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Sample records for central cord syndrome

  1. Outcome after incomplete spinal cord injury: central cord versus Brown-Sequard syndrome

    OpenAIRE

    Wirz, M.; Zörner, B; Rupp, R; Dietz, V.

    2010-01-01

    Study design : A retrospective analysis of prospectively collected data.Objective:A hemisection of the spinal cord is a frequently used animal model for spinal cord injury (SCI), the corresponding human condition, that is, the Brown-Sequard syndrome (BS), is relatively rare as compared with the central cord syndrome (CC). The time course of neurological deficit, functional recovery, impulse conductivity and rehabilitation length of stay in BS and CC subjects were compared.Setting:Nine Europea...

  2. Acute traumatic central cord syndrome: MRI-pathological correlations

    International Nuclear Information System (INIS)

    The acute traumatic central cord syndrome (ATCCS) is commonly stated to result from an injury which affects primarily the center of the spinal cord and is frequently hemorrhagic. To test the validity of this widely disseminated hypothesis, the magnetic resonance images [MRI] of 11 consecutive cases of ATCCS caused by closed injury to the spine were analyzed and correlated with the gross pathological and histological features of 3 cervical spinal cords obtained at post mortem from patients with ATCCS, including 2 of patients studied by MRI. In this study, the MRI and pathological observations indicate that ATCCS is predominantly a white matter injury and that intramedullary hemorrhage is not a necessary feature of the syndrome; indeed, it is probably an uncommon event in ATCCS. We suggest that the most common mechanism of injury in ATCCS may be direct compression of the cervical spinal cord by buckling of the ligamenta flava into an already narrowed cervical spinal canal; this would explain the predominance of axonal injury in the white matter of the lateral columns. (orig./GDG)

  3. MAGNETIC RESONANCE IMAGING OF ACUTE CENTRAL CORD SYNDROME: CORRELATION WITH PROGNOSIS

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective. To report the magnetic resonance imaging (MRI) findings of acute cervical central cord syndrome and to determine their correlation with the prognosis. Methods. MRI findings of 35 patients with acute central cord syndrome were studied and compared with the recovery rate of ASIA score at presentation and in follow-up. Results. MRI data demonstrated spinal cord compression for 32 patients, spinal cord swelling for 16 patients, and abnormal signal intensity within the spinal cord for 19 patients, including 14 with edema and 3 with hematoma. No significant difference of the recovery rate was noted between the patients treated nonoperatively and operatively (P >0.05). There was a significant inverse correlation between the recovery rate and the degree of spinal cord compression as shown in MRI scans (P<0.01). The presence of hematoma in MRI scans was associated with poor prognosis, as demonstrated by a significant difference of the recovery rate (P< 0.01) among the patients with normal intensity, edema and hematoma within the spinal cord. Conclusions. MRI scans provide an efficient assistance for decision-making and accurate prognostic information regarding neurological function, and therefore should routinely be performed within the early phase of acute central cord syndrome.

  4. Anterior cervical surgery methods for central cord syndrome without radiographic spinal fracture-dislocation

    Institute of Scientific and Technical Information of China (English)

    Chengwei JING; Qin FU; Xiaojun XU

    2009-01-01

    This study was aimed to explore the anterior cervical surgery methods to treat central cord syndrome without radiographic spinal fracture-dislocation (CCSWORFD), retrospectively analyze the cases of CCSWORFD, and evaluate the curative effect of anterior cervical surgery methods for CCSWORFD. Twenty four cases of CCSWORFD (19 males and 5 females), all suffering from cervical hyperextension injury, between 45-68 (average 59) years old, were operated on by anterior cervical surgery methods. Among these, 18 cases had been followed up for 6-24 (average 15) months; 18 cases, who had anterior decompression and plate fixation with titanium mesh bone grafting or iliac bone grafting achieved reliable effects based on the Japanese Orthopedics Association (JOA) evaluation (improved scores of cases with titanium mesh bone grafting, t = 2.800, P0.05). Most of these cases had degeneration of cervical vertebra. The decompression which relieves the oppression to the spinal cord can help lessen edema of the spinal cord, and early fixation for stability of cervical vertebra is better for the recovery of spinal cord injury. Anterior operation with titanium mesh bone grafting or iliac bone grafting are both reliable curative methods for CCSWORFD, and titanium mesh bone grafting can avoid the trauma of the supplying graft. Mesh bone grafting can also shorten hospital stay.

  5. Gait kinematic analysis in patients with a mild form of central cord syndrome

    Directory of Open Access Journals (Sweden)

    Crespo-Ruiz Beatriz

    2011-02-01

    Full Text Available Abstract Background Central cord syndrome (CCS is considered the most common incomplete spinal cord injury (SCI. Independent ambulation was achieved in 87-97% in young patients with CCS but no gait analysis studies have been reported before in such pathology. The aim of this study was to analyze the gait characteristics of subjects with CCS and to compare the findings with a healthy age, sex and anthropomorphically matched control group (CG, walking both at a self-selected speed and at the same speed. Methods Twelve CCS patients and a CG of twenty subjects were analyzed. Kinematic data were obtained using a three-dimensional motion analysis system with two scanner units. The CG were asked to walk at two different speeds, at a self-selected speed and at a slower one, similar to the mean gait speed previously registered in the CCS patient group. Temporal, spatial variables and kinematic variables (maximum and minimum lower limb joint angles throughout the gait cycle in each plane, along with the gait cycle instants of occurrence and the joint range of motion - ROM were compared between the two groups walking at similar speeds. Results The kinematic parameters were compared when both groups walked at a similar speed, given that there was a significant difference in the self-selected speeds (p Conclusions The gait pattern of CCS patients showed a decrease of knee and ankle sagittal ROM during level walking and an increase in hip abduction to increase base of support. The findings of this study help to improve the understanding how CCS affects gait changes in the lower limbs.

  6. Tethered cord syndrome: case report

    International Nuclear Information System (INIS)

    Tethered cord syndrome is one of the filum terminale congenital defects. It can coexist with anomalies of the spinal canal and column, as well as with anorectal defects. The authors present a case of tethered cord syndrome diagnosed in a 45-year-old woman. She showed typical lumbo-sacral radicular syndrome with no neurological deficits and no bowel/bladder dysfunction. The anomaly coexisted with fibrolipoma, spina bifida and Tarlov cyst. Magnetic resonance imaging is the method of choice in diagnostics of tethered cord syndrome. It provides crucial information, which is necessary for planning surgical treatment of the anomaly. (author)

  7. Central Pain Syndrome

    Science.gov (United States)

    ... Enhancing Diversity Find People About NINDS NINDS Central Pain Syndrome Information Page Table of Contents (click to ... being done? Clinical Trials Organizations What is Central Pain Syndrome? Central pain syndrome is a neurological condition ...

  8. Vocal cord collapse during phrenic nerve-paced respiration in congenital central hypoventilation syndrome [v1; ref status: indexed, http://f1000r.es/Sh1pwj

    Directory of Open Access Journals (Sweden)

    Mark C Domanski

    2012-11-01

    Full Text Available Objective: Phrenic nerve pacing can be used to treat congenital central hypoventilation syndrome (CCHS. We report how the lack of normal vocal cord tone during phrenic paced respiration can result in passive vocal cord collapse and produce obstructive symptoms. Methods: We describe a case of passive vocal cord collapse during phrenic nerve paced respiration in a patient with CCHS. As far as we know, this is the first report of this etiology of airway obstruction. The patient, a 7-year-old with CCHS and normal waking vocal cord movement, continued to require nightly continuous positive airway pressure (CPAP despite successful utilization of phrenic nerve pacers. On direct laryngoscopy, the patient’s larynx was observed while the diaphragmatic pacers were sequentially engaged. Results: No abnormal vocal cord stimulation was witnessed during engaging of either phrenic nerve stimulator. However, the lack of normal inspiratory vocal cord abduction during phrenic nerve-paced respiration resulted in vocal cord collapse and partial obstruction due to passive adduction of the vocal cords through the Bernoulli effect. Bilateral phrenic nerve stimulation resulted in more vocal cord collapse than unilateral stimulation. Conclusions: The lack of vocal cord abduction on inspiration presents a limit to phrenic nerve pacers.

  9. Anorgasmia in anterior spinal cord syndrome.

    OpenAIRE

    Berić, A; Light, J K

    1993-01-01

    Three male and two female patients with anorgasmia and dissociated sensory loss due to an anterior spinal cord syndrome are described. Clinical, neurophysiological and quantitative sensory evaluation revealed preservation of the large fibre dorsal column functions from the lumbosacral segments with concomitant severe dysfunction or absence of the small fibre neospinothalamic mediated functions. These findings indicate a role for the spinothalamic system in orgasm.

  10. MAGNETIC RESONANCE IMAGING OF ACUTE CENTRAL CORD SYNDROME: CORRELATION WITH PROGNOSIS

    Institute of Scientific and Technical Information of China (English)

    戴力扬

    2001-01-01

    Objective. To report the magnetic resonance imaging (MRI) findings of acute cervical central cord syndrome and to determine their correlation with the prognosis. Methods. MRI findings of 35 patients with acute central cord syndrome were studied and compared with the recovery rate of ASIA score at presentation and in follow-up. Results. MRI data demonstrated spinal cord compression for 32 patients, spinal cord swelling for 16 patients,and abnormal signal intensity within the spinal cord for 19 patients, including 14 with edema and 3 with hematoma. No significant difference of the recovery rate was noted between the patients treated nonoperatively and operatively ( P > 0. 05). There was a significant inverse correlation between the recovery rate and the degree of spinal cord compression as shown in MRI scans ( P < 0.01 ). The presence of hematoma in MRI scans was associated with poor prognosis, as demonstrated by a significant difference of the recovery rate ( P < 0. 01) among the patients with normal intensity, edema and hematoma within the spinal cord. Conclusions. MRI scans provide an efficient assistance for decision-making and accurate prognostic information regarding neurological function, and therefore should routinely be performed within the early phase of acute central cord syndrome.

  11. Spinal Cord Anatomy and Clinical Syndromes.

    Science.gov (United States)

    Diaz, Eric; Morales, Humberto

    2016-10-01

    We review the anatomy of the spinal cord, providing correlation with key functional and clinically relevant neural pathways, as well as magnetic resonance imaging. Peripherally, the main descending (corticospinal tract) and ascending (gracilis or cuneatus fasciculi and spinothalamic tracts) pathways compose the white matter. Centrally, the gray matter can be divided into multiple laminae. Laminae 1-5 carry sensitive neuron information in the posterior horn, and lamina 9 carries most lower motor neuron information in the anterior horn. Damage to the unilateral corticospinal tract (upper motor neuron information) or gracillis-cuneatus fasciculi (touch and vibration) correlates with ipsilateral clinical findings, whereas damage to unilateral spinothalamic tract (pain-temperature) correlates with contralateral clinical findings. Damage to commissural fibers correlates with a suspended bilateral "girdle" sensory level. Autonomic dysfunction is expected when there is bilateral cord involvement. PMID:27616310

  12. [Central anticholinergic syndrome].

    Science.gov (United States)

    Fernández Urretavizcaya, P; Cenoz Osinaga, J C; Jáuregui Garía, M L; Gállego Culleré, J

    1991-10-01

    Two new cases of anticolinergic central syndrome are described. The first case, a 8 year old girl, suffered a severe encefalopathy after topical application of mydriatic cholirio as an aid in a rutine study of ocular refraction. The second case, 67 year old man presented a severe neurological picture after accidental intake of a silvester plantground (Atropa belladonna). His neurological condition returned quickly to normal whith administration of physostigmine. Differents aspects of the etiology, clinical picture and diagnosis are discussed with special emphasis in patients with delirium or acute confusional states. Finally, attention is drawn in the necessity of a properly use of anticholinergic drugs overcoat in aged or children.

  13. Transitory spinal cord swelling in a 6-year-old boy with Guillain-Barre syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Delhaas, T. [Section of Child Neurology, Wilhelmina Children`s Hospital, University Hospital for Children and Youth, Utrecht (Netherlands)]|[Department of Pediatrics, University Hospital Maastricht, P0 Box 5800, 6202 AZ Maastricht (Netherlands); Kamphuis, D.J. [Section of Child Neurology, Wilhelmina Children`s Hospital, University Hospital for Children and Youth, Utrecht (Netherlands); Witkamp, T.D. [Department of Radiology, University Hospital Utrecht, Utrecht (Netherlands)

    1998-07-01

    A 6-year-old boy developed progressive motor weakness and areflexia. The clinical picture, combined with electrophysiological findings, indicated a diagnosis of Guillain-Barre syndrome (GBS). MRI on admission revealed spinal cord swelling and increased signal intensity within the cord. It is concluded that, since a degree of central nervous system involvement can occasionally be part of the spectrum of GBS, swelling of the spinal cord without contrast enhancement does not exclude a diagnosis of GBS. (orig.) With 2 figs., 8 refs.

  14. Spinal cord injury-induced immune deficiency syndrome enhances infection susceptibility dependent on lesion level.

    Science.gov (United States)

    Brommer, Benedikt; Engel, Odilo; Kopp, Marcel A; Watzlawick, Ralf; Müller, Susanne; Prüss, Harald; Chen, Yuying; DeVivo, Michael J; Finkenstaedt, Felix W; Dirnagl, Ulrich; Liebscher, Thomas; Meisel, Andreas; Schwab, Jan M

    2016-03-01

    Pneumonia is the leading cause of death after acute spinal cord injury and is associated with poor neurological outcome. In contrast to the current understanding, attributing enhanced infection susceptibility solely to the patient's environment and motor dysfunction, we investigate whether a secondary functional neurogenic immune deficiency (spinal cord injury-induced immune deficiency syndrome, SCI-IDS) may account for the enhanced infection susceptibility. We applied a clinically relevant model of experimental induced pneumonia to investigate whether the systemic SCI-IDS is functional sufficient to cause pneumonia dependent on spinal cord injury lesion level and investigated whether findings are mirrored in a large prospective cohort study after human spinal cord injury. In a mouse model of inducible pneumonia, high thoracic lesions that interrupt sympathetic innervation to major immune organs, but not low thoracic lesions, significantly increased bacterial load in lungs. The ability to clear the bacterial load from the lung remained preserved in sham animals. Propagated immune susceptibility depended on injury of central pre-ganglionic but not peripheral postganglionic sympathetic innervation to the spleen. Thoracic spinal cord injury level was confirmed as an independent increased risk factor of pneumonia in patients after motor complete spinal cord injury (odds ratio = 1.35, P paralysis, spinal cord injury also induces a functional SCI-IDS ('immune paralysis'), sufficient to propagate clinically relevant infection in an injury level dependent manner. PMID:26754788

  15. Imaging central pain syndromes.

    Science.gov (United States)

    Veldhuijzen, Dieuwke S; Greenspan, Joel D; Kim, Jong H; Coghill, Robert C; Treede, Rolf-Detlef; Ohara, Shinji; Lenz, Frederick A

    2007-06-01

    Anatomic, functional, and neurochemical imaging studies have provided new investigative tools in the study of central pain. High-resolution imaging studies allow for precise determination of lesion location, whereas functional neuroimaging studies measure pathophysiologic consequences of injury to the central nervous system. Additionally, magnetic resonance spectroscopy evaluates lesion-induced neurochemical changes in specific brain regions that may be related to central pain. The small number of studies to date precludes definitive conclusions, but the recent findings provide information that either supports or refutes current hypotheses and can serve to generate new ideas.

  16. Spinal cord stimulation in chronic pain syndromes

    NARCIS (Netherlands)

    ten Vaarwerk, IAM; Staal, MJ

    1998-01-01

    Spinal cord stimulation (SCS) has been used for more than 30 years now, and although it has shown to be effective under certain well-described conditions of chronic pain, conclusive evidence on its effectiveness is still sparse. There is a need for more prospective and methodological good studies, i

  17. Tethered spinal cord syndrome with symptomatic onset in adulthood

    Institute of Scientific and Technical Information of China (English)

    HE Shi-sheng; ZHAO Ying-chuan; SHI Zhi-cai; LI Ming; HOU Tie-sheng; ZHANG Ye; WU Yun-gang

    2009-01-01

    @@ Tethered spinal cord syndrome(TCS)is a condition of overstretching or compression of the caudal part of the spinal cord caused by various spinal lesions,such as a tight filum terminale or an intraspinal lipoma.~(1-9) Though it is a well-recognized cause of neurological deterioration in childhood,its symptomatic onset in adulthood is uncommon.~(10-23) Eleven cases of TCS are presented here.In addition,their related clinical features,surgical procedures and outcomes are investigated.

  18. Spinal cord ischemia: aetiology, clinical syndromes and imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Weidauer, Stefan [Frankfurt Univ., Sankt Katharinen Hospital Teaching Hospital, Frankfurt am Main (Germany). Dept. of Neurology; Hattingen, Elke; Berkefeld, Joachim [Frankfurt Univ., Frankfurt am Main (Germany). Inst. of Neuroradiology; Nichtweiss, Michael

    2015-03-01

    The purpose of this study was to analyse MR imaging features and lesion patterns as defined by compromised vascular territories, correlating them to different clinical syndromes and aetiological aspects. In a 19.8-year period, clinical records and magnetic resonance imaging (MRI) features of 55 consecutive patients suffering from spinal cord ischemia were evaluated. Aetiologies of infarcts were arteriosclerosis of the aorta and vertebral arteries (23.6 %), aortic surgery or interventional aneurysm repair (11 %) and aortic and vertebral artery dissection (11 %), and in 23.6 %, aetiology remained unclear. Infarcts occurred in 38.2 % at the cervical and thoracic level, respectively, and 49 % of patients suffered from centromedullar syndrome caused by anterior spinal artery ischemia. MRI disclosed hyperintense pencil-like lesion pattern on T2WI in 98.2 %, cord swelling in 40 %, enhancement on post-contrast T1WI in 42.9 % and always hyperintense signal on diffusion-weighted imaging (DWI) when acquired. The most common clinical feature in spinal cord ischemia is a centromedullar syndrome, and in contrast to anterior spinal artery ischemia, infarcts in the posterior spinal artery territory are rare. The exclusively cervical location of the spinal sulcal artery syndrome seems to be a likely consequence of anterior spinal artery duplication which is observed preferentially here. (orig.)

  19. Central nociceptive sensitization vs. spinal cord training: opposing forms of plasticity that dictate function after complete spinal cord injury

    OpenAIRE

    Ferguson, Adam R.; Huie, J. Russell; Crown, Eric D; Grau, James W.

    2012-01-01

    The spinal cord demonstrates several forms of plasticity that resemble brain-dependent learning and memory. Among the most studied form of spinal plasticity is spinal memory for noxious (nociceptive) stimulation. Numerous papers have described central pain as a spinally-stored memory that enhances future responses to cutaneous stimulation. This phenomenon, known as central sensitization, has broad relevance to a range of pathological conditions. Work from the spinal cord injury (SCI) field in...

  20. Central nociceptive sensitization vs. spinal cord training: Opposing forms of plasticity that dictate function after complete spinal cord injury

    Directory of Open Access Journals (Sweden)

    Adam R Ferguson

    2012-10-01

    Full Text Available The spinal cord demonstrates several forms of plasticity that resemble brain-dependent learning and memory. Among the most studied form of spinal plasticity is spinal memory for noxious (nociceptive stimulation. Numerous papers have described central pain as a spinally-stored memory that enhances future responses to cutaneous stimulation. This phenomenon, known as central sensitization, has broad relevance to a range of pathological conditions. Work from the spinal cord injury (SCI field indicates that the lumbar spinal cord demonstrates several other forms of plasticity, including formal learning and memory. After complete thoracic SCI, the lumbar spinal cord can be trained by delivering stimulation to the hindleg when the leg is extended. In the presence of this response-contingent stimulation the spinal cord rapidly learns to hold the leg in a flexed position, a centrally mediated effect that meets the formal criteria for instrumental (response-outcome learning. Instrumental flexion training produces a central change in spinal plasticity that enables future spinal learning on both the ipsilateral and contralateral leg. However, if stimulation is given in a response-independent manner, the spinal cord develops central maladaptive plasticity that undermines future spinal learning on both legs. The present paper tests for interactions between spinal cord training and central nociceptive sensitization after complete spinal cord transection. We found that spinal training alters future central sensitization by intradermal formalin (24 h post-training. Conversely intradermal formalin impaired future spinal learning (24 h post-injection. Because the NMDA receptor has been implicated in formalin-induced central sensitization, we tested whether pretreatment with NMDA affects spinal learning. We found intrathecal NMDA impaired learning in a dose-dependent fashion, and that this effect endures for at least 24h. These data provide strong evidence for an

  1. Magnetic resonance imaging study of lumbosacral spinal cord nerves before artificial somatic-central nervous system-autonomic reflex pathway establish ment

    International Nuclear Information System (INIS)

    Objective: To investigate the value of MRI as imaging technique for lumbosacral spinal nerves before artificial somatic-central nervous system-autonomic reflex pathway establish ment. Methods: Conventional MRI and T2W CISS 3D were performed in 10 patients with neurogenic bladder planned for the operation of artificial somatic-central nervous system-autonomic reflex pathway. The Three-dimensional data were then constructed into composite images using a standard multiple planar reformation (MPR). Results: Five patients showed tethered spinal cord syndrome, whose spinal cord nerves were circuitous distributed and had abnormity number when penetrated the dura. Of these 5 patients, one patient was accompanied by spinal cord vas malformation. Four patients had vertebral fracture and spinal injury, and the other one patients demonstrated tumor in vertebral canal on MRI examinations. The spinal cord nerves in these 5 patients floated down river and had normal number of spinal cord nerves. Conclusion: Conventional MRI and T2W CISS 3D MRI were essential for the pre-operative planning of artificial somatic-central nervous system-autonomic reflex pathway, especially in patients with tethered spinal cord syndrome. Spinal cord nerves distribute and anterior and posterior roots array can be clearly showed by MPR. (authors)

  2. Tethered Cord Syndrome Secondary to the Unusual Constellation of a Split Cord Malformation, Lumbar Myelomeningocele, and Coexisting Neurenteric Cyst

    Directory of Open Access Journals (Sweden)

    Humphrey Okechi

    2012-01-01

    Full Text Available We describe a seminal case report of a child with a tethered cord syndrome secondary to the unusual constellation of a split cord malformation, lumbar myelomeningocele, and coexisting neurenteric cyst. A 17-year-old adolescent girl with a several-month history of myelopathy and urinary incontinence was examined whose spinal MRI scan demonstrated a type II split cord malformation with a large bone spur and an intradural neurenteric cyst in addition to lumbar myelomeningocele. Untethering of the spinal cord was achieved via a lumbar laminectomy. Pathological examination confirmed the intradural cyst to be a neurenteric cyst. Postoperatively there was stabilization of the neurological symptoms. Prophylactic surgery with total resection of the neurenteric cyst when feasible and spinal cord un-tethering appears to be associated with excellent outcomes.

  3. Reaction to topical capsaicin in spinal cord injury patients with and without central pain

    DEFF Research Database (Denmark)

    Finnerup, Nanna Brix; Pedersen, Louise H.; Terkelsen, Astrid J.;

    2007-01-01

    Central neuropathic pain is a debilitating and frequent complication to spinal cord injury (SCI). Excitatory input from hyperexcitable cells around the injured grey matter zone is suggested to play a role for central neuropathic pain felt below the level of a spinal cord injury. Direct evidence...... of a spinal cord injury which already is hyperexcitable, would cause enhanced responses in patients with central pain at the level of injury compared to patients without neuropathic pain and healthy controls. Touch, punctuate stimuli, cold stimuli and topical capsaicin was applied above, at, and below injury...... at the level of injury. Keywords: Spinal cord injury; Neuropathic pain; Capsaicin; Neuronal hyperexcitability; Hyperalgesia; Blood flow...

  4. Evaluationof efficiency of methods of neuromodulation in the treatment of spastic syndromes in patients with spinal cord injury

    OpenAIRE

    Smolkin А.А.; Ninel V.G.; Korshunova G.A.

    2014-01-01

    Objective: to evaluate the efficiency of the neuromodulation methods and to determine their role in complex rehabilitation of patients with spastic syndromes after spinal cord injury. Material and methods: Based on the study and treatment of 105 patients with spastic syndromes after injury of the spinal cord, electrical stimulation of the spinal cord, epidural drug therapy in combination with electrical nerve stimulation and local hypothermia spinal cord have been consistently applied for the...

  5. Intramedullary spinal cord metastasis from colonic carcinoma presenting as Brown-Sequard syndrome: a case report

    LENUS (Irish Health Repository)

    Kaballo, Mohammed A

    2011-08-02

    Abstract Introduction Intramedullary spinal cord metastasis is very rare. The majority are discovered incidentally during autopsy. Most symptomatic patients present with rapidly progressive neurological deficits and require immediate examination. Few patients demonstrate features of Brown-Séquard syndrome. Radiotherapy is the gold-standard of therapy for Intramedullary spinal cord metastasis. The overall prognosis is poor and the mortality rate is very high. We present what is, to the best of our knowledge, the first case of Intramedullary spinal cord metastasis of colorectal carcinoma presenting as Brown-Séquard syndrome. Case presentation We present the case of a 71-year-old Caucasian man with colonic adenocarcinoma who developed Intramedullary spinal cord metastasis and showed features of Brown-Séquard syndrome, which is an uncommon presentation of Intramedullary spinal cord metastasis. Conclusion This patient had an Intramedullary spinal cord metastasis, a rare form of metastatic disease, secondary to colonic carcinoma. The metastasis manifested clinically as Brown-Séquard syndrome, itself a very uncommon condition. This syndrome is rarely caused by intramedullary tumors. This unique case has particular interest in medicine, especially for the specialties of medical, surgical and radiation oncology. We hope that it will add more information to the literature about these entities.

  6. Complications after spinal anesthesia in adult tethered cord syndrome.

    Science.gov (United States)

    Liu, Jing-Jie; Guan, Zheng; Gao, Zhen; Xiang, Li; Zhao, Feng; Huang, Sheng-Li

    2016-07-01

    Since little has been reported about complications of spinal anesthesia in adult tethered cord syndrome (TCS), we sought to delineate the characteristics of the condition.A total of 4 cases of adult TCS after spinal anesthesia were reviewed. The medical charts of the patients were obtained. Anesthesia, which was combined spinal and epidural anesthesia or spinal anesthesia was performed, and follow-up were carried out in all patients.The most common neurological symptom of adult TCS before surgery was occasional severe pain in back, perineal region, or legs. Frequent micturition, diminished knee and ankle reflexes, and difficulty in bending were exhibited in partial patients. Paraesthesia of perineal region or/and lower extremities existed 2 to 3 days after spinal anesthesia in all the cases. Weakness of lower extremities existed in 1 case. Lumbar magnetic resonance imaging showed the low location of conus medullaris. At follow-up, 3 cases recovered completely within 3 weeks, and 1 case underwent permanent disability.These cases suggest anesthesiologists and surgeons alert to the association of adult TCS and spinal anesthesia. Spinal anesthesia should be prohibited in patients with adult TCS to prevent neurological damages. PMID:27442670

  7. MR diagnosis and clinical management of whiplash injury syndrome of spinal cord

    International Nuclear Information System (INIS)

    Objective: To study the MR manifestations of whiplash injury syndrome of spinal cord. Methods: MR images of 21 cases diagnosed as whiplash injury syndrome were retrospectively studied. Those images included transverse and sagittal views and coronal scan had been performed in some cases. Results: MRI inspection safely and objectively reveals the extent of the spinal injury, and helps the anticipation of the prognosis. Conclusion: MRI is the first choice of the imaging modalities assessing the whiplash injury syndrome of the spinal cord. An early diagnosis is valuable to clinical management and rehabilitation

  8. Loss of central inhibition: implications for behavioral hypersensitivity after contusive spinal cord injury in rats.

    Science.gov (United States)

    Berrocal, Yerko A; Almeida, Vania W; Puentes, Rocio; Knott, Eric P; Hechtman, Jaclyn F; Garland, Mary; Pearse, Damien D

    2014-01-01

    Behavioral hypersensitivity is common following spinal cord injury (SCI), producing significant discomfort and often developing into chronic pain syndromes. While the mechanisms underlying the development of behavioral hypersensitivity after SCI are poorly understood, previous studies of SCI contusion have shown an increase in amino acids, namely, aspartate and glutamate, along with a decrease in GABA and glycine, particularly below the injury. The current study sought to identify alterations in key enzymes and receptors involved in mediating central inhibition via GABA and glycine after a clinically-relevant contusion SCI model. Following thoracic (T8) 25.0 mm NYU contusion SCI in rodents, significant and persistent behavioral hypersensitivity developed as evidenced by cutaneous allodynia and thermal hyperalgesia. Biochemical analyses confirmed upregulation of glutamate receptor GluR3 with downregulation of the GABA synthesizing enzyme (GAD65/67) and the glycine receptor α3 (GLRA3), notably below the injury. Combined, these changes result in the disinhibition of excitatory impulses and contribute to behavioral hyperexcitability. This study demonstrates a loss of central inhibition and the development of behavioral hypersensitivity in a contusive SCI paradigm. Future use of this model will permit the evaluation of different antinociceptive strategies and help in the elucidation of new targets for the treatment of neuropathic pain. PMID:25180088

  9. Loss of Central Inhibition: Implications for Behavioral Hypersensitivity after Contusive Spinal Cord Injury in Rats

    Directory of Open Access Journals (Sweden)

    Yerko A. Berrocal

    2014-01-01

    Full Text Available Behavioral hypersensitivity is common following spinal cord injury (SCI, producing significant discomfort and often developing into chronic pain syndromes. While the mechanisms underlying the development of behavioral hypersensitivity after SCI are poorly understood, previous studies of SCI contusion have shown an increase in amino acids, namely, aspartate and glutamate, along with a decrease in GABA and glycine, particularly below the injury. The current study sought to identify alterations in key enzymes and receptors involved in mediating central inhibition via GABA and glycine after a clinically-relevant contusion SCI model. Following thoracic (T8 25.0 mm NYU contusion SCI in rodents, significant and persistent behavioral hypersensitivity developed as evidenced by cutaneous allodynia and thermal hyperalgesia. Biochemical analyses confirmed upregulation of glutamate receptor GluR3 with downregulation of the GABA synthesizing enzyme (GAD65/67 and the glycine receptor α3 (GLRA3, notably below the injury. Combined, these changes result in the disinhibition of excitatory impulses and contribute to behavioral hyperexcitability. This study demonstrates a loss of central inhibition and the development of behavioral hypersensitivity in a contusive SCI paradigm. Future use of this model will permit the evaluation of different antinociceptive strategies and help in the elucidation of new targets for the treatment of neuropathic pain.

  10. CD11d integrin blockade reduces the systemic inflammatory response syndrome after spinal cord injury

    OpenAIRE

    Bao, Feng; Brown, Arthur; Dekaban, Gregory A.; Omana, Vanessa; Weaver, Lynne C.

    2011-01-01

    Traumatic injury to the spinal cord triggers a systemic inflammatory response syndrome (SIRS), in which inflammatory cells from the circulation invade organs such as the liver, lung and kidney, leading to damage of these organs. Our previous study (Gris, et al, Exp. Neurol, 2008) demonstrated that spinal cord injury (SCI) activates circulating neutrophils that then invade the lung and kidney from 2 to 24 h after injury, increasing myeloperoxidase activity, cyclooxygenase-2 and matrix metallop...

  11. Apoptosis of lumbar spinal cord neurons in cauda equina syndrome rats

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Objective To explore the law of apoptosis of lumbar spinal cord neurons in cauda equina syndrome (CES). Methods Cauda equina of rats was compressed by a piece of silica gel stick. From day 1 to day 28,the lumbar spinal cord specimens were harvested and assessed by Nissl's staining and TUNEL staining. Results Compression of cauda equina caused lesion and apoptosis of neurons in lumbar spinal cord,and the extent of apoptosis reached the peak on 7th day after compression. Conclusion Apoptosis of neurons in lum...

  12. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    Science.gov (United States)

    ... About NINDS Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet See a list of all NINDS ... 496-5717 "Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet", NINDS, Publication date July 2011. NIH ...

  13. Metabolic syndrome and central retinal artery occlusion

    Directory of Open Access Journals (Sweden)

    Kosanović-Jaković Natalija

    2005-01-01

    Full Text Available Background. The accumulation of risk factors for central retinal artery occlusion can be seen in a single person and might be explained by the metabolic syndrome. Case report. We presented the case of a 52-year-old man with no light perception in his right eye. The visual loss was monocular and painless, fundoscopy showed central retinal artery occlusion and the laboratory investigation showed the raised erythrocyte sedimentation rate of 105 mm/h and the raised C-reactive protein of 22 mg/l. Specific laboratory investigations and fluorescein angiography excluded the presence of vasculitis, collagen vascular diseases, hypercoagulable state and antiphospholipid syndrome. Conclusion. The patient met all the five of the National Cholesterol Education Program (NCEP criteria for the metabolic syndrome: hypertension, abnormal lipid profile, abnormal glucose metabolism, obesity and hyperuricemia. Measurement of C-reactive protein is useful for the assessment of therapeutic systemic effect on any abnormality in the metabolic syndrome. Individual therapy for all risk factors in the metabolic syndrome is necessary to prevent complications such as cardiovascular, retinal vascular diseases and stroke.

  14. Ischemic spinal cord syndrome after transthoracic esophagectomy: two cases of a rare neurologic complication.

    Science.gov (United States)

    Zantl, N; Stein, H J; Brücher, B L; Bartels, H; Siewert, J R

    2000-01-01

    Anterior spinal artery syndrome (ASAS) is a rare complication after surgery of the thoracic or abdominal aorta. The sulco commissuralis syndrome represents a partial or incomplete ASAS. We report two cases of ischemic spinal cord syndromes after transthoracic esophagectomy. This represents a prevalence of this syndrome of 0.2% in more than 1000 consecutive esophagectomies performed at our institution. Patient 1 developed an ASAS on the first day after esophagectomy. Patient 2 showed the pathognomonic clinical signs associated with sulco commissuralis syndrome after an asymptomatic window. In both patients, the extent of the neurologic symptoms initially improved but then remained unchanged for the rest of the follow-up of 9 and 12 months. Although the prognosis of neurologic syndromes resulting from spinal cord infarction is poor, preoperative tests to identify patients at risk appear not to be justified because of the very low incidence of these syndromes after esophagectomy and the poor sensitivity and specificity of currently available diagnostic modalities. However, the possibility of ischemic spinal cord syndrome should be kept in mind when patients present with neurologic symptoms after esophagectomy. PMID:11284985

  15. Solitary median maxillary central incisor (SMMCI) syndrome

    OpenAIRE

    Hall Roger K

    2006-01-01

    Abstract Solitary median maxillary central incisor syndrome (SMMCI) is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35th–38th day(s) from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI. The SMMCI tooth differs from the normal central incisor, in that the crown form is symmetric; i...

  16. Metabolic syndrome and central retinal artery occlusion

    OpenAIRE

    Kosanović-Jaković Natalija; Petrović Lidija; Risimić Dijana; Milenković Svetislav; Matić Danica

    2005-01-01

    Background. The accumulation of risk factors for central retinal artery occlusion can be seen in a single person and might be explained by the metabolic syndrome. Case report. We presented the case of a 52-year-old man with no light perception in his right eye. The visual loss was monocular and painless, fundoscopy showed central retinal artery occlusion and the laboratory investigation showed the raised erythrocyte sedimentation rate of 105 mm/h and the raised C-reactive protein of 22 mg/l. ...

  17. Peripheral chemoreceptors in congenital central hypoventilation syndrome.

    Science.gov (United States)

    Perez, Iris A; Keens, Thomas G

    2013-01-01

    Congenital central hypoventilation syndrome is a rare disorder caused by a mutation in the PHOX2B gene resulting in hypoventilation that is worse during sleep. Human physiologic studies show that patients with CCHS have absent or decreased rebreathing ventilatory responses to hypercapnia and hypoxemia during sleep as well as during wakefulness. Some ventilatory responses to hypoxia and hyperoxia can be demonstrated using a step change in inspired oxygen. However, these suggest that both central and peripheral chemoreceptor functions are generally defective in all states in children with CCHS. The defect in CCHS may lie in central nervous system pathways regulating ventilation, whose development and function are controlled by PHOX2B. Moreover, the retrotrapezoid nucleus (RTN) may be the major defect in CCHS, where central and peripheral inputs converge. Human physiological studies predicted that the defect in CCHS lies in central integration of the central and peripheral chemoreceptor signals. New evidence suggests the RTN may be the respiratory controller where chemoreceptor inputs are integrated. In this review we present the clinical presentation of CCHS, revisit results of human physiologic studies, and discuss the findings in light of new knowledge about the role of PHOX2B and RTN in CCHS.

  18. Postpartum spinal cord injury in a woman with HELLP syndrome.

    NARCIS (Netherlands)

    Groothuis, J.T.; Kuppevelt, DH van

    2008-01-01

    OBJECTIVE: To report a rare cause of spinal cord injury. STUDY DESIGN: Case report. CASE REPORT: A 36-year-old woman presented with acute onset of paresis of the upper and lower extremity (level C5, ASIA B) the day after delivering a healthy daughter (39 weeks' gestation). Prior to giving birth, she

  19. Central sensitization in spinal cord injured humans assessed by reflex receptive fields

    DEFF Research Database (Denmark)

    Biurrun Manresa, José Alberto; Finnerup, Nanna Susanne Brix; Johannesen, Inger Lauge;

    2014-01-01

    OBJECTIVE: To investigate the effects of central sensitization, elicited by intramuscular injection of capsaicin, by comparing the reflex receptive fields (RRF) of spinally-intact volunteers and spinal cord injured volunteers that present presensitized spinal nociceptive mechanisms. METHODS......: Fifteen volunteers with complete spinal cord injury (SCI) and fourteen non-injured (NI) volunteers participated in the experiment. Repeated electrical stimulation was applied on eight sites on the foot sole to elicit the nociceptive withdrawal reflex (NWR). RRF were assessed before, 1min after and 60min...

  20. A Review of Select Centralized Pain Syndromes

    Directory of Open Access Journals (Sweden)

    David R. Spiegel

    2015-01-01

    Full Text Available Pain can be broadly divided into 3 classes, including nociceptive or inflammatory pain (protective, neuropathic (pathological, occurring after damage to the nervous system, or centralized (pathological, due to abnormal function but with no damage or inflammation to the nervous system. The latter has been posited to occur when descending analgesic pathways are attenuated and/or glutamatergic transmission is facilitated. Additionally, this “pain prone phenotype” can be associated with early life trauma and a suboptimal response to opiates. This article will review the relationships between centralized pain syndromes (ie, fibromyalgia, chronic low back pain, childhood sexual abuse, and opiate misuse. Finally, treatment implications, potentially effecting primary care physicians, will be discussed.

  1. Toxoplasmosis of spinal cord in acquired immunodeficiency syndrome patient presenting as paraparesis: A rare entity

    Directory of Open Access Journals (Sweden)

    Sachin R Agrawal

    2014-01-01

    Full Text Available Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.

  2. Disc prolapse and cord contusion in a case of Klippel-Feil syndrome following minor trauma

    Directory of Open Access Journals (Sweden)

    Agrawal Amit

    2009-01-01

    Full Text Available Klippel-Feil syndrome (KFS is defined as congenital fusion of two or more cervical vertebrae and patients with KFS are frequently asymptomatic. However, these patients are especially prone to cervical cord injury after a minor fall or a major traumatic episode. We report an unusual case of KFS where the patient had disc prolapse between two Klippel-Feil segments and discuss the difficulties in the management of this case.

  3. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Science.gov (United States)

    Lo, Tony Chung Tung; Yeung, Stephen Tung; Lee, Sujin; Skavinski, Kira; Liao, Solomon

    2016-01-01

    Objective Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of physiological balance between pain inhibition and facilitation. The present report shows that this treatment option can be used in patients with refractory central pain syndrome in the setting of spinal cord myelopathy secondary to Ehlers–Danlos syndrome. In addition, as seen in this case, this protocol can potentially decrease the chronic use of pain medication, such as opioids.

  4. Association between chromosomal aberration of COX8C and tethered spinal cord syndrome:array-based comparative genomic hybridization analysis

    Institute of Scientific and Technical Information of China (English)

    Qiu-jiong Zhao; Shao-cong Bai; Cheng Cheng; Ben-zhang Tao; Le-kai Wang; Shuang Liang; Ling Yin; Xing-yi Hang; Ai-jia Shang

    2016-01-01

    Copy number variations have been found in patients with neural tube abnormalities. In this study, we performed genome-wide screening using high-resolution array-based comparative genomic hybridization in three children with tethered spinal cord syndrome and two healthy parents. Of eight copy number variations, four were non-polymorphic. These non-polymorphic copy number variations were associated with Angelman and Prader-Willi syndromes, and microcephaly. Gene function enrichment analysis revealed that COX8C, a gene associated with metabolic disorders of the nervous system, was located in the copy number variation region of Patient 1. Our results indicate that ar-ray-based comparative genomic hybridization can be used to diagnose tethered spinal cord syndrome. Our results may help determine the pathogenesis of tethered spinal cord syndrome and prevent occurrence of this disease.

  5. Association between chromosomal aberration of COX8C and tethered spinal cord syndrome: array-based comparative genomic hybridization analysis.

    Science.gov (United States)

    Zhao, Qiu-Jiong; Bai, Shao-Cong; Cheng, Cheng; Tao, Ben-Zhang; Wang, Le-Kai; Liang, Shuang; Yin, Ling; Hang, Xing-Yi; Shang, Ai-Jia

    2016-08-01

    Copy number variations have been found in patients with neural tube abnormalities. In this study, we performed genome-wide screening using high-resolution array-based comparative genomic hybridization in three children with tethered spinal cord syndrome and two healthy parents. Of eight copy number variations, four were non-polymorphic. These non-polymorphic copy number variations were associated with Angelman and Prader-Willi syndromes, and microcephaly. Gene function enrichment analysis revealed that COX8C, a gene associated with metabolic disorders of the nervous system, was located in the copy number variation region of Patient 1. Our results indicate that array-based comparative genomic hybridization can be used to diagnose tethered spinal cord syndrome. Our results may help determine the pathogenesis of tethered spinal cord syndrome and prevent occurrence of this disease. PMID:27651783

  6. Association between chromosomal aberration of COX8C and tethered spinal cord syndrome: array-based comparative genomic hybridization analysis

    Directory of Open Access Journals (Sweden)

    Qiu-jiong Zhao

    2016-01-01

    Full Text Available Copy number variations have been found in patients with neural tube abnormalities. In this study, we performed genome-wide screening using high-resolution array-based comparative genomic hybridization in three children with tethered spinal cord syndrome and two healthy parents. Of eight copy number variations, four were non-polymorphic. These non-polymorphic copy number variations were associated with Angelman and Prader-Willi syndromes, and microcephaly. Gene function enrichment analysis revealed that COX8C, a gene associated with metabolic disorders of the nervous system, was located in the copy number variation region of Patient 1. Our results indicate that array-based comparative genomic hybridization can be used to diagnose tethered spinal cord syndrome. Our results may help determine the pathogenesis of tethered spinal cord syndrome and prevent occurrence of this disease.

  7. [Subcutaneous stimulation as additional therapy to spinal cord stimulation in a post-laminectomy syndrome patient].

    Science.gov (United States)

    Akbaş, Mert; Yeğin, Mehmet Arif; Özdemir, İrem; Göksu, Ethem; Akyüz, Mahmut

    2016-01-01

    Spinal cord stimulation as treatment of chronic low back pain via neuromodulation has been frequently performed in recent years. The dorsal column is stimulated by an electrode placed at the epidural region. In the case presently described, subcutaneous lead was implanted in a patient with failed back syndrome after spinal cord stimulation was inadequate to treat back and gluteal pain. A 65-year-old male had undergone surgery to treat lumbar disc herniation, after which he received physical therapy and multiple steroid injections due to unrelieved pain. He was admitted to the pain clinic with pain radiating to right gluteal muscle and leg. Spinal cord stimulation was performed and, as pain was not relieved, subcutaneous lead was applied to the right cluneal nerve distribution. Following treatment, the patient scored 1-2 on visual analog scale. Pain had been reduced by over 80%. Octad electrode was placed between T8 and T10 vertebrae after Tuohy needle was introduced to intervertebral area between L1 and L2. Paresthesia occurred in the right extremity. Boundaries were determined by area of right gluteal region in which paresthesia did not occur. Octad electrode was placed subcutaneously after vertical line was drawn from center point. Paresthesia occurred throughout the region. Pulse wave was 390-450 msec; frequency was 10-30 Hz. Subcutaneous electrode replacement is effective additional therapy when pain is not relieved by spinal cord stimulation. PMID:27225614

  8. Solitary median maxillary central incisor (SMMCI) syndrome.

    Science.gov (United States)

    Hall, Roger K

    2006-01-01

    Solitary median maxillary central incisor syndrome (SMMCI) is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35th-38th day(s) from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI. The SMMCI tooth differs from the normal central incisor, in that the crown form is symmetric; it develops and erupts precisely in the midline of the maxillary dental arch in both primary and permanent dentitions. Congenital nasal malformation (choanal atresia, midnasal stenosis or congenital pyriform aperture stenosis) is positively associated with SMMCI. The presence of an SMMCI tooth can predict associated anomalies and in particular the serious anomaly holoprosencephaly. Common congenital anomalies associated with SMMCI are: severe to mild intellectual disability, congenital heart disease, cleft lip and/or palate and less frequently, microcephaly, hypopituitarism, hypotelorism, convergent strabismus, oesophageal and duodenal atresia, cervical hemivertebrae, cervical dermoid, hypothyroidism, scoliosis, absent kidney, micropenis and ambiguous genitalia. Short stature is present in half the children. Diagnosis should be made by eight months of age, but can be made at birth and even prenatally at 18-22 weeks from the routine mid-trimester ultrasound scan. Management depends upon the individual anomalies present. Choanal stenosis requires emergency surgical treatment. Short stature may require growth hormone therapy. SMMCI tooth itself is mainly an aesthetic problem, which is ideally managed by combined orthodontic, prosthodontic and oral surgical treatment; alternatively, it can be left untreated. PMID:16722608

  9. Solitary median maxillary central incisor (SMMCI syndrome

    Directory of Open Access Journals (Sweden)

    Hall Roger K

    2006-04-01

    Full Text Available Abstract Solitary median maxillary central incisor syndrome (SMMCI is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s operating in utero about the 35th–38th day(s from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F at 7q36 may be associated with SMMCI. The SMMCI tooth differs from the normal central incisor, in that the crown form is symmetric; it develops and erupts precisely in the midline of the maxillary dental arch in both primary and permanent dentitions. Congenital nasal malformation (choanal atresia, midnasal stenosis or congenital pyriform aperture stenosis is positively associated with SMMCI. The presence of an SMMCI tooth can predict associated anomalies and in particular the serious anomaly holoprosencephaly. Common congenital anomalies associated with SMMCI are: severe to mild intellectual disability, congenital heart disease, cleft lip and/or palate and less frequently, microcephaly, hypopituitarism, hypotelorism, convergent strabismus, oesophageal and duodenal atresia, cervical hemivertebrae, cervical dermoid, hypothyroidism, scoliosis, absent kidney, micropenis and ambiguous genitalia. Short stature is present in half the children. Diagnosis should be made by eight months of age, but can be made at birth and even prenatally at 18–22 weeks from the routine mid-trimester ultrasound scan. Management depends upon the individual anomalies present. Choanal stenosis requires emergency surgical treatment. Short stature may require growth hormone therapy. SMMCI tooth itself is mainly an aesthetic problem, which is ideally managed by combined orthodontic, prosthodontic and oral surgical treatment; alternatively, it can be left untreated.

  10. Umbilical cord blood and neonatal endothelin-1 levels in preterm newborns with and without respiratory distress syndrome

    OpenAIRE

    A.C.W. Benjamin; R.C. Silveira; R.S. Procianoy

    2005-01-01

    Increased pulmonary vascular resistance in preterm newborn infants with respiratory distress syndrome is suggested, and endothelin-1 plays an important role in pulmonary vascular reactivity in newborns. We determined umbilical cord blood and neonatal (second sample) levels of endothelin-1 in 18 preterm newborns with respiratory distress syndrome who had no clinical or echocardiographic diagnosis of pulmonary hypertension and 22 without respiratory distress syndrome (gestational ages: 31.4 ± 1...

  11. Klippel-Feil syndrome – the risk of cervical spinal cord injury: A case report

    Directory of Open Access Journals (Sweden)

    Singh Gurpreet

    2002-04-01

    Full Text Available Abstract Background Klippel-Feil syndrome is defined as congenital fusion of two or more cervical vertebrae and is believed to result from faulty segmentation along the embryo's developing axis during weeks 3–8 of gestation. Persons with Klippel-Feil syndrome and cervical stenosis may be at increased risk for spinal cord injury after minor trauma as a result of hypermobility of the various cervical segments. Persons with Klippel-Feil Syndrome often have congenital anomalies of the urinary tract as well. Case presentation A 51-year male developed incomplete tetraplegia in 1997 when he slipped and fell backwards hitting his head on the floor. X-rays of cervical spine showed fusion at two levels: C2 and C3 vertebrae, and C4 and C5 vertebrae. Intravenous urography (IVU revealed no kidneys in the renal fossa on both sides, but the presence of crossed, fused renal ectopia in the left ilio-lumbar region. This patient had a similar cervical spinal cord injury about 15 years ago, when he developed transient numbness and paresis of the lower limbs following a fall. Discussion and Conclusion 1 Persons with Klippel-Feil syndrome should be made aware of the increased risk of sustaining transient neurologic deterioration after minor trauma if there is associated radiographic evidence of spinal stenosis. 2 Patients with Klippel-Feil syndrome often have congenital anomalies of the urinary tract. Our patient had crossed, fused, ectopia of kidney. 3 When patients with Klippel-Feil syndrome sustain tetraplegia they have increased chances of developing urinary tract calculi. Treatment of kidney stones may pose a challenge because of associated renal anomalies. 4 Health professionals caring for cervical spinal cord injury patients with Klippel-Feil syndrome and renal anomalies should place emphasis on prevention of kidney stones. A large fluid intake is recommended for these patients, as a high intake of fluids is still the most powerful and certainly the most

  12. Monoaminergic and catecholaminergic activation of the central pattern generator for locomotion following spinal cord injury Innovative therapeutic approaches

    Institute of Scientific and Technical Information of China (English)

    Pierre A.Guertin

    2009-01-01

    The development of secondary health complications following spinal cord injury has been increasingly recognized by healthcare professionals as a major concern.These problems most specifically affect complete or near-complete spinal cord injury patients (e.g.,those with minimal mobility),who are not typically rehabilitated with treadmill training approaches,because motor control and leg movements are largely impaired.However,recent pharmaceutical advances in central pattern generator activation may provide new therapeutic hopes for these spinal cord injury patients.This article provides a comprehensive overview,for the non-specialist,of the most recent advances in this field.

  13. Diagnosis and management of traumatic cervical central spinal cord injury: A review

    Directory of Open Access Journals (Sweden)

    Nancy E Epstein

    2015-01-01

    Full Text Available Background: The classical clinical presentation, neuroradiographic features, and conservative vs. surgical management of traumatic cervical central spinal cord (CSS injury remain controversial. Methods: CSS injuries, occurring in approximately 9.2% of all cord injuries, are usually attributed to significant hyperextension trauma combined with congenital/acquired cervical stenosis/spondylosis. Patients typically present with greater motor deficits in the upper vs. lower extremities accompanied by patchy sensory loss. T2-weighted magnetic resonance (MR scans usually show hyperintense T2 intramedullary signals reflecting acute edema along with ligamentous injury, while noncontrast computed tomography (CT studies typically show no attendant bony pathology (e.g. no fracture, dislocation. Results: CSS constitute only a small percentage of all traumatic spinal cord injuries. Aarabi et al. found CSS patients averaged 58.3 years of age, 83% were male and 52.4% involved accidents/falls in patients with narrowed spinal canals (average 5.6 mm; their average American Spinal Injury Association (ASIA motor score was 63.8, and most pathology was at the C3-C4 and C4-C5 levels (71%. Surgery was performed within 24 h (9 patients, 24-48 h (10 patients, or after 48 h (23 patients. In the Brodell et al. study of 16,134 patients with CSS, 39.7% had surgery. In the Gu et al. series, those with CSS and stenosis/ossification of the posterior longitudinal ligament (OPLL exhibited better outcomes following laminoplasty. Conclusions: Recognizing the unique features of CSS is critical, as the clinical, neuroradiological, and management strategies (e.g. conservative vs. surgical management: early vs. late differ from those utilized for other spinal cord trauma. Increased T2-weighted MR images best document CSS, while CT studies confirm the absence of fracture/dislocation.

  14. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Lo TC

    2016-09-01

    Full Text Available Tony Chung Tung Lo,1,* Stephen Tung Yeung,2,* Sujin Lee,1 Kira Skavinski,3 Solomon Liao,4 1Department of Physical Medicine and Rehabilitation, University of California Irvine, Orange, CA, 2Department of Immunology, University of Connecticut School of Medicine, Farmington, CT, 3Department of Palliative Medicine, University of California San Diego, La Jolla, 4Department of Palliative Medicine, University of California Irvine, Orange, CA, USA *These authors contributed equally to this work Objective: Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report: A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion: Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of

  15. Predicting chronic stinger syndrome using the mean subaxial space available for the cord index.

    Science.gov (United States)

    Greenberg, Jared; Leung, Dan; Kendall, Jenny

    2011-05-01

    A 21-year-old division I collegiate football player who had a history of several stingers presented with 5 days of persistent left neck and shoulder pain associated with paresthesias and upper extremity weakness. His symptoms began immediately during a game when he was struck on the right side of his helmet, which induced a compression-extension mechanism of injury to his neck. Clinical and electrodiagnostic evaluation was consistent with a left C5 radiculopathy, but magnetic resonance imaging of the cervical spine yielded normal results. The mean subaxial cervical space available for the cord (MSCSAC) index is a novel tool to predict chronic stinger syndrome. It is calculated by subtracting the sagittal diameter of the spinal cord from the disc-level sagittal diameter of the spinal canal at levels C3 through C6 and then averaging these values. A cutoff of < 4.3 mm has been shown to predict a greater-than-13-fold increase in risk of developing chronic stinger syndrome. This patient had a MSCSAC index of 3.2 mm, which correlated with his history of multiple stingers. The MSCSAC index may be a useful tool to help counsel athletes on the risk of developing future stingers, although more extensive research on this measurement tool is indicated.

  16. Central and peripheral actions of the NSAID ketoprofen on spinal cord nociceptive reflexes.

    Science.gov (United States)

    Herrero, J F; Parrado, A; Cervero, F

    1997-10-01

    Ketoprofen is a non-steroidal antiinflammatory drug (NSAID) which provides effective analgesia in situations of pain provoked by tissue inflammation. However, the location of its analgesic effects, (peripheral tissues versus central nervous system), have not been clearly identified and separated. In the present study the effectiveness of ketoprofen was examined in two different types of experiments: (i) Open field behavioural tests in conscious rats, and (ii) spinal cord nociceptive reflexes (single motor units) activated by electrical and thermal stimulation in chloralose anaesthetised rats. The experiments were performed in rats with carrageenan-induced inflammation of one hindpaw, or of one knee joint. The administration of ketoprofen significantly inhibited the reduction of exploratory movements caused by inflammation in open field experiments. Ketoprofen was also effective in depressing reflex activity evoked by electrical and noxious thermal stimulation of the skin, either in inflamed tissue or in normal tissue of monoarthritic animals. It was also effective in the reduction of reflex wind-up; a phenomenon in which the activity of spinal cord neurones increases progressively with high frequency electrical stimulation. We therefore conclude that ketoprofen has central as well as peripheral analgesic activity.

  17. Protein phosphatase 2A regulates central sensitization in the spinal cord of rats following intradermal injection of capsaicin

    Directory of Open Access Journals (Sweden)

    Fang Li

    2006-03-01

    Full Text Available Abstract Background Intradermal injection of capsaicin into the hind paw of rats induces spinal cord central sensititzation, a process in which the responsiveness of central nociceptive neurons is amplified. In central sensitization, many signal transduction pathways composed of several cascades of intracellular enzymes are involved. As the phosphorylation state of neuronal proteins is strictly controlled and balanced by the opposing activities of protein kinases and phosphatases, the involvement of phosphatases in these events needs to be investigated. This study is designed to determine the influence of serine/threonine protein phosphatase type 2A (PP2A on the central nociceptive amplification process, which is induced by intradermal injection of capsaicin in rats. Results In experiment 1, the expression of PP2A protein in rat spinal cord at different time points following capsaicin or vehicle injection was examined using the Western blot method. In experiment 2, an inhibitor of PP2A (okadaic acid, 20 nM or fostriecin, 30 nM was injected into the subarachnoid space of the spinal cord, and the spontaneous exploratory activity of the rats before and after capsaicin injection was recorded with an automated photobeam activity system. The results showed that PP2A protein expression in the spinal cord was significantly upregulated following intradermal injection of capsaicin in rats. Capsaicin injection caused a significant decrease in exploratory activity of the rats. Thirty minutes after the injection, this decrease in activity had partly recovered. Infusion of a phosphatase inhibitor into the spinal cord intrathecal space enhanced the central sensitization induced by capsaicin by making the decrease in movement last longer. Conclusion These findings indicate that PP2A plays an important role in the cellular mechanisms of spinal cord central sensitization induced by intradermal injection of capsaicin in rats, which may have implications in

  18. Impact of spinal cord stimulation on sensory characteristics in complex regional pain syndrome type I - A randomized trial

    NARCIS (Netherlands)

    Kemler, MA; Reulen, JPH; Barendse, GAM; van Kleef, M; de Vet, HCW; van den Wildenberg, FAJM

    2001-01-01

    Background: A randomized trial was performed to assess the effect of spinal cord stimulation (SCS) on detection and pain thresholds for pressure, warmth, and cold and on the extent of mechanical hyperalgesia in patients with chronic complex regional pain syndrome type I. Methods: Fifty-four chronic

  19. Sjogrens Syndrome Presenting with Central Nervous System Involvement

    Directory of Open Access Journals (Sweden)

    Tülay Terzi

    2012-01-01

    Full Text Available Sjogren’s syndrome is a slowly progressive autoimmune disease. Neurological involvement occurs in approximately 20-25% cases in Sjogren’s syndrome. 87% of the neurological involvement is peripheral nervous system, almost 13% in the form of central nervous system involvement. Affected central nervous system may show similar clinical and radiological findings as in multiple sclerosis (MS. In this paper, a 43-year-old patient is discussed who was referred with the complaint of dizziness, there was MS- like lesions in brain imaging studies and was diagnosed with Sjogren’s syndrome. MS- like clinical and radiologic tables can be seen, albeit rarely in Sjogren’s syndrome. In these cases, early diagnosis and early treatment for the sjögren has a great importance for the prognosis of the disease.

  20. Paraneoplastic Syndromes of the Central Nervous System

    NARCIS (Netherlands)

    J.W.B. Moll (Wibe)

    1996-01-01

    textabstractIn recent years a continuous stream of new information on clinical, pathological and immunological aspects of paraneoplastic neurological syndromes has been published. In this survey, we will discuss current opinions on the value of anti-neuronal antibody detection for establishing a dia

  1. Diaphragm pacers as a treatment for congenital central hypoventilation syndrome.

    Science.gov (United States)

    Chen, Maida Lynn; Tablizo, Mary Anne; Kun, Sheila; Keens, Thomas G

    2005-09-01

    Congenital central hypoventilation syndrome is a rare syndrome present from birth, and is defined as the failure of automatic control of breathing. All patients with congenital central hypoventilation syndrome require life-long ventilatory support during sleep, although approximately a third of patients require ventilatory support 24 h per day. Diaphragm pacers offer a modality of ventilatory support that affords congenital central hypoventilation syndrome patients with maximal mobility for full-time ventilatory patients, and they may allow for a more normal lifestyle in the appropriate patient. They may permit tracheostomy decannulation in those requiring only support during sleep. Diaphragm pacing entails surgical placement of an electrode onto the phrenic nerve, connected to a subcutaneous receiver. There is an external battery-operated transmitter and antenna placed on the skin over the receiver. The transmitter emits energy, similar to radio transmission, which is converted into an electrical current by the receiver. This stimulates the phrenic nerve resulting in a diaphragmatic contraction. Settings on the transmitter include respiratory rate and electrical voltage, and are adjusted to give enough tidal volume to allow for adequate oxygenation and ventilation. Therefore, diaphragm pacing is an attractive alternative mode of mechanically assisted ventilation for many patients with congenital central hypoventilation syndrome.

  2. Guillain-Barre syndrome: A possibility in a spinal cord injured patient

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    Jagatsinh Yogendrasinh

    2007-01-01

    Full Text Available A 28-year-old male had paraplegia as a result of fracture dislocation of T12/L1 six years ago. He was functioning independently until four weeks ago, when he started complaining of trunkal paraesthesia which later progressed to include the upper extremities. The initial diagnosis was that of posttraumatic syringomyelia (PTS. While awaiting the MRI scan he developed weakness of upper limbs. The weakness restricted his self-care activities including transfers. The MRI did not show any evidence of syringomyelia. Neurological consultation and assessment yielded provisional diagnosis of Guillain-Barre syndrome (GBS. The patient was treated with immunoglobulins and regained 90% of his previous neurological status. This case is reported to raise awareness among clinicians to include the possibility of the GBS in the differential diagnosis of progressive neurological loss on top of existing neurological deficiency in spinal cord injured patients.

  3. Umbilical cord blood and neonatal endothelin-1 levels in preterm newborns with and without respiratory distress syndrome

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    A.C.W. Benjamin

    2005-09-01

    Full Text Available Increased pulmonary vascular resistance in preterm newborn infants with respiratory distress syndrome is suggested, and endothelin-1 plays an important role in pulmonary vascular reactivity in newborns. We determined umbilical cord blood and neonatal (second sample levels of endothelin-1 in 18 preterm newborns with respiratory distress syndrome who had no clinical or echocardiographic diagnosis of pulmonary hypertension and 22 without respiratory distress syndrome (gestational ages: 31.4 ± 1.6 and 29.3 ± 2.3 weeks, respectively. Umbilical cord blood and a second blood sample taken 18 to 40 h after birth were used for endothelin-1 determination by enzyme immunoassay. Median umbilical cord blood endothelin-1 levels were similar in both groups (control: 10.9 and respiratory distress syndrome: 11.4 pg/mL and were significantly higher than in the second sample (control: 1.7 pg/mL and respiratory distress syndrome: 3.5 pg/mL, P < 0.001 for both groups. Median endothelin-1 levels in the second sample were significantly higher in children with respiratory distress syndrome than in control infants (P < 0.001. There were significant positive correlations between second sample endothelin-1 and Score for Neonatal Acute Physiology and Perinatal Extension II (r = 0.36, P = 0.02, and duration of mechanical ventilation (r = 0.64, P = 0.02. A slower decline of endothelin-1 from birth to 40 h of life was observed in newborns with respiratory distress syndrome when compared to controls. A significant correlation between neonatal endothelin-1 levels and some illness-severity signs suggests that endothelin-1 plays a role in the natural course of respiratory distress syndrome in preterm newborns.

  4. Split cord malformation as a cause of tethered cord syndrome in a 78-Year-old female.

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    Pallatroni, Henry F; Ball, Perry A; Duhaime, Ann-Christine

    2004-01-01

    A 78-year-old woman presented for evaluation of back pain, urinary dysfunction, leg weakness and progressive equinovarus foot deformity. She reported that shortly after her birth in 1924, she underwent resection of a subcutaneous 'cyst' in the lower lumbar area. Seven years prior to evaluation at our institution, she had undergone bilateral total knee arthroplasty for osteoarthritis. After the procedure, she began to experience severe low back pain that radiated into her legs. Weakness of the foot inverters, urinary dysfunction and worsening bilateral equinovarus foot deformity developed in the years following the surgery. MRI revealed a split cord malformation with a tethered spinal cord. Because of the patient's age and poor medical condition, her symptoms were managed conservatively. This case demonstrates symptomatic deterioration in an elderly patient with a tethered spinal cord after many years of clinical stability. PMID:15292638

  5. The central anticholinergic syndrome in the postoperative period

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    J. Rupreht (Joze); B. Dworacek (B.)

