal disorders. A total of 79 patients with cervical spondylotic myelopathy (40 women and 39 men, mean age 61.2 years) underwent cervical laminoplasty between January 2006 and July 2007. Ten of these 79 patients (7 women and 3 men, mean age 65.2 years) complained of moderate to severe memory disturbances. These 10 patients underwent neuroimaging studies and a battery of neuropsychological tests consisting of the mini-mental state examination, Kohs Block Design Test, Miyake Memory Test, Benton Visual Retention Test (BVRT), and 'kana-hiroi' test before and 3 months after surgery. Brain magnetic resonance imaging showed no organic brain lesions in the 10 patients, but single photon emission computed tomography demonstrated reduced regional cerebral blood flow in the posterior cortical areas in eight patients before surgery. Neuropsychological test scores showed statistically significant improvement after surgery in the Kohs Block Design Test and the BVRT, which measure visuospatial perception and reflect the function of the parietal and/or occipital lobes (p<0.05). The practice effect may have contributed to the neuropsychological improvements, but this study suggests that cervical spinal disorders may affect cognitive functions and that surgical treatment can ameliorate such effects. (author)

The contribution of cognition and spasticity to driving performance in multiple sclerosis.

Science.gov (United States)

Marcotte, Thomas D; Rosenthal, Theodore J; Roberts, Erica; Lampinen, Sara; Scott, J Cobb; Allen, R Wade; Corey-Bloom, Jody

2008-09-01

To examine the independent and combined impact of cognitive dysfunction and spasticity on driving tasks involving high cognitive workload and lower-limb mobility in persons with multiple sclerosis (MS). Single-visit cohort study. Clinical research center. Participants included 17 drivers with MS and 14 referent controls. The group with MS exhibited a broad range of cognitive functioning and disability. Of the 17 patients with MS, 8 had significant spasticity in the knee used to manipulate the accelerator and brake pedals (based on the Modified Ashworth Scale). Not applicable. A brief neuropsychologic test battery and 2 driving simulations. Simulation 1 required participants to maintain a constant speed and lane position while attending to a secondary task. Simulation 2 required participants to adjust their speed to accelerations and decelerations of a lead car in front of them. Patients with MS showed greater variability in lane position (effect size, g=1.30), greater difficulty in maintaining a constant speed (g=1.25), and less ability to respond to lead car speed changes (g=1.85) compared with controls. Within the MS group, in a multivariate model that included neuropsychologic and spasticity measures, cognitive functioning was the strongest predictor of difficulty in maintaining lane position during the divided attention task and poor response time to lead car speed changes, whereas spasticity was associated with reductions in accuracy of tracking the lead car movements and speed maintenance. In this preliminary study, cognitive and physical impairments associated with MS were related to deficits in specific components of simulated driving. Assessment of these factors may help guide the clinician regarding the types of driving behaviors that would put patients with MS at an increased risk for an automobile crash.

Cervical spondylotic myelopathy

African Journals Online (AJOL)

function. Seventy-five per cent of patients with mild symptoms (sensory changes but no functional ... of both motor and sensory function as well as ... pathological and compressive) structures, ... management of cervical degenerative disease:.

Spasticity Measurement Based on Tonic Stretch Reflex Threshold in Children with Cerebral Palsy Using the PediAnklebot

Directory of Open Access Journals (Sweden)

Marco Germanotta

2017-05-01

Full Text Available Nowadays, objective measures are becoming prominent in spasticity assessment, to overcome limitations of clinical scales. Among others, Tonic Stretch Reflex Threshold (TSRT showed promising results. Previous studies demonstrated the validity and reliability of TSRT in spasticity assessment at elbow and ankle joints in adults. Purposes of the present study were to assess: (i the feasibility of measuring TSRT to evaluate spasticity at the ankle joint in children with Cerebral Palsy (CP, and (ii the correlation between objective measures and clinical scores. A mechatronic device, the pediAnklebot, was used to impose 50 passive stretches to the ankle of 10 children with CP and 3 healthy children, to elicit muscles response at 5 different velocities. Surface electromyography, angles, and angular velocities were recorded to compute dynamic stretch reflex threshold; TSRT was computed with a linear regression through angles and angular velocities. TSRTs for the most affected side of children with CP resulted into the biomechanical range (95.7 ± 12.9° and 86.7 ± 17.4° for Medial and Lateral Gastrocnemius, and 75.9 ± 12.5° for Tibialis Anterior. In three patients, the stretch reflex was not elicited in the less affected side. TSRTs were outside the biomechanical range in healthy children. However, no correlation was found between clinical scores and TSRT values. Here, we demonstrated the capability of TSRT to discriminate between spastic and non-spastic muscles, while no significant outcomes were found for the dorsiflexor muscle.

Spastic pelvic floor syndrome: Definition in double-exposure defaecography

International Nuclear Information System (INIS)

Helzel, M.V.

1989-01-01

Double-exposure defaecography and the so-called pinching test improve conventional defaecography in the diagnosis of functional rectal outlet disorders. In particular, the pinching test makes quantitative evaluation of the m. puborectalis possible. 'Spastic pelvic floor syndrome' is defined by quantitative parameters in double-exposure defaecography and the pinching test. (orig.) [de

Classification of topographical pattern of spasticity in cerebral palsy: a registry perspective.

Science.gov (United States)

Reid, Susan M; Carlin, John B; Reddihough, Dinah S

2011-01-01

This study used data from a population-based cerebral palsy (CP) registry and systematic review to assess the amount of heterogeneity between registries in topographical patterns when dichotomised into unilateral (USCP) and bilateral spastic CP (BSCP), and whether the terms diplegia and quadriplegia provide useful additional epidemiological information. From the Victorian CP Register, 2956 individuals (1658 males, 1298 females), born 1970-2003, with spastic CP were identified. The proportions with each topographical pattern were analysed overall and by gestational age. Binary logistic regression analysis was used to assess temporal trends. For the review, data were systematically collected on topographical patterns from 27 registries. Estimates of heterogeneity were obtained, overall and by region, reporting period and definition of quadriplegia. Among individuals born <32 weeks, 48% had diplegia, whereas the proportion for children born ≥ 32 weeks was 24% (p < 0.001). Evidence was weak for a temporal trend in the relative proportions of USCP and BSCP (p = 0.038), but much clearer for an increase in the proportion of spastic diplegia relative to quadriplegia (p < 0.001). The review revealed wide variations across studies in the proportion of diplegia (range 34-90%) and BSCP (range 51-86%). These findings argue against a topographical classification based solely on laterality. Copyright © 2011 Elsevier Ltd. All rights reserved.

A review of the properties and limitations of the Ashworth and modified Ashworth Scales as measures of spasticity.

Science.gov (United States)

Pandyan, A D; Johnson, G R; Price, C I; Curless, R H; Barnes, M P; Rodgers, H

1999-10-01

The Ashworth Scale and the modified Ashworth Scale are the primary clinical measures of spast city. A prerequisite for using any scale is a knowledge of its characteristics and limitations, as these will play a part in analysing and interpreting the data. Despite the current emphasis on treating spasticity, clinicians rarely measure it. To determine the validity and the reliability of the Ashworth and modified Ashworth Scales. A theoretical analysis following a structured literature review (key words: Ashworth; Spasticity; Measurement) of 40 papers selected from the BIDS-EMBASE, First Search and Medline databases. The application of both scales would suggest that confusion exists on their characteristics and limitations as measures of spasticity. Resistance to passive movement is a complex measure that will be influenced by many factors, only one of which could be spasticity. The Ashworth Scale (AS) can be used as an ordinal level measure of resistance to passive movement, but not spasticity. The modified Ashworth Scale (MAS) will need to be treated as a nominal level measure of resistance to passive movement until the ambiguity between the '1' and '1+' grades is resolved. The reliability of the scales is better in the upper limb. The AS may be more reliable than the MAS. There is a need to standardize methods to apply these scales in clinical practice and research.

Cervical spine abnormalities and instability with myelopathy in warfarin-related chondrodysplasia: 17-year follow-up

International Nuclear Information System (INIS)

Takano, Hideyuki; Smith, W.L.; Sato, Yutaka; Kao, S.C.S.

1998-01-01

A patient with warfarin embryopathy developed progressive cervical spinal myelopathy owing to bony cervical spinal damage. While there are several descriptions of warfarin embryopathy, the long-term complication of cervical spinal instability has not been reported. This cervical instability may, as in our patient, cause severe neurological dysfunction or even sudden death; therefore, it is important that pediatric radiologists should be alert to this condition. (orig.)

Neuropathic pain and spasticity: intricate consequences of spinal cord injury

DEFF Research Database (Denmark)

Finnerup, Nanna Brix

2017-01-01

of SCI, and a careful examination and characterization of the symptoms and signs, are a prerequisite for understanding the relationship between neuropathic pain and spasticity and the intricate underlying mechanisms.Spinal Cord advance online publication, 11 July 2017; doi:10.1038/sc.2017.70....

Functional electrical stimulation of the ankle dorsiflexors during walking in spastic cerebral palsy: a systematic review.

Science.gov (United States)

Moll, Irene; Vles, Johannes S H; Soudant, Dan L H M; Witlox, Adhiambo M A; Staal, Heleen M; Speth, Lucianne A W M; Janssen-Potten, Yvonne J M; Coenen, Marcel; Koudijs, Suzanne M; Vermeulen, R Jeroen

2017-12-01

To assess the effect of functional electrical stimulation (FES) of ankle dorsiflexors in children and adolescents with spastic cerebral palsy (CP) during walking. A systematic review was performed using the American Academy of Cerebral Palsy and Developmental Medicine methodology and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Six databases were searched for studies applying interventions to patients aged younger than 20 years. Outcomes were classified according to the International Classification of Functioning, Disability and Health (ICF). Seven hundred and eighty abstracts were found, 35 articles were fully screened, and 14 articles were used for analysis. Only five articles (three studies) were of level I to III evidence. At ICF participation and activity level, there is limited evidence for a decrease in self-reported frequency of toe-drag and falls. At ICF body structure and function level, there is clear evidence (I-III) that FES increased (active) ankle dorsiflexion angle, strength, and improved selective motor control, balance, and gait kinematics, but decreased walking speed. Adverse events include skin irritation, toleration, and acceptation issues. There are insufficient data supporting functional gain by FES on activity and participation level. However, evidence points towards a role for FES as an alternative to orthoses in children with spastic CP. Effects of functional electrical stimulation (FES) point towards a potential role as an alternative to orthoses for patients with spastic cerebral palsy (CP). Some evidence for a decrease in self-reported frequency of toe-drag and falls with the use of FES in spastic CP. Limited evidence for improvements in activity and participation in patients with spastic CP using FES. © 2017 Mac Keith Press.

Effects of hippotherapy on gait parameters in children with bilateral spastic cerebral palsy.

Science.gov (United States)

Kwon, Jeong-Yi; Chang, Hyun Jung; Lee, Ji Young; Ha, Yumi; Lee, Peter K; Kim, Yun-Hee

2011-05-01

To evaluate the effects of hippotherapy on temporospatial parameters and pelvic and hip kinematics of gait in children with bilateral spastic cerebral palsy. Nonrandomized prospective controlled trial. Outpatient therapy center. Children (N=32) with bilateral spastic cerebral palsy, Gross Motor Function Classification System level 1 or 2. Hippotherapy (30 min twice weekly for 8 consecutive weeks). Temporospatial parameters and pelvic and hip kinematic parameters in 3-dimensional motion analysis, Gross Motor Function Measure (GMFM)-88, and score for dimensions D (standing) and E (walking, running, jumping) of the GMFM, GMFM-66, and Pediatric Balance Scale (PBS). Hippotherapy significantly improved walking speed, stride length, and pelvic kinematics (average pelvic anterior tilt, pelvic anterior tilt at initial contact, pelvic anterior tilt at terminal stance). Scores for dimension E of the GMFM, GMFM-66 and PBS also increased. Hippotherapy provided by licensed health professionals using the multidimensional movement of the horse may be used in conjunction with standard physical therapy for improvement of gait and balance in children with bilateral spastic cerebral palsy. Copyright © 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Subacute combined degeneration of the spinal cord: MRI detection of preferential involvement of the posterior columns in a child

International Nuclear Information System (INIS)

Wolansky, L.J.; Goldstein, G.; Gozo, A.; Lee, H.J.; Sills, I.; Chatkupt, S.

1995-01-01

Subacute combined degeneration of the spinal cord (vitamin B 12 -deficient myelopathy) is a neurologic disorder manifesting progressive symptoms of paresthesia and spastic paralysis. As shown by pathology, it initially involves the posterior columns of the thoracic cord. We present a case of vitamin B 12 deficiency with preferential posterior column involvement of the thoracic cord in a child. Theoretically, this should be the earliest, most reversible stage of the disease, making recognition of this MRI pattern of critical importance. (orig.)

Subacute combined degeneration of the spinal cord: MRI detection of preferential involvement of the posterior columns in a child

Energy Technology Data Exchange (ETDEWEB)

Wolansky, L.J. [Dept. of Radiology, UMDNJ-New Jersey Medical School, Newark, NJ (United States); Goldstein, G. [Dept. of Radiology, UMDNJ-New Jersey Medical School, Newark, NJ (United States); Gozo, A. [Dept. of Neurosciences, UMDNJ-New Jersey Medical School, Newark, NJ (United States)]|[Dept. of Pediatrics, UMDNJ-New Jersey Medical School, Newark, NJ (United States)]|[Children`s Hospital of New Jersey, Newark, NJ (United States); Lee, H.J. [Dept. of Radiology, UMDNJ-New Jersey Medical School, Newark, NJ (United States); Sills, I. [Dept. of Pediatrics, UMDNJ-New Jersey Medical School, Newark, NJ (United States)]|[Children`s Hospital of New Jersey, Newark, NJ (United States); Chatkupt, S. [Dept. of Neurosciences, UMDNJ-New Jersey Medical School, Newark, NJ (United States)]|[Dept. of Pediatrics, UMDNJ-New Jersey Medical School, Newark, NJ (United States)]|[Children`s Hospital of New Jersey, Newark, NJ (United States)

1995-03-01

Subacute combined degeneration of the spinal cord (vitamin B{sub 12}-deficient myelopathy) is a neurologic disorder manifesting progressive symptoms of paresthesia and spastic paralysis. As shown by pathology, it initially involves the posterior columns of the thoracic cord. We present a case of vitamin B{sub 12} deficiency with preferential posterior column involvement of the thoracic cord in a child. Theoretically, this should be the earliest, most reversible stage of the disease, making recognition of this MRI pattern of critical importance. (orig.)

The prevalence of cervical myelopathy among subjects with narrow cervical spinal canal in a population-based magnetic resonance imaging study: the Wakayama Spine Study.

Science.gov (United States)

Nagata, Keiji; Yoshimura, Noriko; Hashizume, Hiroshi; Muraki, Shigeyuki; Ishimoto, Yuyu; Yamada, Hiroshi; Takiguchi, Noboru; Nakagawa, Yukihiro; Minamide, Akihito; Oka, Hiroyuki; Kawaguchi, Hiroshi; Nakamura, Kozo; Akune, Toru; Yoshida, Munehito

2014-12-01

A narrow cervical spinal canal (CSC) is a well-known risk factor for cervical myelopathy (CM). However, no epidemiologic data of the CSC based on a population-based cohort are available. The purpose of the study was to investigate the age-related differences in CSC diameters on plain radiographs and to examine the associated magnetic resonance imaging (MRI) abnormalities including cervical cord compression and increased signal intensity (ISI) as well as the clinical CM with the narrow CSC. This was a cross-sectional study. Data were obtained from the baseline survey of the Wakayama Spine Study that was performed from 2008 to 2010 in a western part of Japan. Finally, a total of 959 subjects (319 men and 640 women; mean age, 66.4 years) were included. The outcome measures included in the study were the CSC diameter at C5 level on plain radiographs, cervical cord compression and ISI on sagittal T2-weighted MRI, and physical signs related to CM (eg, the Hoffmann reflex, hyperreflexia of the patellar tendon, the Babinski reflex, sensory and motor function, and bowel/bladder symptoms). The age-related differences of CSC diameters in men and women were investigated by descriptive statistics. The prevalence of MRI abnormalities and clinical CM was compared among the groups divided by the CSC diameter (less than 13, 13-15, and 15 mm or more). In addition, a logistic regression analysis was performed to determine the association of the CSC diameter with cervical cord compression/clinical CM after overall adjustment for age, sex, and body mass index. The CSC diameter was narrower with increasing age in both men and women. The prevalence of cervical cord compression, ISI, and the clinical CM was significantly higher in the narrower CSC group. The prevalence of cervical cord compression, ISI, and CM among subjects with CSC diameter less than 13 mm was 38.0%, 5.4%, and 10.1%, respectively. In the logistic model, the CSC diameter was a significant predictive factor for the

[Experience in using xeomin in the treatment of arm and hand spasticity in the early rehabilitation phase of stroke].

Science.gov (United States)

Kostenko, E V; Petrova, L V; Ganzhula, P A; Lisenker, L N; Otcheskaia, O V; Khozova, A A; Boĭko, A N

2012-01-01

To reduce arm and hand spasticity, 28 patients in the early rehabilitation phase of ischemic hemisphere stroke received injections of the botulinum toxin A preparation xeomin in the content of complex rehabilitation programs. The following muscles: m. biceps brachii, m. flexor digitorum profundus, m. flexor digitorum superficialis, m. flexor carpi ulnaris, m. flexor carpi radialis were injected according to standard scheme. The total dose of drug was 200U in moderate (2-3 scores on the Ashworth scale) and 300U in marked (3-4 scores on the Ashworth scale) spasticity. Efficacy and safety of treatment was assessed at baseline and 2, 4, 8, 12, 16 weeks after injections. Xeomin significantly (parm (due to patient's and caregiver's reports) remained for to 12 weeks. The treatment was most effective in the group of patients with moderate spasticity. The correlation analysis confirmed that the severity of spasticity increased with the disease duration that reduced rehabilitation efficiency. The treatment with xeomin was safe, no serious side-effects were found.

Physical Therapy for an Adult with Chronic Stroke after Botulinum Toxin Injection for Spasticity: A Case Report

Science.gov (United States)

Phadke, Chetan P.; Ismail, Farooq; Boulias, Chris

2015-01-01

ABSTRACT Purpose: In this case report, we describe the type and duration of a physical therapy and botulinum toxin type A (BoNTA) intervention directed at lower limb spasticity and the gait and balance improvement in a patient post-stroke. Treatment of focal spasticity with BoNTA intramuscular injections combined with physical therapy is recommended by rehabilitation experts. However, the optimal type and duration of physical therapy intervention to optimize any functional gains that follow chemodenervation induced by BoNTA has not been established. Method: One individual with chronic stroke who received BoNTA injections for upper and lower extremity spasticity was included. Physical therapy intervention consisted of 45- to 60-min sessions twice weekly for 12 weeks, based on the Bobath–neurodevelopmental therapy approach, and an activity-based home program. Results: After BoNTA injections and physical therapy, the patient made clinically significant improvements in balance and gait speed and became more independent with his ambulation. Conclusions: This case report demonstrates that physical therapy after BoNTA injections can result in significant functional improvements for individuals with spasticity after chronic stroke that may not be possible with BoNTA injections alone. PMID:25931655

Muscle torque of healthy individuals and individuals with spastic hemiparesis after passive static streching.

Science.gov (United States)

Tatsukawa DE Freitas, Sérgio Takeshi; DE Carvalho Abreu, Elizângela Márcia; Dos Reis, Mariane Cecilia; DE Souza Cunha, Bruna; Souza Moreira Prianti, Tamires; Pupio Silva Lima, Fernanda; Oliveira Lima, Mário

2016-01-01

Spasticity is one of the main causes of contracture, muscle weakness and subsequent functional incapacity. The passive static stretching can be included as having the purpose of increasing musculoskeletal flexibility, however, it also can influence the muscle torque. The objective is to verify the immediate effect of passive static stretching in the muscle strength of healthy and those who present spastic hemiparesis. There were assessed 20 subjects, 10 spastic hemiparetic (EG) and 10 healthy individuals (CG), including both sexes, aged between 22 and 78 years. The torque of extensor muscles of the knee was analyzed using isokinetic dynamometer. Results have shown that EG has less muscle torque compared to CG ( p torque after stretching ( p torque of CG after performing the program that was prescribed. Immediately after the passive stretch, a significant torque decrease can be seen in hypertonic muscle; it is believed that this reduction may be associated with the physiological overlap between actin and myosin filaments and so preventing the muscle to develop a maximum contraction.

New approaches in the management of spasticity in multiple sclerosis patients: role of cannabinoids

Directory of Open Access Journals (Sweden)

Paul F Smith

2010-02-01

Full Text Available Paul F SmithDepartment of Pharmacology and Toxicology, School of Medical Sciences, University of Otago, Dunedin, New ZealandAbstract: Cannabinoids such as Cannabis-based medicinal extracts (CBMEs are increasingly being used in the treatment of spasticity associated with multiple sclerosis (MS. They have been shown to have a beneficial effect on spasticity; however, this evidence is largely based on subjective rating scales. Objective measurements using the Ashworth scale have tended to show no significant effect; however, the validity of this scale has been questioned. The available clinical trial data suggest that the adverse side effects associated with using CBMEs are generally mild, such as dry mouth, dizziness, somnolence, nausea and intoxication. However, most of these trials were run over a period of months and it is possible that other adverse side effects could develop with long-term use. There may be reason to be concerned about the use of therapeutic cannabinoids by adolescents, people predisposed to psychosis and pregnant women.Keywords: multiple sclerosis, spasticity, cannabinoids, Cannabis

Unstable gait due to spasticity of the rectus femoris: gait analysis and motor nerve block.

Science.gov (United States)

Gross, R; Leboeuf, F; Rémy-Néris, O; Perrouin-Verbe, B

2012-12-01

We present the case of a 54 year-old man presenting with a right Brown-Séquard plus syndrome (BSPS) after a traumatic cervical spinal cord injury. After being operated on with selective tibial neurotomy and triceps surae lengthening because of a right spastic equinus foot, he developed a gait disorder at high speed. The patient complained about an instability of the right knee. Observational gait analysis exhibited an oscillating, flexion/extension motion of the right knee during stance, which was confirmed by gait analysis. Dynamic electromyographic recordings exhibited a clonus of the right rectus femoris (RF) during stance. The spastic activity of the RF and the abnormal knee motion totally reversed after a motor nerve block of the RF, as well as after botulinum toxin type A injection into the RF. We emphasize that complex, spastic gait disorders can benefit from a comprehensive assessment including gait analysis and nerve blocks. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

The role of kinesiotaping combined with botulinum toxin to reduce plantar flexors spasticity after stroke.

Science.gov (United States)

Karadag-Saygi, Evrim; Cubukcu-Aydoseli, Koza; Kablan, Nilufer; Ofluoglu, Demet

2010-01-01

To evaluate the effect of kinesiotaping as an adjuvant therapy to botulinum toxin A (BTX-A) injection in lower extremity spasticity. This is a single-center, randomized, and double-blind study. Twenty hemiplegic patients with spastic equinus foot were enrolled into the study and randomized into 2 groups. The first group (n=10) received BTX-A injection and kinesiotaping, and the second group (n=10) received BTX-A injection and sham-taping. Clinical assessment was done before injection and at 2 weeks and 1, 3, and 6 months. Outcome measures were modified Ashworth scale (MAS), passive ankle dorsiflexion, gait velocity, and step length. Improvement was recorded in both kinesiotaping and sham groups for all outcome variables. No significant difference was found between groups other than passive range of motion (ROM), which was found to have increased more in the kinesiotaping group at 2 weeks. There is no clear benefit in adjuvant kinesiotaping application with botulinum toxin for correction of spastic equinus in stroke.

Ankle Plantarflexor Spasticity Does Not Restrict the Recovery of Ankle Plantarflexor Strength or Ankle Power Generation for Push-Off During Walking Following Traumatic Brain Injury.

Science.gov (United States)

Williams, Gavin; Banky, Megan; Olver, John

2016-01-01

The main aim of this project was to determine the impact of plantarflexor spasticity on muscle performance for ambulant people with traumatic brain injury (TBI). A large metropolitan rehabilitation hospital. Seventy-two ambulant people with TBI who were attending physiotherapy for mobility limitations. Twenty-four participants returned for a 6-month follow-up reassessment. Cross-sectional cohort study. Self-selected walking speed, Tardieu scale, ankle plantarflexor strength, and ankle power generation (APG). Participants with ankle plantarflexor spasticity had significantly lower self-selected walking speed; however, there was no significant difference in ankle plantarflexor strength or APG. Participants with ankle plantarflexor spasticity were not restricted in the recovery of self-selected walking speed, ankle plantarflexor strength, or APG, indicating equivalent ability to improve their mobility over time despite the presence of spasticity. Following TBI, people with ankle plantarflexor spasticity have significantly greater mobility limitations than those without spasticity, yet retain the capacity for recovery of self-selected walking speed, ankle plantarflexor strength, and APG.

Does electrical stimulation reduce spasticity after stroke? A randomized controlled study.

Science.gov (United States)

Bakhtiary, Amir H; Fatemy, Elham

2008-05-01

To investigate the therapeutic effect of electrical stimulation on plantarflexor spasticity in stroke patients. A randomized controlled clinical trial study. Rehabilitation clinic of Semnan University of Medical Sciences. Forty stroke patients (aged from 42 to 65 years) with ankle plantarflexor spasticity. Fifteen minutes of inhibitory Bobath techniques were applied to one experimental group and a combination of 9 minutes of electrical stimulation on the dorsiflexor muscles and inhibitory Bobath techniques was applied to another group for 20 sessions daily. Passive ankle joint dorsiflexion range of motion, dorsiflexion strength test, plantarflexor muscle tone by Modified Ashworth Scale and soleus muscle H-reflex. The mean change of passive ankle joint dorsiflexion in the combination therapy group was 11.4 (SD 4.79) degrees versus 6.1 (SD 3.09) degrees, which was significantly higher (P = 0.001). The mean change of plantarflexor muscle tonicity measured by the Modified Ashworth Scale in the combination therapy group was -1.6 (SD 0.5) versus -1.1 (SD 0.31) in the Bobath group (P = 0.001). Dorsiflexor muscle strength was also increased significantly (P = 0.04) in the combination therapy group (0.7 +/- 0.37) compared with the Bobath group (0.4 +/- 0.23). However, no significant change in the amplitude of H-reflex was found between combination therapy (-0.41 +/- 0.29) and Bobath (-0.3 +/- 0.28) groups. Therapy combining Bobath inhibitory technique and electrical stimulation may help to reduce spasticity effectively in stroke patients.

