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Sample records for central cord syndrome

  1. Spinal cord injury with central cord syndrome from surfing.

    Steinfeld, Yaniv; Keren, Yaniv; Haddad, Elias

    2018-01-01

    Central cord syndrome (CCS) is an injury to the center of the spinal cord. It is well known as a hyperextension injury, but it has never been described as a surfing injury. Our report describes this injury in detail. A 35-year-old male novice surfer presented to the emergency department with acute tetraplegia following falling off his surfboard and hitting sea floor at a shallow beach break. He was rescued by a fellow surfer while floating in the sea and unable to raise his head above sea level. Upon arrival at the hospital, tetraplegia and sensory deficits were noted. Radiological investigations showed advanced spinal stenosis at C4-6 levels. T2 magnetic resonance imaging (MRI) demonstrated myelopathy at C5-C6 level. He was diagnosed as having central cord syndrome, treated conservatively, and regained near full neurologic recovery after a month of rehabilitation. Unique sport activities lead to unique injuries. It is important to accurately describe these injuries in order to create protective measures against them. Neurologic injuries in surfers are uncommon. With low-energy trauma, surfer's myelopathy is still the most common diagnosis, but central cord syndrome should be in the differential diagnosis.

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

    Quencer, R.M.; Bunge, R.P.; Egnor, M.; Green, B.A.; Puckett, W.; Naidich, T.P.; Post, M.J.D.; Norenberg, M.

    1992-01-01

    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. Acute traumatic central cord syndrome: MRI-pathological correlations

    Quencer, R.M. (Dept. of Radiology, Univ. of Miami MRI Center, FL (United States) Miami Project to Cure Paralysis, FL (United States)); Bunge, R.P.; Egnor, M.; Green, B.A. (Miami Project to Cure Paralysis, FL (United States) Dept. of Neurological Surgery, Univ. of Miami School of Medicine, FL (United States)); Puckett, W. (Miami Project to Cure Paralysis, FL (United States)); Naidich, T.P. (Dept. of Radiology, Univ. of Miami MRI Center, FL (United States) Miami Project to Cure Paralysis, FL (United States) Baptist Hospital of Greater Miami, FL (United States)); Post, M.J.D. (Dept. of Radiology, Univ. of Miami MRI Center, FL (United States)); Norenberg, M. (Dept. of Neuropathology, Univ. of Miami School of Medicine, FL (United States))

    1992-04-01

    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).

  4. Acute traumatic central cord syndrome: analysis of clinical and radiological correlations.

    Miranda, P; Gomez, P; Alday, R

    2008-12-01

    In patients with traumatic spinal cord injury, several studies correlate neurological impairment and radiological findings. However, little information is available about this correspondence in the particular group of acute traumatic central cord syndrome. The object of the present work was to describe the clinical and radiological features of a series of patients presenting with acute traumatic central cord syndrome and to analyze clinical and radiological correlations on admission and at last follow-up. Retrospective review of 15 patients diagnosed of acute traumatic central cord syndrome between 1995 and 2005. Global motor score and motor score in upper extremities were determined on admission and at last follow-up (6 months-4 years, mean 16 months). Plain films, cervical computed tomography and magnetic resonance (MR) were performed in every patient and retrieved for the study. In seven patients, serial MR studies were performed during follow-up. Clinical and radiological correlations were statistically analyzed with non-parametric tests. Cervical spondylosis appeared associated with older age, falls, and absence of fracture. Spinal cord edema was the most common finding in MR studies but hemorrhage was also observed. The length of spinal cord edema significantly correlated with initial motor score. The decrease in T2-weighted hyperintensity in serial MR studies correlated with the gain of motor power in upper limbs at last follow-up. Elderly patients with more degenerated cervical spines commonly develop acute traumatic central cord syndrome after incidental falls. Length of spinal cord edema correlates with neurological impairment on admission and may provide significant prognostic information.

  5. Vocal cord collapse during phrenic nerve-paced respiration in congenital central hypoventilation syndrome.

    Domanski, Mark C; Preciado, Diego A

    2012-01-01

    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. 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. 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. The lack of vocal cord abduction on inspiration presents a limit to phrenic nerve pacers.

  6. The significance of dorsal migration of the cord after extensive cervical laminectomy for patients with traumatic central cord syndrome.

    Levi, L; Wolf, A; Mirvis, S; Rigamonti, D; Fianfaca, M S; Monasky, M

    1995-08-01

    Central cord syndrome (CCS) resulting from traumatic cervical injury is often associated with cervical stenosis and/or spondylosis. The efficacy of multilevel laminectomy in the treatment of this condition has been controversial. The objective of this study was to validate by magnetic resonance (MR) imaging the occurrence of dorsal cord migration after extensive laminectomy for patients with the clinical syndrome of central cord damage and its relationship to the short-term outcome. During a 28-month period, the authors evaluated 20 patients (mean age 54 years) who sustained CCS after cervical spine trauma. Pre- and postoperative MR imaging assessed the adequacy of cervical cord decompression by multilevel laminectomy. All patients had cervical canal stenosis with complete obliteration of the anterior subarachnoid space over multiple levels. Seventeen patients initially had CCS of different degrees of severity. One had no motor deficit and two had motor complete with sensory deficits greater in their arms. Laminectomy, generally from C2 to C7 without facetectomy, was performed after plateau of neurological recovery (mean 17 days postinjury). Neurological assessment 3 months after operation revealed improvement in 12, stable function in 7, and progression of deficit in one with no mortality. The postoperative midsagittal MR images demonstrated adequate decompression with restoration of anterior cerebrospinal fluid space and posterior cord migration in 12 patients (60% of the 20), but only 8 of them also had improved function. MR imaging demonstrated that, in the presence of multilevel spondylosis/stenosis, laminectomy may provide adequate spinal cord decompression in patients with traumatic CCS.(ABSTRACT TRUNCATED AT 250 WORDS)

  7. A traumatic central cord syndrome occurring after adequate decompression for cervical spondylosis: biomechanics of injury: case report.

    Dickerman, Rob D; Lefkowitz, Michael; Epstein, Joseph A

    2005-10-15

    Case report with review of the literature. To present the first case of a central cord syndrome occurring after adequate decompression, and review the mechanics of the cervical spinal cord injury and postoperative biomechanical and anatomic changes occurring after cervical decompressive laminectomy. Cervical spondylosis is a common pathoanatomic occurrence in the elderly population and is thought to be one of the primary causes for a central cord syndrome. Decompressive laminectomy with or without fusion has been a primary treatment for spondylotic disease and is thought to be protective against further injury. To our knowledge, there are no cases of a central cord syndrome occurring after adequate decompression reported in the literature. Case study with extensive review of the literature. The patient underwent C3-C7 cervical laminectomy without complications. After surgery, the patient's spasticity and gait difficulties improved. She was discharged to inpatient rehabilitation for further treatment of upper extremity weakness. The patient fell in the rehabilitation center, with a central cord syndrome despite adequate decompression of her spinal canal. The patient was treated conservatively for the central cord and had minimal improvement. Decompressive laminectomy provides an immediate decompressive effect on the spinal cord as seen by the dorsal migration of the cord, however, the biomechanics of the cervical spine after decompressive laminectomy remain uncertain. This case supports the ongoing research and need for more intensive research on postoperative cervical spine biomechanics, including decompressive laminectomies, decompressive laminectomy and fusion, and laminoplasty.

  8. Surgery for acute subaxial traumatic central cord syndrome without fracture or dislocation.

    Song, Joonsuk; Mizuno, Junichi; Nakagawa, Hiroshi; Inoue, Tatsushi

    2005-05-01

    Twenty-two patients with subaxial acute traumatic central cord syndrome (CCS) without fracture or dislocation who underwent surgery between 1995 and 2002 were reviewed, retrospectively. There were 13 males and nine females ranging in age from 24 to 84 years (mean 61.2). Falls were the most common injury (68%), followed by motor vehicle accidents (32%). All patients had dynamic cervical lateral radiographs and magnetic resonance imaging (MRI). Cord compression was present in all cases and cervical instability in 11. Associated pathology included disc herniation in seven patients, cervical spondylosis (CS) in 11 and ossification of the posterior longitudinal ligament (OPLL) in four. Anterior decompression and fusion was performed in 12 patients with 1- or 2-level lesions. Posterior decompression and fusion was performed for multilevel lesions in 11 patients, including one patient who required re-operation. The interval between injury and surgery ranged from 1 to 37 days (mean 8.0). Postoperatively, all patients improved clinically. We conclude that surgical management of subaxial acute traumatic CCS without fracture or dislocation improved neurological status and prevented delayed neurological deterioration in our patients.

  9. [Central cervical cord syndrome: a case report on rehabilitation, with special references to accidental falls in the elderly].

    Ina, G; Eto, F; Furuichi, T; Suzuki, H; Shibuya, K

    1995-03-01

    An 81-year-old man with Parkinson's disease was admitted to our hospital with impaired function of all extremities. Four weeks before his symptoms developed, he had tripped on the steps, fallen and bruised his jaw. Following this episode he experienced a few more falls inside his house. On examination his greatest weakness was in the hands and wrists. He was hyper-reflexic in all extremities and had bilateral Babinski's sign. He could not walk and needed physical assistance in most of his daily living activities. X-ray films of the cervical spine showed significant degenerative changes. The magnetic resonance images suggested central cervical cord damage at the level of the C6 vertebral body. After three months' rehabilitation treatment, he became able to walk with a cane and became independent in all the basic activities of daily living except for bathing. He never regained skillful function of his hands despite later levodopa treatment of Parkinson's disease. His clinical features were consistent with the central cervical cord syndrome, described by Schnneider and co-workers in 1954. This syndrome may occur as a result of hyperextension neck injury, occasionally associated with an accidental fall in the elderly with cervical spondylosis. Thirteen patients with cervical spinal cord injury above 65 of age were admitted to our department from 1983 to 1993. Six of them presented with the central cervical cord syndrome, and all patients had a history of accidental injuries related to falling.

  10. Cervical Klippel-Feil syndrome predisposing an elderly African man to central cord myelopathy following minor trauma

    Olufemi Adeleye, A; Olusola Akinyemi, R

    2010-01-01

    An otherwise-healthy, active 83-year-old Nigerian man developed reversible central cord myelopathy from a mild fall on a level surface. Cervical spine magnetic resonance imaging (MRI) revealed C5, 6, and 7 block vertebrae and marked disc extrusions only at the immediately adjoining upper and lower non-fused segments of the cervical spine. There was no spinal canal stenosis otherwise. We think that the unique presentation of this case of Klippel-Feil syndrome further supports the impression th...

  11. Acute traumatic central cord syndrome--experience using surgical decompression with open-door expansile cervical laminoplasty.

    Uribe, Juan; Green, Barth A; Vanni, Steven; Moza, Kapil; Guest, James D; Levi, Allan D

    2005-06-01

    Open-door expansile cervical laminoplasty (ODECL) is an effective surgical technique in the treatment of multilevel cervical spondylotic myelopathy. In the present study, we reviewed the safety and short-term neurological outcome after expansile cervical laminoplasty in the treatment of acute central cord syndrome. We retrospectively reviewed our database over a 3-year period (January 1997-January 2001) and identified 69 surgically treated cervical spinal cord injuries, including 29 cases of acute traumatic central cord syndrome (ATCCS). Fifteen of these patients underwent expansile cervical laminoplasty, whereas 14 did not because of radiographic evidence of sagittal instability. We collected data on the preoperative and the immediate postoperative and 3-month neurological examinations. Neurological function was assessed using the Asia Spinal Injury Association (ASIA) grading system. We also reviewed the occurrence of complications and short-term radiological stability after the index procedure. The median age was 56 years. All patients had hyperextension injuries with underlying cervical spondylosis and stenosis in the absence of overt fracture or instability. The average delay from injury to surgery was 3 days. The preoperative ASIA grade scale was grade C, 8 patients, and grade D, 7 patients. There were no cases of immediate postoperative deterioration or at 3 months follow-up. Neurological outcome: 71.4% (10/14) of patients improved 1 ASIA grade when examined 3 months post injury. Surgical intervention consisting of ODECL can be safely applied in the subset of patients with ATCCS without instability who have significant cervical spondylosis/stenosis. Open-door expansile cervical laminoplasty is a safe, low-morbidity, decompressive procedure, and in our patients did not produce neurological deterioration.

  12. Anterior spinal cord syndrome of unknown etiology

    Klakeel, Merrine; Thompson, Justin; Srinivasan, Rajashree; McDonald, Frank

    2015-01-01

    A spinal cord injury encompasses a physical insult to the spinal cord. In the case of anterior spinal cord syndrome, the insult is a vascular lesion at the anterior spinal artery. We present the cases of two 13-year-old boys with anterior spinal cord syndrome, along with a review of the anatomy and vasculature of the spinal cord and an explanation of how a lesion in the cord corresponds to anterior spinal cord syndrome.

  13. Tethered Spinal Cord Syndrome

    ... cord over time and may be exacerbated during sports or pregnancy, or may be due to narrowing of the ... cord over time and may be exacerbated during sports or pregnancy, or may be due to narrowing of the ...

  14. Twiddler's syndrome in spinal cord stimulation.

    Al-Mahfoudh, Rafid; Chan, Yuen; Chong, Hsu Pheen; Farah, Jibril Osman

    2016-01-01

    The aims are to present a case series of Twiddler's syndrome in spinal cord stimulators with analysis of the possible mechanism of this syndrome and discuss how this phenomenon can be prevented. Data were collected retrospectively between 2007 and 2013 for all patients presenting with failure of spinal cord stimulators. The diagnostic criterion for Twiddler's syndrome is radiological evidence of twisting of wires in the presence of failure of spinal cord stimulation. Our unit implants on average 110 spinal cord stimulators a year. Over the 5-year study period, all consecutive cases of spinal cord stimulation failure were studied. Three patients with Twiddler's syndrome were identified. Presentation ranged from 4 to 228 weeks after implantation. Imaging revealed repeated rotations and twisting of the wires of the spinal cord stimulators leading to hardware failure. To the best of our knowledge this is the first reported series of Twiddler's syndrome with implantable pulse generators (IPGs) for spinal cord stimulation. Hardware failure is not uncommon in spinal cord stimulation. Awareness and identification of Twiddler's syndrome may help prevent its occurrence and further revisions. This may be achieved by implanting the IPG in the lumbar region subcutaneously above the belt line. Psychological intervention may have a preventative role for those who are deemed at high risk of Twiddler's syndrome from initial psychological screening.

  15. Central Pain Syndrome

    ... such as neurontin (gabapentin) can be useful. Lowering stress levels appears to reduce pain. View Full Treatment Information Definition Central pain syndrome is a neurological condition caused ...

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

    Delhaas, T.; Kamphuis, D.J.; Witkamp, T.D.

    1998-01-01

    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.)

  17. Magnetic resonance imaging of spinal cord syndromes

    Einsiedel, H. von; Stepan, R.

    1985-01-01

    Thirty-four patients with intramedullary space-occupying lesions or cord compression syndromes were examined with a resistive and two different superconductive magnetic resonance (MR) imaging units. Studies were done primarily by the spin-echo (SE) technique and in the majority of patients different pulse sequences were used. Images with short echo-time (TE) and short recovery-time (TR) were best for demonstration of spinal cord anatomy, for depicting cystic portions in intramedullary tumours and for showing syringomyelia. Solid intramedullary tumours showed normal cord signal intensity. Images with prolonged TE and TR predominantly enhanced CSF signal intensity and, to a more considerable extent, solid intramedullary tumours. Thus, the diameter of the subarachnoid space and the presence of a solid intramedullary tumour, not concomittant with a significant enlargement of the spinal cord, could only be recognized on these prolonged SE images. Major advantages of MR in comparison to CT are that the spinal cord can be imaged in the sagittal plane and that beam hardening artifacts do not occur; in comparison to myelography the cord can be imaged directly by MR. Partial volume is a major limitation of MR, not only in the preferably applied sagittal plane. The choice of slice thickness adequate to the diameter of the lesion and straight positioning of the patient for sagittal single slice midline images are fundamental for reliable MR investigations. Another limitation to MR is that cortical bone gives no signal. The actual diameter of the spinal canal therefore cannot be correctly appreciated and consequently it was difficult or impossible to assess spinal stenosis. (orig.)

  18. Central Neuropathic Pain in Spinal Cord Injury

    Lee, Sujin; Zhao, Xing; Hatch, Maya; Chun, Sophia; Chang, Eric

    2015-01-01

    Spinal cord injury (SCI) is a devastating medical condition affecting 1.2 million people in the United States. Central neuropathic pain is one of the most common medical complications of SCI. Current treatment options include opioids, antiepileptic agents such as gabapentin, antispastic agents such as baclofen or tizanidine, and tricyclic acid. Other options include complementary, nonpharmacological treatment such as exercise or acupuncture, interventional treatments, and psychological approaches. Although these treatment options exist, central neuropathic pain in patients with SCI is still extremely difficult to treat because of its complexity. To develop and provide more effective treatment options to these patients, proper assessment of and classification tools for central neuropathic pain, as well as a better understanding of the pathophysiology, are needed. A combination of approaches, from standard general pain assessments to medically specific questions unique to SCI pathophysiology, is essential for this population. A multidisciplinary approach to patient care, in addition with a better understanding of pathophysiology and diagnosis, will lead to improved management and treatment of patients with SCI displaying central neuropathic pain. Here we summarize the most recent classification tools, pathophysiology, and current treatment options for patients with SCI with central neuropathic pain. PMID:25750485

  19. Vocal cord paralysis associated with Ramsay Hunt syndrome

    Rasmussen, Eva Rye; Mey, Kristianna

    2014-01-01

    Ramsay Hunt syndrome is defined by herpes zoster oticus and peripheral facial nerve palsy which is often associated with otalgia. The syndrome is, in rare cases, associated with other cranial nerve paralyses including the vagal nerve causing unilateral vocal cord paralysis. Vocal cord paralysis...

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

    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.

  1. Spinal cord stimulation therapy for localized central pain

    Hirato, Masafumi; Takahashi, Akio; Watanabe, Katsushige; Kazama, Ken; Yoshimoto, Yuhei

    2008-01-01

    We studied the pathophysiology of localized central pain and the surgical result of spinal cord stimulation. There were 10 cases; 7 males and 3 females from 24 to 77 years old. Pain was caused by peripheral nerve injury in one case, spinal cord injury in two cases and cerebrovascular disease (CVD) (thalamic pain) in 7 cases. All cases were treated by epidural spinal cord stimulation and followed from 0.8 to 8.8 years. Sufficient pain relief was achieved in one case of peripheral nerve and spinal cord injury and in 4 cases of CVD. Moderate pain control was achieved in 2 cases of CVD. In one each case of spinal cord injury and of CVD, pain control was ineffective. In cases with thalamic pain, we studied the correlation between the surgical result of spinal cord stimulation and the clinical features, MRI, fluoro-deoxyglucose (FDG)-positron emission tomography (PET), and somatosensory evoked potentials (SEP) findings before operation. MRI revealed a small to moderate sized lesion on the thalamus or putamen in each case. PET also showed decreased accumulation of FDG on the affected thalamus. In all cases without one fair responder to spinal cord stimulation, we could recognize definite SEP originating in the sensory cortex ipsilateral side to the CVD lesion during contralateral median or posterior tibial nerve stimulation. In the good responders, we could recognize SEP originating in the sensory cortex of the lesion side with less delayed latency or decreased amplitude than in the moderate responders. In this group, test stimulation with low voltage on the spinal cord evoked a sensory effect (paresthesia) over the painful part of the body. Spinal cord stimulation proved to be an effective treatment for localized central pain. In cases with localized central pain after CVD, we could expect to ameliorate the intractable pain in those cases in which SEP or spinal cord test stimulation revealed that the thalamo-cortical system was preserved. (author)

  2. Psychosocial Factors and Central Sensitivity Syndromes

    Adams, Leah M.; Turk, Dennis C.

    2015-01-01

    Central sensitivity syndromes (CSSs) represent a heterogeneous group of disorders (e.g., fibromyalgia [FM], irritable bowel syndrome [IBS], chronic headache, temporomandibular disorders [TMDs], pelvic pain syndromes) that share common symptoms, with persistent pain being the most prominent feature.

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

    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 spinal cord injury directly causes increased risk for bacterial infection in mice as well as in patients. Besides obvious motor and sensory paralysis, spinal cord injury also induces a functional SCI-IDS ('immune paralysis'), sufficient to propagate clinically relevant infection in an injury level dependent manner. © The Author (2016). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  4. Bilateral complex regional pain syndrome following spinal cord injury and bilateral calcaneus fracture

    Ahmet Boyacı

    2013-09-01

    Full Text Available Complex regional pain syndrome (CRPS is a disease affectingone or more extremities, characterized by spontaneouspain, allodynia, hyperpathia and hyperalgesia.CRPS is separated into Type 1 and Type 2. CRPS whichdevelops after a nociceptive event is labeled as Type 1and when it develops following peripheral nerve damage,Type 2. Although the pathogenesis is not fully understood,peripheral and central sensitivity are held responsible.Bilateral lower extremity involvement is extremely rare.However, it should be borne in mind that it can develop intraumatic injuries which occur in more than one area anddiagnosis and commencement of a rehabilitation programshould be made in the early period. The case is presentedhere of bilateral Type 1 CRPS developing after incompletespinal cord injury and bilateral calcaneus fracture. JClin Exp Invest 2013; 4 (3: 360-363Key words: complex regional pain syndrome, calcaneusfracture, spinal cord injury

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

    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

  6. Diagnosis and surgical treatment of terminal syringomyelia within spinal cord combined with tethered cord syndrome

    Jing-cheng XIE

    2016-04-01

    Full Text Available Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS.  Methods and Results Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 years old. The course of disease ranged from 3 months to 20 years (average 42.17 months. Among those patients, one patient presented hydromyelia, 8 patients suffered from meningeal cyst within the sacral canal, and one patient were concurrent with sacral dermal sinus. The weakness of lower extremities, especially distal limbs, was the main clinical manifestation. Five patients were accompanied with bowel and bladder dysfunction and 5 patients with sensory disturbance below the level of syringomyelia, especially hypesthesia. Preoperative MRI showed conus medullaris disappeared at the end of spinal cord, and there was fluid signal in the lower spinal cord with hypo-intensity signal in T1WI and hyper-intensity signal in T2WI without enhancement. All patients underwent surgical procedures. Under microscope, filum terminale was cut off, drainage was performed, meningeal cyst within the sacral canal was removed, and tethered cord was released. The success rate of operations was 100%. The duration of surgery ranged from 1.52 to 3.07 h (average 2.15 h, with average intraoperative blood loss 220 ml (ranged from 100 to 410 ml. The tethering filum had been totally resected and histological examination showed typical filum tissue in all cases. No operative complication was found. Visual Analogue Scale (VAS score was decreased, and the lower limbs weakness as well as bowel and bladder dysfunction was gradually relieved after operation. The period of follow-up was ranged from 6

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

    Deng Xianbo; Kong Xiangquan; Feng Gansheng; Han Ping; Liu Dingxi; Ma Hui

    2005-01-01

    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 T 2 W 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 T 2 W 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)

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

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

    2007-01-01

    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......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...... at the level of injury. Keywords: Spinal cord injury; Neuropathic pain; Capsaicin; Neuronal hyperexcitability; Hyperalgesia; Blood flow...