    1990-01-01

    markdownabstractRésumé Le syndrome anticholinergique central (SAC) comporte des signes centraux (somnolence, confusion, amnésie, agitation, hallucination, dysarthrie, ataxie, délire, stupeur, coma) et des signes périphériques (sécheresse buccale et/ou cutanée, tachycardie, troubles visuels et mict

  6. Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

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    Silvana Guerriero

    2011-01-01

    Full Text Available Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis.

  7. Spatial and temporal activation of spinal glial cells: role of gliopathy in central neuropathic pain following spinal cord injury in rats.

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    Gwak, Young S; Kang, Jonghoon; Unabia, Geda C; Hulsebosch, Claire E

    2012-04-01

    In the spinal cord, neuron and glial cells actively interact and contribute to neurofunction. Surprisingly, both cell types have similar receptors, transporters and ion channels and also produce similar neurotransmitters and cytokines. The neuroanatomical and neurochemical similarities work synergistically to maintain physiological homeostasis in the normal spinal cord. However, in trauma or disease states, spinal glia become activated, dorsal horn neurons become hyperexcitable contributing to sensitized neuronal-glial circuits. The maladaptive spinal circuits directly affect synaptic excitability, including activation of intracellular downstream cascades that result in enhanced evoked and spontaneous activity in dorsal horn neurons with the result that abnormal pain syndromes develop. Recent literature reported that spinal cord injury produces glial activation in the dorsal horn; however, the majority of glial activation studies after SCI have focused on transient and/or acute time points, from a few hours to 1 month, and peri-lesion sites, a few millimeters rostral and caudal to the lesion site. In addition, thoracic spinal cord injury produces activation of astrocytes and microglia that contributes to dorsal horn neuronal hyperexcitability and central neuropathic pain in above-level, at-level and below-level segments remote from the lesion in the spinal cord. The cellular and molecular events of glial activation are not simple events, rather they are the consequence of a combination of several neurochemical and neurophysiological changes following SCI. The ionic imbalances, neuroinflammation and alterations of cell cycle proteins after SCI are predominant components for neuroanatomical and neurochemical changes that result in glial activation. More importantly, SCI induced release of glutamate, proinflammatory cytokines, ATP, reactive oxygen species (ROS) and neurotrophic factors trigger activation of postsynaptic neuron and glial cells via their own receptors

  8. Symptomatic epidural lipomatosis of the spinal cord in a child: MR demonstration of spinal cord injury

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    Munoz, Alberto [Department of Radiology, Section of Neuroradiology, 505 Parnassus Av, L-371, University of California-San Francisco, CA 94143-0628 (United States); Servicio de Radiodiagnostico, Seccion de Neurorradiologia, Hospital Universitario ' ' 12 de Octubre' ' , 28040 Madrid (Spain); Barkovich, James A. [Department of Radiology, Section of Neuroradiology, 505 Parnassus Av, L-371, University of California-San Francisco, CA 94143-0628 (United States); Mateos, Fernando; Simon, Rogelio [Seccion de Neurpediatria, Servicio de Neurologia, Hospital Universitario ' ' 12 de Octubre' ' , 28041 Madrid (Spain)

    2002-12-01

    We report a case of symptomatic epidural lipomatosis in an 8-year-old girl with Cushing's syndrome secondary to longstanding high-dose steroid therapy for Crohn's disease. MR imaging of the spine revealed massive diffuse epidural fat compressing the entire spinal cord with T2 prolongation in the central gray matter of the cord suggesting ischemic myelopathy. This finding has not been previously demonstrated on imaging. A proposed mechanism underlying these findings is discussed. (orig.)

  9. Rare association of central pontine myelinolysis with infantile tremor syndrome

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    Kalpana Datta

    2012-01-01

    Full Text Available Central pontine myelinolysis (CPM is an acute demyelination within the central basis pontis. Though exact mechanism is not known it is seen commonly with rapid correction of hyponatremia and also with pontine ischemia or infarction, demyelinating diseases, pontine neoplasm and different metabolic diseases. We report a rare association of CPM in a patient of Infantile Tremor Syndrom (ITS. ITS is a syndrome of tremor, mental and physical retardation, pigmentary changes of hair and skin and anemia in malnourished children. Though first reported in Indian subcontinent many identical cases were reported from around the world. Our case is a 15 month old child with generalized tremor, mild hepatosplenomegaly with features of grade II malnutrition including skin and hair changes. All the signs and symtoms of tremor improved after treatment with the World Health Organization (WHO protocol for protein energy malnutrition (PEM and administration of propranolol without any side effects.

  10. Congenital central hypoventilation syndrome: diagnostic and management challenges

    OpenAIRE

    Kasi, Ajay; Perez,Iris; Kun,Sheila; Keens, Thomas

    2016-01-01

    Ajay S Kasi,1 Iris A Perez,1,2 Sheila S Kun,1 Thomas G Keens1,2 1Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, 2Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA Abstract: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder with failure of central control of breathing and of the autonomic nervous system function due to a mutation in the paired-like homeobox 2B (PHOX2B) gene. Affec...

  11. An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain

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    Shotaro Kanda

    2015-01-01

    Full Text Available Adult tethered cord syndrome (ATCS is a rare entity that usually presents with multiple neurological symptoms, including lower extremity pain, backache, lower extremity muscle weakness, and bowel/bladder disturbances. Prompt surgical treatment is often necessary to avoid permanent sequelae. We report a 63-year-old man with sudden-onset severe right chest and upper back pain, followed by urinary retention. His initial workup included computed tomography of the abdomen and pelvis, which showed a presacral mass. His symptom-driven neurological workup focused on the cervical and thoracic spine, the results of which were normal. Pelvic radiographs and magnetic resonance imaging of the lumbosacral spine showed spina bifida occulta, meningocele, and presacral masses consistent with a teratomatous tumor. His symptoms, except for urinary retention, improved dramatically with surgical treatment. The excised specimen contained a teratomatous lesion plus an organized hematoma. Hematoma formation was suspected as the trigger of his sudden-onset right chest and upper back pain.

  12. Respiratory and autonomic dysfunction in congenital central hypoventilation syndrome.

    Science.gov (United States)

    Moreira, Thiago S; Takakura, Ana C; Czeisler, Catherine; Otero, Jose J

    2016-08-01

    The developmental lineage of the PHOX2B-expressing neurons in the retrotrapezoid nucleus (RTN) has been extensively studied. These cells are thought to function as central respiratory chemoreceptors, i.e., the mechanism by which brain Pco2 regulates breathing. The molecular and cellular basis of central respiratory chemoreception is based on the detection of CO2 via intrinsic proton receptors (TASK-2, GPR4) as well as synaptic input from peripheral chemoreceptors and other brain regions. Murine models of congenital central hypoventilation syndrome designed with PHOX2B mutations have suggested RTN neuron agenesis. In this review, we examine, through human and experimental animal models, how a restricted number of neurons that express the transcription factor PHOX2B play a crucial role in the control of breathing and autonomic regulation. PMID:27226447

  13. Spinal cord stimulation for treatment of pain in a patient with post thoracotomy pain syndrome.

    Science.gov (United States)

    Graybill, Jordan; Conermann, Till; Kabazie, Abraham J; Chandy, Sunil

    2011-01-01

    Post Thoracotomy Pain Syndrome (PTPS) is defined as pain that occurs or persists in the area of the thoracotomy incision for at least 2 months following the initial procedure.  The true incidence of PTPS is hard to define as literature reports a wide range of occurrence from 5% to 90%.  Thoracotomy is associated with a high risk of severe chronic postoperative pain.  Presenting symptoms include both neuropathic pain in the area of the incision, as well as myofascial pain commonly in the ipsilateral scapula and shoulder.  Pain management can be challenging in these patients.  Multiple treatments have been described including conservative treatments with oral nonsteroidal anti-inflammatory drugs (NSAIDs); topically applied, peripherally acting drugs; neuromodulating agents; physical therapy; transcutaneous electrical nerve stimulation as well as more invasive treatments including intercostal nerve blocks, trigger point steroid injections, epidural steroid injections, radiofrequency nerve ablation, cryoablation, and one case report of spinal cord stimulation.  Unfortunately, a portion of these patients will have persistent pain in spite of multiple treatment modalities, and in some cases will experience worsening of pain. This case report describes the novel utility and complete resolution of symptoms with spinal cord stimulation (SCS) in treatment of a patient with persistent PTPS. In the operating room, a percutaneous octet electrode lead was placed using sterile technique under fluoroscopic guidance and loss-of-resistance technique.  The octet electrode lead was subsequently advanced with the aid of fluoroscopy to the level of the T3 superior endplate just right of midline.  The patient's pain distribution was captured optimally with stimulation at this level.  With the assistance of a neurosurgeon, the lead was anchored, tunneled, and connected to a generator, which was implanted over the right iliac crest.  The patient tolerated the procedure well with

  14. Ascending central canal dilation and progressive ependymal disruption in a contusion model of rodent chronic spinal cord injury

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    Keirstead Hans S

    2007-09-01

    Full Text Available Abstract Background Chronic spinal cord injury (SCI can lead to an insidious decline in motor and sensory function in individuals even years after the initial injury and is accompanied by a slow and progressive cytoarchitectural destruction. At present, no pathological mechanisms satisfactorily explain the ongoing degeneration. Methods Adult female Sprague-Dawley rats were anesthetized laminectomized at T10 and received spinal cord contusion injuries with a force of 250 kilodynes using an Infinite Horizon Impactor. Animals were randomly distributed into 5 groups and killed 1 (n = 4, 28 (n = 4, 120 (n = 4, 450 (n = 5, or 540 (n = 5 days after injury. Morphometric and immunohistochemical studies were then performed on 1 mm block sections, 6 mm cranial and 6 mm caudal to the lesion epicenter. The SPSS 11.5 t test was used to determine differences between quantitative measures. Results Here, we document the first report of an ascending central canal dilation and progressive ependymal disruption cranial to the epicenter of injury in a contusion model of chronic SCI, which was characterized by extensive dural fibrosis and intraparenchymal cystic cavitation. Expansion of the central canal lumen beyond a critical diameter corresponded with ependymal cell ciliary loss, an empirically predictable thinning of the ependymal region, and a decrease in cell proliferation in the ependymal region. Large, aneurysmal dilations of the central canal were accompanied by disruptions in the ependymal layer, periependymal edema and gliosis, and destruction of the adjacent neuropil. Conclusion Cells of the ependymal region play an important role in CSF homeostasis, cellular signaling and wound repair in the spinal cord. The possible effects of this ascending pathology on ependymal function are discussed. Our studies suggest central canal dilation and ependymal region disruption as steps in the pathogenesis of chronic SCI, identify central canal dilation as a marker of

  15. Preclinical evidence supporting the clinical development of central pattern generator-modulating therapies for chronic spinal cord-injured patients

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    Pierre eGuertin

    2014-05-01

    Full Text Available Ambulation or walking is one of the main gaits of locomotion. In terrestrial animals, it may be defined as a series of rhythmic and bilaterally coordinated movement of the limbs which creates a forward movement of the body. This applies regardless of the number of limbs - from arthropods with six or more limbs to bipedal primates. These fundamental similarities among species may explain why comparable neural systems and cellular properties have been found, thus far, to control in similar ways locomotor rhythm generation in most animal models. The aim of this article is to provide a comprehensive review of the known structural and functional features associated with central nervous system (CNS networks that are involved in the control of ambulation and other stereotyped motor patterns - specifically Central Pattern Generators (CPGs that produce basic rhythmic patterned outputs for locomotion, micturition, ejaculation, and defecation. Although there is compelling evidence of their existence in humans, CPGs have been most studied in reduced models including in vitro isolated preparations, genetically-engineered mice and spinal cord-transected animals. Compared with other structures of the CNS, the spinal cord is generally considered as being well-preserved phylogenetically. As such, most animal models of SCI should be considered as valuable tools for the development of novel pharmacological strategies aimed at modulating spinal activity and restoring corresponding functions in chronic spinal cord-injured patients.

  16. [Two cases of solitary median maxillary central incisor syndrome].

    Science.gov (United States)

    Catania, P; Conti, C; Poggi, G M; Bardelli, T; Lasagni, D; De Martino, M

    2010-02-01

    Solitary median maxillary central incisor syndrome (SMMCI) syndrome is a unique developmental abnormality arising from an unknown event occurring between the 35th and 38th days in utero, and involving mieline structure of the head including the cranial bones, the maxilla and its container dentition (specifically the central incisor tooth germ), together with other midline structures of the body. The SMMCI tooth may be possibly occur as an isolated trait or in association with many other midline developmental anomalies. It is estimated to occur in 1:50000 live births. There is a wide variability in the phenotypic spectrum. SMMCI is considered one of the most minimal expressions of the holoprosencephaly spectrum. Mutation in the Sonic Hedgehog homolog (SHH) gene may be associated with SSMMCI, but recent studies suggests the existence of several other candidate genes. We described two patients with SMMCI. They presented a solitary median maxillary incisor, short stature, hipotelorism and corpus callosus anomalies found on magnetic resonance imaging (MRI). They also present severe hiponatremia. At the best of our knowledge, this is the first report of cases of SMMCI with hiponatremia. We suggest that the sodium disorder may be secondary to syndrome of inappropriate secretion of antidiuretic hormone (SIADH). PMID:20212404

  17. Pregnancy in Woman with Spinal Cord Stimulator for Complex Regional Pain Syndrome: A Case Report and Review of the Literature

    OpenAIRE

    Yoo, Hyung Seok; Nahm, Francis Sahngun; Yim, Kyoung Hoon; Moon, Jee Youn; Kim, Yung Suk; Lee, Pyung Bok

    2010-01-01

    Spinal cord stimulation (SCS) is used to manage chronic pain syndromes and it is accepted a cost-effective therapy. Child-bearing women who had SCS become or choose to become pregnant despite these policies pregnancy is a relative contraindication. A 32-year-old woman had SCS as a treatment for the CRPS I of the left lower extremity. During various check up tests, we happen to find out that her serum beta-hCG was positive and confirmed pregnancy. SCS is not recommended in pregnancy because th...

  18. 'Pseudofailure' of spinal cord stimulation for neuropathic pain following a new severe noxious stimulus: learning points from a case series of failed spinal cord stimulation for complex regional pain syndrome and failed back surgery syndrome.

    Science.gov (United States)

    Muquit, Samiul; Moussa, Ahmad Abdelhai; Basu, Surajit

    2016-05-01

    Failure of spinal cord stimulation (SCS) may be due to hardware problems, migration of electrodes and, in the long-term, plasticity in the spinal cord with habituation to the stimulation current. We describe a series of seven patients who experienced acute therapeutic loss of SCS effects following an acute nociceptive event unrelated to primary pathology. There were no hardware problems. We called this 'Pseudofailure', as the effective stimulation returned in all patients following a period off stimulation or reprogramming. This phenomenon has not been reported previously in the literature. Over a 4-year period, we managed seven patients with this feature: four had received SCS for complex regional pain syndrome and three for failed back surgery syndrome. In all seven cases, there was cessation of the pain relief afforded by SCS following an acute painful event: four patients had trauma, two patients had domestic electric shock and one patient suffered shingles (varicella zoster infection). We excluded hardware-related problems in all cases. In two patients, SCS effects could be regained by an initial attempt at reprogramming. In the remaining five cases reprogramming was unsuccessful, and stimulation was switched off for several months before recommencing, when we discovered a return of good therapeutic effect. We conclude that SCS may seem to fail following a separate strong nociceptive stimulus. Stimulation may be regained with reprogramming or following a period with stimulation switched off. We would, therefore, advise against removal of SCS hardware in the first instance. PMID:27551417

  19. Small for Gestational Age and Magnesium in Cord Blood Platelets: Intrauterine Magnesium Deficiency May Induce Metabolic Syndrome in Later Life

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    Junji Takaya

    2011-01-01

    Full Text Available Magnesium deficiency in pregnancy frequently occurs because of inadequate or low intake of magnesium. Magnesium deficiency during pregnancy can induce not only maternal and fetal nutritional problems, but also consequences that might last in offspring throughout life. Many epidemiological studies have disclosed that small for gestational age (SGA is associated with an increased risk of insulin resistance in adult life. We reported that intracellular magnesium of cord blood platelets is lower in SGA groups than that in appropriate for gestational age groups, suggesting that intrauterine magnesium deficiency may result in SGA. Taken together, intrauterine magnesium deficiency in the fetus may lead to or at least program insulin resistance after birth. In this review, we propose that intrauterine magnesium deficiency may induce metabolic syndrome in later life. We discuss the potential contribution of aberrant magnesium regulation to SGA and to the pathogenesis of metabolic syndrome.

  20. Congenital central hypoventilation syndrome: diagnostic and management challenges

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    Kasi AS

    2016-08-01

    Full Text Available Ajay S Kasi,1 Iris A Perez,1,2 Sheila S Kun,1 Thomas G Keens1,2 1Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, 2Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA Abstract: Congenital central hypoventilation syndrome (CCHS is a rare genetic disorder with failure of central control of breathing and of the autonomic nervous system function due to a mutation in the paired-like homeobox 2B (PHOX2B gene. Affected patients have absent or negligible ventilatory sensitivity to hypercapnia and hypoxemia, and they do not exhibit signs of respiratory distress when challenged with hypercarbia or hypoxia. The diagnosis of CCHS must be confirmed with PHOX2B gene mutation. Generally, the PHOX2B mutation genotype can aid in anticipating the severity of the phenotype. They require ventilatory support for life. Home assisted ventilation options include positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and diaphragm pacing via phrenic nerve stimulation, but each strategy has its associated limitations and challenges. Since all the clinical manifestations of CCHS may not manifest at birth, periodic monitoring and early intervention are necessary to prevent complications and improve outcome. Life-threatening arrhythmias can manifest at different ages and a normal cardiac monitoring study does not exclude future occurrences leading to the dilemma of timing and frequency of cardiac rhythm monitoring and treatment. Given the rare incidence of CCHS, most health care professionals are not experienced with managing CCHS patients, particularly those with diaphragm pacers. With early diagnosis and advances in home mechanical ventilation and monitoring strategies, many CCHS children are surviving into adulthood presenting new challenges in their care. Keywords: congenital central hypoventilation syndrome, PHOX2B, home mechanical ventilation, diaphragm

  1. Hippocampal volume reduction in congenital central hypoventilation syndrome.

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    Paul M Macey

    Full Text Available Children with congenital central hypoventilation syndrome (CCHS, a genetic disorder characterized by diminished drive to breathe during sleep and impaired CO(2 sensitivity, show brain structural and functional changes on magnetic resonance imaging (MRI scans, with impaired responses in specific hippocampal regions, suggesting localized injury.We assessed total volume and regional variation in hippocampal surface morphology to identify areas affected in the syndrome. We studied 18 CCHS (mean age+/-std: 15.1+/-2.2 years; 8 female and 32 healthy control (age 15.2+/-2.4 years; 14 female children, and traced hippocampi on 1 mm(3 resolution T1-weighted scans, collected with a 3.0 Tesla MRI scanner. Regional hippocampal volume variations, adjusted for cranial volume, were compared between groups based on t-tests of surface distances to the structure midline, with correction for multiple comparisons. Significant tissue losses emerged in CCHS patients on the left side, with a trend for loss on the right; however, most areas affected on the left also showed equivalent right-sided volume reductions. Reduced regional volumes appeared in the left rostral hippocampus, bilateral areas in mid and mid-to-caudal regions, and a dorsal-caudal region, adjacent to the fimbria.The volume losses may result from hypoxic exposure following hypoventilation during sleep-disordered breathing, or from developmental or vascular consequences of genetic mutations in the syndrome. The sites of change overlap regions of abnormal functional responses to respiratory and autonomic challenges. Affected hippocampal areas have roles associated with memory, mood, and indirectly, autonomic regulation; impairments in these behavioral and physiological functions appear in CCHS.

  2. Selective adenosine A2A receptor agonists and antagonists protect against spinal cord injury through peripheral and central effects

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    Esposito Emanuela

    2011-04-01

    Full Text Available Abstract Background Permanent functional deficits following spinal cord injury (SCI arise both from mechanical injury and from secondary tissue reactions involving inflammation. Enhanced release of adenosine and glutamate soon after SCI represents a component in the sequelae that may be responsible for resulting functional deficits. The role of adenosine A2A receptor in central ischemia/trauma is still to be elucidated. In our previous studies we have demonstrated that the adenosine A2A receptor-selective agonist CGS21680, systemically administered after SCI, protects from tissue damage, locomotor dysfunction and different inflammatory readouts. In this work we studied the effect of the adenosine A2A receptor antagonist SCH58261, systemically administered after SCI, on the same parameters. We investigated the hypothesis that the main action mechanism of agonists and antagonists is at peripheral or central sites. Methods Spinal trauma was induced by extradural compression of SC exposed via a four-level T5-T8 laminectomy in mouse. Three drug-dosing protocols were utilized: a short-term systemic administration by intraperitoneal injection, a chronic administration via osmotic minipump, and direct injection into the spinal cord. Results SCH58261, systemically administered (0.01 mg/kg intraperitoneal. 1, 6 and 10 hours after SCI, reduced demyelination and levels of TNF-α, Fas-L, PAR, Bax expression and activation of JNK mitogen-activated protein kinase (MAPK 24 hours after SCI. Chronic SCH58261 administration, by mini-osmotic pump delivery for 10 days, improved the neurological deficit up to 10 days after SCI. Adenosine A2A receptors are physiologically expressed in the spinal cord by astrocytes, microglia and oligodendrocytes. Soon after SCI (24 hours, these receptors showed enhanced expression in neurons. Both the A2A agonist and antagonist, administered intraperitoneally, reduced expression of the A2A receptor, ruling out the possibility that the

  3. Central poststroke pain: somatosensory abnormalities and the presence of associated myofascial pain syndrome

    OpenAIRE

    de Oliveira Rogério Adas; de Andrade Daniel; Machado André Guelman; Teixeira Manoel

    2012-01-01

    Abstract Background Central post-stroke pain (CPSP) is a neuropathic pain syndrome associated with somatosensory abnormalities due to central nervous system lesion following a cerebrovascular insult. Post-stroke pain (PSP) refers to a broader range of clinical conditions leading to pain after stroke, but not restricted to CPSP, including other types of pain such as myofascial pain syndrome (MPS), painful shoulder, lumbar and dorsal pain, complex regional pain syndrome, and spasticity-related ...

  4. Central canal ependymal cells proliferate extensively in response to traumatic spinal cord injury but not demyelinating lesions.

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    Steve Lacroix

    Full Text Available The adult mammalian spinal cord has limited regenerative capacity in settings such as spinal cord injury (SCI and multiple sclerosis (MS. Recent studies have revealed that ependymal cells lining the central canal possess latent neural stem cell potential, undergoing proliferation and multi-lineage differentiation following experimental SCI. To determine whether reactive ependymal cells are a realistic endogenous cell population to target in order to promote spinal cord repair, we assessed the spatiotemporal dynamics of ependymal cell proliferation for up to 35 days in three models of spinal pathologies: contusion SCI using the Infinite Horizon impactor, focal demyelination by intraspinal injection of lysophosphatidylcholine (LPC, and autoimmune-mediated multi-focal demyelination using the active experimental autoimmune encephalomyelitis (EAE model of MS. Contusion SCI at the T9-10 thoracic level stimulated a robust, long-lasting and long-distance wave of ependymal proliferation that peaked at 3 days in the lesion segment, 14 days in the rostral segment, and was still detectable at the cervical level, where it peaked at 21 days. This proliferative wave was suppressed distal to the contusion. Unlike SCI, neither chemical- nor autoimmune-mediated demyelination triggered ependymal cell proliferation at any time point, despite the occurrence of demyelination (LPC and EAE, remyelination (LPC and significant locomotor defects (EAE. Thus, traumatic SCI induces widespread and enduring activation of reactive ependymal cells, identifying them as a robust cell population to target for therapeutic manipulation after contusion; conversely, neither demyelination, remyelination nor autoimmunity appears sufficient to trigger proliferation of quiescent ependymal cells in models of MS-like demyelinating diseases.

  5. The Cost-Effectiveness of Spinal Cord Stimulation for Complex Regional Pain Syndrome

    NARCIS (Netherlands)

    Kemler, Marius A.; Raphael, Jon H.; Bentley, Anthony; Taylor, Rod S.

    2010-01-01

    Objectives: Health-care policymakers and payers require cost-effectiveness evidence to inform their treatment funding decisions. The aims of this study were to assess the cost-effectiveness of the addition of spinal cord stimulation (SCS) compared with conventional management alone (CMM) in patients

  6. Ethical considerations with the management of congenital central hypoventilation syndrome.

    Science.gov (United States)

    Massie, John; Gillam, Lynn

    2015-05-01

    Congenital central hypoventilation syndrome (CCHS) is a well-recognized disorder of the autonomic nervous system caused by mutations in the PHOX2B gene. The most characteristic feature is failure of ventilatory control, resulting in the need for respiratory support while asleep, and in some cases when awake also. Most cases present in infancy or early childhood. Technological advances allow patients with mild to moderate phenotypesto receive adequate support by non-invasive ventilation (NIV), or diaphragm pacing (or combination of the two) avoiding the need for long-term ventilation by tracheostomy. Daytime functioning of patients with CCHS who require sleep-time ventilation only is expected to be good, with some additional surveillance to ensure they don't accidentally fall asleep without respiratory support available. Some children with CCHS have other complications, such as Hirschprung's disease, learning difficulties, and cardiac arrhythmias (leading in some instances to heart block and the requirement for a pacemaker). In a few cases, patients can develop neurogenic malignancies. Parents bear a significant burden for the care of their child with CCHS including provision of NIV at home, close monitoring, and regular surveillance for complications. Information about patients with CCHS comes from databases in the United States and Europe, but these don't include infants or children for whom ventilator support was not offered. In this paper we use a case study to explore the ethical issues of provision of treatment, or non-treatment, of children with CCHS.

  7. Solitary median maxillary central incisor in association with Goldenhar's syndrome: a case report.

    Science.gov (United States)

    Garcia de Paula e Silva, Francisco Wanderley; de Carvalho, Fabricio Kitazono; Diaz-Serrano, Kranya Victória; de Freitas, Aldevina Campos; Borsatto, Maria Cristina; de Queiroz, Alexandra Mussolino

    2007-01-01

    Goldenhar's syndrome is a rare disorder characterized by several anomalies that include dermal epibulbar cysts, auricular appendices and malformations and vertebral anomalies. In this article, the authors report a case of Goldenhar's syndrome in a 10-year-old child who presented with the classical signs of this condition and a solitary median maxillary central incisor (SMMCI). PMID:17658185

  8. Mild Moxibustion Decreases the Expression of Prokineticin 2 and Prokineticin Receptor 2 in the Colon and Spinal Cord of Rats with Irritable Bowel Syndrome

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    Cili Zhou

    2014-01-01

    Full Text Available It has been proven that prokineticin 2 (PK2 and its receptor PKR2 play an important role in hyperalgesia, while mild moxibustion can relieve visceral hypersensitivity in a rat model of irritable bowel syndrome (IBS. The goal of the present study was to determine the effects of mild moxibustion on the expression of PK2 and PKR2 in colon and spinal cord in IBS rat model, which was induced by colorectal distension using inflatable balloons. After mild moxibustion treatment, abdominal withdrawal reflex (AWR scores were assessed by colorectal distension; protein and mRNA expression of PK2 and PKR2 in rat colon and spinal cord was determined by immunohistochemistry and fluorescence quantitative PCR. Compared with normal rats, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly increased in the model group; compared with the model group, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly decreased in the mild moxibustion group. These findings suggest that the analgesia effect of mild moxibustion may be associated with the reduction of the abnormally increased expression of the PK2/PKR2 proteins and mRNAs in the colon and spinal cord.

  9. Functional and metabolic changes in the brain in neuropathic pain syndrome against the background of chronic epidural electrostimulation of the spinal cord.

    Science.gov (United States)

    Sufianov, A A; Shapkin, A G; Sufianova, G Z; Elishev, V G; Barashin, D A; Berdichevskii, V B; Churkin, S V

    2014-08-01

    Changes in functional and metabolic activities of the brain were evaluated by EEG and positron-emission/computer tomography with 18F-fluorodeoxyglucose in patients with neuropathic pain syndrome previous to and 3 months after implantation of a system for chronic epidural spinal cord stimulation. In most cases, the use of a nerve stimulator was followed by alleviation of neuropathic pain and partial normalization of functional and metabolic activities of brain structures responsible for pain perception, emotiogenic, behavioral, and autonomic responses.

  10. Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular Klippel-Trénaunay-Parkes-Weber syndrome with spinal cord angioma: a case report

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    James Pitagoras de Mattos

    1975-09-01

    Full Text Available É relatado um caso de síndrome de Klippel-Trénaunay-Parkes-Weber associada a angiomatose medular. O autor salienta ter encontrado na literatura somente uma referência com tal associação.A case of Klippel-Trénaunay-Parkes-Weber syndrome associated with spinal cord angioma is reported. The author points out that it was found only one reference in literature with such association.

  11. The Great Deceiver: A Case of Central Sensitization Presenting as Carcinoid Syndrome.

    Science.gov (United States)

    Ramos, Juan A

    2016-06-01

    Central sensitization defines a state of amplified sensory input within the nervous system across many organ systems; it overlaps syndromes as fibromyalgia, chronic fatigue, irritable bowel, and interstitial cystitis. Commonly, individuals will experience multiple syndromes during the course of their lifetime. A 62-year-old patient presented for evaluation of multiple medically unexplained symptoms postsurgically including chronic left chest wall and flank pain with concomitant diarrhea, abdominal pain, and facial flushing. After extensive multidisciplinary evaluations, he was diagnosed as having central sensitization in which the initial presentation mimicked carcinoid syndrome. He was subsequently treated with extensive multidisciplinary pain rehabilitation, and it did well. PMID:27144900

  12. Central nervous system involvement in primary Sjogren`s syndrome manifesting as multiple sclerosis.

    Science.gov (United States)

    Liu, Jing-Yao; Zhao, Teng; Zhou, Chun-Kui

    2014-04-01

    Central nervous system symptoms in patients with primary Sjogren`s syndrome are rare. They can present as extraglandular manifestations and require a differential diagnosis from multiple sclerosis. Due to a variety of presentations, Sjogren`s syndrome with neurologic involvement may be difficult to diagnose. Here, we report a case of a 75-year-old woman who was first diagnosed with multiple sclerosis in 2010, but who was subsequently diagnosed with primary Sjogren`s syndrome 2 years later after showing signs of atypical neurologic manifestations. Therefore, primary Sjogren`s syndrome should be suspected in patients who present with atypical clinical and radiologic neurologic manifestations.

  13. Dynamic Changes in Local Protein Synthetic Machinery in Regenerating Central Nervous System Axons after Spinal Cord Injury

    Science.gov (United States)

    Sachdeva, Rahul; Farrell, Kaitlin; McMullen, Mary-Katharine; Twiss, Jeffery L.; Houle, John D.

    2016-01-01

    Intra-axonal localization of mRNAs and protein synthesis machinery (PSM) endows neurons with the capacity to generate proteins locally, allowing precise spatiotemporal regulation of the axonal response to extracellular stimuli. A number of studies suggest that this local translation is a promising target to enhance the regenerative capacity of damaged axons. Using a model of central nervous system (CNS) axons regenerating into intraspinal peripheral nerve grafts (PNGs) we established that adult regenerating CNS axons contain several different mRNAs and protein synthetic machinery (PSM) components in vivo. After lower thoracic level spinal cord transection, ascending sensory axons regenerate into intraspinal PNGs but axon growth is stalled when they reach the distal end of the PNG (3 versus 7 weeks after grafting, resp.). By immunofluorescence with optical sectioning of axons by confocal microscopy, the total and phosphorylated forms of PSMs are significantly lower in stalled compared with actively regenerating axons. Reinjury of these stalled axons increased axonal localization of the PSM proteins, indicative of possible priming for a subcellular response to axotomy. These results suggest that axons downregulate protein synthetic capacity as they cease growing, yet they retain the ability to upregulate PSM after a second injury.

  14. Evidence-based and heuristic approaches for customization of care in cardiometabolic syndrome after spinal cord injury.

    Science.gov (United States)

    Nash, Mark S; Cowan, Rachel E; Kressler, Jochen

    2012-09-01

    Component and coalesced health risks of the cardiometabolic syndrome (CMS) are commonly reported in persons with spinal cord injuries (SCIs). These CMS hazards are also co-morbid with physical deconditioning and elevated pro-atherogenic inflammatory cytokines, both of which are common after SCI and worsen the prognosis for all-cause cardiovascular disease. This article describes a systematic procedure for individualized CMS risk assessment after SCI, and emphasizes evidence-based and intuition-centered countermeasures to disease. A unified approach will propose therapeutic lifestyle intervention as a routine plan for aggressive primary prevention in this risk-susceptible population. Customization of dietary and exercise plans then follow, identifying shortfalls in diet and activity patterns, and ways in which these healthy lifestyles can be more substantially embraced by both stakeholders with SCI and their health care providers. In cases where lifestyle intervention utilizing diet and exercise is unsuccessful in countering risks, available pharmacotherapies and a preferred therapeutic agent are proposed according to authoritative standards. The over-arching purpose of the monograph is to create an operational framework in which existing evidence-based approaches or heuristic modeling becomes best practice. In this way persons with SCI can lead more active and healthy lives.

  15. Solitary median maxillary central incisor syndrome associated with unique cleft palate: a rare case report.

    Science.gov (United States)

    Holla, Goda; Ramakrishna, Yeluri; Holla, Anup; Munshi, Autar Krishen

    2014-01-01

    Solitary median maxillary central incisor (SMMCI) syndrome is a rare dental anomaly that affects 1:50,000 live births. SMMCI syndrome is characterized by the presence of a single central incisor located on the maxillary midline in both primary and permanent dentitions. It may occur as an isolated finding or in association with developmental defects and systemic involvement. Congenital anomalies associated with SMMCI syndrome can include short stature, mild forms of deviation in craniofacial morphology, mild to severe intellectual disability, congenital heart disease, and cleft lip and/or palate. This report describes a clinical case of a 7-year-old girl with SMMCI syndrome--in addition to bilateral residual cleft and associated nasal regurgitation--that was treated with a removable maxillary obturator. PMID:24598504

  16. The Frequency of Exfoliation Syndrome in the Central Anatolia Region of Turkey

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    Raşit Kılıç

    2014-01-01

    Full Text Available Aim. The aim of this study was to investigate the frequency of exfoliation syndrome in the Central Anatolia region of Turkey and to evaluate its relationship with cardiovascular and ocular diseases. Methods. Patients over the age of 45 years who presented to the clinic were included in the study. All cases underwent a comprehensive ophthalmology examination. Exfoliation syndrome was diagnosed with the presence of exfoliative material on the lens anterior capsule or iris on slit lamp examination. The patients were divided into two groups as the exfoliation syndrome group and nonexfoliation syndrome group according to the presence of exfoliative material. Results. Exfoliative material was found in one or both eyes of 212 of the 2103 patients (10.1% evaluated within the scope of the study. A significant relationship was found between exfoliation syndrome and increasing age and male gender. A significant relationship was found between exfoliation syndrome and glaucoma, cataracts, age-related macular degeneration, and phacodonesis. While no relationship was found between exfoliation syndrome and hypertension or diabetes mellitus, a significant relationship was found with coronary artery disease. Conclusion. The unilateral or bilateral exfoliation syndrome frequency was 10.1% in this hospital-based study. A statistically significant relationship was found between exfoliation syndrome and advancing age, gender, and coronary artery disease.

  17. Myelodysplastic syndrome complicated by central diabetes insipidus and cerebral salt wasting syndrome with peculiar change in magnetic resonance images.

    Science.gov (United States)

    Sano, Soichi; Yamagami, Keiko; Morikawa, Takashi; Yoshioka, Katsunobu

    2010-01-01

    Central diabetes insipidus (CDI) could occurs in patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), because of infiltration of leukemic cells into the neurohypophysis or some other reason and it is closely associated with abnormalities of chromosome 7. We report a case of MDS with abnormalities of chromosome 7, presenting as CDI followed by deterioration of polyuria and hyponatremia with a decreased extracellular fluid volume. Magnetic resonance imaging (MRI) revealed symmetrically enhanced lesions in the hypothalamus. Fludrocortisone treatment normalized his serum sodium level and cerebral salt wasting syndrome (CSWS) was suspected.

  18. Isolated Richter's syndrome in central nervous system: case report Sindrome de Richter isolada em sistema nervoso central: relato de caso

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    Lucilene S.R. Resende

    2005-06-01

    Full Text Available Diffuse large cell non Hodgkin's lymphoma associated with chronic lymphoid leukemia (CLL, or Richter's syndrome, is a rare and serious complication. Isolated Richter's syndrome in the central nervous system is very rare; only 12 cases have been reported. We describe a 74-year-old patient with diffuse large cell non Hodgkin's lymphoma in the right frontal region with the appearance of multiform glioblastoma.Linfoma não Hodgkin difuso de grandes células em paciente portador de leucemia linfóide crônica (LLC, ou síndrome de Richter, é complicação rara e grave nesta leucemia. Síndrome de Richter isolada no sistema nervoso central é muito rara, tendo sido encontrados apenas 12 casos descritos. Descrevemos paciente de 74 anos, que apresentou linfoma não Hodgkin difuso de grandes células em região frontal direita, simulando glioblastoma multiforme.

  19. Central Sensitization in Functional Chronic Pain Syndromes: Overview and Clinical Application.

    Science.gov (United States)

    Bettini, Liz; Moore, Ki

    2016-10-01

    The purpose of this review and clinical application article is to offer nurses up-to-date knowledge on peripheral and central sensitization in chronic functional pain syndromes, and to discuss therapies that have shown efficacy in treating various aspects of these disorders. Central sensitization is a result of changes in the peripheral and central nervous system due to noxious stimuli, such as illness or trauma. Once these changes occur, treatment for the associated syndromes requires a multimodal approach that includes behavioral pain psychology, physical therapy, and pharmacological agents that specifically target neuroinflammation, pain modulation, and amplification of pain pathways. More research needs to be conducted on the basis and patient perception of functional pain syndromes to reduce the morbidity and significant disability associated with these illnesses. Nurses have the opportunity to be at the forefront of this research because of their holistic and multidimensional approach to patient care, assessment, and symptom management. PMID:27553129

  20. Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

    Science.gov (United States)

    Yelnik, Cécile M; Kozora, Elizabeth; Appenzeller, Simone

    2016-02-01

    Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases. PMID:26923254

  1. Combined central diabetes insipidus and cerebral salt wasting syndrome in children.

    Science.gov (United States)

    Lin, Jainn-Jim; Lin, Kuang-Lin; Hsia, Shao-Hsuan; Wu, Chang-Teng; Wang, Huei-Shyong

    2009-02-01

    Central diabetes insipidus, a common consequence of acute central nervous system injury, causes hypernatremia; cerebral salt wasting syndrome can cause hyponatremia. The two conditions occurring simultaneous are rarely described in pediatric patients. Pediatric cases of combined diabetes insipidus and cerebral salt wasting after acute central nervous system injury between January 2000 and December 2007 were retrospectively reviewed, and clinical characteristics were systemically assessed. Sixteen patients, aged 3 months to 18 years, met study criteria: 11 girls and 5 boys. The most common etiologies were severe central nervous system infection (n = 7, 44%) and hypoxic-ischemic event (n = 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury. Onset of cerebral salt wasting syndrome occurred 2-8 days after the onset of diabetes insipidus. In terms of outcome, 13 patients died (81%) and 3 survived under vegetative status (19%). Central diabetes insipidus and cerebral salt wasting syndrome may occur after acute central nervous system injury. A combination of both may impede accurate diagnosis. Proper differential diagnoses are critical, because the treatment strategy for each entity is different.

  2. Spinal cord stimulation for complex regional pain syndrome type 1 with dystonia: a case report and discussion of the literature [v1; ref status: indexed, http://f1000r.es/348

    Directory of Open Access Journals (Sweden)

    Caroline Voet

    2014-04-01

    Full Text Available Background: Complex Regional Pain Syndrome type 1 (CRPS-1 is a debilitating chronic pain disorder, the physiopathology of which can lead to dystonia associated with changes in the autonomic, central and peripheral nervous system. An interdisciplinary approach (pharmacological, interventional and psychological therapies in conjunction with a rehabilitation pathway is central to progress towards pain reduction and restoration of function. Aim: This case report aims to stimulate reflection and development of mechanism-based therapeutic strategies concerning CRPS associated with dystonia. Case description: A 31 year old female CRPS-1 patient presented with dystonia of the right foot following ligamentoplasty for chronic ankle instability. She did not have a satisfactory response to the usual therapies. Multiple anesthetic blocks (popliteal, epidural and intrathecal were not associated with significant anesthesia and analgesia. Mobilization of the foot by a physiotherapist was not possible. A multidisciplinary approach with psychological support, physiotherapy and spinal cord stimulation (SCS brought pain relief, rehabilitation and improvement in the quality of life. Conclusion: The present case report demonstrates the occurrence of multilevel (peripheral and central pathological modifications in the nervous system of a CRPS-1 patient with dystonia. This conclusion is based on the patient’s pain being resistant to anesthetic blocks at different levels and the favourable, at least initially, response to SCS. The importance of the bio-psycho-social model is also suggested, permitting behavioural change.

  3. [Case of neuromyelitis optica spectrum disorder associated with central pontine and extrapontine myelinolysis preceded by syndrome of inappropriate antidiuretic hormone secretion].

    Science.gov (United States)

    Sakai, Waka; Matsui, Naoko; Fujita, Koji; Izumi, Yuishin; Nishida, Yoshihiko; Takahashi, Toshiyuki; Kanbayashi, Takashi; Kaji, Ryuji

    2014-01-01

    A 36-year-old woman complained of general malaise. She presented with hyponatremia and plasma osmotic pressure was lower than urinary osmotic pressure. In addition, serum antidiuretic hormone level was higher than the measurement sensitivity. She was diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). She fell into a coma despite correction of serum sodium level. Brain magnetic resonance imaging (MRI) revealed high signal intensities in the cerebral cortex, striatum, thalamus, hypothalamus, midbrain, and pons in fluid-attenuated inversion recovery images. Spinal MRI revealed a longitudinally extending lesion in the cervical cord. Serum sample was positive for anti-aquaporin-4 antibody, supporting the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) combined with central pontine and extrapontine myelinolysis. In patients with NMOSD, the immune reaction can gradually cause destructive changes of the hypothalamus and lead to unstable ADH secretion in the absence of immunomodulatory treatment. PMID:25087556

  4. Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation

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    Jorge Marques do Vale

    2014-10-01

    Full Text Available The Chiari malformation type I (CM-I has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

  5. Central obesity measurements predict metabolic syndrome in a retrospective cohort study of postmenopausal women

    Directory of Open Access Journals (Sweden)

    Manuel Rosety-Rodríguez

    2013-12-01

    Full Text Available Introduction: The various diagnostic classifications in the literature concur as regards the important role of abdominal obesity in the onset and progression of metabolic syndrome. Accordingly, this study was aimed at clarifying whether central obesity measurements assessed by dual X-ray absorptiometry (DXA may predict metabolic syndrome in Spanish postmenopausal women. Material and methods: This historical cohort study included a total of 1326 postmenopausal women aged > 45 years old who had routinely undergone DXA to measure their bone mineral density between january 2006 and january 2011. The regions of interest (ROI envisaged in our study by using DXA were the lumbar regions L1-L4 and L4-L5. At the same time, they underwent a complete medical examination including personal medical history assessment, biochemical blood analysis, blood pressure measurement and anthropometrical evaluation. Metabolic syndrome was diagnosed attending to the criteria established by National Cholesterol Education Program Adult Treatment Panel III (NECP-ATP-III. Results: During the observation period, 537 women, representing 40.5% of the total studied, met the diagnostic criteria for metabolic syndrome. L1-L4 and L4-L5 abdominal fat mass determinations were associated with the development of metabolic syndrome in all regression models tested, showing an increasing gradient from the lowest to highest quintile. Conclusion: Central adiposity measurements assessed by DXA, especially L1-L4 region of interest, could be considered a powerful predictor of metabolic syndrome in postmenopausal women.

  6. Catheter-directed Thrombolysis in Acute Superior Vena Cava Syndrome Caused by Central Venous Catheters.

    Science.gov (United States)

    Cui, Jie; Kawai, Tasuo; Irani, Zubin

    2015-01-01

    Indwelling central venous catheters have been reported to increase the risk of superior venous cava (SVC) syndrome. This case report describes the development of acute SVC syndrome in a 28-year-old woman with end-stage renal disease implanted with a left-side hemodialysis reliable outflow graft and a right-side double lumen hemodialysis catheter via internal jugular veins. Her symptoms were not alleviated after catheter removal and systemic anticoagulation therapy. She was eventually treated with catheter-directed thrombolysis and a predischarge computer tomographic venogram on postthrombolytic procedure day 7 showed patent central veins and patient remained asymptomatic. This case demonstrates that catheter-directed thrombolysis can be safely employed to treat refractory catheter-induced acute SVC syndrome in end-stage renal disease patients.

  7. Spinal Cord Stimulation

    DEFF Research Database (Denmark)

    Meier, Kaare

    2014-01-01

    Spinal cord stimulation (SCS) is a surgical treatment for chronic neuropathic pain that is refractory to other treatment. Originally described by Shealy et al. in 1967(1), it is used to treat a range of conditions such as complex regional pain syndrome (CRPS I)(2), angina pectoris(3), radicular...... pain after failed back surgery syndrome (FBSS)(4), pain due to peripheral nerve injury, stump pain(5), peripheral vascular disease(6) and diabetic neuropathy(7,8); whereas phantom pain(9), postherpetic neuralgia(10), chronic visceral pain(11), and pain after partial spinal cord injury(12) remain more...

  8. Cerebral salt wasting syndrome in children with acute central nervous system injury.

    Science.gov (United States)

    Jiménez, Raquel; Casado-Flores, Juan; Nieto, Monserrat; García-Teresa, María Angeles

    2006-10-01

    The purpose of this investigation was to describe the causes, clinical pattern, and treatment of cerebral salt wasting syndrome in children with acute central nervous system injury. This retrospective study focused on patientscerebral salt wasting syndrome, over a period of 7 years, in the pediatric intensive care unit of a tertiary care hospital. Selection criteria included evidence of hyponatremia (serum sodium120 mEq/L), and volume depletion. Fourteen patients were identified with cerebral salt wasting syndrome, 12 after a neurosurgical procedure (8 brain tumor, 4 hydrocephalus) and 2 after severe brain trauma. In 11 patients the cerebral salt wasting syndrome was diagnosed during the first 48 hours of admission. Prevalence of cerebral salt wasting syndrome in neurosurgical children was 11.3/1000 surgical procedures. The minimum sodium was 122+/-7 mEq/L, the maximum urine osmolarity 644+/-59 mOsm/kgH2O. The maximum sodium supply was 1 mEq/kg/h (range, 0.1-2.4). The mean duration of cerebral salt wasting syndrome was 6+/-5 days (range 1-9). In conclusion, cerebral salt wasting syndrome can complicate the postoperative course of children with brain injury; it is frequently present after surgery for brain tumors and hydrocephalus and in patients with severe head trauma. Close monitoring of salt and fluid balance is essential to prevent severe neurologic and hemodynamic complications.

  9. The primary Maxillary Central Incisor in the Solitary Median Maxillary Central Incisor syndrome

    DEFF Research Database (Denmark)

    Kjaer, I; Balslev-Olesen, M

    2012-01-01

    Solitary Median Maxillary Central Incisor (SMMCI) is a developmental anomaly in the permanent dentition with one single central incisor in the maxilla, positioned exactly in the midline. This condition has been associated with extra- and intraoral malformations in the frontonasal segment of the c...

  10. Testosterone Plus Finasteride Treatment After Spinal Cord Injury

    Science.gov (United States)

    2016-07-07

    Spinal Cord Injury; Spinal Cord Injuries; Trauma, Nervous System; Wounds and Injuries; Central Nervous System Diseases; Nervous System Diseases; Spinal Cord Diseases; Gonadal Disorders; Endocrine System Diseases; Hypogonadism; Genital Diseases, Male

  11. Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome.

    Science.gov (United States)

    Hall, R K; Bankier, A; Aldred, M J; Kan, K; Lucas, J O; Perks, A G

    1997-12-01

    This article describes a series of 21 consecutive cases, each involving a solitary median maxillary central incisor; the patients were seen in the Department of Dentistry or the Victorian Clinical Genetics Unit, Murdoch Institute, at the Royal Children's Hospital, Melbourne, from 1966 to 1997. The spectrum of anomalies and associated features present in these cases--solitary median maxillary central incisor, choanal atresia, and holoprosencephaly--is described, and the literature related to the features, including genetic studies in these conditions, is reviewed. We relate our findings in these cases to current knowledge of developmental embryology. It is hoped that the findings, together with our interpretation of them, will help to clarify understanding of solitary median maxillary central incisor syndrome. This syndrome was previously considered a simple midline defect of the dental lamina, but it is now recognized as a possible predictor of holoprosencephalies of varying degrees in the proband, in members of the proband's family, and in the family's descendants. PMID:9431535

  12. Central nervous system syndromes in solid organ transplant recipients.

    Science.gov (United States)

    Wright, Alissa J; Fishman, Jay A

    2014-10-01

    Solid organ transplant recipients have a high incidence of central nervous system (CNS) complications, including both focal and diffuse neurologic deficits. In the immunocompromised host, the initial clinical evaluation must focus on both life-threatening CNS infections and vascular or anatomic lesions. The clinical signs and symptoms of CNS processes are modified by the immunosuppression required to prevent graft rejection. In this population, these etiologies often coexist with drug toxicities and metabolic abnormalities that complicate the development of a specific approach to clinical management. This review assesses the multiple risk factors for CNS processes in solid organ transplant recipients and establishes a timeline to assist in the evaluation and management of these complex patients.

  13. MRI morphometric characterisation of the paediatric cervical spine and spinal cord in children with MPS IVA (Morquio-Brailsford syndrome).

    Science.gov (United States)

    Solanki, Guirish A; Lo, William B; Hendriksz, Christian J

    2013-03-01

    Nearly all children with MPS IVA develop skeletal deformities affecting the spine. At the atlanto-axial spine, odontoid hypoplasia occurs. GAG deposition around the dens, leads to peri-odontoid infiltration. Transverse/alar ligament incompetence causes instability. Atlanto-axial instability is associated with cord compression and myelopathy, leading to major morbidity and mortality. Intervention is often required. Does the presence of widened bullet shaped vertebra in platyspondily encroach on the spinal canal and cause spinal stenosis in MPS IVA? So far, there have been no standardised morphometric measurements of the paediatric MPS IVA cervical spine to evaluate whether there is pre-existing spinal stenosis predisposing to compressive myelopathy or whether this is purely an acquired process secondary to instability and compression. This study provides the first radiological quantitative analysis of the cervical spine and spinal cord in a series of affected children. MRI morphometry indicates that the MPS IVA spine is narrower at C1-2 level giving an inverted funnel shape. There is no evidence of a reduction in the Torg ratio (canal-body ratio) in the cervical spine. The spinal canal does not exceed 11 mm at any level, significantly smaller than normal historical cohorts (14 mm). The sagittal diameter and axial surface area of both spinal canal and cord are reduced. C1-2 level cord compression was evident in the canal-cord ratio but the Torg ratio was not predictive of cord compression. In MPS IVA the reduction in the space available for the cord (SAC) is multifactorial rather than due to congenital spinal stenosis. PMID:23404316

  14. MRI morphometric characterisation of the paediatric cervical spine and spinal cord in children with MPS IVA (Morquio-Brailsford syndrome).

    Science.gov (United States)

    Solanki, Guirish A; Lo, William B; Hendriksz, Christian J

    2013-03-01

    Nearly all children with MPS IVA develop skeletal deformities affecting the spine. At the atlanto-axial spine, odontoid hypoplasia occurs. GAG deposition around the dens, leads to peri-odontoid infiltration. Transverse/alar ligament incompetence causes instability. Atlanto-axial instability is associated with cord compression and myelopathy, leading to major morbidity and mortality. Intervention is often required. Does the presence of widened bullet shaped vertebra in platyspondily encroach on the spinal canal and cause spinal stenosis in MPS IVA? So far, there have been no standardised morphometric measurements of the paediatric MPS IVA cervical spine to evaluate whether there is pre-existing spinal stenosis predisposing to compressive myelopathy or whether this is purely an acquired process secondary to instability and compression. This study provides the first radiological quantitative analysis of the cervical spine and spinal cord in a series of affected children. MRI morphometry indicates that the MPS IVA spine is narrower at C1-2 level giving an inverted funnel shape. There is no evidence of a reduction in the Torg ratio (canal-body ratio) in the cervical spine. The spinal canal does not exceed 11 mm at any level, significantly smaller than normal historical cohorts (14 mm). The sagittal diameter and axial surface area of both spinal canal and cord are reduced. C1-2 level cord compression was evident in the canal-cord ratio but the Torg ratio was not predictive of cord compression. In MPS IVA the reduction in the space available for the cord (SAC) is multifactorial rather than due to congenital spinal stenosis.

  15. Preschool Weight and Body Mass Index in Relation to Central Obesity and Metabolic Syndrome in Adulthood

    DEFF Research Database (Denmark)

    Graversen, Lise; Sørensen, Thorkild I A; Petersen, Liselotte;

    2014-01-01

    BACKGROUND: If preschool measures of body size routinely collected at preventive health examinations are associated with adult central obesity and metabolic syndrome, a focused use of these data for the identification of high risk children is possible. The aim of this study was to test...... the associations between preschool weight and body mass index (BMI) and adult BMI, central obesity and metabolic alterations. METHODS: The Northern Finland Birth Cohort 1966 (NFBC1966) (N = 4111) is a population-based cohort. Preschool weight (age 5 months and 1 year) and BMI (age 2-5 years) were studied...... of adult obesity of 6.2(95% CI:4.2-9.3), of adult central obesity of 2.4(95% CI:2.0-2.9), and of early onset adult metabolic syndrome of 2.5(95% CI:1.7-3.8). CONCLUSIONS: High preschool BMI is consistently associated with adult obesity, central obesity and early onset metabolic syndrome. Routinely...

  16. Assessment of Cord Blood Vascular Endothelial Growth Factor Levels and Circulating CD34+ Cells in Preterm Infants with Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Azza Tawfeek Moawed, Nihad Ahmed El Nashar

    2012-04-01

    Full Text Available Respiratory distress syndrome (RDS secondary to surfactant deficiency is a common cause of mobility and mortality in premature infants. Vascular endothelial growth factor (VEGF is a major angiogenic factor and prime regulator of endothelial cells proliferation. So, VEGF may contribute to surfactant secretion and pulmonary maturation. Additionally, circulating CD34+ stem – progenitor cells are elevated along with its mobilizing cytokines in neonatal RDS. Aim of work: This study aimed to elucidate the role of cord blood VEGF and the circulating CD34+ cells in preterm infants with and without RDS. Patients & method: This study was conducted on 55 preterm neonates divided into 25 preterm (15 males/ 10 females without RDS with mean age of 31.60 ± 1.56 weeks and 30 preterm neonates with RDS (18 males/ 12 females with mean age of 29.95 ± 1.09 weeks . Twenty healthy neonates (14 males/ 6 females served as controls with mean age of 38.20 ± 3.57 weeks. All neonates were subjected to full history taking; thorough clinical examination and laboratory investigations including determination of VEGF levels in cord blood samples using ELISA and circulating CD34+ cells in peripheral blood by flowcytometery. Results:The results of this study revealed that cord blood VEGF levels were significantly decreased in preterms with RDS versus preterms without RDS and controls with p values of both < 0.0001. Furthermore, the circulating CD34+ cells were significantly increased in preterm infants with RDS versus preterm infants without RDS and controls (p < 0.05 & < 0.0001 respectively. Premature rupture of the membrane, gender of the newborn, birth weight and antenatal steroid administration had neither significant effect on the cord blood VEGF nor on the number of CD34+ cells. There was inverse significant correlation between GA and the number of CD34+ cells. Conclusion:It was concluded that low cord blood VEGF is associated with RDS and its level negatively

  17. DiGeorge syndrome associated with solitary median maxillary central incisor.

    Science.gov (United States)

    Yang, Huai-Chih; Shyur, Shyh-Dar; Huang, Li-Hsin; Chang, Yi-Chi; Wen, Da-Chin; Liang, Pei-Hsuan; Lin, Mao-Tsair

    2005-01-01

    DiGeorge syndrome is a primary immunodeficiency disease characterized by dysgenesis of the thymus and parathyroid glands, conotruncal cardiac anomalies, and other dysmorphic features. Although most patients have a common microscopic deletion in chromosome 22q11.2, marked clinical variability exists. A solitary median maxillary central incisor (SMMCI) is a rare dental anomaly which may be an isolated occurrence or associated with congenital nasal airway abnormalities or holoprosencephaly. We report a patient with DiGeorge syndrome who was diagnosed at nearly 1 month of age and was later found to have a solitary median central incisor. Initially, the patient presented with recurrent episodes of respiratory distress attributed to partial airway obstruction, one of the phenotypic features of SMMCI. A fluorescence in situ hybridization study showed a chromosome 22q11.2 deletion. PMID:16252847

  18. PHOX2B mutations in three Chinese patients with congenital central hypoventilation syndrome

    Institute of Scientific and Technical Information of China (English)

    Siu-Fong June Or; Ming-for Tony Tong; Fai-Man Ivan Lo; Chi-Wai Law; Ting-Yat Miu; Delphine Trochet; Tak-Sum Stephen Lam

    2006-01-01

    @@ Congenital central hypoventilation syndrome(CCHS, OMIM #209880) is a rare autosomal dominant disorder of the autonomic nervous system(ANS) characterized by an abnormal autonomic ventilatory response to progressive hypercarbia and sustained hypoxemia. Patients typically present in the newborn period with hypoventilation or apnea asleep, awake, or both, without any associated cardiac, pulmonary, neuromuscular or brainstem lesions. Rarely, some patients may present at a later age and are diagnosed to have late onset central hypoventilation syndrome (LOCHS).1 Other features of ANS dysfunction such as feeding difficulty due to oesophageal dysmotility, severe constipation in the absence of Hirschsprung disease, poor regulation of basal body temperature, episodes of profuse sweating, pupillary and ocular abnormalities,decreased beat-to-beat variability of heart rate,attenuated response of heart rate to exercise,abnormal fluctuation of blood pressure, decreased perception to pain, and decreased perception to anxiety may be variably present but not essential for diagnosis.

  19. Ramsay Hunt Syndrome Associated with Central Nervous System Involvement in an Adult.

    Science.gov (United States)

    Chan, Tommy L H; Cartagena, Ana M; Bombassaro, Anne Marie; Hosseini-Moghaddam, Seyed M

    2016-01-01

    Ramsay Hunt syndrome associated with varicella zoster virus reactivation affecting the central nervous system is rare. We describe a 55-year-old diabetic female who presented with gait ataxia, right peripheral facial palsy, and painful vesicular lesions involving her right ear. Later, she developed dysmetria, fluctuating diplopia, and dysarthria. Varicella zoster virus was detected in the cerebrospinal fluid by polymerase chain reaction. She was diagnosed with Ramsay Hunt syndrome associated with spread to the central nervous system. Her facial palsy completely resolved within 48 hours of treatment with intravenous acyclovir 10 mg/kg every 8 hours. However, cerebellar symptoms did not improve until a tapering course of steroid therapy was initiated. PMID:27366189

  20. Neuroradiographic diagnosis and surgical repair of tethered cord syndrome in an English Bulldog with spina bifida and myeloschisis

    International Nuclear Information System (INIS)

    A 3-month-old English Bulldog had excretory incontinence and sensory deficits in the distribution of pudendal nerves. Noncontrast radiography, myelography, and computed tomography revealed spina bifida beginning at L7, an expanded subarachnoid space caudal to L7, and a taut, thick filum terminale. Microsurgical exploration of the lumbosacral spine confirmed the presence of a tethered cord, and the filum terminale was transected. The spinal cord immediately migrated cranially about 1 cm. Although some sensory improvement was evident during a 2-week postoperative period, the dog was euthanatized. Postmortem examination confirmed spina bifida and atrophy of sacral nerve roots and perineal muscles, thoracic hemivertebrae, and hydrocephalus

  1. Two uncommon manifestations of leptospirosis:Sweet’s syndrome and central nervous system vasculitis

    Institute of Scientific and Technical Information of China (English)

    Peter George

    2011-01-01

    To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide. The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries, at most clinical suspicion it is essential in the diagnosis of this disease. In this report, we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.

  2. Traumatic cervical cord transection without facet dislocations--a proposal of combined hyperflexion-hyperextension mechanism: a case report.

    Science.gov (United States)

    Cha, Yoo-Hyun; Cho, Tai-Hyoung; Suh, Jung-Keun

    2010-08-01

    A patient is presented with a cervical spinal cord transection which occurred after a motor vehicle accident in which the air bag deployed and the seat belt was not in use. The patient had complete quadriplegia below the C5 level and his imaging study showed cervical cord transection at the level of the C5/6 disc space with C5, C6 vertebral bodies and laminar fractures. He underwent a C5 laminectomy and a C4-7 posterior fusion with lateral mass screw fixation. Previous reports have described central cord syndromes occurring in hyperextension injuries, but in adults, acute spinal cord transections have only developed after fracture-dislocations of the spine. A case involving a post-traumatic spinal cord transection without any evidence of radiologic facet dislocations is reported. Also, we propose a combined hyperflexion-hyperextension mechanism to explain this type of injury.

  3. Diagnostic reliability of magnetic resonance imaging for central nervous system syndromes in systemic lupus erythematosus: a prospective cohort study

    OpenAIRE

    Nishimura Katsuji; Kanno Tokiko; Soejima Makoto; Fukasawa Chikako; Kawaguchi Yasushi; Harigai Masayoshi; Katsumata Yasuhiro; Yamada Takayuki; Yamanaka Hisashi; Hara Masako

    2010-01-01

    Abstract Background Previous studies of magnetic resonance imaging (MRI) as a diagnostic tool for central nervous system (CNS) syndromes in systemic lupus erythematosus (SLE) contained several limitations such as study design, number of enrolled patients, and definition of CNS syndromes. We overcame these problems and statistically evaluated the diagnostic values of abnormal MRI signals and their chronological changes in CNS syndromes of SLE. Methods We prospectively studied 191 patients with...

  4. A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia.

    Science.gov (United States)

    Blackmore, Kate; Wynne, David M

    2010-08-01

    Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed. PMID:20627328

  5. Immediate Return to Ambulation and Improved Functional Capacity for Rehabilitation in Complex Regional Pain Syndrome following Early Implantation of a Spinal Cord Stimulation System

    Directory of Open Access Journals (Sweden)

    Brandon Jesse Goff

    2014-01-01

    Full Text Available Complex regional pain syndrome (CRPS is a neuropathic pain condition that is characterized by vasomotor, sensory, sudomotor, and motor symptoms. Spinal cord stimulation (SCS has been successfully utilized for the treatment of pain refractory to conventional therapies. We present a case of a previously highly functioning 54-year-old female who developed a rarely reported case of idiopathic CRPS of the right ankle which spontaneously occurred four months after an uncomplicated anterior cervical disc fusion. This condition resulted in severe pain and functional impairment that was unresponsive to pharmacological management. The patient’s rehabilitation was severely stymied by her excruciating pain. However, with the initiation of spinal cord stimulation, her pain was adequately controlled allowing for progression to full unassisted ambulation, advancing functional capacity, and improving quality of life. This case report supports the concept that rapid progression to neuromodulation, rather than delays that occur due to attempts at serial sympathetic blocks, may better control symptoms leading allowing for a more meaningful recovery.

  6. Immediate Return to Ambulation and Improved Functional Capacity for Rehabilitation in Complex Regional Pain Syndrome following Early Implantation of a Spinal Cord Stimulation System.