Swedish Massage and Abnormal Reflexes of Children with Spastic Cerebral Palsy

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Vida Alizad

2007-09-01

Full Text Available Objectives: Massage therapy is one of the most widely used complementary and alternative medicine therapies for children. This study was conducted to determine the effect of wedish massage on abnormal reflexes in children with spastic cerebral palsy (CP. Methods: This study was a single blind clinical trial conducted on forty children with spastic CP who were recruited from clinics of the University of Social Welfare & Rehabilitation Sciences. They were randomly assigned to intervention and control groups. The routine occupational therapy (OT techniques were performed during a 3 month-period in both groups. The intervention group also received Swedish massage for 30 minutes before every OT session. Primary, spinal, brain stem, midbrain, cortical and automatic reflexes were evaluated at the beginning of the study and 3 months later. The data analysis was done by parametric and nonparametric tests. Results: Finally, thirteen subjects in the intervention group and 14 subjects in the control group were remained and studied. The average ages in the intervention and control groups were 49.5 and 42.1 months respectively. There were no statistically significant differences in abnormal reflexes in the intervention group in comparison to the control (P>0.05. Discussion: Adding Swedish massage to traditional OT techniques had no significant effects on abnormal reflexes in children with spastic cerebral palsy. Evidently more research is required in order to completely reject the effects of Swedish massage on abnormal reflexes of children with CP.

Cost analysis of glatiramer acetate versus interferon-β for relapsing-remitting multiple sclerosis in patients with spasticity: the Escala study.

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Sánchez-de la Rosa, Rainel; García-Bujalance, Laura; Meca-Lallana, José

2015-12-01

The Escala Study evidenced that the administration of glatiramer acetate for relapsing-remitting multiple sclerosis improved the spasticity of patients previously treated with interferon-β. However, whether such an improvement was translated into cost savings remained unclear. We therefore conducted a cost analysis of glatiramer acetate versus interferon-β in these patients with multiple sclerosis and spasticity. This cost analysis encompassed data from the observational Escala Study, which included patients with relapsing-remitting multiple sclerosis and spasticity whose treatment had been switched from interferon-β to glatiramer acetate. Costs prior to starting glatiramer acetate (interferon-β period) were compared to the subsequent six months on glatiramer acetate (glatiramer acetate period). The analysis was carried out following the recommendations for conducting pharmacoeconomic studies and from the Spanish National Health System perspective. Costs associated with multiple sclerosis treatment, spasticity treatment and relapse management were expressed in 2014 euros (€); a 7.5 % discount was applied-when needed-as stipulated in Spanish law. The management of relapsing-remitting multiple sclerosis, spasticity and relapses accounted for a 6-month cost per patient of 7,078.02€ when using interferon-β and 4,671.31€ when using glatiramer acetate. Switching from interferon-β to glatiramer acetate therefore represented a cost saving of 2,406.72€ per patient in favour of glatiramer acetate, which resulted from savings in treatment costs, relapse management and spasticity treatment of 1,890.02€, 430.48€ and 86.21€, respectively. The ratio of the costs during interferon-β was 1.5 times the costs during glatiramer acetate; thus, a fixed budget of 5,000,000€ would enable 1,070 patients to be treated with glatiramer acetate and only 706 patients with interferon-β. The treatment of relapsing-remitting multiple sclerosis with glatiramer acetate

Ossificação do ligamento longitudinal posterior na coluna cervical: relato de caso Ossification of the posterior longitudinal ligament in the cervical spine: case report

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ROSANA HERMINIA SCOLA

1998-09-01

Full Text Available A ossificação do ligamento longitudinal posterior (OLLP é causa incomum de mielopatia compressiva na população caucasiana. É relatado o caso de um paciente do sexo masculino com um quadro de paraparesia espástica, cuja investigação radiológica mostrou OLLP. O raio-X de coluna cervical mostrou imagem laminar, vertical, com densidade óssea, posterior aos corpos vertebrais, que se estendia de C2 a T1. A tomografia computadorizada (TC e a mielotomografia mostravam OLLP causando compressão medular ântero-posterior no segmento descrito. Na ressonância magnética, observou-se área de hiperintensidade em T2 no segmento C7-T1, compatível com mielomalácia. O paciente foi submetido a laminoplastia tipo "open-door", com melhora do quadro parético dos membros inferiores. A OLLP deve entrar no diagnóstico diferencial das mielopatias cervicais, sendo facilmente diagnosticada através de radiografias e TC da coluna cervical. São revisados os aspectos clínicos e radiológicos e o tratamento da OLLP.Ossification of the posterior longitudinal ligament (OPLL is an uncommon cause of compressive myelopathy in the Caucasian population. A case of spastic paraparesis in a Caucasian man whose radiological investigation showed OPLL is presented. The radiographs of the cervical spine showed a strip of bony density posterior to the vertebral bodies, extending from C2 to T1. Computerized tomography (CT and CT myelography showed OPLL at the same level. Magnetic resonance showed an area of increased signal on T2-weighted sequences at C7-T1 level suggestive of myelomalacia. The patient underwent an open-door laminoplasty (C2 to C7 with improvement of the paraparesis. OPLL should be included in the differential diagnosis of cervical myelopathy. It can be easily detected by plain radiographs and CT of the cervical spine. A review of the clinical and radiological features and the treatment of OPLL is presented.

Cervical spine abnormalities and instability with myelopathy in warfarin-related chondrodysplasia: 17-year follow-up

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Takano, Hideyuki; Smith, W.L.; Sato, Yutaka; Kao, S.C.S. [Department of Radiology, The University of Iowa Clinics and Hospitals, 200 Hawkins Dr., F3966 JPP, Iowa City, IA 52242-1077 (United States)

1998-07-01

A patient with warfarin embryopathy developed progressive cervical spinal myelopathy owing to bony cervical spinal damage. While there are several descriptions of warfarin embryopathy, the long-term complication of cervical spinal instability has not been reported. This cervical instability may, as in our patient, cause severe neurological dysfunction or even sudden death; therefore, it is important that pediatric radiologists should be alert to this condition. (orig.) With 5 figs., 9 refs.

Negative MRI findings in a case of degenerative myelopathy in a dog : clinical communication

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M. Okada

2009-05-01

Full Text Available An 11-year-old male Rough collie was submitted with paraparesis, but did not respond to medical treatment. Clinical signs worsened and the dog displayed paralysis, inability to stand and loss of voluntary bladder control, whereupon magnetic resonance imaging (MRI was performed. No significant abnormalities were identified from MRI, blood tests, cerebrospinal fluid tests or radiography. After MRI, the dog developed dyspnoea and died. Autopsy and subsequent histopathological examination led to a diagnosis of degenerative myelopathy.

A COMPARATIVE STUDY ON EFFECTIVENESS OF OPEN VERSUS CLOSED KINETIC CHAIN EXERCISES TO IMPROVE GAIT IN SPASTIC DIPLEGIC CEREBRAL PALSY

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Trishna Saikia Baruah

2016-04-01

Full Text Available Background: Cerebral Palsy (CP describes a non- progressive but not unchanging disorder of movement and posture due to an insult to or anomaly of the developing brain. People with spastic diplegia typically walk slowly and have difficulties in performing activities such as walking running or jumping. Children with spastic diplegic cerebral palsy are relied more on cadence to increase speed. Hence, the purpose of this study is to compare the effectiveness of open and closed kinetic chain (OKC and CKC exercises in improving gait in spastic diplegic cerebral palsy. Methods: 30 children with spastic diplegic cerebral palsy of both genders with age 4-12 years was taken. Cadence and distance covered in 1Minute Walk Test was calculated before and after the test. The intervention for group A was CKC exercises and group B was OKC exercises for 3 days a week for 6 weeks and each session lasted for 30-45 minutes was given for both the groups. Results: Paired t-test was performed to find effectiveness of CKC and OKC improving gait in spastic diplegic CP to see the difference of means of 1minute walk, t = 10.789 which is significant (p = 0.000 and for cadence, t = 3.37 which is highly significant (p = 0.00 implying that cadence and distance covered in1minute walk was more with CKC exercises. Conclusion: Based on the result it is concluded that CKC exercises are effective in improving gait than OKC exercises in spastic diplegic cerebral palsy.

Evaluating the functional outcomes of ultrasound-guided botulinum toxin type A injections using the Euro-musculus approach for upper limb spasticity treatment in post-stroke patients; an observational study.

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Buyukavci, Raikan; Akturk, Semra; Ersoy, Yüksel

2018-02-07

Ultrasound-guided botulinum toxin type A injection is an effective treatment for spasticity. Euro-musculus spasticity approach is a new method for administering injections to the correct point of the correct muscle. The clinical outcomes of this practical approach is not yet available in the literature. The purpose of this study was to evaluate the effects on spasticity and the functional outcomes of ultrasound guided botulinum toxin type A injections via the Euro-musculus spasticity approach to treat upper limb spasticity in post-stroke patients. An observational study. Inpatient post-stroke patients. Twenty five post-stroke patients with post-stroke upper limb spasticity were recruited. The ultrasound-guided botulinum toxin type A injections were administered into the spastic target muscles using the Euro-musculus spasticity approach, and all of the patients were enrolled in rehabilitation programmes after the injections. This research included the innervation zone and injection site figures and ultrasound images of each muscle in the upper limb. The degree of spasticity was assessed via the Modified Ashworth Scale and the upper limb motor function via the Fugl Meyer Upper Extremity Scale at the baseline and 4 and 12 weeks after the botulinum toxin type A injection. Significant decreases in the Modified Ashworth Scale scores of the upper limb flexor muscle tone measured 4 and 12 weeks after the botulinum toxin type A injection were found when compared to the baseline scores (pbotulinum toxin type A injection via the Euro- musculus spasticity approach is a practical and effective method for administering injections to the correct point of the correct muscle. Ultrasound-guided botulinum toxin type A injections combined with rehabilitation programmes decrease spasticity and improve the upper extremity motor functions in stroke patients. This new approach for ultrasound- guided botulinum toxin type A injection is very practical and effective method for upper

The reporting of study and population characteristics in degenerative cervical myelopathy: A systematic review.

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Benjamin M Davies

Full Text Available Degenerative cervical myelopathy [DCM] is a disabling and increasingly prevalent condition. Variable reporting in interventional trials of study design and sample characteristics limits the interpretation of pooled outcomes. This is pertinent in DCM where baseline characteristics are known to influence outcome. The present study aims to assess the reporting of the study design and baseline characteristics in DCM as the premise for the development of a standardised reporting set.A systematic review of MEDLINE and EMBASE databases, registered with PROSPERO (CRD42015025497 was conducted in accordance with PRISMA guidelines. Full text articles in English, with >50 patients (prospective or >200 patients (retrospective, reporting outcomes of DCM were deemed to be eligible.A total of 108 studies involving 23,876 patients, conducted world-wide, were identified. 33 (31% specified a clear primary objective. Study populations often included radiculopathy (51, 47% but excluded patients who had undergone previous surgery (42, 39%. Diagnositic criteria for myelopathy were often uncertain; MRI assessment was specified in only 67 (62% of studies. Patient comorbidities were referenced by 37 (34% studies. Symptom duration was reported by 46 (43% studies. Multivariate analysis was used to control for baseline characteristics in 33 (31% of studies.The reporting of study design and sample characteristics is variable. The development of a consensus minimum dataset for (CODE-DCM will facilitate future research synthesis in the future.

Disruption of basal lamina components in neuromotor synapses of children with spastic quadriplegic cerebral palsy.

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Karyn G Robinson

Full Text Available Cerebral palsy (CP is a static encephalopathy occurring when a lesion to the developing brain results in disordered movement and posture. Patients present with sometimes overlapping spastic, athetoid/dyskinetic, and ataxic symptoms. Spastic CP, which is characterized by stiff muscles, weakness, and poor motor control, accounts for ∼80% of cases. The detailed mechanisms leading to disordered movement in spastic CP are not completely understood, but clinical experience and recent studies suggest involvement of peripheral motor synapses. For example, it is recognized that CP patients have altered sensitivities to drugs that target neuromuscular junctions (NMJs, and protein localization studies suggest that NMJ microanatomy is disrupted in CP. Since CP originates during maturation, we hypothesized that NMJ disruption in spastic CP is associated with retention of an immature neuromotor phenotype later in life. Scoliosis patients with spastic CP or idiopathic disease were enrolled in a prospective, partially-blinded study to evaluate NMJ organization and neuromotor maturation. The localization of synaptic acetylcholine esterase (AChE relative to postsynaptic acetylcholine receptor (AChR, synaptic laminin β2, and presynaptic vesicle protein 2 (SV2 appeared mismatched in the CP samples; whereas, no significant disruption was found between AChR and SV2. These data suggest that pre- and postsynaptic NMJ components in CP children were appropriately distributed even though AChE and laminin β2 within the synaptic basal lamina appeared disrupted. Follow up electron microscopy indicated that NMJs from CP patients appeared generally mature and similar to controls with some differences present, including deeper postsynaptic folds and reduced presynaptic mitochondria. Analysis of maturational markers, including myosin, syntrophin, myogenin, and AChR subunit expression, and telomere lengths, all indicated similar levels of motor maturation in the two groups

Gait asymmetry, ankle spasticity, and depression as independent predictors of falls in ambulatory stroke patients.

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Ta-Sen Wei

Full Text Available Falls are the leading cause of injury in stroke patients. However, the cause of a fall is complicated, and several types of risk factors are involved. Therefore, a comprehensive model to predict falls with high sensitivity and specificity is needed.This study was a prospective study of 112 inpatients in a rehabilitation ward with follow-up interviews in patients' homes. Evaluations were performed 1 month after stroke and included the following factors: (1 status of cognition, depression, fear of fall and limb spasticity; (2 functional assessments [walking velocity and the Functional Independence Measure (FIM]; and (3 objective, computerized gait and balance analyses. The outcome variable was the number of accidental falls during the 6-month follow-up period after baseline measurements.The non-faller group exhibited significantly better walking velocity and FIM scale compared to the faller group (P < .001. The faller group exhibited higher levels of spasticity in the affected limbs, asymmetry of gait parameters in single support (P < .001, double support (P = .027, and step time (P = .003, and lower stability of center of gravity in the medial-lateral direction (P = .008. Psychological assessments revealed that the faller group exhibited more severe depression and lower confidence without falling. A multivariate logistic regression model identified three independent predictors of falls with high sensitivity (82.6% and specificity (86.5%: the asymmetry ratio of single support [adjusted odds ratio, aOR = 2.2, 95% CI (1.2-3.8], the level of spasticity in the gastrocnemius [aOR = 3.2 (1.4-7.3], and the degree of depression [aOR = 1.4 (1.2-1.8].This study revealed depression, in additional to gait asymmetry and spasticity, as another independent factor for predicting falls. These results suggest that appropriate gait training, reduction of ankle spasticity, and aggressive management of depression may be critical to prevent falls in stroke patients.

[Microsurgical drezotomy for the treatment of spasticity of the lower limbs].

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Mertens, P; Sindou, M

1998-09-01

Ablative functional neurosurgery can be useful in some selected patients for the treatment of harmful spasticity in the lower limbs. Microsurgical drezotomy was introduced in 1972, on the basis of anatomical studies of the human dorsal root entry zone (DREZ) showing a topographical segregation of the afferent fibers according to their size and thus functional destinations. It consists of a 3 mm deep microsurgical lesion directed at a 45 degree angle in the postero-lateral sulcus, penetrating the DREZ in its ventro-lateral aspect, at the level of all the rootlets considered as involved in spasticity (and pain). It destroys mainly the lateral (nociceptive) and central (myotatic) afferent fibers as well as the facilitatory medial part of the Lissauer tract, whilst sparing most of the medial (lemniscal) fibers and the inhibitor lateral part of the Lissauer tract. We report a series of 121 bedridden patients suffering from harmful spasticity in one (15) or both (106) lower limbs and treated with microsurgical drezotomy. Surgery was decided on because of abnormal postures in flexion in two-thirds of the patients and in hyperextension in one-third, additional pain in 75 of them, and hyperactive bladder in 38 cases. The post-operative results were evaluated after a mean follow-up time of 5 years and 6 months. Both spasticity and spasms were significantly decreased or suppressed respectively in 78% and 88% of the patients. When present, pain was relieved without abolition of sensation in 82%. These benefits resulted in either disappearance or marked reduction of the abnormal postures and articular limitation in 90% of the patients. When present preoperatively, urinary leakage disappeared in 85% of the cases. Mild to severe complications occurred in 32 patients and precipitated or were responsible for death in 6 cases (5%). This is explained by the fact that most of the patients, especially those affected by multiple sclerosis, were in very precarious general and

Physical Therapy in the Management of Pelvic Floor Muscles Hypertonia in a Woman with Hereditary Spastic Paraplegia

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Aline Moreira Ribeiro

2014-01-01

Full Text Available Background. Pelvic floor (PF hypertonic disorders are a group of conditions that present with muscular hypertonia or spasticity, resulting in a diminished capacity to isolate, contract, and relax the PF. Their presentation includes voiding and sexual dysfunctions, pelvic pain, and constipation. Various factors are associated, such as complicated vaginal birth, muscular injury, scar tissue formation, and neuropathies. Study Design. The case of a single patient will be presented, together with the management strategies employed. Case Description. A woman with hereditary spastic paraparesis and a history of muscle spasticity and urinary and fecal complaints since childhood. She presented to this institution seeking treatment for pelvic pain, pain during intercourse, constipation, and micturition problems. A physical therapy protocol was developed, with the trial of several treatment modalities. Outcome. After some failed attempts, perineal and pelvic floor stretching proved to be very efficacious therapies for this patient’s complaint, leading to improved pain during intercourse, constipation, pelvic pain, and urinary stream. Discussion. PF spasticity can lead to severe disability and interfere with daily basic functions, such as micturition and evacuation. Physical therapy plays an essential role in the management of these patients and can lead to significant improvement in quality of life.

Phase-II Clinical Validation of a Powered Exoskeleton for the Treatment of Elbow Spasticity

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Simona Crea

2017-05-01

Full Text Available Introduction: Spasticity is a typical motor disorder in patients affected by stroke. Typically post-stroke rehabilitation consists of repetition of mobilization exercises on impaired limbs, aimed to reduce muscle hypertonia and mitigate spastic reflexes. It is currently strongly debated if the treatment's effectiveness improves with the timeliness of its adoption; in particular, starting intensive rehabilitation as close as possible to the stroke event may counteract the growth and postpone the onset of spasticity. In this paper we present a phase-II clinical validation of a robotic exoskeleton in treating subacute post-stroke patients.Methods: Seventeen post-stroke patients participated in 10 daily rehabilitation sessions using the NEUROExos Elbow Module exoskeleton, each one lasting 45 min: the exercises consisted of isokinetic passive mobilization of the elbow, with torque threshold to detect excessive user's resistance to the movement. We investigated the safety by reporting possible adverse events, such as mechanical, electrical or software failures of the device or injuries or pain experienced by the patient. As regards the efficacy, the Modified Ashworth Scale, was identified as primary outcome measure and the NEEM metrics describing elbow joint resistance to passive extension (i.e., maximum extension torque and zero-torque angle as secondary outcomes.Results: During the entire duration of the treatments no failures or adverse events for the patients were reported. No statistically significant differences were found in the Modified Ashworth Scale scores, between pre-treatment and post-treatment and between post-treatment and follow-up sessions, indicating the absence of spasticity increase throughout (14 days and after (3–4 months follow-up the treatment. Exoskeleton metrics confirmed the absence of significant difference in between pre- and post-treatment data, whereas intra-session data highlighted significant differences in the

Phase-II Clinical Validation of a Powered Exoskeleton for the Treatment of Elbow Spasticity.

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Crea, Simona; Cempini, Marco; Mazzoleni, Stefano; Carrozza, Maria Chiara; Posteraro, Federico; Vitiello, Nicola

2017-01-01

Introduction: Spasticity is a typical motor disorder in patients affected by stroke. Typically post-stroke rehabilitation consists of repetition of mobilization exercises on impaired limbs, aimed to reduce muscle hypertonia and mitigate spastic reflexes. It is currently strongly debated if the treatment's effectiveness improves with the timeliness of its adoption; in particular, starting intensive rehabilitation as close as possible to the stroke event may counteract the growth and postpone the onset of spasticity. In this paper we present a phase-II clinical validation of a robotic exoskeleton in treating subacute post-stroke patients. Methods: Seventeen post-stroke patients participated in 10 daily rehabilitation sessions using the NEUROExos Elbow Module exoskeleton, each one lasting 45 min: the exercises consisted of isokinetic passive mobilization of the elbow, with torque threshold to detect excessive user's resistance to the movement. We investigated the safety by reporting possible adverse events, such as mechanical, electrical or software failures of the device or injuries or pain experienced by the patient. As regards the efficacy , the Modified Ashworth Scale, was identified as primary outcome measure and the NEEM metrics describing elbow joint resistance to passive extension (i.e., maximum extension torque and zero-torque angle) as secondary outcomes. Results: During the entire duration of the treatments no failures or adverse events for the patients were reported. No statistically significant differences were found in the Modified Ashworth Scale scores, between pre-treatment and post-treatment and between post-treatment and follow-up sessions, indicating the absence of spasticity increase throughout (14 days) and after (3-4 months follow-up) the treatment. Exoskeleton metrics confirmed the absence of significant difference in between pre- and post-treatment data, whereas intra-session data highlighted significant differences in the secondary outcomes

A hereditary spastic paraplegia mutation in kinesin-1A/KIF5A disrupts neurofilament transport

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Brown Anthony

2010-11-01

Full Text Available Abstract Background Hereditary spastic paraplegias are a group of neurological disorders characterized by progressive distal degeneration of the longest ascending and descending axons in the spinal cord, leading to lower limb spasticity and weakness. One of the dominantly inherited forms of this disease (spastic gait type 10, or SPG10 is caused by point mutations in kinesin-1A (also known as KIF5A, which is thought to be an anterograde motor for neurofilaments. Results We investigated the effect of an SPG10 mutation in kinesin-1A (N256S-kinesin-1A on neurofilament transport in cultured mouse cortical neurons using live-cell fluorescent imaging. N256S-kinesin-1A decreased both anterograde and retrograde neurofilament transport flux by decreasing the frequency of anterograde and retrograde movements. Anterograde velocity was not affected, whereas retrograde velocity actually increased. Conclusions These data reveal subtle complexities to the functional interdependence of the anterograde and retrograde neurofilament motors and they also raise the possibility that anterograde and retrograde neurofilament transport may be disrupted in patients with SPG10.

Коррекция трофологической недостаточности у больных с синдромом компрессионной миелопатии различной этиологии на уровне верхнешейного отдела позвоночника

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Bajanov S.P.

2012-09-01

Full Text Available Objective: To improve the treatment results of patients with traumatic injuries, and neoplastic lesions of the upper cervical spine on the basis of a differentiated algorithm of nutritional-metabolic therapy. Materials and methods: Based on clinical and instrumental methods of examination typical metabolic characteristics have been studied in 21 patients with compressive myelopathy at the level of the upper cervical spine. Results: Due to the received data differentiated algorithm of nutritional-metabolic therapy has been worked out. Conclusion: The use of nutritional-metabolic therapy in patients with compressive myelopathy at the level of the upper cervical spine may improve the treatment.

Delayed finger tapping and cognitive responses in preterm-born male teenagers with mild spastic diplegia.

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Gao, Fei; Mei, Xi; Chen, Andrew C N

2015-02-01

Information on fine motor and basic cognitive functions in spastic diplegia is sparse in the literature. The aim of this study was to investigate index finger's tapping speed and cognitive functions in categorization and old/new recognition of pictures in patients with mild spastic diplegia. Fifteen preterm-born male teenagers with mild spastic diplegia and 15 healthy male teenagers participated in this study. Finger-tapping tests and cognitive tests were performed on all participants. Outcomes were compared between the two groups. In the finger-tapping tests, the tapping speed was significantly slower in patients than in controls. In the tests of tapping one key persistently and tapping two keys alternately, the reaction time gaps between the left and right digits were larger in patients than in controls. In the categorization tests, the accuracies and reaction times for animal/plant and girl face pictures, but not for boy face pictures, were significantly worse in patients than in controls. In the recognition tests, the accuracies for old/new, animal/plant, and boy/girl face pictures were significantly lower in patients than in controls. The reaction times for old/new, animal/plant, and new face pictures, but not for old face pictures, were significantly longer in patients compared with controls. Our results demonstrate delayed finger tapping and cognitive responses in preterm-born male teenagers with mild spastic diplegia. Our experimental paradigm is sensitive for the study of fine motor and cognitive functions between patients and healthy controls. Copyright © 2015 Elsevier Inc. All rights reserved.

Procedure Oriented Torsional Anatomy of the Forearm for Spasticity Injection.

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Chiou-Tan, Faye; Cianca, John; John, Joslyn; Furr-Stimming, Erin; Pandit, Sindhu; Taber, Katherine H

2015-01-01

: This is the second in a series of articles related to the concept of "torsional" anatomy. The objective of this article is to provide musculoskeletal ultrasound (MSKUS) anatomy of the forearm in the position of hemispastic flexion as a reference relevant to needle procedures. The MSKUS images were obtained in a healthy human subject. Marker dots were placed over common injection sites in the forearm for spasticity. The MSKUS probe was centered over each dot to obtain a cross-sectional view. A pair of MSKUS images was recorded for each site: the first in anatomic neutral and second in hemiparetic spastic position. The images were compared side to side. In addition, a video recording was made at each site to track the movement of the muscles and nerves during internal rotation. The pronator teres (PT) rotated medially and the brachialis and biceps tendon rotated in view. In addition, the median nerve became more superficial. The flexor carpi radialis rotated medially and was replaced by PT and the median nerve. The flexor carpi ulnaris and flexor digitorum profundus rotated medially and were replaced by the flexor carpi radialis, PT and median nerve. The flexor digitorum superficialis was replaced by the brachioradialis, extensor carpi radialis brevis, and radial nerve. The brachioradialis was replaced by the extensor carpi radialis brevis and extensor digitorum communis. Intended muscle targets rotate out of view and injection range. These are replaced by other muscles and nerves that could inadvertently be injected. This potentially could result in both increased complications and decreased efficacy of the procedure. It is hoped that this series of images will increase the accuracy and safety of needle placement for spasticity injections in the forearm.

The effect of Hominis Placenta Pharmacopuncture on Leg spasticity of stroke patients (A Pilot study, Double blind, Randomized, Controlled Clinical Trial

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Ju-hwan Noh

2009-12-01

Full Text Available Objective : The purpose of this study is to determine the effect of Hominis Placenta Pharmacopuncture(HPP on lower limb spasticity control in stroke patients. Methods : Twenty stroke patients with Leg spasticity were randomly divided into two groups, a Distilled water Pharmacopuncture(group I and a HPP(group II. The number of Pharmacopuncture was 5 times a week and acupuncture treatment was 3 times a week for 3 weeks. Modified Ashworth Scale(MAS, H-reflex/M-response ratio(H/M ratio, Berg Balance Scale(BBS and Time Up & Go(TUG were used for evaluation of spasticity control before experiment, after 1 week, 2 weeks, 3 weeks. Results : Group I showed significant improvement(p<.05 in BBS but no significant improvement in MAS, H/M ratio, and TUG. Group II showed significant improvement(p<.05 in MAS, BBS, and TUG, but no significant improvement in H/M ratio. The results showed significant difference in TUG, but no significant difference in MAS, H/M ratio and BBS between 2 groups. Conclusion : These results showed that HPP might decrease lower limb spasticity and increase leg motor function in stroke patients. Further studies will be required to examine more cases in the long period for the effect on lower limb in spasticity by HPP.