  9. The urgency of surgical decompression in acute central cord injuries with spondylosis and without instability.

    Lenehan, Brian; Fisher, Charles G; Vaccaro, Alex; Fehlings, Michael; Aarabi, Bizhan; Dvorak, Marcel F

    2010-10-01

    Systematic review, ambispective analysis of observational data. To make recommendations as to whether or not urgent surgical decompression is ever indicated as the optimal treatment for enhancing neurologic recovery in a patient with acute central cord injury without fracture or instability. There are currently no standards regarding the role and timing of decompression in acute traumatic central cord syndrome. In the setting of TCCS without spinal column instability, much controversy exists. We have performed a thorough literature search based on the following question: "Is there a role for urgent (within 24 hours from injury to surgery) surgical decompression in acute central cord syndrome without fracture or instability specifically to enhance neurologic recovery?" Data including patient demographics, mechanism of injury, comorbidities, neurologic status, and surgical treatment was analyzed from a multicenter STSG observational database. Outcome measured included ASIA Motor Score, ASIA Grade, Functional Independence Measure (FIM) Score, SF-36, Sphincter Disturbance, and Ambulatory status. Measures were recorded on admission, discharge, 6 months and 1 year. At 12-month follow-up, early surgery resulted in a 6.31 point greater improvement in total motor score than did the late surgery group, with a P = 0.0358. At 6-month follow-up, early surgery result in higher chance of improvement in ASIA Grade than late surgery, with an odds ratio = 3.39, while at 12-month follow-up early surgery resulted in a higher chance of improvement in ASIA Grade, with an odds ratio of 2.81. Patients who were operated on within 24 hours had 7.79 U more improvement in FIM Total Score than late surgery at 6 month follow-up, with P = 0.0474. The consensus of experts following review of relevant and examination of observational dataset concluded that it is reasonable and safe to consider early surgical decompression in patients with profound neurologic deficit (ASIA = C) and persistent

  10. Genetics Home Reference: congenital central hypoventilation syndrome

    ... Kravis EM, Zhou L, Rand CM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B mutations and phenotype. Am J Respir Crit ... BA, Leurgans SE, Berry-Kravis EM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr ...

  11. Central Nervous System (CNS Disease Triggering Takotsubo Syndrome

    Josef Finsterer

    2016-01-01

    Full Text Available Takotsubo syndrome (TTS is usually triggered by psychological or physical stress. One of the many physical sources of stress are central nervous system (CNS disorders. CNS disorders most frequently triggering TTS include subarachnoid bleeding, epilepsy, ischemic stroke, migraine, and intracerebral bleeding. More rare CNS-triggers of TTS include posterior reversible encephalopathy syndrome (PRES, amyotrophic lateral sclerosis, encephalitis, or traumatic brain or spinal cord injury. TTS triggered by any of the CNS disorders needs to be recognized since adequate treatment of TTS may improve the general outcome from the CNS disorder as well. Neurologists need to be aware of TTS as a complication of specific CNS disorders but TTS may be triggered also by CNS disorders so far not recognised as causes of TTS.

  12. Choroid Plexus in the Central Canal of the Spinal Cord Causing Recurrent Syringomyelia.

    Shtaya, Anan; Sadek, Ahmed-Ramadan; Nicoll, James A R; Nader-Sepahi, Ali

    2018-03-01

    Syringomyelia is a fluid-filled cavitation within the substance of the spinal cord. This condition usually follows a primary pathology that disrupts the normal cerebrospinal fluid circulation or disturbs the microcirculation and cytoarchitecture of the spinal cord parenchyma. However, an etiology of recurrent syringomyelia resulting from an ectopic choroid plexus (CP) has not been discussed. Ectopic CP rests may be found within the central nervous system. Although there has been a single report, describing ectopic intramedullary spinal cord CP, to our knowledge, extra-cranial nonmalignant CP in the central canal of the spinal cord has not been reported. We report CP in the central canal of the spinal cord in a 23-year-old male patient who had developmental delay and diabetes mellitus type I who presented with dissociated sensory changes and muscle wastage predominantly on the right upper and lower limbs. Magnetic resonance imaging demonstrated a multiloculated spinal cord syringomyelia stretching from cervical (C3) to the conus medullaris causing recurrent neurologic deficits. A biopsy of the central canal spinal cord lesion revealed CP. Decompression and syringosubarachnoid shunt insertion stabilized the patient's neurology. Our illustrative case reveals the presence of CP in the central canal of the spinal cord that may suggest a role in the etiology of recurrent syringomyelia. Although management poses a challenge to neurosurgeons, prompt decompression and shunting of the syringomyelia remains a favorable approach with acceptable outcomes. Further investigation into the pathophysiology of central canal CP ectopic causing recurrent syringomyelia and its correlation with spinal cord development may help future treatments. Copyright © 2017 Elsevier Inc. All rights reserved.

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

    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.

  14. Diet and Mobility in the Corded Ware of Central Europe.

    Karl-Göran Sjögren

    Full Text Available Isotopic investigations of two cemetery populations from the Corded Ware Culture in southern Germany reveal new information on the dating of these graves, human diet during this period, and individual mobility. Corded Ware Culture was present across much of temperate Europe ca. 2800-2200 cal. BC and is represented by distinctive artifacts and burial practices. Corded Ware was strongly influenced by the Yamnaya Culture that arose in the steppes of eastern Europe and western Eurasia after 3000 BC, as indicated by recent aDNA research. However, the development of CW on different chronological and spatial scales has to be evaluated. Examination of the CW burials from southern Germany supports an argument for substantial human mobility in this period. Several burials from gravefields and larger samples from two large cemeteries at Lauda-Königshofen "Wöllerspfad" and at Bergheinfeld "Hühnerberg" contributed the human remains for our study of bone and tooth enamel from the Corded Ware Culture. Our results suggest that Corded Ware groups in this region at least were subsisting on a mix of plant and animal foods and were highly mobile, especially the women. We interpret this as indicating a pattern of female exogamy, involving different groups with differing economic strategies.

  15. Diet and Mobility in the Corded Ware of Central Europe.

    Sjögren, Karl-Göran; Price, T Douglas; Kristiansen, Kristian

    2016-01-01

    Isotopic investigations of two cemetery populations from the Corded Ware Culture in southern Germany reveal new information on the dating of these graves, human diet during this period, and individual mobility. Corded Ware Culture was present across much of temperate Europe ca. 2800-2200 cal. BC and is represented by distinctive artifacts and burial practices. Corded Ware was strongly influenced by the Yamnaya Culture that arose in the steppes of eastern Europe and western Eurasia after 3000 BC, as indicated by recent aDNA research. However, the development of CW on different chronological and spatial scales has to be evaluated. Examination of the CW burials from southern Germany supports an argument for substantial human mobility in this period. Several burials from gravefields and larger samples from two large cemeteries at Lauda-Königshofen "Wöllerspfad" and at Bergheinfeld "Hühnerberg" contributed the human remains for our study of bone and tooth enamel from the Corded Ware Culture. Our results suggest that Corded Ware groups in this region at least were subsisting on a mix of plant and animal foods and were highly mobile, especially the women. We interpret this as indicating a pattern of female exogamy, involving different groups with differing economic strategies.

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

    Lin Shixu; Lin Daiying; Wu Xianheng; Zeng Xianting

    2003-01-01

    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

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

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

    2007-01-01

    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......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...... for this hypothesis is difficult to obtain. Capsaicin, activating TRPV1 receptors on small sensory afferents, induces enhanced cellular activity in dorsal horn neurons and produces a central mediated area of secondary hyperalgesia. We hypothesized that sensory stimuli and capsaicin applied at and just above the level...

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

    Epstein, Nancy E.; Hollingsworth, Renee

    2015-01-01

    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 accomp...

  19. Congenital Central Hypoventilation Syndrome And Intestinal

    Congenital central hypoventilation syndrome (CCHS) or 'Ondine's curse' is characterised by an abnormal ventilatory response to progressive hypercapnia and sustained hypoxaemia. During the neonatal period, most patients with this condition present with hypoventilation or apnoea while asleep. Patients with CCHS may ...

  20. Congenital central hypoventilation syndrome mimicking mitochondrial disease.

    Rojnueangnit, Kitiwan; Descartes, Maria

    2018-03-01

    Later-onset congenital central hypoventilation syndrome (LO-CCHS) does not present only breathing problems but can be present as episodic multiple organs involvement. Our unique case demonstrated LO-CCHS should be considered in the differential diagnosis of mitochondrial diseases and having nontypical polysomnography result.

  1. MRI of anterior spinal artery syndrome of the cervical spinal cord

    Takahashi, S. (Dept. of Radiology, Tohoku Univ. School of Medicine, Sendai (Japan)); Yamada, T. (Dept. of Radiology, Tohoku Univ. School of Medicine, Sendai (Japan)); Ishii, K. (Dept. of Radiology, Tohoku Univ. School of Medicine, Sendai (Japan)); Saito, H. (Dept. of Neurology, Tohoku Univ. School of Medicine, Sendai (Japan)); Tanji, H. (Dept. of Neurology, Tohoku Univ. School of Medicine, Sendai (Japan)); Kobayashi, T. (Inst. of Rehabilitation Medicine, Tohoku Univ. School of Medicine, Miyagi (Japan)); Soma, Y. (Div. of Neurology, Takeda Hospital, Aizuwakamatsu (Japan)); Sakamoto, K. (Dept. of Radiology, Tohoku Univ. School of Medicine, Sendai (Japan))

    1992-12-01

    Cervical spinal cord lesions in the anterior spinal artery syndrome were delineated on magnetic resonance images (MRI) in four patients. The lesion was always seen anteriorly in the cervical cord. On T2-weighted images, the lesions appeared hyperintense relative to the normal spinal cord, while on T1-weighted images, two chronic lesions appeared hypointense, with local atrophy of the cord. In one case, repeated T1-weighted images showed no signal abnormality 4 days after the ictus, but the lesion became hypointense 18 days later, when contrast enhancement was also recognized after injection of Gd-DTPA; this sequence of intensity changes was similar to that of cerebral infarction. The extent of the lesion seen MRI correlated closely with neurological findings in all cases. Although the findings may not be specific, MRI is now the modality of choice for confirming the diagnosis in patients suspected of having an anterior spinal artery syndrome. (orig.)

  2. Hyperthyroidism hidden by congenital central hypoventilation syndrome.

    Fox, Danya A; Weese-Mayer, Debra E; Wensley, David F; Stewart, Laura L

    2015-05-01

    Congenital central hypoventilation syndrome (CCHS) is a rare neurocristopathy with severe central hypoventilation. CCHS results from a mutation in the paired-like homeobox 2B gene (PHOX2B). In addition to hypoventilation, patients with CCHS display a wide array of autonomic nervous system abnormalities, including decreased heart rate variability and abrupt sinus pauses, esophageal dysmotility, abnormal pupillary light response, and temperature dysregulation, to name a few. To date, there has been no documentation of a child with both CCHS and hyperthyroidism. We report the case of a young child with CCHS who presented with tachycardia, which was later found to be due to Grave's disease, after many months of investigation.

  3. "White Cord Syndrome" of Acute Hemiparesis After Posterior Cervical Decompression and Fusion for Chronic Cervical Stenosis.

    Antwi, Prince; Grant, Ryan; Kuzmik, Gregory; Abbed, Khalid

    2018-05-01

    "White cord syndrome" is a very rare condition thought to be due to acute reperfusion of chronically ischemic areas of the spinal cord. Its hallmark is the presence of intramedullary hyperintense signal on T2-weighted magnetic resonance imaging sequences in a patient with unexplained neurologic deficits following spinal cord decompression surgery. The syndrome is rare and has been reported previously in 2 patients following anterior cervical decompression and fusion. We report an additional case of this complication. A 68-year-old man developed acute left-sided hemiparesis after posterior cervical decompression and fusion for cervical spondylotic myelopathy. The patient improved with high-dose steroid therapy. The rare white cord syndrome following either anterior cervical decompression and fusion or posterior cervical decompression and fusion may be due to ischemic-reperfusion injury sustained by chronically compressed parts of the spinal cord. In previous reports, patients have improved following steroid therapy and acute rehabilitation. Copyright © 2018 Elsevier Inc. All rights reserved.

  4. A Review of Select Centralized Pain Syndromes

    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.

  5. Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults.

    Aldave, Guillermo; Hansen, Daniel; Hwang, Steven W; Moreno, Amee; Briceño, Valentina; Jea, Andrew

    2017-06-01

    OBJECTIVE Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique. METHODS The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome. RESULTS Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8-30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26-37 months). Computed tomography-based radiographic outcomes showed solid

  6. Skin vasomotor hemiparesis followed by overactivity: characteristic thermography findings in a patient with Horner syndrome due to spinal cord infarction.

    Kobayashi, Makoto

    2016-04-01

    We present a 21-year-old female with Horner syndrome due to spinal cord infarction. In this patient, infrared thermography revealed a hemibody skin temperature increase followed by excessive focal decreases, indicating skin vasomotor hemiparesis and overactivity.

  7. The relationship between central motor conduction time and spinal cord compression in patients with cervical spondylotic myelopathy.

    Rikita, T; Tanaka, N; Nakanishi, K; Kamei, N; Sumiyoshi, N; Kotaka, S; Adachi, N; Ochi, M

    2017-04-01

    Retrospective study. Few studies have reported a relationship between central motor conduction time (CMCT), which evaluates corticospinal function, and degree of spinal cord compression in patients with myelopathy. Thus, there is no consensus on predicting the degree of prolonged CMCT on the basis of the degree of spinal cord compression. If a correlation exists between CMCT and spinal cord compression, then spinal cord compression may be a useful noninvasive clinical indicator of corticospinal function. Therefore, this study evaluated the relationship between CMCT and cervical spinal cord compression measured by magnetic resonance imaging (MRI) in patients with cervical spondylotic myelopathy (CSM). Hiroshima University Hospital in Japan. We studied 33 patients undergoing laminoplasty. Patients exhibited significant cervical spinal cord compression on both MRI and intraoperative electrophysiological examination. We assessed transcranial magnetic stimulation measurement of CMCT; spinal cord compression parameters such as area, lateral diameter, anteroposterior diameter and flattening of the spinal cord at the lesion site and C2/3 levels on MRI; and pre- versus postoperative Japanese Orthopaedic Association (JOA) scores. Correlations between CMCT and flattening as well as anteroposterior diameter of the spinal cord at the lesion level were observed. Strong correlations between CMCT and the ratio of the flattening and anteroposterior diameter parameters at the lesion level to that at the C2/3 level were also observed. Measurement of spinal cord compression may be useful for the evaluation of corticospinal function as a proxy for CMCT in patients with CSM.

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

    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.

  9. SPINAL CORD STIMULATION IN TREATMENT OF THE NEUROPATHIC PAIN SYNDROMES: INITIAL EXPERIENCE

    D. A. Rzaev

    2010-01-01

    Full Text Available In the article initial experience of spinal cord stimulation for chronic pain syndromes is described. The trial was done for 62 patients, in 52 cases trial was successful and subcutaneous pulse generator were implanated. Maximal follow-up is 26 months. The level of pain evaluates at VAS. Permanent pain-relieve results were achieved in 46 patients (74,2%. These results correspond to literature data.

  10. Acute intraparenchymal spinal cord injury in a cat due to high-rise syndrome.

    Cruz-Arámbulo, Robert; Nykamp, Stephanie

    2012-03-01

    A 9-year-old spayed female Bengal Red cat was evaluated for high-rise syndrome. The cat had paraplegia of the hind limbs, intact reflexes and pain perception, and hyperesthesia in the caudal thoracic area. Mentation, cranial nerve function, forelimb proprioceptive responses, and spinal reflexes were normal. There were no abnormalities on radiographs or computed tomography scan, but magnetic resonance imaging revealed a hyperintense intraparenchymal spinal cord lesion on T2-weighted and T2 fat saturation images.

  11. Acute intraparenchymal spinal cord injury in a cat due to high-rise syndrome

    Cruz–Arámbulo, Robert; Nykamp, Stephanie

    2012-01-01

    A 9-year-old spayed female Bengal Red cat was evaluated for high-rise syndrome. The cat had paraplegia of the hind limbs, intact reflexes and pain perception, and hyperesthesia in the caudal thoracic area. Mentation, cranial nerve function, forelimb proprioceptive responses, and spinal reflexes were normal. There were no abnormalities on radiographs or computed tomography scan, but magnetic resonance imaging revealed a hyperintense intraparenchymal spinal cord lesion on T2-weighted and T2 fat...

  12. Congenital central hypoventilation syndrome: four families.

    Trivedi, Amit; Waters, Karen; Suresh, Sadasivam; Nair, Rashmi

    2011-12-01

    Congenital central hypoventilation syndrome (CCHS) is a rare condition that usually presents soon after birth and is potentially life-shortening if not treated. The defining abnormality is hypoventilation during sleep which requires life-long treatment with artificial ventilation. This syndrome may also be associated with generalised dysfunction of the autonomic nervous system and a sub-group with associated Hirschsprung's disease. The genetic basis of CCHS has been identified as mutations in the PHOX2B gene. We present four families, three with autosomal dominant inheritance and familial clustering, and one with a de novo mutation resulting in CCHS. We demonstrate that nasal mask ventilation from birth can provide adequate treatment and improved quality of life for these children. Phenotypic variability in expression of disease is seen in families with the same mutations in PHOX2B gene. The psychosocial costs of the disease and the unrecognised 'morbidity barter' that is part of current management needs to be factored into in all stages of management from childhood to adolescence to adulthood.

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

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

    2016-01-01

    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. 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. 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.

  14. Electroencephalographic evoked pain response is suppressed by spinal cord stimulation in complex regional pain syndrome: a case report.

    Hylands-White, Nicholas; Duarte, Rui V; Beeson, Paul; Mayhew, Stephen D; Raphael, Jon H

    2016-12-01

    Pain is a subjective response that limits assessment. The purpose of this case report was to explore how the objectivity of the electroencephalographic response to thermal stimuli would be affected by concurrent spinal cord stimulation. A patient had been implanted with a spinal cord stimulator for the management of complex regional pain syndrome of both hands for 8 years. Following ethical approval and written informed consent we induced thermal stimuli using the Medoc PATHWAY Pain & Sensory Evaluation System on the right hand of the patient with the spinal cord stimulator switched off and with the spinal cord stimulator switched on. The patient reported a clinically significant reduction in thermal induced pain using the numerical rating scale (71.4 % reduction) with spinal cord stimulator switched on. Analysis of electroencephalogram recordings indicated the occurrence of contact heat evoked potentials (N2-P2) with spinal cord stimulator off, but not with spinal cord stimulator on. This case report suggests that thermal pain can be reduced in complex regional pain syndrome patients with the use of spinal cord stimulation and offers objective validation of the reported outcomes with this treatment.

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

    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...... after an intramuscular injection of capsaicin in the foot sole in order to induce central sensitization. RESULTS: Both groups presented RRF expansion and lowered NWR thresholds immediately after capsaicin injection, reflected by the enlargement of RRF sensitivity areas and RRF probability areas....... Moreover, the topography of the RRF sensitivity and probability areas were significantly different in SCI volunteers compared to NI volunteers in terms of size and shape. CONCLUSIONS: SCI volunteers can develop central sensitization, despite adaptive/maladaptive changes in synaptic plasticity and lack...

  16. Design of COSMIC: a randomized, multi-centre controlled trial comparing conservative or early surgical management of incomplete cervical cord syndrome without spinal instability

    Bartels, R.H.M.; Hosman, A.J.F.; Meent, H.; Hofmeijer, Jeannette; Vos, P.E.; Slooff, W.B.; Öner, F.C.; Coppes, M.H.; Peul, W.C.; Verbeek, A.L.M.

    2013-01-01

    Background Incomplete cervical cord syndrome without spinal instability is a very devastating event for the patient and the family. It is estimated that up to 25 % of all traumatic spinal cord lesions belong to this category. The treatment for this type of spinal cord lesion is still subject of

  17. Design of COSMIC: a randomized, multi-centre controlled trial comparing conservative or early surgical management of incomplete cervical cord syndrome without spinal instability

    Bartels, R.H.M.A.; Hosman, A.J.F.; Meent, H. van de; Hofmeijer, J.; Vos, P.E.; Slooff, W.B.; Oner, F.C.; Coppes, M.H.; Peul, W.C.; Verbeek, A.L.M.

    2013-01-01

    ABSTRACT: BACKGROUND: Incomplete cervical cord syndrome without spinal instability is a very devastating event for the patient and the family. It is estimated that up to 25% of all traumatic spinal cord lesions belong to this category. The treatment for this type of spinal cord lesion is still

  18. Asymmetric operation of the locomotor central pattern generator in the neonatal mouse spinal cord

    Endo, Toshiaki; Kiehn, Ole

    2008-01-01

    The rhythmic voltage oscillations in motor neurons (MNs) during locomotor movements reflect the operation of the pre-MN central pattern generator (CPG) network. Recordings from MNs can thus be used as a method to deduct the organization of CPGs. Here, we use continuous conductance measurements...... of locomotor CPG. The extracted excitatory and inhibitory synaptic conductances varied between 2 and 56% of the mean total conductance. Analysis of the phase tuning of the extracted synaptic conductances in flexor and extensor MNs in the rostral lumbar cord showed that the flexor-phase-related synaptic...