    Science.gov (United States)

    Goff, Brandon Jesse; Naber, Jeremy Wingseng; McCallin, John Patrick; Lopez, Edward Michael; Guthmiller, Kevin Brant; Lautenschlager, Karl Alan; Lai, Tristan Toll; Hommer, Dean Harry; Marin, Gonzalez Raul

    2014-01-01

    Complex regional pain syndrome (CRPS) is a neuropathic pain condition that is characterized by vasomotor, sensory, sudomotor, and motor symptoms. Spinal cord stimulation (SCS) has been successfully utilized for the treatment of pain refractory to conventional therapies. We present a case of a previously highly functioning 54-year-old female who developed a rarely reported case of idiopathic CRPS of the right ankle which spontaneously occurred four months after an uncomplicated anterior cervical disc fusion. This condition resulted in severe pain and functional impairment that was unresponsive to pharmacological management. The patient's rehabilitation was severely stymied by her excruciating pain. However, with the initiation of spinal cord stimulation, her pain was adequately controlled allowing for progression to full unassisted ambulation, advancing functional capacity, and improving quality of life. This case report supports the concept that rapid progression to neuromodulation, rather than delays that occur due to attempts at serial sympathetic blocks, may better control symptoms leading allowing for a more meaningful recovery. PMID:25525522

  7. Editorial review: an update on central sensitivity syndromes and the issues of nosology and psychobiology.

    Science.gov (United States)

    Yunus, Muhammad B

    2015-01-01

    Central sensitization (CS), simply defined as an amplified response of the central nervous system to peripheral input, is a concept of great importance in clinical medicine. It has helped to explain aspects of the pathophysiology of common diseases, e.g. fibromyalgia syndrome (FMS), irritable bowel syndrome, vulvodynia, headaches, chronic pelvic pain and other overlapping conditions (collectively called central sensitivity syndromes, or CSS). It also applies to pain of complex regional pain syndrome, osteoarthritis (OA), rheumatoid arthritis (RA) and post-operative pain. The pathology-pain gap in CSS is readily explained by CS. Many FMS and other CSS patients have peripheral pathology, e.g. nociceptive areas in the muscles, arthritis, small fiber neuropathy and inflammation. Pro-inflammatory cytokines are elevated in some patients. Identification of CS in patients with structural pathology, e.g. OA and RA, has helped to explain why not all patients benefit from nonsteroidal anti-inflammatory drugs or joint replacement surgery, and require therapy directed at CS. Glial cells are important in pain processing. Remarkable advances have been achieved in neuroimaging, including visualization of grey matter and white matter, not only during provoked pain but also pain at rest. Based on CS mechanisms, targeted individual therapy may now be possible. Appropriate nosology is important particularly for effective patient care. Dichotomy of neurochemical-structural ("functional") and structural ("organic") pathology should be abandoned; many patients have both. Psychobiology is also biology. Patient-blaming terms like somatization, somatizer and catastrophizing should be avoided. For therapy, both pharmacological and non- pharmacological approaches are important, including recognition of subgroups and person/patient-centered care.

  8. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Cord Injury Psychological Realities After Spinal Cord Injury Psychology of Spinal Cord Injury Rehabilitation Psychology of Spinal Cord Injury Rehabilitation How Psychologists Help ...

  9. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Cord Injury Psychological Realities after Spinal Cord Injury Psychology of Spinal Cord Injury Rehabilitation Psychology of Spinal Cord Injury Rehabilitation How Psychologists Help ...

  10. Surgical strategy of tethered cord syndrome with lipoma%脂肪瘤型脊髓栓系综合征的手术技巧

    Institute of Scientific and Technical Information of China (English)

    刘志坚; 蒋健; 赵寅涛

    2008-01-01

    目的 探讨脂肪瘤型脊髓栓系综合征的手术治疗技巧. 方法 自2003年至2006年,我科共收治33例脂肪瘤型脊髓栓系患者,均在电生理监测下切除脂肪瘤,显微手术分离粘连、松解脊髓,以无损伤不可吸收缝线(Prolene线,5-0)连续缝合硬脊膜,一期行硬脊膜重建;翻转腰背部筋膜行先天性缺损修补与加固. 结果 29例患者切口一期愈合:4例患者出现脂肪坏死导致皮下积液,其中2例患者出现脑脊液漏,经缝合及引流后治愈.随访1~3年,28例患者临床症状有显著改善,5例患者虽无改善亦无神经系统症状加重;复查均无再栓系形成. 结论 脂肪瘤型脊髓栓系综合征早期电生理监测下行栓系松解术,同时行硬脊膜的修补与重建,对于预防术后再栓系至关重要.%Objective To explore the surgical strategy for tethered cord syndrome with lipoma.Methods Operations for releasing of tethered conus medullaris were composed of lipomas excision,adhesion releasing, terminal filum resection and reconstruction of dura mate of spinal cord. Fascia of the surrounding muscle with nonabsorbable suture (Prolene, 5-0) was used in duraplasty operation.Microsurgical release of the tethered cord was performed in each case while using multimodality intraoperative neurophysiological monitoring. Results Complication of subcutaneous fluidify included superficial wound infection in 4 patients and cerebrospinal fluid leakage in 2 patients, which were managed by reinforcement sutures and temporary external drainage. During the follow-up of 1-3years, 28 (84.8%) cases get better obviously in symptoms, and MRI re-examination showed no tethered cord in them. The other 5 cases did not suffer from an aggravation of nervous symptoms although a significant improvement was not achieved in them. Conclusions Early operation for tethered cord release should be performed under intraoperative neurophysiological monitoring to prevent the development of

  11. Central Diabetes Insipidus and Cisplatin-Induced Renal Salt Wasting Syndrome: A Challenging Combination.

    Science.gov (United States)

    Cortina, Gerard; Hansford, Jordan R; Duke, Trevor

    2016-05-01

    We describe a 2-year-old female with a suprasellar primitive neuroectodermal tumor and central diabetes insipidus (DI) who developed polyuria with natriuresis and subsequent hyponatremia 36 hr after cisplatin administration. The marked urinary losses of sodium in combination with a negative sodium balance led to the diagnosis of cisplatin-induced renal salt wasting syndrome (RSWS). The subsequent clinical management is very challenging. Four weeks later she was discharged from ICU without neurological sequela. The combination of cisplatin-induced RSWS with DI can be confusing and needs careful clinical assessment as inaccurate diagnosis and management can result in increased neurological injury.

  12. Clinical course, management and in-hospital outcomes of acute coronary syndrome in Central Asian women

    Directory of Open Access Journals (Sweden)

    Ravshanbek Kurbanov

    2011-03-01

    Full Text Available The paper describes features history, risk factors and hospital management of acute coronary syndrome (ACS in women of the Central Asia (829 ACS patients are included, and also comparison of hospital outcomes of ACS in groups of men and women.ACS in women in the Central Asia comprises features as more non Q-wave myocardial infarction frequency, smaller adherence to treatment of dyslipidemia, more obesity degrees. Women arrive to hospital average in 4 hours after men; have more percent of the atypical form. In-hospital outcomes in men and women significantly did not differ because of general tendency to late arriving to the hospital and small quantity of revascularization procedures. The reasons of differences between men and women in the region are mostly connected to social sphere (late arriving, non-treated dyslipidemia than to physiological peculiarities.

  13. Cytomegalovirus upregulates expression of CCR5 in central memory cord blood mononuclear cells, which may facilitate in utero HIV type 1 transmission.

    Science.gov (United States)

    Johnson, Erica L; Howard, Chanie L; Thurman, Joy; Pontiff, Kyle; Johnson, Elan S; Chakraborty, Rana

    2015-01-15

    Administration of combination antiretroviral therapy to human immunodeficiency virus type 1 (HIV-1)-infected pregnant women significantly reduces vertical transmission. In contrast, maternal co-opportunistic infection with primary or reactivated cytomegalovirus (CMV) or other pathogens may facilitate in utero transmission of HIV-1 by activation of cord blood mononuclear cells (CBMCs). Here we examine the targets and mechanisms that affect fetal susceptibility to HIV-1 in utero. Using flow cytometry, we demonstrate that the fraction of CD4(+)CD45RO(+) and CD4(+)CCR5(+) CBMCs is minimal, which may account for the low level of in utero HIV-1 transmission. Unstimulated CD4(+) CBMCs that lack CCR5/CD45RO showed reduced levels of HIV-1 infection. However, upon in vitro stimulation with CMV, CBMCs undergo increased proliferation to upregulate the fraction of T central memory cells and expression of CCR5, which enhances susceptibility to HIV-1 infection in vitro. These data suggest that activation induced by CMV in vivo may alter CCR5 expression in CD4(+) T central memory cells to promote in utero transmission of HIV-1.

  14. Cord Blood

    Directory of Open Access Journals (Sweden)

    Saeed Abroun

    2014-05-01

    Full Text Available   Stem cells are naïve or master cells. This means they can transform into special 200 cell types as needed by body, and each of these cells has just one function. Stem cells are found in many parts of the human body, although some sources have richer concentrations than others. Some excellent sources of stem cells, such as bone marrow, peripheral blood, cord blood, other tissue stem cells and human embryos, which last one are controversial and their use can be illegal in some countries. Cord blood is a sample of blood taken from a newborn baby's umbilical cord. It is a rich source of stem cells, umbilical cord blood and tissue are collected from material that normally has no use following a child’s birth. Umbilical cord blood and tissue cells are rich sources of stem cells, which have been used in the treatment of over 80 diseases including leukemia, lymphoma and anemia as bone marrow stem cell potency.  The most common disease category has been leukemia. The next largest group is inherited diseases. Patients with lymphoma, myelodysplasia and severe aplastic anemia have also been successfully transplanted with cord blood. Cord blood is obtained by syringing out the placenta through the umbilical cord at the time of childbirth, after the cord has been detached from the newborn. Collecting stem cells from umbilical blood and tissue is ethical, pain-free, safe and simple. When they are needed to treat your child later in life, there will be no rejection or incompatibility issues, as the procedure will be using their own cells. In contrast, stem cells from donors do have these potential problems. By consider about cord blood potency, cord blood banks (familial or public were established. In IRAN, four cord blood banks has activity, Shariati BMT center cord blood bank, Royan familial cord blood banks, Royan public cord blood banks and Iranian Blood Transfusion Organ cord blood banks. Despite 50,000 sample which storage in these banks, but the

  15. Prospective double-blind preoperative pain clinic screening before microsurgical denervation of the spermatic cord in patients with testicular pain syndrome.

    Science.gov (United States)

    Oomen, Robert J A; Witjens, Annemijke C; van Wijck, Albert J M; Grobbee, Diederik E; Lock, Tycho M T W

    2014-09-01

    Testicular pain syndrome (TPS), defined as an intermittent or constant pain in one or both testicles for at least 3 months, resulting in significant reduction of daily activities, is common. Microsurgical denervation of the spermatic cord (MDSC) has been suggested as an effective treatment option. The study population comprised 180 TPS patients admitted to our outpatient urology clinic between 1999 and 2011. On 3 different occasions, patients were offered a double-blind, placebo-controlled temporary blockade of the spermatic cord. A single blockade consisted of 10 mL 2% lidocaine, 10 mL 0.25% bupivacaine, or 10 mL 0.9% sodium chloride. If the results of these blockades were positive, MDSC was offered. All MDSCs were performed by a single urologist (M.T.W.T.L.) using an inguinal approach. Pain reduction was determined at prospective follow-up. This study evaluated 180 patients. Most patients (61.1%) had undergone a scrotal or inguinal procedure. Patients had complaints during sexual activities (51.7%), sitting (37.5%), and/or cycling (36.7%); 189 randomized blockades were offered to all patients. There was a positive response in 37% and a negative response in 51%. MDSC was performed on 58 testicular units, including 3 patients with a negative outcome of the blockades. At mean follow-up of 42.8 months, 86.2% had a ≥ 50% reduction of pain and 51.7% were completely pain free. MDSC is a valuable treatment option for TPS patients because in this study 86.2% experienced a ≥ 50% reduction of pain. To prevent superfluous diagnostics and treatment, it is mandatory to follow a systematic protocol in the treatment of TPS.

  16. Central poststroke pain: somatosensory abnormalities and the presence of associated myofascial pain syndrome

    Directory of Open Access Journals (Sweden)

    de Oliveira Rogério Adas

    2012-09-01

    Full Text Available Abstract Background Central post-stroke pain (CPSP is a neuropathic pain syndrome associated with somatosensory abnormalities due to central nervous system lesion following a cerebrovascular insult. Post-stroke pain (PSP refers to a broader range of clinical conditions leading to pain after stroke, but not restricted to CPSP, including other types of pain such as myofascial pain syndrome (MPS, painful shoulder, lumbar and dorsal pain, complex regional pain syndrome, and spasticity-related pain. Despite its recognition as part of the general PSP diagnostic possibilities, the prevalence of MPS has never been characterized in patients with CPSP patients. We performed a cross-sectional standardized clinical and radiological evaluation of patients with definite CPSP in order to assess the presence of other non-neuropathic pain syndromes, and in particular, the role of myofascial pain syndrome in these patients. Methods CPSP patients underwent a standardized sensory and motor neurological evaluation, and were classified according to stroke mechanism, neurological deficits, presence and profile of MPS. The Visual Analogic Scale (VAS, McGill Pain Questionnaire (MPQ, and Beck Depression Scale (BDS were filled out by all participants. Results Forty CPSP patients were included. Thirty-six (90.0% had one single ischemic stroke. Pain presented during the first three months after stroke in 75.0%. Median pain intensity was 10 (5 to 10. There was no difference in pain intensity among the different lesion site groups. Neuropathic pain was continuous-ongoing in 34 (85.0% patients and intermittent in the remainder. Burning was the most common descriptor (70%. Main aggravating factors were contact to cold (62.5%. Thermo-sensory abnormalities were universal. MPS was diagnosed in 27 (67.5% patients and was more common in the supratentorial extra-thalamic group (P Conclusions The presence of MPS is not an exception after stroke and may present in association with CPSP

  17. Myoclonic status and central fever in Angelman syndrome due to paternal uniparental disomy.

    Science.gov (United States)

    Nicita, Francesco; Garone, Giacomo; Papetti, Laura; Consoli, Federica; Magliozzi, Monia; De Luca, Alessandro; Spalice, Alberto

    2015-01-01

    Myoclonic status in nonprogressive encephalopathy (MSNE) is an early-onset, drug-resistant epileptic syndrome characterized by occurrence of continuous diffuse epileptiform abnormalities, associated with positive and/or negative phenomena and accompanied by transient and recurring motor, cognitive, and behavioral impairment. MSNE has been reported in Angelman syndrome (AS) secondary to 15q11-13 deletions or UBE3A mutations but not to paternal uniparental disomy (UPD). We describe the case of a male patient with AS caused by UPD who developed a myoclonic status (MS) associated with long-lasting fever of central origin, both promptly regressed with introduction of levetiracetam. Only three descriptions of thermal dysregulation in AS exist, and none of the previously reported cases were associated with MS or with UPD. Association of MS and central fever expands the spectrum of epileptic and non-epileptic features in UPD-related AS and provides a further evidence of hypothalamus involvement in the pathogenesis of this neurodevelopmental disorder. PMID:26559560

  18. Central nervous system

    Science.gov (United States)

    The central nervous system is composed of the brain and spinal cord. Your brain and spinal cord serve as the main "processing center" for your entire nervous system. They control all the workings of your body.

  19. Central leptin resistance and hypothalamic inflammation are involved in letrozole-induced polycystic ovary syndrome rats.

    Science.gov (United States)

    Lian, Yuling; Zhao, Fangui; Wang, Wenjun

    2016-08-01

    Accumulating evidence indicates that leptin acts as an important mediator in energy homeostasis and reproduction. Since dysfunction of reproduction and metabolism are major characteristics of polycystic ovarian syndrome (PCOS), the role of leptin in pathogenesis of PCOS needs further research. Many studies have shown that central leptin resistance existed in obesity rats through leptin intracerebroventricular (icv) injection; however, central leptin resistance in PCOS rats has not been reported. This study aimed to investigate whether there was a state of central leptin resistance in PCOS rats, as well as explore the possible association of hypothalamic inflammation with central leptin resistance. First, letrozole was used to induce the PCOS model, 24 h food intake, 24 h body weight changes and the expression of p-STAT3 were determined following leptin or artificial cerebrospinal fluid (aCSF) icv injection in rats. Second, we further evaluated the expressions of IL-1β, IL-6, TNF-α, p-IKKβ, NF-κB, p-NF-κB, IκBα, p-IκBα and SOCS3 in hypothalamus. The results showed that 24 h food intake and body weight were decreased, while the expression of p-STAT3 was increased in control group rats following leptin icv injection compared with aCSF icv injection; however, both of them showed no significant difference in PCOS rats. Furthermore, inflammatory markers were upregulated in the hypothalami of PCOS rats. Taken together, our data indicated that there was a state of chronic low-grade inflammation in hypothalamus which might be the possible mechanism for central leptin resistance in PCOS rats. PMID:27233601

  20. Toward a Broader View of Ube3a in a Mouse Model of Angelman Syndrome: Expression in Brain, Spinal Cord, Sciatic Nerve and Glial Cells.

    Directory of Open Access Journals (Sweden)

    Mark D Grier

    Full Text Available Angelman Syndrome (AS is a devastating neurodevelopmental disorder characterized by developmental delay, speech impairment, movement disorder, sleep disorders and refractory epilepsy. AS is caused by loss of the Ube3a protein encoded for by the imprinted Ube3a gene. Ube3a is expressed nearly exclusively from the maternal chromosome in mature neurons. While imprinting in neurons of the brain has been well described, the imprinting and expression of Ube3a in other neural tissues remains relatively unexplored. Moreover, given the overwhelming deficits in brain function in AS patients, the possibility of disrupted Ube3a expression in the infratentorial nervous system and its consequent disability have been largely ignored. We evaluated the imprinting status of Ube3a in the spinal cord and sciatic nerve and show that it is also imprinted in these neural tissues. Furthermore, a growing body of clinical and radiological evidence has suggested that myelin dysfunction may contribute to morbidity in many neurodevelopmental syndromes. However, findings regarding Ube3a expression in non-neuronal cells of the brain have varied. Utilizing enriched primary cultures of oligodendrocytes and astrocytes, we show that Ube3a is expressed, but not imprinted in these cell types. Unlike many other neurodevelopmental disorders, AS symptoms do not become apparent until roughly 6 to 12 months of age. To determine the temporal expression pattern and silencing, we analyzed Ube3a expression in AS mice at several time points. We confirm relaxed imprinting of Ube3a in neurons of the postnatal developing cortex, but not in structures in which neurogenesis and migration are more complete. This furthers the hypothesis that the apparently normal window of development in AS patients is supported by an incompletely silenced paternal allele in developing neurons, resulting in a relative preservation of Ube3a expression during this crucial epoch of early development.

  1. Toward a Broader View of Ube3a in a Mouse Model of Angelman Syndrome: Expression in Brain, Spinal Cord, Sciatic Nerve and Glial Cells.

    Science.gov (United States)

    Grier, Mark D; Carson, Robert P; Lagrange, Andre Hollis

    2015-01-01

    Angelman Syndrome (AS) is a devastating neurodevelopmental disorder characterized by developmental delay, speech impairment, movement disorder, sleep disorders and refractory epilepsy. AS is caused by loss of the Ube3a protein encoded for by the imprinted Ube3a gene. Ube3a is expressed nearly exclusively from the maternal chromosome in mature neurons. While imprinting in neurons of the brain has been well described, the imprinting and expression of Ube3a in other neural tissues remains relatively unexplored. Moreover, given the overwhelming deficits in brain function in AS patients, the possibility of disrupted Ube3a expression in the infratentorial nervous system and its consequent disability have been largely ignored. We evaluated the imprinting status of Ube3a in the spinal cord and sciatic nerve and show that it is also imprinted in these neural tissues. Furthermore, a growing body of clinical and radiological evidence has suggested that myelin dysfunction may contribute to morbidity in many neurodevelopmental syndromes. However, findings regarding Ube3a expression in non-neuronal cells of the brain have varied. Utilizing enriched primary cultures of oligodendrocytes and astrocytes, we show that Ube3a is expressed, but not imprinted in these cell types. Unlike many other neurodevelopmental disorders, AS symptoms do not become apparent until roughly 6 to 12 months of age. To determine the temporal expression pattern and silencing, we analyzed Ube3a expression in AS mice at several time points. We confirm relaxed imprinting of Ube3a in neurons of the postnatal developing cortex, but not in structures in which neurogenesis and migration are more complete. This furthers the hypothesis that the apparently normal window of development in AS patients is supported by an incompletely silenced paternal allele in developing neurons, resulting in a relative preservation of Ube3a expression during this crucial epoch of early development. PMID:25894543

  2. Lack of differential pattern in central adiposity and metabolic syndrome in Barrett's esophagus and gastroesophageal reflux disease.

    LENUS (Irish Health Repository)

    Healy, L A

    2012-02-01

    Obesity is an established risk factor for esophageal adenocarcinoma, although the mechanism is unclear. A pathway from reflux to inflammation through metaplasia is the dominant hypothesis, and an added role relating to visceral adiposity and the metabolic syndrome has been mooted in Barrett\\'s esophagus (BE) patients. Whether BE differs from gastroesophageal reflux disease (GERD) in obesity and metabolic syndrome profiles is unclear, and this was the focus of this study. Patients with proven BE or GERD were randomly selected from the unit data registry and invited to attend for metabolic syndrome screening, anthropometry studies including segmental body composition analysis, and laboratory tests including fasting lipids, insulin, and C-reactive protein. Metabolic syndrome was defined using the National Cholesterol Education Program (NCEP) and the International Diabetes Federation (IDF) criteria. One hundred and eighteen BE patients and 113 age- and sex-matched GERD controls were studied. The incidence of obesity (body mass index >30 kg\\/m(2)) was 36% and 38%, respectively, with the pattern of fat deposition predominantly central and an estimated trunk fat mass of 13 and 14 kg, respectively. Using the NCEP criteria, metabolic syndrome was significantly more common in the BE cohort (30% vs 20%, P < 0.05), but there was no significant difference using IDF criteria (42% vs 37%, P= 0.340). Central obesity and the metabolic syndrome are common in both Barrett\\'s and GERD cohorts, but not significantly different, suggesting that central obesity and the metabolic syndrome does not per se impact on the development of BE in a reflux population. In BE, the importance of obesity and the metabolic syndrome in disease progression merits further study.

  3. Diagnostic reliability of magnetic resonance imaging for central nervous system syndromes in systemic lupus erythematosus: a prospective cohort study

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    Nishimura Katsuji

    2010-01-01

    Full Text Available Abstract Background Previous studies of magnetic resonance imaging (MRI as a diagnostic tool for central nervous system (CNS syndromes in systemic lupus erythematosus (SLE contained several limitations such as study design, number of enrolled patients, and definition of CNS syndromes. We overcame these problems and statistically evaluated the diagnostic values of abnormal MRI signals and their chronological changes in CNS syndromes of SLE. Methods We prospectively studied 191 patients with SLE, comparing those with (n = 57 and without (n = 134 CNS syndrome. CNS syndromes were characterized using the American College of Rheumatology case definitions. Results Any abnormal MRI signals were more frequently observed in subjects in the CNS group (n = 25 than in the non-CNS group (n = 32 [relative risk (RR, 1.7; 95% confidence interval (CI, 1.1-2.7; p = 0.016] and the positive and negative predictive values for the diagnosis of CNS syndrome were 42% and 76%, respectively. Large abnormal MRI signals (ø ≥ 10 mm were seen only in the CNS group (n = 7; RR, 3.7; CI, 2.9-4.7; p = 0.0002, whereas small abnormal MRI signals (ø p = 0.029, whereas small signals did not (p = 1.000. Conclusions Abnormal MRI signals, which showed statistical associations with CNS syndrome, had insufficient diagnostic values. A large MRI signal was, however, useful as a diagnostic and surrogate marker for CNS syndrome of SLE, although it was less common.

  4. A Case of Turner Syndrome with Concomitant Transient Hypogammaglobulinaemia of Infancy and Central Diabetes Insipidus

    Science.gov (United States)

    Korkmaz, Hüseyin Anıl; Özkan, Behzat; Hazan, Filiz; Büyükinan, Muammer; Çelik, Tanju

    2013-01-01

    Turner syndrome (TS) is a genetic disorder that affects development in females and is characterized by the complete or partial absence of the second sex chromosome, or monosomy X. TS is associated with abnormalities in lymphatic and skeletal development, in growth, and in gonadal function. Cardiac and renal malformations and a number of specific cognitive findings may also be encountered in these patients. An increased risk for hypothyroidism, sensorineural hearing loss, hypertension, and other problems has also been reported. We present the case of a patient with TS accompanied by transient hypogammaglobulinaemia of infancy (THI) and central diabetes insipidus, which we believe is the first reported TS patient with these concomitant disorders. Conflict of interest:None declared. PMID:23419422

  5. Mutation of fibulin-1 causes a novel syndrome involving the central nervous system and connective tissues.

    Science.gov (United States)

    Bohlega, Saeed; Al-Ajlan, Huda; Al-Saif, Amr

    2014-05-01

    Fibulin-1 is an extracellular matrix protein that has an important role in the structure of elastic fibers and basement membranes of various tissues. Using homozygosity mapping and exome sequencing, we discovered a missense mutation, p.(Cys397Phe), in fibulin-1 in three patients from a consanguineous family presented with a novel syndrome of syndactyly, undescended testes, delayed motor milestones, mental retardation and signs of brain atrophy. The mutation discovered segregated with the phenotype and was not found in 374 population-matched alleles. The affected cysteine is highly conserved across vertebrates and its mutation is predicted to abolish a disulfide bond that defines the tertiary structure of fibulin-1. Our findings emphasize the crucial role fibulin-1 has in development of the central nervous system and various connective tissues.

  6. A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome.

    Science.gov (United States)

    Yamashita, Chikara; Shigeto, Hiroshi; Maeda, Norihisa; Torii, Takako; Ohyagi, Yasumasa; Kira, Jun-Ichi

    2015-01-01

    Central pontine myelinolysis (CPM), which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI) at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

  7. A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome

    Directory of Open Access Journals (Sweden)

    Chikara Yamashita

    2015-10-01

    Full Text Available Central pontine myelinolysis (CPM, which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

  8. Endocrine and anatomical findings in a case of Solitary Median Maxillary Central Incisor Syndrome.

    Science.gov (United States)

    Szakszon, Katalin; Felszeghy, Enikő; Csízy, István; Józsa, Tamás; Káposzta, Rita; Balogh, Erzsébet; Oláh, Eva; Balogh, István; Berényi, Ervin; Knegt, Alida C; Ilyés, István

    2012-02-01

    Solitary Median Maxillary Central Incisor Syndrome (SMMCI) is a rare malformation syndrome consisting of multiple, mainly midline defects. Some authors suggest that it is a mild manifestation of the wide spectrum of holoprosencephaly, others classify it rather as a distinct entity. Authors report a case of SMMCI presenting with growth retardation, mild intellectual disability and absence of puberty. Cytogenetic and molecular cytogenetic investigations could identify no abnormalities. The presence of a single maxillary incisor called for further investigations to clarify hidden anomalies, these were empty sella, panhypopituitarism, hypothyroidism, and hypoplasia of the inner genitals. Based on the above findings, growth hormone, estrogen, and L-thyroxine substitution was introduced, which resulted in satisfactory longitudinal growth and onset of sexual maturation. We suggest genetic counselling and if needed, invasive investigations in female patients with short stature and absent/delayed puberty, with or without sex chromosomal anomalies, as the adequate therapy and even the quality of life of patient depends largely on the knowledge of their anatomical and endocrine status. PMID:22138217

  9. Effect of electroacupuncture on P2X3 receptor regulation in the peripheral and central nervous systems of rats with visceral pain caused by irritable bowel syndrome.

    Science.gov (United States)

    Weng, Z J; Wu, L Y; Zhou, C L; Dou, C Z; Shi, Y; Liu, H R; Wu, H G

    2015-09-01

    The aim of this study is to investigate the role of the purinergic receptor P2X3 in the peripheral and central nervous systems during acupuncture treatment for the visceral pain of irritable bowel syndrome (IBS). A total of 24 8-day-old Sprague-Dawley (SD) neonatal male rats (SPF grade) were stimulated using colorectal distention (CRD) when the rats were awake. The modeling lasted for 2 weeks with one stimulation per day. After 6 weeks, the rats were randomly divided into three groups of eight each: (1) the normal group (NG, n = 8); (2) the model group (MG, n = 8); and (3) the model + electroacupuncture group (EA, n = 8) that received electroacupuncture at a needling depth of 5 mm at the Shangjuxu (ST37, bilateral) and Tianshu (ST25, bilateral) acupoints. The parameters of the Han's acupoint nerve stimulator (HANS) were as follows: sparse-dense wave with a frequency of 2/100 Hz, current of 2 mA, 20 min/stimulation, and one stimulation per day; the treatment was provided for seven consecutive days. At the sixth week after the treatment, the abdominal withdrawal reflex (AWR) score was determined; immunofluorescence and immunohistochemistry were used to measure the expression of the P2X3 receptor in myenteric plexus neurons, prefrontal cortex, and anterior cingulate cortex; and, a real-time PCR assay was performed to measure the expression of P2X3 messenger RNA (mRNA) in the dorsal root ganglion (DRG) and spinal cord. After stimulation with CRD, the expression levels of the P2X3 receptor in the inter-colonic myenteric plexus, DRG, spinal cord, prefrontal cortex, and anterior cingulate cortex were upregulated, and the sensitivity of the rats to IBS visceral pain was increased. Electroacupuncture (EA) could downregulate the expression of the P2X3 receptor and ease the sensitivity to visceral pain. The P2X3 receptor plays an important role in IBS visceral pain. The different levels of P2X3 in the peripheral enteric nervous system and central nervous system mediate the

  10. Neurological involvement in primary Sjogren syndrome: a focus on central nervous system.

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    Manuela Morreale

    Full Text Available OBJECTIVES: Sjögren syndrome is an autoimmune disease involving mainly salivary and lacrimal glands. Beyond widely described PNS involvement, high variable prevalence of CNS manifestations ranging from 2.5 and 60% of all pSS patients has been reported, without specific syndrome definition. The aim of this cohort study was to evaluate the prevalence of CNS signs and symptoms in pSS patients and to identify possible biomarkers of CNS damage. METHODS: 120 patients with pSS diagnosis according to the 2002 American-European Consensus Group criteria were enrolled after exclusion of secondary causes. All patients underwent to a wide neurological, neuropsychological, psychiatric, neuroradiological and ultrasonographic evaluation. RESULTS: Central and peripheral nervous system involvement was observed in 81 patients with a prevalence of 67.5%. The prevalence of CNS involvement was significantly higher than PNS disease (p 0.001. 68 patients (84% shown non-focal CNS symptoms and 64 (79% focal CNS deficits with headache as the most common feature (46.9%, followed by cognitive (44.4% and mood disorders (38.3%. Particularly, we observed a high prevalence of migraine without aura, subcortical frontal executive functions and verbal memory impairment and apathy/alexythimia. MR spectroscopy revealed a reduction of NAA levels or NAA/Cr ratio decrease in subcortical frontal and basal ganglia white matter, while ultrasonography showed an impairment of microvasculature response. At multivariate analysis, headache, cognitive disorders and psychiatric symptoms was significantly associated to serological markers (anti-SSA, MRS and ultrasonographic features. CONCLUSIONS: The higher prevalence of MWO-mimic headache, cognitive dys-executive syndrome and mood disorders observed in this series confirmed previous evidences of a higher diffused CNS compromission rather than focal involvement such as SM-like clinical course or NMO-like syndrome. The association with

  11. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Injury Psychological Issues After Spinal Cord Injury Psychological Health After Spinal Cord Injury Psychological Health After Spinal Cord Injury The Psychologist's Role After ...

  12. Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

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    Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2007-12-15

    We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition.

  13. Evaluation of the Endothelial Cell Density and the Central Corneal Thickness in Pseudoexfoliation Syndrome and Pseudoexfoliation Glaucoma

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    Bożydar T. Tomaszewski

    2014-01-01

    Full Text Available Purpose. Evaluation of central corneal thickness (CCT and endothelial cell density (ECD in patients with senile cataract and coexisting pseudoexfoliation (PEX syndrome with glaucoma (PEXG and without glaucoma using specular microscopy. Participants and Methods. The study included 122 patients (217 eyes. In this group of patients we identified 133 eyes with PEX syndrome (65 with glaucoma, 68 without glaucoma and 84 eyes without PEX syndrome. ECD and CCT were measured in each eye by specular microscopy. Results. ECD in eyes with PEX syndrome without glaucoma (2297 ± 359 cell/mm2 and in eyes with PEXG (2241 ± 363 cell/mm2 was lower than in the control group (2503 ± 262 cell/mm2 (P<0.001. CCT in eyes with PEXG (508.2 ± 32.6 μm was thinner than in eyes with PEX syndrome without glaucoma (529.7 ± 30.3 μm and control group (527.7 ± 29.4 μm (P<0.001. Conclusions. This research shows that in eyes with PEX syndrome, both with and without glaucoma, ECD was statistically significantly lower than in the control group. In patients with PEXG, CCT was statistically significantly thinner than in the PEX syndrome and control group.

  14. The clinical analysis of central neurological dysfunctions in primary Sjogren's syndrome: 4 cases report%原发性干燥综合征中枢神经系统损害4例分析

    Institute of Scientific and Technical Information of China (English)

    姚晓萍; 李智文; 林仕芳

    2012-01-01

    目的 探讨原发性干燥综合征中枢神经系统损害的临床特点,以提高其早期诊断、治疗效果.方法 回顾性分析4例原发性干燥综合征中枢神经系统损害患者的临床特征、诊疗情况,并结合国内外相关文献进行分析.结果 4例患者均为女性,年龄30~52岁.3例患者中枢神经系统损害表现先于干燥综合征诊断,1例患者先诊断干燥综合征后出现中枢神经系统损害.1例患者出现脊髓损害表现,2例患者为脑实质损害表现,1例具有脊髓、脑、视神经损害表现.4例患者MR均表现为点状或小片状长T1长T2信号影.治疗上1例患者单用糖皮质激素,其余3例患者均合用免疫抑制剂,治疗后病情均好转,随访无再新发中枢神经系统损害表现.结论 对不能用其他原因解释的中枢神经系统损害,尤其是中老年女性,应警惕原发性干燥综合征的可能.重视干燥综合征症状的询问,加深对干燥综合征中枢神经系统损害临床表现的认识,进行相关检查,以提高早期诊断、治疗效果,减少患者的致残率.%Objective To explore the clinical features of central neurological dysfunctions in primary Sjogren' s syndrome , and to improve its early diagnosis and treatment. Methods Retrospective review the clinical features, diagnosis and treatment in four patients of central neurological dysfunctions in primary Sjogren s syndrome, and analyze the relative literatures. Results The four patients were female, aged from 30 years old to 52 years old. Three patients of them presented the manifestation of central nervous system damage ahead of the diagnosis of Sjogren' s syndrome. One patient with spinal cord injury , two patients with brain parenchymal damage, and one patient with spinal cord injury, brain parenchyma and optic nerve damage. All of the patients showed punctate or small pieces of long Tl, T2 signal intensity in the MR examination. In terms of treatment, one patient was treated

  15. Spinal cord involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and MRI study.

    Science.gov (United States)

    Ioannidis, Panagiotis; Parissis, Dimitris; Karapanayiotides, Theodoros; Maiovis, Pantelis; Karacostas, Dimitris; Grigoriadis, Nikolaos

    2015-06-01

    Concomitant central nervous system (CNS) involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare. Although the spinal nerve roots may present MRI abnormalities in CIDP, hitherto, the spinal cord has been investigated in a single study. We retrospectively investigated clinically and with MRI a cohort of patients with definite CIDP diagnosis (EFNS/PNS criteria) for evidence of brain and spinal cord involvement, who were initially admitted in our department during the last 4 years. Among 12 patients with CIDP (men: 8, mean age: 59.3 years, mean disease duration: 3.8 years), nine patients had their MRI scan during a clinical relapse and three during remission. Brain MRI did not document typical multiple sclerosis lesions in any patient. We did not identify any MRI abnormalities in ten patients without clinical evidence of spinal cord involvement. Conversely, MRI disclosed extensive lesions of the thoracic cord in two patients with an overt spinal cord syndrome, whom we describe. This represents the biggest MRI study of CIDP patients who have been investigated for spinal cord involvement. Our data support earlier observations that a minority of CIDP patients may additionally develop CNS involvement of variable degree.

  16. Bilateral Central Retinal Vein Occlusions Combined with Artery Occlusions in A Patient with Acquired Immune Deficiency Syndrome

    Institute of Scientific and Technical Information of China (English)

    Feng Wen; Xuemei Chen; Haitai Li; Ruiduan Liao; Dezheng Wu

    2002-01-01

    Purpose: This is the first report of a bilateral nonischemic central retinal vein occlusionscombined with artery occlusions in a patient with acquired immune deficiency syndrome(AIDS). Methods: Case report. Results: A 22-year-old Chinese(male) with a positive human immunodeficiency virus(HIV) infection developed bilateral nonischemic central retinal vein occlusions combinedwith artery occlusions and severe vision loss. The manifestations of the fundus andfluorescein angiography were similar in both eyes.Conclusion: This case report provides the evidences that central retinal vein and arteryocclusions are probably part of the spectrum of AIDS vascular diseases.

  17. Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: an overview of Down syndrome, autism, Fragile X and Rett syndrome.

    Science.gov (United States)

    Valenti, Daniela; de Bari, Lidia; De Filippis, Bianca; Henrion-Caude, Alexandra; Vacca, Rosa Anna

    2014-10-01

    Clinical manifestations typical of mitochondrial diseases are often present in various genetic syndromes associated with intellectual disability, a condition leading to deficit in cognitive functions and adaptive behaviors. Until now, the causative mechanism leading to intellectual disability is unknown and the progression of the condition is poorly understood. We first report latest advances on genetic and environmental regulation of mitochondrial function and its role in brain development. Starting from the structure, function and regulation of the oxidative phosphorylation apparatus, we review how mitochondrial biogenesis and dynamics play a central role in neurogenesis and neuroplasticity. We then discuss how dysfunctional mitochondria and alterations in reactive oxygen species homeostasis are potentially involved in the pathogenesis of various neurodevelopmental syndromes with a special focus on Down, Rett, Fragile X syndromes and autism spectrum disorders. Finally, we review and suggest novel therapeutic approaches aimed at improving intellectual disability by activating mitochondrial function and reducing oxidative stress to amiliorate the quality of life in the subjects affected.

  18. Systemic 5-fluorouracil treatment causes a syndrome of delayed myelin destruction in the central nervous system

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    Han Ruolan

    2008-04-01

    Full Text Available Abstract Background Cancer treatment with a variety of chemotherapeutic agents often is associated with delayed adverse neurological consequences. Despite their clinical importance, almost nothing is known about the basis for such effects. It is not even known whether the occurrence of delayed adverse effects requires exposure to multiple chemotherapeutic agents, the presence of both chemotherapeutic agents and the body's own response to cancer, prolonged damage to the blood-brain barrier, inflammation or other such changes. Nor are there any animal models that could enable the study of this important problem. Results We found that clinically relevant concentrations of 5-fluorouracil (5-FU; a widely used chemotherapeutic agent were toxic for both central nervous system (CNS progenitor cells and non-dividing oligodendrocytes in vitro and in vivo. Short-term systemic administration of 5-FU caused both acute CNS damage and a syndrome of progressively worsening delayed damage to myelinated tracts of the CNS associated with altered transcriptional regulation in oligodendrocytes and extensive myelin pathology. Functional analysis also provided the first demonstration of delayed effects of chemotherapy on the latency of impulse conduction in the auditory system, offering the possibility of non-invasive analysis of myelin damage associated with cancer treatment. Conclusions Our studies demonstrate that systemic treatment with a single chemotherapeutic agent, 5-FU, is sufficient to cause a syndrome of delayed CNS damage and provide the first animal model of delayed damage to white-matter tracts of individuals treated with systemic chemotherapy. Unlike that caused by local irradiation, the degeneration caused by 5-FU treatment did not correlate with either chronic inflammation or extensive vascular damage and appears to represent a new class of delayed degenerative damage in the CNS.

  19. Impaired Neural Structure and Function Contributing to Autonomic Symptoms in Congenital Central Hypoventilation Syndrome

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    Ronald M Harper

    2015-10-01

    Full Text Available Congenital central hypoventilation syndrome (CCHS patients show major autonomic alterations in addition to their better-known breathing deficiencies. The processes underlying CCHS, mutations in the PHOX2B gene, target autonomic neuronal development, with frame shift extent contributing to symptom severity. Many autonomic characteristics, such as impaired pupillary constriction and poor temperature regulation, reflect parasympathetic alterations, and can include disturbed alimentary processes, with malabsorption and intestinal motility dyscontrol. The sympathetic nervous system changes can exert life-threatening outcomes, with dysregulation of sympathetic outflow leading to high blood pressure, time-altered and dampened heart rate and breathing responses to challenges, cardiac arrhythmia, profuse sweating, and poor fluid regulation. The central mechanisms contributing to failed autonomic processes are readily apparent from structural and functional magnetic resonance imaging studies, which reveal substantial cortical thinning, tissue injury, and disrupted functional responses in hypothalamic, hippocampal, posterior thalamic, and basal ganglia sites and their descending projections, as well as insular, cingulate, and medial frontal cortices, which influence subcortical autonomic structures. Midbrain structures are also compromised, including the raphe system and its projections to cerebellar and medullary sites, the locus coeruleus, and medullary reflex integrating sites, including the dorsal and ventrolateral medullary nuclei. The damage to rostral autonomic sites overlaps metabolic, affective and cognitive regulatory regions, leading to hormonal disruption, anxiety, depression, behavioral control, and sudden death concerns. The injuries suggest that interventions for mitigating hypoxic exposure and nutrient loss may provide cellular protection, in the same fashion as interventions in other conditions with similar malabsorption, fluid turnover

  20. Similar deficits of central histaminergic system in patients with Down syndrome and Alzheimer disease.

    Science.gov (United States)

    Schneider, C; Risser, D; Kirchner, L; Kitzmüller, E; Cairns, N; Prast, H; Singewald, N; Lubec, G

    1997-02-01

    In order to study whether Alzheimer-like neuropathological changes involve the central histaminergic system we measured the concentration of histamine, its precursor histidine as well as the activity of histidine decarboxylase (HDC) and histamine-N-methyl-transferase (HMT) in frontal cortex of aging Down syndrome (DS) patients, Alzheimer patients and control individuals. The study populations were also investigated for choline acetyltransferase (ChAT) activity, since reduced ChAT activity is an established biochemical hallmark in DS and Alzheimer disease (AD). HDC and ChAT activity were reduced in brains of both DS and Alzheimer patients versus control patients. Additionally, we observed a significant decrease of histamine levels in the DS group. Histamine levels in AD brains tended to be decreased. Histidine concentrations and HMT activities were comparable between the three groups. Thus, our results for the first time show histaminergic deficits in brains of patients with DS resembling the neurochemical pattern in AD. Neuropathological changes may be responsible for similar neurochemical alterations of the histaminergic system in both dementing disorders.

  1. An assistive device for congenital central hypoventilation syndrome outpatients during sleep.

    Science.gov (United States)

    Biffi, Emilia; Piazza, Caterina; Cavalleri, Matteo; Taddeo, Peter; Carcano, Alessandro; Morandi, Francesco; Reni, Gianluigi

    2014-10-01

    Congenital Central Hypoventilation Syndrome is a genetic disease characterized by alveolar hypoventilation and autonomic dysregulation. Patients have hypoventilations, especially during sleep, conditioning hypercapnia which can lead to neurological damage and death. They therefore need mechanical ventilators, that provide sufficient gas exchange, and pulse-oximeters that monitor oxy-hemoglobin blood concentration. Due to the restrictions regarding domiciliary assistive devices, the presence of a caregiver is required all night long. Currently, the only alarm systems available are the ones integrated in the ventilators and monitoring systems. During the night, multiple false alarms may occur, interrupting the sleep and causing anxiety. In this work we describe an assistive device that acquires real-time data from a pulse-oximeter, provides a multisensory stimulation if oxygen saturation falls under a certain threshold, and wakes up the patient if the hypoxia is severe. Tests on healthy subjects have shown that the device guarantees rapid awakenings, with a stimulator-dependent efficacy, and that it does not affect sleep efficiency. The purpose of the device is to determine a gentle awakening if mild hypoxia conditions persist, and to assure rapid awakening when a severe hypoxia occurs, reducing false alarms, improving the quality of sleep and increasing the self-sufficiency of the patients. PMID:25069973

  2. Genotype-phenotype relationship in Japanese patients with congenital central hypoventilation syndrome.

    Science.gov (United States)

    Shimokaze, Tomoyuki; Sasaki, Ayako; Meguro, Toru; Hasegawa, Hisaya; Hiraku, Yuka; Yoshikawa, Tetsushi; Kishikawa, Yumiko; Hayasaka, Kiyoshi

    2015-09-01

    Examine the genotype-phenotype relationship in Japanese congenital central hypoventilation syndrome (CCHS) patients and estimate the incidence of CCHS in Japan. Subjects were 92 Japanese patients with PHOX2B mutations; 19 cases carried 25 polyalanine repeat expansion mutations (PARMs); 67 cases carried 26 or more PARMs; and 6 had non-PARMs (NPARMs). We collected clinical data in all patients and estimated the development or intelligent quotients only in the patients carrying 25 PARM. The estimated incidence of CCHS was greater than one case per 148 000 births. Polyhydramnios was observed in three cases. Twelve infants exhibited depressed respiration at birth. In 19 cases carrying 25 PARM, the male-to-female ratio was ~3, no cases had Hirschsprung disease; 7 cases (37%) developed hypoventilation after the neonatal period, and 8 cases (42%) had mental retardation. In other 73 cases carrying 26 or more PARMs or NPARMs, male-to-female ratio was equal; patients frequently complicated with Hirschsprung disease and constipation, and all patients presented with hypoventilation in the neonatal period. Clinical symptoms were severe in most patients carrying long PARMs and NPARMs. In 25 PARM, additional genetic and/or epigenetic factors were required for CCHS development and male sex is likely a predisposing factor. The patients carrying 25 PARM frequently had mental retardation likely because they were not able to receive appropriate ventilation support following a definitive diagnosis owing to subtle and or irregular hypoventilation. Molecular diagnosis provides a definitive diagnosis and enables to receive appropriate ventilator support. PMID:26063465

  3. Subarachnoid disseminative hemangiopericytoma of the spinal cord

    Institute of Scientific and Technical Information of China (English)

    LIN Guo-zhong; WANG Zhen-yu; LI Zhen-dong; ZHONG Yan-feng; WANG Lei-ming

    2010-01-01

    @@ Hemangiopericytomas (HPCs) originating from central nervous system were increasingly reported recently.1 Intravertebral HPCs are predominantly epidural. Primary intradural HPCs of spinal cord are rare.2-5 Little subarachnoid dissemination has been reported. We reported a HPC of the cervical spinal cord with subarachnoid dissemination.

  4. Treatment of neuroblastoma in congenital central hypoventilation syndrome with a PHOX2B polyalanine repeat expansion mutation: New twist on a neurocristopathy syndrome.

    Science.gov (United States)

    Armstrong, Amy E; Weese-Mayer, Debra E; Mian, Amir; Maris, John M; Batra, Vandana; Gosiengfiao, Yasmin; Reichek, Jennifer; Madonna, Mary Beth; Bush, Jonathan W; Shore, Richard M; Walterhouse, David O

    2015-11-01

    Neuroblastoma in patients with congenital central hypoventilation syndrome (CCHS) as part of a neurocristopathy syndrome is a rare finding and has only been associated with paired-like homeobox 2b (PHOX2B) non-polyalanine-repeat-expansion mutations. To the best of our knowledge, we report the first case of a child with CCHS and Hirschsprung disease who had a PHOX2B polyalanine-repeat-expansion mutation (PARM) (genotype 20/33) and developed high-risk neuroblastoma. We further describe his treatment including chemotherapy and therapeutic I(131) -metaiodobenzylguanidine. This case highlights the need to consider neuroblastoma in patients with CCHS and the longest PHOX2B PARMs and to individualize treatment based on co-morbidities.

  5. Multifocal spinal hemangioblastoma in von Hippel-Lindau syndrome: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa [Dept. of Radiology, Inje University College of Medicine, Haeundae Paik Hospital, Busan (Korea, Republic of)

    2015-03-15

    Hemangioblastoma is a benign vascular neoplasm of the central nervous system that occurs frequently in the cerebellum and other areas of the central nervous system including spinal cord and brainstem. Spinal hemangioblastoma can present as a sporadic isolated lesion or as a component of von Hippel-Lindau syndrome. The author presents a case of 32-year-old man with von Hippel-Lindau syndrome and spinal hemangioblastomas represented by multiple small spinal lesions, with an emphasis on the magnetic resonance imaging findings and clinical characteristics of von Hippel-Lindau syndrome-associated spinal hemangioblastomas.

  6. 儿童脊髓栓系综合征的手术治疗%Surgical treatment of tethered cord syndrome in children

    Institute of Scientific and Technical Information of China (English)

    张华楸; 雷霆; 孙炜; 淦超; 李朝曦; 舒凯; 郭东生; 陈劲草; 陈坚; 万锋

    2014-01-01

    Objective To explore the clinical features, treatment method, surgical outcome and prognosis of pediatric tethered cord syndrome (TCS). Methods Clinical data of 30 children with TCS undergoing microsurgery was analyzed retrospectively. Results The clinical pathological diagnosis showed lipoma type in 10 patients and non-lipoma type in 20. During the follow-up period of 0.5 to 4 years, the local phyma and malformation of the sinus tract were cured in all the patients, the motor function was improved in 9, sensory function improved in 5 and fecal and urinary dysfunction improved in 5. The postoperative compilations included wound healing difficulty in 4 patients, dysuria in 1 and numbness of lower limb in 2, and all the patients recovered well 1 to 3 months after operation. No long-term complications occurred. Conclusions Pediatric TCS is frequently associated with congenital malformation. Most of them could get good prognosis from early operation. The recovery of fecal and urinary function is more difficult than improvement of motor and sensory function.%目的:探讨儿童脊髓栓系的临床特点、治疗方法、效果和预后。方法回顾性分析30例显微手术治疗的脊髓栓系病儿的临床资料。结果临床病理诊断院脂肪瘤型10例,非脂肪瘤型20例。术后随访0.5~4年,所有病儿局部皮肤包块和窦道畸形治愈,运动功能改善9例,感觉功能改善4例,二便功能改善5例。术后并发症院伤口愈合困难4例,新发小便困难1例,下肢麻木2例;1~3个月均恢复。所有病例无远期并发症。结论儿童脊髓栓系常合并先天畸形,多数病儿早期手术治疗能取得较好的预后。二便功能恢复较下肢感觉运动功能改善更为困难。

  7. The cerebral cost of breathing: an FMRI case-study in congenital central hypoventilation syndrome.

    Science.gov (United States)

    Sharman, Mike; Gallea, Cécile; Lehongre, Katia; Galanaud, Damien; Nicolas, Nathalie; Similowski, Thomas; Cohen, Laurent; Straus, Christian; Naccache, Lionel

    2014-01-01

    Certain motor activities--like walking or breathing--present the interesting property of proceeding either automatically or under voluntary control. In the case of breathing, brainstem structures located in the medulla are in charge of the automatic mode, whereas cortico-subcortical brain networks--including various frontal lobe areas--subtend the voluntary mode. We speculated that the involvement of cortical activity during voluntary breathing could impact both on the "resting state" pattern of cortical-subcortical connectivity, and on the recruitment of executive functions mediated by the frontal lobe. In order to test this prediction we explored a patient suffering from central congenital hypoventilation syndrome (CCHS), a very rare developmental condition secondary to brainstem dysfunction. Typically, CCHS patients demonstrate efficient cortically-controlled breathing while awake, but require mechanically-assisted ventilation during sleep to overcome the inability of brainstem structures to mediate automatic breathing. We used simultaneous EEG-fMRI recordings to compare patterns of brain activity between these two types of ventilation during wakefulness. As compared with spontaneous breathing (SB), mechanical ventilation (MV) restored the default mode network (DMN) associated with self-consciousness, mind-wandering, creativity and introspection in healthy subjects. SB on the other hand resulted in a specific increase of functional connectivity between brainstem and frontal lobe. Behaviorally, the patient was more efficient in cognitive tasks requiring executive control during MV than during SB, in agreement with her subjective reports in everyday life. Taken together our results provide insight into the cognitive and neural costs of spontaneous breathing in one CCHS patient, and suggest that MV during waking periods may free up frontal lobe resources, and make them available for cognitive recruitment. More generally, this study reveals how the active

  8. Donor Umbilical Cord Blood Transplant With or Without Ex-vivo Expanded Cord Blood Progenitor Cells in Treating Patients With Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, Chronic Myelogenous Leukemia, or Myelodysplastic Syndromes

    Science.gov (United States)

    2016-09-09

    Acute Biphenotypic Leukemia; Acute Lymphoblastic Leukemia in Remission; Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Acute Myeloid Leukemia in Remission; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Mixed Phenotype Acute Leukemia; Myelodysplastic Syndrome; Pancytopenia; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Secondary Acute Myeloid Leukemia

  9. Cord blood transplant: current and future issues.

    Science.gov (United States)

    Inoue, S

    1998-01-01

    Cord blood as the source of hematopoietic stem cells has several advantages over bone marrow cells for transplant purpose. It is readily available, and causes no physical harm or inconveniences to the donor in the processing of harvesting cells. Waiting time between initiating the search and the time to transplant from an unrelated donor is much shorter with cord blood than with unrelated donor bone marrow. The incidence of graft-versus-host diseases is much less. Because of these advantages, cord blood has been increasingly used as the source of stem cells. As of this writing, more than 200 cord blood transplants have been done in patients with hematological malignancies, solid tumors, hematological diseases, immunodeficiency syndromes, and metabolic diseases. One of the limitations inherent in the cord blood is its limited number of hematopoietic stem cells. Thus it has been primarily used for pediatric patients, though more recently, adult patients also have been transplanted with cord blood as people have become more experienced in harvesting cord blood thus yielding a larger number of stem cells in a given specimen. Efforts have been made to amplify stem cells in vitro following harvesting cord blood stem cells, so that adult recipients also would routinely benefit from this resource. Cord blood lymphocytes are functionally "naive", do not generate vigorous mixed lymphocyte culture reactivities. The low incidence of graft-versus-host disease in the recipients of cord blood is due to this particular property. It is highly desirable that the world wide cord blood registry, similar to the international bone marrow registry would be instituted, but there are logistic, ethical and financial problems that need to be resolved. Cord blood is one of the best stem cell sources, and its application is quite wide. PMID:10771961

  10. The central role of cognitive processes in the perpetuation of chronic fatigue syndrome.

    NARCIS (Netherlands)

    Knoop, H.; Prins, J.B.; Moss-Morris, R.; Bleijenberg, G.

    2010-01-01

    OBJECTIVE: Chronic fatigue syndrome (CFS) is considered to be one of the functional somatic syndromes (FSS). Cognitions and behavior are thought to perpetuate the symptoms of CFS. Behavioral interventions based on the existing models of perpetuating factors are quite successful in reducing fatigue a

  11. Spinal Cord Injury 101

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    Full Text Available Experts \\ Spinal Cord Injury 101 Topics Adult Injuries Spinal Cord Injury 101 Spinal Cord Injury 101 The Basics of Spinal Cord Injury Rehabilitation ... in countries outside the US ? A spinal cord injury affects the entire family FacingDisability is designed to ...

  12. Anterior spinal artery syndrome of the cervical hemicord.

    Science.gov (United States)

    Baumgartner, R W; Waespe, W

    1992-01-01

    Three patients developed signs of a unilateral cervical cord lesion 6 to 36 h after the acute onset of severe cervico-brachial pain. The neurological deficit progressed over 6 to 18 h. On the painful side a central Horner's syndrome, a hemiparesis with plegia of the hand, and a slight pallhypaesthesia were found. On the opposite side thermhypaesthesia and hypalgesia were noted with a level at the dermatome C5 or C6. T2-weighted MR images revealed in one patient a small area of increased signal intensity restricted to one half of the cervical cord, and electromyography in another patient showed after 6 months evidence of segmental chronic denervation. Both abnormalities were found at the clinically expected level. The findings are consistent with a small infarction of the cervical cord in the perfusion territory of a central (sulco-commissural) artery, a duplicated anterior spinal artery or an anterior spinal branch of the vertebral artery. PMID:1315578

  13. Clinical observation of umbilical cord mesenchymal stem cell transplantation in treatment for sequelae of thoracolumbar spinal cord injury

    OpenAIRE

    Cheng, Hongbin; Liu, Xuebin; Hua, Rongrong; Dai, Guanghui; Wang, Xiaodong; Gao, Jianhua; An, Yihua

    2014-01-01

    Background Umbilical cord mesenchymal stem cells (UCMSCs) have a considerable advantage and potential in treating for central nervous system diseases and have become a novel alternative treatment for spinal cord injury. This study aims to compare the neurological function outcome of stem cell transplantation, rehabilitation therapy, and self-healing for sequelae of spinal cord injury. Methods Thirty-four cases of thoracolumbar spinal cord injury were randomly divided into three groups: the st...

  14. Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report

    Directory of Open Access Journals (Sweden)

    Samira Luiza dos Apóstolos-Pereira

    2013-06-01

    Full Text Available We report the case of a 24-year-old man presenting with sudden visual loss in the left eye from a central retinal artery occlusion. An extensive clinical investigation revealed no etiology. Three weeks later, however, the patient developed hearing loss followed by encephalopathy and multiple branch retinal artery occlusions in the right eye. Fluorescein angiography confirmed retinal vascular occlusions with no sign of vasculitis. The neurological examination revealed a diffuse encephalopathy while the MRI scan disclosed several small areas of infarcts in the brain. Bilateral sensorineural hearing loss was confirmed on audiometry. The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. This case shows that Susac syndrome may be diagnosed late due to the absence at onset of one or more of the symptoms of the classic triad (encephalopathy, multiple branch retinal artery occlusions and hearing loss. This case also serves to emphasize that Susac syndrome should be considered in the differential diagnosis of central retinal artery occlusion, even in apparently healthy young men.

  15. Dor neuropática central após lesão medular traumática: capacidade funcional e aspectos sociais Dolor neuropático central después de lesión medular traumática: capacidad funcional y aspectos sociales Central neuropathic pain after traumatic spinal cord injury: functional capacity and social aspects

    Directory of Open Access Journals (Sweden)

    Janaina Vall

    2005-12-01

    Full Text Available Estudo de caso comparativo com o objetivo de avaliar a capacidade funcional e os aspectos sociais de dois pacientes, ambos com lesão medular traumática, sem e com dor neuropática central associada, respectivamente. Para avaliar a capacidade funcional, foi utilizado como instrumento o Functional Independence Measure ou Escala de Independência Funcional. E para avaliar os aspectos sociais foi construído o ecomapa de cada paciente, preconizado pelo modelo Calgary de avaliação de famílias. Ambos foram aplicados no domicílio do paciente. Os resultados mostraram que o paciente com dor neuropática central secundária à lesão medular possui baixa capacidade funcional e precária rede social de apoio, quando comparado com o paciente com as mesmas condições, porém sem dor associada.Estudio de caso comparativo con el objetivo de evaluar la capacidad funcional y los aspectos sociales de dos paciente, ambos con lesión medular traumática, sin y con el dolor neuropático central, respectivamente. Para evaluar la capacidad funcional, se usó como instrumento la Escala de Independencia Funcional. Y para evaluar los aspectos sociales, el ecomapa de cada paciente fue construido, preconizado por el modelo Calgary de evaluación de familias. Los dos furon aplicados en la casa del paciente. Los resultados mostraron que el paciente con dolor neuropatico central secundario a la lesión medular posee capacidad funcional baja y precaria red social de apoyo, cuando comparado con el paciente con las mismas condiciones, pero sin el dolor asociado.Comparative study of case with the aim of evaluating the functional capacity and social aspects of two patients, both with traumatic spinal cord injury, without and with central neuropathic pain associated, respectively. To evaluate the functional capacity it was used as instrument Functional Independence Measure. And to evaluate the social aspects the ecomap of each patient one it was built, extolled by the model

  16. Spinal Cord Injury 101

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    Full Text Available ... Substance Abuse and Spinal Cord Injury How Family Life Changes After Spinal Cord Injury How Family Life Changes After Spinal Cord Injury Empowering the Patient After Spinal ...

  17. Dispersal syndromes related to edge distance in cerrado sensu stricto fragments of central-western Brazil

    Directory of Open Access Journals (Sweden)

    André Vitor Fleuri Jardim

    2009-10-01

    Full Text Available The main selective forces affecting the fruiting strategies are related to the environment in which plants occur. As a savanna, microclimatic conditions should not vary in relation to distance from edge in cerrado sensu stricto fragments. Thus, we postulated that the importance of different dispersal syndromes would not vary towards the fragment core. Our aim was to test in four cerrado sensu stricto fragments in central Brazil whether the absolute density of anemo-, auto-, and zoochorous individuals varied in relation to edge distance. According to results, the absolute density of anemo-and autochorous individuals did not vary, whereas those of zoochorous individuals increased with edge distance, pointing out that there were other factors rather than abiotic conditions shifting zoochorous species to the interior of cerrado sensu stricto fragments.As principais forças seletivas que afetam as estratégias de frutificação estão relacionadas ao ambiente em que as plantas ocorrem. Como fragmentos de cerrado sensu stricto são savânicos, as condições microclimáticas não devem variar em relação à distância da borda. Assim, postulamos que a importância das diferentes síndromes de dispersão não varia da borda ao interior de um fragmento de vegetação. O objetivo deste trabalho foi testar, em quatro fragmentos de cerrado sensu stricto (centro-oeste do Brasil, se a densidade absoluta de indivíduos anemo, auto e zoocóricos variava em função da distância da borda. Segundo nossos resultados, a densidade absoluta de indivíduos anemo e autocóricos não variou significativamente borda em direção ao interior dos fragmentos, enquanto que a dos indivíduos zoocóricos aumentou, indicando que existem outros fatores, que não as condições abióticas, deslocando as espécies zoocóricas para o interior dos fragmentos de cerrado sensu stricto.

  18. Central nervous system PET-CT imaging reveals regional impairments in pediatric patients with Wolfram syndrome.

    Directory of Open Access Journals (Sweden)

    Agnieszka Zmyslowska

    Full Text Available Wolfram syndrome (WFS is inherited as an autosomal recessive disease with main clinical features of diabetes mellitus, optic atrophy, diabetes insipidus and deafness. However, various neurological defects may also be detected. The aim of this study was to evaluate aspects of brain structure and function using PET-CT (positron emission tomography and computed tomography and MRI (magnetic resonance imaging in pediatric patients with WFS. Regional changes in brain glucose metabolism were measured using standardized uptake values (SUVs based on images of (18F fluorodeoxyglucose (FDG uptake in 7 WFS patients aged 10.1-16.0 years (mean 12.9±2.4 and in 20 healthy children aged 3-17.9 years (mean 12.8±4.1. In all patients the diagnosis of WFS was confirmed by DNA sequencing of the WFS1 gene. Hierarchical clustering showed remarkable similarities of glucose uptake patterns among WFS patients and their differences from the control group. SUV data were subsequently standardized for age groups 13 years old to account for developmental differences. Reduced SUVs in WFS patients as compared to the control group for the bilateral brain regions such as occipital lobe (-1.24±1.20 vs. -0.13±1.05; p = 0.028 and cerebellum (-1.11±0.69 vs. -0.204±1.00; p = 0.036 were observed and the same tendency for cingulate (-1.13±1.05 vs. -0.15±1.12; p = 0.056, temporal lobe (-1.10±0.98 vs. -0.15±1.10; p = 0.057, parietal lobe (-1.06±1.20 vs. -0.08±1.08; p = 0.058, central region (-1.01±1.04 vs. -0.09±1.06; p = 0.060, basal ganglia (-1.05±0.74 vs. -0.20±1.07; p = 0.066 and mesial temporal lobe (-1.06±0.82 vs. -0.26±1.08; p = 0.087 was also noticed. After adjusting for multiple hypothesis testing, the differences in glucose uptake were non-significant. For the first time, regional differences in brain glucose metabolism among patients with WFS were shown using PET-CT imaging.