Electrophysiological and MRI study on poor outcome after surgery for cervical myelopathy

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Kameyama, Osamu; Kawakita, Hirofumi; Ogawa, Ryokei [Kansai Medical Univ., Moriguchi, Osaka (Japan)

1995-11-01

Occasionally, the outcome from laminoplasty for cervical spondylosis is disappointing despite an adequate operation. Before surgery, it is difficult to diagnose the pathological extent of the involvement of the spinal cord. The purpose of this study is to determine the efficacy of magnetic resonance imaging (MRI) and of the motor evoked potentials (MEPs) for the indication of the surgery and prognosis. Retrospectively, we investigated the MEPs and the MRI of 31 patients in surgery for cervical myelopathy, involving 21 with cervical spondylosis and 10 with ossification of the posterior longitudinal ligamentum, and compared the findings from those with a poor outcome (n=3l) with the findings from those with a good outcome (n=32). The MEPs from the thenar muscle and the tibialis anterior were evoked by transcranial magnetic brain stimulation. In the poor-outcome patients, the spinal canal was narrow and lumbar spinal canal stenosis was seen in 5 cases which required lumbar laminectomy. Before operation, the MEPs from the thenar muscle could not be evoked in 5 cases while there was a remarkably prolonged central motor conduction time in the other 26 cases. MRI revealed the deformed spinal cord in the involved area, and the signal intensity of the involved spinal cord in the T2 weighted image was remarkably high. The signal intensity ratio was significantly higher in the poor-outcome patients than in the good-outcome patients. This study suggested that a high signal intensity in the T2 weighted image and a prolonged conduction time or absence of MEPs largely corresponded to the clinical and other investigative features of myelopathy responsible for a poor outcome. (author).

Electrophysiological and MRI study on poor outcome after surgery for cervical myelopathy

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Kameyama, Osamu; Kawakita, Hirofumi; Ogawa, Ryokei

1995-01-01

Occasionally, the outcome from laminoplasty for cervical spondylosis is disappointing despite an adequate operation. Before surgery, it is difficult to diagnose the pathological extent of the involvement of the spinal cord. The purpose of this study is to determine the efficacy of magnetic resonance imaging (MRI) and of the motor evoked potentials (MEPs) for the indication of the surgery and prognosis. Retrospectively, we investigated the MEPs and the MRI of 31 patients in surgery for cervical myelopathy, involving 21 with cervical spondylosis and 10 with ossification of the posterior longitudinal ligamentum, and compared the findings from those with a poor outcome (n=3l) with the findings from those with a good outcome (n=32). The MEPs from the thenar muscle and the tibialis anterior were evoked by transcranial magnetic brain stimulation. In the poor-outcome patients, the spinal canal was narrow and lumbar spinal canal stenosis was seen in 5 cases which required lumbar laminectomy. Before operation, the MEPs from the thenar muscle could not be evoked in 5 cases while there was a remarkably prolonged central motor conduction time in the other 26 cases. MRI revealed the deformed spinal cord in the involved area, and the signal intensity of the involved spinal cord in the T2 weighted image was remarkably high. The signal intensity ratio was significantly higher in the poor-outcome patients than in the good-outcome patients. This study suggested that a high signal intensity in the T2 weighted image and a prolonged conduction time or absence of MEPs largely corresponded to the clinical and other investigative features of myelopathy responsible for a poor outcome. (author)

Subscales correlations between MSSS-88 and PRISM scales in evaluation of spasticity for patients with multiple sclerosis

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Knežević Tatjana

2017-01-01

Full Text Available Introduction/Objective. Patient-reported outcomes have been recognized as an important way of assessing health and well-being of patients with multiple sclerosis (MS. The aim of the study is to determine the correlation between different subscales of Patient-Reported Impact of Spasticity Measure (PRISM and Multiple Sclerosis Spasticity Scale (MSSS-88 scales in the estimation of spasticity influence on different domains Methods. The study is a cross-sectional observational study. MSSS-88 and PRISM scales were analyzed in five domains (body-function domain, activity domain, participation domain, personal factors/wellbeing domain, and hypothesis. For statistical interpretation of the correlation we performed the Spearman’s ρ-test, concurrent validity, divergent validity, and the linear regression model. Results. We found a significant correlation between subscales of evaluated MSSS-88 and PRISM scales for body domains; the highest correlation was between the need for assistance/positioning (NA/P and walking (W. Spasticity has the weakest correlation with the need for intervention (NI. The presence of pain has a negative impact and significant positive correlation between pain discomfort and NI. In the domain of body function for males, there was a non-significant correlation between muscle spasms and NI. The same applies for social functioning and social embarrassment domains, as well as for emotional health and psychological agitation for personal factors / wellbeing domain. The differences between genders of MS patients persist in different domains; muscle spasms are strong predictors for NI, and body movement is a strong predictor versus W for NA/P. Conclusion. MSSS-88 and PRISM scales can be considered reliable in measuring different domains of disability for MS patients with spasticity. Because it is shorter, quicker, and simple to use, it is concluded that the PRISM scale can successfully compete with and replace the MSSS-88 scale in

Short- and long-term effects of selective dorsal rhizotomy on gross motor function in ambulatory children with spastic diplegia Clinical article

NARCIS (Netherlands)

van Schie, P.E.M.; Schothorst, M.; Dallmeijer, A.J.; Vermeulen, R.J.; van Ouwerkerk, W.J.R.; Strijers, R.L.M.; Becher, J.G.

2011-01-01

Object. The primary aim of this prospective cohort study was to evaluate the short-term (1 year) and long-term (mean 6 years) effects of selective dorsal rhizotomy (SDR) on gross motor function and spasticity in ambulatory children with spastic diplegia. Secondary aims were to investigate side

Effects of interactive games on motor performance in children with spastic cerebral palsy.

Science.gov (United States)

AlSaif, Amer A; Alsenany, Samira

2015-06-01

[Purpose] Motor control and muscle strength impairments are the prime reasons for motor behavior disorders in children with spastic cerebral palsy. These impairments lead to histological changes in muscle growth and the learning of motor skills. Therefore, such children experience reduced muscle force generation and decreased muscle flexibility. We investigated the effect of training with Nintendo Wii Fit games on motor performance in children with spastic cerebral palsy. [Subjects and Methods] Forty children with cerebral palsy spastic diplegia aged 6-10 years diagnosed with level-3 functional capabilities according to the Gross Motor Classification System (GMFCS) were enrolled. Participants were divided randomly into equal groups: group (A) that practiced with the Nintendo Wii Fit game for at least 20 minutes/day for 12 weeks and group (B) that underwent no training (control group). The Movement Assessment Battery for Children-2 (mABC-2) was used to assess motor performance, because it mainly involves motor tasks very similar to those involved in playing Nintendo Wii Fit games, e.g., goal-directed arm movements, balancing, and jumping. [Results] There were significant improvements in the subscales of the motor performance test of those who practiced with the Nintendo Wii, while the control group showed no significant changes. [Conclusion] Using motion interactive games in home rehabilitation is feasible for children with cerebral palsy.

Cine MRI of patients with cervical myelopathy

International Nuclear Information System (INIS)

Ukita, Yasutaka

1993-01-01

Forty-six patients with cervical myelopathy were examined before and after surgery by cine magnetic resonance imaging (MRI). According to the occurrence site and degree of flow void, cerebrospinal fluid (CSF) flow void was classified into five: anterior type (flow void mainly in the anterior part of subarachnoid space), posterior type (mainly in the posteiror part), anteroposterior type (in the anterior and posterior parts), incomplete block type (flow void limited to the upper and lower parts of the block), and complete block type (no flow void). None of the 46 patients had normal CSF flow void on cine MRI before surgery. CSF flow void was seen in systolic phase on ECG (from 150 to 300 msec from R's wave) in all patients after spinal cord decompression. Postoperative CBF flow void types correlated well with surgical method, disease, and postoperative vertebral alignment. Postoperative outcome was the most excellent in the group of posterior type and the poorest in the group of anteroposterior type, showing a significant difference between the groups. Cine MRI is a useful noninvasive, dynamic method for assessing postoperative decompression effect. (N.K.)

Catheter Migration After Implantationan Intrathecal Baclofen Infusion Pump for Severe Spasticity: A Case Report

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Tung-Chou Li

2008-09-01

Full Text Available We report a case of intrathecal baclofen infusion pump implantation complicated by migration of the catheter tip. A 55-year-old man required an intrathecal baclofen infusion for severe spasticity 4 years after a cervical spinal cord injury with incomplete tetraparesis. Twelve months after initial implantation of the device, the patient began to experience a recurrence of trunk tightness and spasticity. Subsequent X-ray and computed tomography evaluations of the catheter system revealed pooling of contrast medium outside of the intrathecal distribution in the lumbar subcutaneous region of the back and therefore migration of the pump catheter tip. At surgical revision, emphasis was placed on minimizing the length of catheter outside of the spine and securing the catheter in the supraspinous fascia with a right-angled anchor. The distance between the anchors and the entry point of the catheter into the supraspinous fascia was also reduced to prevent slipping when the patient bends forward. After surgery, the patient's spasticity improved and, 1 year later, he has experienced no further complications during follow-up, requiring an average baclofen dose of 150 mg/day. Here, we describe several surgical methods intended to secure the intrathecal catheter and prevent catheter migration. Other complications related to catheter failure are also highlighted.

A new approach to assess the spasticity in hamstrings muscles using mechanomyography antagonist muscular group.

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Krueger, Eddy; Scheeren, Eduardo M; Nogueira-Neto, Guilherme N; Button, Vera Lúcia da S N; Nohama, Percy

2012-01-01

Several pathologies can cause muscle spasticity. Modified Ashworth scale (MAS) can rank spasticity, however its results depend on the physician subjective evaluation. This study aims to show a new approach to spasticity assessment by means of MMG analysis of hamstrings antagonist muscle group (quadriceps muscle). Four subjects participated in the study, divided into two groups regarding MAS (MAS0 and MAS1). MMG sensors were positioned over the muscle belly of rectus femoris (RF), vastus lateralis (VL) and vastus medialis (VM) muscles. The range of movement was acquired with an electrogoniometer placed laterally to the knee. The system was based on a LabVIEW acquisition program and the MMG sensors were built with triaxial accelerometers. The subjects were submitted to stretching reflexes and the integral of the MMG (MMG(INT)) signal was calculated to analysis. The results showed that the MMG(INT) was greater to MAS1 than to MAS0 [muscle RF (p = 0.004), VL (p = 0.001) and VM (p = 0.007)]. The results showed that MMG was viable to detect a muscular tonus increase in antagonist muscular group (quadriceps femoris) of spinal cord injured volunteers.

The usage of playing studies for development of spatial reasoning of 5-8-year-old children with spastic diplegi.

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Shlapachenko O.A.

2012-09-01

Full Text Available In the article is shown the influence of rehabilitation-correction program with the usage of outdoor games on the level of influence of spatial thinking and constructive activity of 5-8-year-old children with spastic diplegi. 64 children took part in the experiment, all had main diagnosis - children cerebral paralysis, spastic diplegi. The experiment was conducted in a few phases during the year. It is proved that conducting of outdoor games promote positive influence on forming the level of influence of spatial thinking of children, increasing capacity of work. It is revealed that the level of development of spatial thinking of children with spastic diplegi depends on clinical display of an illness. It is mentioned that involving parents for participation in games with children promote rehabilitation of children in home conditions.

Canine degenerative myelopathy: biochemical characterization of superoxide dismutase 1 in the first naturally occurring non-human amyotrophic lateral sclerosis model.

Science.gov (United States)

Crisp, Matthew J; Beckett, Jeffrey; Coates, Joan R; Miller, Timothy M

2013-10-01

Mutations in canine superoxide dismutase 1 (SOD1) have recently been shown to cause canine degenerative myelopathy, a disabling neurodegenerative disorder affecting specific breeds of dogs characterized by progressive motor neuron loss and paralysis until death, or more common, euthanasia. This discovery makes canine degenerative myelopathy the first and only naturally occurring non-human model of amyotrophic lateral sclerosis (ALS), closely paralleling the clinical, pathological, and genetic presentation of its human counterpart, SOD1-mediated familial ALS. To further understand the biochemical role that canine SOD1 plays in this disease and how it may be similar to human SOD1, we characterized the only two SOD1 mutations described in affected dogs to date, E40K and T18S. We show that a detergent-insoluble species of mutant SOD1 is present in spinal cords of affected dogs that increases with disease progression. Our in vitro results indicate that both canine SOD1 mutants form enzymatically active dimers, arguing against a loss of function in affected homozygous animals. Further studies show that these mutants, like most human SOD1 mutants, have an increased propensity to form aggregates in cell culture, with 10-20% of cells possessing visible aggregates. Creation of the E40K mutation in human SOD1 recapitulates the normal enzymatic activity but not the aggregation propensity seen with the canine mutant. Our findings lend strong biochemical support to the toxic role of SOD1 in canine degenerative myelopathy and establish close parallels for the role mutant SOD1 plays in both canine and human disorders. Copyright © 2013 Elsevier Inc. All rights reserved.

Atlantoaxial subluxation

International Nuclear Information System (INIS)

Yamashita, Y.; Takahashi, M.; Sakamoto, Y.; Kojima, R.

1989-01-01

Twenty-nine patients with atlantoaxial subluxation (18 with rheumatoid arthritis, 2 due to trauma, 4 with os odontoideum, and one each with polyarteritis nodosa, rheumatic fever, Klippel-Feil syndrome, achondroplasia, and cause unknown) were evaluated using a 0.22 tesla resistive MRI unit. Cord compression was classified into four grades according to the degree on magnetic resonance imaging. There were 7 patients with no thecal sac compression (grade 0), 10 with a minimal degree of subarachnoid space compression without cord compression (grade 1), 7 with mild cord compression (grade 2), and 5 with severe cord compression or cord atrophy (grade 3). Although the severity of myelopathy showed poor correlation with the atlantodental interval on conventional radiography, high correlation was observed between MR grading and the degree of myelopathy. The high signal intensity foci were observed in 7 or 12 patients with cord compression (grades 2 and 3) on T2 weighted images. Other frequently observed findings in rheumatoid arthritis included soft tissue masses of low to intermediate signal intensity in the paraodontoid space, erosions of the odontoid processes, and atlanto-axial impaction on T1 and T2 weighted images. (orig.)

Evaluation of Spasticity Variations at the Elbow Joint of CVA Patients According to the Biomechanical Indices

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Nima Soleimanzadeh-Ardabili

2013-10-01

Full Text Available Objective: The goal of the present study was to evaluate spasticity variations by increase the velocity of motion and MAS value in the elbow flexor and extensor muscles at extension and flexion of CVA patients elbow joint according to the biomechanical indices. Materials & Methods: Fifteen adult patients with a history of stroke and upper-extremity spasticity volunteered to participate in this study and fifteen healthy subjects were recruited in order to establish the control group. The degree of spasticity was evaluated for each patient using the MAS. CPM tests were imposed in elbow extension and flexion and the biomechanical indices were calculated at each of the following velocities: 15, 45, 75 and 120 º/s. Results: It seemed a regular increment of the viscoelastic and viscose stiffness indices by increasing the velocity of motion and the rate of MAS value in both extension and flexion in all test groups and also there was significant regular increment of elastic stiffness index by increasing the velocity in both extension and flexion between the control group and group 1 and also irregular increment between group1 and group 2 and 3 and also the effect of mentioned index was decreased at higher level of MAS. Conclusion: it seemed the more effect of elastic stiffness in spasticity in CVA patients at lower level of MAS and more effect of viscose stiffness in higher level of MAS and also results showed the increment of viscose stiffness by increment of speed of motion and the rate of MAS.

Spastic quadriplegia in Down syndrome with congenital duodenal stenosis/atresia.

Science.gov (United States)

Kurosawa, Kenji; Enomoto, Keisuke; Tominaga, Makiko; Furuya, Noritaka; Sameshima, Kiyoko; Iai, Mizue; Take, Hiroshi; Shinkai, Masato; Ishikawa, Hiroshi; Yamanaka, Michiko; Matsui, Kiyoshi; Masuno, Mitsuo

2012-06-01

Down syndrome is an autosomal chromosome disorder, characterized by intellectual disability and muscle hypotonia. Muscle hypotonia is observed from neonates to adulthood in Down syndrome patients, but muscle hypertonicity is extremely unusual in this syndrome. During a study period of nine years, we found three patients with severe spastic quadriplegia among 20 cases with Down syndrome and congenital duodenal stenosis/atresia (3/20). However, we could find no patient with spastic quadriplegia among 644 cases with Down syndrome without congenital duodenal stenosis/atresia during the same period (0/644, P quadriplegia among 17 patients with congenital duodenal stenosis/atresia without Down syndrome admitted during the same period to use as a control group (0/17, P quadriplegia in patients with Down syndrome. Long-term survival is improving, and the large majority of people with Down syndrome are expected to live well into adult life. Management and further study for the various problems, representing a low prevalence but serious and specific to patients with Down syndrome, are required to improve their quality of life. © 2012 The Authors. Congenital Anomalies © 2012 Japanese Teratology Society.

Wall-eyed bilateral internuclear ophthalmoplegia (webino syndrome and myelopathy in pyoderma gangrenosum

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Marco Aurélio Lana

1990-12-01

Full Text Available A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-opthalmologic findings. Although it was initially attributed to lesions affecting the medial longitudinal fasciculus and the medial rectus subnuclei of the oculomotor complex in the midbrain the exact location of the lesion is still disputed. In the present case both myelopathy and Webino syndrome were probably due to vascular occlusive disease resulting from central nervous system vasculitis occurring in concomitance to pyoderma gangrenosum.

Classification of Topographical Pattern of Spasticity in Cerebral Palsy: A Registry Perspective

Science.gov (United States)

Reid, Susan M.; Carlin, John B.; Reddihough, Dinah S.

2011-01-01

This study used data from a population-based cerebral palsy (CP) registry and systematic review to assess the amount of heterogeneity between registries in topographical patterns when dichotomised into unilateral (USCP) and bilateral spastic CP (BSCP), and whether the terms diplegia and quadriplegia provide useful additional epidemiological…

Postural control in children with spastic diplegia : Muscle activity during perturbations in sitting

NARCIS (Netherlands)

Brogren, E; HaddersAlgra, M; Forssberg, H

To clarify the neural mechanisms controlling equilibrium during sitting, and the implications for the optimal sitting position for children with CP, automatic postural adjustments after perturbations of the support surface during sitting were investigated in seven children with spastic diplegia and

USE OF BOTULINUM TOXIN TYPE A IN THE TREATMENT OF SPASTICITY IN CHILDREN WITH CEREBRAL PALSY

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Ljiljana Lazić

2011-06-01

Full Text Available Cerebral palsy has an incidence of about 1-2 per 1000 live births, and in spite of the progress of neonatal medicine, it seems that the incidence will not subside in the near future. The most important characteristic of cerebral palsy is movement abnormality: spasticity, chorea, athetosis, ataxia, dystonia, as well as their different combinations. About 70% of children who suffer from cerebral palsy also suffer from some form of spasticity. Spasticity is a type of muscle hypertonicity characterized by rapid increase in resistance to passive stretching of muscles. The interest for botulinum toxin application in the treatment of spasticity has dramatically increased in the last 10 years. Botulinum toxin is the most powerful neurotoxin that is found in nature. It is produced by anaerobic bacteria – clostridium botulinum. It is produced in eight serotypes of which type A is the most commonly used. Botulinum toxin blocks neuromuscular transmission and causes irreversible weakness of the treated muscle. It has been used since 1993 in the treatment of cerebral palsy in children. The toxin effect is permanent and it results in irreversible denervation. Functional recovery is possible after 2-4 months, due to sprouting of nerve endings and the formation of new synaptic contacts. Treatment with botulinum toxin is safe. Adverse effects are rare, temporary and completely reversible. Application of botulinum toxin prevents or reduces contractures and deformities, and thus delays or avoids surgical treatment. Yet, physical therapy, which prolongs and improves the effects of botulinum toxin, remains an essential and most important form of therapy in the treatment of children with cerebral palsy.

Bone mineral density and insulin-like growth factor-1 in children with spastic cerebral palsy.

Science.gov (United States)

Nazif, H; Shatla, R; Elsayed, R; Tawfik, E; Osman, N; Korra, S; Ibrahim, A

2017-04-01

Children with cerebral palsy (CP) have significant decrease linear growth rate and low bone mineral density (BMD). This study is to evaluate BMD in children with CP and its relation to the levels of insulin-like growth factor-1 (IGF-1). This cross-sectional study was carried out on 58 children suffering from spastic CP with the age range 4-12 years compared to 19 controls. All assessed by dual energy x-ray absorptiometry (DXA) to measure BMD, serum level of IGF-1, and serum vitamin D. The patients were classified according to their GMFCS. Fractures were reported in seven (12.1%) of cases. Our study demonstrated that, IGF-1 level and BMD decrease in correlation with the severity of CP. IGF-1correlates positively with serum vitamin D, BMI, and BMD. CP children with severe GMFCS level or who use anticonvulsive drugs are at a high risk for low BMD and low levels of IGF-1. Both BMD and IGF-1 were significantly in low children with spastic CP; IGF-1 negatively correlates with the severity of osteopenia in children with spastic. Children with CP who are not independently ambulant or with severe GMFCS level or who use anticonvulsive drugs are at a high risk for developing low BMD.

Clinical characteristics of canine fibrocartilaginous embolic myelopathy (FCE): a systematic review of 393 cases (1973-2013).

Science.gov (United States)

Bartholomew, K A; Stover, K E; Olby, N J; Moore, S A

2016-12-24

Fibrocartilaginous embolic myelopathy (FCE) is common in dogs; however, there is conflicting information in the veterinary literature regarding clinical characteristics and data on recovery in severe cases is sparse. A systematic review of canine FCE was performed to delineate the natural history of this disease. 322 previously reported cases and 71 previously unreported cases were identified for inclusion. Source publications were identified via PubMed central search and by references from review articles. Previously unreported cases were identified via computerised medical records search at two veterinary institutions. FCE was most common in middle-aged large breed dogs (30 per cent); however, the miniature schnauzer was the most frequently reported individual breed and small breeds comprised 24 per cent of all reported cases. The most common neuroanatomical localisation was a T3-L3 myelopathy (33.1 per cent). Prognosis for recovery of ambulation was good to excellent with 85 per cent of cases regaining the ability to walk unassisted, most within 3 weeks. Persistent neurological deficits were common in patients that recovered ambulation (49.1 per cent). When nociception was absent in the affected limbs at initial presentation, rate of recovery was lower (10 per cent); however, this data is likely biased by limited follow-up in more severe cases. Future prospective studies should evaluate prognosis for more severely affected patients. British Veterinary Association.

On Denny-Brown's 'spastic dystonia' - What is it and what causes it?

DEFF Research Database (Denmark)

Lorentzen, Jakob; Pradines, Maud; Gracies, Jean-Michel

2018-01-01

In this review, we will work around two simple definitions of two different entities, which most often co-exist in patients with lesions to central motor pathways: Spasticity is “Enhanced excitability of velocity-dependent responses to phasic stretch at rest”, which will not be the subject of thi...

SPG10 is a rare cause of spastic paraplegia in European families.

NARCIS (Netherlands)

Schule, R.; Kremer, H.P.H.; Kassubek, J.; Auer-Grumbach, M.; Kostic, V.; Klopstock, T.; Klimpe, S.; Otto, S.; Boesch, S.; Warrenburg, B.P.C. van de; Schols, L.

2008-01-01

BACKGROUND: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date. OBJECTIVE: To determine the

SPG10 is a rare cause of spastic paraplegia in European families

NARCIS (Netherlands)

Schuele, R.; Kremer, B. P. H.; Kassubek, J.; Auer-Grumbach, M.; Kostic, V.; Klopstock, T.; Klimpe, S.; Otto, S.; Boesch, S.; van de Warrenburg, B. P.; Schoels, L.

Background: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date. Objective: To determine the

A comparative study of human T-cell lymphotropic virus-associated myelopathy in HIV-positive and HIV-negative patients in KwaZulu-Natal

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Hoosain F. Paruk

2017-12-01

Full Text Available Background: KwaZulu-Natal is an endemic area for HIV and human T-cell lymphotropic virus (HTLV infection. The main neurological manifestation of HTLV is HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP. The effect of HIV co-infection in patients with HAM/TSP is not well documented. Aims: To determine the prevalence of HIV seropositivity in patients with HAM/TSP and compare the clinical, laboratory and radiological features of patients mono-infected with HTLV and those dually infected with HTLV and HIV. Methods: Adult patients referred to the Neurology Department at Inkosi Albert Luthuli Central Hospital in KwaZulu-Natal, South Africa, for the period 01 January 2004 to 31 December 2015 with a positive HTLV serology were identified from the National Health Laboratory Service database. A retrospective chart review was conducted to identify all patients who had a diagnosis of HAM/TSP and to record their HIV status. Clinical, laboratory and radiological data were compared for HIV-positive and HIV-negative patients. Results: A total of 52 patients with HAM/TSP were identified. HIV results were available in 44 patients of whom 23 (52% patients were HIV co-infected. Patients who were HIV-positive had a younger age of presentation compared to HIV-negative patients (median: 31 vs 50 years, p = 0.002. HIV-positive patients had a median duration of symptoms at presentation of 12 months compared to 16 months for HIV-negative patients, but the difference did not reach statistical significance (p = 0.082. The CD4 cell counts of HIV-positive patients were well preserved with a median count of 781 cells/µL. Conclusions: HIV co-infection is commonly seen in the setting of HAM/TSP in KwaZulu-Natal. An interaction between the viruses may accelerate the development of HAM/TSP, leading to a younger age of presentation. Co-infection may have treatment implications because of CD4 counts being preserved in these patients.

The Effects of Exercise with TENS on Spasticity, Balance, and Gait in Patients with Chronic Stroke: A Randomized Controlled Trial

OpenAIRE

Park, Junhyuck; Seo, Dongkwon; Choi, Wonjae; Lee, Seungwon

2014-01-01

Background Transcutaneous electrical nerve stimulation (TENS) is a useful modality for pain control. TENS has recently been applied to decrease spasticity. The purpose of this study is to determine whether the addition of TENS to an exercise program reduces spasticity and improves balance and gait in chronic stroke patients. Material/Methods This was a single-blinded, multicenter, randomized controlled trial. Thirty-four ambulatory individuals with chronic stroke participated and were randoml...

In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11.

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Rita-Eva Varga

2015-08-01

Full Text Available Hereditary spastic paraplegia (HSP is characterized by a dying back degeneration of corticospinal axons which leads to progressive weakness and spasticity of the legs. SPG11 is the most common autosomal-recessive form of HSPs and is caused by mutations in SPG11. A recent in vitro study suggested that Spatacsin, the respective gene product, is needed for the recycling of lysosomes from autolysosomes, a process known as autophagic lysosome reformation. The relevance of this observation for hereditary spastic paraplegia, however, has remained unclear. Here, we report that disruption of Spatacsin in mice indeed causes hereditary spastic paraplegia-like phenotypes with loss of cortical neurons and Purkinje cells. Degenerating neurons accumulate autofluorescent material, which stains for the lysosomal protein Lamp1 and for p62, a marker of substrate destined to be degraded by autophagy, and hence appears to be related to autolysosomes. Supporting a more generalized defect of autophagy, levels of lipidated LC3 are increased in Spatacsin knockout mouse embryonic fibrobasts (MEFs. Though distinct parameters of lysosomal function like processing of cathepsin D and lysosomal pH are preserved, lysosome numbers are reduced in knockout MEFs and the recovery of lysosomes during sustained starvation impaired consistent with a defect of autophagic lysosome reformation. Because lysosomes are reduced in cortical neurons and Purkinje cells in vivo, we propose that the decreased number of lysosomes available for fusion with autophagosomes impairs autolysosomal clearance, results in the accumulation of undegraded material and finally causes death of particularly sensitive neurons like cortical motoneurons and Purkinje cells in knockout mice.