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

    Epstein, Nancy E; Hollingsworth, Renee

    2015-01-01

    The classical clinical presentation, neuroradiographic features, and conservative vs. surgical management of traumatic cervical central spinal cord (CSS) injury remain controversial. 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). 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. 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.

  20. Central serous choroidopathy in the Hallermann-Streiff Syndrome.

    Blair, N P; Brockhurst, R J; Lee, W

    1981-08-01

    Central serous choroidopathy was observed in a young patient with the Hallermann-Streiff syndrome. Typical features of this syndrome include microphthalmos, proportionate dwarfism, dyscephaly with birdlike facies, dental abnormalities, and hypotrichosis. Exceptional aspects of this case include age of onset (11 years), high hyperopic refractive error (+ 13.00 sphere), and multiple recurrences caused by six separate documented leaks from the choroid. Fundus changes previously reported in the Hallermann-Streiff syndrome, interpreted as chorioretinal pigmentary changes, may have been secondary to previous undiagnosed central serous choroidopathy. Periodic ophthalmoscopy should be performed and may detect unrecognized episodes of central serous choroidopathy for which photocoagulation would be beneficial.

  1. Spinal cord toxoplasmosis in human immunodeficiency virus infection/acquired immunodeficiency syndrome.

    García-García, Concepción; Castillo-Álvarez, Federico; Azcona-Gutiérrez, José M; Herraiz, María J; Ibarra, Valvanera; Oteo, José A

    2015-05-01

    Neurological complications in patients with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS) are still common, even in the era of highly active antiretroviral therapy. Opportunistic infections, immune reconstitution, the virus itself, antiretroviral drugs and neurocognitive disorders have to be considered when establishing the differential diagnosis. Toxoplasmic encephalitis remains the major cause of space-occupying lesions in the brain of patients with HIV/AIDS; however, spinal cord involvement has been reported infrequently. Here, we review spinal cord toxoplasmosis in HIV infection and illustrate the condition with a recent case from our hospital. We suggest that most patients with HIV/AIDS and myelitis with enhanced spine lesions, multiple brain lesions and positive serology for Toxoplasma gondii should receive immediate empirical treatment for toxoplasmosis, and a biopsy should be performed in those cases without clinical improvement or with deterioration.

  2. Mini-open spinal column shortening for the treatment of adult tethered cord syndrome.

    Safaee, Michael M; Winkler, Ethan A; Chou, Dean

    2017-10-01

    Tethered cord syndrome (TCS) is a challenging entity characterized by adhesions at the caudal spinal cord that prevent upward movement during growth and result in stretching of the cord with a concomitant constellation of neurologic symptoms. Although growth in height stops in adulthood, some patients still develop progressive symptoms; many underwent detethering as a child or adolescent, resulting in significant scar tissue and re-tethering. Recent strategies have focused on spinal column shortening to reduce tension on the spinal cord without exposing the previous de-tethering site. Mini-open and minimally invasive approaches avoid the large dissection and exposure associated with traditional approaches and are associated with reduced blood loss, shorter hospital stay, and similar outcomes when compared to conventional open approaches. We describe a technique for mini-open spinal column shortening. Using intraoperative navigation pedicle screws were placed at T10, T11, L1, and L2. A mini-open 3-column "egg shell" decancellation osteotomy of T12 was performed through a transpedicular approach with preservation of the superior and inferior endplates. This procedure was performed on a 28year old male with recurrent TCS and neurogenic bladder. Postoperative imaging showed a reduction in spinal column length of 1.5cm and evidence of decreased tension on the spinal cord. At last follow-up he was recovering well with improved urinary function. Spinal column shortening for adult TCS can be safely achieved through a mini-open approach. Future studies should compare the efficacy of this technique to both traditional de-tethering and open spinal column shortening. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. Rapid recovery and altered neurochemical dependence of locomotor central pattern generation following lumbar neonatal spinal cord injury.

    Züchner, Mark; Kondratskaya, Elena; Sylte, Camilla B; Glover, Joel C; Boulland, Jean-Luc

    2018-01-15

    Spinal compression injury targeted to the neonatal upper lumbar spinal cord, the region of highest hindlimb locomotor rhythmogenicity, leads to an initial paralysis of the hindlimbs. Behavioural recovery is evident within a few days and approaches normal function within about 3 weeks. Fictive locomotion in the isolated injured spinal cord cannot be elicited by a neurochemical cocktail containing NMDA, dopamine and serotonin 1 day post-injury, but can 3 days post-injury as readily as in the uninjured spinal cord. Low frequency coordinated rhythmic activity can be elicited in the isolated uninjured spinal cord by NMDA + dopamine (without serotonin), but not in the isolated injured spinal cord. In both the injured and uninjured spinal cord, eliciting bona fide fictive locomotion requires the additional presence of serotonin. Following incomplete compression injury in the thoracic spinal cord of neonatal mice 1 day after birth (P1), we previously reported that virtually normal hindlimb locomotor function is recovered within about 3 weeks despite substantial permanent thoracic tissue loss. Here, we asked whether similar recovery occurs following lumbar injury that impacts more directly on the locomotor central pattern generator (CPG). As in thoracic injuries, lumbar injuries caused about 90% neuronal loss at the injury site and increased serotonergic innervation below the injury. Motor recovery was slower after lumbar than thoracic injury, but virtually normal function was attained by P25 in both cases. Locomotor CPG status was tested by eliciting fictive locomotion in isolated spinal cords using a widely used neurochemical cocktail (NMDA, dopamine, serotonin). No fictive locomotion could be elicited 1 day post-injury, but could within 3 days post-injury as readily as in age-matched uninjured control spinal cords. Burst patterning and coordination were largely similar in injured and control spinal cords but there were differences. Notably, in both groups there

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

    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

  5. Dynamic oscillatory signatures of central neuropathic pain in spinal cord injury.

    Vuckovic, Aleksandra; Hasan, Muhammad A; Fraser, Matthew; Conway, Bernard A; Nasseroleslami, Bahman; Allan, David B

    2014-06-01

    Central neuropathic pain (CNP) is believed to be accompanied by increased activation of the sensorimotor cortex. Our knowledge of this interaction is based mainly on functional magnetic resonance imaging studies, but there is little direct evidence on how these changes manifest in terms of dynamic neuronal activity. This study reports on the presence of transient electroencephalography (EEG)-based measures of brain activity during motor imagery in spinal cord-injured patients with CNP. We analyzed dynamic EEG responses during imaginary movements of arms and legs in 3 groups of 10 volunteers each, comprising able-bodied people, paraplegic patients with CNP (lower abdomen and legs), and paraplegic patients without CNP. Paraplegic patients with CNP had increased event-related desynchronization in the theta, alpha, and beta bands (16-24 Hz) during imagination of movement of both nonpainful (arms) and painful limbs (legs). Compared to patients with CNP, paraplegics with no pain showed a much reduced power in relaxed state and reduced event-related desynchronization during imagination of movement. Understanding these complex dynamic, frequency-specific activations in CNP in the absence of nociceptive stimuli could inform the design of interventional therapies for patients with CNP and possibly further understanding of the mechanisms involved. This study compares the EEG activity of spinal cord-injured patients with CNP to that of spinal cord-injured patients with no pain and also to that of able-bodied people. The study shows that the presence of CNP itself leads to frequency-specific EEG signatures that could be used to monitor CNP and inform neuromodulatory treatments of this type of pain. Copyright © 2014 American Pain Society. Published by Elsevier Inc. All rights reserved.

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

    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

  7. Effect of oxidative stress induced by intracranial iron overload on central pain after spinal cord injury.

    Meng, Fan Xing; Hou, Jing Ming; Sun, Tian Sheng

    2017-02-08

    Central pain (CP) is a common clinical problem in patients with spinal cord injury (SCI). Recent studies found the pathogenesis of CP was related to the remodeling of the brain. We investigate the roles of iron overload and subsequent oxidative stress in the remodeling of the brain after SCI. We established a rat model of central pain after SCI. Rats were divided randomly into four groups: SCI, sham operation, SCI plus deferoxamine (DFX) intervention, and SCI plus nitric oxide synthase (NOS) inhibitor treatment. Pain behavior was observed and thermal pain threshold was measured regularly, and brain levels of iron, transferrin receptor 1 (TfR1), ferritin (Fn), and lactoferrin (Lf), were detected in the different groups 12 weeks after establishment of the model. Rats demonstrated self-biting behavior after SCI. Furthermore, the latent period of thermal pain was reduced and iron levels in the hind limb sensory area, hippocampus, and thalamus increased after SCI. Iron-regulatory protein (IRP) 1 levels increased in the hind limb sensory area, while Fn levels decreased. TfR1 mRNA levels were also increased and oxidative stress was activated. Oxidative stress could be inhibited by ferric iron chelators and NOS inhibitors. SCI may cause intracranial iron overload through the NOS-iron-responsive element/IRP pathway, resulting in central pain mediated by the oxidative stress response. Iron chelators and oxidative stress inhibitors can effectively relieve SCI-associated central pain.

  8. Congenital central hypoventilation syndrome and intestinal ...

    ... hypoventilation syndrome (CCHS), also called 'Ondine's curse', is characterised by an abnormal ventilatory response to progressive hypercapnia and sustained hypoxaemia. Neonates with this condition experience hypoventilation or apnoea while asleep. Patients may also have congenital intestinal aganglionosis (CIA), ...

  9. Evaluating the evidence: is phrenic nerve stimulation a safe and effective tool for decreasing ventilator dependence in patients with high cervical spinal cord injuries and central hypoventilation?

    Sieg, Emily P; Payne, Russell A; Hazard, Sprague; Rizk, Elias

    2016-06-01

    Case reports, case series and case control studies have looked at the use of phrenic nerve stimulators in the setting of high spinal cord injuries and central hypoventilation syndromes dating back to the 1980s. We evaluated the evidence related to this topic by performing a systematic review of the published literature. Search terms "phrenic nerve stimulation," "phrenic nerve and spinal cord injury," and "phrenic nerve and central hypoventilation" were entered into standard search engines in a systematic fashion. Articles were reviewed by two study authors and graded independently for class of evidence according to published guidelines. The published evidence was reviewed, and the overall body of evidence was evaluated using the grading of recommendations, assesment, development and evaluations (GRADE) criteria Balshem et al. (J Clin Epidemiol 64:401-406, 2011). Our initial search yielded 420 articles. There were no class I, II, or III studies. There were 18 relevant class IV articles. There were no discrepancies among article ratings (i.e., kappa = 1). A meta-analysis could not be performed due to the low quality of the available evidence. The overall quality of the body of evidence was evaluated using GRADE criteria and fell within the "very poor" category. The quality of the published literature for phrenic nerve stimulation is poor. Our review of the literature suggests that phrenic nerve stimulation is a safe and effective option for decreasing ventilator dependence in high spinal cord injuries and central hypoventilation; however, we are left with critical questions that provide crucial directions for future studies.

  10. Metabolic syndrome in people with a long-standing spinal cord injury : associations with physical activity and capacity

    de Groot, Sonja; Adriaansen, Jacinthe J.; Tepper, Marga; Snoek, Govert J.; van der Woude, Lucas H. V.; Post, Marcel W. M.

    This study investigated (i) the prevalence of the metabolic syndrome (MetS) in people with a long-standing spinal cord injury (SCI); (ii) whether personal or lesion characteristics are determinants of the MetS; and (iii) the association with physical activity or peak aerobic capacity on the MetS. In

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

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

    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

  12. Sjogrens Syndrome Presenting with Central Nervous System Involvement

    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.

  13. [Peripheral, central and functional vertigo syndromes].

    Strupp, M; Dieterich, M; Zwergal, A; Brandt, T

    2015-12-01

    Depending on the temporal course, three forms of vertigo syndrome can be differentiated: 1) vertigo attacks, e.g. benign paroxysmal positional vertigo (BPPV), Menière's disease and vestibular migraine, 2) acute spontaneous vertigo lasting for days, e.g. acute unilateral vestibulopathy, brainstem or cerebellar infarction and 3) symptoms lasting for months or years, e.g. bilateral vestibulopathy and functional vertigo. The specific therapy of the various syndromes is based on three principles: 1) physical treatment with liberatory maneuvers for BPPV and balance training for vestibular deficits, 2) pharmacotherapy, e.g. for acute unilateral vestibulopathy (corticosteroids) and Menière's disease (transtympanic administration of gentamicin or steroids and high-dose betahistine therapy); placebo-controlled pharmacotherapy studies are currently being carried out for acute unilateral vestibulopathy, vestibular paroxysmia, prophylaxis of BPPV, vestibular migraine, episodic ataxia type 2 and cerebellar ataxia; 3) psychotherapy for functional dizziness.

  14. Development of a tree shrew metabolic syndrome model and use of umbilical cord mesenchymal stem cell transplantation for treatment.

    Pan, Xing-Hua; Zhu, Lu; Yao, Xiang; Liu, Ju-Fen; Li, Zi-An; Yang, Jian-Yong; Pang, Rong-Qing; Ruan, Guang-Ping

    2016-12-01

    The aim of this study was to establish a tree shrew metabolic syndrome model and demonstrate the utility of MSCs in treating metabolic syndrome. We used tree shrew umbilical cord mesenchymal stem cell (TS-UC-MSC) transplantation for the treatment of metabolic syndrome to demonstrate the clinical application of these stem cells and to provide a theoretical basis and reference methods for this treatment. Tree shrew metabolic syndrome model showed significant insulin resistance, high blood sugar, lipid metabolism disorders, and hypertension, consistent with the diagnostic criteria. TS-UC-MSC transplantation at 16 weeks significantly reduced blood sugar and lipid levels, improved insulin resistance and the regulation of insulin secretion, and reduced the expression levels of the pro-inflammatory cytokines IL-1 and IL-6 (P metabolic syndrome model and showed that MSC migrate in diseased organs and can attenuate metabolic syndrome severity in a tree shrew model.

  15. Continuous Thoracic Sympathetic Ganglion Block in Complex Regional Pain Syndrome Patients with Spinal Cord Stimulation Implantation

    EungDon Kim

    2016-01-01

    Full Text Available The sympathetic block is widely used for treating neuropathic pain such as complex regional pain syndrome (CRPS. However, single sympathetic block often provides only short-term effect. Moreover, frequent procedures for sympathetic block may increase the risk of complications. The use of epidural route may be limited by concern of infection in case of previous implantation of the spinal cord stimulation (SCS. In contrast, a continuous sympathetic block can be administered without such concerns. The continuous thoracic sympathetic block (TSGB has been used to treat the ischemic disease and other neuropathic conditions such as postherpetic neuralgia. We administered continuous thoracic sympathetic block using catheter in CRPS patients who underwent SCS implantations and achieved desirable outcomes. We believe a continuous sympathetic block is a considerable option before performing neurolysis or radiofrequency rhizotomy and even after SCS implantation.

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

    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.

  17. JNK-induced MCP-1 production in spinal cord astrocytes contributes to central sensitization and neuropathic pain.

    Gao, Yong-Jing; Zhang, Ling; Samad, Omar Abdel; Suter, Marc R; Yasuhiko, Kawasaki; Xu, Zhen-Zhong; Park, Jong-Yeon; Lind, Anne-Li; Ma, Qiufu; Ji, Ru-Rong

    2009-04-01

    Our previous study showed that activation of c-jun-N-terminal kinase (JNK) in spinal astrocytes plays an important role in neuropathic pain sensitization. We further investigated how JNK regulates neuropathic pain. In cultured astrocytes, tumor necrosis factor alpha (TNF-alpha) transiently activated JNK via TNF receptor-1. Cytokine array indicated that the chemokine CCL2/MCP-1 (monocyte chemoattractant protein-1) was strongly induced by the TNF-alpha/JNK pathway. MCP-1 upregulation by TNF-alpha was dose dependently inhibited by the JNK inhibitors SP600125 (anthra[1,9-cd]pyrazol-6(2H)-one) and D-JNKI-1. Spinal injection of TNF-alpha produced JNK-dependent pain hypersensitivity and MCP-1 upregulation in the spinal cord. Furthermore, spinal nerve ligation (SNL) induced persistent neuropathic pain and MCP-1 upregulation in the spinal cord, and both were suppressed by D-JNKI-1. Remarkably, MCP-1 was primarily induced in spinal cord astrocytes after SNL. Spinal administration of MCP-1 neutralizing antibody attenuated neuropathic pain. Conversely, spinal application of MCP-1 induced heat hyperalgesia and phosphorylation of extracellular signal-regulated kinase in superficial spinal cord dorsal horn neurons, indicative of central sensitization (hyperactivity of dorsal horn neurons). Patch-clamp recordings in lamina II neurons of isolated spinal cord slices showed that MCP-1 not only enhanced spontaneous EPSCs but also potentiated NMDA- and AMPA-induced currents. Finally, the MCP-1 receptor CCR2 was expressed in neurons and some non-neuronal cells in the spinal cord. Together, we have revealed a previously unknown mechanism of MCP-1 induction and action. MCP-1 induction in astrocytes after JNK activation contributes to central sensitization and neuropathic pain facilitation by enhancing excitatory synaptic transmission. Inhibition of the JNK/MCP-1 pathway may provide a new therapy for neuropathic pain management.

  18. The central anticholinergic syndrome in the postoperative period

    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

  19. Metabolic Syndrome in a Rural Nigerian Community: Is Central ...

    Alasia Datonye

    ABSTRACT. Background. Metabolic syndrome (MS) is primarily the consequence of excess central adiposity but can also result from low grade systemic inflammation inducing insulin resistance. There is a global increase in the prevalence of MS; it is on this background that evaluation of the prevalence of MS in a poor rural ...

  20. Hypercalculia in savant syndrome: central executive failure?

    González-Garrido, Andrés Antonio; Ruiz-Sandoval, José Luis; Gómez-Velázquez, Fabiola R; de Alba, José Luis Oropeza; Villaseñor-Cabrera, Teresa

    2002-01-01

    The existence of outstanding cognitive talent in mentally retarded subjects persists as a challenge to present knowledge. We report the case of a 16-year-old male patient with exceptional mental calculation abilities and moderate mental retardation. The patient was clinically evaluated. Data from standard magnetic resonance imaging (MRI) and two 99mTc-ethyl cysteine dimer (ECD)-single photon emission computer tomography (SPECT) (in resting condition and performing a mental calculation task) studies were analyzed. Main neurologic findings were brachycephalia, right-side neurologic soft signs, obsessive personality profile, low color-word interference effect in Stroop test, and diffuse increased cerebral blood flow during calculation task in 99mTc-ECD SPECT. MRI showed anatomical temporal plane inverse asymmetry. Evidence appears to support the hypothesis that savant skill is related to excessive and erroneous use of cognitive processing resources instigated by probable failure in central executive control mechanisms.

  1. Electroversion in treatment of arrhythmia in a patient with Wolff-Parkinson-White syndrome and cervical spinal cord injury

    SHEN Peng

    2013-06-01

    Full Text Available 【Abstract】We report electroversion in treatment of atrial fibrillation (AF and atrioventricular nodal reentry ta-chycardia (AVNRT in a patient with Wolff-Parkinson-White syndrome and cervical spinal cord injury. At first, the pa-tient sustained respiratory failure and weak cough reflex, thereafter repeated bronchoscopy was used to aspirate the sputum as well as control the pneumonia, which resulted in arrhythmia (AF and AVNRT. Two doses of intravenous amiodarone failed to correct the arrhythmia. After restora-tion of sinus rhythm by electroversion, he was successfully weaned from mechanical ventilation and discharged from the intensive care unit without recurrent arrhythmia. Key words: Arrhythmia, cardiac; Atrial fibrillation; Electric countershock; Wolff-Parkinson-White syndrome; Spinal cord injuries

  2. Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

    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.

  3. Tripolar spinal cord stimulation for the treatment of abdominal pain associated with irritable bowel syndrome.

    Rana, Maunak V; Knezevic, Nebojsa Nick

    2013-01-01

    The objective of this case report is to describe the use of transverse tripolar dorsal column stimulation in a patient with a history of irritable bowel syndrome (IBS) associated with abdominal pain resistant to conservative treatments. We report a 36-year-old man who presented to the pain clinic with an eight-year history of IBS (constipation predominant with occasional diarrheal episodes), with "crampy and sharp" abdominal pain. He also had nonradicular thoracic spine pain due to thoracic scoliosis. Both pains were affecting his ability to function as an attorney. Prior conservative therapy, including psychologic treatment, antidepressants, and opioids, was without any benefits. The use of a spinal cord stimulator (SCS) was discussed with the patient. The procedure was performed after Institutional Review Board approval. A tripolar SCS was implanted at the T8 level using one-eight contact and two-four contact percutaneous leads based on paresthesia reproduction of patient's areas of discomfort. This tripolar spinal cord stimulation provided relief of abdominal and thoracic pain, and better management of gastrointestinal symptoms. The patient was followed-up for one year, and his quality of life also was improved via the IBS-Severity Scoring System quality of life tool. The use of the tripolar SCS in this patient provided relief of abdominal and thoracic spine pain, regulated bowel habits, and improved the patient's quality of life. We believe that the use of SCS should be considered as a treatment option in patients with IBS when all conservative treatments failed. © 2012 International Neuromodulation Society.

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

    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.)

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

    Munoz, Alberto; Barkovich, James A.; Mateos, Fernando; Simon, Rogelio

    2002-01-01

    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.)

  6. Amyotrophic lateral sclerosis with extension of the central canal of the spinal cord according to magnetic resonance imaging data

    E. G. Mendelevich; G. R. Mukhamedzhanova; E. I. Bogdanov

    2016-01-01

    The paper describes an atypical case of amyotrophic lateral sclerosis (ALS) concurrent with extension of the central canal – hydromyelia.Diagnostic difficulty is due to the presence of symptoms which are atypical for ALS, such as early age of onset, slow progression with long-term involvement of one leg, as well as extension of the central canal of the spinal cord at the level of TIII-IX bodies, as evidenced by magnetic resonance imaging (MRI). The paper presents a clinical case, a review of ...