  19. Spinal Cord Contusion

    Institute of Scientific and Technical Information of China (English)

    Gong Ju; Jian Wang; Yazhou Wang; Xianghui Zhao

    2014-01-01

    Spinal cord injury is a major cause of disability with devastating neurological outcomes and lim-ited therapeutic opportunities, even though there are thousands of publications on spinal cord injury annually. There are two major types of spinal cord injury, transaction of the spinal cord and spinal cord contusion. Both can theoretically be treated, but there is no well documented treatment in human being. As for spinal cord contusion, we have developed an operation with fabulous result.

  20. Spinal Cord Injury 101

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    Full Text Available ... Injury 101 The Basics of Spinal Cord Injury Rehabilitation The Basics of Spinal Cord Injury Rehabilitation Preventing Pressure Sores Preventing Pressure Sores Transition from ...

  1. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Workers Help Transitions How Social Workers Help Transitions Occupational Therapy After Spinal Cord Injury Occupational Therapy After Spinal Cord Injury How Occupational Therapists Work ...

  2. Animal models of Central Diabetes Insipidus: Human relevance of acquired beyond hereditary syndromes and the role of oxytocin.

    Science.gov (United States)

    Bernal, Antonio; Mahía, Javier; Puerto, Amadeo

    2016-07-01

    The aim of this study was to review different animal models of Central Diabetes Insipidus, a neurobiological syndrome characterized by the excretion of copious amounts of diluted urine (polyuria), a consequent water intake (polydipsia), and a rise in the serum sodium concentration (hypernatremia). In rodents, Central Diabetes Insipidus can be caused by genetic disorders (Brattleboro rats) but also by various traumatic/surgical interventions, including neurohypophysectomy, pituitary stalk compression, hypophysectomy, and median eminence lesions. Regardless of its etiology, Central Diabetes Insipidus affects the neuroendocrine system that secretes arginine vasopressin, a neurohormone responsible for antidiuretic functions that acts trough the renal system. However, most Central Diabetes Insipidus models also show disorders in other neurobiological systems, specifically in the secretion of oxytocin, a neurohormone involved in body sodium excretion. Although the hydromineral behaviors shown by the different Central Diabetes Insipidus models have usually been considered as very similar, the present review highlights relevant differences with respect to these behaviors as a function of the individual neurobiological systems affected. Increased understanding of the relationship between the neuroendocrine systems involved and the associated hydromineral behaviors may allow appropriate action to be taken to correct these behavioral neuroendocrine deficits.

  3. Animal models of Central Diabetes Insipidus: Human relevance of acquired beyond hereditary syndromes and the role of oxytocin.

    Science.gov (United States)

    Bernal, Antonio; Mahía, Javier; Puerto, Amadeo

    2016-07-01

    The aim of this study was to review different animal models of Central Diabetes Insipidus, a neurobiological syndrome characterized by the excretion of copious amounts of diluted urine (polyuria), a consequent water intake (polydipsia), and a rise in the serum sodium concentration (hypernatremia). In rodents, Central Diabetes Insipidus can be caused by genetic disorders (Brattleboro rats) but also by various traumatic/surgical interventions, including neurohypophysectomy, pituitary stalk compression, hypophysectomy, and median eminence lesions. Regardless of its etiology, Central Diabetes Insipidus affects the neuroendocrine system that secretes arginine vasopressin, a neurohormone responsible for antidiuretic functions that acts trough the renal system. However, most Central Diabetes Insipidus models also show disorders in other neurobiological systems, specifically in the secretion of oxytocin, a neurohormone involved in body sodium excretion. Although the hydromineral behaviors shown by the different Central Diabetes Insipidus models have usually been considered as very similar, the present review highlights relevant differences with respect to these behaviors as a function of the individual neurobiological systems affected. Increased understanding of the relationship between the neuroendocrine systems involved and the associated hydromineral behaviors may allow appropriate action to be taken to correct these behavioral neuroendocrine deficits. PMID:27118135

  4. Chemo-port insertion: A cause of vocal cord palsy.

    Science.gov (United States)

    Alazzawi, Sarmad; Hindi, Khalid; Malik, Ausama; Wee, Chong Aun; Prepageran, Narayanan

    2015-11-01

    We describe extremely rare cases of vocal cord palsy following surgical insertion of a chemo port. Our cohort consisted of patients with cancer who developed hoarseness immediately after central venous line placement for the administration of chemotherapy, with vocal cord palsy confirmed with flexible laryngoscopy. Given the timing, central venous line placement appears to be the most likely cause. PMID:26108861

  5. 无骨折脱位型急性中央型颈脊髓损伤治疗心得%Type type of acute central cervical spinal cord injury without fracture dislocation treatment result

    Institute of Scientific and Technical Information of China (English)

    谢海明; 陈胜; 李勇; 金正帅

    2014-01-01

    Objective Explore type of acute central type spinal cord injury without fracture dislocation treatment. Methods Type of 32 cases of acute central cervical spinal cord injury patients, upper limbs of 17 cases, limbs 15 cases. Admitted to hospital after skul traction, braking, dehydration. 20 underwent surgical treatment, 12 routine non-surgical treatment. Results 32 patients had no serious complications occurred, 29 cases of patients with injury after 3 months to 18 months folow-up, no complications occurred, neural function classification method (ASIA) are 1 ~ 2 levels of recovery. 23 patients work, the life can provide for oneself, cervical spine MRI review, 14 patients had different degrees of syringomyelia imaging findings. Conclusions For cervical spondylosis, cervical intervertebral disc herniation, cervical stenosis and spinal canal without obstruction of acute central spinal cord injury (edema) appropriate USES non-surgical treatment. Continued to pressure on the spinal cord or type cervical instability in the middle of the spinal cord injury patients should be timely surgical decompression, reconstruction of cervical stability.%目的:探讨无骨折脱位型急性中央型脊髓损伤治疗方法。方法32例急性中央型颈脊髓损伤患者,上肢型17例,四肢型15例。入院后行颅骨牵引、制动、脱水。20例行手术治疗,12例行非手术治疗。结果32例患者均无严重手术并发症发生,29例患者伤后3月-18月获随访,未见手术并发症发生,神经功能(ASIA法分类)均获1~2级的恢复。23例患者工作,生活可自理,颈椎MRI复查,14例患者有不同程度的脊髓空洞影像学表现。结论对无颈椎病、颈椎间盘突出、颈椎管狭窄、椎管内无梗阻的急性中央型脊髓损伤(水肿型)宜采用非手术治疗。对脊髓的持续存在致压物或颈椎失稳的中央型脊髓损伤患者应及时行手术减压,重建颈椎稳定性。对脊髓前方

  6. Assessment of central chemosensitivity and cardiac sympathetic nerve activity using I-123 MIBG imaging in central sleep apnea syndrome in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Iodine-123 m-iodobenzylguanidine (MIBG) imaging has been used to study cardiac sympathetic function in various cardiac diseases. Central sleep apnea syndrome (CSAS) occurs frequently in patients with chronic heart failure (CHF) and is reported to be associated with a poor prognosis. One of the mechanisms of its poor prognosis may be related to impaired cardiac sympathetic activity. However, the relationship between chemosensitivity to carbon dioxide, which is reported to correlate with the severity of CSAS, and cardiac sympathetic activity has not been investigated. Therefore, this study was undertaken to assess cardiac sympathetic function and chemosensitivity to carbon dioxide in CHF patients. The oxygen desaturation index (ODI) was evaluated in 21 patients with dilated cardiomyopathy (male/female: 19/2, left ventricular ejection fraction (LVEF)5 times/h underwent polysomnography. Patients with an apnea hypopnea index >15/h but without evidence of obstructive apnea were defined as having CSAS. Early (15 min) and delayed (4 hr) planar MIBG images were obtained from these patients. The mean counts in the whole heart and the mediastinum were obtained. The heart-to-mediastinum count ratio of the delayed image (H/M) and the corrected myocardial washout rate (WR) were also calculated. The central chemoreflex was assessed with the rebreathing method using a hypercapnic gas mixture (7% CO2 and 93% O2). Ten of the 21 patients had CSAS. The H/M ratio was similar in patients both with and without CSAS (1.57±0.18 vs. 1.59±0.14, p=0.82). However, the WR was higher in patients with CSAS than in patients without CSAS (40±8% vs. 30±12%, p<0.05). ODI significantly correlated with central chemosensitivity to carbon dioxide. Moreover, there was a highly significant correlation between WR and central chemosensitivity (r=0.65, p<0.05). However, there was no correlation between ODI and the WR (r=0.36, p=0.11). Cardiac sympathetic nerve activity in patients with CHF and CSAS is

  7. Bisphenol-A (BPA), BPA glucuronide, and BPA sulfate in mid-gestation umbilical cord serum in a Northern and Central California population

    Science.gov (United States)

    Gerona, Roy R.; Woodruff, Tracey J.; Dickenson, Carrie A.; Pan, Janet; Schwartz, Jackie M.; Sen, Saunak; Friesen, Matthew M.; Fujimoto, Victor Y.; Hunt, Patricia A.

    2013-01-01

    Bisphenol-A (BPA) is an endocrine disrupting chemical used in numerous consumer products, resulting in universal exposure in the United States. Prenatal exposure to BPA is associated with numerous reproductive and developmental effects in animals. However, little is known about human fetal exposure or metabolism of BPA during mid-gestation. In the present study, we present a new liquid chromatography-tandem mass spectrometry method to directly measure concentrations of BPA and two predominant metabolic conjugates – BPA glucuronide and BPA sulfate – in umbilical cord serum collected from elective 2nd trimester pregnancy terminations. We detected at least one form of BPA in all umbilical cord serum samples: BPA (GM 0.16; range

  8. Síndrome do incisivo central superior solitário: relato de caso Solitary median maxillary central incisor syndrome: case report

    Directory of Open Access Journals (Sweden)

    Eduardo Machado

    2010-08-01

    Full Text Available INTRODUÇÃO: a presença de um incisivo central superior solitário é um evento bastante incomum na população. A prevalência da chamada Síndrome do Incisivo Central Superior Solitário (SICSS é verificada em 1:50.000 nascimentos, sendo registrado um maior acometimento no sexo feminino. Essa alteração no desenvolvimento da oclusão dentária é caracterizada por más formações estruturais, sobretudo na região de linha média do paciente. O diagnóstico precoce e o tratamento adequado dessa síndrome são de grande importância, pois essa condição talvez seja um indicativo de que o paciente pode apresentar outras más formações congênitas severas, não devendo ser a SICSS considerada uma simples anomalia dentária. Os procedimentos ortodônticos, nesses casos, variam dependendo do grau de comprometimento das estruturas ósseas da maxila, da oclusão em si, e principalmente da sutura palatina mediana. OBJETIVO: discutir, baseado em evidências científicas, aspectos importantes relacionados à SICSS, bem como apresentar um caso clínico de paciente do sexo feminino com SICSS, que foi submetida a tratamento ortodôntico na Clínica Odontológica Integrada Infantil da Universidade Federal de Santa Maria / RS. CONCLUSÃO: pela análise crítica da literatura, verifica-se ser muito importante o diagnóstico correto e precoce acerca dessa síndrome, visto que há possibilidade da mesma estar associada a outros problemas de desenvolvimento. Além disso, o paciente acometido pela SICSS deve ser assistido por uma equipe multidisciplinar de saúde, de forma a otimizar os resultados clínicos e devolver-lhe qualidade de vida.INTRODUCTION: The presence of a single median maxillary central incisor is an uncommon event in the population. The prevalence of the Solitary Median Maxillary Central Incisor (SMMCI syndrome is about 1:50,000 live births, occurring more in women. This alteration in the development of the dental occlusion is

  9. A regeneration strategy for spinal cord injury

    OpenAIRE

    Nordblom, Jonathan

    2012-01-01

    A severe traumatic spinal cord injury (SCI) frequently leads to a devastating and permanent disability. Due to glial scarring and an inhibitory local environment, regrowth of disrupted axons in the injured spinal cord beyond a lesion is obstructed, thus preventing reconnection with neurons at the other side. Many experimental strategies have been presented to limit the damage and improve outcome after SCI, but few options are available for the patient. Neurons in the central nervous sys...

  10. [Solitary median maxillary central incisor syndrome:a case of report].

    Science.gov (United States)

    Kang, Li-ying; Liu, Xin-qiang

    2014-04-01

    Solitary median maxillary central incisor (SMMCI) is a rare dental anomaly characterized by a symmetric central incisor of normal size, developed and erupted precisely in the midline of the maxilla in both primary and permanent dentitions. SMMCI may occur alone or be associated with other midline structures defects of the body or other systemic disorders. The best known association is holoprosencephaly (HPE). This paper reported a case of SMMCI that companied with other midline structures defects of the body. PMID:24935855

  11. The neurobiology of the spinal cord in experimental parkinsonism and Parkinson's disease.

    Science.gov (United States)

    Ferrucci, Michela; Biagioni, Francesca; Vivacqua, Giorgio; Busceti, Carla Letizia; Bartalucci, Alessia; Soldani, Paola; D'Este, Loredana; Fumagalli, Lorenzo; Fornai, Francesco

    2013-12-01

    The neurobiology of non-motor symptoms in Parkinson's disease (PD) reveals a number of unexpected areas which once were not recognized a priori as part of the neuropathology underlying PD. These areas may belong either to central nervous system or periphery. Among central areas major efforts in the last decade led to recognize a number of brain nuclei as part of the disease spreading or disease onset in PD patients. Unexpectedly recent evidence deriving from pathological studies in PD patients and corroborated by experimental models of PD provided clear evidence that the spinal cord is often recruited in PD pathology. Such an involvement is intriguing since the major degenerative disease of the spinal cord (amyotrophic lateral sclerosis) features the involvement of dopaminergic neurons of the substantia nigra pars compacta, while some environmental (parkinsonism, ALS, and dementia of Guam) and genetic (Kufor-Rakeb syndrome) diseases are known to be characterized by mixed degeneration of pyramidal and extrapyramidal regions. Thus, the clear-cut between degeneration of dopaminergic neurons in the substantia nigra and the loss of pyramidal motor system appears now more as a continuum of   degeneration which converge in abnormal activity and cell pathology of motor neurons as a final common pathway. Among motor neurons, visceral efferent cells of the spinal cord are involved and provide a robust neurobiological findings which may justify a variety of non-motor autonomic symptoms which characterize PD. Neurodegeneration in the spinal cord extends to the dorsal horn of the grey matter posing an intriguing link between PD and sensory alterations. The present manuscript reviews the involvement of multiple regions of the spinal cord in PD and experimental parkinsonism in the attempt to provide both a neurobiological background to understand non motor symptoms and to provide the anatomical basis for disease spreading. PMID:24873929

  12. Mechanism of umbilical cord mesenchymal stem cell therapy for metabolic syndrome%脐带间充质干细胞治疗代谢综合征的作用机制

    Institute of Scientific and Technical Information of China (English)

    胡爽爽; 潘兴华

    2015-01-01

    BACKGROUND:Studies have shown that stem cels are directly involved in injury repair, secretion of growth factors to promote wound healing, improvement of blood circulation and promotion of angiogenesis, regulation of immune and inflammation, oxidative resistance, which may be used to treat a variety of chronic diseases. OBJECTIVE:To review the mechanism of umbilical cord mesenchymal stem cel therapy for metabolic syndrome. METHODS:A computer-based search of VIP, CNKI and PubMed was performed for articles concerning stem cel transplantation therapy for metabolic syndrome published from January 2004 to October 2014. The search terms were “stem cels, umbilical cord-derived mesenchymal stem cels, stem cels transplantation, metabolic syndrome, insulin resistance, diabetes, hyperlipidemia” in Chinese and English, respectively. Finaly, 42 articles were included in result analysis. RESULTS AND CONCLUSION: Umbilical cord mesenchymal stem cels have the ability of self replication and differentiation, which are ideal seed cels for cel replacement therapy. Meanwhile, umbilical cord mesenchymal stem cels also have paracrine and immunomodulatory functions. Currently, technologies for isolation, culture, identification and in vitro amplification of umbilical cord mesenchymal stem cels are quite mature. Moreover, stem cel transplantation as a new method for the treatment of metabolic syndrome can fundamentaly make the target organ damage better as wel as improve insulin resistance and glucose and lipid metabolism disorder, which have been proved in animal experiments. However, there are stil many clinical problems to be solved.%背景:研究表明,干细胞的主要功能有直接参与损伤修复、分泌生长因子促进损伤修复、促血管再生改善血液循环、调节免疫和炎症、抗氧化应激等,可用于治疗多种慢性疾病。目的:综述脐带间充质干细胞治疗代谢综合征的作用机制。方法:以“干细胞,脐带间充

  13. Spinal cord injury reveals multilineage differentiation of ependymal cells.

    Directory of Open Access Journals (Sweden)

    Konstantinos Meletis

    2008-07-01

    Full Text Available Spinal cord injury often results in permanent functional impairment. Neural stem cells present in the adult spinal cord can be expanded in vitro and improve recovery when transplanted to the injured spinal cord, demonstrating the presence of cells that can promote regeneration but that normally fail to do so efficiently. Using genetic fate mapping, we show that close to all in vitro neural stem cell potential in the adult spinal cord resides within the population of ependymal cells lining the central canal. These cells are recruited by spinal cord injury and produce not only scar-forming glial cells, but also, to a lesser degree, oligodendrocytes. Modulating the fate of ependymal progeny after spinal cord injury may offer an alternative to cell transplantation for cell replacement therapies in spinal cord injury.

  14. Association of CHRDL1 mutations and variants with X-linked megalocornea, Neuhauser syndrome and central corneal thickness.

    Directory of Open Access Journals (Sweden)

    Alice E Davidson

    Full Text Available We describe novel CHRDL1 mutations in ten families with X-linked megalocornea (MGC1. Our mutation-positive cohort enabled us to establish ultrasonography as a reliable clinical diagnostic tool to distinguish between MGC1 and primary congenital glaucoma (PCG. Megalocornea is also a feature of Neuhäuser or megalocornea-mental retardation (MMR syndrome, a rare condition of unknown etiology. In a male patient diagnosed with MMR, we performed targeted and whole exome sequencing (WES and identified a novel missense mutation in CHRDL1 that accounts for his MGC1 phenotype but not his non-ocular features. This finding suggests that MMR syndrome, in some cases, may be di- or multigenic. MGC1 patients have reduced central corneal thickness (CCT; however no X-linked loci have been associated with CCT, possibly because the majority of genome-wide association studies (GWAS overlook the X-chromosome. We therefore explored whether variants on the X-chromosome are associated with CCT. We found rs149956316, in intron 6 of CHRDL1, to be the most significantly associated single nucleotide polymorphism (SNP (p = 6.81×10(-6 on the X-chromosome. However, this association was not replicated in a smaller subset of whole genome sequenced samples. This study highlights the importance of including X-chromosome SNP data in GWAS to identify potential loci associated with quantitative traits or disease risk.

  15. Metabolic syndrome in the rural population of Wardha, Central India: An exploratory factor analysis

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    Pradeep R Deshmukh

    2013-01-01

    Full Text Available Background and Objectives: Metabolic syndrome - a plausible precondition for type II diabetes and cardiovascular diseases is also on rise. To understand the mechanistic complexity of metabolic syndrome it is imperative to study the specific contribution of the determinants of metabolic syndrome. Such study can help to identify the most significant factor which may be of use in early detection as well as prevention efforts. Such information is scarcely available from India and especially from rural India. Hence, the present study was undertaken to explore for such factor which might be considered crucial for development of such pathogenesis particularly in rural population of Wardha. Methods: A cross-sectional study comprising of 300 subjects was carried out in rural area of Primary Health Center, attached to medical college with approximate 31,000 populations. The anthropometric parameters such as height, weight, waist circumference were measured. Overnight fasting samples were collected for lipid profile (total cholesterol, triglyceride, high density lipoproteins, low density lipoproteins, very low density lipoproteins and fasting blood glucose levels. The National Cholesterol Education Programme Adult Treatment Panel, ATP-III guidelines were used to categorize the study subjects. As many of the variables are highly intercorrelated, exploratory factor analysis was carried out to reduce the data to a smaller number of independent factors that accounts for the most of the variances in the data. Principal component analysis was used as a method of extraction. Results: For both sexes, three factors were extracted accounting for about 71% variance in the measured variables. An adiposity factor which accounted for highest explained variance (28%, was the initial factor extracted. It was loaded positively by waist circumference, triglyceride, and very low density lipoprotein and negatively loaded by high density lipoprotein. Second factor extracted

  16. Nevirapine-induced Stevens-Johnson syndrome from a tertiary care hospital in Central India

    Directory of Open Access Journals (Sweden)

    Vandana Badar

    2014-04-01

    Full Text Available A 42 year old man, a known case of AIDS receiving antiretroviral therapy – stavudine, lamivudine, and nevirapine since 4 weeks, was admitted with erythematous rash, with blisters, and abnormal liver function test. A diagnosis of Steven-Johnson syndrome was made excluding other opportunistic infection and differential diagnosis. Nevirapine was discontinued until the patient recovered. After recovery patient was started with stavudine, lamivudine, and efavirenz treatment without re-challenge with nevirapine. [Int J Basic Clin Pharmacol 2014; 3(2.000: 408-409

  17. Spinal Cord Dysfunction (SCD)

    Data.gov (United States)

    Department of Veterans Affairs — The Spinal Cord Dysfunction (SCD) module supports the maintenance of local and national registries for the tracking of patients with spinal cord injury and disease...

  18. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... the use of electrical stimulation for spinal cord injuries? What is "Braingate" research? What is the status of stem-cell research? How would stem-cell therapies work in the treatment of spinal cord injuries? ...

  19. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... injury? What is the "Spinal Cord Injury Model Systems" program? ... family FacingDisability is designed to provide Internet-based information and support for people with spinal cord injuries ...

  20. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Patient Partnerships How Social Workers Help Transitions How Social Workers Help Transitions Occupational Therapy After Spinal Cord Injury Occupational Therapy After Spinal Cord Injury How Occupational Therapists Work How Occupational Therapists Work Occupational Therapy Enables Daily ...

  1. 脊髓栓系综合征患儿盆底神经电生理检测特点和临床意义%Electrophysiological study of pelvic floor musculature of tethered cord syndrome

    Institute of Scientific and Technical Information of China (English)

    张丽; 李金良; 孙小兵; 李艳华; 陈雨历

    2012-01-01

    目的 利用神经电生理检测技术分析脊髓栓系综合征(tethered cord syndrome,TCS)患儿盆底神经和肌肉功能改变的特点,评价TCS患儿盆底神经肌肉损伤的程度.方法 2008年5月至2010年12月对88例TCS患儿组和26例正常患儿行双侧肛提肌、耻骨直肠肌和肛门外括约肌肌电图检查,以及阴部神经运动传导和阴部神经体感诱发电位(PN-SSEP)检查.结果 正常对照组患儿肛提肌、肛门外括约肌和耻骨直肠肌在静息状态时均可见持续收缩动作电位,大力收缩动作电位密集,呈干扰相.患儿组盆底肌肌电图(pelvic floor muscles electromyographic,PFM-EMG)表现为不同程度的神经源性损害,甚至呈现完全失神经支配,肛提肌较肛门外括约肌病变严重.患儿组阴部神经运动传导波幅降低,潜伏期延长,其中15例患儿出现双侧不对称.阴部神经体感诱发电位潜伏期延长,甚至波形消失.结论 盆底神经电生理学检测对了解TCS盆底神经肌肉损害程度,评价大小便功能,估计预后具有重要意义.%Objective We aim to analyze the electrophysiological characteristics of pelvic floor musculature of the patients with tethered cord syndrome(TCS),and to evaluate the extent of pelvic floor neuromuscular injury.Methods 88 Patients with tethered cord syndrome and 26 normal children had electromyography of levator ani,puborectalis and external anal sphincter performed.Pudendalmotor nerve conduction,as well as pudendal nerve somatosensory evoked potential study(PN-SSEP) were carried out from May,2008 to December,2010.Results Levator ani,external anal sphincter and puborectalis all had tonic activity in normal children at rest.During maximum voluntary contraction,action potential was seenas an increase of the firing frequency of these motor units,and recruitment of motor units was of interference pattern(IP).In patients suffering from TCS,PFM-EMG indicated different degrees of neurogenic damage,even to the

  2. The Effects of Lamotrigine on Pain, Sleep, and Mood in Refractory Form of Central Post-Stroke Pain Syndrome

    Directory of Open Access Journals (Sweden)

    Peyman Petramfar

    2010-12-01

    Full Text Available Background: Central post-stroke pain (CPSP is a distressingpain syndrome, sometimes become refractory to the conventionalpain managements. Anticonvulsants have been used toalleviate different central pains. Lamotrigine is a novel anticonvulsantand its proper dosage and its efficacy have notbeen well studied yet. The aim of this study was to evaluatethe effect of 100 mg lamotrigine on refractory form of CPSP.Methods: The medical files of 17 patients with CPSP who hadnot responded to the other drugs and were treated with lamotriginewere studied. Using Brief Pain Inventory, pain, sleepand mood were assessed before, and after 8 and 24 weeks oftreatment.Results: After 24 weeks, 70.5 % of the patients responded tolamotrigine, and there was an improvement of 2.41 in themean score of average pain (P=0.001.Conclusion: Lamotrigine 100 mg daily was effective in thetreatment of refractory CPSP, and might be prescribed beforeplanning for more aggressive surgical managements.Iran J Med Sci 2010; 35(4: 299-303.

  3. Pathology of tissue loss (white syndrome) in Acropora sp. corals from the Central Pacific

    Science.gov (United States)

    Work, Thierry M.; Aeby, Greta S.

    2011-01-01

    We performed histological examination of 69 samples of Acropora sp. manifesting different types of tissue loss (Acropora White Syndrome-AWS) from Hawaii, Johnston Atoll and American Samoa between 2002 and 2006. Gross lesions of tissue loss were observed and classified as diffuse acute, diffuse subacute, and focal to multifocal acute to subacute. Corals with acute tissue loss manifested microscopic evidence of necrosis sometimes associated with ciliates, helminths, fungi, algae, sponges, or cyanobacteria whereas those with subacute tissue loss manifested mainly wound repair. Gross lesions of AWS have multiple different changes at the microscopic level some of which involve various microorganisms and metazoa. Elucidating this disease will require, among other things, monitoring lesions over time to determine the pathogenesis of AWS and the potential role of tissue-associated microorganisms in the genesis of tissue loss. Attempts to experimentally induce AWS should include microscopic examination of tissues to ensure that potentially causative microorganisms associated with gross lesion are not overlooked.

  4. Central nervous system mechanisms contributing to the cachexia-anorexia syndrome.

    Science.gov (United States)

    Plata-Salamán, C R

    2000-10-01

    The cachexia-anorexia syndrome occurs in chronic pathophysiologic processes including cancer, infection with human immunodeficiency virus, bacterial and parasitic diseases, inflammatory bowel disease, liver disease, obstructive pulmonary disease, cardiovascular disease, and rheumatoid arthritis. Cachexia makes an organism susceptible to secondary pathologies and can result in death. Cachexia-anorexia may result from pain, depression or anxiety, hypogeusia and hyposmia, taste and food aversions, chronic nausea, vomiting, early satiety, malfunction of the gastrointestinal system (delayed digestion, malabsorption, gastric stasis and associated delayed emptying, and/or atrophic changes of the mucosa), metabolic shifts, cytokine action, production of substances by tumor cells, and/or iatrogenic causes such as chemotherapy and radiotherapy. The cachexia-anorexia syndrome also involves metabolic and immune changes (mediated by either the pathophysiologic process, i.e., tumor, or host-derived chemical factors, e.g., peptides, neurotransmitters, cytokines, and lipid-mobilizing factors) and is associated with hypertriacylglycerolemia, lipolysis, and acceleration of protein turnover. These changes result in the loss of fat mass and body protein. Increased resting energy expenditure in weight-losing cachectic patients can occur despite the reduced dietary intake, indicating a systemic dysregulation of host metabolism. During cachexia, the organism is maintained in a constant negative energy balance. This can rarely be explained by the actual energy and substrate demands by tumors in patients with cancer. Overall, the cachectic profile is significantly different than that observed during starvation. Cachexia may result not only from anorexia and a decreased caloric intake but also from malabsorption and losses from the body (ulcers, hemorrhage, effusions). In any case, the major deficit of a cachectic organism is a negative energy balance. Cytokines are proposed to participate

  5. Contemporary Insights and Novel Treatment Approaches to Central Sleep Apnea Syndrome in Heart Failure

    OpenAIRE

    Grayburn, Ryan L.; Kaka, Yaquta; Wilson Tang, W. H.

    2014-01-01

    Central sleep apnea (CSA) is a common and under-diagnosed condition commonly associated with Cheyne-Stokes respiration. It is particularly prevalent in the heart failure population affecting up to 40% of all patients with heart failure. The pathophysiology associated with CSA is based on the underlying effects of hypoventilation and hyperventilation, with neurologic dysregulation of respiratory control as the primary defect. However, therapeutic options are limited due to the prevailing perce...

  6. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Coping with a New Injury Adjusting to Social Life in a Wheelchair Adjusting to Social Life in a Wheelchair Substance Abuse and Spinal Cord ... Substance Abuse and Spinal Cord Injury How Family Life Changes After Spinal Cord Injury How Family Life ...

  7. Spinal Cord Injuries

    Science.gov (United States)

    ... forth between your body and your brain. A spinal cord injury disrupts the signals. Spinal cord injuries usually begin with a blow that fractures or ... down on the nerve parts that carry signals. Spinal cord injuries can be complete or incomplete. With a complete ...

  8. 脊柱裂致脊髓栓系综合征患者性功能现状评估%Evaluation of sexual function in men with tethred cord syndrome associated with spina bifida

    Institute of Scientific and Technical Information of China (English)

    马彦; 韩晓敏; 汪隆旺; 李兵; 潘峰

    2012-01-01

    Objective:To assess sexual function in men with tethered cord syndrome (TCS) caused by Spinal Bifida. Methods: A cross-sectional study was performed in 16 adult men with TCS who had have stabilized sexual partners in recent 10 years. The International Index of Erectile Function (IIEF) and Erection Hardness Score (EHS) questionnaire was mailed to each man. Results:Of the 16 patients with TCS, 12 completed the IIEF questionnaire and EHS finally. Of the 12 men who replied, 10 (83%) had erectile dysfunction (ED). 70% of the patients were mild to moderate and moderate ED. Only 1 patients (8%) with TCS developed grade 4 on the EHS, and 58% of the patients developed grade 3. Conclusions:The incidence rate of ED in TCS patients was high. IIEF is equally effective to assess sexual function in men with TCS.%目的:研究脊柱裂(spina bifida,SB)致脊髓栓系综合征(tethered cord syndrome,TCS)患者勃起功能状况.方法:近10年来收治TCS患者中,成年男性且有稳定性伴侣者16例.采用邮件问卷方法,对这些患者行国际勃起功能指数(IIEF)问卷及阴茎硬度分级问卷评估.结果:16例TCS患者中,4例未完成问卷.余12例患者中,10例(83%)诊断为勃起功能障碍(erectile dysfunction,ED),其中轻-中度和中度ED占总例数的70%.仅有1例(8%)TCS患者阴茎硬度达到4级,阴茎达到3级硬度的占58%.结论:TCS患者有较高的ED发生.IIEF自评量表能很好地反映TCS患者ED的发病情况.

  9. APOPTOSIS AFTER SPINAL CORD INJURY IN RATS

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To confirm the role played by apoptosis in spinal cord injury. Methods 36 rats models of spinal cord injury were made by Allen method. Histological examinations using HE staining and in situ end-labeling were used to observe apoptosis in spinal cord tissues from 1h to 21d after injury. Results HE staining sections showed hemorrhage and necrosis, neuronal degeneration and gliai cell proliferation. In situ end-labeling sections showed the appearance of apoptosis in both gray and white matter as well as in both central and surrounding region. The number of apoptotic cells increased from 12h after injury, increased to the peak at 4d and declined to normal at 21d. Conclu sion The results suggest that apoptosis, especially glial apoptosis, plays a role in the pathogenesis of spinal cord in jury.

  10. Solitary median maxillary central incisor syndrome: clinical case with a novel mutation of sonic hedgehog.

    Science.gov (United States)

    Garavelli, Livia; Zanacca, C; Caselli, G; Banchini, G; Dubourg, C; David, V; Odent, S; Gurrieri, F; Neri, G

    2004-05-15

    Solitary median maxillary central incisor (SMMCI) is a rare dental anomaly. It is usually considered as a minor manifestation of holoprosencephaly (HPE). Some reported families had severe cases of HPE in some members and SMMCI in others. Mutations of Sonic Hedgehog (SHH) have been documented in these families. SMMCI has also been found as an isolated finding or together with other anomalies such as microcephaly, short stature, endocrine pathology, and choanal atresia. We describe a patient with SMMCI and a novel SHH mutation: Val332Ala. PMID:15103725

  11. Bevacizumab for the treatment of surgically unresectable cervical cord hemangioblastoma: a case report

    Directory of Open Access Journals (Sweden)

    Omar Ayman I

    2012-08-01

    Full Text Available Abstract Introduction Hemangioblastomas are highly vascular tumors that can arise within the central nervous system as well as other organ systems within the body. They can arise sporadically or as part of von Hippel-Lindau syndrome. Those arising in critical locations within the central nervous system can be difficult to resect surgically and therefore pose a significant challenge and result in morbidity and even mortality. Hemangioblastomas express high levels of vascular endothelial growth factor that drives angiogenesis and tumor progression. We hypothesized that bevacizumab through its inhibitory effect on vascular endothelial growth factor will result in hemangioblastoma tumor regression as well as a meaningful clinical response. Case presentation We present the case of a 51-year-old Caucasian man with surgically unresectable cervical cord hemangioblastoma presenting with progressive weakness leading to quadriparesis. He was treated with bevacizumab and his follow up magnetic resonance imaging scans showed marked tumor regression. After only six cycles of intravenous bevacizumab (10mg/kg every two weeks, he started ambulating after being wheelchair bound. He is currently still receiving treatment almost two years after initiation of bevacizumab. Conclusions We have shown for the first time that bevacizumab can result in significant tumor regression and a sustained clinical improvement in a patient with an otherwise unresectable spinal cord hemangioblastoma. This novel approach can be immensely useful for patients with difficult to resect hemangioblastomas or those with multiple lesions such as in von Hippel-Lindau syndrome.

  12. Role of central histaminergic system in lorazepam withdrawal syndrome in rats.

    Science.gov (United States)

    Nath, C; Gupta, M B

    2001-04-01

    Effects of histaminergic agonists and antagonists were investigated on withdrawal signs in lorazepam-dependent rats. Physical dependence was developed by giving lorazepam admixed with the food in the following dose schedule (in mg/kg given daily x days): 10 x 4, 20 x 4, 40 x 4, 80 x 4, and 120 x 7. The parameters observed during the periods of administration of lorazepam and after its withdrawal were spontaneous locomotor activity (SLA), reaction time to pain, foot shock aggression (FSA), and audiogenic seizures. During the withdrawal period, the rats were divided into groups of 10 each. Control-withdrawal group did not receive any drug. The drugs (in mg/kg administered intramuscularly)--L-histidine (50), histamine-N-methyl (2), promethazine (10), pheniramine (10), astemizole (10), and thioperamide (1)--were given separately in other groups daily during the withdrawal period. The withdrawal signs in control group were hyperkinesia, hyperaggression, and audiogenic seizures. L-Histidine, precursor of histamine, and thioperamide, antagonist of H3 receptor, potentiated hyperkinesia, hyperaggression, and audiogenic seizures. Histamine-N-methyl, agonist of H3 receptor, and H1 receptor antagonists, promethazine and pheniramine, blocked all the withdrawal signs. Astemizole, a peripheral antagonist of H1 receptor, could not affect any withdrawal sign. It may be concluded that histamine H1 receptors are facilitatory and H3 receptors are inhibitory for benzodiazepine (BZD) withdrawal syndrome.

  13. Ictal central apnea and bradycardia in temporal lobe epilepsy complicated by obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    Yoko Nishimura

    2015-01-01

    Full Text Available We describe the case of a 12-year-old boy who developed temporal lobe epilepsy (TLE with daily complex partial seizures (CPS and monthly generalized seizures. Moreover, he frequently snored while asleep since early childhood. Polysomnography (PSG revealed severe obstructive sleep apnea with apnea–hypopnea index (AHI of 37.8/h. Video-PSG with simultaneous electroencephalography (EEG recording captured two ictal apneic episodes during sleep, without any motor manifestations. The onset of rhythmic theta activity in the midtemporal area on EEG was preceded by the onset of apnea by several seconds and disappeared soon after cessation of central apnea. One episode was accompanied by ictal bradycardia of <48 beats/min which persisted for 50 s beyond the end of epileptic activity. After treatment with carbamazepine and tonsillectomy/adenoidectomy, the seizures were well controlled and AHI decreased to 2.5/h. Paroxysmal discharges also disappeared during this time. Uncontrolled TLE complicated by sleep apnea should be evaluated for the presence of ictal central apnea/bradycardia.

  14. Does repair of spinal cord injury follow the evolutionary theory?

    Institute of Scientific and Technical Information of China (English)

    Zhicheng Zhang; Fang Li; Tiansheng Sun

    2012-01-01

    Lower vertebrates, such as fish and amphibians, and higher vertebrates in embryonic development can acquire complete regeneration of complex body structures, including the spinal cord, an important part of the central nervous system. However, with species evolution and development, this regenerative capacity gradually weakens and even disappears, but the cellular and molecular mechanisms remain poorly understood. We explored the differences in mechanisms of spinal cord regeneration capability between lower and higher vertebrates, investigated differences in their cellular and molecular mechanisms and between the spinal cord structures of lower vertebrates and mammals, such as rat and monkey, to search for theoretical evidence and therapeutic targets for nerve regeneration in human spinal cord.

  15. Central Anticholinergic Syndrome due to Hypoxia-Induced Bradycardia in a Child with Difficult Intubation Undergoing Complete Dental Restoration: A Case Report.

    Science.gov (United States)

    Gharavifard, Mohamad; Razavi, Majid; Ghandehari Motlagh, Mehdi; Ziyaeifard, Mohsen

    2014-09-01

    Central anticholinergic syndrome (CAS) following general anesthesia (GA) is a well known syndrome in children and adults. Many cases of CAS have been previously reported in the literature. However, there are only two reports of post resuscitation CAS after administration of small doses of atropine. Hereby, we report a case of CAS in a child undergoing complete dental restoration under GA after receiving a small dose of atropine to reverse hypoxia induced bradycardia. Intraoperative events such as hypoxia or cardiac arrest may play a role as triggers for CAS. However, we cannot establish a causal relationship between the occurrence of CAS and such critical events.

  16. Central Anticholinergic Syndrome due to Hypoxia-Induced Bradycardia in a Child with Difficult Intubation Undergoing Complete Dental Restoration: A Case Report.

    Directory of Open Access Journals (Sweden)

    Mohamad Gharavifard

    2014-10-01

    Full Text Available Central anticholinergic syndrome (CAS following general anesthesia (GA is a well known syndrome in children and adults. Many cases of CAS have been previously reported in the literature. However, there are only two reports of post resuscitation CAS after administration of small doses of atropine. Hereby, we report a case of CAS in a child undergoing complete dental restoration under GA after receiving a small dose of atropine to reverse hypoxia induced bradycardia. Intraoperative events such as hypoxia or cardiac arrest may play a role as triggers for CAS. However, we cannot establish a causal relationship between the occurrence of CAS and such critical events.

  17. Implanted central venous catheter-related acute superior vena cava syndrome: management by metallic stent and endovascular repositioning of the catheter tip

    Energy Technology Data Exchange (ETDEWEB)

    Qanadli, S.D.; Mesurolle, B.; Sissakian, J.F.; Chagnon, S.; Lacombe, P. [Service de Radiologie, Hopital Ambroise Pare, 92 - Boulogne (France)

    2000-08-01

    We describe a case of a 49-year-old woman with stage-IIIB lung adenocarcinoma who experienced an acute superior vena cava syndrome related to an implanted central venous catheter without associated venous thrombosis. The catheter was surgically implanted for chemotherapy. Superior vena cava syndrome appeared after the procedure and was due to insertion of the catheter through a subclinical stenosis of the superior vena cava. Complete resolution of the patient's symptoms was obtained using stent placement and endovascular repositioning of the catheter tip. (orig.)

  18. Neuropathological changes of central nervous system in Guillain-Barré syndrome.%吉兰-巴雷综合征中枢神经系统病理改变

    Institute of Scientific and Technical Information of China (English)

    林世和; 赵节绪; 江新梅; 宋晓南

    2001-01-01

    Objective  To investigate the neuropathological changes of central nervous system in Guillain-Barré syndrom. Methods Brain, spinal cord and sciatic nerve were obtained from 22 cases of Guillain-Barré syndrome. Eight cases were examined by general autopsy, 14 cases were examined by limited autopsy. HE, KB, Bielschowsky, Weil and Sudan Ⅲ staining were carried out, the sections were observed by light microscopy. Results 1.Cerebral superficial veins congested, widening of the cortical sulci, narrowed gyri and mild cerebellar tonsillar hernia were present. 2. Majority of cerbral neurons presented an ischemic changes. Slightly loss of hippocampal pyramidal neurons were found. There was chromatolysis of motor neurons of brain stem. Lymphocytic infiltration around the small vessels occurred in the pons and medullary oblongata in 8 cases. Focal demyelination was noted in pons and frontal white matter in 2 cases. Loss of Purkinje cells and appearance of glial nodules were observed in molecular layer of cerebellum. 3. Swellin, central chromatolysis and eccentric nuclei of anterior horn cells appeared in 16 cases, which were pronounced in cervical and lumbal segment of spinal cord. Vaculated neuroplasma and lymphacytic infiltrition could be seen. 4. Segmental demyelination and lymphocytic infiltration were the main neuropathological changes observed in 20 cases. There were two other cases in which the axon were severely involved, which showed swelling and breakdown of axons and as well as axonal bulbs. Conclusions 1. Lymphocytic infiltration in brain stem and spinal cord were in continuousness of pathological changes of peripheral nerves. 2. Finding of glial nodules suggested that there was possibility of infection of neurotropic virus. 3. Occurence of focal demyelination in cerebrum and brain stem indicated that Guillain-Barré syndrome may have combined involvement of central and peripheral nervous system.%目的观察

  19. Acute Cerebrovascular Radiation Syndrome: Radiation Neurotoxicity , mechanisms of CNS radiation injury, advanced countermeasures for Radiation Protection of Central Nervous System.

    Science.gov (United States)

    Popov, Dmitri; Jones, Jeffrey; Maliev, Slava

    Key words: Cerebrovascular Acute Radiation Syndrome (Cv ARS), Radiation Neurotoxins (RNT), Neurotransmitters, Radiation Countermeasures, Antiradiation Vaccine (ArV), Antiradiation Blocking Antibodies, Antiradiation Antidote. Psychoneuroimmunology, Neurotoxicity. ABSTRACT: To review the role of Radiation Neurotoxins in triggering, developing of radiation induced central nervous system injury. Radiation Neurotoxins - rapidly acting blood toxic lethal agent, which activated after irradiation and concentrated, circulated in interstitial fluid, lymph, blood with interactions with cell membranes, receptors and cell compartments. Radiation Neurotoxins - biological molecules with high enzymatic activity and/or specific lipids and activated or modified after irradiation. The Radiation Neurotoxins induce increased permeability of blood vessels, disruption of the blood-brain barrier, blood-cerebrospinal fluid (CSF) barrier and developing severe disorder of blood macro- and micro-circulation. Principles of Radiation Psychoneuro-immunology and Psychoneuro-allergology were applied for determination of pathological processes developed after irradiation or selective administration of Radiation Neurotoxins to radiation naïve mammals. Effects of radiation and exposure to radiation can develop severe irreversible abnormalities of Central Nervous System, brain structures and functions. Antiradiation Vaccine - most effective, advanced methods of protection, prevention, mitigation and treatment and was used for of Acute Radiation Syndromes and elaboration of new technology for immune-prophylaxis and immune-protection against ϒ, Heavy Ion, Neutron irradiation. Results of experiments suggested that blocking, antitoxic, antiradiation antibodies can significantly reduce toxicity of Radiation Toxins. New advanced technology include active immune-prophylaxis with Antiradiation Vaccine and Antiradiation therapy that included specific blocking antibodies to Radiation Neurotoxins

  20. Cerebral salt wasting syndrome following atlantoaxial fracture dislocation in Down syndrome.

    Science.gov (United States)

    Abdel-Latif, Mohamed El-Amin; Chan, Patrick W-K; Goh, Adrian Yu-Teik; Lum, Lucy Chai-See

    2009-01-01

    We describe cerebral salt wasting syndrome (CSWS) in a 5-year-old female child with Down syndrome who had acute myelopathy secondary to chronic atlantoaxial subluxation and fracture dislocation of the odontoid process. The patient developed hyponatraemia associated with excessive urine output and elevated urine sodium concentration following her injury. An administered volume-for-volume replacement of urine loss with 0.9% sodium chloride resulted in an excellent outcome. This patient illustrates the importance of ascertaining CSWS in children with spinal cord disorders, in addition to the syndrome of inappropriate antidiuretic hormone (SIADH) secretion and diabetes insipidus (DI) commonly encountered following a central nervous system (CNS) injury, as the specific treatment approaches is clearly associated with an excellent outcome.

  1. BURNOUT SYNDROME, JOB SATISFACTION LEVELS AND RELATED FACTORS IN CENTRAL TRABZON PROVINCE PRIMARY HEALTH CENTER WORKERS

    Directory of Open Access Journals (Sweden)

    Asuman YAVUZYILMAZ

    2007-02-01

    Full Text Available Burnout manifests itself in individuals working in professions involving face-to-face contact with the public in depersonalization towards others, feelings of emotional exhaustion, and reduced feelings of personal achievement and adequacy. The objective in this study was to determine burnout and job satisfaction levels and related factors in primary health center personnel in the central part of the Turkish province of Trabzon. A total of 227 people working in central Trabzon province primary health centers participated in this cross-sectional study, a level of 90.4%. The Maslach Burnout Inventory was used to determine burnout level and the Job Satisfaction Inventory for job satisfaction. Burnout levels in health personnel were high among women (15.06±5.57, married individuals (14.80±5.65 and those dissatisfied with their working conditions (16.80±5.81; physicians (5.00±2.79, those without children (5.19±2.54, those whose spouses were not working (4.69±2.70 and smokers (4.71±3.29 had a high level of depersonalization; and married individuals were determined to have a low personal achievement level (10.24±4.14 (p=0.020, p=0.028, p=0.011, p=0.038, p=0.028, p=0.012 and p=0.010, respectively. In conclusion, gender, marital status, age, satisfaction with working conditions and income level were determined to be related to burnout and job satisfaction. [TAF Prev Med Bull 2007; 6(1.000: 41-50

  2. BURNOUT SYNDROME, JOB SATISFACTION LEVELS AND RELATED FACTORS IN CENTRAL TRABZON PROVINCE PRIMARY HEALTH CENTER WORKERS

    Directory of Open Access Journals (Sweden)

    Murat TOPBAS

    2007-02-01

    Full Text Available Burnout manifests itself in individuals working in professions involving face-to-face contact with the public in depersonalization towards others, feelings of emotional exhaustion, and reduced feelings of personal achievement and adequacy. The objective in this study was to determine burnout and job satisfaction levels and related factors in primary health center personnel in the central part of the Turkish province of Trabzon. A total of 227 people working in central Trabzon province primary health centers participated in this cross-sectional study, a level of 90.4%. The Maslach Burnout Inventory was used to determine burnout level and the Job Satisfaction Inventory for job satisfaction. Burnout levels in health personnel were high among women (15.06±5.57, married individuals (14.80±5.65 and those dissatisfied with their working conditions (16.80±5.81; physicians (5.00±2.79, those without children (5.19±2.54, those whose spouses were not working (4.69±2.70 and smokers (4.71±3.29 had a high level of depersonalization; and married individuals were determined to have a low personal achievement level (10.24±4.14 (p=0.020, p=0.028, p=0.011, p=0.038, p=0.028, p=0.012 and p=0.010, respectively. In conclusion, gender, marital status, age, satisfaction with working conditions and income level were determined to be related to burnout and job satisfaction. [TAF Prev Med Bull. 2007; 6(1: 41-50

  3. Magnetic resonance imaging: early detection of central nervous system involvement in acquired immunodeficiency syndrome (AIDS)

    International Nuclear Information System (INIS)

    Central Nervous System (CNS) involvement, whether primary by the Human Immunodeficiency Virus - HIV - itself, or secondary (toxoplasmosis or lymphoma) is remarkably frequent in AIDS, in 40 to 70% of cases, depending upon the author. In order to study the natural history of this illness, a cohort of 25 asymptomatic seropositive patients have been established. Every 6 months these patients undergo biological and clinical examinations, as well as Magnetic Resonance brain scans. After two examinations at a 6 month's interval, the first results are reported. Out of these 25 cases, 9 present anomalies: One patient with diffuse cerebral atrophy and 8 others with high signal intensity areas on T2 weighted sequences, like those of the Multiple Sclerosis. No relationship could be demonstrated between the existence of these lesions and various criteria such as age, sex, risk factors and T4 cells count. The nature of these lesions is not lear. They certainly indicate early involvement of the CNS after primary infection by the HIV virus. They may either represent scars of the primary infection or early alterations announcing developing encephalopathy

  4. Central obesity and atherogenic dyslipidemia in metabolic syndrome are associated with increased risk for colorectal adenoma in a Chinese population

    Directory of Open Access Journals (Sweden)

    Lin Tsann

    2010-05-01

    Full Text Available Abstract Background Metabolic syndrome (MetS is composed of cardiovascular risk factors including insulin resistance, obesity, dyslipidemia, and hypertension. Most of the components of MetS have been linked to the development of neoplasm. The purpose of this study was to evaluate the relationship between individual components of MetS and colorectal adenoma. Methods The study subjects were recruited from a pool of 4872 individuals who underwent a health check-up examination during the period January 2006 to May 2008. Each participant fulfilled a structured questionnaire. MetS was defined based on the America Heart Association and National Heart Lung Blood Institute criteria. Subjects with history of colon cancer, colon polyps, colitis, or prior colonic surgery were excluded. Results A total of 4122 subjects were included for final analysis (2367 men and 1755 women; mean age, 49.6 ± 11.7 years. Of them, MetS was diagnosed in 708 men (29.9% and in 367 women (20.9%. Among the patients with MetS, 34.6% had adenoma, 31.7% had hyperplastic polyps and 23.3% were polyp-free (p Conclusions Of the components of MetS analyzed in this study, central obesity and dyslipidemia are independent risk factors for colorectal adenoma. With regard to the prevention of colorectal neoplasm, life-style modification such as weight reduction is worthwhile.

  5. Double-level Incomplete Spinal Cord Injuries: A case report

    Directory of Open Access Journals (Sweden)

    Saeed Bin Ayaz

    2014-04-01

    Full Text Available Brown-Séquard Syndrome is a type of Incomplete Spinal Cord Injury characterized by a relatively greater ipsilateral loss of proprioception and motor function, with contralateral loss of pain and temperature sensations. The residual deficits in balance produced by such injury may render a person liable to fall that may result in vertebral fracture and another injury to the spinal cord. We present here a case who initially had Brown-Séquard Syndrome due to penetrating knife injury to the neck and later on developed Cauda Equina Syndrome (another Incomplete Spinal Cord Injury due to fractured LV1 following a fall. The fracture was fixed through Pedicle Screws and the patient underwent effective rehabilitation to gain maximum achievable independence in functional activities. [Cukurova Med J 2014; 39(2.000: 392-398

  6. Serum thyroid-stimulating hormone and anti-thyroglobulin antibody are independently associated with lesions in spinal cord in central nervous system demyelinating diseases.

    Directory of Open Access Journals (Sweden)

    Youming Long

    Full Text Available Transverse myelitis (TM is associated with neuromyelitis optica (NMO and multiple sclerosis (MS. Early recognition of useful parameters may be helpful to distinguish their difference. This retrospective study analyzed thyroid parameters from 243 serum samples (relapse = 128; remission = 115 of 178 patients with demyelinating diseases (NMO, n = 25; TM, n = 48; MS, n = 105. The relationship between thyroid and clinical parameters was analyzed. Patients with NMO and TM had a higher frequency of abnormal thyroid-stimulating hormone (TSH, anti-thyroglobulin antibodies (TG-Ab, and antithyroid peroxidase antibody (TPO-Ab than MS patients (p<0.05. The level of TSH and TG-Ab returned to normal levels after administration of high-dose intravenous methylprednisolone (p<0.05. In 96 patients (NMO, n = 19; TM, n = 25; MS, n = 52 without treatment, serum levels of TSH, TG-Ab and TPO-Ab were significantly different between patients with and without myelitis (p<0.01. Patients positive for aquaporin-4 (AQP4 antibodies showed higher abnormalities of TSH (p = 0.001, TG-Ab (p = 0.004 and TPO-Ab (p<0.0001 levels than AQP4 antibodies negative patients. Logistic regression analyses revealed independent relationships between TSH (odds ratio [OR]  = 33.994; p<0.0001, TG-Ab (OR = 7.703; p = 0.017 and myelitis occurrence in 96 patients at the active stage. In 52 MS patients experiencing their first attack, MS patients with myelitis were associated with TSH abnormalities (OR = 42.778; p<0.0001. This study showed increased abnormalities of thyroid parameters in patients with NMO and TM than in MS patients. MS patients with myelitis also had greater TSH abnormality than in MS patients without myelitis. Abnormal TSH and TG-Ab were independently associated with myelitis occurrence in central nervous system demyelinating disorders.

  7. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... US ? A spinal cord injury affects the entire family FacingDisability is designed to provide Internet-based information ... spinal cord injuries and the members of their families. Our website has more than 1,500 videos ...

  8. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Adjusting to Social Life in a Wheelchair Substance Abuse and Spinal Cord Injury Substance Abuse and Spinal Cord Injury How Family Life Changes ... Patient Partnerships How Social Workers Help Transitions How Social Workers Help ... advice, recommend or endorse health care products or services, or control the information found on external websites. ...

  9. Spinal Cord Diseases

    Science.gov (United States)

    ... damages the vertebrae or other parts of the spine, this can also injure the spinal cord. Other spinal cord problems include Tumors Infections such as meningitis and polio Inflammatory diseases Autoimmune diseases Degenerative diseases such as amyotrophic lateral sclerosis and spinal ...

  10. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Fertility After Spinal Cord Injury Coping with a New Injury Coping with a New Injury Adjusting to Social Life in a Wheelchair ... after an injury? What are the most promising new treatments for spinal cord injuries? What are the ...

  11. Simultaneous Brain-Cervical Cord fMRI Reveals Intrinsic Spinal Cord Plasticity during Motor Sequence Learning.

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    Shahabeddin Vahdat

    2015-06-01

    Full Text Available The spinal cord participates in the execution of skilled movements by translating high-level cerebral motor representations into musculotopic commands. Yet, the extent to which motor skill acquisition relies on intrinsic spinal cord processes remains unknown. To date, attempts to address this question were limited by difficulties in separating spinal local effects from supraspinal influences through traditional electrophysiological and neuroimaging methods. Here, for the first time, we provide evidence for local learning-induced plasticity in intact human spinal cord through simultaneous functional magnetic resonance imaging of the brain and spinal cord during motor sequence learning. Specifically, we show learning-related modulation of activity in the C6-C8 spinal region, which is independent from that of related supraspinal sensorimotor structures. Moreover, a brain-spinal cord functional connectivity analysis demonstrates that the initial linear relationship between the spinal cord and sensorimotor cortex gradually fades away over the course of motor sequence learning, while the connectivity between spinal activity and cerebellum gains strength. These data suggest that the spinal cord not only constitutes an active functional component of the human motor learning network but also contributes distinctively from the brain to the learning process. The present findings open new avenues for rehabilitation of patients with spinal cord injuries, as they demonstrate that this part of the central nervous system is much more plastic than assumed before. Yet, the neurophysiological mechanisms underlying this intrinsic functional plasticity in the spinal cord warrant further investigations.

  12. Vocal Polyps in Tourette Syndrome

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    Michael P Chu

    2011-10-01

    Full Text Available Hoarseness and dysphonia are often a result of vocal cord polyps which in turn, are linked to vocal trauma. We report the case of vocal polyps in the setting of a 27-year old male with a history only remarkable for Tourette syndrome. We review the literature regarding etiology and pathophysiology of vocal cord lesions and propose vocal tics in Tourette syndrome as an under-recognized etiology. In this way, we also review therapies that may aid in treating not only the vocal cord lesions but also particularly in the setting of vocal tics.

  13. Split Cord Malformations

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    Yurdal Gezercan

    2015-06-01

    Full Text Available Split cord malformations are rare form of occult spinal dysraphism in children. Split cord malformations are characterized by septum that cleaves the spinal canal in sagittal plane within the single or duplicated thecal sac. Although their precise incidence is unknown, split cord malformations are exceedingly rare and represent %3.8-5 of all congenital spinal anomalies. Characteristic neurological, urological, orthopedic clinical manifestations are variable and asymptomatic course is possible. Earlier diagnosis and surgical intervention for split cord malformations is associated with better long-term fuctional outcome. For this reason, diagnostic imaging is indicated for children with associated cutaneous and orthopedic signs. Additional congenital anomalies usually to accompany the split cord malformations. Earlier diagnosis, meticuolus surgical therapy and interdisciplinary careful evaluation and follow-up should be made for good prognosis. [Cukurova Med J 2015; 40(2.000: 199-207

  14. Primary multifocal gliosarcoma of the spinal cord

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    Ramesh M. Kumar

    2016-03-01

    Full Text Available Gliosarcoma (GS is a rare and exceedingly malignant neoplasm of the central nervous system. It displays clinical features similar to glioblastoma, yet is histologically unique as it harbors both gliomatous and sarcomatous cellular components. Involvement of the neuroaxis is predominantly limited to the cerebral parenchyma and meninges. Primary GS of the spinal cord is rarely encountered. We report a case of a 54 year old male who presented with 2 months of progressive, bilateral lower extremity sensory deficits. Magnetic resonance imaging of the neuro-axis revealed multiple intradural lesions involving the cervical and thoracic spinal cord without evidence of intracranial involvement. Surgical resection of a dural based, extramedullary cervical lesion and two exophytic, intramedullary thoracic lesions revealed gliosarcoma, WHO grade IV. The patient died approximately 11 months after presentation. This report confirms that GS is not limited to supratentorial involvement and can primarily affect the spinal cord.

  15. Congenital Central Hypoventilation Syndrome

    Science.gov (United States)

    ... 12/2016 Symptoms Symptoms Listen The Human Phenotype Ontology (HPO) provides the following list of features that ... The in-depth resources contain medical and scientific language that may be hard to understand. You may ...

  16. Dental trauma clinically mimicking single central incisor syndrome = Traumatismo dentário mimetizando clinicamente a síndrome do incisivo central único

    OpenAIRE

    Moreira, Rafaela Nogueira

    2012-01-01

    Objetivo: Apresentar um caso clínico de ausência de um incisivo central perdido por traumatismo e encaminhado ao ortodontista com suspeita de apresentar síndrome do incisivo central único (SMMCI). Descrição do caso: Paciente gênero feminino, 12 anos com queixa da aparência de seus dentes. Ao exame intrabucal observou-se a presença de um incisivo central único localizado na linha média sem espaçamentos em relação aos incisivos laterais superiores. A paciente relatou dois episódios de avulsã...

  17. Flail arm–like syndrome associated with HIV-1 infection

    Science.gov (United States)

    Nalini, A.; Desai, Anita; Mahato, Simendra Kumar

    2009-01-01

    During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. In this report we describe the clinical picture of a young man with HIV-1 clade C infection and flail arm-like syndrome, who we were able to follow-up for a long period. We investigated and prospectively monitored a 34-year-old man with features of flail arm syndrome, who developed the weakness and wasting 1 year after being diagnosed with HIV-1 infection after a routine blood test. He presented in 2003 with progressive, symmetrical wasting and weakness of the proximal muscles of the upper limb of 2 years' duration. He had severe wasting and weakness of the shoulder and arm muscles. There were no pyramidal signs. He has been on HAART for the last 4 years and the weakness or wasting has not worsened. At the last follow-up in July 2007, the patient had the same neurological deficit and no other symptoms or signs of HIV-1 infection. MRI of the spinal cord in 2007 showed characteristic T2 hyperintense signals in the central part of the spinal cord, corresponding to the central gray matter. Thus, our patient had HIV-1 clade C infection associated with a ‘flail arm–like syndrome.’ The causal relationship between HIV-1 infection and amyotrophic lateral sclerosis (ALS)-like syndrome is still uncertain. The syndrome usually manifests as a lower motor neuron syndrome, as was seen in our young patient. It is known that treatment with antiretroviral therapy (ART) stabilizes/improves the condition. In our patient the weakness and atrophy remained stable over a period of 3.5 years after commencing HAART regimen. PMID:20142861

  18. Flail arm-like syndrome associated with HIV-1 infection

    Directory of Open Access Journals (Sweden)

    Nalini A

    2009-01-01

    Full Text Available During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. In this report we describe the clinical picture of a young man with HIV-1 clade C infection and flail arm-like syndrome, who we were able to follow-up for a long period. We investigated and prospectively monitored a 34-year-old man with features of flail arm syndrome, who developed the weakness and wasting 1 year after being diagnosed with HIV-1 infection after a routine blood test. He presented in 2003 with progressive, symmetrical wasting and weakness of the proximal muscles of the upper limb of 2 years′ duration. He had severe wasting and weakness of the shoulder and arm muscles. There were no pyramidal signs. He has been on HAART for the last 4 years and the weakness or wasting has not worsened. At the last follow-up in July 2007, the patient had the same neurological deficit and no other symptoms or signs of HIV-1 infection. MRI of the spinal cord in 2007 showed characteristic T2 hyperintense signals in the central part of the spinal cord, corresponding to the central gray matter. Thus, our patient had HIV-1 clade C infection associated with a ′flail arm-like syndrome.′ The causal relationship between HIV-1 infection and amyotrophic lateral sclerosis (ALS-like syndrome is still uncertain. The syndrome usually manifests as a lower motor neuron syndrome, as was seen in our young patient. It is known that treatment with antiretroviral therapy (ART stabilizes/improves the condition. In our patient the weakness and atrophy remained stable over a period of 3.5 years after commencing HAART regimen.

  19. Modeling spinal cord biomechanics

    Science.gov (United States)

    Luna, Carlos; Shah, Sameer; Cohen, Avis; Aranda-Espinoza, Helim

    2012-02-01

    Regeneration after spinal cord injury is a serious health issue and there is no treatment for ailing patients. To understand regeneration of the spinal cord we used a system where regeneration occurs naturally, such as the lamprey. In this work, we analyzed the stress response of the spinal cord to tensile loading and obtained the mechanical properties of the cord both in vitro and in vivo. Physiological measurements showed that the spinal cord is pre-stressed to a strain of 10%, and during sinusoidal swimming, there is a local strain of 5% concentrated evenly at the mid-body and caudal sections. We found that the mechanical properties are homogeneous along the body and independent of the meninges. The mechanical behavior of the spinal cord can be characterized by a non-linear viscoelastic model, described by a modulus of 20 KPa for strains up to 15% and a modulus of 0.5 MPa for strains above 15%, in agreement with experimental data. However, this model does not offer a full understanding of the behavior of the spinal cord fibers. Using polymer physics we developed a model that relates the stress response as a function of the number of fibers.

  20. Association of misoprostol, moebius syndrome and congenital central alveolar hypoventilation: case report Associação de misoprostol, síndrome de Moebius e hipoventilação central congênita: relato de caso

    Directory of Open Access Journals (Sweden)

    MAGDA LAHORGUE NUNES

    1999-03-01

    Full Text Available We report a case showing the association of Moebius syndrome, the use of misoprostol during pregnancy and the development of central congenital alveolar hypoventilation. Pathophysiological aspects of these three diseases are discussed and also the unfavorable prognosis of this association.Descrevemos o caso de um paciente com Síndrome de Moebius associada ao uso de misoprostol durante a gestação. A criança necessitou de suporte ventilatório desde o primeiro dia de vida e evoluiu com quadro de hipoventilação alveolar central congênita, persistindo dependente de ventilação mecânica. São discutidos aspectos fisiopatológicos que poderiam justificar a comorbidade destes três eventos, assim como o prognóstico reservado desta associação.