The use of hydrotherapy for the management of spasticity.

Science.gov (United States)

Kesiktas, N; Paker, N; Erdogan, N; Gülsen, G; Biçki, D; Yilmaz, H

2004-12-01

Spasticity is a major problem for the rehabilitation team. Physiotherapy is a vital component of therapy. Oral medication and other modalities such as heat, cold, ultrasound, electrical stimulation, and surgery (neuro-surgical or orthopedic) can also be used. The aim of this study was to compare the effects of hydrotherapy on spasticity and Functional Independence Measure (FIM) scores of patients with spinal cord injury (SCI). This is a control case matched study. Twenty SCI patients were divided into 2 groups and matched for age, gender, injury time, Ashworth scores, oral baclofen intake, American Spinal Injury Association, and FIM scores. The control group received passive range of motion exercise twice a day and oral baclofen for 10 weeks. The study group also received passive range of motion and oral baclofen, as well as 20 min of water exercises (at 71 degrees F, full immersion) 3 times per week. The authors evaluated spasm severity, FIM scores, oral baclofen intake, and Ashworth scales, between groups at the beginning and at the end of the treatment period. Both groups demonstrated a significant increase in FIM scores. However, the hydrotherapy group demonstrated a larger increase (P hydrotherapy group (P hydrotherapy produced a significant decrease in spasm severity (P hydrotherapy to the rehabilitation program can be helpful in decreasing the amount of medication required. Future studies must evaluate benefits of hydrotherapy for rehabilitation.

Cervical Klippel-Feil syndrome predisposing an elderly African man to central cord myelopathy following minor trauma

OpenAIRE

Olufemi Adeleye, A; Olusola Akinyemi, R

2010-01-01

An otherwise-healthy, active 83-year-old Nigerian man developed reversible central cord myelopathy from a mild fall on a level surface. Cervical spine magnetic resonance imaging (MRI) revealed C5, 6, and 7 block vertebrae and marked disc extrusions only at the immediately adjoining upper and lower non-fused segments of the cervical spine. There was no spinal canal stenosis otherwise. We think that the unique presentation of this case of Klippel-Feil syndrome further supports the impression th...

[Efficacy on hemiplegic spasticity treated with plum blossom needle tapping therapy at the key points and Bobath therapy: a randomized controlled trial].

Science.gov (United States)

Wang, Fei; Zhang, Lijuan; Wang, Jianhua; Shi, Yan; Zheng, Liya

2015-08-01

To evaluate the efficacy on hemiplegic spasticity after cerebral infarction treated with plum blossom needle tapping therapy at the key points and Bobath therapy. Eighty patients were collected, in compliance with the inclusive criteria of hemiplegic spasticity after cerebral infarction, and randomized into an observation group and a control group, 40 cases in each one. In the control group, Bobath manipulation therapy was adopted to relieve spasticity and the treatment of 8 weeks was required. In the observation group, on the basis of the treatment as the control group, the tapping therapy with plum blossom needle was applied to the key points, named Jianyu (LI 15), Jianliao (LI 14), Jianzhen (SI 9), Hegu (LI 4), Chengfu (BL 36), Zusanli (ST 36), Xiyangguan (GB 33), etc. The treatment was given for 15 min each time, once a day. Before treatment, after 4 and 8 weeks of treatment, the Fugl-Meyer assessment (FMA) and Barthel index (BI) were adopted to evaluate the motor function of the extremity and the activity of daily life in the patients of the two groups separately. The modified Ashworth scale was used to evaluate the effect of anti-spasticity. In 4 and 8 weeks of treatment, FMA: scores and BI scores were all significantly increased as compared with those before treatment in the two groups: (both PBobath therapy effectively relieves hemiplegic spasticity in the patients of cerebral infarction and improves the motor function of extremity and the activity of daily life.

Speech Respiratory Measures in Spastic Cerebral Palsied and Normal Children

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Hashem Shemshadi

2007-10-01

Full Text Available Objective: Research is designed to determine speech respiratory measures in spastic cerebral palsied children versus normal ones, to be used as an applicable tool in speech therapy plans.  Materials & Methods: Via a comparative cross-sectional study (case–control, and through a directive goal oriented sampling in case and convenience approach for controls twenty spastic cerebral palsied and twenty control ones with age (5-12 years old and sex (F=20, M=20 were matched and identified. All possible inclusion and exclusion criteria were considered by thorough past medical, clinical and para clinical such as chest X-ray and Complete Blood Counts reviews to rule out any possible pulmonary and/or systemic disorders. Their speech respiratory indices were determined by Respirometer (ST 1-dysphonia, made and normalized by Glasgow University. Obtained data were analyzed by independent T test. Results: There were significant differences between cases and control groups for "mean tidal volume", "phonatory volume" and "vital capacity" at a=0/05 values and these values in patients were less (34% than normal children (P<0/001. Conclusion: Measures obtained are highly crucial for speech therapist in any speech therapy primary rehabilitative plans for spactic cerebral palsied children.

Clinical and Radiographic Outcomes of C1 Laminectomy Without Fusion in Patients With Cervical Myelopathy That Is Associated With a Retro-odontoid Pseudotumor.

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Takemoto, Mitsuru; Neo, Masashi; Fujibayashi, Shunsuke; Sakamoto, Takeshi; Ota, Masato; Otsuki, Bungo; Kaneko, Hiroki; Umebayashi, Takeshi

2016-12-01

A retro-odontoid pseudotumor that is not associated with rheumatoid arthritis or hemodialysis is clinically rare. The majority of surgeons select transoral resection as the surgical treatment, often followed by posterior fusion or posterior decompression and fusion. In contrast, some authors have reported success with simple decompression without posterior stabilization in cases where atlanto-axial instability (AAI) is either absent or minor. In this study, we have evaluated the clinical and radiographic outcomes of C1 laminectomy without fusion as the surgical treatment for patients with cervical myelopathy that is associated with a retro-odontoid pseudotumor. A retrospective chart review was conducted on 10 patients who underwent C1 laminectomy without fusion for cervical myelopathy associated with a retro-odontoid pseudotumor. The average follow-up time was 29 months. All cases were graded as Ranawat grade 3a or 3b. After surgery, myelopathy improved in all of the patients. In 2 patients, the atlas-dens interval increased in the flexed position; however, this did not result in any clinical problems. The size of the retro-odontoid mass (measured on magnetic resonance images at least 12 mo after surgery) decreased in 4 of the 10 cases. AAI progression and mass enlargement were our primary concerns for this surgical option; however, C1 laminectomy did not cause severe AAI progression, no patients showed serious mass enlargement, and all patients demonstrated neurological improvement. This surgical strategy is beneficial especially for elderly patients given the risks of other surgical options that use an anterior transoral approach or posterior fusion.

Robot-Assisted Rehabilitation of Ankle Plantar Flexors Spasticity: A Three-Month Study with Proprioceptive Neuromuscular Facilitation

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Zhihao Zhou

2016-11-01

Full Text Available In this paper, we aim to investigate the effect of Proprioceptive Neuromuscular Facilitation (PNF based rehabilitation for ankle plantar flexors spasticity by using a Robotic Ankle-foot Rehabilitation System (RARS. A modified robot-assisted system was proposed and seven post-stroke patients with hemiplegic spastic ankles participated a three-month of robotic PNF training. Their impaired sides were used as the experimental group while their unimpaired sides as the control group. A robotic intervention for the experimental group generally started from a two minutes passive stretching to warm-up or relax the soleus and gastrocnemius muscle and also ended with the same one. Then a PNF training session included 30 trails was activated between them. The rehabilitation trainings were carried out three times a week as an addition of their regular rehabilitation exercise. Passive ankle joint range of motion, resistance torque and stiffness were measured in both ankles before and after the intervention. The changes in Achilles' tendon length, walking speed, and lower limb function were also evaluated by the same physician or physiotherapist for each participant. Biomechanical measurements before interventions showed significant difference between the experimental group and the control group due to ankle spasticity. For the control group, there was no significant difference in the three months with no robotic intervention. But for the experimental group, passive dorsiflexion range of motion increased ( p0.05 . The robotic rehabilitation also improved the muscle strength ( p0.05 and fast walking speed ( p<0.05 . These results indicated that PNF based robotic intervention could significantly alleviate lower limb spasticity and improve the motor function in chronic stroke participant. The robotic system could potentially be used as an effective tool in post-stroke rehabilitation training.

Progression of scoliosis in patients with spastic quadriplegia after the insertion of an intrathecal baclofen pump.

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Ginsburg, Glen M; Lauder, Anthony J

2007-11-15

Medical and radiographic review of 19 consecutive patients with spastic quadriplegia before and after intrathecal baclofen pump insertion with special attention paid to progression of scoliosis. Many orthopedic surgeons who treat spastic quadriplegic patients have noticed a trend of marked scoliosis progression after the administration of intrathecal baclofen (ITB) via subcutaneous pump and catheter. The purpose of this study is to quantify scoliosis progression in this patient population before and after baclofen administration and compare this to published natural history data. The authors had noted rapid progression of scoliosis in spastic quadriplegic patients after intrathecal baclofen pump insertion. This had been noted at other centers, but no significant statistical analysis had been done comparing prepump to postpump scoliosis progression in these patients. To document the magnitude and rate of scoliosis progressions after the placement of an ITB pump, the charts and radiographs of 19 consecutive nonambulatory patients with spastic quadriplegia and an ITB pump were reviewed. To document the rate of scoliosis progression, each patient had at least 2 pre and 2 postpump insertion spinal radiographs made. All radiographs were made with the patients in the supine position without orthoses. A board-certified orthopedic surgeon reviewed these radiographs. Skeletal maturity was assessed using Risser grading. Catheter tip location and rate of baclofen administration were recorded. For 19 patients with complete radiographic data, average Cobb angles were 10.2 degrees before pump insertion and 25 degrees at an average of 20.9 months after pump insertion (P quadriplegia and ITB pump. The authors are now performing spinal fusions for curves that exceed 40 degrees to 50 degrees in the presence of an ITB pump as recommended by previous reviews of scoliosis and accompanying quadriplegia.

Clinical experience with THC:CBD oromucosal spray in patients with multiple sclerosis-related spasticity.

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Koehler, Jürgen; Feneberg, Wolfgang; Meier, Martin; Pöllmann, Walter

2014-09-01

This detailed medical charts' data collection study conducted at a multiple sclerosis (MS) clinic in Germany evaluated the effectiveness of tetrahydrocannabinol (THC)/cannabidiol (CBD) oromucosal spray in patients with resistant MS spasticity. Over a 15-month timeframe, THC:CBD spray was initiated in 166 patients. Mean follow-up was 9 months. In all, 120 patients remained on treatment for a response rate of 72%. THC:CBD spray was used as add-on therapy in 95 patients and as monotherapy in 25 patients to achieve best-possible therapeutic results. Among responders, the mean spasticity 0-10 numerical rating scale (NRS) score decreased by 57%, from 7.0 before treatment to 3.0 within 10 days of starting THC:CBD spray. The mean dosage was 4 sprays/day. Most patients who withdrew from treatment (40/46) had been receiving THC:CBD spray for less than 60 days. Main reasons for treatment discontinuation were: adverse drug reactions, mainly dizziness, fatigue and oral discomfort (23 patients; 13.9%); lack of efficacy (14 patients; 8.4%); or need for a baclofen pump (9 patients; 5.4%). No new safety signals were noted with THC:CBD spray during the evaluation period. In this routine clinical practice setting at an MS clinic in Germany, THC:CBD spray was effective and well tolerated as add-on therapy or as monotherapy in a relevant proportion of patients with resistant MS spasticity.

The 25-question Geriatric Locomotive Function Scale predicts the risk of recurrent falls in postoperative patients with cervical myelopathy.

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Kimura, Atsushi; Takeshita, Katsushi; Inoue, Hirokazu; Seichi, Atsushi; Kawasaki, Yosuke; Yoshii, Toshitaka; Inose, Hiroyuki; Furuya, Takeo; Takeuchi, Kazuhiro; Matsunaga, Shunji; Seki, Shoji; Tsushima, Mikito; Imagama, Shiro; Koda, Masao; Yamazaki, Masashi; Mori, Kanji; Nishimura, Hirosuke; Endo, Kenji; Yamada, Kei; Sato, Kimiaki; Okawa, Atsushi

2018-01-01

Fall-induced injuries represent a major public health concern for older individuals. The relationship between risk of falling and the severity of locomotive syndrome (LS) remains largely unknown. We conducted a retrospective analysis of patients who had undergone surgery from January 2012 to December 2013 and completed at least 1 year of follow-up at 12 participating institutes. Patients completed a questionnaire survey regarding their fall experience during a routine postoperative follow-up. Questionnaire items included the number of falls during the prior postoperative year and the 25-question Geriatric Locomotive Function Scale (GLFS-25). The severity of cervical myelopathy was assessed using the Japanese Orthopaedic Association (JOA) score. We analyzed the association between the incidence of falling and the severity of LS measured by the GLFS-25. Of 360 patients, 61 (16.9%) experienced 1 fall; 31 (8.6%), 2-3 falls; 4 (1.1%), 4-5 falls; and 6 (1.7%), ≥6 falls during the first postoperative year. Thus, 102 (28%) patients experienced at least 1 fall, and 41 (11%) experienced recurrent falls (2 or more falls) during the time period. The mean GLFS-25 score was 30.2 ± 22.7, and 242 (62%) patients had GLFS-25 scores of 16 or higher, which fulfilled the diagnostic criteria for LS. When subjects were categorized into recurrent fallers and non-recurrent fallers, recurrent fallers had a significantly higher GLFS-25 score and a significantly lower extremity motor function score of the JOA score than non-recurrent fallers. The GLFS-25 and lower extremity motor function score of the JOA score yielded the areas under the receiver operating characteristic curves of 0.674 and 0.607, respectively, to differentiate recurrent fallers from non-recurrent fallers. Postoperative patients with cervical myelopathy had a 62% prevalence of LS. The GLFS-25 may be useful to predict the risk of recurrent falls in patients with cervical myelopathy. Copyright © 2017 The Japanese

Evaluation of low-level laser therapy in the treatment of masticatory muscles spasticity in children with cerebral palsy

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Santos, Maria Teresa Botti Rodrigues; Diniz, Michele Baffi; Gouw-Soares, Sheila Cynthia; Lopes-Martins, Rodrigo Alvaro Brandão; Frigo, Lucio; Baeder, Fernando Martins

2016-02-01

Spasticity is a motor disorder frequently present in individuals with cerebral palsy (CP). This study aimed to evaluate the effect of low-level laser therapy (LLLT) on the spasticity of the masseter and anterior temporal muscle fibers in children with CP over three weeks of intermittent laser exposures. The bite force (BF) of the masticatory muscles and the amplitude of mouth opening were evaluated before and after laser irradiation in 30 children with CP. Both sides of the masseter and temporalis muscles were irradiated with low-intensity diode laser pulses of 808-nm wavelength six times over three consecutive weeks. During the subsequent three weeks of postlaser exposures, although no laser treatment was applied, the evaluation parameters were measured and recorded. A significant improvement in the amplitude of mouth opening and a decrease in the BF were observed in the weeks following LLLT (Peffective short-term therapeutic tool. This method increased the amplitude of mouth opening and decreased the muscle tonus of children with spastic CP over a time course of three weeks of intermittent laser applications.

Increased low-frequency oscillation amplitude of sensorimotor cortex associated with the severity of structural impairment in cervical myelopathy.

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Fuqing Zhou

Full Text Available Decreases in metabolites and increased motor-related, but decreased sensory-related activation of the sensorimotor cortex (SMC have been observed in patients with cervical myelopathy (CM using advanced MRI techniques. However, the nature of intrinsic neuronal activity in the SMC, and the relationship between cerebral function and structural damage of the spinal cord in patients with CM are not fully understood. The purpose of this study was to assess intrinsic neuronal activity by calculating the regional amplitude of low frequency fluctuations (ALFF using resting-state functional MRI (rs-fMRI, and correlations with clinical and imaging indices. Nineteen patients and 19 age- and sex-matched healthy subjects underwent rs-fMRI scans. ALFF measurements were performed in the SMC, a key brain network likely to impaired or reorganized patients with CM. Compared with healthy subjects, increased amplitude of cortical low-frequency oscillations (LFO was observed in the right precentral gyrus, right postcentral gyrus, and left supplementary motor area. Furthermore, increased z-ALFF values in the right precentral gyrus and right postcentral gyrus correlated with decreased fractional anisotropy values at the C2 level, which indicated increased intrinsic neuronal activity in the SMC corresponding to the structural impairment in the spinal cord of patients with CM. These findings suggest a complex and diverging relationship of cortical functional reorganization and distal spinal anatomical compression in patients with CM and, thus, add important information in understanding how spinal cord integrity may be a factor in the intrinsic covariance of spontaneous low-frequency fluctuations of BOLD signals involved in cortical plasticity.

Correlations between risk factors and functional evolution in patients with spastic quadriplegia.

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Rogoveanu, O C; Tuțescu, N C; Kamal, D; Alexandru, D O; Kamal, C; Streba, L; Trăistaru, M R

2016-01-01

Cerebral palsy is the most common cause of developing neuro-motor disability in children, in many cases, the triggering cause remaining unknown. Quadriplegia is the most severe spastic cerebral palsy, characterized by severe mental retardation and bi-pyramidal syndrome. The purpose of this paper was to demonstrate the importance of knowing the risk factors and the psychosomatic ones, determining to what extent they influence the functional evolution in patients diagnosed with spastic quadriplegia. 23 children diagnosed with spastic quadriplegia were included in the study, being aged between 1 year and half and 12 years. Patients were assessed at baseline (T1), at one year (T2) and after two years at the end of the study (T3). Patients received a comprehensive rehabilitation program for the motor and sensory deficits throughout the study. Initially, a comprehensive evaluation (etiopathogenic, clinical and functional) that started from a thorough medical history of children (the older ones), was conducted but chose parents to identify the risk factors, and a complete physical exam. At each assessment, joint and muscle balance was conducted. To assess functionality, the gross motor function classification systems (GMFCS) and manual ability (MACS) were used. Many risk factors that were classified according to the timeline in prenatal factors, perinatal and postnatal, were identified from a thorough history. A direct correlation was noticed between the decrease of coarse functionality and manual ability, both initially and in dynamic and low APGAR scores, low gestational age, low birth weight and a higher body mass index of the mother. A direct link was observed between the gross motor function and the manual ability. A significant improvement in the MACS score was noticed in patients with a better GMFCS score.

MRI of the spinal cord in myelopathy complicating vitamin B12 deficiency: two additional cases and a review of the literature

International Nuclear Information System (INIS)

Bassi, S.S.; Bulundwe, K.K.; Gledhill, R.F.; Labuscagne, J.H.

1999-01-01

Focal spinal cord lesions have been present in all previously reported cases of MRI appearances in myelopathy complicating vitamin B 12 deficiency. We describe two further cases showing mild atrophy only and review the salient features of the previous 11 publications. MRI findings reflect quite closely the known pathological changes in this condition. (orig.)

DNABIT Compress - Genome compression algorithm.

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Rajarajeswari, Pothuraju; Apparao, Allam

2011-01-22

Data compression is concerned with how information is organized in data. Efficient storage means removal of redundancy from the data being stored in the DNA molecule. Data compression algorithms remove redundancy and are used to understand biologically important molecules. We present a compression algorithm, "DNABIT Compress" for DNA sequences based on a novel algorithm of assigning binary bits for smaller segments of DNA bases to compress both repetitive and non repetitive DNA sequence. Our proposed algorithm achieves the best compression ratio for DNA sequences for larger genome. Significantly better compression results show that "DNABIT Compress" algorithm is the best among the remaining compression algorithms. While achieving the best compression ratios for DNA sequences (Genomes),our new DNABIT Compress algorithm significantly improves the running time of all previous DNA compression programs. Assigning binary bits (Unique BIT CODE) for (Exact Repeats, Reverse Repeats) fragments of DNA sequence is also a unique concept introduced in this algorithm for the first time in DNA compression. This proposed new algorithm could achieve the best compression ratio as much as 1.58 bits/bases where the existing best methods could not achieve a ratio less than 1.72 bits/bases.

Assessing the immediate impact of botulinum toxin injection on impedance of spastic muscle.

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Li, Xiaoyan; Shin, Henry; Li, Le; Magat, Elaine; Li, Sheng; Zhou, Ping

2017-05-01

This study aimed to investigate the immediate impacts of Botulinum Toxin A (BoNT-A) injections on the inherent electrical properties of spastic muscles using a newly developed electrical impedance myography (EIM) technique. Impedance measures were performed before and after a BoNT-A injection in biceps brachii muscles of 14 subjects with spasticity. Three major impedance variables, resistance (R), reactance (X) and phase angle (θ) were obtained from three different configurations, and were evaluated using the conventional EIM frequency at 50kHz as well as multiple frequency analysis. Statistical analysis demonstrated a significant decrease of resistance in the injected muscles (Multiple-frequency: R pre =25.17±1.94Ohm, R post =23.65±1.63Ohm, ptoxin effects on the muscle. This study demonstrated high sensitivity of the EIM technique in the detection of alterations to muscle composition. Copyright © 2017 IPEM. Published by Elsevier Ltd. All rights reserved.

Pathological investigation of radiation necrosis. A case report and histo-pathological analysis of radiation myelopathy

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Nakamura, N; Yoshimura, N; Ikuta, F [Niigata Univ. (Japan). School of Medicine

1975-05-01

The brain and spinal cord of an 18-year-old male, who suffered from cerebellar medulloblastoma with subarachnoid spread, had been irradiated by a large amount of Linac X-ray: 14,450 rads to the lower thoracic segments and 7,400 rads to the lumbar segments. The tumor at the roof of the 4th ventricle had disseminated along the ventricular system but was limited to the subarachnoid space of the cervical spinal cord. No remarkable changes were found in the volume or consistency of the thoracic and lumbar cord. Elasticity of the lower thoracic segment was greatly diminished and the cut surfaces were yellowish white and fragile. Microscopically extensive coagulation necrosis was observed with complete disintegration of myelin and axon. Vascular changes were most prominent in the smaller vessels, eg. hyalinous thickening, concentric cleavage, adventitial fibrosis and edema of small artery perivascular spaces, fibrin thrombi occulusion of arterioles and capillaries, and telangiectasia. In the lumbar spinal cord, moderate neuronal degeneration and protoplasmic astrocytosis were observed. Changes in the lumbar posterior white column were considered to be not only secondary degeneration but also a primary lesion caused by irradiation. Liquefactive necrosis in the gray matter of the cervical cord was thought to be a nonspecific circulatory disturbance because of the absence of vascular changes. Vascular changes were thought to be very important in the histological diagnosis of radiation myelopathy and it was supposed that increased permeability of the vessel walls was a factor in coagulation necrosis. They considered this case to have typical histology of radiation myelopathy.

Heterozygous KIDINS220/ARMS nonsense variants cause spastic paraplegia, intellectual disability, nystagmus, and obesity

NARCIS (Netherlands)

Josifova, Dragana J.; Monroe, Glen R.; Tessadori, Federico; de Graaff, Esther; van der Zwaag, Bert; Mehta, Sarju G.; Harakalova, Magdalena; Duran, Karen J.; Savelberg, Sanne M. C.; Nijman, Isaäc J.; Jungbluth, Heinz; Hoogenraad, Casper C.; Bakkers, Jeroen; Knoers, Nine V.; Firth, Helen V.; Beales, Philip L.; van Haaften, Gijs; van Haelst, Mieke M.

2016-01-01

We identified de novo nonsense variants in KIDINS220/ARMS in three unrelated patients with spastic paraplegia, intellectual disability, nystagmus, and obesity (SINO). KIDINS220 is an essential scaffold protein coordinating neurotrophin signal pathways in neurites and is spatially and temporally

Warm-needle moxibustion for spasticity after stroke: A systematic review of randomized controlled trials.

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Yang, Liu; Tan, Jing-Yu; Ma, Haili; Zhao, Hongjia; Lai, Jinghui; Chen, Jin-Xiu; Suen, Lorna K P

2018-03-22

Spasticity is a common post-stroke complication, and it results in substantial deterioration in the quality of life of patients. Although potential positive effects of warm-needle moxibustion on spasticity after stroke have been observed, evidence on its definitive effect remains uncertain. This study aimed to summarize clinical evidence pertaining to therapeutic effects and safety of warm-needle moxibustion for treating spasticity after stroke. Randomized controlled trials were reviewed systematically on the basis of the Cochrane Handbook for Systematic Reviews of Interventions. The report follows the PRISMA statement. Ten electronic databases (PubMed, CENTRAL, EMBASE, AMED, CINAHL, Web of Science, CBM, CNKI, WanFang, and VIP) were explored, and articles were retrieved manually from two Chinese journals (The Journal of Traditional Chinese Medicine and Zhong Guo Zhen Jiu) through retrospective search. Randomized controlled trials with warm-needle moxibustion as treatment intervention for patients with limb spasm after stroke were included in this review. The risk of bias assessment tool was utilized in accordance with Cochrane Handbook 5.1.0. All included studies reported spasm effect as primary outcome. Effect size was estimated using relative risk, standardized mean difference, or mean difference with a corresponding 95% confidence interval. Review Manager 5.3 was utilized for meta-analysis. Twelve randomized controlled trials with certain methodological flaws and risk of bias were included, and they involved a total of 878 participants. Warm-needle moxibustion was found to be superior to electroacupuncture or acupuncture in reducing spasm and in promoting motor function and daily living activities. Pooled results for spasm effect and motor function were significant when warm-needle moxibustion was compared with electroacupuncture or acupuncture. A comparison of daily living activities indicated significant differences between warm-needle moxibustion and

Neuromuscular properties of different spastic human joints vary systematically.

Science.gov (United States)

Mirbagheri, M M; Settle, K

2010-01-01

We quantified the mechanical abnormalities of the spastic wrist in chronic stroke survivors, and determined whether these findings were representative of those recorded at the elbow and ankle joints. System identification techniques were used to characterize the mechanical abnormalities of these joints and to identify the contribution of intrinsic and reflex stiffness to these abnormalities. Modulation of intrinsic and reflex stiffness with the joint angle was studied by applying PRBS perturbations to the joints at different joint angles over the range of motion. Age-matched healthy subjects were used as control.

Autosomal dominant spastic paraplegia with peripheral neuropathy maps to chr12q23-24.

NARCIS (Netherlands)

Schule, R.; Bonin, M.; Durr, A.; Forlani, S.; Sperfeld, A.D.; Klimpe, S.; Mueller, J.C.; Seibel, A.; Warrenburg, B.P.C. van de; Bauer, P.; Schols, L.