  7. Central xanthoma of the jaw in association with Noonan syndrome.

    Olson, Nicholas J; Addante, Rocco R; de Abreu, Francine B; Memoli, Vincent A

    2018-05-01

    Xanthomas are histiocytic lesions of the skin, soft tissue and bone and are generally considered to be reactive in nature. When they arise in the bones of the jaw, they are referred to as central xanthomas. New evidence supports the hypothesis that central xanthomas are a separate and distinct entity from their extragnathic counterparts. Noonan syndrome (NS) is an autosomal dominant disorder that has been associated with giant cell lesions which also commonly occur in the jaw. We present a case of a 15year-old-male with NS who presented with a radiolucent lesion of the mandible that on excision, was found to be a central xanthoma. Although giant cell lesions have been well described in NS, xanthomas of the jaw have not been reported. We will also discuss the entities that must be excluded prior to making a diagnosis of central xanthoma, as this can affect both treatment and follow up. Copyright © 2018. Published by Elsevier Inc.

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

    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

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

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

    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.

  11. Dopaminergic treatment of restless legs syndrome in spinal cord injury patients with neuropathic pain.

    Kumru, Hatice; Albu, Sergiu; Vidal, Joan; Barrio, Manuela; Santamaria, Joan

    2016-01-01

    Recent studies report high incidence of restless legs syndrome (RLS) in patients with spinal cord injury (SCI), who may also present pain and sensory disturbances. In the present manuscript, we examine and discuss diagnostic and treatment challenges of comorbid RLS and neuropathic pain (NP) in SCI. We evaluated seven men with a mean age of 55.6 (s.d.=14.0) years, with chronic complete or incomplete SCI at the thoracic or lumbar level, for complaints of sensory disturbances in the legs, which initially were attributed to drug-resistant NP. Because overlapped RLS was suspected, clinical evaluation of NP and RLS, serum ferritin and iron level assessment, and video polysomnographic (VPSG) studies were conducted. Pramipexole (0.18 mg q.d. -1 ) was added to treat RLS, and a follow-up was performed at 2 months. We found that in six subjects the RLS was comorbid with NP and in one subject the symptoms of RLS were misdiagnosed as NP. VPSG revealed periodic limb movements (PLMs) in all patients, including PLMs of the legs, arms or both. Serum ferritin was patients. RLS improved significantly after 2 months with pramipexole. On the basis of current findings, we recommend physicians to be aware of the comorbidity between RLS and NP secondary to SCI to include suitable diagnostic procedures and effective treatments.

  12. Clinical Evidence for Spinal Cord Stimulation for Failed Back Surgery Syndrome (FBSS): Systematic Review.

    Kapural, Leonardo; Peterson, Erika; Provenzano, David A; Staats, Peter

    2017-07-15

    A systematic review. A systematic literature review of the clinical data from prospective studies was undertaken to assess the efficacy of spinal cord stimulation (SCS) in the treatment of failed back surgery syndrome (FBSS) in adults. For patients with unrelenting back pain due to mechanical instability of the spine, degenerative disc disease, spinal injury, or deformity, spinal surgery is a well-accepted treatment option; however, even after surgical intervention, many patients continue to experience chronic back pain that can be notoriously difficult to treat. Clinical evidence suggests that for patients with FBSS, repeated surgery will not likely offer relief. Additionally, evidence suggests long-term use of opioid pain medications is not effective in this population, likely presents additional complications, and requires strict management. A systematic literature review was performed using several bibliographic databases, prospective studies in adults using SCS for FBSS were included. SCS has been shown to be a safe and efficacious treatment for this patient population. Recent technological developments in SCS offer even greater pain relief to patients' refractory to other treatment options, allowing patients to regain functionality and improve their quality of life with significant reductions in pain. N/A.

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

    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.

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

    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

  15. Bilateral vocal cord paralysis and hypothyroidism as presenting symptoms of Williams-Beuren syndrome: a case report.

    Koren, Ilana; Kessel, Ira; Rotschild, Avi; Cohen-Kerem, Raanan

    2015-09-01

    Williams-Beuren syndrome is a rare neurodevelopmental disorder caused by deletion of 1.5-1.8Mb genes on chromosome 7q11.23. The syndrome was first described as a triad of supra-valvular aortic stenosis, mental retardation, and distinctive facial features. Our patient was referred due to audible inspiratory stridor when he was seven days old. Following endoscopy he was diagnosed with bilateral vocal cord paralysis and was eventually intubated due to respiratory de-compensation followed by tracheotomy. On further workup he was diagnosed with hypothyroidism. Genetic workup supported the diagnosis of Williams-Beuren syndrome. We report here a case with an unusual clinical presentation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  16. HLA-A, -B, -DRB1 allele and haplotype frequencies of 920 cord blood units from Central Chile.

    Schäfer, Christian; Sauter, Jürgen; Riethmüller, Tobias; Kashi, Zahra Mehdizadeh; Schmidt, Alexander H; Barriga, Francisco J

    2016-08-01

    We present human leukocyte antigen (HLA) haplotype and allele/antigenic group frequencies derived from a data set of 920 umbilical cord blood units collected in Central Chile. HLA-A and -B genotypes were typed using sequence specific oligonucleotide probe methods while HLA-DRB1 genotypes were obtained from sequencing-based typing. The most frequent haplotype is A*29~B*44~DRB1*07:01 with an estimated frequency of 2.1%. Copyright © 2016 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.

  17. Pre-differentiated GABAergic neural precursor transplants for alleviation of dysesthetic central pain following excitotoxic spinal cord injury

    Jeung Woon eLee

    2012-05-01

    Full Text Available Intraspinal quisqualic acid (QUIS injury induce (i mechanical and thermal hyperalgesia, (ii progressive self-injurious overgrooming of the affected dermatome. The latter is thought to resemble painful dysesthesia observed in spinal cord injury (SCI patients. We have reported previously loss of endogenous GABA immunoreactive (IR cells in the superficial dorsal horn of QUIS rats 2 weeks post-injury. Further histological evaluation showed that GABA-, glycine-, and synaptic vesicular transporter VIAAT-IR persisted but were substantially decreased in the injured spinal cord. In this study, partially-differentiated GABA-IR embryonic neural precursor cells (NPCs were transplanted into the spinal cord of QUIS rats to reverse overgrooming by replenishing lost inhibitory circuitry. Rat E14 NPCs were predifferentiated in 0.1 ng/ml FGF-2 for 4 hrs prior to transplantation. In vitro immunocytochemistry of transplant cohort showed large population of GABA-IR NPCs that double labeled with nestin but few co-localized with NeuN, indicating partial maturation. Two weeks following QUIS lesion at T12-L1, and following the onset of overgrooming, NPCs were transplanted into the QUIS lesion sites; bovine adrenal fibroblast cells were used as control. Overgrooming was reduced in >55.5% of NPC grafted animals, with inverse relationship between the number of surviving GABA-IR cells and the size of overgrooming. Fibroblast-control animals showed a progressive worsening of overgrooming. At 3 weeks post-transplantation, numerous GABA-, nestin-, and GFAP-IR cells were present in the lesion site. Surviving grafted GABA-IR NPCs were NeuN+ and GFAP-. These results indicate that partially-differentiated NPCs survive and differentiate in vivo into neuronal cells following transplantation into an injured spinal cord. GABA-IR NPC transplants can restore lost dorsal horn inhibitory signaling and are useful in alleviating central pain following SCI.

  18. [Munchausen syndrome: need for centralized administrative health-care management].

    Altmark, D; Sigal, M; Gelkopf, M

    1991-03-01

    The clinical picture of Munchausen syndrome is that of feigned or self-induced illness with the aim of being hospitalized and/or receiving unnecessary medical interventions, peregrination from 1 hospital to another, and disruptive behavior when hospitalized. These patients are a danger to themselves and heavily burden inpatient facilities that care for them. We present a case that illustrates the problems of diagnosing and managing such patients. We stress the need for adequate centralization and distribution of the relevant information concerning these patients.

  19. Case report: Noonan-like multiple central giant cell granuloma syndrome.

    Bitton, Natalie; Alexander, Stanley; Ruggiero, Salvatore; Parameswaran, Ashish; Russo, Antonino; Ferguson, Fred

    2012-01-01

    The purpose of this report was to: summarize the care of a child between the ages of 12 to 16 years old born with Noonan-like central giant cell syndrome and unrelated common variable immune deficiency; provide information on the dental management of patients with Noonan's syndrome; and present a brief discussion of the recent associated genetic findings. A review of the common features of Noonan syndrome and Noonan-like central giant cell syndrome is also provided.

  20. Amyotrophic lateral sclerosis with extension of the central canal of the spinal cord according to magnetic resonance imaging data

    E. G. Mendelevich

    2016-01-01

    Full Text Available The paper describes an atypical case of amyotrophic lateral sclerosis (ALS concurrent with extension of the central canal – hydromyelia.Diagnostic difficulty is due to the presence of symptoms which are atypical for ALS, such as early age of onset, slow progression with long-term involvement of one leg, as well as extension of the central canal of the spinal cord at the level of TIII-IX bodies, as evidenced by magnetic resonance imaging (MRI. The paper presents a clinical case, a review of current literature on the clinical and MRI signs of ALS and on the differential diagnosis of motor neuron disease.

  1. '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.

    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.

  2. Acrolein contributes to TRPA1 up-regulation in peripheral and central sensory hypersensitivity following spinal cord injury.

    Park, Jonghyuck; Zheng, Lingxing; Acosta, Glen; Vega-Alvarez, Sasha; Chen, Zhe; Muratori, Breanne; Cao, Peng; Shi, Riyi

    2015-12-01

    Acrolein, an endogenous aldehyde, has been shown to be involved in sensory hypersensitivity after rat spinal cord injury (SCI), for which the pathogenesis is unclear. Acrolein can directly activate a pro-algesic transient receptor protein ankyrin 1 (TRPA1) channel that exists in sensory neurons. Both acrolein and TRPA1 mRNA are elevated post SCI, which contributes to the activation of TRPA1 by acrolein and consequently, neuropathic pain. In the current study, we further showed that, post-SCI elevation of TRPA1 mRNA exists not only in dorsal root ganglias but also in both peripheral (paw skin) and central endings of primary afferent nerves (dorsal horn of spinal cord). This is the first indication that pain signaling can be over-amplified in the peripheral skin by elevated expressions of TRPA1 following SCI, in addition over-amplification previously seen in the spinal cord and dorsal root ganglia. Furthermore, we show that acrolein alone, in the absence of physical trauma, could lead to the elevation of TRPA1 mRNA at various locations when injected to the spinal cord. In addition, post-SCI elevation of TRPA1 mRNA could be mitigated using acrolein scavengers. Both of these attributes support the critical role of acrolein in elevating TRPA1 expression through gene regulation. Taken together, these data indicate that acrolein is likely a critical causal factor in heightening pain sensation post-SCI, through both the direct binding of TRPA1 receptor, and also by boosting the expression of TRPA1. Finally, our data also further support the notion that acrolein scavenging may be an effective therapeutic approach to alleviate neuropathic pain after SCI. We propose that the trauma-mediated elevation of acrolein causes neuropathic pain through at least two mechanisms: acrolein stimulates the production of transient receptor protein ankyrin 1 (TRPA1) in both central and peripheral locations, and it activates TRPA1 channels directly. Therefore, acrolein appears to be a critical

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

    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

  4. Congenital central hypoventilation syndrome: diagnostic and management challenges

    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

  5. Intrauterine Reprogramming of the Polycystic Ovary Syndrome: Evidence from a Pilot Study of Cord Blood Global Methylation Analysis

    Luca Lambertini

    2017-12-01

    Full Text Available Polycystic ovary syndrome (PCOS affects 5–15% of women. PCOS is a heterogeneous disorder displaying endocrine, metabolic, and reproductive dysfunction and cardiovascular risk manifestations. Evidence of heritability exists, but only a portion of the genetic transmission has been identified by genome-wide association studies and linkage studies, suggesting epigenetic phenomena may play a role. Evidence implicates intrauterine influences in the genesis of PCOS. This was a pilot study that aimed at identifying an epigenetic PCOS reprogramming signature by profiling the methylation of the DNA extracted from umbilical cord blood (UCB from 12 subjects undergoing in vitro fertilization. Six subjects were anovulatory PCOS women diagnosed by Rotterdam criteria and six ovulatory non-PCOS women matched for age and body mass index. UCB was collected at delivery of the placenta; the DNA was extracted and submitted to methylation analysis. A differential methylation picture of prevalent hypomethylation affecting 918 genes was detected. Of these, 595 genes (64.8% carried single or multiple hypomethylated CpG dinucleotides and 323 genes (35.2% single or multiple hypermethylated CpG dinucleotides. The Ingenuity Pathway Analysis (IPA online platform enlisted 908 of the 918 input genes and clustered 794 of them into 21 gene networks. Key features of the primary networks scored by IPA included carbohydrate and lipid metabolism, neurotransmitter signaling, cardiovascular system development and function, glycosaminoglycan signaling regulation and control of amino acid biosynthesis. Central to the network activities were genes controlling hormonal regulation (ESR1, mitochondrial activity (APP, PARK2, and glucose metabolism (INS. Regulatory pathways such as G-protein coupled receptor signaling, inositol metabolism, and inflammatory response were also highlighted. These data suggested the existence of a putative “PCOS epigenomic superpathway” with three main

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

    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.

  7. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis

    Izumi M

    2011-12-01

    Full Text Available Manabu Izumi, Shoko Teraoka, Keisuke Yamashita, Kenji Matsumoto, Tomohiro Muronoi, Yoshimitsu Izawa, Chikara Yonekawa, Masaki Ano, Masayuki SuzukawaDepartment of Emergency and Critical Care Medicine, Jichi Medical University, Tochigi, JapanAbstract: A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.Keywords: intra-aortic thrombus, antiphospholipid antibody syndrome, spinal cord infarction

  8. Comparable Enhanced Prothrombogenesis in Simple Central Obesity and Metabolic Syndrome

    Noor Shafina Mohd Nor

    2018-01-01

    Full Text Available Objective. There is limited data comparing prothrombogenic or fibrinolysis biomarkers (tissue plasminogen activator (tPA and plasminogen activator inhibitor-1 (PAI-1 simultaneously in subjects with Metabolic Syndrome (MS, simple central obesity without MS (COB and normal controls (NC. We investigated the concentrations of fibrinolysis biomarkers in subjects with MS, COB and NC. Methods. A cross-sectional study involving 503 drug naive subjects (163 males, aged 30–65 years old (mean age ± SD = 47.4 ± 8.3 years divided into MS, COB and NC groups. COB was defined as central obesity (waist circumference (WC males ≥90 cm, females ≥80 cm in the absence of MS according to the International Diabetes Federation 2006. Fasting blood levels of tPA and PAI-1were analyzed. Results. MS and COB had significantly higher concentration of all biomarkers compared to NC. The MS group had significantly higher concentration of tPA and PAI-1 compared to COB. WC and HDL-c had significant correlation with all biomarkers (tPA p<0.001, PAI-1 p<0.001. Fasting plasma glucose and diastolic blood pressure were independent predictors after correcting for confounding factors. Conclusion. Central obesity with or without MS both demonstrated enhanced prothrombogenesis. This suggests that simple obesity possibly increases the risk of coronary artery disease in part, via increased susceptibility to thrombogenesis.

  9. Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

    Steven S. Saraf

    2015-01-01

    Full Text Available A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient’s vision improved and remained stable at 20/200 and 20/80.

  10. Hippocampal volume reduction in congenital central hypoventilation syndrome.

    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.

  11. Therapy for sleep hypoventilation and central apnea syndromes.

    Selim, Bernardo J; Junna, Mithri R; Morgenthaler, Timothy I

    2012-10-01

    • Primary Central Sleep Apnea (CSA): We would recommend a trial of Positive Airway Pressure (PAP), acetazolamide, or zolpidem based on thorough consideration of risks and benefits and incorporation of patient preferences.• Central Sleep Apnea Due to Cheyne-Stokes Breathing Pattern in Congestive Heart Failure (CSR-CHF): We would recommend PAP devices such as continuous positive airway pressure (CPAP) or adaptive servo-ventilation (ASV) to normalize sleep-disordered breathing after optimizing treatment of heart failure. Oxygen may also be an effective therapy. Acetazolamide and theophylline may be considered if PAP or oxygen is not effective.• Central Sleep Apnea due to High-Altitude Periodic Breathing: We would recommend descent from altitude or supplemental oxygen. Acetazolamide may be used when descent or oxygen are not feasible, or in preparation for ascent to high altitude. Slow ascent may be preventative.• Central Sleep Apnea due to Drug or Substance: If discontinuation or reduction of opiate dose is not feasible or effective, we would recommend a trial of CPAP, and if not successful, treatment with ASV. If ASV is ineffective or if nocturnal hypercapnia develops, bilevel positive airway pressure-spontaneous timed mode (BPAP-ST) is recommended.• Obesity hypoventilation syndrome: We would recommend an initial CPAP trial. If hypoxia or hypercapnia persists on CPAP, BPAP, BPAP-ST or average volume assured pressure support (AVAPS™) is recommended. Tracheostomy with nocturnal ventilation should be considered when the above measures are not effective. Weight loss may be curative.• Neuromuscular or chest wall disease: We would recommend early implementation of BPAP-ST based on thorough consideration of risks and benefits and patient preferences. AVAPS™ may also be considered. We recommend close follow up due to disease progression.

  12. CENTRAL SENSITIZATION AND MEDICATION IN SPINAL CORD INJURED IN-PATIENTS. A CROSS-SECTIONAL CLINICAL STUDY

    Rosendahl, A; Kasch, Helge

    Background and aims: A major proportion of spinal cord injured subjects (SCIS) suffers from chronic pain. A majority with neuropathic pain, being: shooting, burning and stabbing. Neurological examination reveals signs of central sensitization (CS) e.g. allodynia and hyperalgesia. CS plays...... an important role in maintained neuropathic pain conditions and may lead to or be induced by analgesics. Medication-overuse-headaches (MOH) alter CNS pain processing systems, and the situation is reversed after discontinuation of headache medication. Aim: To determine the occurrence of CS and conditions...... pressure algometry, Von Frey filaments and pinprick test. Patients fulfill McGill Pain Questionnaire and the International SCI pain data-set. All participants undergo examination of the Pressure Pain Detection Threshold, Pressure Pain Tolerance Threshold, Mechanical Detection Threshold, and Wind...

  13. Cervical Retrograde Spinal Cord Stimulation Lead Placement to Treat Failed Back Surgery Syndrome: A Case Report

    Helmond, N. van; Kardaszewski, C.N.; Chapman, K.B.

    2017-01-01

    Spinal cord stimulation is an effective treatment modality for refractory neuropathic pain conditions, but the placement of leads can be challenging due to unforeseen anatomical variations. We used a retrograde C7-T1 approach to place a lead at the bottom of T8 in a patient suffering from failed

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

    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

  15. High-cervical spinal cord electrical stimulation in brain low perfusion syndromes: experimental basis and preliminary clinical report.

    Broseta, J; García-March, G; Sánchez-Ledesma, M J; Gonçalves, J; Silva, I; Barcia, J A; Llácer, J L; Barcia-Salorio, J L

    1994-01-01

    Previous studies of our group showed that C1-C2 spinal cord stimulation increases carotid and brain blood flow in normal conditions in the goat and dog and it has a beneficial vasomotor effect in a model of vasospasm in the rat. For further clinical application it seemed rational to investigate the possible vascular changes mediated by this technique in experimental brain infarction. To this aim, 45 New Zealand rabbits were used. Brain infarction was produced by bilateral carotid ligation in 15, unilateral microcoagulation of the middle cerebral artery in 15 and by microcoagulation of the vertebral artery at the craniocervical junction in the other 15. One week later, following daily clinical scoring and cortical and posterior fossa blood flow readings by laser Doppler, a period of 120 min of right C1-C2 spinal cord electric stimulation was performed. A mean of 27% increase in previous blood flow recordings was obtained at the right hemisphere and a mean of 32% in the posterior fossa. This procedure was used in 10 patients presenting with various cerebral low perfusion syndromes. Though not constant, an increase in alertness, retention, speech, emotional lability and performance in skilled acts was achieved. No MR changes were observed, though SPECT readings showed an increase in blood flow in the penumbral perilesional area.

  16. Central-Variant Posterior Reversible Encephalopathy Syndrome with Albuminocytologic Dissociation.

    Lee, Sang-Woo; Lee, Seung-Jae

    2018-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a disorder of reversible vasogenic brain edema which mainly involves the parieto-occipital lobes in various clinical settings. The main mechanism is known to be cerebral autoregulation failure and endothelial dysfunction leading to the disruption of the blood-brain barrier. We report the case of a 47-year-old woman with PRES which involved the brain stem and thalami, sparing the cerebral hemispheres. She was admitted to the emergency room because of acute-onset confusion. Her initial blood pressure was 270/220 mm Hg. Routine blood lab tests showed pleocytosis, hyperglycemia, and azotemia. Brain magnetic resonance imaging (MRI) showed a lesion of vasogenic edema involving nearly the whole area of pons, the left side of the midbrain, and the bilateral medial thalami. Cerebrospinal fluid (CSF) examination revealed an increased level of protein with normal white blood cell count. With conservative care, the patient markedly recovered 3 days after symptom onset, and a follow-up MRI confirmed complete resolution of the vasogenic edema. This case suggests that PRES can rarely involve the "central zone" only, sparing the cerebral hemispheres, which may be confused with other neurological diseases. Besides, the CSF albuminocytologic dissociation may suggest the disruption of the blood-brain barrier in patients with PRES.