  1. Imbalance between thyroid hormones and the dopaminergic system might be central to the pathophysiology of restless legs syndrome: a hypothesis

    Directory of Open Access Journals (Sweden)

    Jose Carlos Pereira Jr.

    2010-01-01

    Full Text Available Data collected from medical literature indicate that dopaminergic agonists alleviate Restless Legs Syndrome symptoms while dopaminergic agonists antagonists aggravate them. Dopaminergic agonists is a physiological regulator of thyroid-stimulating hormone. Dopaminergic agonists infusion diminishes the levels of thyroid hormones, which have the ability to provoke restlessness, hyperkinetic states, tremors, and insomnia. Conditions associated with higher levels of thyroid hormones, such as pregnancy or hyperthyroidism, have a higher prevalence of Restless Legs Syndrome symptoms. Low iron levels can cause secondary Restless Legs Syndrome or aggravate symptoms of primary disease as well as diminish enzymatic activities that are involved in dopaminergic agonists production and the degradation of thyroid hormones. Moreover, as a result of low iron levels, dopaminergic agonists diminishes and thyroid hormones increase. Iron therapy improves Restless Legs Syndrome symptoms in iron deprived patients. Medical hypothesis. To discuss the theory that thyroid hormones, when not counterbalanced by dopaminergic agonists, may precipitate the signs and symptoms underpinning Restless Legs Syndrome. The main cause of Restless Legs Syndrome might be an imbalance between the dopaminergic agonists system and thyroid hormones.

  2. Imbalance between thyroid hormones and the dopaminergic system might be central to the pathophysiology of restless legs syndrome: a hypothesis.

    Science.gov (United States)

    Pereira, Jose Carlos; Pradella-Hallinan, Marcia; Lins Pessoa, Hugo de

    2010-05-01

    Data collected from medical literature indicate that dopaminergic agonists alleviate Restless Legs Syndrome symptoms while dopaminergic agonists antagonists aggravate them. Dopaminergic agonists is a physiological regulator of thyroid-stimulating hormone. Dopaminergic agonists infusion diminishes the levels of thyroid hormones, which have the ability to provoke restlessness, hyperkinetic states, tremors, and insomnia. Conditions associated with higher levels of thyroid hormones, such as pregnancy or hyperthyroidism, have a higher prevalence of Restless Legs Syndrome symptoms. Low iron levels can cause secondary Restless Legs Syndrome or aggravate symptoms of primary disease as well as diminish enzymatic activities that are involved in dopaminergic agonists production and the degradation of thyroid hormones. Moreover, as a result of low iron levels, dopaminergic agonists diminishes and thyroid hormones increase. Iron therapy improves Restless Legs Syndrome symptoms in iron deprived patients. Medical hypothesis. To discuss the theory that thyroid hormones, when not counterbalanced by dopaminergic agonists, may precipitate the signs and symptoms underpinning Restless Legs Syndrome. The main cause of Restless Legs Syndrome might be an imbalance between the dopaminergic agonists system and thyroid hormones. PMID:20535374

  3. Evaluation of spinal cord stimulation for the treatment of fail back surgery syndrome%脊髓电刺激治疗腰椎手术失败综合征的疗效分析

    Institute of Scientific and Technical Information of China (English)

    杨立强; 倪家骧

    2012-01-01

    Objective: To investigate the efficacy of spinal cord stimulation (SCS) for the treatment of fail back surgery syndrome (FBSS). Method: Thirty-one patients with FBSS were randomly assigned to A group and B group. A group was given conventional interventional management as continuous epidural infusion combined with collagenase injection, or in-tervertebral disc radiofrequency combined with ozone injection (n=16). B group was given epidural spinal cord stimulation treatment (n=15). Patients were asked to rate their pain with visual analogue scale (VAS) 1 week, 1 month,6 months and 1 year after treatment. Oswestry disability index (ODI), Japanese orthopedic association(JOA) low back pain score were also collected. Result: The VAS and ODI scores decreased significantly in both groups 1 week, 6 months and 1 year after treatment (P< 0.01). JOA scores improved also in both group at the same time. Compared with A group, patients in B group reported superior pain relief, ODI and JOA score improvements. Conclusion: SCS provided better clinical effect than conventional interventional management.%目的:探讨脊髓电刺激(SCS)治疗腰椎手术失败综合征(FBSS)的临床疗效评定.方法:选取FBSS患者31例,随机分为两组:A组进行连续硬膜外腔镇痛联合胶原酶注射或椎间盘射频联合臭氧注射等传统介入手术治疗组(16例).B组进行硬膜外腔SCS治疗组(15例).在治疗前、治疗后1周、1个月、6个月、12个月,分别采用视觉模拟评分法(VAS)、Oswestry功能障碍指数(ODI)、日本骨科学会(JOA)腰痛评估表进行疗效分析.结果:两组治疗后1周、1个月、6个月、12个月VAS值均较治疗前明显降低(P<0.01),ODI指数均较治疗前明显降低(P<0.01),JOA值均较治疗前明显提高(P<0.01).B组在治疗后12个月的VAS值、ODI指数、JOA值较A组明显改善(P<0.05).结论:脊髓电刺激(SCS)治疗腰椎术后失败综合征(FBSS)疗效优于传统的微创介入手术.

  4. Spinal cord compression in {beta}-thalassemia: follow-up after radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Fonseca, Silvana Fahel da; Figueiredo, Maria Stella; Cancado, Rodolfo Delfini; Nakadakare, Fernando; Segreto, Roberto; Kerbauy, Jose [Universidade Federal de Sao Paulo (UNIFESP), SP (Brazil). Escola Paulista de Medicina

    1998-12-01

    Spinal cord compression due to extramedullary hematopoiesis is a well-described bu rare syndrome encountered in several hematologic disorders, including {beta}-thalassemia. We report a case of a patient with intermediate {beta}-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms. (author)

  5. Effect of electroacupuncture on P2X3 receptor regulation in the peripheral and central nervous systems of rats with visceral pain caused by irritable bowel syndrome

    OpenAIRE

    Weng, Z. J.; L. Y. Wu; Zhou, C. L.; Dou, C. Z.; Shi, Y; H. R. Liu; Wu, H. G.

    2015-01-01

    The aim of this study is to investigate the role of the purinergic receptor P2X3 in the peripheral and central nervous systems during acupuncture treatment for the visceral pain of irritable bowel syndrome (IBS). A total of 24 8-day-old Sprague–Dawley (SD) neonatal male rats (SPF grade) were stimulated using colorectal distention (CRD) when the rats were awake. The modeling lasted for 2 weeks with one stimulation per day. After 6 weeks, the rats were randomly divided into three groups of eigh...

  6. Spinal cord abscess

    Science.gov (United States)

    ... abscess: Back injuries or trauma, including minor ones Boils on the skin, especially on the back or ... of spinal cord abscess. Prevention Thorough treatment of boils, tuberculosis, and other infections decreases the risk. Early ...

  7. Spinal Cord Injury 101

    Science.gov (United States)

    ... is "Braingate" research? What is the status of stem-cell research? How would stem-cell therapies work in the treatment of spinal cord injuries? What does stem-cell research on animals tell us? When can we expect ...

  8. Spinal Cord Infarction

    Science.gov (United States)

    ... treatments Functional and Dysfunctional Spinal Circuitry: Role for Rehabilitation and Neural Prostheses Summary of NINDS New Strategies in Spinal Cord Injury workshop held June, 2000. NINDS Workshop on Re- ...

  9. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... family FacingDisability is designed to provide Internet-based information and support for people with spinal cord injuries ... health care products or services, or control the information found on external websites. The Hill Foundation is ...

  10. Spinal Cord Injury Map

    Science.gov (United States)

    ... Videos Videos by Topic and Question Videos by Family Relationship Videos by Experts Resources The Short List Government ... Home Videos by Topic and Question Videos by Family Relationship Videos by Spinal Cord Experts Resources Forums Peer ...

  11. Cord-Blood Banking

    Science.gov (United States)

    ... cord blood mainly because of the promise that stem cell research holds for the future. Most of us would have little use for stem cells now, but research into using them to treat diseases is ongoing — ...

  12. Spinal cord trauma

    Science.gov (United States)

    ... Oh's Intensive Care Manual . 7th ed. Philadelphia, PA: Elsevier; 2014:chap 78. Bryce TN. Spinal cord injury. ... Physical Medicine and Rehabilitation . 5th ed. Philadelphia, PA: Elsevier; 2016:chap 49. Dalzell K, Nouri A, Fehlings ...

  13. Radiation nephritis causing nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Jennette, J.C.; Ordonez, N.G.

    1983-12-01

    Clinical symptoms of acute radiation nephritis with nephrotic syndrome developed in a fifty-six-year-old woman after abdominal radiation therapy for an astrocytoma of the spinal cord. The diagnosis of radiation nephritis was confirmed by renal biopsy. To our knowledge, this is the first documented case of radiation nephritis associated with nephrotic syndrome.

  14. Spinal cord injury as a trigger to develop periodic leg movements during sleep: an evolutionary perspective

    Directory of Open Access Journals (Sweden)

    Susana Cristina Lerosa Telles

    2012-11-01

    Full Text Available The primary trigger to periodic limb movement (PLM during sleep is still unknown. Its association with the restless legs syndrome (RLS is established in humans and was reported in spinal cord injury (SCI patients classified by the American Spinal Injury Association (ASIA as A. Its pathogenesis has not been completely unraveled, though recent advances might enhance our knowledge about those malfunctions. PLM association with central pattern generator (CPG is one of the possible pathologic mechanisms involved. This article reviewed the advances in PLM and RLS genetics, the evolution of CPG functioning, and the neurotransmitters involved in CPG, PLM and RLS. We have proposed that SCI might be a trigger to develop PLM.

  15. A Systematic Review of Peripheral and Central Nervous System Involvement of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Primary Sjögren’s Syndrome, and Associated Immunological Profiles

    Directory of Open Access Journals (Sweden)

    Anastasia Bougea

    2015-01-01

    Full Text Available Both central (CNS and peripheral nervous system (PNS complications are frequent and varied in connective tissue diseases. A systematic review was conducted between 1989 and 2014 in the databases Medline, Scopus, and Cochrane Library using the search terms, peripheral and central nervous complications and immunological profiles, to identify studies in specific connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome. A total of 675 references were identified, of which 118 were selected for detailed analysis and 22 were included in the final review with a total of 2338 participants. Our search focused only on studies upon connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome associated with seroimmunological data. The reported prevalence of CNS involvement ranges from 9 to 92% across the reported studies. However, the association between CNS and PNS manifestations and seroimmunological profiles remains controversial. Τo date, no laboratory test has been shown as pathognomonic neither for CNS nor for PNS involvement.

  16. Síndrome do incisivo central superior solitário: relato de caso Solitary median maxillary central incisor syndrome: case report

    OpenAIRE

    Eduardo Machado; Patricia Machado; Betina Grehs; Renésio Armindo Grehs

    2010-01-01

    INTRODUÇÃO: a presença de um incisivo central superior solitário é um evento bastante incomum na população. A prevalência da chamada Síndrome do Incisivo Central Superior Solitário (SICSS) é verificada em 1:50.000 nascimentos, sendo registrado um maior acometimento no sexo feminino. Essa alteração no desenvolvimento da oclusão dentária é caracterizada por más formações estruturais, sobretudo na região de linha média do paciente. O diagnóstico precoce e o tratamento adequado dessa síndrome são...

  17. Imbalance between thyroid hormones and the dopaminergic system might be central to the pathophysiology of restless legs syndrome: a hypothesis

    OpenAIRE

    Jose Carlos Pereira Jr.; Marcia Pradella-Hallinan; Hugo de Lins Pessoa

    2010-01-01

    Data collected from medical literature indicate that dopaminergic agonists alleviate Restless Legs Syndrome symptoms while dopaminergic agonists antagonists aggravate them. Dopaminergic agonists is a physiological regulator of thyroid-stimulating hormone. Dopaminergic agonists infusion diminishes the levels of thyroid hormones, which have the ability to provoke restlessness, hyperkinetic states, tremors, and insomnia. Conditions associated with higher levels of thyroid hormones, such as pregn...

  18. Phrenic nerve afferents elicited cord dorsum potential in the cat cervical spinal cord

    Directory of Open Access Journals (Sweden)

    Davenport Paul W

    2005-05-01

    Full Text Available Abstract Background The diaphragm has sensory innervation from mechanoreceptors with myelinated axons entering the spinal cord via the phrenic nerve that project to the thalamus and somatosensory cortex. It was hypothesized that phrenic nerve afferent (PnA projection to the central nervous system is via the spinal dorsal column pathway. Results A single N1 peak of the CDP was found in the C4 and C7 spinal segments. Three peaks (N1, N2, and N3 were found in the C5 and C6 segments. No CDP was recorded at C8 dorsal spinal cord surface in cats. Conclusion These results demonstrate PnA activation of neurons in the cervical spinal cord. Three populations of myelinated PnA (Group I, Group II, and Group III enter the cat's cervical spinal segments that supply the phrenic nerve

  19. Spinal cord stimulation with hybrid lead relieves pain in low back and legs

    NARCIS (Netherlands)

    Vos, de Cecile C.; Dijkstra, Cindy; Lenders, Mathieu W.P.M.; Holsheimer, Jan; Levy, R.M.

    2012-01-01

    Objective: The failed back surgery syndrome (FBSS) is the most common chronic pain syndrome. Whereas it is relatively easy to achieve pain relief in the lower limbs of FBSS patients with spinal cord stimulation (SCS), it is difficult to manage low back pain with SCS. The performance of a paddle-shap

  20. Effect of Microgene pSVPoMcat Implantation to Modify Genetically Schwann Cells on Central Conduction Function after Spinal Cord Injury%微基因修饰雪旺氏细胞移植对脊髓损伤中枢传导功能的作用

    Institute of Scientific and Technical Information of China (English)

    陈礼刚; 高立达; 周虎田; 曾凡俊; 毛伯镛; 李讯; 卢敏

    2001-01-01

    Objective To approach the effect of microgene pSVPoMcat to modify genetically Schwann cells (SC) on central conduction functionafter spinal cord injury (SCI) . Method Experimental animals were divided into three groups: the group of microgene pSVPoMcat implanted to genetically modify SC (group A), SC implanted group (group B), and the control group (group C). The cortical somatasensory evoked potentials (CESP) and combined behavioral score (CBS) were continuous monitored from operation second week to twelfth week Result The result showed that the peak latency and peak amplitudes of group A, B have a recovery tendency and it was constant with CBS. Conclusion We concluded that the microgene pSVPoMcat to modify genetically Sc may play a promotion role in recovery of central conduction function after SCI.

  1. [Spinal cord infarction].

    Science.gov (United States)

    Naumann, N; Shariat, K; Ulmer, S; Stippich, C; Ahlhelm, F J

    2012-05-01

    Infarction of the spinal cord can cause a variety of symptoms and neurological deficits because of the complex vascular supply of the myelon. The most common leading symptom is distal paresis ranging from paraparesis to tetraplegia caused by arterial ischemia or infarction of the myelon. Venous infarction, however, cannot always be distinguished from arterial infarction based on the symptoms alone.Modern imaging techniques, such as computed tomography angiography (CTA) and magnetic resonance angiography (MRA) assist in preoperative planning of aortic operations to reliably identify not only the most important vascular structure supplying the spinal cord, the artery of Adamkiewicz, but also other pathologies such as tumors or infectious disorders. In contrast to CT, MRI can reliably depict infarction of the spinal cord.

  2. Spinal cord swelling and candidiasis

    Energy Technology Data Exchange (ETDEWEB)

    Ho, K.; Gronseth, G.; Aldrich, M.; Williams, A.

    1982-11-01

    Fusiform swelling of the spinal cord was noted myelographically in a patient with Hodgkin's disease. Autopsy revealed that the swelling was caused by Candida infection of the spinal cord. It is suggested that fungal infection be included in the differential diagnosis of spinal cord swelling in the immunosuppressed cancer patient.

  3. Spinal cord swelling and candidiasis

    International Nuclear Information System (INIS)

    Fusiform swelling of the spinal cord was noted myelographically in a patient with Hodgkin's disease. Autopsy revealed that the swelling was cauused by Candida infection of the spinal cord. It is suggested that fungal infection be included in the differential diagnosis of spinal cord swelling in the immunsupporessed cancer patient. (orig.)

  4. Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: protean additions to the autoimmune central neuropathies.

    Science.gov (United States)

    Ali, Fatima; Rowley, Merrill; Jayakrishnan, Bindu; Teuber, Suzanne; Gershwin, M Eric; Mackay, Ian R

    2011-09-01

    Stiff Person Syndrome (SPS) is a rare autoimmune neurological disease attributable to autoantibodies to glutamic acid decarboxylase (anti-GAD) more usually associated with the islet beta cell destruction of autoimmune type 1 diabetes (T1D). SPS is characterized by interference in neurons with the synthesis/activity of the inhibitory neurotransmitter gamma amino butyric acid (GABA) resulting in the prototypic progressive spasmodic muscular rigidity of SPS, or diverse neurological syndromes, cerebellar ataxia, intractable epilepsy, myoclonus and several others. Remarkably, a single autoantibody, anti-GAD, can be common to widely different disease expressions, i.e. T1D and SPS. One explanation for these data is the differences in epitope engagement between the anti-GAD reactivity in SPS and T1D: in both diseases, anti-GAD antibody reactivity is predominantly to a conformational epitope region in the PLP- and C-terminal domains of the 65 kDa isoform but, additionally in SPS, there is reactivity to conformational epitope(s) on GAD67, and short linear epitopes in the C-terminal region and at the N-terminus of GAD65. Another explanation for disease expressions in SPS includes ready access of anti-GAD to antigen sites due to immune responsiveness within the CNS itself according to intrathecal anti-GAD-specific B cells and autoantibody. Closer study of the mysterious stiff-person syndrome should enhance the understanding of this disease itself, and autoimmunity in general. PMID:21680149

  5. Metabolic Syndrome in Italian Obese Children and Adolescents: Stronger Association with Central Fat Depot than with Insulin Sensitivity and Birth Weight

    Directory of Open Access Journals (Sweden)

    Claudia Brufani

    2011-01-01

    Full Text Available Aim. To evaluate whether body fat distribution, birth weight, and family history for diabetes (FHD were associated with metabolic syndrome (MetS in children and adolescents. Methods. A total of 439 Italian obese children and adolescents (5–18 years were enrolled. Subjects were divided into 2 groups: prepubertal and pubertal. MetS was diagnosed according to the adapted National Cholesterol Education Program criteria. Birth weight percentile, central obesity index (measured by dual-energy X-ray absorptiometry, insulin sensitivity (ISI, and disposition index were evaluated. Multivariate logistic regression models were used to determine variables associated with MetS. Results. The prevalence of MetS was 17%, with higher percentage in adolescents than in children (21 versus 12%. In the overall population, central obesity index was a stronger predictor of MetS than insulin sensitivity and low birth weight. When the two groups were considered, central fat depot remained the strongest predictor of MetS, with ISI similarly influencing the probability of MetS in the two groups and birth weight being negatively associated to MetS only in pubertal individuals. Neither FHD nor degree of fatness was a significant predictor of MetS. Conclusion. Simple clinical parameters like increased abdominal adiposity and low birth weight could be useful tools to identify European obese adolescents at risk for metabolic complications.

  6. Trombose de veia central da retina bilateral associada à síndrome de hiperviscosidade sanguínea: relato de caso Bilateral central retinal vein occlusion associated with blood hyperviscosity syndrome: case report

    Directory of Open Access Journals (Sweden)

    John Helal Jr

    2005-02-01

    Full Text Available Relato de caso de um paciente masculino de 16 anos de idade com queixa inicial de baixa da acuidade visual e que no exame oftalmológico foi encontrado edema de papila bilateral, que evoluiu para trombose da veia central da retina em ambos os olhos. Na investigação laboratorial, foi feito diagnóstico de um mieloma múltiplo tipo IgA que cursava com síndrome de hiperviscosidade sanguínea, o que explicava o quadro oftalmológico. Após tratamento específico, o paciente apresentou melhora tanto da acuidade visual quanto do aspecto fundoscópico. O achado de oclusão de veia central da retina bilateral pode levar ao diagnóstico de importantes doenças sistêmicas. os achados fundoscópicos podem servir de parâmetro na avaliação do tratamento.The authors report the case of a 16-year-old male patient who presented with blurred vision and bilateral optic disc edema, then developing bilateral central retinal vein occlusion. On laboratory work-up, he was found to have multiple myeloma IgA along with hyperviscosity syndrome, which led to the ophthalmological features. After proper treatment, the patient recovered visual acuity and normalized his eye fundus changes. Bilateral central retinal vein occlusion finding may yield the diagnosis of major systemic diseases. Fundoscopic features may serve as parameters on treatment evaluation.

  7. 终丝脂肪瘤型脊髓栓系综合征的诊断及治疗%Management and diagnosis of tethered cord syndrome with filum terminale lipoma

    Institute of Scientific and Technical Information of China (English)

    刘福云; 于洋; 牛学强; 李新伟; 贺盼盼

    2014-01-01

    目的:了解终丝脂肪瘤型脊髓栓系综合征(TCS)患儿的临床表现和诊治特点。方法回顾性分析61例(7个月~14岁)终丝脂肪瘤型 TCS 患儿的临床资料,术前均行放射影像学、尿流动力学、肌电图检查,手术方式采用脂肪瘤切除,终丝离断加椎板成形术治疗。术后复查 MRI 及尿流动力学,比较患儿手术前后脊髓受牵拉情况及尿流动力学等各指标的变化。结果术前 MRI 显示终丝处有不同程度的脂肪信号,其中52例 TCS 患儿脊髓圆锥末端位置在第2腰椎椎体下缘以下,9例脊髓圆锥末端位置正常;术后显示圆锥位置无明显变化,脂肪瘤移除、终丝紧张解除。尿流动力学结果显示术前37例患儿存在逼尿肌过度活动,19例逼尿肌收缩乏力,5例逼尿肌部分收缩功能受损伴膀胱残余尿量增多;术后49例患儿取得较好疗效,余12例下肢情况得到改善,但尿流动力学表现改善不明显。结论终丝脂肪瘤型脊髓栓系综合征起病隐匿,诊断需综合考虑其临床表现、放射影像学、肌电图及尿流动力学检查结果,而 MRI 及尿流动力学检查是诊断的关键指标。%Objective To investigate the clinical characteristics,diagnosis and management of tethered cord syndrome(TCS)with filum terminale lipomain children. Methods The clinical data of 61 children(7 months - 14 years old)with filum terminale lipoma were analyzed retrospectively. The auxil-iary examinations including radiography examination,electromyogram examination and urodynamictest. Every child was surgically treated by transecting the filumterminalelipoma and laminoplasty MRI and uro-dynamic index were reviewed after operation,and the spinal cord tethering condition by MRI scaning and the parameters of urodynamic study before operation and after operation were compared. Results Before operation,MRI showed fat signal in the filum,and the conusmedullaris was found at

  8. Clinical Analysis of Talipes Equinovarus Caused by Tethered Cord Syndrome in 64 Children%脊髓栓系综合征后遗马蹄内翻足64例

    Institute of Scientific and Technical Information of China (English)

    刘福云; 王志岭; 卜建文; 李剑峰; 骆晓飞

    2012-01-01

    目的 分析儿童脊髓柃系综合征(TCS)后遗马蹄内翻足的临床特点,并对其疗效进行评估.方法 对2005年5月-2010年12月手术治疗的64例TCS后遗马蹄内翻足畸形患儿的病例资料进行回顾性研究,从发病情况、临床表现、并发症、手术方式等几个方面进行分析,并根据国际马蹄内翻足畸形研究学组(ICFSG)的评分标准对其进行疗效评估.结果 单侧足受累35例(左侧13例,右侧22例),双侧足受累29例.原发型TCS 53例,继发型TCS 11例.并发症:感觉障碍者26例,大小便功能障碍者23例(部分丧失18例,失禁5例),并发育性髋关节脱位和肌营养不良者1例,小脑扁桃体疝2例,脊柱侧弯10例,肋骨发育畸形3例和脊髓空洞22例.疗效:优10例16足,良21例36足,可25例31足,差8例10足.本组病例中,11例既往在多家医院未能明确发病原因,误诊或者漏诊率为17.2%(11/64例).结论 儿童TCS继发足畸形以马蹄内翻足居多.临床误诊漏诊现象较普遍,应提高对该病的认识,明确病因及对因治疗至关重要.%Objective To analyze the clinical features of children with talipes equinovarus caused by tethered cord syndrome(TCS) and assess their effects. Methods The clinical data of 64 children with talipes equinovarus caused by TCS who suffered surgery were researched retrospectively from May 2005 to Dec. 2010, whose incidence, clinical manifestations, complications and operative methods were analyzed, and effects were also evaluated in accordance with international clubfoot studies group( ICFSG). Results Unilateral foot deformity in 35 cases (left in 13 cases, right in 22 cases), bilateral foot deformity in 29 cases. Primary TCS in 53 cases, secondary TCS in 11 cases. Complications; 26 cases of sensory disturbance, bowel and bladder dysfunction in 23 cases (partial loss in 18 cases, incontinence in 5 cases) , developmental dislocation of the hip and muscular dystrophy in 1 case, tonsillar herniation in 2

  9. Infantile Hemophagocytic Lymphohistiocytosis in a Case of Chediak-Higashi Syndrome Caused by a Mutation in the LYST/CHS1 Gene Presenting With Delayed Umbilical Cord Detachment and Diarrhea

    DEFF Research Database (Denmark)

    Nielsen, Christian; Agergaard, Charlotte N; Jakobsen, Marianne A;

    2015-01-01

    A 2-month-old female infant, born to consanguineous parents, presented with infections in skin and upper respiratory tract. She was notable for delayed umbilical cord detachment, partial albinism, and neurological irritability. Giant granules were present in white blood cells. The intracellular p...

  10. Sleep apnea syndrome: central sleep apnea and pulmonary hypertension worsened during treatment with auto-CPAP, but improved by adaptive servo-ventilation.

    Science.gov (United States)

    Ono, Hiroshi; Fujimoto, Hiroyuki; Kobayashi, Yoshinori; Kudoh, Shoji; Gemma, Akihiko

    2010-01-01

    In this 71-year-old man diagnosed as obstructive sleep apnea syndrome initially, the apnea-hypopnea index in polysomnography was 31.3/hour. He started auto-adjusted continuous positive airway pressure (auto-CPAP) treatment in July 2005 but developed congestive heart failure in December 2007. Pulmonary arterial pressure (PAP), estimated by echocardiography, was 71 mmHg. In January 2008, during simplified sleep examination with a breath-movement sensor under auto-CPAP, many central-type apneas were recognized. After replacing auto-CPAP with adaptive servo-ventilation (ASV), the apnea-hypopnea index was 5.3/hour and PAP became 36 mmHg after 3 months. It was thought that the increase of PAP was due to long-term inadequate use of auto-CPAP.

  11. Unified-planning, graded-administration, and centralized-controlling: a management modality for treating acquired immune deficiency syndrome with Chinese medicine in Henan Province of China.

    Science.gov (United States)

    Xu, Li-Ran; Guo, Hui-jun; Liu, Zhi-bin; Li, Qiang; Yang, Ji-ping; He, Ying

    2015-04-01

    Henan Province in China has a major epidemic of human immunodeficiency virus/acquired immune deficiency syndrome (HIV/AIDS). Chinese medicine (CM) has been used throughout the last decade, and a management modality was developed, which can be described by unified-planning, graded-administration, and centralized-controlling (UGC). The UGC modality has one primary concept (patient-centered medicine from CM theory), four basic foundations (classifying administrative region, characteristics of CM on disease treatment, health resource conditions, and distribution of patients living with HIV), six important relationships (the "three uniformities and three combinations," and the six relationships therein guide the treatment of AIDS with CM), and four key sections (management, operation, records, and evaluation). In this article, the authors introduce the UGC modality, which could be beneficial to developing countries or resource-limited areas for the management of chronic infectious disease. PMID:25877652

  12. Unified-planning, graded-administration, and centralized-controlling: a management modality for treating acquired immune deficiency syndrome with Chinese medicine in Henan Province of China.

    Science.gov (United States)

    Xu, Li-Ran; Guo, Hui-jun; Liu, Zhi-bin; Li, Qiang; Yang, Ji-ping; He, Ying

    2015-04-01

    Henan Province in China has a major epidemic of human immunodeficiency virus/acquired immune deficiency syndrome (HIV/AIDS). Chinese medicine (CM) has been used throughout the last decade, and a management modality was developed, which can be described by unified-planning, graded-administration, and centralized-controlling (UGC). The UGC modality has one primary concept (patient-centered medicine from CM theory), four basic foundations (classifying administrative region, characteristics of CM on disease treatment, health resource conditions, and distribution of patients living with HIV), six important relationships (the "three uniformities and three combinations," and the six relationships therein guide the treatment of AIDS with CM), and four key sections (management, operation, records, and evaluation). In this article, the authors introduce the UGC modality, which could be beneficial to developing countries or resource-limited areas for the management of chronic infectious disease.

  13. Immune reconstitution inflammatory syndrome involving the central nervous system in a patient with HIV infection: a case report and review of literature.

    Science.gov (United States)

    Zaffiri, Lorenzo; Verma, Rajanshu; Struzzieri, Kevin; Monterroso, Joanne; Batts, Donald H; Loehrke, Mark E

    2013-01-01

    IRIS is described as a paradoxical deterioration of clinical status upon initiation of combined anti-retroviral therapy (cART) in patients with HIV infection. Immune reconstitution inflammatory syndrome (CNS-IRIS) involving the central nervous system is rarely reported. We describe the case of 57-year-old man who developed a fatal case of CNS- IRIS. A rapid deterioration of neurological status was associated with progression of patchy T2-weighted hyperintensities involving different vascular territories on brain MRI. Diagnosis of CNS-IRIS is based of laboratory and radiologic findings, however brain biopsy is supportive. Despite immune restoration being involved in clinical deterioration, discontinuation of cART is not recommended. The use of corticosteroids is highly controversial. Prompt recognition of CNS-IRIS is crucial for preventing neurological complications and ensuing sequelae. PMID:23435821

  14. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Braingate" research? What is the status of stem-cell research? How would stem-cell therapies work in the treatment of spinal cord injuries? What does stem-cell research on animals tell us? When can we ...

  15. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Home Videos by Topic and Question Videos by Family Relationship Videos by Spinal Cord Experts Resources Forums Peer Counseling Blog About Us Contact Donate Sitemap Privacy ... © 2011 – 2016 Hill Foundation for Families Living With Disabilities FacingDisability.com is an informational ...

  16. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... Home Videos by Topic and Question Videos by Family Relationship Videos by Spinal Cord Experts Resources Forums Peer Counseling Blog About Us Contact Donate Sitemap Privacy Statement Terms of Use © 2011 – 2016 Hill Foundation for Families Living With Disabilities FacingDisability.com is an informational ...

  17. Cord blood testing

    Science.gov (United States)

    ... to evaluate the oxygen, carbon dioxide, and pH levels) Blood sugar level Blood type and Rh Complete blood count ( ... means you have a blood infection (septicemia). High levels of blood sugar (glucose) in the cord blood may be found ...

  18. Spinal Cord Injury

    Science.gov (United States)

    ... How much do you know about taking good care of yourself? Links to more information girlshealth glossary girlshealth.gov home http://www.girlshealth.gov/ Home Illness & disability Types of ... Spinal cord injury Read advice from Dr. Jeffrey Rabin , a pediatric rehabilitation specialist at the Children’s National Medical Center. ...

  19. Spinal Cord Injury 101

    Medline Plus

    Full Text Available ... is "Braingate" research? What is the status of stem-cell research? How would stem-cell therapies work in the treatment of spinal cord injuries? What does stem-cell research on animals tell us? When can we ...

  20. Segmental hypersensitivity and spinothalamic function in spinal cord injury pain

    DEFF Research Database (Denmark)

    Finnerup, Nanna Brix; Sørensen, Leif Hougaard; Biering-Sørensen, Fin;

    2007-01-01

    The mechanisms underlying central pain following spinal cord injury (SCI) are unsettled. The purpose of the present study was to examine differences in spinothalamic tract function below injury level and evoked pain in incomplete SCI patients with neuropathic pain below injury level (central pain...... in central SCI pain and furthermore - in contrast to previous findings - that loss of spinothalamic functions does not appear to be a predictor for central neuropathic pain in spinal cord injury....... and thermal detection thresholds below injury level. SCI patients with central pain had sensory hypersensitivity in dermatomes corresponding to the lesion level more frequently than SCI patients without pain, but this may in part be explained by the exclusion of at-level spontaneous pain in the pain...

  1. Central nervous determination of food storage--a daily switch from conservation to expenditure: implications for the metabolic syndrome.

    Science.gov (United States)

    Kreier, Felix; Kalsbeek, Andries; Ruiter, Marieke; Yilmaz, Ajda; Romijn, Johannes A; Sauerwein, Hans P; Fliers, Eric; Buijs, Ruud M

    2003-11-01

    Here, we present a neuroendocrine concept to review the circularly interacting energy homeostasis system between brain and body. Body-brain interaction is circular because the brain immediately integrates an input to an output, and because part of this response may be that the brain modulates the sensitivity of this perception. First, we describe how the brain senses the body through neurons and blood-borne factors. Direct neuronal connections report the state of various organs. In addition, humoral factors are perceived by the blood-brain barrier and circumventricular organs. We describe how circulating energy carriers are sensed and what signals reach the brain during food intake, exercise and an immune response. We describe that the brain regulates the homeostatic process at two fundamentally different levels during the active and inactive states. The unbalanced output of the brain in the metabolic syndrome is discussed in relation with such circadian rhythms and with regional activity of the autonomic nervous system. In line with the above, we suggest a new approach for the diagnosis and therapy of the metabolic syndrome.

  2. Moderate voluntary exercise attenuates the metabolic syndrome in melanocortin-4 receptor-deficient rats showing central dopaminergic dysregulation

    Directory of Open Access Journals (Sweden)

    Silvana Obici

    2015-10-01

    Conclusions: Central dopamine dysregulation during VWR reinforces the link between MC4R function and molecular and behavioral responding to rewards. The data also suggest that exercise can be a successful lifestyle intervention in MC4R-haploinsufficient individuals despite reduced positive reinforcement during exercise training.

  3. Cord blood transplantation in Japan.

    Science.gov (United States)

    Uchida, Naoyuki

    2016-05-01

    Cord blood transplantation (CBT) has increasingly been used in Japan and the annual number of CBT now exceeds 1,200. The cumulative number of CBT reached 12,853 in 2015, accounting for almost 1/3 of total CBT performed worldwide. It is true that smaller body size and lower costs, as compared to western countries, have been advantages for Japanese people in using CB as graft alternative. In addition, several novel findings regarding serious issues following CBT have been obtained, which further enhanced the use of CB. First, several mechanisms of engraftment failure following CBT other than cell dose have been reported, such as the presence of donor-specific anti-HLA antibodies or the development of hemophagocytic syndrome. Second, unique profiles of infectious complications following CBT have been reported, such as higher incidences of early bacterial infections and HHV-6 encephalitis, as compared to those following bone marrow (BM)/peripheral blood (PB) transplants. Third, the incidence of disease relapse was comparable to those following BM/PB transplants. Novel pre-transplant conditioning regimens using intravenous busulfan have been investigated with promising results being obtained to date. A recent analysis of Japanese transplant registry data revealed similar survival following CBT to HLA-matched unrelated BM/PB transplants. PMID:27263776

  4. Pain in Down's Syndrome

    Directory of Open Access Journals (Sweden)

    Federica Mafrica

    2006-01-01

    Full Text Available Pain is a homeostatic mechanism that intervenes to protect the organism from harmful stimuli that could damage its integrity. It is made up of two components: the sensory-discriminative component, which identifies the provenance and characteristics of the type of pain; and the affective-motivational component, on which emotional reflexes, following the painful sensation, depend.There is a system for pain control at an encephalic and spinal level, principally made up of the periaqueductal grey matter, the periventricular area, the nucleus raphe magnus, and the pain-inhibition complex situated in the posterior horns of the spinal cord. Through the activation of these pain-control systems, the nervous system suppresses the afference of pain signals. Endogenous opioids represent another analgesic system.In the course of various studies on pain transmission in Down patients, the reduced tolerance of pain and the incapacity to give a qualitative and quantitative description emerged in a powerful way. All of these aspects cause difficulty in evaluating pain. This is linked to several learning difficulties. However, it cannot be excluded that in these anomalies of pain perception, both the anatomical and the neurotransmitter alteration, typical of this syndrome, may hold a certain importance.This fact may have important clinical repercussions that could affect the choice of therapeutic and rehabilitative schemes for treatment of pathologies in which pain is the dominant symptom, such as postoperative pain. It could influence research on analgesics that are more suitable for these patients, the evaluation of the depth of analgesia during surgical operation, and ultimately, absence of obvious pain manifestations. In conclusion, alterations of the central nervous system, neurotransmitters, pain transmission, and all related problems should be considered in the management of pain in patients with Down's syndrome, especially by algologists and

  5. Central leptin insufficiency syndrome: an interactive etiology for obesity, metabolic and neural diseases and for designing new therapeutic interventions

    OpenAIRE

    Kalra, Satya P.

    2007-01-01

    This review critically reappraises recent scientific evidence concerning central leptin insufficiency versus leptin resistance formulations to explain metabolic and neural disorders resulting from subnormal or defective leptin signaling in various sites in the brain. Research at various fronts to unravel the complexities of the neurobiology of leptin is surveyed to provide a comprehensive account of the neural and metabolic effects of environmentally-imposed fluctuations in leptin availabilit...

  6. A rare case report of cord entanglement in a post-dated monochorionic monoamniotic twins

    Directory of Open Access Journals (Sweden)

    Kasturi Donimath

    2016-01-01

    Full Text Available Monochorionic Monoamniotic (MCMA twin gestations have been associated perinatal mortality rates as high as 28 to 47%. Umbilical cord entanglements and knots, twin-to-twin transfusion syndrome, congenital anomalies, prematurity and intertwin locking during labour is responsible for their high perinatal morbidity and mortality. We report here a case of Cord entanglement in a post-dated MCMA twin pregnancy without any complications. [Int J Reprod Contracept Obstet Gynecol 2016; 5(1.000: 230-231

  7. Astrocytoma in an African hedgehog (Atelerix albiventris) suspected wobbly hedgehog syndrome.

    Science.gov (United States)

    Nakata, Makoto; Miwa, Yasutsugu; Itou, Takuya; Uchida, Kazuyuki; Nakayama, Hiroyuki; Sakai, Takeo

    2011-10-01

    A 28-month-old African hedgehog was referred to our hospital with progressive tetraparesis. On the first presentation, the hedgehog was suspected as having wobbly hedgehog syndrome (WHS) and the animal was treated with medication and rehabilitation. The animal died 22 days after onset. Pathological examination revealed that the animal was involved in astrocytoma between the medulla oblongata and the spinal cord (C1). This report indicates that a primary central nervous system tumor should be considered as one of the differential diagnoses for hedgehogs presenting with progressive paresis, together with WHS. PMID:21628867

  8. The acute radiation syndrome

    International Nuclear Information System (INIS)

    Symptoms and signs from medical aspects resulting from whole body exposure, or in the main part, to ionizing radiation are described. The dose-response relationship is studied and the exposure is divided in three parts: central nervous system syndrome, gastrointestinal syndrome and hematopoietic syndrome. Brief comments about the treatment are reported. (M.A.C.)

  9. MRI and clinical symptoms in chronic cervical cord injury

    Energy Technology Data Exchange (ETDEWEB)

    Soeda, Shuichi; Maruiwa, Hirofumi; Yokoi, Masahiro; Saitoh, Seiya (Tsukigase Rehabilitation Center, Shizuoka (Japan)); Yamauchi, Kenji

    1992-08-01

    To assess the ability of magnetic resonance (MR) imaging to determine the prognosis of spinal cord injury in the chronic stage and to detect the injured myelomere, 39 patients were examined with MR images obtained by T1-weighted spin echo method 5 months to 4 years and 8 months (mean, one year and 5 months) after they had sustained spinal cord injury. According to hypointensity area of the ventrodorsad diameter of the spinal cord, MR images were classified as non-hypointensity (I), discrete (II), central (III), large cavity (IV), and transverse (V). The most common type was III (25%), followed by IV (26%), II (18%), V (15%), and I (13%). In 21 patients with bone injury, 14 (67%) had type IV or V, in contrast to 2 (11%) of 18 patients without bone injury. Increased hypointensity on MR images was associated with severer injury of the spinal cord. When hypointensity accounted for less than 1/2 of the ventrodorsad diameter of the spinal cord, walking ability was recovered in more than 80% of the patients. When less than 1/3 of the ventrodorsad diameter of the spinal cord was seen as hypointensity, arm function was well preserved, and the anterior horn of gray matter was found less injured. In 60% of the patients, there was difference in the injured level of myelomere between MR images and the neurological examination; the injured level of myelomere tended to be more cephalad level in the neurological examination than MR appearance.(N.K.).

  10. MRI and clinical symptoms in chronic cervical cord injury

    International Nuclear Information System (INIS)

    To assess the ability of magnetic resonance (MR) imaging to determine the prognosis of spinal cord injury in the chronic stage and to detect the injured myelomere, 39 patients were examined with MR images obtained by T1-weighted spin echo method 5 months to 4 years and 8 months (mean, one year and 5 months) after they had sustained spinal cord injury. According to hypointensity area of the ventrodorsad diameter of the spinal cord, MR images were classified as non-hypointensity (I), discrete (II), central (III), large cavity (IV), and transverse (V). The most common type was III (25%), followed by IV (26%), II (18%), V (15%), and I (13%). In 21 patients with bone injury, 14 (67%) had type IV or V, in contrast to 2 (11%) of 18 patients without bone injury. Increased hypointensity on MR images was associated with severer injury of the spinal cord. When hypointensity accounted for less than 1/2 of the ventrodorsad diameter of the spinal cord, walking ability was recovered in more than 80% of the patients. When less than 1/3 of the ventrodorsad diameter of the spinal cord was seen as hypointensity, arm function was well preserved, and the anterior horn of gray matter was found less injured. In 60% of the patients, there was difference in the injured level of myelomere between MR images and the neurological examination; the injured level of myelomere tended to be more cephalad level in the neurological examination than MR appearance.(N.K.)

  11. Akhirin regulates the proliferation and differentiation of neural stem cells in intact and injured mouse spinal cord.

    Science.gov (United States)

    Abdulhaleem, Felemban Athary M; Song, Xiaohong; Kawano, Rie; Uezono, Naohiro; Ito, Ayako; Ahmed, Giasuddin; Hossain, Mahmud; Nakashima, Kinichi; Tanaka, Hideaki; Ohta, Kunimasa

    2015-05-01

    Although the central nervous system is considered a comparatively static tissue with limited cell turnover, cells with stem cell properties have been isolated from most neural tissues. The spinal cord ependymal cells show neural stem cell potential in vitro and in vivo in injured spinal cord. However, very little is known regarding the ependymal niche in the mouse spinal cord. We previously reported that a secreted factor, chick Akhirin, is expressed in the ciliary marginal zone of the eye, where it works as a heterophilic cell-adhesion molecule. Here, we describe a new crucial function for mouse Akhirin (M-AKH) in regulating the proliferation and differentiation of progenitors in the mouse spinal cord. During embryonic spinal cord development, M-AKH is transiently expressed in the central canal ependymal cells, which possess latent neural stem cell properties. Targeted inactivation of the AKH gene in mice causes a reduction in the size of the spinal cord and decreases BrdU incorporation in the spinal cord. Remarkably, the expression patterns of ependymal niche molecules in AKH knockout (AKH-/-) mice are different from those of AKH+/+, both in vitro and in vivo. Furthermore, we provide evidence that AKH expression in the central canal is rapidly upregulated in the injured spinal cord. Taken together, these results indicate that M-AKH plays a crucial role in mouse spinal cord formation by regulating the ependymal niche in the central canal.

  12. Alterations of Spinal Cord in Japanese B Encephalitis

    OpenAIRE

    Kishikawa, Masao

    1993-01-01

    The cytopathologic changes of Japanese B encephalitis (JBE) are basically similar to those of other forms of arbovirus encephalitis. Because the entire central nervous system including the spinal cord is involved to varying degrees, the nomenclature of JBE should actually be Japanese B panencephalomyelitis.

  13. Sleep apnea syndrome after irradiation of the neck

    Energy Technology Data Exchange (ETDEWEB)

    Herlihy, J.P.; Whitlock, W.L.; Dietrich, R.A.; Shaw, T. (Pulmonary Disease Service, Presidio of San Francisco, CA (USA))

    1989-12-01

    After irradiation of the neck for a squamous cell carcinoma of the tonsillar pillar and vocal cord, a 71-year-old man presented with a rapidly progressive sleep apnea syndrome. Previous reports describe the condition of patients with obstructive sleep apnea that developed after neck irradiation and secondary to supraglottic edema. Our patient had an obstructive component to his apnea similar to that described in previous cases, but, in addition, he had hypothyroidism. Myxedema is a well-described cause of both obstructive and central apnea. We believe both contributed to his condition. He was successfully treated by placement of a tracheostomy and by thyroid supplementation. In patients who present with sleep apnea after neck irradiation, especially with acute or severe symptoms, the differential diagnosis should include both a central cause from hypothyroidism as well as a peripheral obstructive cause from laryngeal edema.

  14. Sleep apnea syndrome after irradiation of the neck

    International Nuclear Information System (INIS)

    After irradiation of the neck for a squamous cell carcinoma of the tonsillar pillar and vocal cord, a 71-year-old man presented with a rapidly progressive sleep apnea syndrome. Previous reports describe the condition of patients with obstructive sleep apnea that developed after neck irradiation and secondary to supraglottic edema. Our patient had an obstructive component to his apnea similar to that described in previous cases, but, in addition, he had hypothyroidism. Myxedema is a well-described cause of both obstructive and central apnea. We believe both contributed to his condition. He was successfully treated by placement of a tracheostomy and by thyroid supplementation. In patients who present with sleep apnea after neck irradiation, especially with acute or severe symptoms, the differential diagnosis should include both a central cause from hypothyroidism as well as a peripheral obstructive cause from laryngeal edema

  15. Complications of spinal cord injury

    OpenAIRE

    Dursun, Erbil; Hamamci, Nigar; Ozbey, Aydan; Cakci, Aytul

    2004-01-01

    Spinal cord injury and its complications cause important physical, psychosocial and economical problems. The purpose of this study was to evaluate the complications resulting from spinal cord injury, to show their adverse effects on the rehabilitation program, and to make related clinicians to call attention especially to preventable complications. Sixty-two spinal cord injured patients were included in the study. All the patients were evaluated regarding age, gender, etiology, time since inj...

  16. [Pathology of the internal organs and central nervous system in acquired immunodeficiency syndrome (with special reference to opportunistic infections)].

    Science.gov (United States)

    Masini, T; Chinaglia, D; Riviera, L; Capricci, E; Gullotta, F; Spigolon, G; Bauer, A L

    1990-01-01

    Extracerebral and cerebral pathology in AIDS (with particular emphasis on the opportunistic infections). The Authors present the extracerebral pathology of 27 cases of AIDS observed at the Department of Pathology of Milan and the cerebral pathology of 80 cases of AIDS collected by three Institutes (Department of Pathology of Milan, Department of Pathology of Rimini and Department of Neuropathology of Münster) with particular emphasis on the pathology of the opportunistic infections. In the adults' group, the most frequent infections are the protozoan ones (T. gondii) followed with equal incidence by the viral and fungal diseases. In the pediatric group the viral diseases are the most frequently seen. Almost all of the adults show multiple infections in the same organ or in different organs. Diffuse lesions with heavy pathologic fields were observed also without tissue reaction. As to cerebral pathology AIDS' patients with opportunistic infections show focal symptoms, whereas the so called "subacute microglial encephalitis" generally appears as a demential syndrome. In cases with progressive multifocal leukoencephalopathy JC virus was always found and in one case also SV 40 - and BK virus. The diffuse demyelinization in some cases of HIV-Encephalopathy is aspecific. In HIV-positive newborns with cerebral signs, the lesions are characterized by oedema, spongiosis and microcalcifications of the basal ganglia; these are aspecific lesions which can be found in toxic and infectious encephalopathies.

  17. Umbilical cord blood mercury levels in China

    Institute of Scientific and Technical Information of China (English)

    Meiqin Wu,; Chonghuai Yan; Jian Xu; Wei Wu; Hui Li; Xin Zhou

    2013-01-01

    Mercury (Hg) is a well-known neurotoxicant.Hg exposure at high levels can harm individuals of all ages.Even low level exposure to Hg can damage the brain of fetuses and young children,and affect their central nervous system and cognitive development.The aims of our study were to measure total Hg levels in infant umbilical cord blood and to investigate the risk factors associated with total Hg cord blood levels in various cities in China.Our goal was to provide clues for the prevention of Hg exposure in utero.The results indicated that the average cord blood mercury levels (CBMLs) were (1.81 ± 1.93) μg/L,which were lower than those found in most previous studies.The concentrations also differed according to geographic region.The CBMLs were not only associated with family economic and living conditions,but also with diet in pregnant women,especially the intake of marine fish,shellfish,poultry,formula milk and fruits.

  18. Long-lasting beneficial effects of central serotonin receptor 7 stimulation in female mice modeling Rett syndrome

    Directory of Open Access Journals (Sweden)

    Bianca eDe Filippis

    2015-04-01

    Full Text Available Rett syndrome (RTT is a rare neurodevelopmental disorder, characterized by severe behavioral and physiological symptoms. Mutations in the methyl CpG binding protein 2 gene (MECP2 cause more than 95% of classic cases, and currently there is no cure for this devastating disorder. Recently we have demonstrated that specific behavioral and brain molecular alterations can be rescued in MeCP2-308 male mice, a RTT mouse model, by pharmacological stimulation of the brain serotonin receptor 7 (5-HT7R. This member of the serotonin receptor family – crucially involved in the regulation of brain structural plasticity and cognitive processes – can be stimulated by systemic repeated treatment with LP-211, a brain-penetrant selective 5-HT7R agonist. The present study extends previous findings by demonstrating that the LP-211 treatment (0.25 mg/kg, once per day for 7 days rescues RTT-related phenotypic alterations, motor coordination (Dowel test, spatial reference memory (Barnes maze test and synaptic plasticity (hippocampal long-term-potentiation in MeCP2-308 heterozygous female mice, the genetic and hormonal milieu that resembles that of RTT patients. LP-211 also restores the activation of the ribosomal protein S6, the downstream target of mTOR and S6 kinase, in the hippocampus of RTT female mice. Notably, the beneficial effects on neurobehavioral and molecular parameters of a seven-day long treatment with LP-211 were evident up to two months after the last injection, thus suggesting long-lasting effects on RTT-related impairments. Taken together with our previous study, these results provide compelling preclinical evidence of the potential therapeutic value for RTT of a pharmacological approach targeting the brain 5-HT7R.

  19. Long-lasting beneficial effects of central serotonin receptor 7 stimulation in female mice modeling Rett syndrome.

    Science.gov (United States)

    De Filippis, Bianca; Chiodi, Valentina; Adriani, Walter; Lacivita, Enza; Mallozzi, Cinzia; Leopoldo, Marcello; Domenici, Maria Rosaria; Fuso, Andrea; Laviola, Giovanni

    2015-01-01

    Rett syndrome (RTT) is a rare neurodevelopmental disorder, characterized by severe behavioral and physiological symptoms. Mutations in the methyl CpG binding protein 2 gene (MECP2) cause more than 95% of classic cases, and currently there is no cure for this devastating disorder. Recently we have demonstrated that specific behavioral and brain molecular alterations can be rescued in MeCP2-308 male mice, a RTT mouse model, by pharmacological stimulation of the brain serotonin receptor 7 (5-HT7R). This member of the serotonin receptor family-crucially involved in the regulation of brain structural plasticity and cognitive processes-can be stimulated by systemic repeated treatment with LP-211, a brain-penetrant selective 5-HT7R agonist. The present study extends previous findings by demonstrating that the LP-211 treatment (0.25 mg/kg, once per day for 7 days) rescues RTT-related phenotypic alterations, motor coordination (Dowel test), spatial reference memory (Barnes maze test) and synaptic plasticity (hippocampal long-term-potentiation) in MeCP2-308 heterozygous female mice, the genetic and hormonal milieu that resembles that of RTT patients. LP-211 also restores the activation of the ribosomal protein (rp) S6, the downstream target of mTOR and S6 kinase, in the hippocampus of RTT female mice. Notably, the beneficial effects on neurobehavioral and molecular parameters of a seven-day long treatment with LP-211 were evident up to 2 months after the last injection, thus suggesting long-lasting effects on RTT-related impairments. Taken together with our previous study, these results provide compelling preclinical evidence of the potential therapeutic value for RTT of a pharmacological approach targeting the brain 5-HT7R.

  20. Development of central nervous system autoimmunity is impaired in the absence of Wiskott-Aldrich syndrome protein.

    Directory of Open Access Journals (Sweden)

    Marita Bosticardo

    Full Text Available Wiskott-Aldrich Syndrome protein (WASP is a key regulator of the actin cytoskeleton in hematopoietic cells. Defective expression of WASP leads to multiple abnormalities in different hematopoietic cells. Despite severe impairment of T cell function, WAS patients exhibit a high prevalence of autoimmune disorders. We attempted to induce EAE, an animal model of organ-specific autoimmunity affecting the CNS that mimics human MS, in Was(-/- mice. We describe here that Was(-/- mice are markedly resistant against EAE, showing lower incidence and milder score, reduced CNS inflammation and demyelination as compared to WT mice. Microglia was only poorly activated in Was(-/- mice. Antigen-induced T-cell proliferation, Th-1 and -17 cytokine production and integrin-dependent adhesion were increased in Was(-/- mice. However, adoptive transfer of MOG-activated T cells from Was(-/- mice in WT mice failed to induce EAE. Was(-/- mice were resistant against EAE also when induced by adoptive transfer of MOG-activated T cells from WT mice. Was(+/- heterozygous mice developed an intermediate clinical phenotype between WT and Was(-/- mice, and they displayed a mixed population of WASP-positive and -negative T cells in the periphery but not in their CNS parenchyma, where the large majority of inflammatory cells expressed WASP. In conclusion, in absence of WASP, T-cell responses against a CNS autoantigen are increased, but the ability of autoreactive T cells to induce CNS autoimmunity is impaired, most probably because of an inefficient T-cell transmigration into the CNS and defective CNS resident microglial function.

  1. [Spinal cord stimulation for the management of chronic pain].

    Science.gov (United States)

    Perruchoud, Christophe; Mariotti, Nicolas

    2016-06-22

    Neuromodulation techniques modify the activity of the central or peripheral nervous system. Spinal cord stimulation is a reversible and minimally invasive treatment whose efficacy and cost effectiveness are recognized for the treatment of chronic neuropathic pain or ischemic pain. Spinal cord stimulation is not the option of last resort and should be considered among other options before prescribing long-term opioids or considering reoperation. The selection and regular follow-up of patients are crucial to the success of the therapy. PMID:27506068

  2. Retraining the injured spinal cord

    Science.gov (United States)

    Edgerton, V. R.; Leon, R. D.; Harkema, S. J.; Hodgson, J. A.; London, N.; Reinkensmeyer, D. J.; Roy, R. R.; Talmadge, R. J.; Tillakaratne, N. J.; Timoszyk, W.; Tobin, A.

    2001-01-01

    The present review presents a series of concepts that may be useful in developing rehabilitative strategies to enhance recovery of posture and locomotion following spinal cord injury. First, the loss of supraspinal input results in a marked change in the functional efficacy of the remaining synapses and neurons of intraspinal and peripheral afferent (dorsal root ganglion) origin. Second, following a complete transection the lumbrosacral spinal cord can recover greater levels of motor performance if it has been exposed to the afferent and intraspinal activation patterns that are associated with standing and stepping. Third, the spinal cord can more readily reacquire the ability to stand and step following spinal cord transection with repetitive exposure to standing and stepping. Fourth, robotic assistive devices can be used to guide the kinematics of the limbs and thus expose the spinal cord to the new normal activity patterns associated with a particular motor task following spinal cord injury. In addition, such robotic assistive devices can provide immediate quantification of the limb kinematics. Fifth, the behavioural and physiological effects of spinal cord transection are reflected in adaptations in most, if not all, neurotransmitter systems in the lumbosacral spinal cord. Evidence is presented that both the GABAergic and glycinergic inhibitory systems are up-regulated following complete spinal cord transection and that step training results in some aspects of these transmitter systems being down-regulated towards control levels. These concepts and observations demonstrate that (a) the spinal cord can interpret complex afferent information and generate the appropriate motor task; and (b) motor ability can be defined to a large degree by training.

  3. The astrocytic transporter SLC7A10 (Asc-1) mediates glycinergic inhibition of spinal cord motor neurons

    Science.gov (United States)

    Ehmsen, Jeffrey T.; Liu, Yong; Wang, Yue; Paladugu, Nikhil; Johnson, Anna E.; Rothstein, Jeffrey D.; du Lac, Sascha; Mattson, Mark P.; Höke, Ahmet

    2016-01-01

    SLC7A10 (Asc-1) is a sodium-independent amino acid transporter known to facilitate transport of a number of amino acids including glycine, L-serine, L-alanine, and L-cysteine, as well as their D-enantiomers. It has been described as a neuronal transporter with a primary role related to modulation of excitatory glutamatergic neurotransmission. We find that SLC7A10 is substantially enriched in a subset of astrocytes of the caudal brain and spinal cord in a distribution corresponding with high densities of glycinergic inhibitory synapses. Accordingly, we find that spinal cord glycine levels are significantly reduced in Slc7a10-null mice and spontaneous glycinergic postsynaptic currents in motor neurons show substantially diminished amplitudes, demonstrating an essential role for SLC7A10 in glycinergic inhibitory function in the central nervous system. These observations establish the etiology of sustained myoclonus (sudden involuntary muscle movements) and early postnatal lethality characteristic of Slc7a10-null mice, and implicate SLC7A10 as a candidate gene and auto-antibody target in human hyperekplexia and stiff person syndrome, respectively. PMID:27759100

  4. HERC 1 ubiquitin ligase mutation affects neocortical, CA3 hippocampal and spinal cord projection neurons. An ultrastructural study

    Directory of Open Access Journals (Sweden)

    Rocío eRuiz

    2016-04-01

    Full Text Available The spontaneous mutation tambaleante is caused by the Gly483Glu substitution in the highly conserved N terminal RCC1-like domain of the HERC1 protein, which leads to the increase of mutated protein levels responsible for cerebellar Purkinje cell death by autophagy. Until now, Purkinje cells have been the only central nervous neurons reported as being targeted by the mutation, and their degeneration elicits an ataxic syndrome in adult mutant mice. However, the ultrastructural analysis performed here demonstrates that signs of autophagy, such as autophagosomes, lysosomes, and altered mitochondria, are present in neocortical pyramidal, CA3 hippocampal pyramidal, and spinal cord motor neurons. The main difference is that the reduction in the number of neurons affected in the tambaleante mutation in the neocortex, the hippocampus, and the spinal cord is not so evident as the dramatic loss of cerebellar Purkinje cells. Interestingly, signs of autophagy are absent in both interneurons and neuroglia cells. Affected neurons have in common that they are projection neurons which receive strong and varied synaptic inputs, and possess the highest degree of neuronal activity. Therefore, because the integrity of the ubiquitin-proteasome system is essential for protein degradation and, hence, for normal protein turnover, it could be hypothesized that the deleterious effects of the misrouting of these pathways would depend directly on the neuronal activity.

  5. HERC 1 Ubiquitin Ligase Mutation Affects Neocortical, CA3 Hippocampal and Spinal Cord Projection Neurons: An Ultrastructural Study.

    Science.gov (United States)

    Ruiz, Rocío; Pérez-Villegas, Eva María; Bachiller, Sara; Rosa, José Luis; Armengol, José Angel

    2016-01-01

    The spontaneous mutation tambaleante is caused by the Gly483Glu substitution in the highly conserved N terminal RCC1-like domain of the HERC1 protein, which leads to the increase of mutated protein levels responsible for cerebellar Purkinje cell death by autophagy. Until now, Purkinje cells have been the only central nervous neurons reported as being targeted by the mutation, and their degeneration elicits an ataxic syndrome in adult mutant mice. However, the ultrastructural analysis performed here demonstrates that signs of autophagy, such as autophagosomes, lysosomes, and altered mitochondria, are present in neocortical pyramidal, CA3 hippocampal pyramidal, and spinal cord motor neurons. The main difference is that the reduction in the number of neurons affected in the tambaleante mutation in the neocortex, the hippocampus, and the spinal cord is not so evident as the dramatic loss of cerebellar Purkinje cells. Interestingly, signs of autophagy are absent in both interneurons and neuroglia cells. Affected neurons have in common that they are projection neurons which receive strong and varied synaptic inputs, and possess the highest degree of neuronal activity. Therefore, because the integrity of the ubiquitin-proteasome system is essential for protein degradation and hence, for normal protein turnover, it could be hypothesized that the deleterious effects of the misrouting of these pathways would depend directly on the neuronal activity. PMID:27147983

  6. Effets de l'inhibition du système sympathique central sur les paramètres métaboliques et microcirculatoires chez les rats obèses avec syndrome métabolique

    OpenAIRE

    Nascimento, Alessandro

    2013-01-01

    Cardiovascular and metabolic risk factors that characterize the metabolic syndrome (MS), including high blood pressure, obesity and glucose intolerance, are accompanied by sympathetic hyperactivity. In this study, we investigated the effects of a chronic oral antihypertensive treatment using centrally-acting sympatho-inhibitory drugs on the metabolic and microvascular parameters in rats under long-term high-fat diet with salt supplementation. For that, fifty male adult Wistar rats were mainta...

  7. The spinal cord

    International Nuclear Information System (INIS)

    The spinal cord develops initially as an invagination of the thickened ectodermal neural plate to form the neural groove. This is then closed over by the neural folds, which fuse first in the thoracic region, then progressively rostrad and caudad to form the neural tube. The neural tube is completely formed by the fourth fetal week and is separated from the overlying ectoderm by intervening mesoderm, part of which has simultaneously segmented into somites to become the vertebral column. The cartilaginous and ossifying neural arches of the vertebral column are completely developed and fused by the third month of fetal life. The fetal spine can be detected by US by 12 weeks of gestational age

  8. Central neurobiological mechanism of liver depression and spleen deficiency syndrome based on chronic stress: a review%从慢性应激探讨肝郁脾虚证的中枢神经生物学机制

    Institute of Scientific and Technical Information of China (English)

    李晓红; 李晶晶; 刘玥芸; 陈家旭

    2012-01-01

    Some researchers focus on research of the nature of syndromes. The methods of combining traditional Chinese medicine syndrome and diseases and the correspondence between formulas and syndromes may be used in research of the nature of syndromes. According to combined theories of zang-organ state and seven emotions in traditional Chinese medicine with stress theory in modern medicine, the authors applied the methods of chronic immobilization stress to induce liver depression and spleen deficiency syndrome in rats based on the thinking of relativity on formula and syndrome. The research showed that the central neurobiology mechanism of liver depression and spleen deficiency syndrome closely correlates to the hypo-thalamic-pituitary-adrenal axis, brain-gut axis, myriad central neurotrophic factors, neurotransmitters, neuropep-tides and hormones and their receptors, involving in many encephalic regions such as the hypothalamus, hippocampus, cortex, amygdale, etc. The authors will combine their previous work with multi-disciplinary research, such as genomics, proteomics, metabolomics and bioinformatics in future studies, to reveal the scientific connotations of liver depression and spleen deficiency syndrome.%证实质的研究一直是很多学者研究的重点方向,可利用中医病证结合、方证相关的研究思路来探寻证候的生物学基础.从现代应激理论入手,结合中医脏象理论和七情学说,运用中医方证相关的研究思路,通过慢性束缚应激的方法复制肝郁脾虚证大鼠模型,前期实验结果提示肝郁脾虚证的中枢神经生物学机制与下丘脑-垂体-肾上腺轴、脑肠轴以及中枢多种神经营养因子、神经递质、神经肽、激素及其受体密切相关,涉及下丘脑、海马、皮层、杏仁核等多个脑区.今后将结合基因组学、蛋白质组学、代谢组学,并与生物信息学等多学科结合,以期揭示肝郁脾虚证的科学内涵.