2009-01-01

OBJECTIVE: Hereditary spastic paraplegias (HSP) are genetically exceedingly heterogeneous. To date, 37 genetic loci for HSP have been described (SPG1-41), among them 16 loci for autosomal dominant disease. Notwithstanding, further genetic heterogeneity is to be expected in HSP, as various HSP

Factors Influencing Goal Attainment in Patients with Post-Stroke Upper Limb Spasticity Following Treatment with Botulinum Toxin A in Real-Life Clinical Practice: Sub-Analyses from the Upper Limb International Spasticity (ULIS-II Study

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Klemens Fheodoroff

2015-04-01

Full Text Available In this post-hoc analysis of the ULIS-II study, we investigated factors influencing person-centred goal setting and achievement following botulinum toxin-A (BoNT-A treatment in 456 adults with post-stroke upper limb spasticity (ULS. Patients with primary goals categorised as passive function had greater motor impairment (p < 0.001, contractures (soft tissue shortening [STS] (p = 0.006 and spasticity (p = 0.02 than those setting other goal types. Patients with goals categorised as active function had less motor impairment (0.0001, contracture (p < 0.0001, spasticity (p < 0.001 and shorter time since stroke (p = 0.001. Patients setting goals for pain were older (p = 0.01 with more contractures (p = 0.008. The proportion of patients achieving their primary goal was not impacted by timing of first-ever BoNT-A injection (medium-term (≤1 year vs. longer-term (>1 year post-stroke (80.0% vs. 79.2% or presence or absence of severe contractures (76.7% vs. 80.6%, although goal types differed. Earlier BoNT-A intervention was associated with greater achievement of active function goals. Severe contractures impacted negatively on goal achievement except in pain and passive function. Goal setting by patients with ULS is influenced by impairment severity, age and time since stroke. Our findings resonate with clinical experience and may assist patients and clinicians in selecting realistic, achievable goals for treatment.

A Rare Case of Neuromyelitis Optica Spectrum Disorder in Patient with Sjogren’s Syndrome

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Supat Thongpooswan

2014-01-01

Full Text Available We report a 48-year-old female with the history of Sjogren’s syndrome who presented with 3-week history of tingling, numbness, and shooting back, waist, and bilateral leg pain and numbness in the pelvic region with urinary and bowel incontinence. Physical examination was remarkable for reduced motor power in both lower extremities with spasticity. Sensory deficit was noted at the T6 level. Laboratory investigation revealed elevated ESR and CRP and positive serum antiaquaporin-4 IgG. Thoracic and lumbar magnetic resonance imaging revealed abnormal patchy areas, leptomeningeal enhancement through the thoracic cord extending from T3 through T6 levels, without evidence of cord compression. Impression of neuromyelitis optica spectrum disorder was made and patient was treated with methylprednisolone intravenously followed by tapering oral prednisone. Neurological symptoms gradually improved with resolution of bowel and urinary incontinence. In a patient with Sjogren’s syndrome who presents with neurological complaints, the possibility of neuromyelitis optica or neuromyelitis optica spectrum disorder should be considered. Awareness of the possibility of CNS disease is important due to the serious nature of CNS complications, some of which are treatable with immunosuppressants. Our patient with Sjogren’s syndrome who presented with myelopathy benefited from early recognition and institution of appropriate therapy.

Direct analysis of viral-specific CD8+ T cells with soluble HLA-A2/Tax11-19 tetramer complexes in patients with human T cell lymphotropic virus-associated myelopathy.

Science.gov (United States)

Bieganowska, K; Höllsberg, P; Buckle, G J; Lim, D G; Greten, T F; Schneck, J; Altman, J D; Jacobson, S; Ledis, S L; Hanchard, B; Chin, J; Morgan, O; Roth, P A; Hafler, D A

1999-02-01

Human T cell lymphotropic virus-I (HTLV-I)-associated myelopathy is a slowly progressive neurologic disease characterized by inflammatory infiltrates in the central nervous system accompanied by clonal expansion of HTLV-I-reactive CD8+ T-cells. In patients carrying the HLA-A2 allele, the immune response is primarily directed to the Tax11-19 peptide. The frequency, activation state, and TCR usage of HLA-A2/Tax11-19 binding T cells in patients with HTLV-I-associated myelopathy was determined using MHC class I tetramers loaded with the Tax11-19 peptide. Circulating Tax11-19-reactive T cells were found at very high frequencies, approaching 1:10 circulating CD8+ T cells. T cells binding HLA-A2/Tax11-19 consisted of heterogeneous populations expressing different chemokine receptors and the IL-2R beta-chain but not the IL-2R alpha-chain. Additionally, Tax11-19-reactive CD8+ T cells used one predominant TCR Vbeta-chain for the recognition of the HLA-A2/Tax11-19 complex. These data provide direct evidence for high frequencies of circulating Tax11-19-reactive CD8+ T cells in patients with HTLV-I-associated myelopathy.

[Consensus document on spasticity in patients with multiple sclerosis. Grupo de Enfermedades Desmielinizantes de la Sociedad Española de Neurología].

Science.gov (United States)

Oreja-Guevara, Celia; Montalban, Xavier; de Andrés, Clara; Casanova-Estruch, Bonaventura; Muñoz-García, Delicias; García, Inmaculada; Fernández, Óscar

2013-10-16

Multiple sclerosis is a chronic neurological inflammatory demyelinating disease. Specialists involved in the symptomatic treatment of this disease tend to apply heterogeneous diagnostic and treatment criteria. To establish homogeneous criteria for treating spasticity based on available scientific knowledge, facilitating decision-making in regular clinical practice. A group of multiple sclerosis specialists from the Spanish Neurological Society demyelinating diseases working group met to review aspects related to spasticity in this disease and draw up the consensus. After an exhaustive bibliographic search and following a metaplan technique, a number of preliminary recommendations were established to incorporate into the document. Finally, each argument was classified depending on the degree of recommendation according to the SIGN (Scottish Intercollegiate Guidelines Network) system. The resulting text was submitted for review by the demyelinating disease group. An experts' consensus was reached regarding spasticity triggering factors, related symptoms, diagnostic criteria, assessment methods, quality of life and therapeutic management (drug and non-drug) criteria. The recommendations included in this consensus can be a useful tool for improving the quality of life of multiple sclerosis patients, as they enable improved diagnosis and treatment of spasticity.

Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease.

Science.gov (United States)

Casari, G; De Fusco, M; Ciarmatori, S; Zeviani, M; Mora, M; Fernandez, P; De Michele, G; Filla, A; Cocozza, S; Marconi, R; Dürr, A; Fontaine, B; Ballabio, A

1998-06-12

Hereditary spastic paraplegia (HSP) is characterized by progressive weakness and spasticity of the lower limbs due to degeneration of corticospinal axons. We found that patients from a chromosome 16q24.3-linked HSP family are homozygous for a 9.5 kb deletion involving a gene encoding a novel protein, named Paraplegin. Two additional Paraplegin mutations, both resulting in a frameshift, were found in a complicated and in a pure form of HSP. Paraplegin is highly homologous to the yeast mitochondrial ATPases, AFG3, RCA1, and YME1, which have both proteolytic and chaperon-like activities at the inner mitochondrial membrane. Immunofluorescence analysis and import experiments showed that Paraplegin localizes to mitochondria. Analysis of muscle biopsies from two patients carrying Paraplegin mutations showed typical signs of mitochondrial OXPHOS defects, thus suggesting a mechanism for neurodegeneration in HSP-type disorders.

Spinal cord compression secondary to extramedullary hematopoiesis in a dog.

Science.gov (United States)

Williams, Lindsay M; Skeen, Todd M

2013-03-15

An 11-year-old spayed female Siberian Husky was evaluated because of a 2-week history of progressive paraparesis. Results of neurologic examination were consistent with a T3-L3 myelopathy. There were no abnormalities on CBC, and hypercalcemia was noted on serum biochemical analysis. Several hypoechoic splenic nodules were evident on abdominal ultrasonography, and results of fine-needle aspiration cytology were consistent with splenic extramedullary hematopoiesis (EMH). Two compressive, extradural masses in the dorsal epidural space of the thoracolumbar region of the spinal cord were seen on MRI images. A dorsal laminectomy was performed to remove the extradural spinal masses. Results of histologic examination of tissue samples were consistent with EMH. Following surgery, clinical signs of paraparesis resolved, and there was no recurrence of the masses 24 months after surgery. Extramedullary hematopoesis should be considered as a differential diagnosis in dogs in which results of diagnostic imaging indicate a epidural mass. In human patients, spinal EMH usually occurs secondary to an underlying hematologic disease, but it can also occur spontaneously. Treatment options reported for humans include surgical decompression, radiation therapy, chemotherapy, and blood transfusion. The dog of this report responded favorably to surgical decompression and was clinically normal 2 years after surgery.

Effects of early spasticity treatment on children with hemiplegic cerebral palsy: a preliminary study

Directory of Open Access Journals (Sweden)

Marise Bueno Zonta

2013-07-01

Full Text Available Objective To compare motor and functional performance of two groups of children with hemiplegic cerebral palsy (HCP. Only the study group (SG received early treatment of spasticity with botulinum neurotoxin type A (BXT-A. Methods Gross Motor Function Measure (GMFM, functional performance (Pediatric Evaluation of Disability Inventory - PEDI, range of movement, gait pattern (Physician Rating Scale - PRS and the speed of hand movements were considered. Results The SG, composed of 11 HCP (45.64±6.3 months, was assessed in relation to the comparison group, composed of 13 HCP (45.92±6.4 months. SG showed higher scores in four of the five GMFM dimensions, which included scores that were statistically significant for dimension B, and higher scores in five of the six areas evaluated in the PEDI. Active wrist extension, the speed of hand movements and PRS score were higher in the SG. Conclusion Children who received early BXT-A treatment for spasticity showed higher scores in motor and functional performance.

Time-dependent, bidirectional, anti- and pro-spinal hyper-reflexia and muscle spasticity effect after chronic spinal glycine transporter 2 (GlyT2) oligonucleotide-induced downregulation.

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Kamizato, Kota; Marsala, Silvia; Navarro, Michael; Kakinohana, Manabu; Platoshyn, Oleksandr; Yoshizumi, Tetsuya; Lukacova, Nadezda; Wancewicz, Ed; Powers, Berit; Mazur, Curt; Marsala, Martin

2018-07-01

The loss of local spinal glycine-ergic tone has been postulated as one of the mechanisms contributing to the development of spinal injury-induced spasticity. In our present study using a model of spinal transection-induced muscle spasticity, we characterize the effect of spinally-targeted GlyT2 downregulation once initiated at chronic stages after induction of spasticity in rats. In animals with identified hyper-reflexia, the anti-spasticity effect was studied after intrathecal treatment with: i) glycine, ii) GlyT2 inhibitor (ALX 1393), and iii) GlyT2 antisense oligonucleotide (GlyT2-ASO). Administration of glycine and GlyT2 inhibitor led to significant suppression of spasticity lasting for a minimum of 45-60 min. Treatment with GlyT2-ASO led to progressive suppression of muscle spasticity seen at 2-3 weeks after treatment. Over the subsequent 4-12 weeks, however, the gradual appearance of profound spinal hyper-reflexia was seen. This was presented as spontaneous or slight-tactile stimulus-evoked muscle oscillations in the hind limbs (but not in upper limbs) with individual hyper-reflexive episodes lasting between 3 and 5 min. Chronic hyper-reflexia induced by GlyT2-ASO treatment was effectively blocked by intrathecal glycine. Immunofluorescence staining and Q-PCR analysis of the lumbar spinal cord region showed a significant (>90%) decrease in GlyT2 mRNA and GlyT2 protein. These data demonstrate that spinal GlyT2 downregulation provides only a time-limited therapeutic benefit and that subsequent loss of glycine vesicular synthesis resulting from chronic GlyT2 downregulation near completely eliminates the tonic glycine-ergic activity and is functionally expressed as profound spinal hyper-reflexia. These characteristics also suggest that chronic spinal GlyT2 silencing may be associated with pro-nociceptive activity. Copyright © 2018 Elsevier Inc. All rights reserved.

Complicated spastic paraplegia in patients with AP5Z1 mutations (SPG48)

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Hirst, Jennifer; Madeo, Marianna; Smets, Katrien; Edgar, James R.; Schols, Ludger; Li, Jun; Yarrow, Anna; Deconinck, Tine; Baets, Jonathan; Van Aken, Elisabeth; De Bleecker, Jan; Datiles, Manuel B.; Roda, Ricardo H.; Liepert, Joachim; Züchner, Stephan; Mariotti, Caterina; De Jonghe, Peter; Blackstone, Craig

2016-01-01

Objective: Biallelic mutations in the AP5Z1 gene encoding the AP-5 ζ subunit have been described in a small number of patients with hereditary spastic paraplegia (HSP) (SPG48); we sought to define genotype–phenotype correlations in patients with homozygous or compound heterozygous sequence variants predicted to be deleterious. Methods: We performed clinical, radiologic, and pathologic studies in 6 patients with biallelic mutations in AP5Z1. Results: In 4 of the 6 patients, there was complete loss of AP-5 ζ protein. Clinical features encompassed not only prominent spastic paraparesis but also sensory and motor neuropathy, ataxia, dystonia, myoclonus, and parkinsonism. Skin fibroblasts from affected patients tested positive for periodic acid Schiff and autofluorescent storage material, while electron microscopic analysis demonstrated lamellar storage material consistent with abnormal storage of lysosomal material. Conclusions: Our findings expand the spectrum of AP5Z1-associated neurodegenerative disorders and point to clinical and pathophysiologic overlap between autosomal recessive forms of HSP and lysosomal storage disorders. PMID:27606357

Functional influence of botulinum neurotoxin type A treatment (Xeomin® of multifocal upper and lower limb spasticity on chronic hemiparetic gait

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Maurizio Falso

2012-05-01

Full Text Available This report describes the modification of hemiplegic shoulder pain and walking velocity through injections of Xeomin®, a new botulinum neurotoxin type A formulation, in a 67-year-old woman with chronic residual left hemiparesis and hemiparetic gait attributable to stroke. Clinical evaluation included upper and lower limb spasticity, upper and lower limb pain, trunk control, upper and lower limb motricity index, visual gait analysis, and gait velocity. Assessments were performed before, 1 week after, and 1 month after treatment. Improvement was observed in all clinical parameters assessed. Amelioration of spasticity of the upper and lower limbs and shoulder pain was observed after 1 month. Trunk postural attitude and paraxial muscle recruitment recovered. No adverse events were observed and the patient shows significant improvement of functional impairment derived from chronic spasticity after treatment with Xeomin®. We also provide a simple and useful protocol for clinical evaluation of the treatment.

MRI in the investigation of patients with myelopathy thought to be due to multiple sclerosis

International Nuclear Information System (INIS)

Papadopoulos, A.; Gouliamos, A.; Trakadas, S.; Kalovidouris, A.; Sgouropoulos, P.; Gatzonis, S.; Vlahos, L.

1995-01-01

The role of cerebral and spinal cord MRI was investigated in 65 patients with myelopathy suspected of having demyelinating disease. Cerebral MRI demonstrated lesions compatible with demyelination in 80% and spinal cord MRI in 68.6%. In 28.5% of our patients brain lesions were present with normal spinal cord images, but in 17% spinal cord lesions were depicted with a normal brain MRI. The combination of the two examinations demonstrated lesions in 97% of the patients. The frequency of coexistent cerebral lesions in patients with spinal cord lesions was over 85% in patients with chronic disease but only 28.5% in patients with acute myelitis. (orig.)

MRI in the investigation of patients with myelopathy thought to be due to multiple sclerosis

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Papadopoulos, A. [CT and MRI Unit, X-Ray Dept., `Areteion` Hospital, Univ. Athens (Greece); Gouliamos, A. [CT and MRI Unit, X-Ray Dept., `Areteion` Hospital, Univ. Athens (Greece); Trakadas, S. [CT and MRI Unit, X-Ray Dept., `Areteion` Hospital, Univ. Athens (Greece); Kalovidouris, A. [CT and MRI Unit, X-Ray Dept., `Areteion` Hospital, Univ. Athens (Greece); Sgouropoulos, P. [Neurology Dept., `Eginition` Hospital, Univ. Athens Medical School (Greece); Gatzonis, S. [Neurology Dept., `Eginition` Hospital, Univ. Athens Medical School (Greece); Vlahos, L. [CT and MRI Unit, X-Ray Dept., `Areteion` Hospital, Univ. Athens (Greece)

1995-07-01

The role of cerebral and spinal cord MRI was investigated in 65 patients with myelopathy suspected of having demyelinating disease. Cerebral MRI demonstrated lesions compatible with demyelination in 80% and spinal cord MRI in 68.6%. In 28.5% of our patients brain lesions were present with normal spinal cord images, but in 17% spinal cord lesions were depicted with a normal brain MRI. The combination of the two examinations demonstrated lesions in 97% of the patients. The frequency of coexistent cerebral lesions in patients with spinal cord lesions was over 85% in patients with chronic disease but only 28.5% in patients with acute myelitis. (orig.)

Computed tomography in cervical spondylotic myelopathy and radiculopathy: Visualisation of structures, myelographic comparison, cord measurements and clinical utility

International Nuclear Information System (INIS)

Yu, Y.L.; Du Boulay, G.H.; Stevens, J.M.; Kendall, B.E.

1986-01-01

Sixty-nine patients with cervical spondylotic myelopathy (CSM), radiculopathy (CSR), or both (CSMR) were studied with computed tomography (CT). Computer-assisted myelography (CAM) accurately determines the site and nature of spondylotic protrusions and provides good visualisation of the subarachnoid space and cord deformities even in areas with dilute metrizamide. However, excessive vertebral movement and bulging ligamenta flava with their effects on cord deformity, so easily visualised in myelograms, are completely or partially missed. In the assessment of CSM, metrizamide myelography (MM) followed by CAM should be performed, particularly when the myelographic images are unsatisfactory due to contrast dilution or blockage, when cord compression cannot be ascertained with MM and when cord atrophy is suspected. In CSR, the diagnostic information from MM and CAM is comparable. The diagnostic criteria in CAM are, however, less direct and since MM is adequate in uncomplicated cases, CAM is generally not necessary. The APD, APD/TD ratio, area and circularity are sensitive indices of cord deformity and the first two should be used more often to assist visual assessment of cord deformity. The relation between cord parameters and treatment response is better reflected in CSM cases managed conservatively and the results suggest that the degree of cord deformity is helpful in determining the outcome and hence the choice between surgical and conservative treatment. In plain CT, the osteophytes and calcified discs are adequately visualised and canal dimensions measured with accuracy, but the cervical cord and roots cannot be properly assessed and the diagnosis of CSM or CSR cannot be ascertained. At present, its role in cervical spondylosis is therefore limited. (orig.)

A Descriptive Study of Lower Limb Torsional Kinematic Profiles in Children With Spastic Diplegia.

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Simon, Anne-Laure; Ilharreborde, Brice; Megrot, Fabrice; Mallet, Cindy; Azarpira, Reza; Mazda, Keyvan; Presedo, Ana; Penneçot, Georges F

2015-09-01

Lower limb rotational anomalies in spastic diplegic children with cerebral palsy (CP) are common and difficult to identify through physical examination alone. The identification and treatment of the overall rotational disorders must be considered to restore physiological lever-arms lengths and lever-arms orientation.The aims of the study were to assess the prevalence of lower limb rotational malalignment and to describe the distribution of the different kinematic torsional profiles in children with spastic diplegia. Instrumented gait analysis data from 188 children with spastic diplegia were retrospectively reviewed. None of the patients had undergone surgery previously or received botulinum toxin treatment within 6 months before the review. Kinematic data, collected at the midstance phase, included: pelvic, hip, and ankle rotation and foot progression angle. The prevalence of kinematic rotational deviations was 98.4%. Sixty-one percent of the children walked with an internal foot progression angle and 21% exhibited external alignment. The pelvis was internally rotated in 41% of the cases and externally in another 27%. Hip rotation was internal in 29% and external in 27% of the cases. Ankle rotation was internal in 55% and external in 16% of the cases. Lower limb rotational anomalies involved more than one level in 77% of the limbs. A kinematic compensatory deviation was identified in at least one level in 48% of the limbs. Kinematic rotational anomalies were identified in nearly all the 188 children in the study. The multilevel involvement of lower limb malalignment was not systematically associated with compensatory mechanisms between the levels. Ankle rotational anomalies were the most frequent cause of lower limb torsional deviations followed by pelvic malalignment. Level IV.

Developmental Profiles of Preschool Children With Spastic Diplegic and Quadriplegic Cerebral Palsy

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Ya-Chen Lee

2010-07-01

Full Text Available Cerebral palsy (CP is a disorder of movement and posture control with multiple impairments. The clinical manifestations of CP vary among children. The aim of this study was to compare the developmental profiles of preschool children with either of two types of CP: spastic diplegic (SD CP and spastic quadriplegic (SQ CP. Relationships between the children's various developmental functions were also investigated. We recruited 137 children with spastic CP, aged 1-5 years (mean age = 3.7 ± 2.1 years, and we classified them into two groups: SD (n = 59 and SQ (n = 78. The comparison group comprised 18 children with typical development. Developmental functions were assessed in all the children, using the Chinese Child Development Inventory with the updated norms. This scale addressed eight functional domains: gross motor ability, fine motor ability, expressive language ability, concept comprehension ability, situation comprehension ability, self-help ability, personal-social skills, and general development. A development quotient (DQ was determined for each domain as a percentage of the developmental age divided by the chronological age. The developmental profiles of the CP subtypes were found to differ. Children with SQ were found to have lower DQs than those with SD (p < 0.01. There was also a difference in the distribution of DQs between the SD and SQ groups, although the lowest DQ in both groups was for the gross motor domain. An uneven delay in the development of gross motor function was found in both groups of children with CP. Motor functions, including gross motor and fine motor functions, were significantly related to self-help ability. Complex and significant correlations among developmental functions were also identified in children with CP. The findings in the present study may allow clinicians to anticipate the developmental profile of children with CP on the basis of whether they have the SD or SQ subtype. This, in turn, is likely to

Direct visualization of antigen-specific T cells: HTLV-1 Tax11-19- specific CD8(+) T cells are activated in peripheral blood and accumulate in cerebrospinal fluid from HAM/TSP patients.

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Greten, T F; Slansky, J E; Kubota, R; Soldan, S S; Jaffee, E M; Leist, T P; Pardoll, D M; Jacobson, S; Schneck, J P

1998-06-23

Human T lymphotropic virus type 1 (HTLV-1) -associated myelopathy/tropic spastic paraparesis is a demyelinating inflammatory neurologic disease associated with HTLV-1 infection. HTLV-1 Tax11-19-specific cytotoxic T cells have been isolated from HLA-A2-positive patients. We have used a peptide-loaded soluble HLA-A2-Ig complex to directly visualize HTLV-1 Tax11-19-specific T cells from peripheral blood and cerebrospinal fluid without in vitro stimulation. Five of six HTLV-1-associated myelopathy/tropic spastic paraparesis patients carried a significant number (up to 13.87%) of CD8(+) lymphocytes specific for the HTLV-1 Tax11-19 peptide in their peripheral blood, which were not found in healthy controls. Simultaneous comparison of peripheral blood and cerebrospinal fluid from one patient revealed 2.5-fold more Tax11-19-specific T cells in the cerebrospinal fluid (23.7% vs. 9.4% in peripheral blood lymphocyte). Tax11-19-specific T cells were seen consistently over a 9-yr time course in one patient as far as 19 yrs after the onset of clinical symptoms. Further analysis of HTLV-1 Tax11-19-specific CD8(+) T lymphocytes in HAM/TSP patients showed different expression patterns of activation markers, intracellular TNF-alpha and gamma-interferon depending on the severity of the disease. Thus, visualization of antigen-specific T cells demonstrates that HTLV-1 Tax11-19-specific CD8(+) T cells are activated, persist during the chronic phase of the disease, and accumulate in cerebrospinal fluid, showing their pivotal role in the pathogenesis of this neurologic disease.

Hereditary spastic paraplegia with cerebellar ataxia: a complex phenotype associated with a new SPG4 gene mutation

DEFF Research Database (Denmark)

Nielsen, Jørgen Erik; Johnson, B; Koefoed, Pernille

2004-01-01

Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria......, unipolar depression, epilepsy, migraine, and cognitive impairment was investigated. Genetic linkage analysis and sequencing of the SPG4 gene was performed and electrophysiologic investigations were carried out in six individuals and positron emission tomography (PET) in one patient. The disease was linked...... in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance...

The effect of spasticity, sense and walking aids in falls of people after chronic stroke.

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Soyuer, Ferhan; Oztürk, Ahmet

2007-05-15

To study the effects of spasticity, sensory impairment, and type of walking aid on falls in community dwellers with chronic stroke. Functional Independence Measure (FIM) Instrument, Joint Position Sense Evaluation (JPS), the Rivermead motor assessment scale (RMA), Ashworth Scale, Tinetti Assessment Tool were used to assess 100 cases. Fifty-three of the cases were grouped as nonfallers, 36 as one-time fallers and 11 as repeat fallers. These 3 groups were found to be different from each other in respect to FIM, Tinetti test and RMA (p cane, 41.9% high cane). According to Ordinal logistic regression analysis, it was found that the possibility of fall increased (p fall of the individuals with stroke decreased (p falls, spasticity is also an indicator for chronic stroke patients, as is motor impairment, functional situation, impairment of balance and walking. Sensory impairment, using a walking aid and the type were found to be ineffective.

DNABIT Compress – Genome compression algorithm

Science.gov (United States)

Rajarajeswari, Pothuraju; Apparao, Allam

2011-01-01

Data compression is concerned with how information is organized in data. Efficient storage means removal of redundancy from the data being stored in the DNA molecule. Data compression algorithms remove redundancy and are used to understand biologically important molecules. We present a compression algorithm, “DNABIT Compress” for DNA sequences based on a novel algorithm of assigning binary bits for smaller segments of DNA bases to compress both repetitive and non repetitive DNA sequence. Our proposed algorithm achieves the best compression ratio for DNA sequences for larger genome. Significantly better compression results show that “DNABIT Compress” algorithm is the best among the remaining compression algorithms. While achieving the best compression ratios for DNA sequences (Genomes),our new DNABIT Compress algorithm significantly improves the running time of all previous DNA compression programs. Assigning binary bits (Unique BIT CODE) for (Exact Repeats, Reverse Repeats) fragments of DNA sequence is also a unique concept introduced in this algorithm for the first time in DNA compression. This proposed new algorithm could achieve the best compression ratio as much as 1.58 bits/bases where the existing best methods could not achieve a ratio less than 1.72 bits/bases. PMID:21383923

The ankle-foot orthosis improves balance and reduces fall risk of chronic spastic hemiparetic patients.