  17. Central-Variant Posterior Reversible Encephalopathy Syndrome with Albuminocytologic Dissociation

    Sang-Woo Lee

    2018-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a disorder of reversible vasogenic brain edema which mainly involves the parieto-occipital lobes in various clinical settings. The main mechanism is known to be cerebral autoregulation failure and endothelial dysfunction leading to the disruption of the blood-brain barrier. We report the case of a 47-year-old woman with PRES which involved the brain stem and thalami, sparing the cerebral hemispheres. She was admitted to the emergency room because of acute-onset confusion. Her initial blood pressure was 270/220 mm Hg. Routine blood lab tests showed pleocytosis, hyperglycemia, and azotemia. Brain magnetic resonance imaging (MRI showed a lesion of vasogenic edema involving nearly the whole area of pons, the left side of the midbrain, and the bilateral medial thalami. Cerebrospinal fluid (CSF examination revealed an increased level of protein with normal white blood cell count. With conservative care, the patient markedly recovered 3 days after symptom onset, and a follow-up MRI confirmed complete resolution of the vasogenic edema. This case suggests that PRES can rarely involve the “central zone” only, sparing the cerebral hemispheres, which may be confused with other neurological diseases. Besides, the CSF albuminocytologic dissociation may suggest the disruption of the blood-brain barrier in patients with PRES.

  18. Endogenous neural stem cells in central canal of adult rats acquired limited ability to differentiate into neurons following mild spinal cord injury

    Liu, Yuan; Tan, Botao; Wang, Li; Long, Zaiyun; Li, Yingyu; Liao, Weihong; Wu, Yamin

    2015-01-01

    Endogenous neural stem cells in central canal of adult mammalian spinal cord exhibit stem cell properties following injury. In the present study, the endogenous neural stem cells were labeled with Dil to track the differentiation of cells after mild spinal cord injury (SCI). Compared with 1 and 14 days post mild injury, the number of endogenous neural stem cells significantly increased at the injured site of spinal cord on 3 and 7 days post-injury. Dil-labeled βIII-tublin and GFAP expressing cells could be detected on 7 days post-injury, which indicated that the endogenous neural stem cells in central canal of spinal cord differentiated into different type of neural cells, but there were more differentiated astrocytes than the neurons after injury. Furthermore, after injury the expression of inhibitory Notch1 and Hes1 mRNA began to increase at 6 hours and was evident at 12 and 24 hours, which maintained high levels up to 7 days post-injury. These results indicated that a mild SCI in rat is sufficient to induce endogenous neural stem cells proliferation and differentiation. However, the ability to differentiate into neurons is limited, which may be, at least in part, due to high expression of inhibitory Notch1 and Hes1 genes after injury. PMID:26097566

  19. Frequency of fibromyalgia syndrome in patients with central serous chorioretinopathy

    Ayse Balkarli

    Full Text Available ABSTRACT Purpose: To ınvestigate frequency of fibromyalgia syndrome (FMS among patients with central serous chorioretinopathy (CSCR. Methods: The study included 83 patients with CSCR and 201 age- and sex-matched healthy controls; the mean age was 47.5 ± 11.3 years in the CSCR group (18 women; 21.7% and 47.2 ± 11.2 years in the control group (44 women; 21.9%. All participants were assessed for FMS based on 2010 American College of Rheumatology diagnostic criteria and for depression and anxiety with the Beck Anxiety Inventory (BAI and Beck Depression Inventory (BDI. Results: FMS was diagnosed in 35 patients (42.2% from the CSCR group and in 21 individuals (10.4% from the control group (p<0.001. It was found in 77.77% of the women (14/18 and 32.3% of the men (21/65 in the CSCR group and in 22.7% of the women (10/44 and 7.0% of the men (11/157 in the control group. Familial stress, BDI and BAI scores were higher in the patients with FMS than in those without. When independent risk factors were evaluated by logistic regression analysis, it was found that only the presence of familial stress was a significant risk factor for FMS. Conclusions: Patients with CSCR should be assessed for the presence of FMS, and this should be taken into consideration when developing a treatment plan. Further studies with a larger sample size are needed to clarify the relationship between FMS and CSCR.

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

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

    2012-01-01

    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

  1. Pinch-off syndrome: transection of implantable central venous access device

    Sugimoto, Takuya; Nagata, Hiroshi; Hayashi, Ken; Kano, Nobuyasu

    2012-01-01

    As the population of people with cancer increases so does the number of patients who take chemotherapy. Majority of them are administered parentally continuously. Implantable central venous catheter device is a good choice for those patients; however, severe complication would occur concerning the devices. Pinch-off syndrome is one of the most severe complications. The authors report a severe case of pinch-off syndrome. The patient with the implantable central venous device could not take che...

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

    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.

  3. Association of nicotinic acetylcholine receptors with central respiratory control in isolated brainstem-spinal cord preparation of neonatal rats

    EIKI HATORI

    2006-01-01

    Full Text Available Nicotine exposure is a risk factor in several breathing disorders Nicotinic acetylcholine receptors (nAChRs exist in the ventrolateral medulla, an important site for respiratory control. We examined the effects of nicotinic acetylcholine neurotransmission on central respiratory control by addition of a nAChR agonist or one of various antagonists into superfusion medium in the isolated brainstem-spinal cord from neonatal rats. Ventral C4 neuronal activity was monitored as central respiratory output, and activities of respiratory neurons in the ventrolateral medulla were recorded in whole-cell configuration. RJR-2403 (0.1-10mM, alpha4beta2 nAChR agonist induced dose-dependent increases in respiratory frequency. Non-selective nAChR antagonist mecamylamine (0.1-100mM, alpha4beta2 antagonist dihydro-beta-erythroidine (0.1-100mM, alpha7 antagonist methyllycaconitine (0.1-100mM, and a-bungarotoxin (0.01-10mM all induced dose-dependent reductions in C4 respiratory rate. We next examined effects of 20mM dihydro-beta-erythroidine and 20mM methyllycaconitine on respiratory neurons. Dihydro-beta-erythroidine induces hyperpolarization and decreases intraburst firing frequency of inspiratory and preinspiratory neurons. In contrast, methyllycaconitine has no effect on the membrane potential of inspiratory neurons, but does decrease their intraburst firing frequency while inducing hyperpolarization and decreasing intraburst firing frequency in preinspiratory neurons. These findings indicate that alpha4beta2 nAChR is involved in both inspiratory and preinspiratory neurons, whereas alpha7 nAChR functions only in preinspiratory neurons to modulate C4 respiratory rate

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

    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.

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

    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.

  6. Transplanted Human Stem Cell-Derived Interneuron Precursors Mitigate Mouse Bladder Dysfunction and Central Neuropathic Pain after Spinal Cord Injury.

    Fandel, Thomas M; Trivedi, Alpa; Nicholas, Cory R; Zhang, Haoqian; Chen, Jiadong; Martinez, Aida F; Noble-Haeusslein, Linda J; Kriegstein, Arnold R

    2016-10-06

    Neuropathic pain and bladder dysfunction represent significant quality-of-life issues for many spinal cord injury patients. Loss of GABAergic tone in the injured spinal cord may contribute to the emergence of these symptoms. Previous studies have shown that transplantation of rodent inhibitory interneuron precursors from the medial ganglionic eminence (MGE) enhances GABAergic signaling in the brain and spinal cord. Here we look at whether transplanted MGE-like cells derived from human embryonic stem cells (hESC-MGEs) can mitigate the pathological effects of spinal cord injury. We find that 6 months after transplantation into injured mouse spinal cords, hESC-MGEs differentiate into GABAergic neuron subtypes and receive synaptic inputs, suggesting functional integration into host spinal cord. Moreover, the transplanted animals show improved bladder function and mitigation of pain-related symptoms. Our results therefore suggest that this approach may be a valuable strategy for ameliorating the adverse effects of spinal cord injury. Copyright © 2016 Elsevier Inc. All rights reserved.

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

    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.

  8. Hemorrhagic fever with renal syndrome accompanied by panhypopituitarism and central diabetes insipidus: a case report.

    Ahn, Hee Jung; Chung, Jong-Hoon; Kim, Dong-Min; Yoon, Na-Ra; Kim, Choon-Mee

    2018-03-05

    Central diabetes insipidus (DI) was detected in a patient with hemorrhagic fever with renal syndrome (HFRS) who had been molecularly and serologically diagnosed with Hantaan virus infection. We recommend that clinicians differentiate central DI in HFRS patients with a persistent diuretic phase even when pituitary MRI findings are normal.

  9. Increased psychological distress among individuals with spinal cord injury is associated with central neuropathic pain rather than the injury characteristics.

    Gruener, Hila; Zeilig, Gabi; Laufer, Yocheved; Blumen, Nava; Defrin, Ruth

    2018-02-01

    Cross-sectional study. Central neuropathic pain (CNP) is common after spinal cord injury (SCI). The psychological impact of CNP is not clear. Previous studies reported depression and pain catastrophizing among patients with SCI and CNP; however, the lack of control groups prevented discerning whether these were attributed to CNP or to the SCI itself. The aim was to examine the psychological distress among individuals with SCI with and without CNP and controls to evaluate its impact and possible source. Outpatient clinic of a large rehabilitation center. Individuals with SCI and CNP (n = 27) and without CNP (n = 23), and able-bodied controls (n = 20) participated. Data collection included sociodemographics, SCI characteristics, and level of post-traumatic stress disorder (PTSD), anxiety, stress, depression, and pain catastrophizing. The sensory, affective, and cognitive dimensions of CNP were analyzed. Individuals with SCI and CNP exhibited elevated levels of PTSD, anxiety, stress, depression, and pain catastrophizing compared to the two control groups, which presented similar levels. The psychological variables among the CNP group correlated positively only with the affective dimension of CNP. Neither CNP nor the psychological variables correlated with SCI characteristics. Irrespective of CNP intensity, the affective dimension (suffering) is associated with increased psychological distress. Perhaps individual differences in the response to SCI and/or individual traits rather than the mere exposure to SCI may have a role in the emergence of CNP and psychological distress/mood dysfunction. Rehabilitation programs should prioritize stress management and prevention among individuals with SCI and CNP.

  10. Spinal Cord Stimulation in Failed Back Surgery Syndrome: Effects on Posture and Gait—A Preliminary 3D Biomechanical Study

    L. Brugliera

    2017-01-01

    Full Text Available We studied 8 patients with spinal cord stimulation (SCS devices which had been previously implanted to treat neuropathic chronic pain secondary to Failed Back Surgery Syndrome. The aim of our study was to investigate the effects of SCS on posture and gait by means of clinical scales (Short Form Health Survey-36, Visual Analogue Scale for pain, and Hamilton Depression Rating Scale and instrumented evaluation with 3D Gait Analysis using a stereophotogrammetric system. The latter was performed with the SCS device turned both OFF and ON. We recorded gait and posture using the Davis protocol and also trunk movement during flexion-extension on the sagittal plane, lateral bending on the frontal plane, and rotation on the transversal plane. During and 30 minutes after the stimulation, not only the clinical scales but also spatial-temporal gait parameters and trunk movements improved significantly. Improvement was not shown under stimulation-OFF conditions. Our preliminary data suggest that SCS has the potential to improve posture and gait and to provide a window of pain-free opportunity to optimize rehabilitation interventions.

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

    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.

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

    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.

  13. MRI signal intensity as a maker of impairment in incomplete cervical spinal cord injuries

    Chiba, Hidefumi; Aoki, Haruhito; Hamabe, Masaki; Sasao, Yutaka; Miura, Takehiko

    1998-01-01

    Incomplete cervical spinal cord injuries such as central cord syndrome may result in prolonged spasticity of the limbs, especially disabilities of the upper extremities, even if the patient is able to walk. In this study, relationship between cord impairment and clinical outcome was investigated using MRI. Results showed that small foci of low signal intensity in T 1 -weighted imaging combined with foci of high signal intensity in T 2 -weighted imaging in follow-up MRI are closely related to the severity of sequelae. Small foci of low signal intensity in T 1 -weighted imaging are considered in the literature to indicate myelomalacia or cyst formation with gliosis. (author)

  14. Mirizzi Syndrome: A Case Report | Muthuuri | East and Central ...

    The classical Mirizzi Syndrome described by P. L. Mirizzi in 1948 is characterized by a cholecystocholedochal fistula arising from a calculus in the cystic duct that erodes into the common hepatic duct. The gall bladder eventually collapses due to fibrosis while the terminal bile ducts become dilated. A calculus is usually ...

  15. The adult spinal cord injury without radiographic abnormalities syndrome: magnetic resonance imaging and clinical findings in adults with spinal cord injuries having normal radiographs and computed tomography studies.

    Kasimatis, Georgios B; Panagiotopoulos, Elias; Megas, Panagiotis; Matzaroglou, Charalambos; Gliatis, John; Tyllianakis, Minos; Lambiris, Elias

    2008-07-01

    Spinal cord injury without radiographic abnormalities (SCIWORA) is thought to represent mostly a pediatric entity and its incidence in adults is rather underreported. Some authors have also proposed the term spinal cord injury without radiologic evidence of trauma, as more precisely describing the condition of adult SCIWORA in the setting of cervical spondylosis. The purpose of the present study was to evaluate adult patients with cervical spine injuries and radiological-clinical examination discrepancy, and to discuss their characteristics and current management. During a 16-year period, 166 patients with a cervical spine injury were admitted in our institution (Level I trauma center). Upper cervical spine injuries (occiput to C2, 54 patients) were treated mainly by a Halo vest, whereas lower cervical spine injuries (C3-T1, 112 patients) were treated surgically either with an anterior, or posterior procedure, or both. Seven of these 166 patients (4.2%) had a radiologic-clinical mismatch, i.e., they presented with frank spinal cord injury with no signs of trauma, and were included in the study. Magnetic resonance imaging was available for 6 of 7 patients, showing intramedullary signal changes in 5 of 6 patients with varying degrees of compression from the disc and/or the ligamentum flavum, whereas the remaining patient had only traumatic herniation of the intervertebral disc and ligamentum flavum bulging. Follow-up period was 6.4 years on average (1-10 years). This retrospective chart review provides information on adult patients with cervical spinal cord injuries whose radiographs and computed tomography studies were normal. It furthers reinforces the pathologic background of SCIWORA in an adult population, when evaluated by magnetic resonance imaging. Particularly for patients with cervical spondylosis, special attention should be paid with regard to vascular compromise by predisposing factors such as smoking or vascular disease, since they probably contribute in

  16. Bilateral widespread mechanical pain sensitivity in carpal tunnel syndrome: evidence of central processing in unilateral neuropathy.

    Fernández-de-las-Peñas, César; de la Llave-Rincón, Ana Isabel; Fernández-Carnero, Josué; Cuadrado, María Luz; Arendt-Nielsen, Lars; Pareja, Juan A

    2009-06-01

    The aim of this study was to investigate whether bilateral widespread pressure hypersensitivity exists in patients with unilateral carpal tunnel syndrome. A total of 20 females with carpal tunnel syndrome (aged 22-60 years), and 20 healthy matched females (aged 21-60 years old) were recruited. Pressure pain thresholds were assessed bilaterally over median, ulnar, and radial nerve trunks, the C5-C6 zygapophyseal joint, the carpal tunnel and the tibialis anterior muscle in a blinded design. The results showed that pressure pain threshold levels were significantly decreased bilaterally over the median, ulnar, and radial nerve trunks, the carpal tunnel, the C5-C6 zygapophyseal joint, and the tibialis anterior muscle in patients with unilateral carpal tunnel syndrome as compared to healthy controls (all, P < 0.001). Pressure pain threshold was negatively correlated to both hand pain intensity and duration of symptoms (all, P < 0.001). Our findings revealed bilateral widespread pressure hypersensitivity in subjects with carpal tunnel syndrome, which suggest that widespread central sensitization is involved in patients with unilateral carpal tunnel syndrome. The generalized decrease in pressure pain thresholds associated with pain intensity and duration of symptoms supports a role of the peripheral drive to initiate and maintain central sensitization. Nevertheless, both central and peripheral sensitization mechanisms are probably involved at the same time in carpal tunnel syndrome.

  17. Design of COSMIC: a randomized, multi-centre controlled trial comparing conservative or early surgical management of incomplete cervical cord syndrome without spinal instability.

    Bartels, Ronald H M A; Hosman, Allard J F; van de Meent, Henk; Hofmeijer, Jeannette; Vos, Pieter E; Slooff, Willem Bart; Öner, F Cumhur; Coppes, Maarten H; Peul, Wilco C; Verbeek, André L M

    2013-01-31

    Incomplete cervical cord syndrome without spinal instability is a very devastating event for the patient and the family. It is estimated that up to 25% of all traumatic spinal cord lesions belong to this category. The treatment for this type of spinal cord lesion is still subject of discussion. From a biological point of view early surgery could prevent secondary damage due to ongoing compression of the already damaged spinal cord. Historically, however, conservative treatment was propagated with good clinical results. Proponents for early surgery as well those favoring conservative treatment are still in debate. The proposed trial will contribute to the discussion and hopefully also to a decrease in the variability of clinical practice. A randomized controlled trial is designed to compare the clinical outcome of early surgical strategy versus a conservative approach. The primary outcome is clinical outcome according to mJOA. This also measured by ASIA score, DASH score and SCIM III score. Other endpoints are duration of the stay at a high care department (medium care, intensive care), duration of the stay at the hospital, complication rate, mortality rate, sort of rehabilitation, and quality of life. A sample size of 36 patients per group was calculated to reach a power of 95%. The data will be analyzed as intention-to-treat at regular intervals, but the end evaluation will take place at two years post-injury. At the end of the study, clinical outcomes between treatments attitudes can be compared. Efficacy, but also efficiency can be determined. A goal of the study is to determine which treatment will result in the best quality of life for the patients. This study will certainly contribute to more uniformity of treatment offered to patients with a special sort of spinal cord injury. Gov: NCT01367405.

  18. Design of COSMIC: a randomized, multi-centre controlled trial comparing conservative or early surgical management of incomplete cervical cord syndrome without spinal instability

    Bartels Ronald HMA

    2013-01-01

    Full Text Available Abstract Background Incomplete cervical cord syndrome without spinal instability is a very devastating event for the patient and the family. It is estimated that up to 25% of all traumatic spinal cord lesions belong to this category. The treatment for this type of spinal cord lesion is still subject of discussion. From a biological point of view early surgery could prevent secondary damage due to ongoing compression of the already damaged spinal cord. Historically, however, conservative treatment was propagated with good clinical results. Proponents for early surgery as well those favoring conservative treatment are still in debate. The proposed trial will contribute to the discussion and hopefully also to a decrease in the variability of clinical practice. Methods/Design A randomized controlled trial is designed to compare the clinical outcome of early surgical strategy versus a conservative approach. The primary outcome is clinical outcome according to mJOA. This also measured by ASIA score, DASH score and SCIM III score. Other endpoints are duration of the stay at a high care department (medium care, intensive care, duration of the stay at the hospital, complication rate, mortality rate, sort of rehabilitation, and quality of life. A sample size of 36 patients per group was calculated to reach a power of 95%. The data will be analyzed as intention-to-treat at regular intervals, but the end evaluation will take place at two years post-injury. Discussion At the end of the study, clinical outcomes between treatments attitudes can be compared. Efficacy, but also efficiency can be determined. A goal of the study is to determine which treatment will result in the best quality of life for the patients. This study will certainly contribute to more uniformity of treatment offered to patients with a special sort of spinal cord injury. Trial Registration Gov: NCT01367405

  19. Pinch-off syndrome: transection of implantable central venous access device.

    Sugimoto, Takuya; Nagata, Hiroshi; Hayashi, Ken; Kano, Nobuyasu

    2012-11-30

    As the population of people with cancer increases so does the number of patients who take chemotherapy. Majority of them are administered parentally continuously. Implantable central venous catheter device is a good choice for those patients; however, severe complication would occur concerning the devices. Pinch-off syndrome is one of the most severe complications. The authors report a severe case of pinch-off syndrome. The patient with the implantable central venous device could not take chemotherapy because the device occluded. Further examination revealed the transection of the catheter. The transected fragment of the catheter in the heart was successfully removed by using a loop snare placed through the right femoral vein.

  20. Case Report: Cervical Klippel-Feil syndrome predisposing an ...

    Case Report: Cervical Klippel-Feil syndrome predisposing an elderly African man to central cord myelopathy following minor trauma. ... unique presentation of this case of Klippel-Feil syndrome further supports the impression that following fusion (congenital or acquired) of one segment of the spinal column, hypermobility of ...

  1. Metabolic syndrome in people with a long-standing spinal cord injury: associations with physical activity and capacity.

    de Groot, Sonja; Adriaansen, Jacinthe J; Tepper, Marga; Snoek, Govert J; van der Woude, Lucas H V; Post, Marcel W M

    2016-11-01

    This study investigated (i) the prevalence of the metabolic syndrome (MetS) in people with a long-standing spinal cord injury (SCI); (ii) whether personal or lesion characteristics are determinants of the MetS; and (iii) the association with physical activity or peak aerobic capacity on the MetS. In a cross-sectional study, persons with SCI (N = 223; time since injury of ≥10 years) were tested. The individual components of the MetS were assessed together with the physical activity measured by the Physical Activity Scale for Individuals with Physical Disabilities (PASIPD), while peak aerobic capacity was tested during a graded wheelchair exercise test on a treadmill. Thirty-nine percent of the participants had MetS. In a multivariate logistic regression analyses and after performing a backward regression analysis, only age and education were significant determinants of the MetS. A 10-year increase in age leads to a 1.5 times more chance to have the MetS. Furthermore, people with a low education will multiply the relative risk of MetS compared with people with high education by almost 2. With and without correcting for confounders, no significant relationship was found between PASIPD or peak aerobic capacity and the MetS. It can be concluded that the prevalence of the MetS is high (39%) in people with a long-standing SCI but is comparable to the general Dutch population. Older people and those with a lower education level are most at risk for the MetS. Physical activity and peak aerobic fitness were not related to the MetS in this group with a long-standing SCI.