  9. Central cholinergic regulation of respiration: nicotinic receptors

    Institute of Scientific and Technical Information of China (English)

    Xuesi M SHAO; Jack L FELDMAN

    2009-01-01

    Nicotinic acetylcholine receptors (nAChRs) are expressed in brainstem and spinal cord regions involved in the control of breathing. These receptors mediate central cholinergic regulation of respiration and effects of the exogenous ligand nicotine on respiratory pattern. Activation of a4* nAChRs in the preBotzinger Complex (preBotC), an essential site for normal respiratory rhythm generation in mammals, modulates excitatory glutamatergic neurotransmission and depolarizes preBotC inspiratory neurons, leading to increases in respiratory frequency. nAChRs are also present in motor nuclei innervating respiratory muscles. Activation of post- and/or extra-synaptic a4* nAChRs on hypoglossal (XII) motoneurons depolarizes these neurons, potentiating tonic and respiratory-related rhythmic activity. As perinatal nicotine exposure may contribute to the pathogenesis of sudden infant death syndrome (SIDS), we discuss the effects of perinatal nicotine exposure on development of the cholinergic and other neurotransmitter systems involved in control of breathing. Advances in understanding of the mechanisms underlying central cholinergic/nicotinic modulation of respiration provide a pharmacological basis for exploiting nAChRs as therapeutic targets for neurological disorders related to neural control of breathing such as sleep apnea and SIDS.

  10. Distribution of Traditional Chinese Medicine Syndromes in Psoriasis in Central China%华中地区银屑病中医证候分布情况研究

    Institute of Scientific and Technical Information of China (English)

    李隽; 徐丽敏; 周飞红; 姜一化; 张天柱; 李东升; 周登远

    2011-01-01

    目的 探讨华中地区银屑病中医证候的分布情况.方法 通过填写"银屑病中医证候调查表",采集500例华中地区银屑病患者中医证候四诊资料,确立中医证候,采用Epidata3.1软件包对调查资料进行录入、核查,再转换成SPSS11.5统计软件包进行统计,得出在不同性别、不同年龄组、不同病期、家族史及舌象方面的证候分布情况.结果 银屑病以血热证、血瘀证、血燥证、血热血瘀证、血热血燥证、血瘀血燥证为主要证候,不同性别、不同年龄组、不同病期、家族史及舌质的中医证候分布不同(P<0.05),在舌苔中差异无统计学意义(P>0.05).结论 本研究进一步验证了对"血"的辨证应是银屑病的主要辨证方法,中医证候在不同性别、不同年龄组、不同病情分期、有无家族史及舌质方面分布不同,而在舌苔方面差异无统计学意义.%Objective To explore distribution of traditional chinese medicine(TCM) syndromes in psoriasis in central China.Methods By completing "Questionnaires syndromes of traditional Chinese medicine (TCM) with psoriasis", 500 cases of patients with psoriasis in Huazhong area of TCM syndrome the four diagnostic material were collected and established syndromes.Epidata3.1 package was used to survey data entry and check. And then SPSS11.5 software package was used to statistics, concluded with different gender, different age groups, different disease period, family history and tongue presence of syndromes distribution.Results The main syndrome including blood-heat syndrome, blood-stasis syndrome, blood-dryness syndrome, syndrome of blood stasis and heat, syndrome of blood heat and dryness as well as syndrome of blood stasis and dryness. Distribution of TCM syndrome was different in different gender, different age groups, different disease period, family history and tongue qualitative (P< 0.05). TCM syndrome in tongue coating had no statistical significant (P> 0

  11. Maladaptive spinal plasticity opposes spinal learning and recovery in spinal cord injury

    Directory of Open Access Journals (Sweden)

    Adam R Ferguson

    2012-10-01

    Full Text Available Synaptic plasticity within the spinal cord has great potential to facilitate recovery of function after spinal cord injury (SCI. Spinal plasticity can be induced in an activity-dependent manner even without input from the brain after complete SCI. The mechanistic basis for these effects is provided by research demonstrating that spinal synapses have many of the same plasticity mechanisms that are known to underlie learning and memory in the brain. In addition, the lumbar spinal cord can sustain several forms of learning and memory, including limb-position training. However, not all spinal plasticity promotes recovery of function. Central sensitization of nociceptive (pain pathways in the spinal cord may emerge with certain patterns of activity, demonstrating that plasticity within the spinal cord may contribute to maladaptive pain states. In this review we discuss interactions between adaptive and maladaptive forms of activity-dependent plasticity in the spinal cord. The literature demonstrates that activity-dependent plasticity within the spinal cord must be carefully tuned to promote adaptive spinal training. Stimulation that is delivered in a limb position-dependent manner or on a fixed interval can induce adaptive plasticity that promotes future spinal cord learning and reduces nociceptive hyper-reactivity. On the other hand, stimulation that is delivered in an unsynchronized fashion, such as randomized electrical stimulation or peripheral skin injuries, can generate maladaptive spinal plasticity that undermines future spinal cord learning, reduces recovery of locomotor function, and promotes nociceptive hyper-reactivity after spinal cord injury. We review these basic phenomena, discuss the cellular and molecular mechanisms, and discuss implications of these findings for improved rehabilitative therapies after spinal cord injury.

  12. Central centrifugal cicatricial alopecia

    OpenAIRE

    Collin Blattner; Dennis C Polley; Frank Ferritto; Elston, Dirk M

    2013-01-01

    Central centrifugal cicatricial alopecia is a common cause of progressive permanent apical alopecia. This unique form of alopecia includes entities previously know as “hot comb alopecia,” “follicular degeneration syndrome,” “pseudopelade” in African Americans and “central elliptical pseudopelade” in Caucasians. The etiology appears to be multifactorial and the condition occurs in all races.

  13. Intraspinal microstimulation A novel technique for the functional recovery of spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Hua Zhang; Liqun Feng; Yuping Wang

    2010-01-01

    Nonspecific neuronal activity elicited by intraspinal microstimulation in the intermediate and ventral gray matter of thoracic spinal segments caudal to a complete spinal cord transection significantly increased the rat hindlimb Basso,Beattie,Bresnahan locomotor score by activating the central pattern generator located in the lumbar spinal cord.However,the best region for intraspinal microstimulation is unclear.Using an incomplete spinal cord injury model at T8,we compared the use of intraspinal microstimulation to activate the spinal cord in rats with a spontaneous recovery group.The intraspinal microstimulation group recovered sooner and showed three kinds of movement: the left hindlimb,the left hindlimb toes,and the paraspinal muscles and tails.These had different microstimulation thresholds.There was mild hyperplasia of the astrocytes surrounding the tips of the microelectrodes and slight inflammatory reactions nearby.These results indicate that implantation of microelectrodes was relatively safe and induced minimal damage to the lumbar-sacral spinal cord.Intraspinal microstimulation in the lumbar sacral spinal cord may improve leg movements after spinal cord injury.Non-specific intraspinal microstimulation may be a novel technique for the recovery of spinal cord injuries.

  14. Depression and Spinal Cord Injury

    Science.gov (United States)

    ... of Washington-operated SCI Clinics: Harborview Medical Center Rehabilitation Medicine Clinic 325 9th Ave., Seattle WA 98104 Spinal Cord Injury Clinic nurses: 206-744-5862 University of Washington ...

  15. Spinal Cord Injury Prevention Tips

    Science.gov (United States)

    ... age 1 should not be carried on a bicycle, because their necks are not strong enough to ... may contribute to falls. Secure rugs and loose electrical cords, put away toys, use safety gates, and ...

  16. A report of high triglyceride level in cord blood of Iranian newborns

    Directory of Open Access Journals (Sweden)

    Seyed Ali Naghi Kazemi

    2013-01-01

    Conclusion: The mean and 95 th percentiles of triglyceride levels in cord blood of our newborn infants were higher than other reports. We recommend that larger studies should be conducted in this area to establish preventive ways for increasing epidemic of the metabolic syndrome.

  17. The puerperium alters spinal cord plasticity following peripheral nerve injury

    OpenAIRE

    Gutierrez, Silvia; Hayashida, Ken-ichiro; Eisenach, James C.

    2012-01-01

    Tissue and nerve damage can result in chronic pain. Yet, chronic pain after cesarean delivery is remarkably rare in women and hypersensitivity from peripheral nerve injury in rats resolves rapidly if the injury occurs in the puerperium. Little is known regarding the mechanisms of this protection except for a reliance on central nervous system oxytocin signaling. Here we show that density of inhibitory noradrenergic fibers in the spinal cord is greater when nerve injury is performed in rats du...

  18. A Neural Model of Demyelination of the Mouse Spinal Cord

    OpenAIRE

    Petreska, Biljana; Yovel, Yossi

    2008-01-01

    This paper presents a neural network model of demyelination of the mouse motor pathways, coupled to a central pattern generation (CPG) model for quadruped walking. Demyelination is the degradation of the myelin layer covering the axons which can be caused by several neurodegenerative autoimmune diseases such as multiple sclerosis. We use this model - to our knowledge first of its kind - to investigate the locomotion deficits that appear following demyelination of axons in the spinal cord. Our...

  19. Spinal cord involvement in a child with familial hemophagocytic lymphohistiocytosis

    Directory of Open Access Journals (Sweden)

    Muge Gokce

    2012-01-01

    Full Text Available The involvement of the central nervous system (CNS in familial hemophagocytic lymphohistiocytosis (FHL has known to be limited to the brain, brain stem, and cerebellum. Herein, we report an 11-year-old boy who presented with neurological symptoms and was diagnosed as FHL by molecular diagnosis. The hemophagocytic lesions in the CNS were shown to extend to the thoracal level of spinal cord which completely disappeared after the completion of hemophagocytic lymphohistiocytosis-2004 protocol.

  20. Antiphospholipid syndrome.

    Science.gov (United States)

    Ruiz-Irastorza, Guillermo; Crowther, Mark; Branch, Ware; Khamashta, Munther A

    2010-10-30

    The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs. PMID:20822807

  1. The stress ulcer syndrome

    NARCIS (Netherlands)

    H.A. van Essen

    1986-01-01

    textabstractThe stress ulcer syndrome is described in this thesis. This syndrome is seen in patients admitted to intensive care departments or being treated in field hospitals, in disaster areas, or battle fields. Acute mucosal lesions associated with burns (Curling's ulcers) and central nervous sys

  2. Immunotherapy strategies for spinal cord injury.

    Science.gov (United States)

    Wang, Yong-Tang; Lu, Xiu-Min; Chen, Kai-Ting; Shu, Ya-Hai; Qiu, Chun-Hong

    2015-01-01

    Regeneration in the central nervous system (CNS) of adult mammalian after traumatic injury is limited, which often causes permanent functional motor and sensory loss. After spinal cord injury (SCI), the lack of regeneration is mainly attributed to the presence of a hostile microenvironment, glial scarring, and cavitation. Besides, inflammation has also been proved to play a crucial role in secondary degeneration following SCI. The more prominent treatment strategies in experimental models focus mainly on drugs and cell therapies, however, only a few strategies applied in clinical studies and therapies still have only limited effects on the repair of SCI. Recently, the interests in immunotherapy strategies for CNS are increasing in number and breadth. Immunotherapy strategies have made good progresses in treating many CNS degenerative disorders, such as Alzheimer's disease (AD), Parkinson's disease (PD), stroke, and multiple sclerosis (MS). However, the strategies begin to be considered to the treatment of SCI and other neurological disorders in recent years. Besides anti-inflamatory therapy, immunization with protein vaccines and DNA vaccines has emerged as a novel therapy strategy because of the simplicity of preparation and application. An inflammatory response followed by spinal cord injury, and is controled by specific signaling molecules, such as some cytokines playing a crucial role. As a result, appropriate immunoregulation, the expression of pro-inflammatory cytokines and anti-inflammatory cytokines may be an effective therapy strategy for earlier injury of spinal cord. In addition, myelinassociated inhibitors (MAIs) in the injured spinal cord, such as Nogo, myelin-associated glycoprotein (MAG) and oligodendrocyte- myelin glycoprotein (OMgp) are known to prevent axonal regeneration through their co-receptors, and to trigger demyelinating autoimmunity through T cell-mediated harmful autoimmune response. The antagonism of the MAIs through vaccinating with

  3. Transplantation of olfactory ensheathing cells for promoting regeneration following spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Kaijun Liu

    2007-01-01

    OBJECTIVE: To investigate the status of olfactory ensheathing cells (OECs) transplantation in facilitating the regeneration of spinal cord injury.DATA SOURCES: Articles about OECs transplantation in treating spinal cord injury were searched in Pubmed database published in English from January 1981 to December 2005 by using the keywords of "olfactory ensheathing cells, transplantation, spinal cord injury".STUDY SELECTION: The data were checked primarily, literatures related to OECs transplantation and the regeneration of spinal cord injury were selected, whereas the repetitive studies and reviews were excluded.DATA EXTRACTION: Totally 43 articles about OECs transplantation and the regeneration and repair of spinal cord injury were collected, and the repetitive ones were excluded.DATA SYNTHESIS: There were 35 articles accorded with the criteria. OECs are the olfactory ensheathing glias isolated from olfactory bulb and olfactory nerve tissue. OECs have the characters of both Schwann cells in central nervous system and peripheral astrocytes. The transplanted OECs can migrate in the damaged spinal cord of host, can induce and support the regeneration, growth and extension of damaged neuritis.Besides, transgenic technique can enable it to carry some exogenous genes that promote neuronal regeneration, and express some molecules that can facilitate neural regeneration, so as to ameliorate the internal environment of nerve injury, induce the regeneration of damaged spinal cord neurons, which can stimulate the regeneration potential of the damaged spinal cord to reach the purpose of spinal cord regeneration and functional recovery.CONCLUSION: OECs are the glial cells with the energy for growth at mature phase, they can myelinize axons, secrete various biological nutrition factors, and then protect and support neurons, also facilitate neural regeneration. OECs have been successfully isolated from nasal olfactory mucosa and olfactory nerve.Therefore, autologous transplantation

  4. 脐带间充质干细胞移植对不同证型系统性红斑狼疮患者的效果分析%Therapeutic effect of umbilical cord-derived mesenchymal stem cell transplantation in systemic lupus erythematosus patients with different patterns of syndromes

    Institute of Scientific and Technical Information of China (English)

    汤郁; 刘瑞霞; 裘影影; 芮金兵; 李晶

    2015-01-01

    BACKGROUND:Systemic lupus erythematosus (SLE) is classified into four types, and the major treatment is to tonify kidney and nourishyin, clear blood stasis and toxin by the traditional Chinese medicine (TCM). Even though, there are stil many patients with poor efficacy. Mesenchymal stem cels have the capacity of multiple differentiation, hematopoietic support and immune regulation, thus having been used for the treatment of refractory, recurrent SLE and achieving good effects. OBJECTIVE:To investigate the therapeutic effect of umbilical cord-derived mesenchymal stem cel transplantation on SLE patients with different patterns of syndromes. METHODS: Twenty-one SLE patients were clustered to four syndrome types of TCM, including heat-toxin,yin deficiency of liver and kidney,yang deficiency of spleen and kidney, andqi stagnation and blood stasis. The changes in clinical and laboratory indicators were analyzed statisticaly before and after cel transplantation. RESULTS AND CONCLUSION:The level of 24-hour proteinuria and SLE disease activity index scores in SLE patients were significantly decreased at 1, 3, 6 months after cel transplantation (P < 0.01). Umbilical cord-derived mesenchymal stem cel transplantation could significantly reduce the 24-hour proteinuria in SLE patients withyin deficiency of liver and kidney at 1, 3 and 6 months (P < 0.01), while slightly reduce the 24-hour proteinuria in SLE patients with heat-toxin andqi stagnation and blood stasis at 1, 3 months (P < 0.05) as wel as in SLE patients withyang deficiency of spleen and kidney at 1 month (P < 0.05). Additionaly, umbilical cord-derived mesenchymal stem cel transplantation could increase the serum albumin levels in al the SLE patients (P < 0.01), although the changes in patients with heat-toxin were moderate (P < 0.05). Al the SLE patients of four types had an increasing trend of their platelet counting after cel transplantation, but there was no statistical difference before and after cel

  5. A Case Report of Jarcho–Levin Syndrome and Caudal Regression Syndrome in A Five Year Old Girl

    Directory of Open Access Journals (Sweden)

    H. Emad Momtaz

    2008-07-01

    Full Text Available Introduction: Jarcho- Levin syndrome and caudal regression syndrome are rare syndromes with clinical and radiologic findings of cervical and thoracic vertebral anomalies with rib deformities (jarcho-levin syndrome and sacrococcygeal agenesis, lower extremity anomalies, truncated spinal cord and neurogenic bladder (caudal regression syndrome. There are only one report of simultaneous existence of these two syndromes in a dead fetus in literature.Case Report: The case was a 5 year old girl who had history of bilateral hip dysplasia and spastic dipleigia of lower limbs who presented with recurrent urinary tract infections. X - Ray findings was extensive vertebral anomalies such as hemivertebrae, butterfly vertebrae, coccygeal agenesis and rib deformities. MRI findings was termination of spinal cord at low thoracic level, cystic mass of sacral area.Conclusion: This case is a rare simultaneous occurrence of Jarcho-levin and Cauda regression syndrome in a patient and to our best knowledge is reported for the first time in Iran.

  6. Dexmedetomidine for an awake fiber-optic intubation of a parturient with Klippel-Feil syndrome, Type I Arnold Chiari malformation and status post released tethered spinal cord presenting for repeat cesarean section

    Directory of Open Access Journals (Sweden)

    Tanmay H. Shah

    2011-08-01

    Full Text Available Patients with Klippel-Feil Syndrome (KFS have congenital fusion of their cervical vertebrae due to a failure in the normal segmentation of the cervical vertebrae during the early weeks of gestation and also have myriad of other associated anomalies. Because of limited neck mobility, airway management in these patients can be a challenge for the anesthesiologist. We describe a unique case in which a dexmedetomidine infusion was used as sedation for an awake fiber-optic intubation in a parturient with Klippel-Feil Syndrome, who presented for elective cesarean delivery. A 36-yearold female, G2P1A0 with KFS (fusion of cervical vertebrae who had prior cesarean section for breech presentation with difficult airway management was scheduled for repeat cesarean delivery. After obtaining an informed consent, patient was taken in the operating room and non-invasive monitors were applied. Dexmedetomidine infusion was started and after adequate sedation, an awake fiberoptic intubation was performed. General anesthetic was administered after intubation and dexmedetomidine infusion was continued on maintenance dose until extubation. Klippel-Feil Syndrome (KFS is a rare congenital disorder for which the true incidence is unknown, which makes it even rare to see a parturient with this disease. Patients with KFS usually have other congenital abnormalities as well, sometimes including the whole thoraco-lumbar spine (Type III precluding the use of neuraxial anesthesia for these patients. Obstetric patients with KFS can present unique challenges in administering anesthesia and analgesia, primarily as it relates to the airway and dexmedetomidine infusion has shown promising result to manage the airway through awake fiberoptic intubation without any adverse effects on mother and fetus.

  7. Spinal cord injury pain: mechanisms and management.

    Science.gov (United States)

    Finnerup, Nanna Brix; Baastrup, Cathrine

    2012-06-01

    Patients with spinal cord injury (SCI) may experience several types of chronic pain, including peripheral and central neuropathic pain, pain secondary to overuse, painful muscle spasms, and visceral pain. An accurate classification of the patient's pain is important for choosing the optimal treatment strategy. In particular, neuropathic pain appears to be persistent despite various treatment attempts. In recent years, we have gained increasing knowledge of SCI pain mechanisms from experimental models and clinical studies. Nevertheless, treatment remains difficult and inadequate. In line with the recommendations for peripheral neuropathic pain, evidence from randomized controlled treatment trials suggests that tricyclic antidepressants and pregabalin are first-line treatments. This review highlights the diagnosis and classification of SCI pain and recent improvements in the understanding of underlying mechanisms, and provides an update on treatment of SCI pain. PMID:22392531

  8. Transplant Outcomes (Bone Marrow and Cord Blood)

    Science.gov (United States)

    ... reports show patient survival and transplant data of bone marrow and umbilical cord blood transplants in the transplant ... Data by Center Report —View the number of bone marrow and cord blood transplants performed at a specific ...

  9. Spinal Cord Injury Model System Information Network

    Science.gov (United States)

    ... Go New to Website Managing Bowel Function After Spinal Cord Injury Resilience, Depression and Bouncing Back after SCI Getting ... the UAB-SCIMS Contact the UAB-SCIMS UAB Spinal Cord Injury Model System Newly Injured Health Daily Living Consumer ...

  10. Suicide in a spinal cord injured population

    DEFF Research Database (Denmark)

    Hartkopp, A; Brønnum-Hansen, Henrik; Seidenschnur, A M;

    1998-01-01

    To determine the relation between functional status and risk of suicide among individuals with spinal cord injury (SCI).......To determine the relation between functional status and risk of suicide among individuals with spinal cord injury (SCI)....

  11. Glial implications in transplantation therapy of spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    CHEN Shi-wen; XIE Yu-feng

    2009-01-01

    Spinal cord injuries are damages that result in complete or partial loss of sensation and/or mobility and affect the life qualities of many patients. Their pathophysiology in-cludes primary and secondary processes, which are related with the activation of astrocytes and microgliacytes and the degeneration of oligodendrocytes. Although transplan-tation of embryonic stem cells or neural progenitor cells is an attractive strategy for repair of the injured central ner-vous system (CNS), transplantation of these cells alone for acute spinal cord injuries has not resulted in robust axon regeneration beyond the injury sites. This may be due to the progenitor cells differentiating to the cell types that sup-port axon growth poorly and/or their inability to modify the inhibitory environment of adult CNS after injury. Recent studies indicate that transplantation of glial progenitor cells has exhibited beneficial effects on the recovery and promis-ing future for the therapy strategy of spinal cord injury. In this review, we summarized the data from recent literature regarding glial implications in transplantation therapy of spinal cord injury.

  12. [MR investigation of spinal cord herniation in the thoracic spine].

    Science.gov (United States)

    Kenéz, József; Barsi, Péter; Várallyay, György; Bobest, Mátyás; Veres, Róbert

    2002-05-20

    Transdural herniation of the spinal cord is thought to be previously extremely rare and very often misdiagnosed. Possible reasons may be iatrogenic and traumatic or in about one third of cases it may be unknown, where the probable origin might be a congenital dural defect. The pathology may show characteristic and misleading MR patterns of the thoracic spine, emphasising the importance of these patterns. This anomaly can lead to progressive Brown-Sequard syndrome. Surgical intervention, consisting the repair of the dural defect may result in improvement or even complete regression of the neurologic deficits.

  13. MRI in Lyme disease of the spinal cord

    International Nuclear Information System (INIS)

    We report a case of Lyme myelitis in a 31-year-old man, presenting with a conus medullaris syndrome. MRI demonstrated contrast enhancement on the pial surface of the lower thoracic cord and conus medullaris. Elevated blood immunoglobulins and IgM antibodies against Borrelia burgdorferi in the cerebrospinal fluid (CSF) were found. Leptomeningitis may be the first stage of spinal infection in Lyme disease, preceding parenchymal infection leading to myelitis. Vasculitis is probably the major mechanism. MRI findings are nonspecific and the diagnosis is given by serum and CSF analyses. Early treatment with antibiotics and high doses steroids may result in complete recovery, as in this case. (orig.)

  14. MRI in Lyme disease of the spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Mantienne, C.; Catalaa, I.; Sevely, A.; Cognard, C.; Manelfe, C. [Dept. of Diagnostic and Therapeutic Neuroradiology, Hopital Purpan, Toulouse (France); Albucher, J.F. [Dept. of Neurology, Hopital Purpan, Toulouse (France)

    2001-06-01

    We report a case of Lyme myelitis in a 31-year-old man, presenting with a conus medullaris syndrome. MRI demonstrated contrast enhancement on the pial surface of the lower thoracic cord and conus medullaris. Elevated blood immunoglobulins and IgM antibodies against Borrelia burgdorferi in the cerebrospinal fluid (CSF) were found. Leptomeningitis may be the first stage of spinal infection in Lyme disease, preceding parenchymal infection leading to myelitis. Vasculitis is probably the major mechanism. MRI findings are nonspecific and the diagnosis is given by serum and CSF analyses. Early treatment with antibiotics and high doses steroids may result in complete recovery, as in this case. (orig.)

  15. Beals Syndrome

    Science.gov (United States)

    ... Boards & Staff Annual Report & Financials Contact Us Donate Marfan & Related Disorders What is Marfan Syndrome? What are ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of ...

  16. The role of propriospinal interneurons in recovery from spinal cord injury.

    Science.gov (United States)

    Flynn, Jamie R; Graham, Brett A; Galea, Mary P; Callister, Robert J

    2011-04-01

    Over one hundred years ago, Sir Charles Sherrington described a population of spinal cord interneurons (INs) that connect multiple spinal cord segments and participate in complex or 'long' motor reflexes. These neurons were subsequently termed propriospinal neurons (PNs) and are known to play a crucial role in motor control and sensory processing. Recent work has shown that PNs may also be an important substrate for recovery from spinal cord injury (SCI) as they contribute to plastic reorganisation of spinal circuits. The location, inter-segmental projection pattern and sheer number of PNs mean that after SCI, a significant number of them are capable of 'bridging' an incomplete spinal cord lesion. When these properties are combined with the capacity of PNs to activate and coordinate locomotor central pattern generators (CPGs), it is clear they are ideally placed to assist locomotor recovery. Here we summarise the anatomy, organisation and function of PNs in the uninjured spinal cord, briefly outline the pathophysiology of SCI, describe how PNs contribute to recovery of motor function, and finally, we discuss the mechanisms that underlie PN plasticity. We propose there are two major challenges for PN research. The first is to learn more about ways we can promote PN plasticity and manipulate the 'hostile' micro-environment that limits regeneration in the damaged spinal cord. The second is to study the cellular/intrinsic properties of PNs to better understand their function in both the normal and injured spinal cord. This article is part of a Special Issue entitled 'Synaptic Plasticity & Interneurons'.

  17. Relationship of the fat metabolic parameters and androgen level of umbilical cord blood in newborns of mothers with polycystic ovary syndrome%多囊卵巢综合征母亲的新生儿脂肪代谢指标与脐带血雄激素水平的关系

    Institute of Scientific and Technical Information of China (English)

    黄龙坚; 吕艳; 郑景辉; 陈景霞

    2015-01-01

    Objective To assess the relationship between fat metabolic parameters and androgen concentration in the cord blood of newborns of mothers with polycystic ovary syndrome (PCOS). Methods This cross-sectional study included PCOS women (n=55) and neonatal, and 40 cases with matched body mass index (BMI) were used as control. The clinical data including height, body mass, waist circumference, hip circumference of PCOS group, and length and head circumference in newborns after delivery were measured and compared. Blood lipid level, serum insulin and testosterone level were detected using umbilical artery-vein mixed cord blood after delivery. Regression analysis was used to analyze the influence factors of neonatal cholesterol and testosterone levels. Results The neonatal birth weight, head circumference, cholesterol, low density lipoprotein cholesterol, high density lipoprotein cholesterol and triglyceride level were significantly lower, birth height and testosterone level were significantly higher, in PCOS group than those of control group (P<0.05). Values of waist to hip ratio, BMI, cholesterol, high density lipoprotein cholesterol and testosterone levels were significantly higher in PCOS group than those of control group (P<0.05). The insulin, cholesterol and triglyceride levels of PCOS mother were risk factors for neonatal cholesterol level(P < 0.05). The cholesterol, triglyceride and free testosterone levels of PCOS mother were risk factors for increased neonatal free testosterone (P < 0.05). Conclusion Mother with PCOS may affect fetal birth weight, head circumference and cord blood lipid metabolism, which may be related with the elevated level of testosterone during the fetal period.%目的 评估多囊卵巢综合征(PCOS)母亲所生新生儿的脂肪代谢指标与脐带血雄激素水平的关系.方法 选取55例PCOS母亲及其新生儿的临床资料(PCOS组),40例体质指数(BMI)与PCOS组匹配的健康母亲及其新生儿资料为对照组,

  18. Cysticercosis of the nervous system: less frequent clinical forms III- spinal cord forms

    Directory of Open Access Journals (Sweden)

    Horacio M. Canelas

    1963-06-01

    Full Text Available The spinal forms of cysticercosis are rather rare (2.7% of 296 cases of neurocysticercosis recorded in the Department of Neurology of the University of São Paulo Medical School. In a survey of the literature only 42 cases were found, most of them associated with cerebral symptoms. The reasons for this low incidence, as well as the possible routes followed by the parasite in its approach to the spinal cord, are discussed. After a review of the first cases reported in the literature, the authors refer the main syndromes (meningomyelitides, tabetiform pictures and spinal cord compressions and some of the clinico-pathologic features of spinal cysticercosis. Nine cases of spinal cysticercosis are reported. The diagnosis was based on laboratorial data (mainly the complement fixation test for cysticercosis in the cerebrospinal fluid or in the results of surgical therapy. Other cerebrospinal fluid findings (presence of eosinophile cells, protein contents, and the results of the manometric tests are discussed. Myelographic block was demonstrated in 5 cases. Three of these patients were submitted to laminectomy, with variable results. The prevailing neurological picture was that of spinal cord and/or root compression (4 cases. Two patients showed a dorsal funiculi syndrome closely simulating tabes dorsalis. Two other patients presented a picture of meningomyelitis with no systematization. One patient had a syndrome suggestive of subacute combined degeneration of the spinal cord, but the presence of cerebral symptoms and the laboratorial data pointed to cysti-cercosis as the main disease process.

  19. The paradox of chronic neuroinflammation, systemic immune suppression, autoimmunity after traumatic chronic spinal cord injury.

    Science.gov (United States)

    Schwab, Jan M; Zhang, Yi; Kopp, Marcel A; Brommer, Benedikt; Popovich, Phillip G

    2014-08-01

    During the transition from acute to chronic stages of recovery after spinal cord injury (SCI), there is an evolving state of immunologic dysfunction that exacerbates the problems associated with the more clinically obvious neurologic deficits. Since injury directly affects cells embedded within the "immune privileged/specialized" milieu of the spinal cord, maladaptive or inefficient responses are likely to occur. Collectively, these responses qualify as part of the continuum of "SCI disease" and are important therapeutic targets to improve neural repair and neurological outcome. Generic immune suppressive therapies have been largely unsuccessful, mostly because inflammation and immunity exert both beneficial (plasticity enhancing) and detrimental (e.g. glia- and neurodegenerative; secondary damage) effects and these functions change over time. Moreover, "compartimentalized" investigations, limited to only intraspinal inflammation and associated cellular or molecular changes in the spinal cord, neglect the reality that the structure and function of the CNS are influenced by systemic immune challenges and that the immune system is 'hardwired' into the nervous system. Here, we consider this interplay during the progression from acute to chronic SCI. Specifically, we survey impaired/non-resolving intraspinal inflammation and the paradox of systemic inflammatory responses in the context of ongoing chronic immune suppression and autoimmunity. The concepts of systemic inflammatory response syndrome (SIRS), compensatory anti-inflammatory response syndrome (CARS) and "neurogenic" spinal cord injury-induced immune depression syndrome (SCI-IDS) are discussed as determinants of impaired "host-defense" and trauma-induced autoimmunity. PMID:25017893

  20. Spinal Cord Ventral Horns and Lymphoid Organ Involvement in Powassan Virus Infection in a Mouse Model

    Directory of Open Access Journals (Sweden)

    Rodrigo I. Santos

    2016-08-01

    Full Text Available Powassan virus (POWV belongs to the family Flaviviridae and is a member of the tick-borne encephalitis serogroup. Transmission of POWV from infected ticks to humans has been documented in the USA, Canada, and Russia, causing fatal encephalitis in 10% of human cases and significant neurological sequelae in survivors. We used C57BL/6 mice to investigate POWV infection and pathogenesis. After footpad inoculation, infected animals exhibited rapid disease progression and 100% mortality. Immunohistochemistry and immunofluorescence revealed a very strong neuronal tropism of POWV infection. The central nervous system infection appeared as a meningoencephalitis with perivascular mononuclear infiltration and microglial activation in the brain, and a poliomyelitis-like syndrome with high level of POWV antigen at the ventral horn of the spinal cord. Pathological studies also revealed substantial infection of splenic macrophages by POWV, which suggests that the spleen plays a more important role in pathogenesis than previously realized. This report provides a detailed description of the neuroanatomical distribution of the lesions produced by POWV infection in C57BL/6 mice.

  1. Spinal Cord Ventral Horns and Lymphoid Organ Involvement in Powassan Virus Infection in a Mouse Model.

    Science.gov (United States)

    Santos, Rodrigo I; Hermance, Meghan E; Gelman, Benjamin B; Thangamani, Saravanan

    2016-01-01

    Powassan virus (POWV) belongs to the family Flaviviridae and is a member of the tick-borne encephalitis serogroup. Transmission of POWV from infected ticks to humans has been documented in the USA, Canada, and Russia, causing fatal encephalitis in 10% of human cases and significant neurological sequelae in survivors. We used C57BL/6 mice to investigate POWV infection and pathogenesis. After footpad inoculation, infected animals exhibited rapid disease progression and 100% mortality. Immunohistochemistry and immunofluorescence revealed a very strong neuronal tropism of POWV infection. The central nervous system infection appeared as a meningoencephalitis with perivascular mononuclear infiltration and microglial activation in the brain, and a poliomyelitis-like syndrome with high level of POWV antigen at the ventral horn of the spinal cord. Pathological studies also revealed substantial infection of splenic macrophages by POWV, which suggests that the spleen plays a more important role in pathogenesis than previously realized. This report provides a detailed description of the neuroanatomical distribution of the lesions produced by POWV infection in C57BL/6 mice. PMID:27529273

  2. Spinal Cord Ventral Horns and Lymphoid Organ Involvement in Powassan Virus Infection in a Mouse Model

    Science.gov (United States)

    Santos, Rodrigo I.; Hermance, Meghan E.; Gelman, Benjamin B.; Thangamani, Saravanan

    2016-01-01

    Powassan virus (POWV) belongs to the family Flaviviridae and is a member of the tick-borne encephalitis serogroup. Transmission of POWV from infected ticks to humans has been documented in the USA, Canada, and Russia, causing fatal encephalitis in 10% of human cases and significant neurological sequelae in survivors. We used C57BL/6 mice to investigate POWV infection and pathogenesis. After footpad inoculation, infected animals exhibited rapid disease progression and 100% mortality. Immunohistochemistry and immunofluorescence revealed a very strong neuronal tropism of POWV infection. The central nervous system infection appeared as a meningoencephalitis with perivascular mononuclear infiltration and microglial activation in the brain, and a poliomyelitis-like syndrome with high level of POWV antigen at the ventral horn of the spinal cord. Pathological studies also revealed substantial infection of splenic macrophages by POWV, which suggests that the spleen plays a more important role in pathogenesis than previously realized. This report provides a detailed description of the neuroanatomical distribution of the lesions produced by POWV infection in C57BL/6 mice. PMID:27529273

  3. Psychological Aspects of Spinal Cord Injury

    Science.gov (United States)

    Cook, Daniel W.

    1976-01-01

    Reviewing literature on the psychological impact of spinal cord injury suggests: (a) depression may not be a precondition for injury adjustment; (b) many persons sustaining cord injury may have experienced psychological disruption prior to injury; and (c) indexes of rehabilitation success need to be developed for the spinal cord injured. (Author)

  4. FAQs about Spinal Cord Injury (SCI)

    Science.gov (United States)

    ... of Care? Emergency Medical Services Hospital (Acute) Care Rehabilitation More FAQs about Spinal Cord Injury (SCI) If you or a loved one is ... spinal cord injury? What recovery is expected following spinal cord injury? Where is the ... on Disability, Independent Living, and Rehabilitation Research (NIDILRR grant number 90SI5005). NIDILRR is a ...

  5. Evaluation of spinal cord injury animal models

    Institute of Scientific and Technical Information of China (English)

    Ning Zhang; Marong Fang; Haohao Chen; Fangming Gou; Mingxing Ding

    2014-01-01

    Because there is no curative treatment for spinal cord injury, establishing an ideal animal model is important to identify injury mechanisms and develop therapies for individuals suffering from spinal cord injuries. In this article, we systematically review and analyze various kinds of animal models of spinal cord injury and assess their advantages and disadvantages for further studies.

  6. Augmentation of Voluntary Locomotor Activity by Transcutaneous Spinal Cord Stimulation in Motor-Incomplete Spinal Cord-Injured Individuals.

    Science.gov (United States)

    Hofstoetter, Ursula S; Krenn, Matthias; Danner, Simon M; Hofer, Christian; Kern, Helmut; McKay, William B; Mayr, Winfried; Minassian, Karen

    2015-10-01

    The level of sustainable excitability within lumbar spinal cord circuitries is one of the factors determining the functional outcome of locomotor therapy after motor-incomplete spinal cord injury. Here, we present initial data using noninvasive transcutaneous lumbar spinal cord stimulation (tSCS) to modulate this central state of excitability during voluntary treadmill stepping in three motor-incomplete spinal cord-injured individuals. Stimulation was applied at 30 Hz with an intensity that generated tingling sensations in the lower limb dermatomes, yet without producing muscle reflex activity. This stimulation changed muscle activation, gait kinematics, and the amount of manual assistance required from the therapists to maintain stepping with some interindividual differences. The effect on motor outputs during treadmill-stepping was essentially augmentative and step-phase dependent despite the invariant tonic stimulation. The most consistent modification was found in the gait kinematics, with the hip flexion during swing increased by 11.3° ± 5.6° across all subjects. This preliminary work suggests that tSCS provides for a background increase in activation of the lumbar spinal locomotor circuitry that has partially lost its descending drive. Voluntary inputs and step-related feedback build upon the stimulation-induced increased state of excitability in the generation of locomotor activity. Thus, tSCS essentially works as an electrical neuroprosthesis augmenting remaining motor control.

  7. Cryptic organisation within an apparently irregular rostrocaudal distribution of interneurons in the embryonic zebrafish spinal cord

    International Nuclear Information System (INIS)

    The molecules and mechanisms involved in patterning the dorsoventral axis of the developing vertebrate spinal cord have been investigated extensively and many are well known. Conversely, knowledge of mechanisms patterning cellular distributions along the rostrocaudal axis is relatively more restricted. Much is known about the rostrocaudal distribution of motoneurons and spinal cord cells derived from neural crest but there is little known about the rostrocaudal patterning of most of the other spinal cord neurons. Here we report data from our analyses of the distribution of dorsal longitudinal ascending (DoLA) interneurons in the developing zebrafish spinal cord. We show that, although apparently distributed irregularly, these cells have cryptic organisation. We present a novel cell-labelling technique that reveals that DoLA interneurons migrate rostrally along the dorsal longitudinal fasciculus of the spinal cord during development. This cell-labelling strategy may be useful for in vivo analysis of factors controlling neuron migration in the central nervous system. Additionally, we show that DoLA interneurons persist in the developing spinal cord for longer than previously reported. These findings illustrate the need to investigate factors and mechanisms that determine 'irregular' patterns of cell distribution, particularly in the central nervous system but also in other tissues of developing embryos.

  8. Cryptic organisation within an apparently irregular rostrocaudal distribution of interneurons in the embryonic zebrafish spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Wells, Simon, E-mail: simon.wells@adelaide.edu.au [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); The Special Research Centre for the Molecular Genetics of Development, University of Adelaide, Adelaide, South Australia 5005 (Australia); Conran, John G., E-mail: john.conran@adelaide.edu.au [Ecology and Evolutionary Biology, School of Earth and Environmental Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); Tamme, Richard, E-mail: rtamme@ttu.ee [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); Gaudin, Arnaud, E-mail: a.gaudin@uq.edu.au [School of Biomedical Sciences, University of Queensland, Brisbane, Queensland 4072 (Australia); Webb, Jonathan, E-mail: jonathan.webb@worc.ox.ac.uk [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); Lardelli, Michael, E-mail: michael.lardelli@adelaide.edu.au [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); The Special Research Centre for the Molecular Genetics of Development, University of Adelaide, Adelaide, South Australia 5005 (Australia)

    2010-11-15

    The molecules and mechanisms involved in patterning the dorsoventral axis of the developing vertebrate spinal cord have been investigated extensively and many are well known. Conversely, knowledge of mechanisms patterning cellular distributions along the rostrocaudal axis is relatively more restricted. Much is known about the rostrocaudal distribution of motoneurons and spinal cord cells derived from neural crest but there is little known about the rostrocaudal patterning of most of the other spinal cord neurons. Here we report data from our analyses of the distribution of dorsal longitudinal ascending (DoLA) interneurons in the developing zebrafish spinal cord. We show that, although apparently distributed irregularly, these cells have cryptic organisation. We present a novel cell-labelling technique that reveals that DoLA interneurons migrate rostrally along the dorsal longitudinal fasciculus of the spinal cord during development. This cell-labelling strategy may be useful for in vivo analysis of factors controlling neuron migration in the central nervous system. Additionally, we show that DoLA interneurons persist in the developing spinal cord for longer than previously reported. These findings illustrate the need to investigate factors and mechanisms that determine 'irregular' patterns of cell distribution, particularly in the central nervous system but also in other tissues of developing embryos.

  9. Neuroimaging features of Cornelia de Lange syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Whitehead, Matthew T. [Department of Radiology, Washington, DC (United States); Nagaraj, Usha D. [Department of Radiology, Washington, DC (United States); Cincinnati Children' s Hospital, Department of Radiology, Cincinnati, OH (United States); Pearl, Phillip L. [Department of Radiology, Washington, DC (United States); Boston Children' s Hospital, Department of Neurology, Boston, MA (United States)

    2015-08-15

    Cornelia de Lange syndrome is a rare genetic disease characterized by distinctive facial dysmorphia and dwarfism. Multiple organ system involvement is typical. Various central nervous system (CNS) aberrations have been described in the pathology literature; however, the spectrum of neuroimaging manifestations is less well documented. To present neuroimaging findings from a series of eight patients with Cornelia de Lange syndrome. The CT/MR database at a single academic children's hospital was searched for the terms ''Cornelia'', ''Brachmann'' and ''de Lange.'' The search yielded 18 exams from 16 patients. Two non-CNS and six exams without available images were excluded. Ten exams from eight patients were evaluated by a board-certified neuroradiologist. All patients had skull base dysplasia, most with an unusual coronal basioccipital cleft (7/8). All brain MR exams showed microcephaly, volume loss and gyral simplification (5/5). Six patients had an absent massa intermedia. Four patients had small globe anterior segments; three had optic pathway hypoplasia. Basilar artery fenestration was present in two patients; vertebrobasilar hypoplasia was present in one patient. The inner ear vestibules were dysplastic in two patients. One patient had pachymeningeal thickening. Spinal anomalies included scoliosis, segmentation anomalies, endplate irregularities, basilar invagination, foramen magnum stenosis and tethered spinal cord. Typical imaging manifestations of Cornelia de Lange syndrome include skull base dysplasia with coronal clival cleft, cerebral and brainstem volume loss, and gyral simplification. Membranous labyrinth dysplasia, anterior segment and optic pathway hypoplasia, basilar artery fenestration, absent massa intermedia and spinal anomalies may also be present. (orig.)

  10. Vitamin B(12) dependent changes in mouse spinal cord expression of vitamin B(12) related proteins and the epidermal growth factor system

    DEFF Research Database (Denmark)

    Mutti, Elena; Lildballe, Dorte L; Kristensen, Lise;

    2013-01-01

    Chronic vitamin B(12) (cobalamin) deficiency in the mammalian central nervous system causes degenerative damage, especially in the spinal cord. Previous studies have shown that cobalamin status alters spinal cord expression of epidermal growth factor (EGF) and its receptor in rats. Employing...

  11. Pain and spinal cord imaging measures in children with demyelinating disease

    Directory of Open Access Journals (Sweden)

    Nadia Barakat

    2015-01-01

    Full Text Available Pain is a significant problem in diseases affecting the spinal cord, including demyelinating disease. To date, studies have examined the reliability of clinical measures for assessing and classifying the severity of spinal cord injury (SCI and also to evaluate SCI-related pain. Most of this research has focused on adult populations and patients with traumatic injuries. Little research exists regarding pediatric spinal cord demyelinating disease. One reason for this is the lack of reliable and useful approaches to measuring spinal cord changes since currently used diagnostic imaging has limited specificity for quantitative measures of demyelination. No single imaging technique demonstrates sufficiently high sensitivity or specificity to myelin, and strong correlation with clinical measures. However, recent advances in diffusion tensor imaging (DTI and magnetization transfer imaging (MTI measures are considered promising in providing increasingly useful and specific information on spinal cord damage. Findings from these quantitative imaging modalities correlate with the extent of demyelination and remyelination. These techniques may be of potential use for defining the evolution of the disease state, how it may affect specific spinal cord pathways, and contribute to the management of pediatric demyelination syndromes. Since pain is a major presenting symptom in patients with transverse myelitis, the disease is an ideal model to evaluate imaging methods to define these regional changes within the spinal cord. In this review we summarize (1 pediatric demyelinating conditions affecting the spinal cord; (2 their distinguishing features; and (3 current diagnostic and classification methods with particular focus on pain pathways. We also focus on concepts that are essential in developing strategies for the detection, monitoring, treatment and repair of pediatric myelitis.

  12. Intestinal endotoxemia plays a central role in development of hepatopulmonary syndrome in a cirrhotic rat model induced by multiple pathogenic factors

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    AIM: To characterize the correlation between severity of hepatopulmonary syndrome (HPS) and degree of hepatic dysfunction, and to explore how intestinal endotoxemia (IETM) affects the development of HPS in cirrhotic rats.METHODS: Male Wister rats were fed with a diet containing maize flour, lard, cholesterol, and alcohol and injected subcutaneously with CCl4 oil solution every two days for 8 wk to induce typical cirrhosis and development of HPS. The animals were also given a nitric oxide (NO) production inhibitor, Nω-nitro-L-arginine methyl ester (L-NAME) intraperitoneally, and an iNOS inhibitor, aminoguanidine hydrochloride (AG) via gavage daily from the end of the 4th wk to the end of the 6th or 8th wk, or a HO-1 inhibitor, zinc protoporphyrin (ZnPP) intraperitoneally 12 h prior to killing. Blood, liver and lung tissues were sampled.RESULTS: Histological deterioration of the lung paralleled to that of the liver in the cirrhotic rats. The number of pulmonary capillaries was progressively increased from 6.1±1.1 (count/filed) at the 4th wk to 14.5±2.4 (count/filed) at the 8th wk in the cirrhotic rats. Increased pulmonary capillaries were associated with increased blood levels of lipopolysaccharide (LPS) (0.31±0.08 EU/mL vs control 0.09±0.03 EU/mL),alanine transferase (ALT, 219.1±17.4 U/L vs control 5.9±2.2 U/L) and portal vein pressure. Compared with normal control animals, the number of total cells in bronchoalveolar lavage fluid (BALF) of the cirrhotic rats at the 8th wk was not changed, but the number of macrophages and the ratio of macrophages to total cells were increased by nearly 2-fold, protein expression of inducible nitric oxide synthase (iNOS) and endothelial nitric oxide synthase (eNOS) started to increase significantly at the 4th wk, and reached its peak at the 8th wk in the lung of cirrhotic rats. The increase of iNOS expression appeared to be quicker than that of eNOS.NO2-/NO3-was also increased, which was correlated to the increase of iNOS (r

  13. Unusual presentation in Axenfeld-Rieger syndrome

    Directory of Open Access Journals (Sweden)

    Rajul S Parikh

    2011-01-01

    Full Text Available We report an unusual presentation of a case of Axenfeld-Rieger (A-R syndrome. A 14-year-old male presented with gradual dimness of vision for 1 year and redness of left eye for 3 days. The patient had megalocornea with Haab′s striae in the right eye and posterior embryotoxon in both the eyes. In the left eye, there was a white cord-like structure traversing the anterior chamber with adhesions to iris tissue along its course. On two antiglaucoma medications, his intraocular pressure (IOP was 22 mm Hg in the right eye and 18 mm Hg in the left eye. Gonioscopy revealed a cord-like structure originating at the level of Schwalbe′s line. He underwent right eye trabeculectomy with mitomycin-C. This case highlights a rare presentation of a strange cord-like structure, a rare presentation of A-R syndrome.

  14. Spinal cord injury at birth

    DEFF Research Database (Denmark)

    Fenger-Gron, Jesper; Kock, Kirsten; Nielsen, Rasmus G;

    2008-01-01

    UNLABELLED: A case of perinatally acquired spinal cord injury (SCI) is presented. The foetus was vigorous until birth, the breech presented and delivery was performed by a non-traumatic Caesarean section. The infant displayed symptoms of severe SCI but diagnosis was delayed due to severe co...

  15. Advances in umbilical cord blood transplantation.

    Science.gov (United States)

    Ballen, Karen K

    2006-09-01

    The first successful cord blood transplant was reported in 1989. In the last sixteen years, there has been a substantial increase in the use of cord blood as an alternative stem cell source for patients without matched related or unrelated bone marrow donors. Approximately 5000 cord blood transplants have been performed worldwide. Recently, the results in adult cord blood transplantation appear promising. In this review, the preclinical background, cord blood banking, and ethical issues will be briefly addressed. Outcome data for both pediatric and adult transplantation will be reviewed, with an emphasis on new strategies for adult cord blood transplantation. New indications for cord blood use outside of hematology/oncology will also be explored. PMID:18220876

  16. Neuromyelitis Optica (Devic's Syndrome): an Appraisal.

    Science.gov (United States)

    Crout, Teresa M; Parks, Laura P; Majithia, Vikas

    2016-08-01

    Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic's syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course. The diagnosis of NMO/NMOSD requires a consistent history and examination with typical clinical presentations, findings on spinal cord neuroimaging with MRI, cerebrospinal fluid analysis along with determination of AQP4-IgG serum autoantibody status, and exclusion of other disorders. Two major advances in this field has been the development of diagnostic criteria and treatment recommendations. Consensus diagnostic criteria have been established and were recently revised and published in 2015, enhancing the ability to make a diagnosis and appropriately evaluate these disorders. Expert recommendations and uncontrolled trials form the basis of treatment guidelines. All patients with suspected NMOSD should be treated for acute attacks as soon as possible with high-dose intravenous methylprednisolone -1 gram daily for three to five consecutive days and in some cases, plasma exchange should be used. It is recommended that every patient with NMOSD be started on an immunosuppressive agent, such as, azathioprine, methotrexate, or mycophenolate and in some cases, rituximab, soon after the acute attack and usually be treated for about 5 years after the attack. These advances have helped improve the prognosis and outcome in these disorders. PMID:27402111

  17. MRI of central nervous system anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Izawa, M.; Oikawa, A.; Matoba, A.

    1987-05-01

    MRI was very useful in the evaluation of congenital anomalies of central nervous system as well as other nervous system disease with three-dimensional spatial resolution. We had experienced MRI of central nervous system anomalies, demonstrated characterisitic findings in each anomaly. MRI is useful to observe the coronal, horizontal and sagittal images of the brain and spinal cord in order to discuss the etiological mechanisms of spinal dysraphysm and its associated anomalies. In case of spina bifida cystica MRI was available to decide operative indication for radical operation and tetherd cord developed from postoperative scar or accompanied intraspinal lesions.

  18. MRI of central nervous system anomalies

    International Nuclear Information System (INIS)

    MRI was very useful in the evaluation of congenital anomalies of central nervous system as well as other nervous system disease with three-dimensional spatial resolution. We had experienced MRI of central nervous system anomalies, demonstrated characterisitic findings in each anomaly. MRI is useful to observe the coronal, horizontal and sagittal images of the brain and spinal cord in order to discuss the etiological mechanisms of spinal dysraphysm and its associated anomalies. In case of spina bifida cystica MRI was available to decide operative indication for radical operation and tetherd cord developed from postoperative scar or accompanied intraspinal lesions. (author)

  19. Widespread non-central nervous system organ pathology in fragile X premutation carriers with fragile X-associated tremor/ataxia syndrome and CGG knock-in mice

    NARCIS (Netherlands)

    Hunsaker, M.R.; Greco, C.M.; Spath, M.A.; Smits, A.P.T.; Navarro, C.S.; Tassone, F.; Kros, J.M.; Severijnen, L.A.; Berry-Kravis, E.M.; Berman, R.F.; Hagerman, P.J.; Willemsen, R.; Hagerman, R.J.; Hukema, R.K.

    2011-01-01

    Fragile X-associated tremor/ataxia syndrome (FXTAS) is an adult-onset neurodegenerative disorder generally presenting with intention tremor and gait ataxia, but with a growing list of co-morbid medical conditions including hypothyroidism, hypertension, peripheral neuropathy, and cognitive decline. T

  20. A Case Report of Pfeiffer Syndrome with Spinal Anomaly

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Chung Hee; Yi, Boem Ha; Hong, Hyun Sook; Lee, Hae Kyung; Park, Seong Jin [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of)

    2009-01-15

    Pfeiffer syndrome is a rare genetic disorder characterized by the premature fusion of certain bones of the skull as well as skeletal deformities, including broad thumbs, great toes, and mid-face anomalies. In our case study, the spinal deformity was combined with type II Pfeiffer syndrome. We describe the radiologic findings of the vertebral and spinal cord anomalies, with emphasis on the magnetic resonance imaging (MRI) findings, and a review of the literature on spinal deformities associated with Pfeiffer syndrome.

  1. Macrophage and microglial plasticity in the injured spinal cord.

    Science.gov (United States)

    David, S; Greenhalgh, A D; Kroner, A

    2015-10-29

    Macrophages in the injured spinal cord arise from resident microglia and from infiltrating peripheral myeloid cells. Microglia respond within minutes after central nervous system (CNS) injury and along with other CNS cells signal the influx of their peripheral counterpart. Although some of the functions they carry out are similar, they appear to be specialized to perform particular roles after CNS injury. Microglia and macrophages are very plastic cells that can change their phenotype drastically in response to in vitro and in vivo conditions. They can change from pro-inflammatory, cytotoxic cells to anti-inflammatory, pro-repair phenotypes. The microenvironment of the injured CNS importantly influences macrophage plasticity. This review discusses the phagocytosis and cytokine-mediated effects on macrophage plasticity in the context of spinal cord injury.

  2. Shedding light on restoring respiratory function after spinal cord injury

    Directory of Open Access Journals (Sweden)

    Warren J Alilain

    2009-10-01

    Full Text Available Loss of respiratory function is one of the leading causes of death following spinal cord injury. Because of this, much work has been done in studying ways to restore respiratory function following SCI - including pharmacological and regeneration strategies. With the emergence of new and powerful tools from molecular neuroscience, new therapeutically relevant alternatives to these approaches have become available, including expression of light sensitive proteins called channelrhodopsins. In this article we briefly review the history of various attempts to restore breathing after C2 hemisection, and focus on our recent work using the activation of light sensitive channels to restore respiratory function after experimental spinal cord injury. We also discuss how such light induced activity can help shed light on the inner workings of the central nervous system respiratory circuitry that controls diaphragmatic function.

  3. Central Pattern Generator for Locomotion: Anatomical, Physiological and Pathophysiological Considerations

    Directory of Open Access Journals (Sweden)

    Pierre A. Guertin

    2013-02-01

    Full Text Available This article provides a perspective on major innovations over the past century in research on the spinal cord and, specifically, on specialized spinal circuits involved in the control of rhythmic locomotor pattern generation and modulation. Pioneers such as Charles Sherrington and Thomas Graham Brown have conducted experiments in the early twentieth century that changed our views of the neural control of locomotion. Their seminal work supported subsequently by several decades of evidence has led to the conclusion that walking, flying and swimming are largely controlled by a network of spinal neurons generally referred to as the central pattern generator (CPG for locomotion. It has been subsequently demonstrated across all vertebrate species examined, from lampreys to humans, that this CPG is capable, under some conditions, to self-produce, even in absence of descending or peripheral inputs, basic rhythmic and coordinated locomotor movements. Recent evidence suggests, in turn, that plasticity changes of some CPG elements may contribute to the development of specific pathophysiological conditions associated with impaired locomotion or spontaneous locomotor-like movements. This article constitutes a comprehensive review summarizing key findings on the CPG as well as on its potential role in Restless Leg Syndrome (RLS, Periodic Leg Movement (PLM, and Alternating Leg Muscle Activation (ALMA. Special attention will be paid to the role of the CPG in a recently identified, and uniquely different neurological disorder, called the Uner Tan Syndrome.

  4. Central pattern generator for locomotion: anatomical, physiological, and pathophysiological considerations.

    Science.gov (United States)

    Guertin, Pierre A

    2012-01-01

    This article provides a perspective on major innovations over the past century in research on the spinal cord and, specifically, on specialized spinal circuits involved in the control of rhythmic locomotor pattern generation and modulation. Pioneers such as Charles Sherrington and Thomas Graham Brown have conducted experiments in the early twentieth century that changed our views of the neural control of locomotion. Their seminal work supported subsequently by several decades of evidence has led to the conclusion that walking, flying, and swimming are largely controlled by a network of spinal neurons generally referred to as the central pattern generator (CPG) for locomotion. It has been subsequently demonstrated across all vertebrate species examined, from lampreys to humans, that this CPG is capable, under some conditions, to self-produce, even in absence of descending or peripheral inputs, basic rhythmic, and coordinated locomotor movements. Recent evidence suggests, in turn, that plasticity changes of some CPG elements may contribute to the development of specific pathophysiological conditions associated with impaired locomotion or spontaneous locomotor-like movements. This article constitutes a comprehensive review summarizing key findings on the CPG as well as on its potential role in Restless Leg Syndrome, Periodic Leg Movement, and Alternating Leg Muscle Activation. Special attention will be paid to the role of the CPG in a recently identified, and uniquely different neurological disorder, called the Uner Tan Syndrome.

  5. Experimental Neuromyelitis Optica Induces a Type I Interferon Signature in the Spinal Cord

    DEFF Research Database (Denmark)

    Oji, Satoru; Nicolussi, Eva-Maria; Kaufmann, Nathalie;

    2016-01-01

    Neuromyelitis optica (NMO) is an acute inflammatory disease of the central nervous system (CNS) which predominantly affects spinal cord and optic nerves. Most patients harbor pathogenic autoantibodies, the so-called NMO-IgGs, which are directed against the water channel aquaporin 4 (AQP4) on astr......Neuromyelitis optica (NMO) is an acute inflammatory disease of the central nervous system (CNS) which predominantly affects spinal cord and optic nerves. Most patients harbor pathogenic autoantibodies, the so-called NMO-IgGs, which are directed against the water channel aquaporin 4 (AQP4...... not profit from such treatments. How is I-IFN involved in NMO pathogenesis? To address this question, we made gene expression profiles of spinal cords from Lewis rat models of experimental neuromyelitis optica (ENMO) and experimental autoimmune encephalomyelitis (EAE). We found an upregulation of I...

  6. Auriculotemporal Syndrome (Frey Syndrome).

    Science.gov (United States)

    Motz, Kevin M; Kim, Young J

    2016-04-01

    Frey syndrome is a common sequela of parotidectomy, and although it is not frequently manifested clinically, it can cause significant morbidity for those affected. Frey syndrome results from synkinetic autonomic reinnervation by transected postganglionic parasympathetic nerve fiber within the parotid gland to the overlying sweat glands of the skin. Many surgical techniques have been proposed to prevent the development of Frey syndrome. For those who develop clinical symptoms of Frey syndrome, objective testing can be performed with a Minor starch-iodine test. Some of the current methods to prevent and treat symptomatic Frey syndrome are reviewed. PMID:26902982

  7. Bancos de cordón umbilical Umbilical cord banks

    Directory of Open Access Journals (Sweden)

    L. Madero

    2009-01-01

    Full Text Available La utilización de sangre de cordón como fuente de precursores hematológicos se remonta a 1983 cuando Boyse apuntó el potencial en progenitores existente en la sangre de cordón, realizándose un año más tarde las primeras experiencias sobre modelos murinos. Tuvieron que pasar más de cinco años para que Gluckman realizara la primera experiencia en humanos. Un niño afecto de anemia de Fanconi fue trasplantado con progenitores de sangre de cordón umbilical de su hermana HLA idéntica, realizándose todos los estudios de compatibilidad intraútero. Actualmente, veinte años más tarde, el paciente se encuentra libre de enfermedad y con la hematopoyesis del donante, demostrándose así la capacidad de persistencia del injerto a largo plazo.

  8. Neuromodulation for Refractory Failed Back Surgery Syndrome Patients

    OpenAIRE

    I.N. Morozov; А.I. Ushakov

    2015-01-01

    The aim of the investigation was to assess the capabilities of spinal cord stimulation, and compare it with conventional nonsurgical treatment in failed back syndrome. Materials and Methods. The study involved the patients with at least one anatomically successful lumber disc hernia excision, post-traumatic spinal deformity in their past history, the patients keeping suffering from neuropathic pain. Group 1 patients (n=45) in addition to traditional therapy underwent spinal cord stimu...

  9. Langtidsbehandling med spinal cord-stimulation hos en patient med kronisk regionalt smertesyndrom type 1 og fantomsmerter efter amputation

    DEFF Research Database (Denmark)

    Enggaard, Thomas P.; Scherer, Christian; Nikolajsen, Lone;

    2008-01-01

    The development of stump and phantom pain after limb amputation in patients with complex regional pain syndrome (CRPS) is very frequent. Stump pain is typically recurred CRPS and the possibilities for effective pharmacological pain relief are often limited. Spinal cord stimulation (SCS) has a wel...

  10. A 2-center Comparative Study on Tonic Versus Burst Spinal Cord Stimulation: Amount of Responders and Amount of Pain Suppression

    NARCIS (Netherlands)

    Ridder, D.M.D.; Lenders, M.W.P.M.; Vos, de C.C.; Dijkstra-Scholten, C.R.N.; Wolters, R.R.N.; Vancamp, T.P.T.; Van Looy, P.R.N.; Van Havenbergh, T.M.D.; Vanneste, S.

    2015-01-01

    Introduction: Spinal cord stimulation is a safe and effective procedure applied for medically intractable neuropathic pain and failed back surgery syndrome. Recently, a novel stimulation paradigm was developed, called burst stimulation consisting of intermittent packets of closely spaced high-freque

  11. Spinal-, brainstem- and cerebrally mediated responses at- and below-level of a spinal cord contusion in rats: evaluation of pain-like behavior.