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Cakar, E; Durmus, O; Tekin, L; Dincer, U; Kiralp, M Z

2010-09-01

Ankle foot orthoses (AFO) are commonly used orthotic device in order to restore the ankle foot function and to improve the balance and gait in post-stroke hemiparetic patients. However, there remain some discussions about their effectiveness on long term hemiparetic patients who had mild to moderate spasticity. To investigate the relative effect of prefabricated thermoplastic posterior leaf spring AFO (PLS-AFO) on balance and fall risk. A cross-over interventional study The Department of PMR of a tertiary hospital. Twenty-five chronic post-stroke long duration hemiparetic patients who had Ashworth grade 1-2 spasticity at affected calf muscles and lower limb Brunnstrom stage 2-3 and also able to walk independently without an assistive device. Berg Balance Scale (BERG), and the postural stability test (PST) and the fall risk test (FRT) of Biodex balance systems were used for the assessments. All of the patients were assessed with AFO and without AFO. All assessments were made with footwear. The mean post-stroke duration was 20,32±7,46 months. The BERG scores were 42,12±9,05 without AFO and 47,52±7,77 with AFO; the overall stability scores of FRT were 3,35±1,97 without AFO and 2,69±1,65 with AFO (Pbalance and provide fall risk reduction in chronic post-stroke ambulatory hemiparetic patients who had mild to moderate spasticity on their affected lower limb. These results encourage the usage of AFO on long duration hemiparetic patients in order to provide better balance and lesser fall risk.

Assessment Impact of Foot Sensory Modulation on Inhibition of Hypertonicity of the Lower Limb in Children with Diplegia Spastic

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Saeed Fatoureh-Chi

2005-01-01

Full Text Available Objective: The purpose of this study was to assess impact of foot sensory modulation on inhibition of hypertonicity of the lower limb in children with diplegia spastic cerebral palsy. Materials & Methods: 24 selected children (aged 2.5 to 4.5 years were randomly assigned to a control and experimental groups. Muscle tone was assessed using modified Ashworth scale, passive Range of motion by goniameter (Pedretti, neurodevelopmental level by Bobath scale. All children were pre-post tested in an interval of ten weeks. Results: Significant reduction was observed in hypertonicity of hip extensor (p<0/1 and ankle planter flexor (P<0/05. Significant increase was observed in passive Range of motion of hip flexion (P<0/1, knee extension (P<0/05 and ankle dorsi flexion (P<0/05. There was found no significant difference of reduction in hypertonicity of knee flexor and improvement neurodevelopmental level. Meaningful relationship was observed between reduction hypertonicity of the hip extensor (P<0/05 and improvement of neurodevelopmental level (P<0/05. Conclusion: Impact of sensory modulation on children with diplegia spastic cerebral palsy reduces spasticity of lower limb and also extends joints domain of motion.

Autosomal recessive spastic paraplegia (SPG30) with mild ataxia and sensory neuropathy maps to chromosome 2q37.3.

Science.gov (United States)

Klebe, Stephan; Azzedine, Hamid; Durr, Alexandra; Bastien, Patrick; Bouslam, Naima; Elleuch, Nizar; Forlani, Sylvie; Charon, Celine; Koenig, Michel; Melki, Judith; Brice, Alexis; Stevanin, Giovanni

2006-06-01

The hereditary spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of neurodegenerative diseases characterized by progressive spasticity in the lower limbs. Twenty-nine different loci (SPG) have been mapped so far, and 11 responsible genes have been identified. Clinically, one distinguishes between pure and complex HSP forms which are variably associated with numerous combinations of neurological and extra-neurological signs. Less is known about autosomal recessive forms (ARHSP) since the mapped loci have been identified often in single families and account for only a small percentage of patients. We report a new ARHSP locus (SPG30) on chromosome 2q37.3 in a consanguineous family with seven unaffected and four affected members of Algerian origin living in Eastern France with a significant multipoint lod score of 3.8. Ten other families from France (n = 4), Tunisia (n = 2), Algeria (n = 3) and the Czech Republic (n = 1) were not linked to the newly identified locus thus demonstrating further genetic heterogeneity. The phenotype of the linked family consists of spastic paraparesis and peripheral neuropathy associated with slight cerebellar signs confirmed by cerebellar atrophy on one CT scan.

SPASTICITY PATTERNS OF HAND MUSCLES AND BOTULINUM TOXIN THERAPY APPLICATION IN PATIENTS WITH CEREBRAL PALSY WITH UPPER LIMB INVOLVEMENT

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O. A. Klochkova

2013-01-01

Full Text Available Botulinum toxin therapy is an effective and safe method of treatment of local spasticity in patients with cerebral palsy (CP. Calculation of botulinum toxin A (BTA dosage based on the spasticity patterns and functional capabilities of the patient proved effective for the hypertonic lower limb muscle spasm treatment and is being applied to BTA injections in hand muscles more often. The article presents contemporary scientific data and results of the original study of BTA injections efficacy for pathologic tension reduction in hand muscles of 52 patients with CP. The authors give detailed description of the upper limb spasticity patterns, their frequency and role in the pathological movement pattern formation. The authors propose BTA dosage calculation for the functional segments of upper limbs, which allows minimizing the total amount of the administered drug and avoiding excessive weakness. The authors have also conducted a follow-up analysis of changes in hand muscle tone for the period of 6 months after the first BTA injection, compared results of botulinum toxin therapy at various clinical forms of CP and given recommendations on the optimum duration of the follow-up period.

The effect of Bobath approach on the excitability of the spinal alpha motor neurones in stroke patients with muscle spasticity.

Science.gov (United States)

Ansari, N N; Naghdi, S

2007-01-01

A clinical study was performed to evaluate the efficacy of the Bobath approach on the excitability of the spinal alpha motor neurones in patients with poststroke spasticity. Ten subjects ranging in age from 37 through 76 years (average 60 years) with ankle plantarflexor spasticity secondary to a stroke were recruited and completed the trial. They had physiotherapy according to Bobath concept for ten treatment sessions, three days per week. Two repeated measures, one before and another after treatment, were taken to quantify clinical efficacy. The effect of this type of therapy on the excitability of alpha motor neurones (aMN) was assessed by measuring the latency of the Hoffmann reflex (H-reflex) and the Hmax/Mmax ratio. The original Ashworth scale and ankle range of motion were also measured. The mean HmaxlMmax ratio on the affected side at baseline was high in the study patients. However, there were no statistically significant differences in the HmaxlMmax ratio or in the H-reflex latency between the baseline values and those recorded after therapy intervention. Before treatment, the HmaxlMmax ratio was significantly higher in the affected side than in the unaffected side. However, it was similar at both sides after treatment. Following treatment, the significant reduction in spasticity was clinically detected as measured with the original Ashworth scale. The ankle joint active and passive range of motion was significantly increased. In conclusion, Bobath therapy had a statistically significant effect on the excitability of the aMN in the affected side compared to the unaffected side in stroke patients with muscle spasticity.

Reproducibility and validity of video screen measurements of gait in children with spastic cerebral palsy.

NARCIS (Netherlands)

Grunt, S.; van Kampen, P.M.; van der Krogt, M.M.; Brehm, M.A.; Doorenbosch, C.A.M.; Becher, J.G.

2010-01-01

Purpose: To determine the reproducibility and validity of video screen measurement (VSM) of sagittal plane joint angles during gait. Methods: 17 children with spastic cerebral palsy walked on a 10. m walkway. Videos were recorded and 3d-instrumented gait analysis was performed. Two investigators

Reproducibility and validity of video screen measurements of gait in children with spastic cerebral palsy

NARCIS (Netherlands)

Grunt, Sebastian; van Kampen, Petra J.; van der Krogt, Marjolein M.; Brehm, Merel-Anne; Doorenbosch, Caroline A. M.; Becher, Jules G.

2010-01-01

PURPOSE: To determine the reproducibility and validity of video screen measurement (VSM) of sagittal plane joint angles during gait. METHODS: 17 children with spastic cerebral palsy walked on a 10m walkway. Videos were recorded and 3d-instrumented gait analysis was performed. Two investigators

Epidemiological, humanistic, and economic burden of illness of lower limb spasticity in adults: a systematic review

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Martin A

2014-01-01

Full Text Available Alison Martin,1 Seye Abogunrin,1 Hannah Kurth,2 Jerome Dinet2 1Evidera, London, UK; 2Ipsen, Boulogne Billancourt Cedex, France Background: The purpose of this study was to investigate the epidemiological, humanistic, and economic burden of illness associated with adult lower limb spasticity (LLS and its complications. Methods: A systematic search of MEDLINE and EMBASE identified 23 studies published between January 2002 and October 2012 that assessed the epidemiology, impact, and resource use associated with LLS. A hand-search of four neurology conferences identified abstracts published between 2010 and 2012. Results: LLS was found to occur in one third of adults after stroke, half to two thirds with multiple sclerosis, and three quarters with cerebral palsy. LLS limits mobility and reduces quality of life. No clear association was found between LLS and occurrence of pain, development of contractures, or risk of falls. Conclusion: The evidence on the burden of LLS and its complications is surprisingly limited given the condition's high prevalence among adults with common disorders, such as stroke. Further research is needed to clarify the impact of LLS, including the likelihood of thrombosis in spastic lower limbs. The dearth of high-quality evidence for LLS suggests a lack of awareness of, and interest in, the problem, and therefore, the unmet need among patients and their carers. Keywords: muscle spasticity, cost of illness, complications, quality of life

IMPACT OF BODY WEIGHT SUPPORTED BACKWARD TREADMILL TRAINING ON WALKING SPEED IN CHILDREN WITH SPASTIC DIPLEGIA

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Hamada El Sayed Abd Allah Ayoub

2016-10-01

Full Text Available Background: A lot of the ambulating children with spastic diplegia were able to walk with flexed hips, knees and ankles this gait pattern is known as crouch gait. The most needed functional achievement of diplegic children habilitation is to be able to walk appropriately. The development of an independent and efficient walking is one of the main objectives for children with cerebral palsy especially those with spastic diplegia. Method: Twenty children with spastic diplegia enrolled in this study, they were classified into two groups of equal number, eligibility to our study were ages ranged from seven to ten years, were able to ambulate, They had gait problems and abnormal gait kinematics. The control group (A received selected physical therapy program based on neurodevelopmental approach for such cases, while the study group (B received partial body weight supported backward treadmill training in addition to regular exercise program. Gait pattern was assessed using the Biodex Gait Trainer II for each group pre and post three months of the treatment program. Results: There was statistically significant improvement in walking speed in the study group (P<0.05 with significant difference when comparing post treatment results between groups (p<0.05. Conclusion: These findings suggested that partial body weight supported backward treadmill training can be included as a supplementary therapeutic modality to improve walking speed and functional abilities of children with diplegic cerebral palsy.

Cerebral metabolic and structural alterations in hereditary spastic paraplegia with thin corpus callosum assessed by MRS and DTI

International Nuclear Information System (INIS)

Dreha-Kulaczewski, Steffi; Dechent, Peter; Helms, Gunther; Frahm, Jens; Gaertner, Jutta; Brockmann, Knut

2006-01-01

Hereditary spastic paraplegia with thin corpus callosum (HSP-TCC) is a complicated form of autosomal-recessive hereditary spastic paraplegia. Characteristic clinical features comprise progressive spastic gait, cognitive impairment, and ataxia. Diagnostic MRI findings include thinning of the corpus callosum and non-progressive white matter (WM) alterations. To study the extent of axonal involvement, we performed localized proton magnetic resonance spectroscopy (MRS) of the cerebral WM and cortical grey matter (GM) in a patient with HSP-TCC at 20 and 25 years of age. The second investigation included diffusion tensor imaging (DTI). While MRS of the GM was normal, affected WM was characterized by major metabolic alterations such as reduced concentrations of N-acetylaspartate and N-acetylaspartyl-glutamate, creatine and phosphocreatine, and choline-containing compounds as well as elevated levels of myo-inositol. These abnormalities showed progression over a period of 5 years. DTI revealed increased mean diffusivity as well as reduced fractional anisotropy in periventricular WM. The metabolic and structural findings are consistent with progressive neuroaxonal loss in the WM accompanied by astrocytic proliferation - histopathological changes known to occur in HSP-TCC. Our results are in agreement with the hypothesis that the primary pathological process in HSP-TCC affects the axon, possibly due to impaired axonal trafficking. (orig.)

[Intrathecal baclofen therapy for spastic paraparesis due to aortic dissecting aneurysm; recent progress in treatment strategy].

Science.gov (United States)

Nakajima, T; Akagawa, H; Ochiai, T; Hayashi, M; Goto, S; Taira, T; Okada, Y

2009-11-01

A 48-year-old man suffered from acute dissection of thoracic aortic aneurysm which eventually led to replacement of the ascending aorta with a tube graft. During this clinical course, circulatory failure in intercostal artery resulted in spinal cord infarction followed by moto-sensory disturbance below Th7 dermatomic area. Seven months later, spasticity with pain in both lower extremities became conspicuous that was uncontrollable by any oral medication. Eventually the patient underwent the implantation of continuous infusion pump for intrathecal baclofen therapy (ITB). The clinical condition was remarkably improved and now has been well controlled. ITB, authorized by Japanese Ministry of Health Labour and Welfare in 2006, has notable therapeutic effects on spasticity derived from any sort of central nervous disorder. More promotive enlightenment if ITB is indispensable for enhancement of its medical benefit in Japan.

Palmo-Plantar hyperkeratosis, intellectual disability, and spastic paraplegia in two maternal half brothers: further evidence for an X-linked inheritance.

Science.gov (United States)

Isidor, Bertrand; Lefebvre, Tiphaine; Barbarot, Sébastien; Perrier, Julie; Mercier, Sandra; Péréon, Yann; Le Caignec, Cédric; David, Albert

2013-06-01

In 1983, Fitzsimmons et al. reported four brothers with an unrecognized disorder characterized by intellectual disability, spastic paraplegia, and palmo-plantar hyperkeratosis (OMIM 309500). In this report, we describe a family in which two males, maternal half-brothers, had learning disabilities. Both patients also showed spasticity in the lower limbs and palmo-plantar hyperkeratosis. The mother of the affected boys had learning difficulties but did not show any dermatological symptoms. This report confirms that the association of features reported by Fitzsimmons et al. is a distinct entity and further suggests an X-linked mode of inheritance. Copyright © 2013 Wiley Periodicals, Inc.

Compound heterozygous mutations in two different domains of ALDH18A1 do not affect the amino acid levels in a patient with hereditary spastic paraplegia

DEFF Research Database (Denmark)

Steenhof, Maria; Kibæk, Maria; Larsen, Martin J.

2018-01-01

with mutations affecting the GR5P domain. We present a 19-year old male patient with autosomal recessive spastic paraplegia and compound heterozygosity for two ALDH18A1 mutations, one in each of the P5CS domains. This young man has spastic paraplegia with onset in childhood and temporal lobe epilepsy, but normal...

BoNT-A related changes of cortical activity in patients suffering from severe hand paralysis with arm spasticity following ischemic stroke.

Science.gov (United States)

Veverka, Tomáš; Hluštík, Petr; Tomášová, Zuzana; Hok, Pavel; Otruba, Pavel; Král, Michal; Tüdös, Zbyněk; Zapletalová, Jana; Herzig, Roman; Krobot, Alois; Kaňovský, Petr

2012-08-15

Investigations were performed to localize and analyze the botulinum toxin (BoNT-A) related changes of cerebral cortex activation in chronic stroke patients suffering from severe hand paralysis with arm spasticity. Effects on task- related cerebral activation were evaluated by functional magnetic resonance imaging (fMRI). 14 patients (5 males, 9 females, mean age 55.3 years) suffering from upper limb post-stroke spasticity were investigated. The change of arm spasticity was assessed by using the modified Ashworth scale (MAS). FMRI sessions were performed before (W0), four weeks (W4) and 11 weeks (W11) after BoNT-A application. Patients were scanned while performing imaginary movement with the impaired hand. Group fMRI analysis included patient age as a covariate. BoNT-A treatment was effective in alleviation of arm spasticity. Mean MAS was at Week 0: 2.5 (SD 0.53), at Week 4: 1.45 (SD 0.38), at Week 11: 2.32 (SD 0.44). Task-related fMRI prior to the treatment showed extensive activation of bilateral frontoparietal sensorimotor cortical areas, anterior cingulate gyrus, pallidum, thalamus and cerebellum. Effective BoNT-A treatment (W4) resulted in partial reduction of active network volume in most of the observed areas, whereas BoNT-free data (W11) revealed further volume reduction in the sensorimotor network. On direct comparison, significant activation decreases associated with BoNT-A treatment were located in areas outside the classical sensorimotor system, namely, ipsilesional lateral occipital cortex, supramarginal gyrus and precuneus cortex. On comparison of W4 and W11, no activation increases were found, instead, activation further decreased in ipsilesional insular cortex, contralesional superior frontal gyrus and bilateral frontal pole. Whole brain activation patterns during BoNT-A treatment of post-stroke arm spasticity and further follow up document predominantly gradual changes both within and outside the classical sensorimotor system. Copyright © 2012

Spinal cord edema with contrast enhancement mimicking intramedullary tumor in patient with cervical myelopathy: A case report and a brief literature review.

Science.gov (United States)

Gkasdaris, Grigorios; Chourmouzi, Danai; Karagiannidis, Apostolos; Kapetanakis, Stylianos

2017-01-01

Cervical myelopathy (CM) is a clinical diagnosis that may be associated with hyperintense areas on T2-weighted magnetic resonance imaging (MRI) scan. The use of contrast enhancement in such areas to differentiate between neoplastic and degenerative disease has rarely been described. We present a 41-year-old female with a 5-month course of progressive CM. The cervical MRI revealed spinal cord swelling, stenosis, and a hyperintense signal at the C5-C6 and C5-C7 levels. Both the neurologic and radiologic examinations were consistent with an intramedullary cervical cord tumor. To decompress the spinal canal, an anterior cervical discectomy and fusion was performed from C5 to C7 level. This resulted in immediate and significant improvement of the myelopathy. Postoperatively, over 1.5 years, the hyperintense, enhancing intramedullary lesion gradually regressed on multiple postoperative MRI scans. Spinal cord edema is occasionally seen on MR studies of the cervical spine in patients with degenerative CM. Contrast-enhanced MR studies may help differentiate hyperintense cord signals due to edema vs. atypical intramedullary tumors. Routine successive postoperative MRI evaluations are crucial to confirm the diagnosis of degenerative vs. neoplastic disease.

Severe Spastic Contractures and Diabetes Mellitus Independently Predict Subsequent Minimal Trauma Fractures Among Long-Term Care Residents.

Science.gov (United States)

Lam, Kuen; Leung, Man Fuk; Kwan, Chi Wai; Kwan, Joseph

2016-11-01

The study aimed to examine the epidemiology of hypertonic contractures and its relationship with minimal trauma fracture (MTF), and to determine the incidence and predictors of (MTF) in long-term care residents. This was a longitudinal cohort study of prospectively collected data. Participants were followed from March 2007 to March 2016 or until death. A 300-bed long-term care hospital in Hong Kong. All long-term care residents who were in need of continuous medical and nursing care for their activities of daily living. Information on patients' demographic data, severe contracture defined as a decrease of 50% or more of the normal passive range of joint movement of the joint, and severe limb spasticity defined by the Modified Ashworth Scale higher than grade 3, medical comorbidities, functional status, cognitive status, nutritional status including body mass index and serum albumin, past history of fractures, were evaluated as potential risk factors for subsequent MTF. Three hundred ninety-six residents [148 males, mean ± standard deviation (SD), age = 79 ± 16 years] were included for analysis. The presence of severe contracture was highly prevalent among the study population: 91% of residents had at least 1 severe contracture, and 41% of residents had severe contractures involving all 4 limbs. Moreover, there were a significant proportion of residents who had severe limb spasticity with the elbow flexors (32.4%) and knee flexors (33.9%) being the most commonly involved muscles. Twelve residents (3%) suffered from subsequent MTF over a median follow-up of 33 (SD = 30) months. Seven out of these 12 residents died during the follow-up period, with a mean survival of 17.8 months (SD = 12.6) after the fracture event. The following 2 factors were found to independently predict subsequent MTF in a multivariate Cox regression: bilateral severe spastic knee contractures (hazard ratio = 16.5, P contractures are common morbidities in long-term care residents

Surgical clipping of flow related giant unruptured anterior spinal artery aneurysm secondary to co-arctation of aorta: Management challenges and lessons learnt

Directory of Open Access Journals (Sweden)

Jeena Joseph

2017-12-01

Conclusion: ASAA remains an extremely rare cause of compressive myelopathy. Its association with co-arctation of aorta increases treatment related mortality. Posterolateral approach provides a good exposure of the aneurysm for surgical clipping with minimal retraction of the cord.

Revisiting genotype-phenotype overlap in neurogenetics: triplet-repeat expansions mimicking spastic paraplegias.

Science.gov (United States)

Bettencourt, Conceição; Quintáns, Beatriz; Ros, Raquel; Ampuero, Israel; Yáñez, Zuleima; Pascual, Samuel Ignacio; de Yébenes, Justo García; Sobrido, María-Jesús

2012-09-01

Hereditary spastic paraplegias (HSPs) constitute a heterogeneous group of neurological disorders, characterized primarily by progressive spasticity and weakness of the lower limbs. HSPs are caused by mutations in multiple genes (at least 48 loci and 28 causative genes). The clinical spectrum of HSPs is wide and important differences have been reported between patients with distinct mutations in the same gene, or even between different family members bearing the same mutation. Many patients with HSP present clinical deficits related to the involvement of neuronal systems other than corticospinal tracts, namely, peripheral nerves, sensory, or cerebellar pathways. These cases may be difficult to differentiate from other neurological diseases (e.g., hereditary ataxias), also genetically and clinically heterogeneous. As an illustration of how overlapping this genotype-phenotype relationship is, and the difficulties that it brings upon the development of neurogenetic algorithms and databases, we review the main clinical and genetic features of HSPs, and summarize reports on cases of triplet-repeat spinocerebellar ataxias that can mimic HSP phenotypes. This complex scenario makes the necessity of high-quality, curated mutation databases even more urgent, in order to develop adequate diagnostic guidelines, correct interpretation of genetic testing, and appropriate genetic counseling. © 2012 Wiley Periodicals, Inc.

COMPARISON BETWEEN PHYSIOLOGICAL COST INDEX IN HEALTHY NORMAL CHILDREN AS AGAINST AMBULATORY SPASTIC DIPLEGIC CEREBRAL PALSY (WITH AND WITHOUT ORTHOSIS IN THE AGE GROUP 6 TO 18 YEARS

Directory of Open Access Journals (Sweden)

Swatia Bhise

2016-08-01

Full Text Available Background: Efficacy of rehabilitation program for subjects with orthosis with objective measurement. The study aiming to objectively compare the PCI and walking speed of normal children with ambulatory spastic diaplegic. Also we aimed to analyze whether BMIhad impact on energy cost. Methods: 41 normal children and 41 community walking spastic diaplegic aged between 6 to 18 yrs. were assessed to compare the PCI. Speed of walking and heart rate were checked constantlyboth barefoot and in shoes in normal children and with and without conventional AFO in children with spastic diaplegic at their chosen velocities over four consecutive lengths of a 12.5m walkway i.e. total 50m.,Pre and Post readings are taken. Heart rate is affected by speed; PCI with speed of walking and heart rate was calculated for each child. Results: The mean PCI in shoes and barefoot was same in normal children i.e. 0.05 ±0.039beats/meter. The PCI for children with pathological gait i.e. spastic diaplegic without orthosis and with orthosis is 0.199 ±0.176 and 0.104± 0.093beats/meter appreciably greater than that for normal children(p less than 0.05. Conclusion: This study showed that walking with orthosis in spastic diplegic CP children showed higher costs of energy and slower walking speed compared normal children with age matched. The PCI of walking, with orthosis in children with spastic Diplegic cerebral palsy is less as compared to without orthosis i.e. gait is more energy efficient with orthosis. BMI doesn’t show any correlation with PCI further study may require.

Recovery of neuronal and network excitability after spinal cord injury and implications for spasticity

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Jessica Maria D'Amico

2014-05-01

Full Text Available The state of areflexia and muscle weakness that immediately follows a spinal cord injury is gradually replaced by the recovery of neuronal and network excitability, leading to both improvements in residual motor function and the development of spasticity. In this review we summarize recent animal and human studies that describe how motoneurons and their activation by sensory pathways become hyperexcitable to compensate for the reduction of descending and movement-induced sensory inputs and the eventual impact on the muscle. We discuss how replacing lost patterned activation of the spinal cord by activating synaptic inputs via assisted movements, pharmacology or electrical stimulation may help to recover lost spinal inhibition. This may lead to a reduction of uncontrolled activation of the spinal cord and thus, improve its controlled activation by synaptic inputs to ultimately normalize circuit function. Increasing the excitation of the spinal cord below an injury with spared descending and/or peripheral functional synaptic activation, instead of suppressing it pharmacologically, may provide the best avenue to improve residual motor function and manage spasticity after spinal cord injury.

Passive muscle properties are altered in children with cerebral palsy before the age of 3 years and are difficult to distinguish clinically from spasticity

DEFF Research Database (Denmark)

Willerslev-Olsen, Maria; Lorentzen, Jakob; Sinkjær, Thomas

2013-01-01

AIM: Clinical determination of spasticity is confounded by the difficulty in distinguishing reflex from passive contributions to muscle stiffness. There is, therefore, a risk that children with cerebral palsy (CP) receive antispasticity treatment unnecessarily. To investigate this, we aimed...... to determine the contribution of reflex mechanisms to changes in the passive elastic properties of muscles and tendons in children with CP. METHOD: Biomechanical and electrophysiological measures were used to determine the relative contribution of reflex and passive mechanisms to ankle muscle stiffness in 35...... tetraplegia. According to the Gross Motor Function Classification System, 31 children were classified in level I, two in level II, and two in level III. RESULTS: Only seven children with spastic CP showed reflex stiffness outside the range of the control children. In contrast, 20 children with spastic CP...

Best seating condition in children with spastic cerebral palsy : One type does not fit all

NARCIS (Netherlands)

Angsupaisal, Mattana; Dijkstra, Linze-Jaap; la Bastide-van Gemert, Sacha; van Hoorn, Jessika F.; Burger, Karine; Maathuis, Carel G. B.; Hadders-Algra, Mijna

2017-01-01

Background: The effect of forward-tilting of the seat surface and foot-support in children with spastic cerebral palsy (CP) is debated. Aim: To assess the effect of forward-tilting of the seat surface and foot-support in children with CP on kinematic head stability and reaching. Methods: Nineteen

Restoration of Upper Limb Function in an Individual with Cervical Spondylotic Myelopathy using Functional Electrical Stimulation Therapy: A Case Study

OpenAIRE

Popovic, Milos R.; Zivanovic, Vera; Valiante, Taufik A.

2016-01-01

Non-traumatic spinal cord pathology is responsible for 25–52% of all spinal cord lesions. Studies have revealed that spinal stenosis accounts for 16–21% of spinal cord injury (SCI) admissions. Impaired grips as well as slow unskilled hand and finger movements are the most common complaints in patients with spinal cord disorders, such as myelopathy secondary to cervical spondylosis. In the past, our team carried out couple of successful clinical trials, including two randomized control trials,...

The effect and complication of botulinum toxin type a injection with serial casting for the treatment of spastic equinus foot.