  2. Neuronal activity in the isolated mouse spinal cord during spontaneous deletions in fictive locomotion: insights into locomotor central pattern generator organization

    Zhong, Guisheng; Shevtsova, Natalia A; Rybak, Ilya A; Harris-Warrick, Ronald M

    2012-01-01

    We explored the organization of the spinal central pattern generator (CPG) for locomotion by analysing the activity of spinal interneurons and motoneurons during spontaneous deletions occurring during fictive locomotion in the isolated neonatal mouse spinal cord, following earlier work on locomotor deletions in the cat. In the isolated mouse spinal cord, most spontaneous deletions were non-resetting, with rhythmic activity resuming after an integer number of cycles. Flexor and extensor deletions showed marked asymmetry: flexor deletions were accompanied by sustained ipsilateral extensor activity, whereas rhythmic flexor bursting was not perturbed during extensor deletions. Rhythmic activity on one side of the cord was not perturbed during non-resetting spontaneous deletions on the other side, and these deletions could occur with no input from the other side of the cord. These results suggest that the locomotor CPG has a two-level organization with rhythm-generating (RG) and pattern-forming (PF) networks, in which only the flexor RG network is intrinsically rhythmic. To further explore the neuronal organization of the CPG, we monitored activity of motoneurons and selected identified interneurons during spontaneous non-resetting deletions. Motoneurons lost rhythmic synaptic drive during ipsilateral deletions. Flexor-related commissural interneurons continued to fire rhythmically during non-resetting ipsilateral flexor deletions. Deletion analysis revealed two classes of rhythmic V2a interneurons. Type I V2a interneurons retained rhythmic synaptic drive and firing during ipsilateral motor deletions, while type II V2a interneurons lost rhythmic synaptic input and fell silent during deletions. This suggests that the type I neurons are components of the RG, whereas the type II neurons are components of the PF network. We propose a computational model of the spinal locomotor CPG that reproduces our experimental results. The results may provide novel insights into the

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

    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.

  4. Cushing's Syndrome and Hypothalamic-Pituitary-Adrenal Axis Hyperactivity in Chronic Central Serous Chorioretinopathy.

    van Haalen, Femke M; van Dijk, Elon H C; Dekkers, Olaf M; Bizino, Maurice B; Dijkman, Greet; Biermasz, Nienke R; Boon, Camiel J F; Pereira, Alberto M

    2018-01-01

    Central serous chorioretinopathy (CSC), a specific form of macular degeneration, has been reported as presenting manifestation of Cushing's syndrome. Furthermore, CSC has been associated with both exogenous hypercortisolism and endogenous Cushing's syndrome. It is important to know whether CSC patients should be screened for Cushing's syndrome. Although hypothalamic-pituitary-adrenal (HPA) axis hyperactivity in CSC has been suggested, no detailed evaluation of the HPA axis has been performed in a large cohort of CSC patients. This study aimed to investigate whether Cushing's syndrome prevalence is increased among chronic CSC (cCSC) patients and whether detailed endocrinological phenotyping indicates hyperactivity of the HPA axis. Cross-sectional study. 86 cCSC patients and 24 controls. Prevalence of Cushing's syndrome, HPA axis activity. None of the cCSC patients met the clinical or biochemical criteria of Cushing's syndrome. However, compared to controls, HPA axis activity was increased in cCSC patients, reflected by higher 24 h urinary free cortisol, and accompanying higher waist circumference and diastolic blood pressure, whereas circadian cortisol rhythm and feedback were not different. Chronic CSC patients did not report more stress or stress-related problems on questionnaires. No case of Cushing's syndrome was revealed in a large cohort of cCSC patients. Therefore, we advise against screening for Cushing's syndrome in CSC patients, unless additional clinical features are present. However, our results indicate that cCSC is associated with hyperactivity of the HPA axis, albeit not accompanied with perception of more psychosocial stress.

  5. Capsaicin 8% Patch for Central and Peripheral Neuropathic Pain of Persons with Incomplete Spinal Cord Injury: Two Case Reports.

    Trbovich, Michelle; Yang, Huiqing

    2015-08-01

    Neuropathic pain after spinal cord injury is common and often refractory to standard treatments. The capsaicin 8% patch is a Food and Drug Administration-approved treatment of neuropathic pain in postherpetic neuralgia and has demonstrated significant efficacy in human immunodeficiency virus-autonomic neuropathy. The patch defunctionalizes transient receptor potential vanilloid 1 receptors, impairing cutaneous nociceptors for a prolonged period (i.e., 8-12 wks) with no systemic side effects. A retrospective review was conducted on the effects of the patch in two patients with spinal cord injury and neuropathic pain refractory to standard treatments. Two weeks after application, both patients reported complete pain relief. Average onset of relief of 4 days and average duration of relief of 197 days, requiring only one to four applications per year, paralleled findings reported in postherpetic neuralgia and human immunodeficiency virus-autonomic neuropathy trials. Upregulation of capsaicin-sensitive transient receptor potential vanilloid 1 receptors after spinal cord injury has been reported. The capsaicin 8% patch is a promising therapeutic agent for neuropathic pain in spinal cord injury.

  6. Spinal Cord Stimulation

    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...

  7. Nociceptive transmission and modulation via P2X receptors in central pain syndrome.

    Kuan, Yung-Hui; Shyu, Bai-Chuang

    2016-05-26

    Painful sensations are some of the most frequent complaints of patients who are admitted to local medical clinics. Persistent pain varies according to its causes, often resulting from local tissue damage or inflammation. Central somatosensory pathway lesions that are not adequately relieved can consequently cause central pain syndrome or central neuropathic pain. Research on the molecular mechanisms that underlie this pathogenesis is important for treating such pain. To date, evidence suggests the involvement of ion channels, including adenosine triphosphate (ATP)-gated cation channel P2X receptors, in central nervous system pain transmission and persistent modulation upon and following the occurrence of neuropathic pain. Several P2X receptor subtypes, including P2X2, P2X3, P2X4, and P2X7, have been shown to play diverse roles in the pathogenesis of central pain including the mediation of fast transmission in the peripheral nervous system and modulation of neuronal activity in the central nervous system. This review article highlights the role of the P2X family of ATP receptors in the pathogenesis of central neuropathic pain and pain transmission. We discuss basic research that may be translated to clinical application, suggesting that P2X receptors may be treatment targets for central pain syndrome.

  8. Theory of Mind and Central Coherence in Adults with High-Functioning Autism or Asperger Syndrome

    Beaumont, Renae; Newcombe, Peter

    2006-01-01

    The study investigated theory of mind and central coherence abilities in adults with high-functioning autism (HFA) or Asperger syndrome (AS) using naturalistic tasks. Twenty adults with HFA/AS correctly answered significantly fewer theory of mind questions than 20 controls on a forced-choice response task. On a narrative task, there were no…

  9. Combined central retinal artery and vein occlusion in Churg-Strauss syndrome

    Hamann, Steffen; Johansen, Sven; Hamann, Steffen Ellitsgaard

    2006-01-01

    PURPOSE: To describe a rare case of Churg-Strauss syndrome presenting with severe visual loss due to a combined central retinal vein and artery occlusion. METHODS: A 42-year old man with a medical history of asthma and blood hypereosinophilia developed a sudden loss of vision in his right eye. We...... and dilated and tortuous veins. The diagnosis was confirmed by a fluorescein angiogram showing absence of retinal filling and normal choroidal filling. Churg-Strauss syndrome was diagnosed based on the necessary presence of four of six criteria for the disease proposed by the American College of Rheumatology...... the vascular occlusion and experienced no visual improvement. CONCLUSION: Combined central retinal artery and vein occlusion can occur in Churg-Strauss syndrome. We suggest that regional vasculitis may be the pathological mechanism underlying the vascular occlusions observed in our case. The condition carries...

  10. Gross and fine motor skills of children with Hurler syndrome (MPS-IH) post umbilical cord blood transplantation: a case series report.

    Dusing, Stacey C; Rosenberg, Angela; Hiemenz, Jennifer R; Piner, Shelley; Escolar, Maria

    2005-01-01

    Recent advancements in medical treatment of Hurler syndrome have resulted in longer life expectancies and a greater need for therapeutic services. The purpose of this case series is to provide recommendations for assessing children with Hurler syndrome after umbilical cord blood transplant (UCBT). CLINICAL DESCRIPTIONS: Two children with Hurler syndrome were seen for longitudinal assessments following an UCBT for Hurler syndrome. The raw scores and percentage of fine and gross motor items each child completed on the Motor Scale of the Bayley Scales of Infant Development II (BSID-II) were reviewed. Both children gained new motor skills with each successive motor assessment. Both children were able to complete a higher percentage of fine motor skills than gross motor skills in the most advanced item set assessed. The children presented in these two case reports both had better fine motor skills than gross motor skills, which inflated their standard scores on the BSID-II. Clinicians assessing children with Hurler syndrome should use standardized assessments that allow for differentiation of fine and gross motor skills to prevent this situation.

  11. Fixed cord

    Levy, L.M.; DiChiro, G.; DeSouza, B.; McCullough, D.C.; McVeigh, E.; Hefffez, D.

    1989-01-01

    Pulsatile longitudinal motion of the spinal cord was examined with MR phase imaging in healthy subjects and in cases involving cord tethering and compression. Asymptomatic patients with a low conus medullaris demonstrated normal cord motion. Clinical improvement was associated with improved cord motion after surgical untethering, provided permanent neurologic damage had not occurred. Decreased and unchanged cord motion was associated with unchanged neurologic deficits. In cases of normal cord motion and possible retethering versus syringomyelia, clinical improvement occurred after shunting only. MR imaging of pulsatile cord motion can be clinically useful in the evaluation of diseases restricting motion of the neuraxis

  12. Progress in study on central nervous system injuries caused by obstructive sleep apnea syndrome

    ZHAO Xiang-xiang

    2013-05-01

    Full Text Available Chronic and repetitive intermittent hypoxia and dysfunction of sleep architecture mainly contribute to obstructive sleep apnea syndrome (OSAS. More and more evidences demonstrate it is a systemic disease, which is common encountered in clinic and strongly related to the systemic lesion of central nervous system. The central nervous system complications comprise cognitive impairment, brain atrophy and the growing risk of stroke and so on. Early treatment for OSAS has a positive significance on complications of central nervous system, and even the damage can be completely reversed.

  13. Radiologists need to be aware of secondary central venous stenosis in patients with SAPHO syndrome

    Suzuki, Mizuho; Kanazawa, Hidenori; Shinozaki, Takeshi; Sugimoto, Hideharu [Jichi Medical University, Department of Radiology, Shimotsuke, Tochigi (Japan)

    2017-11-15

    We aimed to define central venous stenosis (CVS) caused by sternocostoclavicular hyperostosis as a feature of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome on routine contrast-enhanced computed tomography (CT) images. The relationship between SAPHO syndrome and CVS without venous thrombosis caused by anterior chest wall compression has not been investigated. Therefore, the present study evaluated CVS in patients with SAPHO syndrome at our hospital. We retrospectively reviewed contrast-enhanced CT images of ten patients with suspected or diagnosed SAPHO syndrome between January 2007 and November 2015. The patients were assessed by contrast-enhanced CT using 16-, 64- or 128-detector row scanners. Two radiologists independently assessed the presence of CVS or obstruction and SAPHO syndrome in a retrospective review of CT images. Six of the ten patients had findings of CVS with SAPHO syndrome. The mean diameter and patency rate at the site of CVS were 1.88 mm and 27.2%, respectively. Stenosis was more significant in terms of the mean diameter of CVS sites than of stenotic sites that crossed the anteroposterior vein (p < 0.05). Radiologists who routinely assess contrast-enhanced CT images should be aware that sternocostoclavicular hyperostosis with SAPHO syndrome could cause secondary CVS. (orig.)

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

    Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae

    2007-01-01

    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

  15. Cushing’s Syndrome and Hypothalamic–Pituitary–Adrenal Axis Hyperactivity in Chronic Central Serous Chorioretinopathy

    Femke M. van Haalen

    2018-02-01

    Full Text Available ObjectiveCentral serous chorioretinopathy (CSC, a specific form of macular degeneration, has been reported as presenting manifestation of Cushing’s syndrome. Furthermore, CSC has been associated with both exogenous hypercortisolism and endogenous Cushing’s syndrome. It is important to know whether CSC patients should be screened for Cushing’s syndrome. Although hypothalamic-pituitary-adrenal (HPA axis hyperactivity in CSC has been suggested, no detailed evaluation of the HPA axis has been performed in a large cohort of CSC patients. This study aimed to investigate whether Cushing’s syndrome prevalence is increased among chronic CSC (cCSC patients and whether detailed endocrinological phenotyping indicates hyperactivity of the HPA axis.DesignCross-sectional study.Patients86 cCSC patients and 24 controls.MeasurementsPrevalence of Cushing’s syndrome, HPA axis activity.ResultsNone of the cCSC patients met the clinical or biochemical criteria of Cushing’s syndrome. However, compared to controls, HPA axis activity was increased in cCSC patients, reflected by higher 24 h urinary free cortisol, and accompanying higher waist circumference and diastolic blood pressure, whereas circadian cortisol rhythm and feedback were not different. Chronic CSC patients did not report more stress or stress-related problems on questionnaires.ConclusionNo case of Cushing’s syndrome was revealed in a large cohort of cCSC patients. Therefore, we advise against screening for Cushing’s syndrome in CSC patients, unless additional clinical features are present. However, our results indicate that cCSC is associated with hyperactivity of the HPA axis, albeit not accompanied with perception of more psychosocial stress.

  16. Central Retinal Artery Occlusion in a Patient with Metabolic Syndrome X

    Sonja Predrag Cekić

    2010-01-01

    Full Text Available Purpose: To report a case of central retinal artery occlusion (CRAO in a patient with metabolic syndrome X. Case Report: A 64 year-old-man presented with abrupt, painless, and severe loss of vision in his left eye. Indirect ophthalmoscopy disclosed signs compatible with CRAO and laboratory investigations revealed erythrocyte sedimentation rate of 74 mm/h, C-reactive protein (CRP level of 21 mg/l, hyperglycemia, hyperuricemia, hypertriglyceridemia and hypercholesterolemia. Fluorescein angiography and immunological studies excluded other systemic disorders. The patient met the full criteria of the National Cholesterol Education Program for metabolic syndrome X. Conclusion: In addition to different vascular complications such as stroke, and cardiovascular disease, metabolic syndrome X may be associated with retinal vascular occlusions.

  17. The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System.

    Ljubisavljevic, Srdjan; Prazic, Ana; Lazarevic, Miodrag; Stojanov, Dragan; Savic, Dejan; Vojinovic, Slobadan

    2017-02-01

    The association of paroxysmal hemicrania with trigeminal neuralgia (TN) has been described and called paroxysmal hemicrania-tic syndrome (PH-tic). We report the case of a patient diagnosed as having chronic PH-tic (CPH-tic) syndrome as a clinically isolated syndrome of the central nervous system (CNS) (CIS).A forty year old woman was admitted to our hospital suffering from right facial pain for the last 2 years. The attacks were paroxysmal, neuralgiform, consisting of throb-like sensations, which developed spontaneously or were triggered by different stimuli in right facial (maxilar and mandibular) areas. Parallel with those, she felt a throbbing orbital and frontal pain with homolateral autonomic symptoms such as conjunctival injection, lacrimation, and the feeling that the ear on the same side was full. This pain lasted most often between 15 and 20 minutes. Beyond hemifacial hypoesthesia in the region of right maxilar and mandibular nerve, the other neurological finding was normal. Magnetic resonance imaging (MRI) study showed a T2-weighted multiple hyperintense paraventricular lesion and hyperintense lesion in the right trigeminal main sensory nucleus and root inlet, all of them being hypointense on T1-weighted image. All of these lesions were hypointense in gadolinium-enhanced T1-weighted images. Neurophysiological studies of trigeminal nerve (somatosensory evoked potentials and blink reflex) correlated with MRI described lesions. The patient's pain bouts were improved immediately after treatment with indomethacin, and were completely relieved with lamotrigine for a longer period. According to the actual McDonald's criteria, clinical state was defined as CIS which was clinically presented by CPH-tic syndrome.Even though it is a clinical rarity and its etiology is usually idiopathic, CPH-tic syndrome can also be symptomatic. When dealing with symptomatic cases, like the one described here, when causal therapy is not possible due to the nature of the primary

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

    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 associ......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...... in relation to metabolic syndrome as well as BMI, waist circumference, lipoproteins, blood pressure, and fasting glucose at the age of 31 years. Linear regression models and generalized linear regression models with log link were used. RESULTS: Throughout preschool ages, weight and BMI were significantly...

  19. A risk factor analysis of outcomes after unrelated cord blood transplantation for children with Wiskott-Aldrich syndrome

    Shekhovtsova, Zhanna; Bonfim, Carmem M.; Ruggeri, Annalisa; Nichele, Samantha; Page, Kristin M.; Alseraihy, Amal; Barriga, Francisco; de Toledo Codina, José Sánchez; Veys, Paul; Boelens, Jaap Jan; Mellgren, Karin; Bittencourt, Henrique; O’Brien, Tracey; Shaw, Peter J.; Chybicka, Alicja; Volt, Fernanda; Giannotti, Federica; Gluckman, Eliane; Kurtzberg, Joanne; Gennery, Andrew R.; Rocha, Vanderson; Eurocord, Cord Blood Committee of Cellular Therapy and Immunobiology Working Party of the EBMT, Federal University of Parana, Duke University Medical Center and Inborn Errors Working Party of the EBMT

    2017-01-01

    Wiskott-Aldrich syndrome is a severe X-linked recessive immune deficiency disorder. A scoring system of Wiskott-Aldrich syndrome severity (0.5-5) distinguishes two phenotypes: X-linked thrombocytopenia and classic Wiskott-Aldrich syndrome. Hematopoietic cell transplantation is curative for

  20. Neurokinin-1 Receptor Immunoreactive Neuronal Elements in the Superficial Dorsal Horn of the Chicken Spinal Cord: With Special Reference to Their Relationship with the Tachykinin-containing Central Axon Terminals in Synaptic Glomeruli

    Sakamoto, Hiroshi; Kawate, Toyoko; Li, Yongnan; Atsumi, Saoko

    2009-01-01

    Synaptic glomeruli that involve tachykinin-containing primary afferent central terminals are numerous in lamina II of the chicken spinal cord. Therefore, a certain amount of noxious information is likely to be modulated in these structures in chickens. In this study, we used immunohistochemistry with confocal and electron microscopy to investigate whether neurokinin-1 receptor (NK-1R)-expressing neuronal elements are in contact with the central primary afferent terminals in synaptic glomeruli of the chicken spinal cord. We also investigated which neuronal elements (axon terminals, dendrites, cell bodies) and which neurons in the spinal cord possess NK-1R, and are possibly influenced by tachykinin in the glomeruli. By confocal microscopy, NK-1R immunoreactivities were seen in a variety of neuronal cell bodies, their dendrites and smaller fibers of unknown origin. Some of the NK-1R immunoreactive profiles also expressed GABA immunoreactivities. A close association was observed between the NK-1R-immunoreactive neurons and tachykinin-immunoreactive axonal varicosities. By electron microscopy, NK-1R immunoreactivity was seen in cell bodies, conventional dendrites and vesicle-containing dendrites in laminae I and II. Among these elements, dendrites and vesicle-containing dendrites made contact with tachykinin-containing central terminals in the synaptic glomeruli. These results indicate that tachykinin-containing central terminals in the chicken spinal cord can modulate second-order neuronal elements in the synaptic glomeruli

  1. Central retinal artery occlusion in a patient with ANCA-negative Churg-Strauss syndrome

    Kumano, Yuji; Yoshida, Noriko; Fukuyama, Satoru; Miyazaki, Masanori; Enaida, Hiroshi; Matsui, Takaaki

    2012-01-01

    Ocular involvement in Churg-Strauss syndrome is infrequent. We describe the case of a 54-year-old woman with eosinophilia and involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for Churg-Strauss syndrome. The patient presented with acute, painless vision loss in her right eye. Central retinal artery occlusion (CRAO) without accompanying retinal vasculitis was diagnosed by angiographic findings and funduscopic findings of retinal whitening with a cherry-red spot. Although her antineutrophil cytoplasmic antibody (ANCA) status was negative, CRAO was thought to be an ocular manifestation of Churg-Strauss syndrome, and appropriate treatment was planned. She was treated with high-dose corticosteroids and anticoagulant therapy. Her macular edema improved, but visual recovery was poor. Specific therapy to alter inflammation, blood coagulation, and rheology reportedly plays an important role in ANCA-positive patients with Churg-Strauss syndrome who develop CRAO. Regardless of ANCA status, high-dose corticosteroids should be considered for CRAO in patients with Churg-Strauss syndrome, as discussed in this case. PMID:22927731

  2. Annular and central heavy pigment deposition on the posterior lens capsule in the pigment dispersion syndrome: pigment deposition on the posterior lens capsule in the pigment dispersion syndrome.

    Turgut, Burak; Türkçüoğlu, Peykan; Deniz, Nurettin; Catak, Onur

    2008-12-01

    To report annular and central heavy pigment deposition on the posterior lens capsule in a case of pigment dispersion syndrome. Case report. A 36-year-old female with bilateral pigment dispersion syndrome presented with progressive decrease in visual acuity in the right eye over the past 1-2 years. Clinical examination revealed the typical findings of pigment dispersion syndrome including bilateral Krunkenberg spindles, iris transillumination defects, and dense trabecular meshwork pigmentation. Remarkably, annular and central dense pigmentation of the posterior lens capsule was noted in the right eye. Annular pigment deposition on the posterior lens capsule may be a rare finding associated with pigment dispersion syndrome. Such a finding suggests that there may be aqueous flow into the retrolental space in some patients with this condition. The way of central pigmentation is the entrance of aqueous to Berger's space. In our case, it is probable that spontaneous detachment of the anterior hyaloid membrane aided this entrance.

  3. Dorsal Root Ganglion Stimulation for Complex Regional Pain Syndrome (CRPS) Recurrence after Amputation for CRPS, and Failure of Conventional Spinal Cord Stimulation.

    Goebel, Andreas; Lewis, Sarah; Phillip, Rhodri; Sharma, Manohar

    2018-01-01

    Limb amputation is sometimes being performed in long-standing complex regional pain syndrome (CRPS), although little evidence is available guiding management decisions, including how CRPS recurrence should be managed. This report details the management of a young soldier with CRPS recurrence 2 years after midtibial amputation for CRPS. Conventional spinal cord stimulation did not achieve paraesthetic coverage, or pain relief in the stump, whereas L4 dorsal root ganglion stimulation achieved both coverage and initially modest pain relief, and over time, substantial pain relief. Current evidence does not support the use of amputation to improve either pain or function in CRPS. Before a decision is made, in exceptional cases, about referral for amputation, dorsal root ganglion stimulation should be considered as a potentially effective treatment, even where conventional spinal cord stimulator treatment has failed to achieve reliable paraesthetic cover. Furthermore, this treatment may provide pain relief in those patients with CRPS recurrence in the stump after amputation. © 2017 World Institute of Pain.