    Science.gov (United States)

    Baastrup, Cathrine; Maersk-Moller, Camilla Charlotte; Nyengaard, Jens Randel; Jensen, Troels Staehelin; Finnerup, Nanna Brix

    2010-12-01

    Pain is a frequent consequence of spinal cord injury (SCI) which may profoundly impair the patients' quality of life. Valid experimental models and methods are therefore desirable in the search for better treatments. Usually, experimental pain assays depend on stimulus-evoked withdrawal responses; however, this spinal-mediated reflex response may be particularly problematic when evaluating below-level SCI pain due to the development of hyperactive reflex circuitries. In this study, we applied and compared assays measuring cold (acetone), static (von Frey filaments), and dynamic mechanical (soft brush) hypersensitivity at different levels of the neuroaxis at and below the level of injury in a rat model of SCI. We induced an experimental SCI (MASCIS 25 mm weight-drop) and evaluated the development of spinal reflexes (withdrawal), spinal-brainstem-spinal reflexes (licking, guarding, struggling, vocalizing, jumping, and biting) and cerebral-dependent behavior (place escape/avoidance paradigm (PEAP)). We demonstrated increased brainstem reflexes and cerebrally mediated aversive reactions to stimuli applied at the level of SCI, suggesting development of at-level evoked pain behavior. Furthermore, stimulation below-level increased innate reflex responses without increasing brainstem reflexes or aversive behavior in the PEAP, suggesting development of the spasticity syndrome rather than pain-like behavior. While spinal reflex measures are acceptable for studying changes in the spinal reflex pathways and spinal cord, they are not suited as nociceptive behavioral measures. Measuring brainstem organized responses eliminates the bias associated with the spastic syndrome, but pain requires cortical involvement. Methods depending on cortical structures, as the PEAP, are therefore optimal endpoints in animal models of central pain. PMID:20863621

  12. The pathophysiology of restless legs syndrome

    International Nuclear Information System (INIS)

    Restless legs syndrome (RLS) is a sensorimotor disorder that is frequently associated with periodic leg movements (PLMS). RLS is generally considered to be a central nervous system (CNS)-related disorder although no specific lesion has been found to be associated with the syndrome. Reduced intracortical inhibition has been demonstrated in RLS by transcranial magnetic stimulation. Some MRI studies have revealed the presence of morphologic changes in the somatosensory cortex, motor cortex and thalamic gray matter. The results of single photon emission computed tomography (SPECT) and positron emission tomography (PET) studies showed that the limbic and opioid systems also play important roles in the pathophysiology of RLS. A functional MRI study revealed abnormal bilateral cerebellar and thalamic activation during the manifestation of sensory symptoms, with additional red nucleus and reticular formation activity during PLMS. PLMS is likely to occur in patients with spinal cord lesions, and some patients with sensory polyneuropathy may exhibit RLS symptoms. RLS symptoms seem to depend on abnormal spinal sensorimotor integration at the spinal cord level and abnormal central somatosensory processing. PLMS appears to depend on increased excitability of the spinal cord and a decreased supraspinal inhibitory mechanism from the A11 diencephalic dopaminergic system. RLS symptoms respond very dramatically to dopaminergic therapy. The results of analysis by PET and SPECT studies of striatal D2 receptor binding in humans are inconclusive. However, studies in animal models suggest that the participation of the A11 dopaminergic system and the D3 receptor in RLS symptoms. The symptoms of RLS are aggravated in those with iron deficiency, and iron treatment ameliorates the symptoms in some patients. Neuroimaging studies, analysis of the cerebrospinal fluid, and studies on postmortem tissue and use of animal models have indicated that low brain iron concentrations and dysfunction of

  13. Establishment and evaluation of a rat model of complete transected spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Xuejun Li; Chunhai Huang; Shangming Liu; Xianrui Yuan

    2008-01-01

    BACKGROUND: The establishment of a rat model of complete transected spinal cord injury lacks technological specifications. The current models lack concordance and reliability, and the death rate of the experimental animals is high. Therefore, there is a great need for a reliable model to apply clinical applications of therapy.OBJECTIVE: To construct a rat model of complete transected spinal cord injury characterized by stability, reproducibility, and a high animal survival rate. DESIGN: Completely randomized controlled study.SETTING: Department of Neurosurgery, Xiangya Hospital of Central South University.MATERIALS: Fifty-five healthy specific pathogen free grade adult female Sprague Dawley rats were provided by the Experimental Animal Department, Xiangya Medical College, Central South University. Olympus BX51 imaging collecting analytic system was provided by Olympus Company, Japan; and SEN-7203 Nihon-Kohden electrical stimulator by Nihon Kohden, Japan. METHODS: This study was performed at the Laboratory of Neurosurgery, Xiangya Hospital of Central South University from April to June 2006. Experimental grouping: 55 rats were randomly divided into model group (n = 40) and sham surgery group (n = 15). In the model group, a self-made sliver hook was passed through the ventral side to support the spinal cord at the T12 segment and to shear it off. A complete transected spinal cord, 2 mm in length, was resected. In the sham surgery group, the spinal cord was identically exposed. The dura mater of the spinal cord was cut open, but the spinal cord was not damaged. MAIN OUTCOME MEASURES: Histopathological changes after spinal cord injury at L2 segment were observed subsequent to hematoxylin and eosin staining under optical microscopy. Olympus BX51 imaging collecting analytic system was used to count spinal cord ventral horn neurons. Motor function of rat hindlimb was evaluated with the Basso, Beattie and Bresnahan (BBB) scale. Paraplegia was evaluated as 0 point, and

  14. Vascularized peripheral nerve trunk autografted in the spinal cord: a new experimental model in adult rats

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective: To investigate the effect of vascularized peripheral nerve trunk autografted in spinal cord. Methods: With modern microsurgical technique,vascularized peripheral median and ulnar nerve trunk autografted in the upper thoracic region of the spinal cord were established in 20 female adult rats. The origin and the termination of axons in the graft were studied by retrograde neuronal labeling with horseradish peroxidase (HRP).Cord, nerve grafts and some normal median and ulnar nerves in the right upper limb were removed and sectioned for Bielschowsky's silver stain and haematoxylin and eosin (H&E) stain. Light and electron microscopic examination and electrophysiological examination were applied.Results: The grafts were innervated by many new fibers. Studies with HRP indicated that new axons in graft were originated from intrinsic central nervous system (CNS) neurons with their cell bodies from brain stem to sacral segments of spinal cord. Other axons arose from dorsal root ganglia at the level of graft and at least 19 distal segments to them. Together with electron microscopy, electrophysiological examination, silver and H&E stain, the results demonstrated that vascularized peripheral nerve trunk grafted in spinal cord attracted many neurons to grow into the nerve grafts.Conclusions: The findings implicate that CNS is able to regenerate much better in vascularized nerve autografted in spinal cord.

  15. Religious perspectives on umbilical cord blood banking.

    Science.gov (United States)

    Jordens, Christopher F C; O'Connor, Michelle A C; Kerridge, Ian H; Stewart, Cameron; Cameron, Andrew; Keown, Damien; Lawrence, Rabbi Jeremy; McGarrity, Andrew; Sachedina, Abdulaziz; Tobin, Bernadette

    2012-03-01

    Umbilical cord blood is a valuable source of haematopoietic stem cells. There is little information about whether religious affiliations have any bearing on attitudes to and decisions about its collection, donation and storage. The authors provided information about umbilical cord blood banking to expert commentators from six major world religions (Catholicism, Anglicanism, Islam, Judaism, Hinduism and Buddhism) and asked them to address a specific set of questions in a commentary. The commentaries suggest there is considerable support for umbilical cord blood banking in these religions. Four commentaries provide moral grounds for favouring public donation over private storage. None attach any particular religious significance to the umbilical cord or to the blood within it, nor place restrictions on the ethnicity or religion of donors and recipients. Views on ownership of umbilical cord blood vary. The authors offer a series of general points for those who seek a better understanding of religious perspectives on umbilical cord blood banking. PMID:22558902

  16. Spinal cord ependymoma presenting with neurological deficits in the setting of trauma.

    Science.gov (United States)

    Saad, Amin F; Nickell, Larry T; Finn, S Sam; Opatowsky, Michael J

    2014-07-01

    Ependymomas represent 4% of all primary central nervous system neoplasms in adults, with 30% occurring in the spinal cord. We describe a young man with neurological deficits following a motor vehicle accident who was found to have an intramedullary cervicothoracic ependymoma.

  17. Astrocytoma with involvement of medulla oblongata, spinal cord and spinal nerves in a raccoon (Procyon lotor)

    Science.gov (United States)

    Neoplasms affecting the central and peripheral nervous systems of wild animals are extremely rare. Described are clinical signs, pathologic and immunohistochemical findings in an adult female raccoon (Procyon lotor) with an astrocytoma which involved brainstem, cervical spinal cord and roots of the ...

  18. Activity-Based Restorative Therapies: Concepts and Applications in Spinal Cord Injury-Related Neurorehabilitation

    Science.gov (United States)

    Sadowsky, Cristina L.; McDonald, John W.

    2009-01-01

    Physical rehabilitation following spinal cord injury-related paralysis has traditionally focused on teaching compensatory techniques, thus enabling the individual to achieve day-to-day function despite significant neurological deficits. But the concept of an irreparable central nervous system (CNS) is slowly being replaced with evidence related to…

  19. Edwards syndrome: prenatal diagnosis by ultrasound

    International Nuclear Information System (INIS)

    Edwards syndrome is the second most common trisomy syndrome occurring in 0.3/1,000 births. The prenatal diagnosis of this trisomy may be suspected in the routine ultrasound exam of the pregnancy by means of the recognition of a constellation of findings: fetal structural anomalies, intrauterine growth retardation (CIR) and umbilical cord or amniotic fluid volume abnormalities. The diagnosis is then confirmed by chromosome analysis of either amniotic fluid or fetal blood or tissue. We present six cases of Edwards syndrome suspected by fetal ultrasonography exam, all of them confirmed by karyotype, describing the major sonographic findings. (Author) 15 refs

  20. Role of plasma membrane calcium ATPase 2 in spinal cord pathology

    Institute of Scientific and Technical Information of China (English)

    Amanda; Kathleen; Fakira; Stella; Elkabes

    2010-01-01

    A number of studies have indicated that plasma membrane calcium ATPases(PMCAs) are expressed in the brain and spinal cord and could play important roles not only in the maintenance of cellular calcium homeostasis but also in the survival and function of central nervous system cells under pathological conditions.The different regional and cellular distributions of the various PMCA isoforms and splice variants in the nervous system and the diverse phenotypes of PMCA knockout mice support the notion that each isoform might play a distinct role. Especially in the spinal cord,the survival of neurons and,in particular,motor neurons could be dependent on PMCA2.This is indicated by the knockdown of PMCA2 in pure spinal cord neuronal cultures that leads to cell death via a decrease in collapsing response mediator protein 1 levels.Moreover,the progressive decline in the number of motor neurons in PMCA2-null mice andheterozygous mice further supports this notion.Therefore,the reported reduction in PMCA2 mRNA and protein levels in the inflamed spinal cord of mice affected by experimental autoimmune encephalomyelitis(EAE) ,an animal model of multiple sclerosis,and after spinal cord contusion injury,suggests that changes in PMCA2 expression could be a cause of neuronal pathology and death during inflammation and injury.Glutamate excitotoxicity mediated via kainate receptors has been implicated in the neuropathology of both EAE and spinal cord injury,and has been identified as a trigger that reduces PMCA2 levels in pure spinal cord neuronal cultures through degradation of the pump by calpain without affecting PMCA2 transcript levels.It remains to be determined which other stimuli modulate PMCA2 mRNA expression in the aforementioned pathological conditions of the spinal cord.

  1. Transplantation of Neural Stem Cells Cultured in Alginate Scaffold for Spinal Cord Injury in Rats

    Science.gov (United States)

    Sharafkhah, Ali; Koohi-Hosseinabadi, Omid; Semsar-Kazerooni, Maryam

    2016-01-01

    Study Design This study investigated the effects of transplantation of alginate encapsulated neural stem cells (NSCs) on spinal cord injury in Sprague-Dawley male rats. The neurological functions were assessed for 6 weeks after transplantation along with a histological study and measurement of caspase-3 levels. Purpose The aim of this study was to discover whether NSCs cultured in alginate transplantation improve recovery from spinal cord injury. Overview of Literature Spinal cord injury is one of the leading causes of disability and it has no effective treatment. Spinal cord injury can also cause sensory impairment. With an impetus on using stem cells therapy in various central nervous system settings, there is an interest in using stem cells for addressing spinal cord injury. Neural stem cell is one type of stem cells that is able to differentiate to all three neural lineages and it shows promise in spinal injury treatment. Furthermore, a number of studies have shown that culturing NSCs in three-dimensional (3D) scaffolds like alginate could enhance neural differentiation. Methods The NSCs were isolated from 14-day-old rat embryos. The isolated NSCs were cultured in growth media containing basic fibroblast growth factor and endothelial growth factor. The cells were characterized by differentiating to three neural lineages and they were cultured in an alginate scaffold. After 7 days the cells were encapsulated and transplanted in a rat model of spinal cord injury. Results Our data showed that culturing in an alginate 3D scaffold and transplantation of the NSCs could improve neurological outcome in a rat model of spinal cord injury. The inflammation scores and lesion sizes and also the activity of caspase-3 (for apoptosis evaluation) were less in encapsulated neural stem cell transplantation cases. Conclusions Transplantation of NSCs that were cultured in an alginate scaffold led to a better clinical and histological outcome for recovery from spinal cord injury in

  2. Nanomedicine for Treating Spinal Cord Injury

    OpenAIRE

    Tyler, Jacqueline Y.; Xu, Xiao-Ming; Cheng, Ji-Xin

    2013-01-01

    Spinal cord injury results in significant mortality and morbidity, lifestyle changes, and difficult rehabilitation. Treatment of spinal cord injury is challenging because the spinal cord is both complex to treat acutely and difficult to regenerate. Nanomaterials can be used to provide effective treatments; their unique properties can facilitate drug delivery to the injury site, enact as neuroprotective agents, or provide platforms to stimulate regrowth of damaged tissues. We review recent use...

  3. KBG syndrome

    Directory of Open Access Journals (Sweden)

    Brancati Francesco

    2006-12-01

    Full Text Available Abstract KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG anomalies (with or without seizures and abnormal hair implantation. Cutaneous syndactyly, webbed short neck, cryptorchidism, hearing loss, palatal defects, strabismus and congenital heart defects are less common findings. Autosomal dominant transmission has been observed in some families, and it is predominantly the mother, often showing a milder clinical picture, that transmits the disease. The diagnosis is currently based solely on clinical findings as the aetiology is unknown. The final diagnosis is generally achieved after the eruption of upper permanent central incisors at 7–8 years of age when the management of possible congenital anomalies should have been already planned. A full developmental assessment should be done at diagnosis and, if delays are noted, an infant stimulation program should be initiated. Subsequent management and follow-up should include an EEG, complete orthodontic evaluation, skeletal investigation with particular regard to spine curvatures and limb asymmetry, hearing testing and ophthalmologic assessment.

  4. Multifocal Dysembryoplastic Neuroepithelial Tumour with Intradural Spinal Cord Lipomas: Report of a Case

    Directory of Open Access Journals (Sweden)

    Richard D. White

    2011-01-01

    Full Text Available We report a case of temporal lobe epilepsy and incomplete Brown-Sequard syndrome of the thoracic cord. Computed tomography and magnetic resonance (MR imaging showed multiple supratentorial masses with the classical radiological appearances of multifocal dysembryoplastic neuroepithelial tumour (DNET. Spinal MR imaging revealed intradural lipomas, not previously reported in association with multifocal DNET. Presentation and imaging findings are discussed along with classification and natural history of the tumour.

  5. Spinal cord compression due to ethmoid adenocarcinoma.

    Science.gov (United States)

    Johns, D R; Sweriduk, S T

    1987-10-15

    Adenocarcinoma of the ethmoid sinus is a rare tumor which has been epidemiologically linked to woodworking in the furniture industry. It has a low propensity to metastasize and has not been previously reported to cause spinal cord compression. A symptomatic epidural spinal cord compression was confirmed on magnetic resonance imaging (MRI) scan in a former furniture worker with widely disseminated metastases. The clinical features of ethmoid sinus adenocarcinoma and neoplastic spinal cord compression, and the comparative value of MRI scanning in the neuroradiologic diagnosis of spinal cord compression are reviewed.

  6. Cellular Scaling Rules for Primate Spinal Cords

    OpenAIRE

    Burish, Mark J.; Peebles, J. Klint; Baldwin, Mary K.; Tavares, Luciano; Kaas, Jon H.; Herculano-Houzel, Suzana

    2010-01-01

    The spinal cord can be considered a major sensorimotor interface between the body and the brain. How does the spinal cord scale with body and brain mass, and how are its numbers of neurons related to the number of neurons in the brain across species of different body and brain sizes? Here we determine the cellular composition of the spinal cord in eight primate species and find that its number of neurons varies as a linear function of cord length, and accompanies body mass raised to an expone...

  7. Mutations in CUL4B, which encodes a ubiquitin E3 ligase subunit, cause an X-linked mental retardation syndrome associated with aggressive outbursts, seizures, relative macrocephaly, central obesity, hypogonadism, pes cavus, and tremor.

    Science.gov (United States)

    Tarpey, Patrick S; Raymond, F Lucy; O'Meara, Sarah; Edkins, Sarah; Teague, Jon; Butler, Adam; Dicks, Ed; Stevens, Claire; Tofts, Calli; Avis, Tim; Barthorpe, Syd; Buck, Gemma; Cole, Jennifer; Gray, Kristian; Halliday, Kelly; Harrison, Rachel; Hills, Katy; Jenkinson, Andrew; Jones, David; Menzies, Andrew; Mironenko, Tatiana; Perry, Janet; Raine, Keiran; Richardson, David; Shepherd, Rebecca; Small, Alexandra; Varian, Jennifer; West, Sofie; Widaa, Sara; Mallya, Uma; Moon, Jenny; Luo, Ying; Holder, Susan; Smithson, Sarah F; Hurst, Jane A; Clayton-Smith, Jill; Kerr, Bronwyn; Boyle, Jackie; Shaw, Marie; Vandeleur, Lucianne; Rodriguez, Jayson; Slaugh, Rachel; Easton, Douglas F; Wooster, Richard; Bobrow, Martin; Srivastava, Anand K; Stevenson, Roger E; Schwartz, Charles E; Turner, Gillian; Gecz, Jozef; Futreal, P Andrew; Stratton, Michael R; Partington, Michael

    2007-02-01

    We have identified three truncating, two splice-site, and three missense variants at conserved amino acids in the CUL4B gene on Xq24 in 8 of 250 families with X-linked mental retardation (XLMR). During affected subjects' adolescence, a syndrome emerged with delayed puberty, hypogonadism, relative macrocephaly, moderate short stature, central obesity, unprovoked aggressive outbursts, fine intention tremor, pes cavus, and abnormalities of the toes. This syndrome was first described by Cazebas et al., in a family that was included in our study and that carried a CUL4B missense variant. CUL4B is a ubiquitin E3 ligase subunit implicated in the regulation of several biological processes, and CUL4B is the first XLMR gene that encodes an E3 ubiquitin ligase. The relatively high frequency of CUL4B mutations in this series indicates that it is one of the most commonly mutated genes underlying XLMR and suggests that its introduction into clinical diagnostics should be a high priority. PMID:17236139

  8. Vocal Cord Paralysis and its Etiologies: A Prospective Study

    OpenAIRE

    Seyed Javad Seyed Toutounchi; Mahmood Eydi; Samad EJ Golzari; Mohammad Reza Ghaffari; Nashmil Parvizian

    2014-01-01

    Introduction: Vocal cord paralysis is a common symptom of numerous diseases and it may be due to neurogenic or mechanical fixation of the cords. Paralysis of the vocal cords is just a symptom of underlying disease in some cases; so, clinical diagnosis of the underlying cause leading to paralysis of the vocal cords is important. This study evaluates the causes of vocal cord paralysis. Methods: In a prospective study, 45 patients with paralyzed vocal cord diagnosis were examined by tests such a...

  9. Effects of electroacupuncture on c-Fos expression in the spinal cord and brain of rats with chronic visceral hypersensitivity

    Institute of Scientific and Technical Information of China (English)

    Xiaomei Wang; Huirong Liu; Guanghong Ding; Yunfei Chen; Huangan Wu; Na Li; Enhua Zhou; Xiudi Qin; Lingsong Yuan

    2009-01-01

    BACKGROUND: Visceral hypersensitivity is the main cause of irritable bowel syndrome, c-Fos is a marker of visceral hypersensitivity in the central nervous system. Electroacupuncture can relieve chronic visceral hypersensitivity in rats, but the mechanism is still unknown.OBJECTIVE: To identify c-Fos expression in the spinal cord and cerebral cortex of rats with chronic visceral hypersensitivity, and to test the effects of electroacupuncture on pain sensitivity in rats with chronic visceral hypersensitivity.DESIGN, TIME AND SETTING: A randomized controlled animal experiment was performed at the Animal Experimental Center, Shanghai University of Traditional Chinese Medicine, from January to April, 2007.MATERIALS: A total of 24 neonatal, male, Sprague Dawley rats, aged five days old, were equally and randomly assigned into a normal group, a model group, and an electroacupuncture group. Rabbit anti-rat c-Fos antibody and Evision secondary antibody kits (Sigma, USA), diaminobenzidine kit (Dako, Denmark), and an LD202H electroacupuncture apparatus (Huawei, Beijing, China) were used in this study.METHODS: Neonatal rats from the model and electroacupuncture groups were used to establish rat models of chronic visceral hypersensitivity by the saccule stimulation method. After model establishment, 0.25 mm diameter electric needles were inserted into Tianshu (ST 25) and Shangjuxu (ST37) at a depth of approximately 0.5 cm, with an square wave (alternating current frequency at 100/20 Hz, amplitude ranged 0.2-0.6 ms, intensify at 1 mA) once for 20 minutes, once a day, for seven days. Rats in the normal and model groups were not treated.MAIN OUTCOME MEASURES: Following 7 days of treatment, c-Fos expression in the spinal cord and cerebral cortex was detected by immunohistochemistry. After the first electroacupuncture treatment, abdominal withdrawal reflex scores were investigated to evaluate the pain threshold for chronic visceral hypersensitivity in rats.RESULTS: Visceral

  10. High-fat simple carbohydrate feeding impairs central and peripheral monoamine metabolic pathway triggering the onset of metabolic syndrome in C57Bl/6J mice

    Directory of Open Access Journals (Sweden)

    Serena S D'Souza

    2016-01-01

    Conclusion: HFSC diet impairs the central and peripheral dopaminergic and noradrenergic pathways in mice as evidenced by the disturbances in their hypothalamic, plasma, and urine levels and this might be one of the early factors contributing towards the development of the MetS.

  11. Central Diabetes Insipidus after Staged Spinal Surgery

    OpenAIRE

    Rosenbaum, Benjamin P.; Steinmetz, Michael P.

    2013-01-01

    Diabetes insipidus (DI) is described following penetrating spinal cord trauma but rarely following instrumented spinal fusion. More commonly, hyponatremia is seen following spine surgery, which may be iatrogenic, attributed to the syndrome of inappropriate antidiuretic hormone release. The authors present a case of a 57-year-old woman who underwent a planned two-stage operation for scoliotic deformity correction. On the third postoperative day, the patient developed hypernatremia (sodium leve...

  12. Gorlin syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Patil K

    2005-01-01

    Full Text Available Gorlin syndrome is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. It is characterized mainly by Basal cell carcinomas, Odontogenic keratocysts and skeletal anomalies. However, medical literature documents both common and lesser known manifestations of the disorder involving the skin, central nervous system, skeletal system etc. Diagnosis of the syndrome in childhood is basically through oral abnormalities. A case of Gorlin syndrome has been reported here, with review of literature.

  13. Genetics Home Reference: Noonan syndrome

    Science.gov (United States)

    ... article on PubMed Central Rohrer T. Noonan syndrome: introduction and basic clinical features. Horm Res. 2009 Dec; ... Library of Medicine Lister Hill National Center for Biomedical Communications 8600 Rockville Pike, Bethesda, MD 20894, USA ...

  14. Plasticity and Recovery After Dorsal Column Spinal Cord Injury in Nonhuman Primates.

    Science.gov (United States)

    Reed, Jamie L; Liao, Chia-Chi; Qi, Hui-Xin; Kaas, Jon H

    2016-01-01

    Here, we review recent work on plasticity and recovery after dorsal column spinal cord injury in nonhuman primates. Plasticity in the adult central nervous system has been established and studied for the past several decades; however, capacities and limits of plasticity are still under investigation. Studies of plasticity include assessing multiple measures before and after injury in animal models. Such studies are particularly important for improving recovery after injury in patients. In summarizing work by our research team and others, we suggest how the findings from plasticity studies in nonhuman primate models may affect therapeutic interventions for conditions involving sensory loss due to spinal cord injury. PMID:27578996

  15. Miliary Tuberculosis with Concurrent Brain and Spinal Cord Involvement: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Sung, Chang Keun; Na, Hyoung Il; Yu, Hyeon; Byun, Jun Soo; Youn, Young Chul; Seo, Jae Seung; Kim, Gi Hyeon [Chung-Ang University, Seoul (Korea, Republic of)

    2008-11-15

    Central nervous system involvement by tuberculosis is rare, and intramedullary involvement is even more rare. A patient that developed intermittent amnesia during anti-tuberculous therapy underwent brain CT and MRI and spine MRI. The latter showed multiple small enhancing nodules in the brain and spinal cord. The patient was treated with anti-tuberculous medication and steroids under the suspected diagnosis of miliary tuberculosis. Follow-up CT showed decreased nodule size and number. We report a case of miliary tuberculosis in the brain and spinal cord and present a review of the literature related to similar cases.

  16. Noninvasive transcutaneous bionic baroreflex system prevents severe orthostatic hypotension in patients with spinal cord injury.

    Science.gov (United States)

    Yoshida, Masayoshi; Murayama, Yoshinori; Chishaki, Akiko; Sunagawa, Kenji

    2008-01-01

    Central baroreflex failure in patients with spinal cord injury results in serious orthostatic hypotension. We examined if transcutaneous electrical stimulation regulates arterial pressure in those patients. We identified skin regions capable of increasing arterial pressure and determined respective transfer function. Using the transfer function, we designed the feedback regulator (i.e., bionic baroreflex system) to control arterial pressure. Orthostatic stress decreased arterial pressure profoundly. Activation of bionic regulator restored and maintained arterial pressure at pre-specified levels. We conclude that the transcutaneous bionic system is noninvasive and capable of stabilizing arterial pressure in patients with spinal cord injury.

  17. Bi Syndrome (Arthralgia Syndrome)

    Institute of Scientific and Technical Information of China (English)

    ZHANG En-qin

    2010-01-01

    @@ The word 'Bi' (痹) in Chinese means an obstruction.Bi Syndrome refers the syndrome characterized by the obstruction of qi and blood in the meridians due to the invasion of external pathogenic wind, cold and dampness, manifested as soreness, pain, numbness,heavy sensation, swelling of joints and limbs, limitation of movements and so on.

  18. Dental approach to craniofacial syndromes

    DEFF Research Database (Denmark)

    Kjær, Inger

    2012-01-01

    is essential for insight into craniofacial syndromes. The dentition, thus, becomes central in diagnostics and evaluation of the pathogenesis. Developmental fields can explore and advance the concept of dental approaches to craniofacial syndromes. Discussion. As deviations in teeth persist and do not reorganize...

  19. Atypical presentations of Wolframs syndrome

    Directory of Open Access Journals (Sweden)

    S Saran

    2012-01-01

    Full Text Available Background: Wolfram syndrome is a rare hereditary or sporadic neurodegenerative disorder also known as DIDMOAD. The classically described presentation is of insulin-dependent diabetes, followed by optic atrophy, central diabetes insipidus, and sensory neural deafness. Also included are less well-described presentations of Wolframs syndrome. We here present three cases of atypical presentation of this syndrome. Case 1: A 15-year-old boy with insulin-dependent diabetes was presented for evaluation of depressive symptoms associated with suicidal tendency. Neuropsychiatric manifestations are described with Wolframs syndrome, and wolframin gene, in recessive inheritance, is associated with psychiatric illnesses without other manifestations of Wolframs syndrome. Case 2: A 17-year-old diabetic boy on insulin with good control of blood sugar presented for evaluation of delayed puberty. Central hypogonadism and other anterior pituitary hormone dysfunctions are the less publicized hormone dysfunctions in Wolframs syndrome. Case 3: A 23-year-old female who was on insulin for diabetes for the past 14 years, got admitted for evaluation of sudden loss of vision. This patient had developed a vitreous hemorrhage and, on evaluation, was found to have optic atrophy, sensory neural hearing loss, and diabetes insipidus, and presented differently from the gradual loss of vision described in Wolframs syndrome. Conclusion: Wolframs syndrome being a multisystem degenerative disorder can have myriad other manifestations than the classically described features. Neuropsychiatric manifestations, depression with suicidal risk, central hypogonadism, and secondary adrenal insufficiency are among the less well-described manifestations of this syndrome.

  20. Ambulation and spinal cord injury.

    Science.gov (United States)

    Hardin, Elizabeth C; Kobetic, Rudi; Triolo, Ronald J

    2013-05-01

    Walking is possible for many patients with a spinal cord injury. Avenues enabling walking include braces, robotics and FES. Among the benefits are improved musculoskeletal and mental health, however unrealistic expectations may lead to negative changes in quality of life. Use rigorous assessment standards to gauge the improvement of walking during the rehabilitation process, but also yearly. Continued walking after discharge may be limited by challenges, such as lack of accessibility in and outside the home, and complications, such as shoulder pain or injuries from falls. It is critical to determine the risks and benefits of walking for each patient.

  1. Large-scale synchronized activity in the embryonic brainstem and spinal cord

    Directory of Open Access Journals (Sweden)

    Yoko eMomose-Sato

    2013-04-01

    Full Text Available In the developing central nervous system, spontaneous activity appears well before the brain responds to external sensory inputs. One of the earliest activities is observed in the hindbrain and spinal cord, which is detected as rhythmic electrical discharges of cranial and spinal motoneurons or oscillations of Ca2+- and voltage-related optical signals. Shortly after the initial expression, the spontaneous activity appearing in the hindbrain and spinal cord exhibits a large-scale correlated wave that propagates over a wide region of the central nervous system, maximally extending to the lumbosacral cord and to the forebrain. In this review, we describe several aspects of this synchronized activity by focusing on the basic properties, development, origin, propagation pattern, pharmacological characteristics, and possible mechanisms underlying the generation of the activity. These profiles differ from those of the respiratory and locomotion pattern generators observed in the mature brainstem and spinal cord, suggesting that the wave is primordial activity that appears during a specific period of embryonic development and plays some important roles in the development of the central nervous system.

  2. [Von Hippel-Lindau syndrome].

    Science.gov (United States)

    Reich, H; Hollwich, F

    1984-06-01

    The von Hippel-Lindau syndrome is an autosomal dominant condition that comprises, apart from angiomas of the retina, the cerebellum, the spinal cord, and the cerebrum, also cystic and blastomatous dysplasias resulting from maldevelopment, namely cystic kidney and pancreas, hypernephroma, and pheochromocytoma. Early observers of the syndrome were the English neurologist John Hughlings Jackson (1872) and the German ophthalmologist Hugo Magnus (1874). The typical association of angiomas of the retina with the cerebellum was first described in 1905 by the Prague ophthalmologist Wilhelm Czermak, long before Lindau (1926). The fact that hypernephromas and pheochromocytomas may form parts of it characterizes the syndrome as a polyneoplastic hereditary disease and the sufferers as members of families at risk. Since the ophthalmologist is often the first to recognize this disease by direct inspection of the fundi, he is responsible for ensuring proper medical care for the affected person and his or her entire family.

  3. Peripheral nerve hyperexcitability syndromes.

    Science.gov (United States)

    Küçükali, Cem Ismail; Kürtüncü, Murat; Akçay, Halil İbrahim; Tüzün, Erdem; Öge, Ali Emre

    2015-01-01

    Peripheral nerve hyperexcitability (PNH) syndromes can be subclassified as primary and secondary. The main primary PNH syndromes are neuromyotonia, cramp-fasciculation syndrome (CFS), and Morvan's syndrome, which cause widespread symptoms and signs without the association of an evident peripheral nerve disease. Their major symptoms are muscle twitching and stiffness, which differ only in severity between neuromyotonia and CFS. Cramps, pseudomyotonia, hyperhidrosis, and some other autonomic abnormalities, as well as mild positive sensory phenomena, can be seen in several patients. Symptoms reflecting the involvement of the central nervous system occur in Morvan's syndrome. Secondary PNH syndromes are generally seen in patients with focal or diffuse diseases affecting the peripheral nervous system. The PNH-related symptoms and signs are generally found incidentally during clinical or electrodiagnostic examinations. The electrophysiological findings that are very useful in the diagnosis of PNH are myokymic and neuromyotonic discharges in needle electromyography along with some additional indicators of increased nerve fiber excitability. Based on clinicopathological and etiological associations, PNH syndromes can also be classified as immune mediated, genetic, and those caused by other miscellaneous factors. There has been an increasing awareness on the role of voltage-gated potassium channel complex autoimmunity in primary PNH pathogenesis. Then again, a long list of toxic compounds and genetic factors has also been implicated in development of PNH. The management of primary PNH syndromes comprises symptomatic treatment with anticonvulsant drugs, immune modulation if necessary, and treatment of possible associated dysimmune and/or malignant conditions. PMID:25719304

  4. Minimally invasive tethered cord release in children: A technical note

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    S. Kağan Başarslan

    2014-03-01

    Full Text Available Tethered cord release is commonly performed in pediatric neurosurgery. Nowadays, minimally invasive procedures are created growing interest due to its highly tolerable nature for surgery. It has been main purpose a minimal damaging on access route and maximum protection of normal structures in surgery. We present a surgical treatment of tethered cord syndrome, by which is provided the cord releasing unlike the many methods being applied with tissue removal. The main advantage of performing this surgery through 2 cm hole is to avoid removing ligamentum flavum and bony structure like lamina in addition to reduce the length of the incision and the related scar tissue. J Clin Exp Invest 2014; 5 (1: 115-117 Technical note: the patient was taken on the operating table in the sitting-prone position, and L5-S1 distance was determined by fluoroscopy. The skin and subcutaneous tissues was passed via a 2 cm vertical incision settled in 0.5 cm laterally from midline. L5-S1 distance and its covering ligamentum flavum are displayed by the guidance of L5 lamina. Williams’s retractor was placed in the distance after fetching microscope. The foregoing procedures are the same with microdiscectomic surgery. By a vertical incision made on the flavum, its both layer was lifted up and hanged with simple suture on the back tissue for a comfortable exposure of the Dura. Thecal sac was opened by 0.5 cm long vertical incision on the Dura after obtaining secure CSF drainage with the help of yellow-tipped syringe needle. With finding by a nerve hook, the phylum was burned and released securely. Then the Dura was sutured primarily for the closure by means of microsurgery instruments, and flavum was laid on it again.

  5. [Acute ischemic spinal cord disease. Spinal cord infarction. A clinical study and MRI in 8 cases].

    Science.gov (United States)

    Pau Serradell, A

    1994-01-01

    Acute spinal cord infarction (ASCI) occurs infrequently and may have diverse causes. The diagnosis of ASCI, and particularly of an anterior spinal artery syndrome (ASAS) can be confirmed nowadays by MRI, whereas in the past only necropsy confirmation was possible. Pathophysiology and long-term prognosis may be better known at present and treatments more consistent. We present the longitudinal study and clinical features of 8 patients suffering from ASCI. All of them were personally studied and had MRI examinations, often with sequential studies. three groups must be considered: one included 4 cases of ASAS at cervical level, the second 2 cases of ASAS at thoracic level and the third group with infarction of the conus medullaris (ICM), one of them developed during surgical repair of an infrarenal aortic aneurysm. Motor and sensory sequelae were assessed in each case together with possible etiological factors. In conclusion, recovery after ASAS tends to be dependent on the severity of the initial deficit. At cervical level, clinical and morphological findings argue in favour of an extrinsic selective compression of the C7 right radiculo-medullary artery as responsible for the ASA. At thoracic level, the artery preferentially occluded seems to be the sulco-commisural artery as a consequence of disc compression. Finally, an underlying peculiarity of the pattern of arterial supply is a probable predisposing factor for ICM. Generally, the long-term prognosis of ASCI is not necessarily bad. PMID:7801036

  6. The MRI manifestations of intramedullary germinoma of the spinal cord: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Feng Chen; Tao Liu; Jianjun Li; Chuanzi Li

    2009-01-01

    Primary germinoma in the spinal cord is very rare. Preoperative diagnosis is important because germinomas are sensitive to radiation therapy and chemotherapy so that treatment trends and prognosis differ from other intramedullary spinal cord tumors. Preoperative radiologic diagnosis is very difficult because of this rarity. In this case a 22-year-old woman with a 4-month history of gradual numbness and weakness of both lower extremities was found to have a solid tumor in the thoracic cord between the T9 and T11 vertebral bodies. No other neoplastic lesion was found inside or outside the central nervous system. The patient underwent surgery, and the intramedullary lesion was almost totally resected. Serum HCG was elevated postoperatively without pregnancy. The pathological diagnosis was conclusively that of a germinoma. The previous 23 cases of primary spinal cord germinoma were reviewed for comparison.

  7. Advances in regenerative therapies for spinal cord injury:a biomaterials approach

    Institute of Scientific and Technical Information of China (English)

    Magdalini Tsintou; Kyriakos Dalamagkas; Alexander Marcus Seifalian

    2015-01-01

    Spinal cord injury results in the permanent loss of function, causing enormous personal, social and economic problems. Even though neural regeneration has been proven to be a natural mech-anism, central nervous system repair mechanisms are ineffective due to the imbalance of the inhibitory and excitatory factors implicated in neuroregeneration. Therefore, there is growing re-search interest on discovering a novel therapeutic strategy for effective spinal cord injury repair. To this direction, cell-based delivery strategies, biomolecule delivery strategies as well as scaf-fold-based therapeutic strategies have been developed with a tendency to seek for the answer to a combinatorial approach of all the above. Here we review the recent advances on regenerative/neural engineering therapies for spinal cord injury, aiming at providing an insight to the most promising repair strategies, in order to facilitate future research conduction.

  8. Advances in regenerative therapies for spinal cord injury: a biomaterials approach

    Directory of Open Access Journals (Sweden)

    Magdalini Tsintou

    2015-01-01

    Full Text Available Spinal cord injury results in the permanent loss of function, causing enormous personal, social and economic problems. Even though neural regeneration has been proven to be a natural mechanism, central nervous system repair mechanisms are ineffective due to the imbalance of the inhibitory and excitatory factors implicated in neuroregeneration. Therefore, there is growing research interest on discovering a novel therapeutic strategy for effective spinal cord injury repair. To this direction, cell-based delivery strategies, biomolecule delivery strategies as well as scaffold-based therapeutic strategies have been developed with a tendency to seek for the answer to a combinatorial approach of all the above. Here we review the recent advances on regenerative/neural engineering therapies for spinal cord injury, aiming at providing an insight to the most promising repair strategies, in order to facilitate future research conduction.

  9. Delayed cord clamping in red blood cell alloimmunization: safe, effective, and free?

    Science.gov (United States)

    McAdams, Ryan M

    2016-04-01

    Hemolytic disease of the newborn (HDN), an alloimmune disorder due to maternal and fetal blood type incompatibility, is associated with fetal and neonatal complications related to red blood cell (RBC) hemolysis. After delivery, without placental clearance, neonatal hyperbilirubinemia may develop from ongoing maternal antibody-mediated RBC hemolysis. In cases refractory to intensive phototherapy treatment, exchange transfusions (ET) may be performed to prevent central nervous system damage by reducing circulating bilirubin levels and to replace antibody-coated red blood cells with antigen-negative RBCs. The risks and costs of treating HDN are significant, but appear to be decreased by delayed umbilical cord clamping at birth, a strategy that promotes placental transfusion to the newborn. Compared to immediate cord clamping (ICC), safe and beneficial short-term outcomes have been demonstrated in preterm and term neonates receiving delayed cord clamping (DCC), a practice that may potentially be effective in cases RBC alloimmunization. PMID:27186530

  10. Transcriptome analyses reveal molecular mechanisms underlying functional recovery after spinal cord injury.

    Science.gov (United States)

    Duan, Hongmei; Ge, Weihong; Zhang, Aifeng; Xi, Yue; Chen, Zhihua; Luo, Dandan; Cheng, Yin; Fan, Kevin S; Horvath, Steve; Sofroniew, Michael V; Cheng, Liming; Yang, Zhaoyang; Sun, Yi E; Li, Xiaoguang

    2015-10-27

    Spinal cord injury (SCI) is considered incurable because axonal regeneration in the central nervous system (CNS) is extremely challenging, due to harsh CNS injury environment and weak intrinsic regeneration capability of CNS neurons. We discovered that neurotrophin-3 (NT3)-loaded chitosan provided an excellent microenvironment to facilitate nerve growth, new neurogenesis, and functional recovery of completely transected spinal cord in rats. To acquire mechanistic insight, we conducted a series of comprehensive transcriptome analyses of spinal cord segments at the lesion site, as well as regions immediately rostral and caudal to the lesion, over a period of 90 days after SCI. Using weighted gene coexpression network analysis (WGCNA), we established gene modules/programs corresponding to various pathological events at different times after SCI. These objective measures of gene module expression also revealed that enhanced new neurogenesis and angiogenesis, and reduced inflammatory responses were keys to conferring the effect of NT3-chitosan on regeneration. PMID:26460053

  11. A clinicopathological analysis of unusual extraventricular neurocytoma of spinal cord

    Directory of Open Access Journals (Sweden)

    LI Zhi

    2013-08-01

    attach the dura mater and invade the surrounding tissues. The tumor was removed totally. Microscopic examination showed that a moderate cellular tumor was composed of uniform cells and arranged in sheets. The tumor cells had round nuclei, finely speckled chromatin and clear cytoplasm. Some cells had perinuclear haloes resembling oligodendroglioma. In some areas, the tumor cells with elongated cytoplasmic processes arranged radially around the blood vessels with myxoid degeneration, forming a structure of "perivascular pseudorosette", resembling the ependymoma. Mitotic activity and necrotic area were not observed. The tumor cells were strongly immunopositive for Syn, focally positive for NSE, S-100 and Oligo-2, but negative for Vim, CK, EMA, NeuN and GFAP. Ki-67 index was less than 1% in our case. Based on clinical presentation and histological findings, a final histological diagnosis of primary EVN in spinal cord was made according to the criteria of WHO classification. The patient has not received radiotherapy and attended follow-up for 6 months, without any neurological deficit or signs of recurrence. Conclusion Spinal EVN is extremely rare and there are no more than 20 bona fide cases reported previously all over the world. It might originate from neuronal precursor cells surrounding the region of central canal in fetal life. The definite diagnosis of this tumor should be made under the microscopical examination because the preoperatively radiological appearance of the tumor does not differ from other tumors occurring in spinal cord. Although good prognosis obtained from gross total resection in most of reported patients with this tumor, adjuvant radiotherapy is recommend for those tumors with atypical histological features to avoid the tumor recurrence. Due to the rarity of its site, the tumor can easily be confused with other tumors of spinal cord with clear cells features and ependymoma-like structure. The strictly differential diagnosis should be made when the

  12. Primary sleep apnoea syndrome.

    OpenAIRE

    Chokroverty, S.; Sharp, J T

    1981-01-01

    Polygraphic study in 18 men with the sleep apnoea syndrome showed central, upper airway obstructive, and mixed apnoeas. Fifty per cent of the total apnoea time was central, 33% was obstructive, and 17% was mixed. Apnoeic episodes were accompanied by oxygen desaturation, relative bradycardia and hypotonia of orofacial muscles innervated by ponto-medullary neurons. During regular breathing these muscles revealed tonic and phasic inspiratory EMG activities. The data suggest that the primary slee...

  13. Extensive neuronal differentiation of human neural stem cell grafts in adult rat spinal cord.

    Directory of Open Access Journals (Sweden)

    Jun Yan

    2007-02-01

    Full Text Available BACKGROUND: Effective treatments for degenerative and traumatic diseases of the nervous system are not currently available. The support or replacement of injured neurons with neural grafts, already an established approach in experimental therapeutics, has been recently invigorated with the addition of neural and embryonic stem-derived precursors as inexhaustible, self-propagating alternatives to fetal tissues. The adult spinal cord, i.e., the site of common devastating injuries and motor neuron disease, has been an especially challenging target for stem cell therapies. In most cases, neural stem cell (NSC transplants have shown either poor differentiation or a preferential choice of glial lineages. METHODS AND FINDINGS: In the present investigation, we grafted NSCs from human fetal spinal cord grown in monolayer into the lumbar cord of normal or injured adult nude rats and observed large-scale differentiation of these cells into neurons that formed axons and synapses and established extensive contacts with host motor neurons. Spinal cord microenvironment appeared to influence fate choice, with centrally located cells taking on a predominant neuronal path, and cells located under the pia membrane persisting as NSCs or presenting with astrocytic phenotypes. Slightly fewer than one-tenth of grafted neurons differentiated into oligodendrocytes. The presence of lesions increased the frequency of astrocytic phenotypes in the white matter. CONCLUSIONS: NSC grafts can show substantial neuronal differentiation in the normal and injured adult spinal cord with good potential of integration into host neural circuits. In view of recent similar findings from other laboratories, the extent of neuronal differentiation observed here disputes the notion of a spinal cord that is constitutively unfavorable to neuronal repair. Restoration of spinal cord circuitry in traumatic and degenerative diseases may be more realistic than previously thought, although major

  14. Voice therapy and assistive techniques in voice disorders caused by unilateral vocal cord pareses

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    Kašterović Bojana

    2014-01-01

    Full Text Available Introduction. Dysphonias due to primary neurogenic disorders are a group of voice disorders that can be caused by both central and peripheral disorders of the larynx innervation. There are numerous causes leading to paralysis of superior and/or inferior laryngeal nerves, particularly of the inferior laryngeal or recurrent nerve. Voice Therapy in Unilateral Vocal Cord Paresis. Vocal therapy is an integral part of the conservative treatment. Specific methods are applied to individual vocal problems, while the non-specific ones are applied to a number of dysphonias. Non-specific methods are further divided into integrated and focused vocal methods. Integrated methods treat the voice and speech disorders as a unified entity of all quality and segments of voice and speech. Focused non-specific methods treat the segments and the quality of voice and speech individually. Assistive Techniques in Voice Disorders Caused by Unilateral Vocal Cord Paresis. Digital compression of the larynx by Seeman includes the treatment of voice with compression of the thyroid cartilage, thus moving the paralyzed and healthy vocal cord medially and upwards, and medially and downwards, respectively. This leads to the proper occlusion of vocal cords because in these conditions the paralyzed vocal cord is lower than the healthy one. According to the theoretical assumption, when the head anc neck are rotated to one or the other side, the anatomic relations in the neck are change and thus the vocal cords are brought into contact with the resulting reduction of the gap between them and the reduced air flow. Conclusion. Studies assessing the efficiency of different methods of vocal therapy are scarce bearing in mind the importance of vocal therapy and the fact that many patients refuse surgical treatment. Research on the efficiency of assistive techniques in phoniatric rehabilitation of patients with unilateral vocal cord paresis yields conflicting results. However, assistive

  15. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, ...

  16. A Clinical Perspective and Definition of Spinal Cord Injury.

    Science.gov (United States)

    Kretzer, Ryan M

    2016-04-01

    Spinal cord injury (SCI) can be complete or incomplete. The level of injury in SCI is defined as the most caudal segment with motor function rated at greater than or equal to 3/5, with pain and temperature preserved. The standard neurological classification of SCI provided by the American Spinal Injury Association (ASIA) assigns grades from ASIA A (complete SCI) through ASIA E (normal sensory/motor), with B, C, and D representing varying degrees of injury between these extremes. The most common causes of SCI include trauma (motor vehicle accidents, sports, violence, falls), degenerative spinal disease, vascular injury (anterior spinal artery syndrome, epidural hematoma), tumor, infection (epidural abscess), and demyelinating processes (). (SDC Figure 1, http://links.lww.com/BRS/B91)(Figure is included in full-text article.).

  17. Pain modulation by nitric oxide in the spinal cord.

    Directory of Open Access Journals (Sweden)

    Marco Aurelio M Freire

    2009-09-01

    Full Text Available Nitric oxide (NO is a versatile messenger molecule first associated with endothelial relaxing effects. In the central nervous system (CNS, NO synthesis is primarily triggered by activation of N-methyl-D-aspartate (NMDA receptors and has a Janus face, with both beneficial and harmful properties, depending on concentration and the identity of its synthetic enzyme isoform. There are three isoforms of the NO synthesizing enzyme nitric oxide synthase (NOS: neuronal (nNOS, endothelial (eNOS, and inducible nitric oxide synthase (iNOS, each one involved with specific events in the brain. In CNS, nNOS is involved with modulation of synaptic transmission through long-term potentiation in several regions, including nociceptive circuits in the spinal cord. Here, we review the role played by NO on central pain sensitization.

  18. Neurochemical profile of the human cervical spinal cord determined by MRS.

    Science.gov (United States)

    Hock, Andreas; Wilm, Bertram; Zandomeneghi, Giorgia; Ampanozi, Garyfalia; Franckenberg, Sabine; Zoelch, Niklaus; Wyss, Patrik Oliver; De Zanche, Nicola; Nordmeyer-Maßner, Jurek; Kraemer, Thomas; Thali, Michael; Ernst, Matthias; Kollias, Spyros; Henning, Anke

    2016-10-01

    MRS enables insight into the chemical composition of central nervous system tissue. However, technical challenges degrade the data quality when applied to the human spinal cord. Therefore, to date detection of only the most prominent metabolite resonances has been reported in the healthy human spinal cord. The aim of this investigation is to provide an extended metabolic profile including neurotransmitters and antioxidants in addition to metabolites involved in the energy and membrane metabolism of the human cervical spinal cord in vivo. To achieve this, data quality was improved by using a custom-made, cervical detector array together with constructive averaging of a high number of echo signals, which is enabled by the metabolite cycling technique at 3T. In addition, the improved spinal cord spectra were extensively cross-validated, in vivo, post-mortem in situ and ex vivo. Reliable identification of up to nine metabolites was achieved in group analyses for the first time. Distinct features of the spinal cord neurochemical profile, in comparison with the brain neurotransmission system, include decreased concentrations of the sum of glutamate and glutamate and increased concentrations of aspartate, γ-amino-butyric acid, scyllo-inositol and the sum of myo-inositol and glycine. PMID:27580498

  19. The central role of vascular extracellular matrix and basement membrane remodeling in metabolic syndrome and type 2 diabetes: the matrix preloaded

    Directory of Open Access Journals (Sweden)

    Tyagi Suresh C

    2005-06-01

    Full Text Available Abstract The vascular endothelial basement membrane and extra cellular matrix is a compilation of different macromolecules organized by physical entanglements, opposing ionic charges, chemical covalent bonding, and cross-linking into a biomechanically active polymer. These matrices provide a gel-like form and scaffolding structure with regional tensile strength provided by collagens, elasticity by elastins, adhesiveness by structural glycoproteins, compressibility by proteoglycans – hyaluronans, and communicability by a family of integrins, which exchanges information between cells and between cells and the extracellular matrix of vascular tissues. Each component of the extracellular matrix and specifically the capillary basement membrane possesses unique structural properties and interactions with one another, which determine the separate and combined roles in the multiple diabetic complications or diabetic opathies. Metabolic syndrome, prediabetes, type 2 diabetes mellitus, and their parallel companion (atheroscleropathy are associated with multiple metabolic toxicities and chronic injurious stimuli. The adaptable quality of a matrix or form genetically preloaded with the necessary information to communicate and respond to an ever-changing environment, which supports the interstitium, capillary and arterial vessel wall is individually examined.

  20. Therapeutic approaches for spinal cord injury

    Directory of Open Access Journals (Sweden)

    Alexandre Fogaça Cristante

    2012-10-01

    Full Text Available This study reviews the literature concerning possible therapeutic approaches for spinal cord injury. Spinal cord injury is a disabling and irreversible condition that has high economic and social costs. There are both primary and secondary mechanisms of damage to the spinal cord. The primary lesion is the mechanical injury itself. The secondary lesion results from one or more biochemical and cellular processes that are triggered by the primary lesion. The frustration of health professionals in treating a severe spinal cord injury was described in 1700 BC in an Egyptian surgical papyrus that was translated by Edwin Smith; the papyrus reported spinal fractures as a ''disease that should not be treated.'' Over the last biological or pharmacological treatment method. Science is unraveling the mechanisms of cell protection and neuroregeneration, but clinically, we only provide supportive care for patients with spinal cord injuries. By combining these treatments, researchers attempt to enhance the functional recovery of patients with spinal cord injuries. Advances in the last decade have allowed us to encourage the development of experimental studies in the field of spinal cord regeneration. The combination of several therapeutic strategies should, at minimum, allow for partial functional recoveries for these patients, which could improve their quality of life.

  1. Therapeutic approaches for spinal cord injury.

    Science.gov (United States)

    Cristante, Alexandre Fogaça; Barros Filho, Tarcísio Eloy Pessoa de; Marcon, Raphael Martus; Letaif, Olavo Biraghi; Rocha, Ivan Dias da

    2012-10-01

    This study reviews the literature concerning possible therapeutic approaches for spinal cord injury. Spinal cord injury is a disabling and irreversible condition that has high economic and social costs. There are both primary and secondary mechanisms of damage to the spinal cord. The primary lesion is the mechanical injury itself. The secondary lesion results from one or more biochemical and cellular processes that are triggered by the primary lesion. The frustration of health professionals in treating a severe spinal cord injury was described in 1700 BC in an Egyptian surgical papyrus that was translated by Edwin Smith; the papyrus reported spinal fractures as a "disease that should not be treated." Over the last biological or pharmacological treatment method. Science is unraveling the mechanisms of cell protection and neuroregeneration, but clinically, we only provide supportive care for patients with spinal cord injuries. By combining these treatments, researchers attempt to enhance the functional recovery of patients with spinal cord injuries. Advances in the last decade have allowed us to encourage the development of experimental studies in the field of spinal cord regeneration. The combination of several therapeutic strategies should, at minimum, allow for partial functional recoveries for these patients, which could improve their quality of life. PMID:23070351

  2. Curcumin protects against ischemic spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Jinhua Zhang; Hao Wei; Meimei Lin; Chunmei Chen; Chunhua Wang; Maobai Liu

    2013-01-01

    Inducible nitric oxide synthase and N-methyl-D-aspartate receptors have been shown to participate in nerve cellinjury during spinal cord ischemia. This study observed a protective effect of curcumin on ischemic spinal cord injury. Models of spinal cord ischemia were established by ligating the lumbar artery from the left renal artery to the bifurcation of the abdominal aorta. At 24 hours after model establishment, the rats were intraperitoneal y injected with curcumin. Reverse transcrip-tion-polymerase chain reaction and immunohistochemical results demonstrated that after spinal cord ischemia, inducible nitric oxide synthase and N-methyl-D-aspartate receptor mRNA and protein expression significantly increased. However, curcumin significantly decreased inducible nitric oxide synthase and N-methyl-D-aspartate receptor mRNA and protein expression in the ischemic spinal cord. Tarlov scale results showed that curcumin significantly improved motor function of the rat hind limb after spinal cord ischemia. The results demonstrate that curcumin exerts a neuroprotective ef-fect against ischemic spinal cord injury by decreasing inducible nitric oxide synthase and N-methyl-D-aspartate receptor expression.

  3. Central nervous system abnormalities in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome Anormalidades do sistema nervoso central em pacientes com espectro óculo-aurículo-vertebral (síndrome de Goldenhar

    Directory of Open Access Journals (Sweden)

    Rafael Fabiano Machado Rosa

    2010-02-01

    Full Text Available OBJECTIVE: To describe the central nervous system (CNS alterations present in a sample of oculo-auriculo-vertebral spectrum (OAVS patients, trying to correlate them with other clinical features. METHOD: Seventeen patients with diagnosis of OAVS were evaluated. All presented radiological evaluation of the CNS, normal GTG-Banding karyotype and clinical features involving at least two from the four following areas: oro-cranio-facial, ocular, auricular and vertebral. RESULTS: CNS alterations were verified in eight from seventeen patients (47%. Diffuse cerebral hypoplasia, dilated lateral cerebral ventricles (asymptomatic hydrocephalus, corpus callosum dysgenesis and frontal hypodensities were the most frequent abnormalities. Presence of ophthalmologic abnormalities was the only clinical association observed, being significantly more frequent among patients with cerebral alterations (63% versus 11%. CONCLUSION: CNS abnormalities are frequent in patients with OAVS, especially in carriers of ophthalmologic alterations. However, the absence of detectable cerebral abnormalities did not exclude the possibility that these subjects will subsequently present neurological symptoms.OBJETIVO: Descrever as alterações do sistema nervoso central (SNC presentes em uma amostra de pacientes com espectro óculo-aurículo-vertebral (EOAV, tentando correlacioná-las com os demais achados clínicos. MÉTODO: Foram avaliados dezessete pacientes com diagnóstico de EOAV. Todos apresentavam avaliação radiológica do SNC, cariótipo por bandas GTG normal e achados clínicos em pelo menos duas das quatro das seguintes áreas: oro-crânio-facial, ocular, auricular e vertebral. RESULTADOS: Alterações do SNC foram verificadas em oito dos dezessete pacientes (47%. Hipoplasia cerebral difusa, dilatação dos ventrículos cerebrais laterais (hidrocefalia assintomática, disgenesia do corpo caloso e hipondesidades frontais foram as anormalidades mais frequentes. A presença de

  4. Cytokines in Sjogren's syndrome

    NARCIS (Netherlands)

    N. Roescher; P.P. Tak; G.G. Illei

    2009-01-01

    Cytokines play a central role in the regulation of immunity and are often found to be deregulated in autoimmune diseases. Sjogren's syndrome is a chronic autoimmune disease characterized by inflammation and loss of secretory function of the salivary and lachrymal glands. This review highlights the c

  5. Advance in spinal cord ischemia reperfusion injury: Blood-spinal cord barrier and remote ischemic preconditioning.

    Science.gov (United States)

    Yu, Qijing; Huang, Jinxiu; Hu, Ji; Zhu, Hongfei

    2016-06-01

    The blood-spinal cord barrier (BSCB) is the physiological and metabolic substance diffusion barrier between blood circulation and spinal cord tissues. This barrier plays a vital role in maintaining the microenvironment stability of the spinal cord. When the spinal cord is subjected to ischemia/reperfusion (I/R) injury, the structure and function of the BSCB is disrupted, further destroying the spinal cord homeostasis and ultimately leading to neurological deficit. Remote ischemic preconditioning (RIPC) is an approach in which interspersed cycles of preconditioning ischemia is followed by reperfusion to tissues/organs to protect the distant target tissues/organs against subsequent lethal ischemic injuries. RIPC is an innovation of the treatment strategies that protect the organ from I/R injury. In this study, we review the morphological structure and function of the BSCB, the injury mechanism of BSCB resulting from spinal cord I/R, and the effect of RIPC on it. PMID:27060223

  6. Human umbilical cord mesenchymal stem cells and the treatment of spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    CAO Fu-jiang; FENG Shi-qing

    2009-01-01

    Objective To review the recent studies about human umbilical cord mesenchymal stem cells (hUCMSCs) and advances in the treatment of spinal cord injury, Data sources Published articles (1983-2007) about hUCMSCs and spinal cord injury were selected using Medline. Study selection Articles selected were relevant to development of mesenchymal stem cells (MSCs) for transplantation in spinal cord injury therapy. Of 258 originally identifiied arises 51 were selected that specifically addressed the stated purpose. Results Recent work has revealed that hUCMSCs share most of the characteristics with MSCs derived from bone marrow and are more appropriate to transplantation for cell based therapies. Conclusions Human umbilical cord could be regarded as a source of MSCs for experimental and clinical needs. In addition, as a peculiar source of stem cells, hUCMSCs may play an important role in the treatment of spinal cord injury.

  7. MR imaging evaluation of tethered spinal cord

    International Nuclear Information System (INIS)

    Seven cases of tethered cord underwent magnetic resonance imaging. The associated findings included the following: case 1: caudal regression, inperforated anus, cutaneovesical fistula, and diverticulum of the bladder; case 2: Arnold-Chiari malformation, hydromyelia, and urinary reflux; case 3; lipoma; case 4: postoperative syringomyelia and residual lipoma; case 5: diastematomyelia and spinal bifida; case 6: dysraphism, diastematomyelia, and myelomeningocele; and case 7: postoperative split cord T1-weighted axial and sagittal images are sufficient for evaluation of tethered cord and associated anomalies and are also useful for postoperative follow-up and the detection of possible complications

  8. Teaching nonlinear dynamics through elastic cords

    Energy Technology Data Exchange (ETDEWEB)

    Chacon, R; Galan, C A; Sanchez-Bajo, F, E-mail: rchacon@unex.e [Departamento de Fisica Aplicada, Escuela de IngenierIas Industriales, Universidad de Extremadura, Apartado Postal 382, E-06071 Badajoz (Spain)

    2011-01-15

    We experimentally studied the restoring force of a length of stretched elastic cord. A simple analytical expression for the restoring force was found to fit all the experimental results for different elastic materials. Remarkably, this analytical expression depends upon an elastic-cord characteristic parameter which exhibits two limiting values corresponding to two nonlinear springs with different Hooke's elastic constants. Additionally, the simplest model of elastic cord dynamics is capable of exhibiting a great diversity of nonlinear phenomena, including bifurcations and chaos, thus providing a suitable alternative model system for discussing the basic essentials of nonlinear dynamics in the context of intermediate physics courses at university level.

  9. Teaching nonlinear dynamics through elastic cords

    International Nuclear Information System (INIS)

    We experimentally studied the restoring force of a length of stretched elastic cord. A simple analytical expression for the restoring force was found to fit all the experimental results for different elastic materials. Remarkably, this analytical expression depends upon an elastic-cord characteristic parameter which exhibits two limiting values corresponding to two nonlinear springs with different Hooke's elastic constants. Additionally, the simplest model of elastic cord dynamics is capable of exhibiting a great diversity of nonlinear phenomena, including bifurcations and chaos, thus providing a suitable alternative model system for discussing the basic essentials of nonlinear dynamics in the context of intermediate physics courses at university level.

  10. Nanomedicine for treating spinal cord injury

    Science.gov (United States)

    Tyler, Jacqueline Y.; Xu, Xiao-Ming; Cheng, Ji-Xin

    2013-09-01

    Spinal cord injury results in significant mortality and morbidity, lifestyle changes, and difficult rehabilitation. Treatment of spinal cord injury is challenging because the spinal cord is both complex to treat acutely and difficult to regenerate. Nanomaterials can be used to provide effective treatments; their unique properties can facilitate drug delivery to the injury site, enact as neuroprotective agents, or provide platforms to stimulate regrowth of damaged tissues. We review recent uses of nanomaterials including nanowires, micelles, nanoparticles, liposomes, and carbon-based nanomaterials for neuroprotection in the acute phase. We also review the design and neural regenerative application of electrospun scaffolds, conduits, and self-assembling peptide scaffolds.

  11. The high cost of motherhood: End-lactation syndrome in southern sea otters (Enhydra lutris nereis) on the central California, USA, coast

    Science.gov (United States)

    Chinn, Sarah S; Miller, Melissa A.; Tinker, M. Tim; Staedler, Michelle M.; Batac, Francesca I.; Dodd, Erin M.; Henkel, Laird A.

    2016-01-01

    Sea otters (Enhydra lutris) have exceptionally high energetic requirements, which nearly double during lactation and pup care. Thus, females are extremely vulnerable to caloric insufficiency. Despite a number of compensatory strategies, the metabolic challenge of reproduction culminates in numerous maternal deaths annually. Massive depletion of energy reserves results in a case presentation that we define as end-lactation syndrome (ELS), characterized by moderate to severe emaciation not attributable to a concurrent, independent disease process in females dying during late pup care or postweaning. We compiled detailed data for 108 adult female southern sea otters (Enhydra lutris nereis) examined postmortem that stranded in California, US, 2005–12, and assessed pathology, reproductive status, and the location and timing of stranding. We introduce simple, grossly apparent, standardized physical criteria to assess reproductive stage for female sea otters. We also describe ELS, examine associated risk factors, and highlight female life history strategies that likely optimize reproduction and survival. Our data suggest that females can reset both the timing and energetic demands of reproduction through fetal loss, pup abandonment, or early weaning as part of specific physiologic checkpoints during each reproductive cycle. Females appear to preload nutritionally during delayed implantation and gestation to increase fitness and reproductive success. We found that ELS was a major cause of death, affecting 56% of enrolled adult females. Peak ELS prevalence occurred in late spring, possibly reflecting the population trend toward fall/winter pupping. Increasing age and number of pregnancies were associated with a higher risk of ELS. Although the proportion of ELS females was highest in areas with dense sea otter populations, cases were recovered throughout the range, suggesting that death from ELS is associated with, but not solely caused by, population resource limitation.