Science.gov (United States)

Lee, Sook Joung; Sung, In Young; Jang, Dae Hyun; Yi, Jin Hwa; Lee, Jin Ho; Ryu, Ju Seok

2011-06-01

To identify the effect of serial casting combined with Botulinum toxin type A (BTX-A) injection on spastic equinus foot. Twenty-nine children with cerebral palsy who had equinus foot were recruited from the outpatient clinic of Rehabilitation Medicine. The children were divided into 2 groups, one of which received serial casting after BTX-A injection, and the other which only received BTX-A injection. Serial casting started 3 weeks after the BTX-A injection, and was changed weekly for 3 times. Spasticity of the ankle joint was evaluated using the modified Ashworth scale (MAS), and the modified Tardieu scale (MTS). Gait pattern was measured using the physician's rating scale (PRS). The degree of ankle dorsiflexion and the MAS improved significantly until 12 weeks following the BTX-A injection in the serial casting group (pcasting. Our study demonstrated that the effect of BTX-A injection with serial casting was superior and lasted longer than the effect of BTX-A injection only in patients with spastic equinus foot. We therefore recommend BTX-A injection with serial casting for the treatment of equinus foot. However, physicians must also consider the possible complications associated with serial casting.

Novel application of a Wii remote to measure spasticity with the pendulum test: Proof of concept.

Science.gov (United States)

Yeh, Chien-Hung; Hung, Chi-Yao; Wang, Yung-Hung; Hsu, Wei-Tai; Chang, Yi-Chung; Yeh, Jia-Rong; Lee, Po-Lei; Hu, Kun; Kang, Jiunn-Horng; Lo, Men-Tzung

2016-01-01

The pendulum test is a standard clinical test for quantifying the severity of spasticity. In the test, an electrogoniometer is typically used to measure the knee angular motion. The device is costly and difficult to set up such that the pendulum test is normally time consuming. The goal of this study is to determine whether a Nintendo Wii remote can replace the electrogroniometer for reliable assessment of the angular motion of the knee in the pendulum test. The pendulum test was performed in three control participants and 13 hemiplegic stroke patients using both a Wii remote and an electrogoniometer. The correlation coefficient and the Bland-Altman difference plot were used to compare the results obtained from the two devices. The Wilcoxon signed-rank test was used to compare the difference between hemiplegia-affected and nonaffected sides in the hemiplegic stroke patients. There was a fair to strong correlation between measurements from the Wii remote and the electrogoniometer (0.513spastic) side from the nonaffected (nonspastic) side (both with p<.0001*). In addition, the intraclass correlation coefficient, standard error of measurement, and minimum detectable differences were highly consistent for both devices. Our findings suggest that the Wii remote may serve as a convenient and cost-efficient tool for the assessment of spasticity. Copyright © 2015 Elsevier B.V. All rights reserved.

Characteristics of spondylotic myelopathy on 3D driven-equilibrium fast spin echo and 2D fast spin echo magnetic resonance imaging: a retrospective cross-sectional study.

Science.gov (United States)

Abdulhadi, Mike A; Perno, Joseph R; Melhem, Elias R; Nucifora, Paolo G P

2014-01-01

In patients with spinal stenosis, magnetic resonance imaging of the cervical spine can be improved by using 3D driven-equilibrium fast spin echo sequences to provide a high-resolution assessment of osseous and ligamentous structures. However, it is not yet clear whether 3D driven-equilibrium fast spin echo sequences adequately evaluate the spinal cord itself. As a result, they are generally supplemented by additional 2D fast spin echo sequences, adding time to the examination and potential discomfort to the patient. Here we investigate the hypothesis that in patients with spinal stenosis and spondylotic myelopathy, 3D driven-equilibrium fast spin echo sequences can characterize cord lesions equally well as 2D fast spin echo sequences. We performed a retrospective analysis of 30 adult patients with spondylotic myelopathy who had been examined with both 3D driven-equilibrium fast spin echo sequences and 2D fast spin echo sequences at the same scanning session. The two sequences were inspected separately for each patient, and visible cord lesions were manually traced. We found no significant differences between 3D driven-equilibrium fast spin echo and 2D fast spin echo sequences in the mean number, mean area, or mean transverse dimensions of spondylotic cord lesions. Nevertheless, the mean contrast-to-noise ratio of cord lesions was decreased on 3D driven-equilibrium fast spin echo sequences compared to 2D fast spin echo sequences. These findings suggest that 3D driven-equilibrium fast spin echo sequences do not need supplemental 2D fast spin echo sequences for the diagnosis of spondylotic myelopathy, but they may be less well suited for quantitative signal measurements in the spinal cord.

The effect of operant-conditioning balance training on the down-regulation of spinal H-reflexes in a spastic patient.

Science.gov (United States)

Hoseini, Najmeh; Koceja, David M; Riley, Zachary A

2011-10-24

Spasticity in chronic hemiparetic stroke patients has primarily been treated pharmacologically. However, there is increasing evidence that physical rehabilitation can help manage hyper-excitability of reflexes (hyperreflexia), which is a primary contributor to spasticity. In the present study, one chronic hemiparetic stroke patient operantly conditioned the soleus H-reflex while training on a balance board for two weeks. The results showed a minimal decrease in the Hmax-Mmax ratio for both the affected and unaffected limb, indicating that the H-reflex was not significantly altered with training. Alternatively, paired-reflex depression (PRD), a measure of history-dependent changes in reflex excitability, could be conditioned. This was evident by the rightward shift and decreased slope of reflex excitability in the affected limb. The non-affected limb decreased as well, although the non-affected limb was very sensitive to PRD initially, whereas the affected limb was not. Based on these results, it was concluded that PRD is a better index of hyperreflexia, and this measurement could be more informative of synapse function than simple H-reflexes. This study presents a novel and non-pharmacological means of managing spasticity that warrants further investigation with the potential of being translated to the clinic. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Classification of upper limb disability levels of children with spastic unilateral cerebral palsy using K-means algorithm.

Science.gov (United States)

Raouafi, Sana; Achiche, Sofiane; Begon, Mickael; Sarcher, Aurélie; Raison, Maxime

2018-01-01

Treatment for cerebral palsy depends upon the severity of the child's condition and requires knowledge about upper limb disability. The aim of this study was to develop a systematic quantitative classification method of the upper limb disability levels for children with spastic unilateral cerebral palsy based on upper limb movements and muscle activation. Thirteen children with spastic unilateral cerebral palsy and six typically developing children participated in this study. Patients were matched on age and manual ability classification system levels I to III. Twenty-three kinematic and electromyographic variables were collected from two tasks. Discriminative analysis and K-means clustering algorithm were applied using 23 kinematic and EMG variables of each participant. Among the 23 kinematic and electromyographic variables, only two variables containing the most relevant information for the prediction of the four levels of severity of spastic unilateral cerebral palsy, which are fixed by manual ability classification system, were identified by discriminant analysis: (1) the Falconer index (CAI E ) which represents the ratio of biceps to triceps brachii activity during extension and (2) the maximal angle extension (θ Extension,max ). A good correlation (Kendall Rank correlation coefficient = -0.53, p = 0.01) was found between levels fixed by manual ability classification system and the obtained classes. These findings suggest that the cost and effort needed to assess and characterize the disability level of a child can be further reduced.

The effect of Sub-maximal exercise-rehabilitation program on cardio-respiratory endurance indexes and oxygen pulse in patients with spastic cerebral palsy

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M Izadi

2006-05-01

Full Text Available Background: Physical or cardio-respiratory fitness are of the best important physiological variables in children with cerebral palsy (CP, but the researches on exercise response of individuals with CP are limited. Our aim was to determine the effect of sub-maximal rehabilitation program (aerobic exercise on maximal oxygen uptake, oxygen pulse and cardio- respiratory physiological variables of children with moderate to severe spastic cerebral palsy diplegia and compare with able-bodied children. Methods: In a controlled clinical trial study, 15 children with diplegia spastic cerebral palsy, were recruited on a voluntarily basis (experimental group and 18 subjects without neurological impairments selected as control group. In CP group, aerobic exercise program performed on the average of exercise intensity (144 beat per minute of heart rate, 3 times a week for 3 months. The time of each exercise session was 20-25 minutes. Dependent variables were measured in before (pretest and after (post test of rehabilitation program through Mac Master Protocol on Tantories cycle ergometer in CP group and compared with the control group. Results: The oxygen pulse (VO2/HR during ergometery protocol was significantly lower in CP group than normal group (P<0.05. No significant statistical difference in maximal oxygen uptake (VO2 max was found between groups. The rehabilitation program leads to little increase of this variable in CP group. After sub-maximal exercise in pretest and post test, the heart rate of patient group was greater than control group, and aerobic exercise leads to significant decrease in heart rate in CP patients(P<0.05. Conclusion: The patients with spastic cerebral palsy, because of high muscle tone, severe spasticity and involuntarily movements have higher energy cost and lower aerobic fitness than normal people. The rehabilitation exercise program can improve physiological function of muscle and cardio-respiratory endurance in these

The early markers for later dyskinetic cerebral palsy are different from those for spastic cerebral palsy

NARCIS (Netherlands)

Einspieler, C; Cioni, G; Paolicelli, PB; Bos, AF; Dressler, A; Ferrari, F; Roversi, MF; Prechtl, HFR

Qualitative abnormalities of spontaneous motor activity in new-borns and young infants are early predictive markers for later spastic cerebral palsy. Aim of this research was to identify which motor patterns may be specific for later dyskinetic cerebral palsy. In a large, prospectively performed

Efficacy of intrathecal baclofen delivery in the management of severe spasticity in upper motor neuron syndrome

NARCIS (Netherlands)

Rietman, Johan Swanik; Geertzen, J.H.B.

In the treatment of patients with severe spasticity, intrathecal administration of baclofen (ITB) was introduced in order to exert its effect directly at the receptor sites in the spinal cord, and have better therapeutic efficacy with smaller drug doses compared to oral antispasmodic medications.

Spasticity improvement in patients with relapsing-remitting multiple sclerosis switching from interferon-β to glatiramer acetate: the Escala Study.

Science.gov (United States)

Meca-Lallana, J E; Balseiro, J J; Lacruz, F; Guijarro, C; Sanchez, O; Cano, A; Costa-Frossard, L; Hernández-Clares, R; Sanchez-de la Rosa, R

2012-04-15

A recent pilot study suggested spasticity improvement during glatiramer acetate (GA) treatment in multiple sclerosis (MS) patients who previously received interferon-β (IFN-β). To evaluate changes in spasticity in MS patients switching from IFN-β to GA. Observational, multicentre study in patients with relapsing-remitting MS (RRMS) and spasticity switching from IFN-β to GA. The primary endpoint comprised changes on Penn Spasm Frequency Scale (PSFS), Modified Ashworth Scale (MAS), Adductor Tone Rating Scale (ATRS), and Global Pain Score (GPS) at months 3 and 6 after starting GA. Sixty-eight evaluable patients were included (mean age,41.7±9.5 years; female,70.6%; mean time from MS diagnosis to starting GA,7.6±5.7 years). Previous treatments were subcutaneous IFN-β1a in 42.6% patients, intramuscular IFN-β1a in 41.2% and IFN-β1b in 32.4%, whose mean durations were 3.5±3.3, 2.7±2.5 and 4.4±3.6 years, respectively. Statistically significant reductions in mean scores on all spasticity measurements were observed from baseline to month 3 (PSFS, 1.7±0.9 vs 1.4±0.6, p<0.01; MAS, 0.7±0.5 vs 0.6±0.5, p<0.01; highest MAS score, 1.9±0.8 vs 1.7±0.8, p<0.01; ATRS, 1.6±0.6 vs 1.4±0.6, p<0.01; GPS, 29.4±22.1 vs 24.7±19.4, p<0.01) and from baseline to month 6 (PSFS, 1.7±0.9 vs 1.3±0.6, p<0.01; MAS, 0.7±0.5 vs 0.5±0.5, p<0.01; highest MAS score, 1.9±0.8 vs 1.5±0.9, p<0.01; ATRS, 1.6±0.6 vs 1.3±0.6, p<0.01; GPS, 29.4±22.1 vs 19.1±14.8, p<0.01). Spasticity improvement in terms of spasm frequency, muscle tone and pain can be noted after three months and prolonged for six months of GA treatment. Copyright © 2011 Elsevier B.V. All rights reserved.

Indication for the operative methods in surgical treatment of cervical spondylotic myelopathy

International Nuclear Information System (INIS)

Kobayashi, Akira; Inoue, Shunichi; Watabe, Tsuneo; Nagase, Joji; Harada, Yoshitada

1984-01-01

Indication for the operative methods for cervical spondylotic myelopathy was examined in 16 patients undergoing CT-myelography before and after operation. There was a highly significant correlation between the anteroposterior (A-P) diameter of the spinal cord and clinical symptoms. Patients with shorter A-P diameter of the spinal cord tended to have severer preoperative clinical symptoms. Clinical symptoms improved as the post operative A-P diameter of the spinal cord increased. Fixation with decompression of the anterior spinal cord should be indicated when constriction of 5 mm or less of the spinal cord is seen segmentally at the level of the intervertebral disc. Dilation of the spinal cavity should be indicated when the constriction of the spinal cord is 5 mm or less at all levels of the cervical spinal cord. In performing fixation with anterior decompression, 15 mm is considered to be the most suitable width for complete and safe decompression of the flattened spinal cord with a wide transverse diameter. (Namekawa, K)

The natural history and clinical syndromes of degenerative cervical spondylosis.

LENUS (Irish Health Repository)

Kelly, John C

2012-01-01

Cervical spondylosis is a broad term which describes the age related chronic disc degeneration, which can also affect the cervical vertebrae, the facet and other joints and their associated soft tissue supports. Evidence of spondylitic change is frequently found in many asymptomatic adults. Radiculopathy is a result of intervertebral foramina narrowing. Narrowing of the spinal canal can result in spinal cord compression, ultimately resulting in cervical spondylosis myelopathy. This review article examines the current literature in relation to the cervical spondylosis and describes the three clinical syndromes of axial neck pain, cervical radiculopathy and cervical myelopathy.

Benefits of Repetitive Transcranial Magnetic Stimulation (rTMS for Spastic Subjects: Clinical, Functional, and Biomechanical Parameters for Lower Limb and Walking in Five Hemiparetic Patients

Directory of Open Access Journals (Sweden)

Luc Terreaux

2014-01-01

Full Text Available Introduction. Spasticity is a disabling symptom resulting from reorganization of spinal reflexes no longer inhibited by supraspinal control. Several studies have demonstrated interest in repetitive transcranial magnetic stimulation in spastic patients. We conducted a prospective, randomized, double-blind crossover study on five spastic hemiparetic patients to determine whether this type of stimulation of the premotor cortex can provide a clinical benefit. Material and Methods. Two stimulation frequencies (1 Hz and 10 Hz were tested versus placebo. Patients were assessed clinically, by quantitative analysis of walking and measurement of neuromechanical parameters (H and T reflexes, musculoarticular stiffness of the ankle. Results. No change was observed after placebo and 10 Hz protocols. Clinical parameters were not significantly modified after 1 Hz stimulation, apart from a tendency towards improved recruitment of antagonist muscles on the Fügl-Meyer scale. Only cadence and recurvatum were significantly modified on quantitative analysis of walking. Neuromechanical parameters were modified with significant decreases in Hmax⁡ /Mmax⁡ and T/Mmax⁡ ratios and stiffness indices 9 days or 31 days after initiation of TMS. Conclusion. This preliminary study supports the efficacy of low-frequency TMS to reduce reflex excitability and stiffness of ankle plantar flexors, while clinical signs of spasticity were not significantly modified.

Benefits of repetitive transcranial magnetic stimulation (rTMS) for spastic subjects: clinical, functional, and biomechanical parameters for lower limb and walking in five hemiparetic patients.

Science.gov (United States)

Terreaux, Luc; Gross, Raphael; Leboeuf, Fabien; Desal, Hubert; Hamel, Olivier; Nguyen, Jean Paul; Pérot, Chantal; Buffenoir, Kévin

2014-01-01

Introduction. Spasticity is a disabling symptom resulting from reorganization of spinal reflexes no longer inhibited by supraspinal control. Several studies have demonstrated interest in repetitive transcranial magnetic stimulation in spastic patients. We conducted a prospective, randomized, double-blind crossover study on five spastic hemiparetic patients to determine whether this type of stimulation of the premotor cortex can provide a clinical benefit. Material and Methods. Two stimulation frequencies (1 Hz and 10 Hz) were tested versus placebo. Patients were assessed clinically, by quantitative analysis of walking and measurement of neuromechanical parameters (H and T reflexes, musculoarticular stiffness of the ankle). Results. No change was observed after placebo and 10 Hz protocols. Clinical parameters were not significantly modified after 1 Hz stimulation, apart from a tendency towards improved recruitment of antagonist muscles on the Fügl-Meyer scale. Only cadence and recurvatum were significantly modified on quantitative analysis of walking. Neuromechanical parameters were modified with significant decreases in H max⁡ /M max⁡ and T/M max⁡ ratios and stiffness indices 9 days or 31 days after initiation of TMS. Conclusion. This preliminary study supports the efficacy of low-frequency TMS to reduce reflex excitability and stiffness of ankle plantar flexors, while clinical signs of spasticity were not significantly modified.

Proximal femoral resection and articulated hip distraction with an external fixator for the treatment of painful spastic hip dislocations in pediatric patients with spastic quadriplegia.

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Lampropulos, Mario; Puigdevall, Miguel H; Zapozko, Daniel; Malvárez, Héctor R

2008-01-01

We describe the results obtained with an alternative method of treatment for spastic painful hip dislocations in nonambulatory patients, which consists of a proximal femoral resection with capsular interposition arthroplasty, and the addition of a hinged external fixator for postoperative articulated hip distraction to allow for an immediate upright position and the ability to sit in a wheelchair. We performed this technique in three patients (four hips) with a mean age at the time of surgery of 15 years. Postoperatively, clinical improvement was observed in all four hips, with respect to pain relief, sitting tolerance, perineal care and functional range of motion.

MRI of autosomal dominant pure spastic paraplegia

DEFF Research Database (Denmark)

Krabbe, K.; Nielsen, J.E.; Fallentin, E.

1997-01-01

We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus...... callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a "corpus-callosum index" expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord...... at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and "corpus-callosum index" than controls. This finding, not reported previously...

Rapidly deteriorating course in Dutch hereditary spastic paraplegia type 11 patients

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de Bot, Susanne T; Burggraaff, Rogier C; Herkert, Johanna C; Schelhaas, Helenius J; Post, Bart; Diekstra, Adinda; van Vliet, Reinout O; van der Knaap, Marjo S; Kamsteeg, Erik-Jan; Scheffer, Hans; van de Warrenburg, Bart P; Verschuuren-Bemelmans, Corien C; Kremer, Hubertus PH

2013-01-01

Although SPG11 is the most common complicated hereditary spastic paraplegia, our knowledge of the long-term prognosis and life expectancy is limited. We therefore studied the disease course of all patients with a proven SPG11 mutation as tested in our laboratory, the single Dutch laboratory providing SPG11 mutation analysis, between 1 January 2009 and 1 January 2011. We identified nine different SPG11 mutations, four of which are novel, in nine index patients. Eighteen SPG11 patients from these nine families were studied by means of a retrospective chart analysis and additional interview/examination. Ages at onset were between 4 months and 14 years; 39% started with learning difficulties rather than gait impairment. Brain magnetic resonance imaging showed a thin corpus callosum and typical periventricular white matter changes in the frontal horn region (known as the ‘ears-of the lynx'-sign) in all. Most patients became wheelchair bound after a disease duration of 1 to 2 decades. End-stage disease consisted of loss of spontaneous speech, severe dysphagia, spastic tetraplegia with peripheral nerve involvement and contractures. Several patients died of complications between ages 30 and 48 years, 3–4 decades after onset of gait impairment. Other relevant features during the disease were urinary and fecal incontinence, obesity and psychosis. Our study of 18 Dutch SPG11-patients shows the potential serious long-term consequences of SPG11 including a possibly restricted life span. PMID:23443022

Multigeneration family with dominant SPG30 hereditary spastic paraplegia.

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Roda, Ricardo H; Schindler, Alice B; Blackstone, Craig

2017-11-01

Autosomal recessive KIF1A missense mutations cause hereditary spastic paraplegia (HSP) type SPG30, while recessive truncations lead to sensory and autonomic neuropathy (HSN2C) and many de novo missense mutations are associated with cognitive impairment. Here, we describe family members across three generations with pure HSP. A heterozygous p.Ser69Leu KIF1A mutation segregates with those afflicted. The same variant was previously reported in a Finnish father and son with pure HSP as well as four members of a Sicilian kindred with more intrafamilial phenotypic variability. This further validates the pathogenicity of the p.Ser69Leu mutation and suggests that it may represent a mutation hot spot.

Effect of joint mobilization on the H Reflex amplitude in people with spasticity

OpenAIRE

Pérez Parra, Julio Ernesto; Henao Lema, Claudia Patricia

2011-01-01

Objective: To determine the effect of ankle joint mobilization on the H reflex amplitude of thesoleus muscle in people with spasticity. Materials and methods: A quasi-experimental study withcrossover design and simple masking was conducted in 24 randomized subjects to initiate thecontrol or experimental group. Traction and rhythmic oscillation were applied for five minutesto the ankle joint. H wave amplitude changes of Hoffmann reflex (electrical equivalent of themonosynaptic spinal reflex) w...

Chronic meningoencephalomyelitis with spastic spinal paralysis. Case report

Energy Technology Data Exchange (ETDEWEB)

Mitsuyama, Y; Thompson, L R; Yuki, I; Tanemori, H

1973-01-01

A case of chronic meningoencephalomyelitis in a 48-year-old housewife is presented. The onset was characterized by spastic paralysis of the lower extremities. The course was progressive with repeated remissions and exacerbations, and the patient died approximately 7 years after the onset of disease. Laboratory tests showed slightly increased cell count in the spinal fluid, accelerated sedimentation rate, positive CRP and RA, and increased ASLO and gamma globulin levels. Neuropathologic examination revealed such changes as perivascular cellular infiltration, glial nodules, poorly demarcated demyelination, and recent necrosis in the spinal cord and basal ganglia. Only mild inflammatory findings were noted in the telencephalon and brain stem. The clinicopathologic findings in this case supported a diagnosis of chronic meningoencepalomyelitis which could not be classified as any known type of encephalomyelitis. (auth)

[Medical care of patients with spasticity following stroke : Evaluation of the treatment situation in Germany with focus on the use of botulinum toxin].

Science.gov (United States)

Kerkemeyer, L; Lux, G; Walendzik, A; Wasem, J; Neumann, A

2017-08-01

Upper limb spasticity is a common complication following stroke. Cohort studies found 19% of post-stroke patients had upper limb spasticity at 3 months and 38% of patients at 12 months. For focal spasticity, intramuscular injections of botulinum toxin are indicated. In Germany, it is assumed that patients with the described indication are undersupplied with botulinum toxin. The aim of the present study is to evaluate the medical care of patients with upper limb spasticity post-stroke with the focus on the use of botulinum toxin as one treatment option. A standardized questionnaire was developed and a postal survey of a representative national random sample of 800 neurologists to capture the actual medical care situation. The response rate amounted to 37% (n = 292). 59% of the neurologists surveyed had never used botulinum toxin. In total, 87% of neurologists noticed barriers regarding the use of botulinum toxin, where the amount of the doctor's remuneration in 40% and the lack of reimbursement of costs in off-label use in 60% were the most commonly used answers. The achievement of an advanced training in using botulinum toxin was also stated as a general obstacle for resident neurologists. Due to a response rate of 37% for the postal survey a selection bias cannot be excluded. Although botulinum toxin is recommended in the national treatment guidelines, many neurologists do not use botulinum toxin. The reasons can be seen from the barriers described.

SPG3A-linked hereditary spastic paraplegia associated with cerebral glucose hypometabolism.

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Terada, Tatsuhiro; Kono, Satoshi; Ouchi, Yasuomi; Yoshida, Kenichi; Hamaya, Yasushi; Kanaoka, Shigeru; Miyajima, Hiroaki

2013-04-01

SPG3A-linked hereditary spastic paraplegia (HSP) is a rare autosomal dominant motor disorder caused by a mutation in the SPG3A gene, and is characterized by progressive motor weakness and spasticity in the lower limbs, without any other neurological abnormalities. SPG3A-linked HSP caused by a R239C mutation has been reported to present a pure phenotype confined to impairment of the corticospinal tract. However, there is still a debate about the etiology of this motor deficit with regard to whether it is peripheral or central. We herein report two patients who were heterozygous for a R239C mutation in the SPG3A gene. Two middle-aged Japanese sisters had been suffering from a pure phenotype of HSP since their childhood. Both patients had a significant decrease in glucose metabolism in the frontal cortex medially and dorsolaterally in a [(18)F]-fluorodeoxyglucose (FDG) positron emission photography (PET) study and low scores on the Frontal Assessment Battery. A real-time PCR analysis in normal subjects showed the frontal cortex to be the major location where SPG3A mRNA is expressed. The present finding that the frontal glucose hypometabolism was associated with frontal cognitive impairment indicates that widespread neuropathology associated with mutations in the SPG3A gene may be present more centrally than previously assumed.