  4. Evaluation of spinal cord stimulation on the symptoms of anxiety and depression and pain intensity in patients with failed back surgery syndrome.

    Robb, L P; Cooney, J M; McCrory, C R

    2017-08-01

    Spinal cord stimulation (SCS) is now established as the primary treatment for failed back surgery syndrome (FBSS). Commonly, patients with chronic pain and FBSS often report symptoms of anxiety and depression resulting from this condition. These factors can modulate and amplify the pain experience, therefore, further challenging treatment success. This study examined the efficacy of SCS on alleviating the symptoms of anxiety and depression associated with chronic pain as well as pain intensity in a group of patients with FBSS. A convenience sample (n = 26) was selected for participation. Questionnaires [Hospital Anxiety and Depression Scale (HADS) and Brief Pain Inventory Short Form (BPI-SF)] were completed and examined pre and post spinal cord implant. Analysis of the data 1 year following SCS indicates that there was a statistical significant improvement in the symptoms of depression and anxiety reported as well as pain intensity in all participants (p anxiety and depression scores on the HADS were significantly lower compared to baseline (p anxiety and depression with an associated reduction in opioid consumption.

  5. DRG Spinal Cord Stimulation as Solution for Patients With Severe Pain Due to Anterior Cutaneous Nerve Entrapment Syndrome: A Case Series.

    Mol, Frédérique Mathilde Ulrike; Roumen, Rudi M H

    2018-04-01

    Anterior Cutaneous Nerve Entrapment Syndrome (ACNES) is a debilitating neuropathic pain condition. A small portion of patients do not respond to any currently available treatment modalities. These patients, often young women, might benefit from targeted spinal cord stimulation of the dorsal root ganglion (DRG). This retrospective case series describes five ACNES patients who were referred from a Dutch dedicated tertiary referral center to collaborating sites with extensive experience in DRG stimulation to be implanted with a DRG Axium System (St. Jude/Abbott, IL, USA) in the period of 2013-2016. Numeric pain rating scores at routine 6- and 12-month follow-up visits were analyzed. Three patients experienced >50% pain reduction at 12 months follow-up. Four patients experienced device-related complications, such as lead dislocation, lead breakage, pain at the battery site, and overstimulation. This case series suggests DRG spinal cord stimulation can be safe and effective for some patients with persistent pain due to ACNES. © 2017 International Neuromodulation Society.

  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

    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.

  7. Dynamic changes of central thyroid functions in the management of Cushing's syndrome.

    Dogansen, Sema Ciftci; Yalin, Gulsah Yenidunya; Canbaz, Bulent; Tanrikulu, Seher; Yarman, Sema

    2018-01-01

    The aim of this study was to determine the frequency of central thyroid dysfunctions in Cushing's syndrome (CS). We also aimed to evaluate the frequency of hyperthyroidism due to the syndrome of the inappropriate secretion of TSH (SITSH), which was recently defined in patients with insufficient hydrocortisone replacement after surgery. We evaluated thyroid functions (TSH and free thyroxine [fT4]) at the time of diagnosis, during the hypothalamo-pituitary-adrenal axis recovery, and after surgery in 35 patients with CS. The patients were separated into two groups: ACTH-dependent CS (group 1, n = 20) and ACTH-independent CS (group 2, n = 15). Patients' clinical and laboratory findings were evaluated in five visits in the outpatient clinic of the endocrinology department. The frequency of baseline suppressed TSH levels and central hypothyroidism were determined to be 37% (n = 13) and 26% (n = 9), respectively. A negative correlation was found between baseline cortisol and TSH levels (r = -0.45, p = 0.006). All patients with central hypothyroidism and suppressed TSH levels showed recovery at the first visit without levothyroxine treatment. SITSH was not detected in any of the patients during the postoperative period. No correlation was found between prednisolone replacement after surgery and TSH or fT4 levels on each visit. Suppressed TSH levels and central hypothyroidism may be detected in CS, independent of etiology. SITSH was not detected in the early postoperative period due to our adequate prednisolone replacement doses.

  8. [Spinal cord stimulation (SCS) as a treatment for the spread phenomenon related to complex regional pain syndrome type- I (CRPS-I )].

    Goto, Shinichi; Taira, Takaomi; Hori, Tomokatsu

    2009-09-01

    The authors describe an experience of spinal cord stimulation (SCS) in a 30-year-old woman who developed complex regional pain syndrome type-I (CRPS-I) with spread phenomenon. She had received lumbar SCS under a diagnosis of CRPS-I in her left leg for 8 years. She had refractory pain in her right arm for the recent two years. There was no new lesion explaining her refractory pain on physical or radiological examination. Thus, the pain in her right upper arm was considered as spread phenomenon of CRPS-I. Test stimulation with cervical epidural spinal electrode showed good results and the pulse generator was implanted. It is suggested that the symptom of CRPS-I involving spread phenomenon was possibly due to a cortical reorganization. But a certain effect of SCS may be contributing to the favorable results of test stimulation for the treatment of CRPS-I with spread phenomenon in this case.

  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

    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...... in this severe phenotype of Chediak-Higashi syndrome was probably induced by rotaviral infection. Interestingly, the intracellular perforin content in CD8 T cells seems to correlate to the immune activation state of the patient. Late separation of the umbilical cord in concordance with clinical symptoms should...

  10. Banking on cord blood stem cells.

    Sullivan, Michael J

    2008-07-01

    Umbilical cord blood gifted to non-profit public cord blood banks is now routinely used as an alternative source of haematopoietic stem cells for allogeneic transplantation for children and adults with cancer, bone marrow failure syndromes, haemoglobinopathies and many genetic metabolic disorders. Because of the success and outcomes of public cord banking, many companies now provide private cord banking services. However, in the absence of any published transplant evidence to support autologous and non-directed family banking, commercial cord banks currently offer a superfluous service.

  11. Back to Future Health Blueprint: The Effects of a Brief Bioenergy Economy Program on a Patient with Tethered Cord Syndrome

    Farzad Goli

    2016-12-01

    Full Text Available Background: The spinal cord congenital abnormalities may prevent normal cephalad movement of the conusmedullaris such as tethered cord. A child or even an adult with these abnormalities may develop progressiveneurological dysfunction due to traction on the cord or nerve roots. As the most problematic technicalconsideration in surgery for the release of the tethered cord is how to preserve functions of neural elementsand rebuild the dural sac to maintain normal cerebrospinal fluid (CSF circulation; the priority is to treat thecondition through less invasive methods. Bioenergy economy (BEE is an integrative healing model which triesto abstract healing modalities and integrate them into a psychosomatic health system. In contrast toreductionistic and pathology-based approach of biomedical treatment, bioenergy healing is a salutogenic,holistic and metadiagnostic approach which creates healing responses from a blueprint of healthy body.Case Report: We report the process of a bioenergy economy intervention in a 10-year-old boy withclinical signs of drop foot, urinary incontinence, urinary reflux, and low back pain who was candidate forsurgeries by neurosurgical and urological criteria. The clinical results indicated that after about two yearsof 12 healing sessions in a brief bioenergy economy package of biofield scanning, biofield attunement,and hand-on self-healing, the patient’s clinical signs remarkably improved to the extent that he returned tonormal activities of his age and followed an athletic lifestyle.Conclusion: From a biosemiotic viewpoint, it can be discussed that bioenergy economy, by focusing onenhancing the pathways of salutogenesis was effective to evoke healing response in the patient’s body.The effect of the bioenergy economy practice may be due to the healing images and intentions flowedthrough patient’s body and healer-healee biofields’ coupling and interactions.

  12. Cord Blood

    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

  13. Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

    Di Stefano, G; Celletti, C; Baron, R; Castori, M; Di Franco, M; La Cesa, S; Leone, C; Pepe, A; Cruccu, G; Truini, A; Camerota, F

    2016-09-01

    Patients with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT) commonly suffer from pain. How this hereditary connective tissue disorder causes pain remains unclear although previous studies suggested it shares similar mechanisms with neuropathic pain and fibromyalgia. In this prospective study seeking information on the mechanisms underlying pain in patients with JHS/EDS-HT, we enrolled 27 consecutive patients with this connective tissue disorder. Patients underwent a detailed clinical examination, including the neuropathic pain questionnaire DN4 and the fibromyalgia rapid screening tool. As quantitative sensory testing methods, we included thermal-pain perceptive thresholds and the wind-up ratio and recorded a standard nerve conduction study to assess non-nociceptive fibres and laser-evoked potentials, assessing nociceptive fibres. Clinical examination and diagnostic tests disclosed no somatosensory nervous system damage. Conversely, most patients suffered from widespread pain, the fibromyalgia rapid screening tool elicited positive findings, and quantitative sensory testing showed lowered cold and heat pain thresholds and an increased wind-up ratio. While the lack of somatosensory nervous system damage is incompatible with neuropathic pain as the mechanism underlying pain in JHS/EDS-HT, the lowered cold and heat pain thresholds and increased wind-up ratio imply that pain in JHS/EDS-HT might arise through central sensitization. Hence, this connective tissue disorder and fibromyalgia share similar pain mechanisms. WHAT DOES THIS STUDY ADD?: In patients with JHS/EDS-HT, the persistent nociceptive input due to joint abnormalities probably triggers central sensitization in the dorsal horn neurons and causes widespread pain. © 2016 European Pain Federation - EFIC®

  14. Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome.

    Grillo, Andrea; Salvi, Paolo; Marelli, Susan; Gao, Lan; Salvi, Lucia; Faini, Andrea; Trifirò, Giuliana; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

    2017-11-07

    Marfan syndrome is characterized by aortic root dilation, beginning in childhood. Data about aortic pulsatile hemodynamics and stiffness in pediatric age are currently lacking. In 51 young patients with Marfan syndrome (12.0±3.3 years), carotid tonometry was performed for the measurement of central pulse pressure, pulse pressure amplification, and aortic stiffness (carotid-femoral pulse wave velocity). Patients underwent an echocardiogram at baseline and at 1 year follow-up and a genetic evaluation. Pathogenetic fibrillin-1 mutations were classified between "dominant negative" and "haploinsufficient." The hemodynamic parameters of patients were compared with those of 80 sex, age, blood pressure, and heart-rate matched controls. Central pulse pressure was significantly higher (38.3±12.3 versus 33.6±7.8 mm Hg; P =0.009), and pulse pressure amplification was significantly reduced in Marfan than controls (17.9±15.3% versus 32.3±17.4%; P Marfan and controls (4.98±1.00 versus 4.75±0.67 m/s). In the Marfan group, central pulse pressure and pulse pressure amplification were independently associated with aortic diameter at the sinuses of Valsalva (respectively, β=0.371, P =0.010; β=-0.271, P =0.026). No significant difference in hemodynamic parameters was found according to fibrillin-1 genotype. Patients who increased aortic Z-scores at 1-year follow-up presented a higher central pulse pressure than the remaining (42.7±14.2 versus 32.3±5.9 mm Hg; P =0.004). Central pulse pressure and pulse pressure amplification were impaired in pediatric Marfan syndrome, and associated with aortic root diameters, whereas aortic pulse wave velocity was similar to that of a general pediatric population. An increased central pulse pressure was present among patients whose aortic dilatation worsened at 1-year follow-up. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  15. Prevalence of metabolic syndrome in Central America: a cross-sectional population-based study

    Roy A. Wong-McClure

    2015-09-01

    Full Text Available OBJECTIVE: To report the prevalence of metabolic syndrome (MetS as found by the Central American Diabetes Initiative (CAMDI study for five major Central American populations: Belize (national; Costa Rica (San José; Guatemala (Guatemala City; Honduras (Tegucigalpa; and Nicaragua (Managua. METHODS: Study data on 6 185 adults aged 20 years or older with anthropometric and laboratory determination of MetS from population-based surveys were analyzed. Overall, the survey response rate was 82.0%. MetS prevalence was determined according to criteria from the Adult Treatment Panel III of the National Cholesterol Education Program. The study's protocol was reviewed and approved by the bioethical committee of each country studied. RESULTS: The overall standardized prevalence of MetS in the Central American region was 30.3% (95% confidence interval (CI: 27.1-33.4. There was wide variability by gender and work conditions, with higher prevalence among females and unpaid workers. The standardized percentage of the population free of any component of MetS was lowest in Costa Rica (9.0%; CI: 6.5-11.4 and highest in Honduras (21.1%; CI: 16.4-25.9. CONCLUSIONS: Overall prevalence of MetS in Central America is high. Strengthening surveillance of chronic diseases and establishing effective programs for preventing cardiovascular diseases might reduce the risk of MetS in Central America.

  16. Sleeping problems in mothers and fathers of patients suffering from congenital central hypoventilation syndrome.

    Paddeu, Erika Maria; Giganti, Fiorenza; Piumelli, Raffaele; De Masi, Salvatore; Filippi, Luca; Viggiano, Maria Pia; Donzelli, Gianpaolo

    2015-09-01

    Advanced medical technology has resulted in an increased survival rate of children suffering from congenital central hypoventilation syndrome. After hospitalization, these technology-dependent patients require special home care for assuring ventilator support and the monitoring of vital parameters mainly during sleep. The daily challenges associated with caring for these children can place primary caregivers under significant stress, especially at night. Our study aimed at investigating how this condition affects mothers and fathers by producing poor sleep quality, high-level diurnal sleepiness, anxiety, and depression. The study included parents of 23 subjects with congenital central hypoventilation syndrome and 23 healthy subjects. All parents filled out the Pittsburgh Sleep Quality Index (PSQI) questionnaire, Epworth Sleepiness Scale (ESS), Beck Depression Inventory (BDI-II), and Beck Anxiety Inventory (BAI). A comparison between the two groups showed that parents of patients had poorer sleep quality, greater sleepiness, and higher BDI-II scores compared to that of parents of healthy subjects (respectively, PSQI score 6.5 vs 3.8, ESS score 6.2 vs 4.3, BDI-II score 8.4 vs 5.7). Specifically, mothers of patients showed poorer sleep quality and higher BDI-II scores compared to that of mothers of controls (respectively, PSQI score 7.5 vs 3.8, BDI-II score 9.3 vs 5.9), whereas fathers of patients showed greater levels of sleepiness with respect to fathers of healthy children (respectively, ESS score 6.8 vs 4.0). These differences emerged in parents of younger children. Congenital central hypoventilation syndrome impacts the family with different consequences for mothers and fathers. Indeed, while the patients' sleep is safeguarded, sleeping problems may occur in primary caregivers often associated with other psychological disorders. Specifically, this disease affects sleep quality and mood in the mothers and sleepiness levels in the fathers.

  17. γ-diketone central neuropathy: quantitative morphometric analysis of axons in rat spinal cord white matter regions and nerve roots

    LoPachin, Richard M.; Jortner, Bernard S.; Reid, Maria L.; Das, Soma

    2003-01-01

    A quantitative analytical method was used to measure myelinated axon morphometric parameters (e.g., axon area, ratio of axon area/fiber area, and index of circularity) in rat nervous tissue during intoxication with 2,5-hexanedione (HD). Parameters were assessed in nerve roots (dorsal and ventral) and in ascending (gracile fasciculus and spinocerebellar tract) and descending (corticospinal and rubrospinal tracts) spinal cord white matter tracts (L4-L5) of rats intoxicated with HD at two different daily dose-rates (175 or 400 mg HD/kg/day, gavage). For each dose-rate, tissue was sampled at four neurological endpoints: unaffected, slight, moderate, and severe toxicity, as determined by gait analysis and measurements of grip strength. Results indicate that, regardless of the HD dose-rate, axon atrophy (reduced axon area) was a widespread, abundant effect that developed in concert with neurological deficits. The atrophy response occurred contemporaneously in both ascending and descending spinal tracts, which suggests that loss of caliber developed simultaneously along the proximodistal axon axis. In contrast, swollen axons were a numerically small component and were present in nerve roots and spinal tracts only during subchronic intoxication at the lower HD dose-rate (i.e., 175 mg/kg/day). Intoxication at the higher dose-rate (400 mg/kg/day) produced neurological deficits in the absence of axonal swellings. These observations in conjunction with our previous studies of HD-induced peripheral neuropathy (Toxicol. Appl. Pharmacol. 135 (1995) 58; and Toxicol. Appl. Pharmacol. 165 (2000) 127) indicate that axon atrophy, and not axonal swelling, is a primary neuropathic phenomenon

  18. Thoracolumbar spinal neurenteric cyst with tethered cord syndrome and extreme cervical lordosis in a child: A case report and literature review.

    Lan, Zhi Gang; Richard, Seidu A; Lei, Chuanfen; Huang, Siqing

    2018-04-01

    Neurenteric cysts, are rare benign tumors of the central nervous system that are mostly located in the spinal cord and predominantly seen in male children although adult form of the disorder also occurs. The etiology and treatment of this disorder is still a matter of debate. Our case further throws more light on the pathogenesis and treatment of this disorder. A 4-year-old boy presented with 5-month history of cervical lordosis and bilateral lower extremity pain that progressed to his abdomen and upper body. The pain was general, recurrent, non-persistent and progressive in nature with no paralysis. The pain was aggravated by trunk stretching and relieved when he assumed opisthotonos position so he preferred sleeping in this position at night. Magnetic resonance imaging (MRI) revealed a cystic lesion at the thoracolumbar spine with tethering of spinal cord and cervical lordosis. He was operated on successfully and the cervical lordosis and pain resolved. The child recovered well with no tumor recurrence and massive improvement of his life. The gold standard treatment for this disorder is surgery although the precise surgical approach is still a matter of debate. We are of the view that surgical approach should be individualized and aim at total excision of the cyst.

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

    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. © 2016 Wiley Periodicals, Inc.

  20. Neurally adjusted ventilatory assist (NAVA) mode as an adjunct diagnostic tool in congenital central hypoventilation syndrome

    Rahmani, A.; Rehman, N.U.; Chedid, F.

    2013-01-01

    A full term female newborn was admitted to the neonatal intensive care unit (NICU) for continuous observation of apnea. Infant was noted to have apnea while asleep requiring intubation and mechanical ventilation. A video EEG was performed which demonstrated normal awake background without any seizure activity. Neurally adjusted ventilatory assist (NAVA) demonstrated the absence of electrical activity of the diaphragm (Edi) when the patient was in quiet phase of sleep. This finding on NAVA monitor raised the suspicion of central hypoventilation syndrome (CCHS) which was confirmed by genetic identification of the PHOX2B mutation. (author)

  1. Neurally adjusted ventilatory assist (NAVA) mode as an adjunct diagnostic tool in congenital central hypoventilation syndrome.

    Rahmani, Aiman; Ur Rehman, Naveed; Chedid, Fares

    2013-02-01

    A full term female newborn was admitted to the neonatal intensive care unit (NICU) for continuous observation of apnea. Infant was noted to have apnea while asleep requiring intubation and mechanical ventilation. A video EEG was performed which demonstrated normal awake background without any seizure activity. Neurally adjusted ventilatory assist (NAVA) demonstrated the absence of electrical activity of the diaphragm (Edi) when the patient was in quiet phase of sleep. This finding on NAVA monitor raised the suspicion of central hypoventilation syndrome (CCHS) which was confirmed by genetic identification of the PHOX2B mutation.

  2. Chronic Central Serous Chorioretinopathy in a Patient with Pigment Dispersion Syndrome: A Possible Correlation.

    Kourkoutas, Dimitrios; Tsakonas, George; Karamaounas, Aristotelis; Karamaounas, Nikolaos

    2017-01-01

    Chronic central serous chorioretinopathy (CSCR) is a progressive chorioretinopathy with widespread atrophic RPE abnormalities and serous retinal detachments (SRDs) present for 6 months or longer. We report a case of CSCR in a 38-year-old patient with Pigment Dispersion Syndrome (PDS). In the presented case of CSCR, the chronic course of the disease may in part be associated with an underlying generalized degenerative dysfunction of the pigmented cells of the eye on grounds of PDS. We suggest that a chronic course of disease may be suspected in the setting of CSCR with concurrent RPE pathology, such as what is found in PDS.

  3. Chronic Central Serous Chorioretinopathy in a Patient with Pigment Dispersion Syndrome: A Possible Correlation

    Dimitrios Kourkoutas

    2017-01-01

    Full Text Available Chronic central serous chorioretinopathy (CSCR is a progressive chorioretinopathy with widespread atrophic RPE abnormalities and serous retinal detachments (SRDs present for 6 months or longer. We report a case of CSCR in a 38-year-old patient with Pigment Dispersion Syndrome (PDS. In the presented case of CSCR, the chronic course of the disease may in part be associated with an underlying generalized degenerative dysfunction of the pigmented cells of the eye on grounds of PDS. We suggest that a chronic course of disease may be suspected in the setting of CSCR with concurrent RPE pathology, such as what is found in PDS.

  4. Central nervous system

    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.

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

    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

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

    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. Copyright © 2014 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

  7. Pericytes Make Spinal Cord Breathless after Injury.

    Almeida, Viviani M; Paiva, Ana E; Sena, Isadora F G; Mintz, Akiva; Magno, Luiz Alexandre V; Birbrair, Alexander

    2017-09-01

    Traumatic spinal cord injury is a devastating condition that leads to significant neurological deficits and reduced quality of life. Therapeutic interventions after spinal cord lesions are designed to address multiple aspects of the secondary damage. However, the lack of detailed knowledge about the cellular and molecular changes that occur after spinal cord injury restricts the design of effective treatments. Li and colleagues using a rat model of spinal cord injury and in vivo microscopy reveal that pericytes play a key role in the regulation of capillary tone and blood flow in the spinal cord below the site of the lesion. Strikingly, inhibition of specific proteins expressed by pericytes after spinal cord injury diminished hypoxia and improved motor function and locomotion of the injured rats. This work highlights a novel central cellular population that might be pharmacologically targeted in patients with spinal cord trauma. The emerging knowledge from this research may provide new approaches for the treatment of spinal cord injury.