  12. Pain in spinal cord injury.

    Science.gov (United States)

    Baastrup, Cathrine; Finnerup, Nanna Brix

    2012-01-01

    SUMMARY An important and detrimental effect of spinal cord injury (SCI) is pain, which develops in approximately two-thirds of all SCI patients, while approximately half of SCI patients develop chronic neuropathic pain (NP). Thus far, there is no cure for SCI NP, and oral pharmacological intervention is often inadequate, commonly resulting in a pain reduction of only 20-30%. In this short review, we will present an overview of the important features of SCI pain including taxonomy, epidemiology and classification, as well as a suggested oral pharmacological treatment strategy for SCI NP and the current evidence available from randomized placebo-controlled trials. Considerations and evidence for the nonpharmacological treatment of SCI will be discussed briefly. PMID:24654622

  13. Muscle after spinal cord injury

    DEFF Research Database (Denmark)

    Biering-Sørensen, Bo; Kristensen, Ida Bruun; Kjaer, Michael;

    2009-01-01

    The morphological and contractile changes of muscles below the level of the lesion after spinal cord injury (SCI) are dramatic. In humans with SCI, a fiber-type transformation away from type I begins 4-7 months post-SCI and reaches a new steady state with predominantly fast glycolytic IIX fibers...... years after the injury. There is a progressive drop in the proportion of slow myosin heavy chain (MHC) isoform fibers and a rise in the proportion of fibers that coexpress both the fast and slow MHC isoforms. The oxidative enzymatic activity starts to decline after the first few months post-SCI. Muscles...... from individuals with chronic SCI show less resistance to fatigue, and the speed-related contractile properties change, becoming faster. These findings are also present in animals. Future studies should longitudinally examine changes in muscles from early SCI until steady state is reached in order...

  14. Anaplastic astrocytoma in the spinal cord of an African pygmy hedgehog (Atelerix albiventris).

    Science.gov (United States)

    Gibson, C J; Parry, N M A; Jakowski, R M; Eshar, D

    2008-11-01

    A 2-year-old, female hedgehog presented with an 8-month history of progressive, ascending paresis/paralysis and was tentatively diagnosed with wobbly hedgehog syndrome. She died awaiting further diagnostic tests, and the owners consented to postmortem examination. Grossly, the bladder was large and flaccid and the cervical and lumbar spinal cord were regionally enlarged, light grey, and friable with multifocal hemorrhages. The thoracic spinal cord was grossly normal. Microscopically all regions of the spinal cord had similar changes, although the cervical and lumbar sections were most severely affected. These regions were completely effaced by a moderately cellular infiltration of highly pleomorphic polygonal to spindle shaped cells, mineralization, and necrosis, which were most consistent with anaplastic astrocytoma. The thoracic spinal cord white matter was similarly infiltrated by the neoplastic cells, with perivascular extension into the otherwise normal grey matter. A diagnosis of anaplastic astrocytoma was confirmed using immunohistochemical stains that were positive for glial fibrillary acidic protein and S100. PMID:18984799

  15. Spinal cord decompression reduces rat neural cell apoptosis secondary to spinal cord injury*

    OpenAIRE

    Xu, Kan; Chen, Qi-xin; Li, Fang-cai; Chen, Wei-Shan; Lin, Min; Wu, Qiong-hua

    2009-01-01

    Objective: To determine whether spinal cord decompression plays a role in neural cell apoptosis after spinal cord injury. Study design: We used an animal model of compressive spinal cord injury with incomplete paraparesis to evaluate neural cell apoptosis after decompression. Apoptosis and cellular damage were assessed by staining with terminal deoxynucleotidyl transferase (TdT)-mediated deoxyuridine triphosphate nick-end labelling (TUNEL) and immunostaining for caspase-3, Bcl-2 and Bax. Meth...

  16. Induced pluripotent stem cell-derived neural stem cell therapies for spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Corinne A Lee-Kubli; Paul Lu

    2015-01-01

    The greatest challenge to successful treatment of spinal cord injury is the limited regenerative capacity of the central nervous system and its inability to replace lost neurons and severed axons following injury. Neural stem cell grafts derived from fetal central nervous system tissue or embryonic stem cells have shown therapeutic promise by differentiation into neurons and glia that have the potential to form functional neuronal relays across injured spinal cord segments. However, implementation of fetal-derived or embryonic stem cell-derived neural stem cell ther-apies for patients with spinal cord injury raises ethical concerns. Induced pluripotent stem cells can be generated from adult somatic cells and differentiated into neural stem cells suitable for therapeutic use, thereby providing an ethical source of implantable cells that can be made in an autologous fashion to avoid problems of immune rejection. This review discusses the therapeutic potential of human induced pluripotent stem cell-derived neural stem cell transplantation for treatment of spinal cord injury, as well as addressing potential mechanisms, future perspectives and challenges.

  17. Effects of intrathecal methotrexate and cytosine arabinoside on the radiation tolerance of the rat spinal cord

    International Nuclear Information System (INIS)

    The effect of intrathecally or intravenously administered methotrexate (MTX) or cytosine arabinoside (ara-C) on the early and late delayed radiation response of the rat cervical spinal cord has been studied. A technique has been developed for intrathecal administration of drugs into the rat lumbar spinal canal. When given shortly before irradiation, intrathecal ara-C significantly reduces the isoeffect doses for the early delayed white matter necrosis syndrome by a factor of 1.2-1.3, while no effect is observed for the late delayed vascular syndrome. The effect disappears when ara-C is given intravenously or 24 h after irradiation. At a maximally tolerated intrathecal MTX dose, no modification of the early or late radiation response of the spinal cord was observed. In constrast to ara-C, intravenous MTX seems to interact with the induction of the late delayed vascular damage in the rat cervical spinal cord, with a dose-modifying factor of 1.1-1.2. (Auth.)

  18. Morquio syndrome

    Science.gov (United States)

    ... include: Abnormal development of bones, including the spine Bell-shaped chest with ribs flared out at the ... may slip and damage the spinal cord, causing paralysis. Surgery to correct such problems should be done ...

  19. The Acrocallosal Syndrome in A Neonate With Further Widening of Phenotypic Expression

    Directory of Open Access Journals (Sweden)

    Ravish SINGHAL*

    2014-04-01

    Full Text Available How to Cite This Article: Singhal R, Pandit S, Saini A, Singh P, Dhawan N. The Acrocallosal Syndrome in A Neonate With Further Widening of Phenotypic Expression. Iran J Child Neurol. 2014 Spring;8(2:60-64. The presentation of the typical characteristics of the acrocallosal syndrome (ACLS are hypoplasia/agenesis of corpus callosum, moderate to severe mental retardation, characteristic craniofacial abnormalities, distinctive digitalmalformation, and growth retardation in a neonate.An Indian neonate presented on day 1 of life (youngest in the literature to be reported with combination of abnormalities consistent with the acrocallosal syndrome and some additional findings. The baby, born to non-consanguineous, healthy parents, presented with macrocephaly, prominent forehead, hypertelorism, polydactyly of the hands and feet, duplication of hallux, hypotonia, recurrent cyanotic episodes, rib anomalies, dextro-positioning of heart, and delayed fallof umbilical cord.As the mode of inheritance of ACLS is autosomal recessive, the risk of recurrence is 25%. Genetic counselling is of prime importance, Polydactyly, and central nervous system malformations can be detected by ultrasonography in the second trimester, but due to variability of presentation, prenatal diagnosis may not always be possible.References1. Schinzel A. Postaxial polydactyly, hallux duplication, absence of corpus callosum, macencephaly and severemental retardation: a new syndrome? Helv Paediatr Acta 1979:34:141-146.2. Schinzel A. The acrocallosal syndrome: expansion of the phenotypic spectrum. Clin Dysmorphol 1994;3:31-34.3. Schinzel A, Schmid W. Hallux duplication, postaxial Polydactyly, absence of corpus callosum, severe mental retardation and additional anomalies in two unrelated patients: a new syndrome. Am J Med Genet 1980;6:241-249.4. Moeschler JB, Pober BR, Holmes LB, Graham JM Jr. Acrocallosal syndrome: new findings. Am J Med Genet 1989;32:195-199.5. Schinzel A

  20. Jacobsen syndrome

    Directory of Open Access Journals (Sweden)

    Grossfeld Paul

    2009-03-01

    Full Text Available Abstract Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears. Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from ~7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be

  1. Morphological aspects of buffaloes (Bubalus bubalis umbilical cord Aspectos Morfológicos do cordão umbilical de búfalos (Bubalus bubalis

    Directory of Open Access Journals (Sweden)

    Guilherme J. Ferreira

    2009-10-01

    Full Text Available Buffalo is an important livestock resource, with a great participation in agricultural systems, providing milk, meat, and work power. Umbilical cord is responsible for maternal-fetal nutrients exchange during pregnancy, and its alterations can compromise the fetal development. We investigated ten pregnant uteruses collected from cross-bread buffaloes in different stages of gestation. Pregnancy and fetal age was determined by measuring the apex sacral length and development period was calculated by previously published formula. Umbilical cords were measured for length determination. Umbilical cord vascular net and anastomosis were observed by injection of Neoprene latex. Histological sections of the umbilical cord were studied after stain with HE, picrossirius, toluidine blue, orceine, and PAS reaction. Buffaloes' umbilical cord was formed by two central arteries, an allantois duct and two peripheral veins. The artery wall was composed by large quantity of collagen, elastic fibers, fibroblasts and large number of vasa vasorum. The allantois duct was located between the arteries and presented a great number of small nourishing vessels. Small nourishing vessels should be carefully considered to avoid to be mistaken to the arterials and veins vasa vasorum. Medium length of umbilical cord from buffalos was 11.8cm (minimum of 6.8cm and maximum of 17.4cm.Búfalo é uma importante fonte de recurso nos rebanhos animais, apresentando uma grande participação na agropecuária, provendo leite, carne e força de trabalho. O Cordão umbilical é responsável pela troca de nutrientes materno-fetais durante a gestação, e suas alterações podem comprometer o desenvolvimento fetal. Nós investigamos dez úteros gravídicos de búfalos de raças cruzadas em fases diferentes de gestação. O período de gestação e a idade fetal foram determinados pelo comprimento ápice sacral, aplicando fórmulas previamente estabelecidas. Posteriormente mediu-se o

  2. Loss of microRNA-124 expression in neurons in the peri-lesion area in mice with spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Yu Zhao; Hui Zhang; Dan Zhang; Cai-yong Yu; Xiang-hui Zhao; Fang-fang Liu; Gan-lan Bian; Gong Ju; Jian Wang

    2015-01-01

    MicroRNA-124 (miR-124) is abundantly expressed in neurons in the mammalian central ner-vous system, and plays critical roles in the regulation of gene expression during embryonic neurogenesis and postnatal neural differentiation. However, the expression proifle of miR-124 after spinal cord injury and the underlying regulatory mechanisms are not well understood. In the present study, we examined the expression of miR-124 in mouse brain and spinal cord after spinal cord injury usingin situ hybridization. Furthermore, the expression of miR-124 was examined with quantitative RT-PCR at 1, 3 and 7 days after spinal cord injury. The miR-124 expression in neurons at the site of injury was evaluated by in situ hybridization combined with NeuN immunohistochemical staining. The miR-124 was mainly expressed in neurons through-out the brain and spinal cord. The expression of miR-124 in neurons significantly decreased within 7 days after spinal cord injury. Some of the neurons in the peri-lesion area were NeuN+/miR-124−. Moreover, the neurons distal to the peri-lesion site were NeuN+/miR-124+. These ifndings indicate that miR-124 expression in neurons is reduced after spinal cord injury, and may relfect the severity of spinal cord injury.

  3. CB1 cannabinoid receptor enrichment in the ependymal region of the adult human spinal cord.

    Science.gov (United States)

    Paniagua-Torija, Beatriz; Arevalo-Martin, Angel; Ferrer, Isidro; Molina-Holgado, Eduardo; Garcia-Ovejero, Daniel

    2015-12-04

    Cannabinoids are involved in the regulation of neural stem cell biology and their receptors are expressed in the neurogenic niches of adult rodents. In the spinal cord of rats and mice, neural stem cells can be found in the ependymal region, surrounding the central canal, but there is evidence that this region is largely different in adult humans: lacks a patent canal and presents perivascular pseudorosettes, typically found in low grade ependymomas. Using Laser Capture Microdissection, Taqman gene expression assays and immunohistochemistry, we have studied the expression of endocannabinoid system components (receptors and enzymes) at the human spinal cord ependymal region. We observe that ependymal region is enriched in CB1 cannabinoid receptor, due to high CB1 expression in GFAP+ astrocytic domains. However, in human spinal cord levels that retain central canal patency we found ependymal cells with high CB1 expression, equivalent to the CB1(HIGH) cell subpopulation described in rodents. Our results support the existence of ependymal CB1(HIGH) cells across species, and may encourage further studies on this subpopulation, although only in cases when central canal is patent. In the adult human ependyma, which usually shows central canal absence, CB1 may play a different role by modulating astrocyte functions.

  4. Anterior horn syndrome: A rare manifestation of primary Sjögren's syndrome.

    Science.gov (United States)

    Zahlane, Safaa; Louhab, Nissrine; El Mellakh, Meriem; Kissani, Najib

    2016-07-01

    The authors report an exceptional case of an anterior horn syndrome associated with Sjögren's syndrome in a 58-year-old patient with a flaccid tetraparesis revealed by asymmetric atrophy and diffuse fasciculations associated with xerostomia and xerophthalmia. The electroneuromyography objectified a diffuse anterior horn syndrome. The brain MRI and spinal cord were normal. Laboratory tests revealed positive anti-SSA and anti-SSB antibody. The salivary glands biopsy objectified lymphocytic sialadenitis grade 3 of Chisholm. The Schirmer's test was abnormally low. Diagnosis of anterior horn syndrome as part of Sjögren's syndrome was retained. The methylprednisolone bolus allowed partial clinical improvement after 12 months of evolution. Therefore, in patients with isolated anterior horn involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable. PMID:27118221

  5. Ortner’s syndrome: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Bruno Landim Dutra

    2015-08-01

    Full Text Available AbstractThe authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner’s syndrome.

  6. Spinal cord injury without radiographic abnormality

    Directory of Open Access Journals (Sweden)

    Singh Anil

    2006-01-01

    Full Text Available Spinal cord injury without radiological abnormality is rare in adults. Below we present a case report of 20 yrs old male with isolated cervical cord injury, without accompanying vertebral dislocation or fracture involving the spinal canal rim. He fell down on plain and smooth ground while carrying 40 kg weight overhead and developed quadriparesis with difficulty in respiration. Plain radiographs of the neck revealed no fractures or dislocations. MRI showed bulky spinal cord and an abnormal hyper intense signal on the T2W image from C2 vertebral body level to C3/4 intervertebral disc level predominantly in the anterior aspect of the cord The patient was managed conservatively with head halter traction and invasive ventilatory support for the initial 7 days period in the ICU. In our patient recovery was good and most of the neurological deficit improved over 4 weeks with conservative management.

  7. Brain and Spinal Cord Tumors in Adults

    Science.gov (United States)

    ... saved articles window. My Saved Articles » My ACS » Brain and Spinal Cord Tumors in Adults Download Printable ... the topics below to get started. What Is Brain/CNS Tumors In Adults? What are adult brain ...

  8. Extramedullary haematopoeisis causing spinal cord compression

    Directory of Open Access Journals (Sweden)

    F Ismail

    2010-08-01

    Full Text Available Extramedullary haematopoeisis (EMH is a rare cause of spinal cord compression. However, in a patient with a haematological disorder and in particular thalassaemia, EMH with paraspinal masses should be considered and imaging planned appropriately.

  9. What Is Vocal Cord Dysfunction (VCD)?

    Science.gov (United States)

    ... your vocal cords. The breathing test is called spirometry and must include a flow-volume loop. This ... be so hard. To learn more about the spirometry test, see the ATS Patient Information Series fact ...

  10. Antenatal umbilical cord parameters and perinatal outcome

    Directory of Open Access Journals (Sweden)

    Athira Narayanan

    2016-04-01

    Conclusions: No association between antenatal umbilical cord characteristics and perinatal outcome was found in pregnancies at high risk for poor perinatal outcome. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000: 1211-1215

  11. Caudal Regression Syndrome

    Directory of Open Access Journals (Sweden)

    Karim Hardani*

    2012-05-01

    Full Text Available A 10-month-old baby presented with developmental delay. He had flaccid paralysis on physical examination.An MRI of the spine revealed malformation of the ninth and tenth thoracic vertebral bodies with complete agenesis of the rest of the spine down that level. The thoracic spinal cord ends at the level of the fifth thoracic vertebra with agenesis of the posterior arches of the eighth, ninth and tenth thoracic vertebral bodies. The roots of the cauda equina appear tightened down and backward and ended into a subdermal fibrous fatty tissue at the level of the ninth and tenth thoracic vertebral bodies (closed meningocele. These findings are consistent with caudal regression syndrome.

  12. Secondary restless legs syndrome

    Directory of Open Access Journals (Sweden)

    SHEN Yun

    2013-05-01

    Full Text Available This paper reviewed the latest findings related to secondary restless legs syndrome (RLS systematically. There were a variety of causes. Renal failure, injury of spinal cord and peripheral nerve were related risk factors. During pregnancy, the levels of folic acid and iron diminished and many hormone levels underwent significant changes, which might also be predisposing factors for RLS. The underlying dopamine dysfunction might affect Pakinson's disease (PD patients with RLS symptoms. In addition, there were some evidences which would illustrate some drugs (mainly antidepressants, antipsychotics, histamine receptor antagonist, smoking, caffeine, alcohol and migraine might be associated with the prevalence of RLS, or even the severity of the disease. Clinicians should pay more attention to early diagnosis and treatment plan to improve the quality of life of these patients.

  13. 上颌正中孤立中切牙综合征1例报告%Solitary median maxillary central incisor syndrome: a case of report

    Institute of Scientific and Technical Information of China (English)

    康丽颖; 刘新强

    2014-01-01

    上颌正中孤立中切牙(solitary median maxillary central incisor,SMMCI)是一种比较罕见的牙畸形,是指在上颌牙弓中仅发育并萌出1颗中切牙且该中切牙位于牙弓的正中,形态左右对称,大小与正常中切牙相似,在乳、恒牙列均可见.SMMCI可单独发生,也可伴发其他先天性中线结构畸形,或与一些系统性疾病同时发生.其中,与前脑无裂畸形(holoprosencephaly,HPE)关系最为密切.本文报告1例伴有其他中线结构缺陷的上颌正中孤立中切牙病例.并对相关问题进行讨论.

  14. Acute Hydrocephalus Following Cervical Spinal Cord Injury

    OpenAIRE

    Son, Seong; Lee, Sang Gu; Park, Chan Woo; Kim, Woo Kyung

    2013-01-01

    We present a case of acute hydrocephalus secondary to cervical spinal cord injury in a patient with diffuse ossification of the posterior longitudinal ligament (OPLL). A 75-year-old male patient visited the emergency department with tetraparesis and spinal shock. Imaging studies showed cervical spinal cord injury with hemorrhage and diffuse OPLL from C1 to C4. We performed decompressive laminectomy and occipitocervical fusion. Two days after surgery, his mental status had deteriorated to drow...

  15. Early treatment of spinal cord injury

    OpenAIRE

    Feng, Ya-Ping

    2016-01-01

    With the rapid development of society, the incidence of spinal cord injury (SCI) is increasing year by year, and the treatment is very difficult with a high disability rate. Correct prehospital first aid transportation can greatly reduce secondary injury of spinal cord caused by improper transportation. Early application of high dose methylprednisolone, internal fixation by using screw-rod system, as well as intramedually and extramedually decompression can protect the residual neurolog...

  16. Intramedullary sarcoidosis of the cervical spinal cord.

    OpenAIRE

    Vighetto, A; Fischer, G.; Collet, P.; Bady, B; Trillet, M

    1985-01-01

    A 26-year-old male with a history of pulmonary sarcoidosis showed clinical, myelographic and intra-operative evidence of a C5-C6 spinal cord tumour, which was diagnosed by biopsy as a pure intramedullary granuloma. Among the 29 published cases of histologically proven cord sarcoidosis, only five presented with pure parenchymatous infiltration without meningeal involvement. Corticosteroids are the most useful therapy, and surgery is indicated only for minimal biopsy when the parenchyma is inva...

  17. Male infertility in spinal cord trauma

    OpenAIRE

    Cristiano Utida; Jose C. Truzzi; Homero Bruschini; Rogerio Simonetti; Cedenho, Agnaldo P.; Miguel Srougi; Valdemar Ortiz

    2005-01-01

    Every year there are 10 thousand new cases of patients victimized by spinal cord trauma (SCT) in the United States and it is estimated that there are 7 thousand new cases in Brazil. Eighty percent of patients are fertile males. Infertility in this patient group is due to 3 main factors resulting from spinal cord lesions: erectile dysfunction, ejaculatory disorder and low sperm counts. Erectile dysfunction has been successfully treated with oral and injectable medications, use of vacuum device...

  18. Umbilical Cord Segmental Hemorrhage and Fetal Distress

    OpenAIRE

    Larciprete, Giovanni; Romanini, Maria Elisabetta; Arduini, Domenico; Cirese, Elio; Slowikowska-Hilczer, Jolanta; Kula, Krzysztof

    2006-01-01

    We describe an unexplained case of umbilical cord segmental hemorrhage linked with meconium-stained amniotic fluid. A severely asphyxiated infant was delivered at term by Caesarean section. There were poor prognostic signs on fetal cardiotocography with rupture of membranes with meconium-stained amniotic fluid. The pathophysiologic mechanism in this case is still unknown, even if we argued a possible role of the umbilical cord shortness.

  19. Tuberculosis of the Spermatic Cord: Case Report

    Directory of Open Access Journals (Sweden)

    Amine Benjelloun

    2014-09-01

    Full Text Available The spermatic cord tuberculoma is uncommon, especially in its lower portion. Most cases were described in Japanese literature. We report a case of tuberculosis of the spermatic cord in a sexually active young man, revealed by a scrotal mass mimicking a tumor of the testicle and discuss the suitable diagnostic and therapeutic procedures, with preservation of the testes and the other sexual organs.

  20. Perturbed cholesterol homeostasis in aging spinal cord.

    Science.gov (United States)

    Parkinson, Gemma M; Dayas, Christopher V; Smith, Doug W

    2016-09-01

    The spinal cord is vital for the processing of sensorimotor information and for its propagation to and from both the brain and the periphery. Spinal cord function is affected by aging, however, the mechanisms involved are not well-understood. To characterize molecular mechanisms of spinal cord aging, microarray analyses of gene expression were performed on cervical spinal cords of aging rats. Of the metabolic and signaling pathways affected, cholesterol-associated pathways were the most comprehensively altered, including significant downregulation of cholesterol synthesis-related genes and upregulation of cholesterol transport and metabolism genes. Paradoxically, a significant increase in total cholesterol content was observed-likely associated with cholesterol ester accumulation. To investigate potential mechanisms for the perturbed cholesterol homeostasis, we quantified the expression of myelin and neuroinflammation-associated genes and proteins. Although there was minimal change in myelin-related expression, there was an increase in phagocytic microglial and astrogliosis markers, particularly in the white matter. Together, these results suggest that perturbed cholesterol homeostasis, possibly as a result of increased inflammatory activation in spinal cord white matter, may contribute to impaired spinal cord function with aging. PMID:27459933

  1. Neuroimaging for spine and spinal cord surgery

    Energy Technology Data Exchange (ETDEWEB)

    Koyanagi, Izumi [Hokkaido Neurosurgical Memorial Hospital (Japan); Iwasaki, Yoshinobu; Hida, Kazutoshi

    2001-01-01

    Recent advances in neuroimaging of the spine and spinal cord are described based upon our clinical experiences with spinal disorders. Preoperative neuroradiological examinations, including magnetic resonance (MR) imaging and computerized tomography (CT) with three-dimensional reconstruction (3D-CT), were retrospectively analyzed in patients with cervical spondylosis or ossification of the posterior longitudinal ligament (130 cases), spinal trauma (43 cases) and intramedullary spinal cord tumors (92 cases). CT scan and 3D-CT were useful in elucidating the spine pathology associated with degenerative and traumatic spine diseases. Visualization of the deformity of the spine or fracture-dislocation of the spinal column with 3D-CT helped to determine the correct surgical treatment. MR imaging was most important in the diagnosis of both spine and spinal cord abnormalities. The axial MR images of the spinal cord were essential in understanding the laterality of the spinal cord compression in spinal column disorders and in determining surgical approaches to the intramedullary lesions. Although non-invasive diagnostic modalities such as MR imaging and CT scans are adequate for deciding which surgical treatment to use in the majority of spine and spinal cord disorders, conventional myelography is still needed in the diagnosis of nerve root compression in some cases of cervical spondylosis. (author)

  2. Transient Serotonin Syndrome by Concurrent Use of Electroconvulsive Therapy and Selective Serotonin Reuptake Inhibitor: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Nagahisa Okamoto

    2012-01-01

    Full Text Available The serotonin syndrome, which is characterized by psychiatric, autonomic nervous and neurological symptoms, is considered to be caused by excessive stimulation of the 5-HT1A and 5-HT2 receptors in the gray matter and spinal cord of the central nervous system, after the start of dosing or increase of the dose of a serotoninergic drug. There have been hardly any reports of induction of serotonin syndrome by electroconvulsive therapy (ECT in combination with antidepressant. We present the case of a female patient with major depressive disorder (MDD who developed transient serotonin syndrome soon after the first session of ECT in combination with paroxetine. Paroxetine was discontinued, and her psychiatric, autonomic nervous and neurological symptoms were gradually relieved and disappeared within 2 days. We performed the second ECT session 5 days after the initial session and performed 12 sessions of ECT without any changes in the procedure of ECT and anesthesia, but no symptoms of SS were observed. Finally, her MDD remitted. ECT might cause transiently increased blood-brain barrier (BBB permeability and enhance the transmissivity of the antidepressant in BBB. Therefore, it is necessary to pay attention to rare side effect of serotonin syndrome by ECT in combination with antidepressant.

  3. The treatment of neurodegenerative disorders using umbilical cord blood and menstrual blood-derived stem cells.

    Science.gov (United States)

    Sanberg, Paul R; Eve, David J; Willing, Alison E; Garbuzova-Davis, Svitlana; Tan, Jun; Sanberg, Cyndy D; Allickson, Julie G; Cruz, L Eduardo; Borlongan, Cesar V

    2011-01-01

    Stem cell transplantation is a potentially important means of treatment for a number of disorders. Two different stem cell populations of interest are mononuclear umbilical cord blood cells and menstrual blood-derived stem cells. These cells are relatively easy to obtain, appear to be pluripotent, and are immunologically immature. These cells, particularly umbilical cord blood cells, have been studied as either single or multiple injections in a number of animal models of neurodegenerative disorders with some degree of success, including stroke, Alzheimer's disease, amyotrophic lateral sclerosis, and Sanfilippo syndrome type B. Evidence of anti-inflammatory effects and secretion of specific cytokines and growth factors that promote cell survival, rather than cell replacement, have been detected in both transplanted cells.

  4. Spinal cord stimulators and radiotherapy: First case report and practice guidelines

    Directory of Open Access Journals (Sweden)

    Walsh Lorraine

    2011-10-01

    Full Text Available Abstract Spinal cord stimulators (SCS are a well-recognised treatment modality in the management of a number of chronic neuropathic pain conditions, particularly failed back syndrome and radiculopathies. The implantable pulse generator (IPG component of the SCS is designed and operates in a similar fashion to that of a cardiac pacemaker. The IPG consists of an electrical generator, lithium battery, transmitter/receiver and a minicomputer. When stimulated, it generates pulsed electrical signals which stimulate the dorsal columns of the spinal cord, thus alleviating pain. Analogous to a cardiac pacemaker, it can be potentially damaged by ionising radiation from a linear accelerator, in patients undergoing radiotherapy. Herein we report our clinical management of the first reported case of a patient requiring adjuvant breast radiotherapy who had a SCS in situ. We also provide useful practical recommendations on the management of this scenario within a radiation oncology department.

  5. Spinal cord stimulators and radiotherapy: First case report and practice guidelines

    International Nuclear Information System (INIS)

    Spinal cord stimulators (SCS) are a well-recognised treatment modality in the management of a number of chronic neuropathic pain conditions, particularly failed back syndrome and radiculopathies. The implantable pulse generator (IPG) component of the SCS is designed and operates in a similar fashion to that of a cardiac pacemaker. The IPG consists of an electrical generator, lithium battery, transmitter/receiver and a minicomputer. When stimulated, it generates pulsed electrical signals which stimulate the dorsal columns of the spinal cord, thus alleviating pain. Analogous to a cardiac pacemaker, it can be potentially damaged by ionising radiation from a linear accelerator, in patients undergoing radiotherapy. Herein we report our clinical management of the first reported case of a patient requiring adjuvant breast radiotherapy who had a SCS in situ. We also provide useful practical recommendations on the management of this scenario within a radiation oncology department

  6. Central Acute Vestibular Syndrome of Vascular Cause and Its Nystagmus Patterns%血管源性中枢急性前庭综合征及其眼震形式

    Institute of Scientific and Technical Information of China (English)

    鞠奕; 杨旭; 赵性泉

    2015-01-01

    The onset of acute vestibular syndrome (AVS) is characterized by rapid permanent vertigo, accompanying nausea, vomiting, spontaneous nystagmus, unsteady gait and intolerant head motion, these clinical symptoms last for more than 24 hours, the majority of AVS patients suffer for several days or weeks. Central AVS often originates from vascular causes, most of which are posterior circulation ischemic stroke. Even some patients with brain stem and cerebellar infarction present acute isolated vertigo, which is extremely challenging for diagnosis. With the development of researches about vestibular and eye movement physiology mechanism, bedside examination including vestibular, eye movement and posture balance system has become increasingly important. In addition to conventional neurology and otology examinations, nystagmus evaluation, which is based on the vestibular-eye reflex, contributes to rapid diagnosis of central AVS among vertigo patients. The present study elucidates the central AVS and nystagmus characteristics.%急性前庭综合征(acute vestibular syndrome,AVS)是以急性持续性眩晕起病,伴有恶心、呕吐,自发眼震,步态不稳,头动不能耐受等症状的临床综合征,持续时间超过24 h,大多为数天或数周。中枢性AVS以血管源性常见,其中大多数为后循环缺血性卒中,尤其部分脑干、小脑梗死常表现为急性孤立性眩晕,这类患者的诊断极具挑战性。随着前庭及眼动生理机制研究的深入,基于前庭、眼动及姿势平衡系统等方面的床旁检查重要性也日益凸显。眩晕患者的床旁检查,除了常规的神经科及耳科查体外,基于前庭-眼反射的各类眼震的评价极有助于快速诊断、识别中枢性AVS。本文对血管源性中枢性AVS的神经血管解剖基础、脑干和小脑卒中所致AVS的眼震特点等进行了综述归纳。

  7. Identification of Multiple Subsets of Ventral Interneurons and Differential Distribution along the Rostrocaudal Axis of the Developing Spinal Cord

    NARCIS (Netherlands)

    C. Francius (Cédric); A Harris (Anna); V. Rucchin (Vincent); T.J. Hendricks (Timothy); F.J. Stam (Floor); M. Barber (Melissa); D. Kurek (Dorota); F.G. Grosveld (Frank); A. Pierani (Alessandra); J. Goulding (John); F. Clotman (Frédéric)

    2013-01-01

    textabstractThe spinal cord contains neuronal circuits termed Central Pattern Generators (CPGs) that coordinate rhythmic motor activities. CPG circuits consist of motor neurons and multiple interneuron cell types, many of which are derived from four distinct cardinal classes of ventral interneurons,

  8. Structural and functional reorganization of propriospinal connections promotes functional recovery after spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Linard Filli; Martin E Schwab

    2015-01-01

    Axonal regeneration and ifber regrowth is limited in the adult central nervous system, but re-search over the last decades has revealed a high intrinsic capacity of brain and spinal cord circuits to adapt and reorganize after smaller injuries or denervation. Short-distance ifber growth and synaptic rewiring was found in cortex, brain stem and spinal cord and could be associated with restoration of sensorimotor functions that were impaired by the injury. Such processes of struc-tural plasticity were initially observed in the corticospinal system following spinal cord injury or stroke, but recent studies showed an equally high potential for structural and functional reorganization in reticulospinal, rubrospinal or propriospinal projections. Here we review the lesion-induced plastic changes in the propriospinal pathways, and we argue that they represent a key mechanism triggering sensorimotor recovery upon incomplete spinal cord injury. The for-mation or strengthening of spinal detour pathways bypassing supraspinal commands around the lesion site to the denervated spinal cord were identiifed as prominent neural substrate inducing substantial motor recovery in different species from mice to primates. Indications for the exis-tence of propriospinal bypasses were also found in humans after cortical stroke. It is mandatory for current research to dissect the biological mechanisms underlying spinal circuit remodeling and to investigate how these processes can be stimulated in an optimal way by therapeutic inter-ventions (e.g., ifber-growth enhancing interventions, rehabilitation). This knowledge will clear the way for the development of novel strategies targeting the remarkable plastic potential of pro-priospinal circuits to maximize functional recovery after spinal cord injury.

  9. Structural and functional reorganization of propriospinal connections promotes functional recovery after spinal cord injury

    Directory of Open Access Journals (Sweden)

    Linard Filli

    2015-01-01

    Full Text Available Axonal regeneration and fiber regrowth is limited in the adult central nervous system, but research over the last decades has revealed a high intrinsic capacity of brain and spinal cord circuits to adapt and reorganize after smaller injuries or denervation. Short-distance fiber growth and synaptic rewiring was found in cortex, brain stem and spinal cord and could be associated with restoration of sensorimotor functions that were impaired by the injury. Such processes of structural plasticity were initially observed in the corticospinal system following spinal cord injury or stroke, but recent studies showed an equally high potential for structural and functional reorganization in reticulospinal, rubrospinal or propriospinal projections. Here we review the lesion-induced plastic changes in the propriospinal pathways, and we argue that they represent a key mechanism triggering sensorimotor recovery upon incomplete spinal cord injury. The formation or strengthening of spinal detour pathways bypassing supraspinal commands around the lesion site to the denervated spinal cord were identified as prominent neural substrate inducing substantial motor recovery in different species from mice to primates. Indications for the existence of propriospinal bypasses were also found in humans after cortical stroke. It is mandatory for current research to dissect the biological mechanisms underlying spinal circuit remodeling and to investigate how these processes can be stimulated in an optimal way by therapeutic interventions (e.g., fiber-growth enhancing interventions, rehabilitation. This knowledge will clear the way for the development of novel strategies targeting the remarkable plastic potential of propriospinal circuits to maximize functional recovery after spinal cord injury.

  10. Serotonergic modulation of post-synaptic inhibition and locomotor alternating pattern in the spinal cord

    Directory of Open Access Journals (Sweden)

    Laurent eVinay

    2014-08-01

    Full Text Available The central pattern generators (CPGs for locomotion, located in the lumbar spinal cord, are functional at birth in the rat. Their maturation occurs during the last few days preceding birth, a period during which the first projections from the brainstem start to reach the lumbar enlargement of the spinal cord. Locomotor burst activity in the mature intact spinal cord alternates between flexor and extensor motoneurons through reciprocal inhibition and between left and right sides through commisural inhibitory interneurons. By contrast, all motor bursts are in phase in the fetus. The alternating pattern disappears after neonatal spinal cord transection which suppresses supraspinal influences upon the locomotor networks. This article will review the role of serotonin (5-HT, in particular 5-HT2 receptors, in shaping the alternating pattern. For instance, pharmacological activation of these receptors restores the left-right alternation after injury. Experiments aimed at either reducing the endogenous level of serotonin in the spinal cord or blocking the activation of 5-HT2 receptors.We then describe recent evidence that the action of 5-HT2 receptors is mediated, at least in part, through a modulation of chloride homeostasis. The postsynaptic action of GABA and glycine depends on the intracellular concentration of chloride ions which is regulated by a protein in the plasma membrane, the K+-Cl− cotransporter (KCC2 extruding both K+ and Cl− ions. Absence or reduction of KCC2 expression leads to a depolarizing action of GABA and glycine and a marked reduction in the strength of postsynaptic inhibition. This latter situation is observed early during development and in several pathological conditions, such as after spinal cord injury, thereby causing spasticity and chronic pain. It was recently shown that specific activation of 5-HT2A receptors is able to up-regulate KCC2, restore endogenous inhibition and reduce spasticity.

  11. Differential expression of Wnts after spinal cord contusion injury in adult rats.

    Directory of Open Access Journals (Sweden)

    Carmen María Fernández-Martos

    Full Text Available BACKGROUND: Spinal cord injury is a major cause of disability that has no clinically accepted treatment. Functional decline following spinal cord injury is caused by mechanical damage, secondary cell death, reactive gliosis and a poor regenerative capacity of damaged axons. Wnt proteins are a family of secreted glycoproteins that play key roles in different developmental processes although little is known of the expression patterns and functions of Wnts in the adult central nervous system in normal or diseased states. FINDINGS: Using qRT-PCR analysis, we demonstrate that mRNA encoding most Wnt ligands and soluble inhibitors are constitutively expressed in the healthy adult spinal cord. Strikingly, contusion spinal cord injury induced a time-dependent increase in Wnt mRNA expression from 6 hours until 28 days post-injury, and a narrow peak in the expression of soluble Wnt inhibitors between 1 and 3 days post-injury. These results are consistent with the increase in the migration shift, from day 1 to 7, of the intracellular Wnt signalling component, Dishevelled-3. Moreover, after an initial decrease by 1 day, we also found an increase in phosphorylation of the Wnt co-receptor, low-density lipoprotein receptor-related protein 6, and an increase in active β-catenin protein, both of which suffer a dramatic change, from a homogeneous expression pattern in the grey matter to a disorganized injury-induced pattern. CONCLUSIONS: Our results suggest a role for Wnts in spinal cord homeostasis and injury. We demonstrate that after injury Wnt signalling is activated via the Wnt/β-catenin and possibly other pathways. These findings provide an important foundation to further address the function of individual Wnt proteins in vivo and the pathophysiology of spinal cord injury.

  12. Thoracic rat spinal cord contusion injury induces remote spinal gliogenesis but not neurogenesis or gliogenesis in the brain.

    Directory of Open Access Journals (Sweden)

    Steffen Franz

    Full Text Available After spinal cord injury, transected axons fail to regenerate, yet significant, spontaneous functional improvement can be observed over time. Distinct central nervous system regions retain the capacity to generate new neurons and glia from an endogenous pool of progenitor cells and to compensate neural cell loss following certain lesions. The aim of the present study was to investigate whether endogenous cell replacement (neurogenesis or gliogenesis in the brain (subventricular zone, SVZ; corpus callosum, CC; hippocampus, HC; and motor cortex, MC or cervical spinal cord might represent a structural correlate for spontaneous locomotor recovery after a thoracic spinal cord injury. Adult Fischer 344 rats received severe contusion injuries (200 kDyn of the mid-thoracic spinal cord using an Infinite Horizon Impactor. Uninjured rats served as controls. From 4 to 14 days post-injury, both groups received injections of bromodeoxyuridine (BrdU to label dividing cells. Over the course of six weeks post-injury, spontaneous recovery of locomotor function occurred. Survival of newly generated cells was unaltered in the SVZ, HC, CC, and the MC. Neurogenesis, as determined by identification and quantification of doublecortin immunoreactive neuroblasts or BrdU/neuronal nuclear antigen double positive newly generated neurons, was not present in non-neurogenic regions (MC, CC, and cervical spinal cord and unaltered in neurogenic regions (dentate gyrus and SVZ of the brain. The lack of neuronal replacement in the brain and spinal cord after spinal cord injury precludes any relevance for spontaneous recovery of locomotor function. Gliogenesis was increased in the cervical spinal cord remote from the injury site, however, is unlikely to contribute to functional improvement.

  13. An ex vivo laser-induced spinal cord injury model to assess mechanisms of axonal degeneration in real-time.

    Science.gov (United States)

    Okada, Starlyn L M; Stivers, Nicole S; Stys, Peter K; Stirling, David P

    2014-01-01

    Injured CNS axons fail to regenerate and often retract away from the injury site. Axons spared from the initial injury may later undergo secondary axonal degeneration. Lack of growth cone formation, regeneration, and loss of additional myelinated axonal projections within the spinal cord greatly limits neurological recovery following injury. To assess how central myelinated axons of the spinal cord respond to injury, we developed an ex vivo living spinal cord model utilizing transgenic mice that express yellow fluorescent protein in axons and a focal and highly reproducible laser-induced spinal cord injury to document the fate of axons and myelin (lipophilic fluorescent dye Nile Red) over time using two-photon excitation time-lapse microscopy. Dynamic processes such as acute axonal injury, axonal retraction, and myelin degeneration are best studied in real-time. However, the non-focal nature of contusion-based injuries and movement artifacts encountered during in vivo spinal cord imaging make differentiating primary and secondary axonal injury responses using high resolution microscopy challenging. The ex vivo spinal cord model described here mimics several aspects of clinically relevant contusion/compression-induced axonal pathologies including axonal swelling, spheroid formation, axonal transection, and peri-axonal swelling providing a useful model to study these dynamic processes in real-time. Major advantages of this model are excellent spatiotemporal resolution that allows differentiation between the primary insult that directly injures axons and secondary injury mechanisms; controlled infusion of reagents directly to the perfusate bathing the cord; precise alterations of the environmental milieu (e.g., calcium, sodium ions, known contributors to axonal injury, but near impossible to manipulate in vivo); and murine models also offer an advantage as they provide an opportunity to visualize and manipulate genetically identified cell populations and subcellular

  14. Umbilical cord blood testosterone and childhood internalizing and externalizing behavior: a prospective study.

    Directory of Open Access Journals (Sweden)

    Monique Robinson

    Full Text Available Antenatal testosterone exposure influences fetal neurodevelopment and gender-role behavior in postnatal life and may contribute to differences in developmental psychopathology during childhood. We prospectively measured the associations between umbilical cord blood testosterone levels at birth and childhood behavioral development in both males and females from a large population based sample. The study comprised 430 females and 429 males from the Western Australian Pregnancy Cohort (Raine Study where umbilical cord blood had been collected. Total testosterone concentrations were determined by mass spectrometry and bioavailable testosterone (BioT levels were calculated. At two, five, eight and ten years of age, the participants completed the Child Behavior Checklist (CBCL. Linear regression models were used to analyse the relationship between BioT concentrations (in quartiles and CBCL scores (total, internalizing, externalizing and selected syndrome. Boys had higher mean CBCL T-scores than girls across all ages of follow-up. There was no significant relationship between cord blood BioT quartiles and CBCL total, internalizing and externalizing T-scores at age two or five to ten combined. In the syndrome score analyses, higher BioT quartiles were associated with significantly lower scores for attention problems for boys at age five, eight and ten, and greater withdrawal symptoms in pre-school girls (age five. We did not identify a consistent relationship between antenatal testosterone exposure and total, internalizing or externalizing behavioral difficulties in childhood. Higher umbilical cord BioT levels were associated with lower scores for attention problems in boys up to 10 years and more withdrawn behavior in 5-year-old girls; however, these findings were not consistent across ages and require further investigation in a larger sample.

  15. Prenatal and postnatal prevalence of Turner's syndrome: a registry study.

    OpenAIRE

    Gravholt, C. H.; Juul, S; Naeraa, R. W.; Hansen, J.

    1996-01-01

    OBJECTIVE--To study prevalence of Turner's syndrome in Denmark and to assess validity of prenatal diagnosis. DESIGN--Study of data on prenatal and postnatal Turner's syndrome in Danish Cytogenetic Central Register. SUBJECTS--All registered Turner's syndrome karyotypes (100 prenatal cases and 215 postnatal cases) during 1970-93. MAIN OUTCOME MEASURES--Prevalence of Turner's syndrome karyotypes among prenatally tested fetuses and Turner's syndrome among liveborn infants. RESULTS--Among infant g...

  16. [Alport's syndrome (author's transl)].

    Science.gov (United States)

    Huismans, H

    1978-05-01

    A case report is given of a 22-years old student (whose brother had Alport's syndrome) with recurrent central corneal swelling and paracentral erosions of the cornea of both eyes. Further signs of beginning Alport's syndrome in this case are disturbance of re-adaptation after dazzling (Mesoptometer) and paracentral scotomata in the visual fields. Remarkable was the small diameter of the disc in both eyes (1.37 mm). Local therapy was Scopolamin-eye-drops, Actihaemyl- and especially Cystein-Gel (2.4%). PMID:672101

  17. Age-Dependent Modulation of Cortical Transcriptomes in Spinal Cord Injury and Repair

    OpenAIRE

    Jaerve, Anne; Kruse, Fabian; Malik, Katharina; Hartung, Hans-Peter; Müller, Hans Werner

    2012-01-01

    Both injury and aging of the central nervous system reportedly produce profound changes in gene expression. Therefore, aging may interfere with the success of therapeutic interventions which were tailored for young patients. Using genome-scale transcriptional profiling, we identified distinct age-dependent expression profiles in rat sensorimotor cortex during acute, subacute and chronic phases of spinal cord injury (SCI). Aging affects the cortical transcriptomes triggered by transection of t...

  18. Chondroitinase ABC Improves Basic and Skilled Locomotion in Spinal Cord Injured Cats

    OpenAIRE

    Tester, Nicole J.; Howland, Dena R.

    2007-01-01

    Chondroitin sulfate proteoglycans (CSPGs) are upregulated in the central nervous system following injury. Chondroitin sulfate glycosaminoglycan (CS GAG) side chains substituted on this family of molecules contribute to the limited functional recovery following injury by restricting axonal growth and synaptic plasticity. In the current study, the effects of degrading CS GAGs with Chondroitinase ABC (Ch’ase ABC) in the injured spinal cords of adult cats were assessed. Three groups were evaluate...

  19. Research progress on the treatment of spinal cord injury with cellular transplantation

    Institute of Scientific and Technical Information of China (English)

    Cheng Lu; Qiang Shen

    2009-01-01

    Spinal cord injury(SCI) is a severe trauma to the central nervous system(CNS). This article reviews recent advances in cellular transplantation to treat SCI. Transplanted cells can supply new neurons to replace injured ones, promote regeneration of axons and myelin sheath, modulate the inflammatory response, and thus promote recovery from traumatic injury of the CNS. Cellular transplantation is a promising potential method for the treatment of SCI.

  20. Management of intramedullary spinal cord abscess: experience with four cases, pathophysiology and outcomes

    OpenAIRE

    Al Barbarawi, Moh’d.; Khriesat, Wadah; Qudsieh, Suhair; Qudsieh, Hanna; Loai, Abu Alia

    2009-01-01

    Intramedullary spinal cord abscess (ISCA) in children is extremely a rare infection of the central nervous system (CNS); and probably a devastating neurological condition. Clinical awareness of patients at risk is crucial for early diagnosis and intervention; as this entity is one of the treatable conditions of paraparesis. Association with congenital neuro-ectodermal abnormality in children is frequent. This pathology highlights the importance of complete neurological checks of infants as a ...

  1. Effect of Fenugreek Seed Extract (Trigonella Foenum-graecum on Brachial Region of the Spinal Cord of an 18-Day Old Rat Offspring with Diabetes

    Directory of Open Access Journals (Sweden)

    Z Khaksar

    2013-08-01

    Full Text Available Abstract Background & aim: Diabetes can affect the spinal cord, an important part of the central nervous system. Fenugreek seed has been suggested to have potential antidiabetic effects. This study was conducted to assess the effects of Fenugreek seed extract (trigonella foenum-graecum on fetal spinal cord structure, specifically in the brachial enlargement cord in an 18-day old fetus of diabetic mothers treated with extracts in comparison with the control groups' mothers. Methods: In the present cross-sectional study, sixteen healthy female rats were randomly divided into four equal groups: Healthy control, Diabetes control, Fenugreek control, Fenugreek treatment. Diabetes was induced by intraperitoneal injection of Streptozotcin (50 mg/kg. All four groups became pregnant by natural mating. After formation of the nervous system, two fetuses from each group were expelled by cesarean after performing anesthesia on the animals on 18th day of pregnancy furthermore their brachial enlargement spinal cord samples were taken. Finally, the tissue sections were prepared by routine procedures and diverse histological parameters were examined. The collected data were analyzed by one-way ANOVA. Results: results showed that fetal body weight on the diabetic control group was significantly higher compared to the other groups (P≤ 0/05. Moreover, significant reductions in the transvers and vertical diameters in central channel of the spinal cord and as well in the number of neurons of the spinal cord gray matter in the diabetic control groups in comparison with the other groups were observed (P≤0/05. Conclusion: The hyperglycemic effect of maternal diabetes during fetal period causes abnormalities, especially in the brachial enlargement of spinal cord, including changes in the spinal cord and neurons number in the gray matter. Disorders occurring in the prenatal remains and may perhaps cause lack of ability to perform certain physical activities. Key words

  2. Neuregulin-1 controls an endogenous repair mechanism after spinal cord injury.

    Science.gov (United States)

    Bartus, Katalin; Galino, Jorge; James, Nicholas D; Hernandez-Miranda, Luis R; Dawes, John M; Fricker, Florence R; Garratt, Alistair N; McMahon, Stephen B; Ramer, Matt S; Birchmeier, Carmen; Bennett, David L H; Bradbury, Elizabeth J

    2016-05-01

    Following traumatic spinal cord injury, acute demyelination of spinal axons is followed by a period of spontaneous remyelination. However, this endogenous repair response is suboptimal and may account for the persistently compromised function of surviving axons. Spontaneous remyelination is largely mediated by Schwann cells, where demyelinated central axons, particularly in the dorsal columns, become associated with peripheral myelin. The molecular control, functional role and origin of these central remyelinating Schwann cells is currently unknown. The growth factor neuregulin-1 (Nrg1, encoded by NRG1) is a key signalling factor controlling myelination in the peripheral nervous system, via signalling through ErbB tyrosine kinase receptors. Here we examined whether Nrg1 is required for Schwann cell-mediated remyelination of central dorsal column axons and whether Nrg1 ablation influences the degree of spontaneous remyelination and functional recovery following spinal cord injury. In contused adult mice with conditional ablation of Nrg1, we found an absence of Schwann cells within the spinal cord and profound demyelination of dorsal column axons. There was no compensatory increase in oligodendrocyte remyelination. Removal of peripheral input to the spinal cord and proliferation studies demonstrated that the majority of remyelinating Schwann cells originated within the injured spinal cord. We also examined the role of specific Nrg1 isoforms, using mutant mice in which only the immunoglobulin-containing isoforms of Nrg1 (types I and II) were conditionally ablated, leaving the type III Nrg1 intact. We found that the immunoglobulin Nrg1 isoforms were dispensable for Schwann cell-mediated remyelination of central axons after spinal cord injury. When functional effects were examined, both global Nrg1 and immunoglobulin-specific Nrg1 mutants demonstrated reduced spontaneous locomotor recovery compared to injured controls, although global Nrg1 mutants were more impaired in

  3. [Central nervous system tumors in pregnancy].

    Science.gov (United States)

    Podciechowski, Lech; Nowakowska, Dorota; Bielak, Adam; Nowosławska, Emilia; Szymański, Wojciech; Polis, Lech; Krasomski, Grzechorz; Fiks, Tomasz; Wilczyński, Jan

    2003-12-01

    Central nervous system tumour in pregnancy constitutes a serious complication. Considering frequent difficulties in diagnostics and therapy, the aim of the study was to present our experience in management with pregnant women with brain and spinal cord tumours. Between 1988-2000, in The Research Institute Polish Mother's Memorial Hospital in Lodzi, 4 pregnant women had been diagnosed with brain and spinal cord tumours. The incidence of tumours complicating pregnancy was 1/11460. Two patients diagnosed at 29 weeks' gestation, underwent craniotomy and tumour resection during pregnancy. Two other women with central nervous system tumours diagnosed at 39 weeks' gestation, were operated in the postpartum period. The analysis of the postoperative period, gestation and/or postpartum period in all women and well-being of their new-borns confirm undertaken medical decisions. PMID:15029742

  4. Neuromesodermal progenitors and the making of the spinal cord

    Science.gov (United States)

    Henrique, Domingos; Abranches, Elsa; Verrier, Laure; Storey, Kate G.

    2016-01-01

    Neuromesodermal progenitors (NMps) contribute to both the elongating spinal cord and the adjacent paraxial mesoderm. It has been assumed that these cells arise as a result of patterning of the anterior neural plate. However, as the molecular mechanisms that specify NMps in vivo are uncovered, and as protocols for generating these bipotent cells from mouse and human pluripotent stem cells in vitro are established, the emerging data suggest that this view needs to be revised. Here, we review the characteristics, regulation, in vitro derivation and in vivo induction of NMps. We propose that these cells arise within primitive streak-associated epiblast via a mechanism that is separable from that which establishes neural fate in the anterior epiblast. We thus argue for the existence of two distinct routes for making central nervous system progenitors. PMID:26329597

  5. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  6. Cushing's Syndrome

    Science.gov (United States)

    Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone ... cause your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

  7. Cushing's Syndrome

    Science.gov (United States)

    ... Cushing's syndrome, also called hypercortisolism , is a rare endocrine disorder caused by chronic exposure of the body's tissues ... removing the tumor while minimizing the chance of endocrine deficiency or long-term ... for Cushing's Syndrome Clinical Trials ...

  8. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of ...

  9. CT and MRI of congenital nasal lesions in syndromic conditions

    Energy Technology Data Exchange (ETDEWEB)

    Ginat, Daniel T. [University of Chicago, Department of Radiology, Chicago, IL (United States); Robson, Caroline D. [Harvard Medical School, Department of Radiology, Boston Children' s Hospital, Boston, MA (United States)

    2015-07-15

    Congenital malformations of the nose can be associated with a variety of syndromes, including solitary median maxillary central incisor syndrome, CHARGE syndrome, Bosma syndrome, median cleft face syndrome, PHACES association, Bartsocas-Papas syndrome, Binder syndrome, duplication of the pituitary gland-plus syndrome and syndromic craniosynsotosis (e.g., Apert and Crouzon syndromes) among other craniofacial syndromes. Imaging with CT and MRI plays an important role in characterizing the nasal anomalies as well as the associated brain and cerebrovascular lesions, which can be explained by the intimate developmental relationship between the face and intracranial structures, as well as certain gene mutations. These conditions have characteristic imaging findings, which are reviewed in this article. (orig.)

  10. CT and MRI of congenital nasal lesions in syndromic conditions.

    Science.gov (United States)

    Ginat, Daniel T; Robson, Caroline D

    2015-07-01

    Congenital malformations of the nose can be associated with a variety of syndromes, including solitary median maxillary central incisor syndrome, CHARGE syndrome, Bosma syndrome, median cleft face syndrome, PHACES association, Bartsocas-Papas syndrome, Binder syndrome, duplication of the pituitary gland-plus syndrome and syndromic craniosynsotosis (e.g., Apert and Crouzon syndromes) among other craniofacial syndromes. Imaging with CT and MRI plays an important role in characterizing the nasal anomalies as well as the associated brain and cerebrovascular lesions, which can be explained by the intimate developmental relationship between the face and intracranial structures, as well as certain gene mutations. These conditions have characteristic imaging findings, which are reviewed in this article. PMID:25573243

  11. Cord Serum Lipid Profile of Infants of Diabetic Mothers

    Directory of Open Access Journals (Sweden)

    Jasim Almusawi

    2015-12-01

    Full Text Available Background: Infants of diabetic mothers (IDM is a critical issue in pediatrics, which is regarded as a major risk factor for birth trauma, respiratory distress syndrome (RDS, birth asphyxia, transient tachypnea of the newborn (TTN and jaundice. IDM is also a risk factor for microvascular (e.g., ocular and renal complications and macrovascular complications (e.g., cerebrovascular accident, atherosclerosis and cardiovascular complications. Lipids are a heterogeneous group of hydrophobic organic molecules which can be extracted from tissues using non-polar solvents. Lipids, due to their hydrophobic property, are mainly found in membranes enclosing various cell organelles. Diabetes mellitus management with insulin (nowadays also with oral hypoglycemic medications has improved the outcomes of gestational diabetes mellitus (GDM (most infants born to diabetic mother are large for gestational age. The neonatal mortality rate in IDM is over five times higher than that of infants of non-diabetic mothers. In this study, therefore, we aimed to assess the effect of maternal diabetes on cord serum lipid profile. Methods: This prospective (case-control study was carried out on 60 infants born in Al-Zahra teaching hospital during February 2014–October 2014. The study group consisted of 30 randomly chosen IDM, and the control group comprised 30 infants who were born to healthy mothers. Results: The results of this study demonstrated that there are significant differences between IDM and infants of healthy mothers regarding lipid profile and birth weight. Conclusion: This study confirms that cord serum lipid profile (serum cholesterol, serum triglycerides and low-density lipoprotein is higher at birth in IDM. Moreover, this study shows a significant association between lipid profile and body weight.

  12. 颅脑创伤后尿崩症合并脑性盐耗综合征%Combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury

    Institute of Scientific and Technical Information of China (English)

    吴雪海; 高亮; 胡锦; 吴惺; 郎黎琴; 金毅; 毛颖; 周良辅

    2011-01-01

    目的 探讨如何正确认识、早期及时诊断和准确治疗尿崩症合并脑性盐耗综合征,以提高救治成功率.方法 回顾性分析6例尿崩症合并脑性盐耗综合征的临床特点:尿量特别异常增多、高脑利钠肽水平和显著尿钠排泄增加是其最显著特征;尿比重、血钠水平和血浆渗透压可以正常;尿量增多单纯加压素疗效不佳,而加用醋酸可的松治疗有效.结果 6例患者及时正确诊断为尿崩症合并脑性盐耗综合征,但其中1例患者因为血钠上升过快出现脑桥脱髓鞘病变,最终植物状态;1例患者因脑疝、多脏衰而死亡;其余4例患者治愈出院.结论 根据尿崩症合并脑性盐耗综合征的临床特点本文提出其参考诊断标准,及时诊断和正确治疗是成功救治的关键.%Objective To discuss the diagnosis coexistence of central diabetes insipidus and cerebral salt wasting syndrome promptly and treat properly.Method Six patients who suffered central diabetes insipidus combined cerebral salt wasting syndrome were analyzed retrospectively.Its characteristics was massive polyuria which can not be controlled by vasopressin alone,but can be controlled by both vasopresin and cortisone acetate.Sodium chloride saline were mainly used to make up for the loss of water and salt.Other charactefistcs were including low CVP,high Pro - BNP,high 24 h urine sodium evacuation with normal serum sodium level,higher osmolarity in urine than in serum which was at normal level,and usually normal urine specific gravity.Results One died and one became vegetative state,other four patients discharged with Glasgow Coma Scale 15.Conclusions Monitoring of water and sodium metabolism systemically were critical to diagnose the concurrent DI and CSW.Slow sodium chloride saline supplement,cortisone acetate and desmopressin were the keys to deal with concurrent DI and CSW.With exact diagnosis and treatment promptly,favourable outcome can be achieved.

  13. National Down Syndrome Society

    Science.gov (United States)

    ... info@ndss.org Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ... More » Follow us Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ...

  14. Learning about Marfan Syndrome

    Science.gov (United States)

    ... genetic terms used on this page Learning About Marfan Syndrome What is Marfan syndrome? What are the ... Syndrome Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most ...

  15. Expression of Nav1.7 in DRG neurons extends from peripheral terminals in the skin to central preterminal branches and terminals in the dorsal horn

    Directory of Open Access Journals (Sweden)

    Black Joel A

    2012-11-01

    Full Text Available Abstract Background Sodium channel Nav1.7 has emerged as a target of considerable interest in pain research, since loss-of-function mutations in SCN9A, the gene that encodes Nav1.7, are associated with a syndrome of congenital insensitivity to pain, gain-of-function mutations are linked to the debiliting chronic pain conditions erythromelalgia and paroxysmal extreme pain disorder, and upregulated expression of Nav1.7 accompanies pain in diabetes and inflammation. Since Nav1.7 has been implicated as playing a critical role in pain pathways, we examined by immunocytochemical methods the expression and distribution of Nav1.7 in rat dorsal root ganglia neurons, from peripheral terminals in the skin to central terminals in the spinal cord dorsal horn. Results Nav1.7 is robustly expressed within the somata of peptidergic and non-peptidergic DRG neurons, and along the peripherally- and centrally-directed C-fibers of these cells. Nav1.7 is also expressed at nodes of Ranvier in a subpopulation of Aδ-fibers within sciatic nerve and dorsal root. The peripheral terminals of DRG neurons within skin, intraepidermal nerve fibers (IENF, exhibit robust Nav1.7 immunolabeling. The central projections of DRG neurons in the superficial lamina of spinal cord dorsal horn also display Nav1.7 immunoreactivity which extends to presynaptic terminals. Conclusions The expression of Nav1.7 in DRG neurons extends from peripheral terminals in the skin to preterminal central branches and terminals in the dorsal horn. These data support a major contribution for Nav1.7 in pain pathways, including action potential electrogenesis, conduction along axonal trunks and depolarization/invasion of presynaptic axons. The findings presented here may be important for pharmaceutical development, where target engagement in the right compartment is essential.

  16. Central blood pressure and pulse wave velocity: relationship to target organ damage and cardiovascular morbidity-mortality in diabetic patients or metabolic syndrome. An observational prospective study. LOD-DIABETES study protocol

    Directory of Open Access Journals (Sweden)

    Castaño-Sánchez Carmen

    2010-03-01

    Full Text Available Abstract Background Diabetic patients show an increased prevalence of non-dipping arterial pressure pattern, target organ damage and elevated arterial stiffness. These alterations are associated with increased cardiovascular risk. The objectives of this study are the following: to evaluate the prognostic value of central arterial pressure and pulse wave velocity in relation to the incidence and outcome of target organ damage and the appearance of cardiovascular episodes (cardiovascular mortality, myocardial infarction, chest pain and stroke in patients with type 2 diabetes mellitus or metabolic syndrome. Methods/Design Design: This is an observational prospective study with 5 years duration, of which the first year corresponds to patient inclusion and initial evaluation, and the remaining four years to follow-up. Setting: The study will be carried out in the urban primary care setting. Study population: Consecutive sampling will be used to include patients diagnosed with type 2 diabetes between 20-80 years of age. A total of 110 patients meeting all the inclusion criteria and none of the exclusion criteria will be included. Measurements: Patient age and sex, family and personal history of cardiovascular disease, and cardiovascular risk factors. Height, weight, heart rate and abdominal circumference. Laboratory tests: hemoglobin, lipid profile, creatinine, microalbuminuria, glomerular filtration rate, blood glucose, glycosylated hemoglobin, blood insulin, fibrinogen and high sensitivity C-reactive protein. Clinical and 24-hour ambulatory (home blood pressure monitoring and self-measured blood pressure. Common carotid artery ultrasound for the determination of mean carotid intima-media thickness. Electrocardiogram for assessing left ventricular hypertrophy. Ankle-brachial index. Retinal vascular study based on funduscopy with non-mydriatic retinography and evaluation of pulse wave morphology and pulse wave velocity using the SphygmoCor system. The

  17. Seminal plasma PSA in spinal cord injured men

    DEFF Research Database (Denmark)

    Brasso, K; Sønksen, J; Sommer, P;

    1998-01-01

    The aim of the study was to evaluate the impact of spinal cord injury on seminal plasma PSA concentration.......The aim of the study was to evaluate the impact of spinal cord injury on seminal plasma PSA concentration....

  18. Vocational Rehabilitation of Persons with Spinal Cord Injuries

    Science.gov (United States)

    Poor, Charles R.

    1975-01-01

    Reviews historical development of organized vocational rehabilitation programming for the spinal cord injured in the United States. Significant factors that affect vocational rehabilitation outcomes with spinal cord injured persons are listed and discussed. (Author)

  19. Spinal-cord swelling in acute multiple sclerosis

    International Nuclear Information System (INIS)

    Despite the frequent involvement of the spinal cord by multiple sclerosis, reports concerning neuroradiological findings regarding these lesions have been limited; most of them have demonstrated a normal or small spinal cord. Two cases of acute paraparesis showed evidence of spinal-cord swelling on myelography and CT myelography, initially suggesting the diagnosis of an intramedullary tumor. Spinal-cord swelling was demonstrated more clearly on CT myelography than on conventional myelography. The diagnosis of multiple sclerosis was made with the aid of the CSF findings, the clinical course, and the contracting-cord sign. The ''contracting-cord sign'' means the diminution of the spinal-cord diameter in the chronic stage. Since acute multiple sclerosis may produce spinal-cord swelling simulating a tumor, careful investigations are necessary to avoid unwarranted surgical interventions. (author)

  20. Mechanisms of symptomatic spinal cord ischemia after TEVAR

    DEFF Research Database (Denmark)

    Czerny, Martin; Eggebrecht, Holger; Sodeck, Gottfried;

    2012-01-01

    To test the hypothesis that simultaneous closure of at least 2 independent vascular territories supplying the spinal cord and/or prolonged hypotension may be associated with symptomatic spinal cord ischemia (SCI) after thoracic endovascular aortic repair (TEVAR)....