Modification of spasticity by transcutaneous spinal cord stimulation in individuals with incomplete spinal cord injury

Science.gov (United States)

Hofstoetter, Ursula S.; McKay, William B.; Tansey, Keith E.; Mayr, Winfried; Kern, Helmut; Minassian, Karen

2014-01-01

Context/objective To examine the effects of transcutaneous spinal cord stimulation (tSCS) on lower-limb spasticity. Design Interventional pilot study to produce preliminary data. Setting Department of Physical Medicine and Rehabilitation, Wilhelminenspital, Vienna, Austria. Participants Three subjects with chronic motor-incomplete spinal cord injury (SCI) who could walk ≥10 m. Interventions Two interconnected stimulating skin electrodes (Ø 5 cm) were placed paraspinally at the T11/T12 vertebral levels, and two rectangular electrodes (8 × 13 cm) on the abdomen for the reference. Biphasic 2 ms-width pulses were delivered at 50 Hz for 30 minutes at intensities producing paraesthesias but no motor responses in the lower limbs. Outcome measures The Wartenberg pendulum test and neurological recordings of surface-electromyography (EMG) were used to assess effects on exaggerated reflex excitability. Non-functional co-activation during volitional movement was evaluated. The timed 10-m walk test provided measures of clinical function. Results The index of spasticity derived from the pendulum test changed from 0.8 ± 0.4 pre- to 0.9 ± 0.3 post-stimulation, with an improvement in the subject with the lowest pre-stimulation index. Exaggerated reflex responsiveness was decreased after tSCS across all subjects, with the most profound effect on passive lower-limb movement (pre- to post-tSCS EMG ratio: 0.2 ± 0.1), as was non-functional co-activation during voluntary movement. Gait speed values increased in two subjects by 39%. Conclusion These preliminary results suggest that tSCS, similar to epidurally delivered stimulation, may be used for spasticity control, without negatively impacting residual motor control in incomplete SCI. Further study in a larger population is warranted. PMID:24090290

Two children with chronic progressive radiation myelopathy

International Nuclear Information System (INIS)

Ohta, Hideaki; Takai, Kenji; Hosoi, Gaku; Okamura, Takayuki; Osugi, Yuko; Ishihara, Shigehiko; Hara, Junichi; Tawa, Akio; Okada, Shintaro

1995-01-01

We report two patients who developed chronic progressive radiation myelopathy (CPRM). Patient 1 was a 16-year-old boy with group IV rhabdomyosarcoma of cervical soft tissue. He underwent partial excision of the tumor and received systemic and intrathecal chemotherapy and 44 Gy of local radiotherapy (C4 through Th3). These therapies were followed by high-dose chemotherapy including thio-TEPA and busulfan with autologous bone marrow rescue. One year after the completion of the therapies, he developed CPRM. Patient 2 was a 15-year-old girl with acute lymphoblastic leukemia on the 3rd complete remission. She received 18 Gy of irradiation to whole brain during the 1st remission and another 18 Gy to whole brain and 9 Gy to spinal cord after her 1st CNS relapse. After successful reinduction therapy for the 2nd relapse in CNS and bone marrow, she underwent an allogeneic bone marrow transplantation (BMT). The preconditioning regimen consisted of 12 Gy total body irradiation, thio-TEPA and cyclophosphamide. Seven months after BMT, she developed CPRM at C0-C1 level, which was included in the area of whole-brain irradiation. In both patients, MR images showed a swelling of the cervical cord and ring-like images by gadolinium enhancement. Their neurological disability transiently responded to the administration of corticosteroid, but they developed progressive quadriplegia. Although it is reported that a dose of 45-50 Gy may be safe, these cases suggest that administration of high-dose chemotherapy combined with intrathecal chemotherapy and radiotherapy to the cord might increase the rink of developing CPRM. (author)

Two children with chronic progressive radiation myelopathy

Energy Technology Data Exchange (ETDEWEB)

Ohta, Hideaki; Takai, Kenji; Hosoi, Gaku; Okamura, Takayuki; Osugi, Yuko; Ishihara, Shigehiko; Hara, Junichi; Tawa, Akio; Okada, Shintaro [Osaka Univ. (Japan). Faculty of Medicine

1995-06-01

We report two patients who developed chronic progressive radiation myelopathy (CPRM). Patient 1 was a 16-year-old boy with group IV rhabdomyosarcoma of cervical soft tissue. He underwent partial excision of the tumor and received systemic and intrathecal chemotherapy and 44 Gy of local radiotherapy (C4 through Th3). These therapies were followed by high-dose chemotherapy including thio-TEPA and busulfan with autologous bone marrow rescue. One year after the completion of the therapies, he developed CPRM. Patient 2 was a 15-year-old girl with acute lymphoblastic leukemia on the 3rd complete remission. She received 18 Gy of irradiation to whole brain during the 1st remission and another 18 Gy to whole brain and 9 Gy to spinal cord after her 1st CNS relapse. After successful reinduction therapy for the 2nd relapse in CNS and bone marrow, she underwent an allogeneic bone marrow transplantation (BMT). The preconditioning regimen consisted of 12 Gy total body irradiation, thio-TEPA and cyclophosphamide. Seven months after BMT, she developed CPRM at C0-C1 level, which was included in the area of whole-brain irradiation. In both patients, MR images showed a swelling of the cervical cord and ring-like images by gadolinium enhancement. Their neurological disability transiently responded to the administration of corticosteroid, but they developed progressive quadriplegia. Although it is reported that a dose of 45-50 Gy may be safe, these cases suggest that administration of high-dose chemotherapy combined with intrathecal chemotherapy and radiotherapy to the cord might increase the rink of developing CPRM. (author).

Effects of intensive physical therapy on the motor function of a child with spastic hemiparesis

Directory of Open Access Journals (Sweden)

María Eugenia Serrano-Gómez

2016-09-01

Full Text Available Introduction: Physical therapy is a health profession whose object of study is the movement of the human body, therefore, it is responsible for cases involving motor development problems, as in the case presented here. Objective: To describe the short-term effect caused by intensive physical therapy treatment, performed with Therasuit, on motor function of a child with spastic right hemiparesis. Materials and methods: Descriptive qualitative research conducted based on the case study methodology with an observation period of two years. Results: The results include, besides the detection and diagnosis of the case, the analysis of information based on the application of the methodology and the observation of results in clinical trials to assess movement, design and implementation of a treatment plan using the Therasuit method. Conclusions: This case study makes possible to observe how the presence of a physical therapy program at a clinical practice institution allowed Therasuit method treatment to a four-year-old child diagnosed with right spastic hemiparesis sequelae, which improved gait and motor function.

NEUROORTHOPEDICAL APPROACH TO THE CORRECTION OF EQUINES CONTRACTURE IN PATIENTS WITH SPASTIC PARALYSIS

Directory of Open Access Journals (Sweden)

Valery Vladimirovich Umnov

2014-03-01

Full Text Available The frequency of recurrent contractures of the joints of the lower limb after their correction by means of tendon-muscle plasty remains significant. Therefore, the search for effective ways to correct contractures with the most resistant long-term result is relevant. The objective of the study is to improve treatment outcomes of equinus contracture in children with spastic paralysis. Materials and methods. We analyzed the results of correction of contractures in joints of lower limbs in 40 patients with cerebral palsy and the influence of spasticity of patognomonic muscles on them. The mean age was 6 years 7 months. In addition, for the correction of hypertonus of triceps muscle of tibia, the 330 lower limb segments were performed selective neurotomy of appropriate motor branches of the general tibial nerve. This operation in 304 cases was combined with achilloplastics or Strayer operation. Results. A mean degree of correlation between the degree of contracture in the ankle and increased tone of triceps tibia was determined (r value ranged from 0.451 to 0.487. Short-term results of the combined neuroorthopedic method for correction of contractures were good in estimating within 1 year post surgery, but a study of its short-run effect requires long-term follow-up.

Orthodontic treatment and follow-up of a patient with cerebral palsy and spastic quadriplegia.

Science.gov (United States)

Çifter, Muhsin; Cura, Nil

2016-10-01

This report describes the clinical orthodontic management of a patient with spastic quadriplegia and cerebral palsy. Guidelines to overcome difficulties encountered during the treatment period are suggested. A 13-year-old boy with cerebral palsy and spastic quadriplegia complained of an undesirable oral appearance because of his malocclusion. He had a Class II molar relationship, with severe maxillary and moderate mandibular anterior crowding. Enamel hypoplasia was apparent on all teeth. He had losses of body function and upper extremity function of 70% and 39%, respectively. His physical limitations necessitated a treatment approach that did not rely on patient-dependent appliances. The treatment plan called for maxillary first premolar extractions, mandibular incisor protrusion, and air rotor stripping. The patient's oral function and esthetic appearance were significantly improved. Aligned dental arches with good occlusion were obtained. The patient's self-confidence improved during the treatment period. Physical appearance can influence personality and social acceptability. Corrective orthodontic treatment for patients with physical handicaps can improve not only oral function, but also self-confidence and self-esteem. Copyright © 2016 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

Outcome of medial hamstring lengthening in children with spastic paresis: A biomechanical and morphological observational study.

Science.gov (United States)

Haberfehlner, Helga; Jaspers, Richard T; Rutz, Erich; Harlaar, Jaap; van der Sluijs, Johannes A; Witbreuk, Melinda M; van Hutten, Kim; Romkes, Jacqueline; Freslier, Marie; Brunner, Reinald; Becher, Jules G; Maas, Huub; Buizer, Annemieke I

2018-01-01

To improve gait in children with spastic paresis due to cerebral palsy or hereditary spastic paresis, the semitendinosus muscle is frequently lengthened amongst other medial hamstring muscles by orthopaedic surgery. Side effects on gait due to weakening of the hamstring muscles and overcorrections have been reported. How these side effects relate to semitendinosus morphology is unknown. This study assessed the effects of bilateral medial hamstring lengthening as part of single-event multilevel surgery (SEMLS) on (1) knee joint mechanics (2) semitendinosus muscle morphology and (3) gait kinematics. All variables were assessed for the right side only. Six children with spastic paresis selected for surgery to counteract limited knee range of motion were measured before and about a year after surgery. After surgery, in most subjects popliteal angle decreased and knee moment-angle curves were shifted towards a more extended knee joint, semitendinosus muscle belly length was approximately 30% decreased, while at all assessed knee angles tendon length was increased by about 80%. In the majority of children muscle volume of the semitendinosus muscle decreased substantially suggesting a reduction of physiological cross-sectional area. Gait kinematics showed more knee extension during stance (mean change ± standard deviation: 34±13°), but also increased pelvic anterior tilt (mean change ± standard deviation: 23±5°). In most subjects, surgical lengthening of semitendinosus tendon contributed to more extended knee joint angle during static measurements as well as during gait, whereas extensibility of semitendinosus muscle belly was decreased. Post-surgical treatment to maintain muscle belly length and physiological cross-sectional area may improve treatment outcome of medial hamstring lengthening.

Plastic changes in spinal synaptic transmission following botulinum toxin A in patients with post-stroke spasticity.

Science.gov (United States)

Kerzoncuf, Marjorie; Bensoussan, Laurent; Delarque, Alain; Durand, Jacques; Viton, Jean-Michel; Rossi-Durand, Christiane

2015-11-01

The therapeutic effects of intramuscular injections of botulinum toxin-type A on spasticity can largely be explained by its blocking action at the neuromuscular junction. Botulinum toxin-type A is also thought to have a central action on the functional organization of the central nervous system. This study assessed the action of botulinum toxin-type A on spinal motor networks by investigating post-activation depression of the soleus H-reflex in post-stroke patients. Post-activation depression, a presynaptic mechanism controlling the synaptic efficacy of Ia-motoneuron transmission, is involved in the pathophysiology of spasticity. Eight patients with chronic hemiplegia post-stroke presenting with lower limb spasticity and requiring botulinum toxin-type A injection in the ankle extensor muscle. Post-activation depression of soleus H-reflex assessed as frequency-related depression of H-reflex was investigated before and 3, 6 and 12 weeks after botulinum toxin-type A injections in the triceps surae. Post-activation depression was quantified as the ratio between H-reflex amplitude at 0.5 and 0.1 Hz. Post-activation depression of soleus H-reflex, which is reduced on the paretic leg, was affected 3 weeks after botulinum toxin-type A injection. Depending on the residual motor capacity of the post-stroke patients, post-activation depression was either restored in patients with preserved voluntary motor control or further reduced in patients with no residual voluntary control. Botulinum toxin treatment induces synaptic plasticity at the Ia-motoneuron synapse in post-stroke paretic patients, which suggests that the effectiveness of botulinum toxin-type A in post-stroke rehabilitation might be partly due to its central effects.

Changes in skeletal muscle perfusion and spasticity in patients with poststroke hemiparesis treated by robotic assistance (Gloreha) of the hand.

Science.gov (United States)

Bissolotti, Luciano; Villafañe, Jorge Hugo; Gaffurini, Paolo; Orizio, Claudio; Valdes, Kristin; Negrini, Stefano

2016-03-01

[Purpose] The purpose of this case series was to determine the effects of robot-assisted hand rehabilitation with a Gloreha device on skeletal muscle perfusion, spasticity, and motor function in subjects with poststroke hemiparesis. [Subjects and Methods] Seven patients, 2 women and 5 men (mean ± SD age: 60.5 ±6.3 years), with hemiparesis (>6 months poststroke), received passive mobilization of the hand with a Gloreha (Idrogenet, Italy), device (30 min per day; 3 sessions a week for 3 weeks). The outcome measures were the total hemoglobin profiles and tissue oxygenation index (TOI) in the muscle tissue evaluated through near-infrared spectroscopy. The Motricity Index and modified Ashworth Scale for upper limb muscles were used to assess mobility of the upper extremity. [Results] Robotic assistance reduced spasticity after the intervention by 68.6% in the upper limb. The Motricity Index was unchanged in these patients after treatment. Regarding changes in muscle perfusion, significant improvements were found in total hemoglobin. There were significant differences between the pre- and posttreatment modified Ashworth scale. [Conclusion] The present work provides novel evidence that robotic assistance of the hand induced changes in local muscle blood flow and oxygen supply, diminished spasticity, and decreased subject-reported symptoms of heaviness and stiffness in subjects with post-stroke hemiparesis.

Brain magnetic resonance imaging and motor and intellectual functioning in 86 patients born at term with spastic diplegia.

Science.gov (United States)

Numata, Yurika; Onuma, Akira; Kobayashi, Yasuko; Sato-Shirai, Ikuko; Tanaka, Soichiro; Kobayashi, Satoru; Wakusawa, Keisuke; Inui, Takehiko; Kure, Shigeo; Haginoya, Kazuhiro

2013-02-01

To investigate the association between magnetic resonance imaging (MRI) patterns and motor function, epileptic episodes, and IQ or developmental quotient in patients born at term with spastic diplegia. Eighty-six patients born at term with cerebral palsy (CP) and spastic diplegia (54 males, 32 females; median age 20 y, range 7-42 y) among 829 patients with CP underwent brain MRI between 1990 and 2008. The MRI and clinical findings were analysed retrospectively. Intellectual disability was classified according to the Enjoji developmental test or the Wechsler Intelligence Scale for Children (3rd edition). The median ages at diagnosis of CP, assignment of Gross Motor Function Classification System (GMFCS) level, cognitive assessment, and MRI were 2 years (range 5 mo-8 y), 6 years (2 y 8 mo-19 y), 6 years (1 y 4 mo-19 y), and 7 years (10 mo-30 y) respectively. MRI included normal findings (41.9%), periventricular leukomalacia, hypomyelination, and porencephaly/periventricular venous infarction. The frequency of patients in GMFCS levels III to V and intellectual disability did not differ between those with normal and abnormal MRI findings. Patients with normal MRI findings had significantly fewer epileptic episodes than those with abnormal ones (p=0.001). Varied MRI findings, as well as the presence of severe motor dysfunction and intellectual disability (despite normal MRI), suggest that patients born at term with spastic diplegia had heterogeneous and unidentified pathophysiology. © The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.

Radiation myelopathy in over-irradiated patients: MR imaging findings

International Nuclear Information System (INIS)

Alfonso, E.R.; Gregorio, M.A. de; Mateo, P.; Esco, R.; Bascon, N.; Morales, F.; Bellosta, R.; Lopez, P.; Gimeno, M.; Roca, M.; Villavieja, J.L.

1997-01-01

The objective of this work is to report the MRI findings in patients with radiation myelopathy due to accidental local over-irradiation syndrome. Eight patients (seven males and one female) were suffering from over-irradiation syndrome as a result of treatments from a malfunctioning linear electron accelerator. The mean accidental estimated dose was 136 Gy delivered to the ''open-neck'' (seven cases) and to the thoracic wall (one case), during a mean of 5.4 sessions (range 1-9 sessions). Paresthesia and weakness in the upper extremities were the earliest symptoms (87.5 %), with evolution to paralysis in all patients. No patient is alive (mean survival time 64 days). In all cases MRI was negative for neurologic lesions in the acute phase (< 90 days from irradiation; Radiation Therapy Oncology Group scoring system). Late signs of radiation myelitis manifested as high-intensity signals on T2-weighted images in three patients, and as Gd-DTPA enhancement of T1-weighted images in one case. Autopsies performed on four patients who died in acute phase showed morphologic alterations in white matter: edema in 75 %, and necrosis and glial reaction as well as obliterative vasculitis in all cases. In cases of over-irradiation, MRI may be normal in acute phase even if the patients have severe neurologic deficit, as positive MRI findings appear only in delayed radiation myelitis. (orig.). With 3 figs., 2 tabs

Radiation myelopathy in over-irradiated patients: MR imaging findings

Energy Technology Data Exchange (ETDEWEB)

Alfonso, E.R. [Radiology Service, Hospital Clinico Universitario, Zaragoza (Spain); Gregorio, M.A. de [Radiology Service, Hospital Clinico Universitario, Zaragoza (Spain); Mateo, P. [Radiation Oncology Service, Hospital Clinico Universitario, Zaragoza (Spain); Esco, R. [Radiation Oncology Service, Hospital Clinico Universitario, Zaragoza (Spain); Bascon, N. [Radiation Oncology Service, Hospital Clinico Universitario, Zaragoza (Spain); Morales, F. [Neurology Service, Hospital Clinico Universitario, Zaragoza (Spain); Bellosta, R. [Radiation Oncology Service, Hospital Clinico Universitario, Zaragoza (Spain); Lopez, P. [Radiation Oncology Service, Hospital Clinico Universitario, Zaragoza (Spain); Gimeno, M. [Hospital Miguel Servet, Zaragoza (Spain); Roca, M. [Radiology Service, Hospital Miguel Servet, E-50 009 Zaragoza (Spain); Villavieja, J.L. [Radiology Service, Hospital Clinico Universitario, Zaragoza (Spain)

1997-04-01

The objective of this work is to report the MRI findings in patients with radiation myelopathy due to accidental local over-irradiation syndrome. Eight patients (seven males and one female) were suffering from over-irradiation syndrome as a result of treatments from a malfunctioning linear electron accelerator. The mean accidental estimated dose was 136 Gy delivered to the ``open-neck`` (seven cases) and to the thoracic wall (one case), during a mean of 5.4 sessions (range 1-9 sessions). Paresthesia and weakness in the upper extremities were the earliest symptoms (87.5 %), with evolution to paralysis in all patients. No patient is alive (mean survival time 64 days). In all cases MRI was negative for neurologic lesions in the acute phase (< 90 days from irradiation; Radiation Therapy Oncology Group scoring system). Late signs of radiation myelitis manifested as high-intensity signals on T2-weighted images in three patients, and as Gd-DTPA enhancement of T1-weighted images in one case. Autopsies performed on four patients who died in acute phase showed morphologic alterations in white matter: edema in 75 %, and necrosis and glial reaction as well as obliterative vasculitis in all cases. In cases of over-irradiation, MRI may be normal in acute phase even if the patients have severe neurologic deficit, as positive MRI findings appear only in delayed radiation myelitis. (orig.). With 3 figs., 2 tabs.

The effect of walking speed on hamstrings length and lengthening velocity in children with spastic cerebral palsy

NARCIS (Netherlands)

Krogt, van der M.M.; Doorenbosch, C.A.M.; Harlaar, J.

2009-01-01

0.001). These data are important as a reference for valid interpretation of hamstrings length and velocity data in gait analyses at different walking speeds. The results indicate that the presence of spasticity is associated with reduced hamstrings length and lengthening velocity during gait, even

DNABIT Compress – Genome compression algorithm

OpenAIRE

Rajarajeswari, Pothuraju; Apparao, Allam

2011-01-01

Data compression is concerned with how information is organized in data. Efficient storage means removal of redundancy from the data being stored in the DNA molecule. Data compression algorithms remove redundancy and are used to understand biologically important molecules. We present a compression algorithm, “DNABIT Compress” for DNA sequences based on a novel algorithm of assigning binary bits for smaller segments of DNA bases to compress both repetitive and non repetitive DNA sequence. Our ...

Intrathecal baclofen pumps do not accelerate progression of scoliosis in quadriplegic spastic cerebral palsy.

Science.gov (United States)

Rushton, Paul R P; Nasto, Luigi A; Aujla, Ranjit K; Ammar, Amr; Grevitt, Michael P; Vloeberghs, Michael H

2017-06-01

To compare scoliosis progression in quadriplegic spastic cerebral palsy with and without intrathecal baclofen (ITB) pumps. A retrospective matched cohort study was conducted. Patients with quadriplegic spastic cerebral palsy, GMFCS level 5, treated with ITB pumps with follow-up >1 year were matched to comparable cases by age and baseline Cobb angle without ITB pumps. Annual and peak coronal curve progression, pelvic obliquity progression and need for spinal fusion were compared. ITB group: 25 patients (9 female), mean age at pump insertion 9.4 and Risser 0.9. Initial Cobb angle 25.6° and pelvic tilt 3.2°. Follow-up 4.3 (1.0-7.8) years. Cobb angle at follow-up 76.1° and pelvic tilt 18.9°. Non-ITB group: 25 patients (14 female), mean age at baseline 9.2 and Risser 1.0. Initial Cobb angle 29.7° and pelvic tilt 7.1°. Follow-up 3.5 (1.0-7.5) years. Cobb angle at follow-up 69.1° and pelvic tilt 21.0°. The two groups were statistically similar for baseline age, Cobb angle and Risser grade. Mean curve progression was 13.6°/year for the ITB group vs 12.6°/year for the non-ITB group (p = 0.39). Peak curve progression was similar between the groups. Pelvic tilt progression was comparable; ITB group 4.5°/year vs non-ITB 4.6°/year (p = 0.97). During follow-up 5 patients in the ITB group and 9 in the non-ITB group required spinal fusion surgery for curve progression (p = 0.35). Patients with quadriplegic spastic cerebral palsy with and without ITB pumps showed significant curve progression over time. ITB pumps do not appear to alter the natural history of curve progression in this population.

Targeted retrograde transfection of adenovirus vector carrying brain-derived neurotrophic factor gene prevents loss of mouse (twy/twy) anterior horn neurons in vivo sustaining mechanical compression.

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Xu, Kan; Uchida, Kenzo; Nakajima, Hideaki; Kobayashi, Shigeru; Baba, Hisatoshi

2006-08-01

Immunohistochemical analysis after adenovirus (AdV)-mediated BDNF gene transfer in and around the area of mechanical compression in the cervical spinal cord of the hyperostotic mouse (twy/twy). To investigate the neuroprotective effect of targeted AdV-BDNF gene transfection in the twy mouse with spontaneous chronic compression of the spinal cord motoneurons. Several studies reported the neuroprotective effects of neurotrophins on injured spinal cord. However, no report has described the effect of targeted retrograde neurotrophic gene delivery on motoneuron survival in chronic compression lesions of the cervical spinal cord resembling lesions of myelopathy. LacZ marker gene using adenoviral vector (AdV-LacZ) was used to evaluate retrograde delivery from the sternomastoid muscle in adult twy mice (16-week-old) and (control). Four weeks after the AdV-LacZ or AdV-BDNF injection, the compressed cervical spinal cord was removed en bloc for immunohistologic investigation of b-galactosidase activity and immunoreactivity and immunoblot analyses of BDNF. The number of anterior horn neurons was counted using Nissl, ChAT and AChE staining. Spinal accessory motoneurons between C1 and C3 segments were successfully transfected by AdV-LacZ in both twy and ICR mice after targeted intramuscular injection. Immunoreactivity to BDNF was significantly stronger in AdV-BDNF-gene transfected twy mice than in AdV-LacZ-gene transfected mice. At the cord level showing the maximum compression in AdV-BDNF-transfected twy mice, the number of anterior horn neurons was sinificantly higher in the topographic neuronal cell counting of Nissl-, ChAT-, and AChE-stained samples than in AdV-LacZ-injected twy mice. Targeted AdV-BDNF-gene delivery significantly increased Nissl-stained anterior horn neurons and enhanced cholinergic enzyme activities in the twy. Our results suggest that targeted retrograde AdV-BDNF-gene in vivo delivery may enhance neuronal survival even under chronic mechanical compression.

Whole-Exome Sequencing Identifies Homozygous AFG3L2 Mutations in a Spastic Ataxia-Neuropathy Syndrome Linked to Mitochondrial m-AAA Proteases

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Martinelli, Paola; Cherukuri, Praveen F.; Teer, Jamie K.; Hansen, Nancy F.; Cruz, Pedro; Mullikin for the NISC Comparative Sequencing Program, James C.; Blakesley, Robert W.; Golas, Gretchen; Kwan, Justin; Sandler, Anthony; Fuentes Fajardo, Karin; Markello, Thomas; Tifft, Cynthia; Blackstone, Craig; Rugarli, Elena I.; Langer, Thomas; Gahl, William A.; Toro, Camilo

2011-01-01

We report an early onset spastic ataxia-neuropathy syndrome in two brothers of a consanguineous family characterized clinically by lower extremity spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. Whole-exome sequencing identified a homozygous missense mutation (c.1847G>A; p.Y616C) in AFG3L2, encoding a subunit of an m-AAA protease. m-AAA proteases reside in the mitochondrial inner membrane and are responsible for removal of damaged or misfolded proteins and proteolytic activation of essential mitochondrial proteins. AFG3L2 forms either a homo-oligomeric isoenzyme or a hetero-oligomeric complex with paraplegin, a homologous protein mutated in hereditary spastic paraplegia type 7 (SPG7). Heterozygous loss-of-function mutations in AFG3L2 cause autosomal-dominant spinocerebellar ataxia type 28 (SCA28), a disorder whose phenotype is strikingly different from that of our patients. As defined in yeast complementation assays, the AFG3L2Y616C gene product is a hypomorphic variant that exhibited oligomerization defects in yeast as well as in patient fibroblasts. Specifically, the formation of AFG3L2Y616C complexes was impaired, both with itself and to a greater extent with paraplegin. This produced an early-onset clinical syndrome that combines the severe phenotypes of SPG7 and SCA28, in additional to other “mitochondrial” features such as oculomotor apraxia, extrapyramidal dysfunction, and myoclonic epilepsy. These findings expand the phenotype associated with AFG3L2 mutations and suggest that AFG3L2-related disease should be considered in the differential diagnosis of spastic ataxias. PMID:22022284

Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases.

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Tyler Mark Pierson

2011-10-01

Full Text Available We report an early onset spastic ataxia-neuropathy syndrome in two brothers of a consanguineous family characterized clinically by lower extremity spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. Whole-exome sequencing identified a homozygous missense mutation (c.1847G>A; p.Y616C in AFG3L2, encoding a subunit of an m-AAA protease. m-AAA proteases reside in the mitochondrial inner membrane and are responsible for removal of damaged or misfolded proteins and proteolytic activation of essential mitochondrial proteins. AFG3L2 forms either a homo-oligomeric isoenzyme or a hetero-oligomeric complex with paraplegin, a homologous protein mutated in hereditary spastic paraplegia type 7 (SPG7. Heterozygous loss-of-function mutations in AFG3L2 cause autosomal-dominant spinocerebellar ataxia type 28 (SCA28, a disorder whose phenotype is strikingly different from that of our patients. As defined in yeast complementation assays, the AFG3L2(Y616C gene product is a hypomorphic variant that exhibited oligomerization defects in yeast as well as in patient fibroblasts. Specifically, the formation of AFG3L2(Y616C complexes was impaired, both with itself and to a greater extent with paraplegin. This produced an early-onset clinical syndrome that combines the severe phenotypes of SPG7 and SCA28, in additional to other "mitochondrial" features such as oculomotor apraxia, extrapyramidal dysfunction, and myoclonic epilepsy. These findings expand the phenotype associated with AFG3L2 mutations and suggest that AFG3L2-related disease should be considered in the differential diagnosis of spastic ataxias.

Classification of Posture in Poststroke Upper Limb Spasticity: A Potential Decision Tool for Botulinum Toxin A Treatment?

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Hefter, Harald; Jost, Wolfgang H.; Reissig, Andrea; Zakine, Benjamin; Bakheit, Abdel Magid; Wissel, Jorg

2012-01-01

A significant percentage of patients suffering from a stroke involving motor-relevant central nervous system regions will develop a spastic movement disorder. Hyperactivity of different muscle combinations forces the limbs affected into abnormal postures or movement patterns. As muscular hyperactivity can effectively and safely be treated with…