  8. Bing-Neel Syndrome: Illustrative Cases and Comprehensive Review of the Literature

    Varettoni, Marzia; Defrancesco, Irene; Diamanti, Luca; Marchioni, Enrico; Farina, Lisa Maria; Pichiecchio, Anna

    2017-01-01

    The Bing-Neel syndrome is a rare neurological complication of Waldenström’s Macroglobulinemia which results from a direct involvement of central nervous system by malignant lymphoplasmacytic cells. The clinical suspicion of Bing-Neel syndrome may be difficult because neurologic symptoms are heterogeneous, non specific and sometimes underhand. A definitive diagnosis of Bing-Neel syndrome can be confidently made using brain and spinal cord magnetic resonance imaging as well as histopathology an...

  9. Therapeutic effects of human umbilical cord blood-derived mesenchymal stem cells on the radiation-induced GI syndrome

    Shim, Se Hwan; Jang, Won Suk; Lee, Sun Joo; Park, Eun Young; Kim, Youn Joo; Jin, Sung Ho; Park, Sun Hoo; Lee, Seung Sook [Korea Institute of Radiological and Medical Sciences, Seoul (Korea, Republic of)

    2011-05-15

    The gastrointestinal (GI) tract is one of the most radiosensitive organ systems in the body. Radiation-induced GI injury is described as destruction of crypt cell, decrease in villous height and number, ulceration, and necrosis of intestinal epithelium. Studies show that mesenchymal stem cells (MSCs) treatment may be useful in the repair or regeneration of damaged organs including bone, cartilage, or myocardium. MSCs from umbilical cord blood (UCB) have many advantages because of the immature nature of newborn cells compared to bone marrow derived MSCs. Moreover, UCB-MSCs provide no ethical barriers for basic studies and clinical applications. In this study, we explore the regeneration capability of human UCB-MSCs after radiation-induced GI injury

  10. Spinal cord compression caused by anaplastic large cell lymphoma in an HIV infected individual

    Kumar Susheel

    2010-01-01

    Full Text Available Lymphomas occur with an increased frequency in patients with Human Immunodeficiency Virus (HIV infection. These are usually high-grade immunoblastic lymphomas and primary central nervous system lymphomas. Anaplastic large cell lymphoma (ALCL is a distinct type of non-Hodgkin′s lymphoma. It is uncommon in HIV infected individuals. We describe here an uncommon presentation of this relatively rare lymphoma in the form of spinal cord compression syndrome in a young HIV infected individual.

  11. Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report

    Sajish Jacob

    2014-01-01

    Full Text Available Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord.

  12. Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report

    Jacob, Sajish; Nikolic, Dejan; Gundogdu, Betul; Ong, Shirley

    2014-01-01

    Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord. PMID:24716023

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

    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.

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

    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.

  15. Four-dimensional real-time sonographically guided cauterization of the umbilical cord in a case of twin-twin transfusion syndrome.

    Timor-Tritsch, Ilan E; Rebarber, Andrei; MacKenzie, Andrew; Caglione, Christopher F; Young, Bruce K

    2003-07-01

    In the past decade, three-dimensional (3D) sonographic technology has matured from a static imaging modality to near-real-time imaging. One of the more notable improvements in this technology has been the speed with which the imaged volume is acquired and displayed. This has enabled the birth of the near-real-time or four-dimensional (4D) sonographic concept. Using the 4D feature of the current 3D sonography machines allows us to follow moving structures, such as fetal motion, in almost real time. Shortly after the emergence of 3D and 4D technology as a clinical imaging tool, its use in guiding needles into structures was explored by other investigators. We present a case in which we used the 4D feature of our sonographic equipment to follow the course and motion of an instrument inserted into the uterus to occlude the umbilical cord of a fetus in a case of twin-twin transfusion syndrome.

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

    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.

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

    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.

  18. Expanding the phenotype of congenital central hypoventilation syndrome impacts management decisions.

    Byers, Heather M; Chen, Maida; Gelfand, Andrew S; Ong, Bruce; Jendras, Marisa; Glass, Ian A

    2018-04-25

    Congenital central hypoventilation syndrome (CCHS) is a neurocristopathy caused by pathogenic heterozygous variants in the gene paired-like homeobox 2b (PHOX2B). It is characterized by severe infantile alveolar hypoventilation. Individuals may also have diffuse autonomic nervous system dysfunction, Hirschsprung disease and neural crest tumors. We report three individuals with CCHS due to an 8-base pair duplication in PHOX2B; c.691_698dupGGCCCGGG (p.Gly234Alafs*78) with a predominant enteral and neural crest phenotype and a relatively mild respiratory phenotype. The attenuated respiratory phenotype reported here and elsewhere suggests an emergent genotype:phenotype correlation which challenges the current paradigm of invoking mechanical ventilation for all infants diagnosed with CCHS. Best treatment requires careful clinical judgment and ideally the assistance of a care team with expertise in CCHS. © 2018 Wiley Periodicals, Inc.

  19. A case of Churg-Strauss syndrome and central retinal artery occlusion with good visual recovery

    Yuki Kamata

    2013-01-01

    Full Text Available Here we report a case of Churg-Strauss syndrome (CSS and central retinal artery occlusion (CRAO, with good visual recovery. A 58-year-old Japanese man with CSS experienced acute painless loss of vision in his right eye. CRAO was diagnosed by fundoscopic findings (retinal whitening with a cherry-red spot. Steroid pulse therapy (methylprednisolone at 1 g daily for 3 days followed by combined treatment with prednisolone (30 mg/day and cyclophosphamide (150 mg/day was administered; his visual acuity recovered to 20/30 in 1 month, and no recurrence has occurred for 1 year. Steroid pulse therapy may be effective for CRAO in CSS patients.

  20. Carotid cavernous fistula with central retinal artery occlusion and Terson syndrome after mid-facial trauma

    Karna, Satya

    2017-05-01

    Full Text Available Objectives: To report a rare occurrence combination of central retinal artery occlusion (CRAO and Terson syndrome in a Barrow’s type A carotid cavernous fistula (CCF patient.Methods: Observational case report.Results: A twenty-year-old male patient with a history of road traffic accident presented with periorbital swelling and redness in the left eye. Examination revealed a CRAO with intraretinal and preretinal hemorrhages. On imaging, type A CCF and subarachnoid hemorrhage were detected. He underwent embolization of the fistula for cosmetic blemish. The possible mechanisms and clinical implications are discussed. Conclusion: Patients with a head injury can have serious ocular damage. Posterior segment manifestations of CCFs are varied and at times can occur in various rare combinations, making it challenging. Early recognition of these rare manifestations and a multi-disciplinary approach are needed in patients with head trauma.

  1. Paresthesia-Free High-Density Spinal Cord Stimulation for Postlaminectomy Syndrome in a Prescreened Population: A Prospective Case Series.

    Sweet, Jennifer; Badjatiya, Anish; Tan, Daniel; Miller, Jonathan

    2016-04-01

    Spinal cord stimulation (SCS) traditionally is thought to require paresthesia, but there is evidence that paresthesia-free stimulation using high-density (HD) parameters might also be effective. The purpose of this study is to evaluate relative effectiveness of conventional, subthreshold HD, and sham stimulation on pain intensity and quality of life. Fifteen patients with response to conventional stimulation (60 Hz/350 μsec) were screened with a one-week trial of subthreshold HD (1200 Hz/200 μsec/amplitude 90% paresthesia threshold) and enrolled if there was at least 50% reduction on visual analog scale (VAS) for pain. Subjects were randomized into two groups and treated with four two-week periods of conventional, subthreshold HD, and sham stimulation in a randomized crossover design. Four of 15 patients responded to subthreshold HD stimulation. Mean VAS during conventional, subthreshold HD, and sham stimulation was 5.32 ± 0.63, 2.29 ± 0.41, and 6.31 ± 1.22, respectively. There was a significant difference in pain scores during the blinded crossover study of subthreshold HD vs. sham stimulation (p Paresthesia are not necessary for pain relief using commercially available SCS devices, and may actually increase attention to pain. Subthreshold HD SCS represents a viable alternative to conventional stimulation among patients who are confirmed to have a clinical response to it. © 2015 International Neuromodulation Society.

  2. Ordovas-Oxidized LDL is associated with metabolic syndrome traits independently of central obesity and insulin resistance

    This study assesses whether oxidative stress, using oxidized LDL (ox-LDL) as a proxy, is associated with metabolic syndrome (MS), whether ox-LDL mediates the association between central obesity and MS, and whether insulin resistance mediates the association between ox-LDL and MS. We examined baselin...

  3. Spinal Cord Injury 101

    Full Text Available ... Cord Injury What is a Spinal Cord Injury Levels of Injury and What They Mean Animated Spinal ... Cord Injury What is a Spinal Cord Injury Levels of Injury and What They Mean Animated Spinal ...

  4. Spinal Cord Injury 101

    Full Text Available menu Understanding Spinal Cord Injury What is a Spinal Cord Injury Levels of Injury and What They Mean Animated Spinal Cord Injury Chart Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal ...

  5. Spinal Cord Injury 101

    Full Text Available ... Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal Cord Injury Rehabilitation Pediatric Spinal ... Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal Cord Injury Rehabilitation Pediatric Spinal ...

  6. Spinal Cord Injury 101

    Full Text Available ... Cord Injury Rehabilitation Pediatric Spinal Cord Injuries Video Library SCI Medical Experts People Living with SCI Personal ... Cord Injury Rehabilitation Pediatric Spinal Cord Injuries Video Library SCI Medical Experts People Living with SCI Personal ...

  7. Spinal Cord Injury 101

    Full Text Available ... Animated Spinal Cord Injury Chart Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal ... Animated Spinal Cord Injury Chart Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal ...

  8. Spinal Cord Injury 101

    Full Text Available ... Injury Chart Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal Cord Injury Rehabilitation ... Injury Chart Spinal Cord Injury Facts and Figures Care and Treatment After SCI Spinal Cord Injury Rehabilitation ...

  9. Cardiometabolic Syndrome in People With Spinal Cord Injury/Disease: Guideline-Derived and Nonguideline Risk Components in a Pooled Sample.

    Nash, Mark S; Tractenberg, Rochelle E; Mendez, Armando J; David, Maya; Ljungberg, Inger H; Tinsley, Emily A; Burns-Drecq, Patricia A; Betancourt, Luisa F; Groah, Suzanne L

    2016-10-01

    To assess cardiometabolic syndrome (CMS) risk definitions in spinal cord injury/disease (SCI/D). Cross-sectional analysis of a pooled sample. Two SCI/D academic medical and rehabilitation centers. Baseline data from subjects in 7 clinical studies were pooled; not all variables were collected in all studies; therefore, participant numbers varied from 119 to 389. The pooled sample included men (79%) and women (21%) with SCI/D >1 year at spinal cord levels spanning C3-T2 (American Spinal Injury Association Impairment Scale [AIS] grades A-D). Not applicable. We computed the prevalence of CMS using the American Heart Association/National Heart, Lung, and Blood Institute guideline (CMS diagnosis as sum of risks ≥3 method) for the following risk components: overweight/obesity, insulin resistance, hypertension, and dyslipidemia. We compared this prevalence with the risk calculated from 2 routinely used nonguideline CMS risk assessments: (1) key cut scores identifying insulin resistance derived from the homeostatic model 2 (HOMA2) method or quantitative insulin sensitivity check index (QUICKI), and (2) a cardioendocrine risk ratio based on an inflammation (C-reactive protein [CRP])-adjusted total cholesterol/high-density lipoprotein cholesterol ratio. After adjustment for multiple comparisons, injury level and AIS grade were unrelated to CMS or risk factors. Of the participants, 13% and 32.1% had CMS when using the sum of risks or HOMA2/QUICKI model, respectively. Overweight/obesity and (pre)hypertension were highly prevalent (83% and 62.1%, respectively), with risk for overweight/obesity being significantly associated with CMS diagnosis (sum of risks, χ(2)=10.105; adjusted P=.008). Insulin resistance was significantly associated with CMS when using the HOMA2/QUICKI model (χ(2)2=21.23, adjusted P<.001). Of the subjects, 76.4% were at moderate to high risk from elevated CRP, which was significantly associated with CMS determination (both methods; sum of risks, χ(2

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

    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.

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

    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.

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

    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.

  13. Long-term outcomes of spinal cord stimulation with percutaneously introduced paddle leads in the treatment of failed back surgery syndrome and lumboischialgia.

    Logé, David; Vanneste, Sven; Vancamp, Tim; Rijckaert, Dirk

    2013-01-01

    The study aims to evaluate the long-term clinical and technical efficacy of recently developed percutaneously introducible plate electrodes for spinal cord stimulation. Twenty-one patients diagnosed with failed back surgery syndrome (FBSS) or lumboischialgia were implanted with a small profile plate-type electrode. Patients were followed-up long term and were asked at baseline, after trial, and during each follow-up visit to score their pain on a visual analog scale (VAS) for pain now, worst pain last week, least pain last week, and mean pain last week. Pain location, electrophysiologic parameters, and number of reprogrammings were collected as well. Furthermore, each patient was asked if he/she would redo the procedure post trial and at each of the follow-up visits. A total of 21 patients were prospectively followed up long term. With a mean follow-up of 40.8 months, a significant mean reduction in patient self-reported pain from baseline to postoperative of 75.79% pain reduction was seen at follow-up 1 and 62.52% at follow-up 2. A significant decrease was obtained for, respectively, pain at the present moment, VAS pain worst last week, VAS pain least last week, and VAS pain mean last week in comparison with baseline VAS scores. All patients indicated that they would redo the procedure. Percutaneous implantation of small profile paddle leads in patients with FBSS and lumboischialgia produces favorable results over the long term that are at least comparable with surgical implanted paddle leads. The percutaneous approach also allows nonsurgically trained pain physicians to introduce paddle leads. Indices like if patients would redo the procedure may be more appropriate for analyzing long-term outcomes than the arbitrarily taking 50% reduction in VAS scores. © 2013 International Neuromodulation Society.

  14. Comprehensive rehabilitation measure treatment on spinal cord injury central pain of clinical observation%综合康复措施治疗脊髓损伤中枢性疼痛的临床观察

    肖登; 夏新蜀

    2011-01-01

    目的:探讨综合康复治疗措施对脊髓损伤后中枢性疼痛的治疗效果.方法:对31例脊髓损伤后中枢性疼痛患者采用阿米替林、按摩、心理治疗及经皮神经电刺激(TENS)进行治疗.采用McGill疼痛问卷(McGill pain questionnaire,MPQ)、疼痛视觉模拟评分法(Visual Analogous Score,VAS)于治疗前和治疗1疗程后对疼痛和日常生活活动(activities of daily living,ADL)能力进评定.结果:治疗后患者疼痛的各项评分均有明显降低(Pcentral pain of patients with spinal cord injury. Methods: 31 cases of spinal cord injury with central pain were treated with amitriptyline, psychotherapy and transcutaneous electric nerve stimulation ( TENS ), and the effects were assessed by McGill Pain Questionnaire (MPQ), Visual Analogous Score (VAS) and activities of daily living (ADL). Results:The scores of pain of all patients significantly decreased and the scores of ADL highly increased after treatment ( P < 0.01 ). Conclusion: Multidisciplinary therapy is effective on central pain in patients with spinal cord injury.

  15. Impaired neural structure and function contributing to autonomic symptoms in congenital central hypoventilation syndrome.

    Harper, Ronald M; Kumar, Rajesh; Macey, Paul M; Harper, Rebecca K; Ogren, Jennifer A

    2015-01-01

    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, or hypoxic exposure.

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

    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

  17. Mazabraud's Syndrome Coexisting with a Uterine Tumor Resembling an Ovarian Sex Cord Tumor (UTROSCT): a Case Report

    Calisir, Cuneyt; Inan, Ulukan; Yavas, Ulas Savas; Isiksoy, Serap; Kaya, Tamer [Eskisehir Osmangazi University, Eskiseh (Korea, Republic of)

    2007-10-15

    Intramuscular myxoma is a relatively uncommon benign soft tissue tumor. Its association with fibrous dysplasia of bone represents a rare syndrome described by Mazabraud and Girard in 1957. The relationship between fibrous dysplasia and myxoma remains unclear. A common histogenesis has been proposed for both lesions. Wirth et al. has suggested a basic metabolic error of both tissues during the initial growth period, restricted to the region of bone involvement. Myxomas may appear at any age, but have a predilection for older individuals, occurring most commonly during the sixth and seventh decades of life. They are often located in the large muscles of the thigh, shoulder and buttocks. The majority of intramuscular myxomas are solitary. Cross-sectional techniques are essential in the preoperative planning of excision of soft tissue tumors. The ability to evaluate soft tissue myxomas is best accomplished with MR imaging. Myxomas typically demonstrate the following MR features: very sharply defined contour and homogeneous signal intensity. In particular, the lesions are significantly low in signal intensity on T1-weighted images and high in signal intensity on T2-weighted images. In the patient of this case, the MR appearance was in agreement with previously reported cases.

  18. Central nervous system immune reconstitution inflammatory syndrome in AIDS: experience of a Mexican neurological centre.

    Guevara-Silva, Erik A; Ramírez-Crescencio, María A; Soto-Hernández, José Luís; Cárdenas, Graciela

    2012-09-01

    Highly active antiretroviral therapy (HAART) restores the inflammatory immune response in AIDS patients and it may unmask previous subclinical infections or paradoxically exacerbate symptoms of opportunistic infections. Up to 25% of patients receiving HAART develop immune reconstitution inflammatory syndrome (IRIS). We describe six patients with IRIS central nervous system (CNSIRIS) manifestations emphasizing the relevance of CSF cultures and neuroimaging in early diagnosis and management. Patients with CNSIRIS were identified among hospitalized HIV-infected patients that started HAART from January 2002 through December 2007 at a referral neurological center in Mexico. One-hundred and forty-two HIV-infected patients with neurological signs were hospitalized, 64 of which had received HAART, and six (9.3%) developed CNSIRIS. Five patients were male. Two cases of tuberculosis, two of cryptococcosis, one of brain toxoplasmosis, and one possible PML case were found. IRIS onset occurred within 12 weeks of HAART in five patients. Anti-infective therapy was continued. In one case, HAART was temporarily suspended. In long-term follow-up the clinical condition improved in all patients. CNSIRIS associated to opportunistic infections appeared in 9% of patients receiving HAART. Interestingly, no cases of malignancy or neoplasm IRIS-related were found. Frequent clinical assessment and neuroimaging studies supported diagnosis and treatment. Risk factors were similar to those found in other series. Copyright © 2012 Elsevier B.V. All rights reserved.

  19. Upper airway resistance syndrome. Central electroencephalographic power and changes in breathing effort.

    Black, J E; Guilleminault, C; Colrain, I M; Carrillo, O

    2000-08-01

    Upper airway resistance syndrome (UARS) is defined by excessive daytime sleepiness and tiredness, and is associated with increased breathing effort. Its polygraphic features involve progressive increases in esophageal pressure (Pes), terminated by arousal (AR) as defined by the American Sleep Disorders Association (ASDA). With the arousal there is an abrupt decrease in Pes, called Pes reversal. However, Pes reversal can be seen without the presence of an AR. We performed spectral analysis on electroencephalographic data from a central lead for both AR and nonarousal (N-AR) events obtained from 15 UARS patients (eight men and seven women). Delta band activity was increased before and surrounding Pes reversal regardless of the presence or absence of AR. In the period after Pes reversal, alpha, sigma, and beta activity showed a greater increase in AR events than in N-AR events. The Pes measures were identical leading up to the point of reversal, but showed a longer-lasting and significantly greater decrease in respiratory effort after an AR. The data indicate that substantial electroencephalographic changes can be identified in association with Pes events, even when ARs cannot be detected according to standard criteria; however, visually identifiable electroencephalographic arousals clearly have a greater impact on ongoing inspiratory effort.

  20. Neuromyelitis optica with Hashimoto′s thyroiditis: A new syndrome or just coincidence

    Abhishek Singhai

    2015-01-01

    Full Text Available Neuromyelitis optica (NMO is an uncommon disease syndrome of the central nervous system that affects the optic nerves and spinal cord. NMO with endocrinopathies has been described as being unique to black Antillean and Afro-Brazilian women. We describe one case of NMO with hashimoto′s thyroiditis in a young female, probably first case report in India.

  1. Spinal cord stimulation and the induction of c-fos and heat shock protein 72 in the central nervous system of rats

    DeJongste, MJL; Hautvast, RWM; Ruiters, MHJ; Ter Horst, GJ

    For more than a decade, spinal cord stimulation (SCS) has been used as an adjuvant treatment for patients who are unresponsive to conventional therapies for angina pectoris. Many studies showed that SCS has both electro-analgesic and anti-ischemic effects. Nonetheless, the biological substrates by

  2. Dorsal border periaqueductal gray neurons project to the area directly adjacent to the central canal ependyma of the C4-T8 spinal cord in the cat

    Mouton, LJ; Kerstens, L; VanderWant, J; Holstege, G

    In a previous study horseradish peroxidase (HRP) injections in the upper thoracic and cervical spinal cord revealed some faintly labeled small neurons at the dorsal border of the periaqueductal gray (PAG). The present light microscopic and electronmicroscopic tracing study describes the precise

  3. [The role of the neurophysiological methods in the assessment of the effectiveness of the rehabilitation of sensorimotor disturbances associated with the lesions of the central nervous system at the spinal cord level].

    Ekusheva, E V; Voitenkov, V B; Skripchenko, N V; Samoilova, I G; Filimonov, V A

    2017-12-28

    The objective of the present study was the evaluation and comparison of the effectiveness of the differential approaches to the neurorehabilitation of the somatosensory disturbances in the patients presenting with the spinal cord lesions. A total of 68 patients with spinal cord lesions were enrolled in the study, including 38 suffering from vascular myelopathy, 18 with the consequences of extramedullar meningioma surgery, 12 with the sequelae of acute transverse myelitis. The control groups was comprised of were 55 subjects. All the participants of the study underwent rehabilitation which included robotized mechanotherapy, stabilography, neuro-muscular stimulation, kinesiotherapy, physical therapy, ergotherapy, massage, etc. Transcranial magnetic stimulation (TMS) and evaluation of somatosensory evoked potentials (SSEP) were carried out before and after the therapy. In those patients who received personalized therapy, significant changes of TMS parameters (central motor conduction time at rest and in facilitation probe), but not SSEP ones were registered. Moreover, the patients who had undergone personalized therapy exhibited better clinical results than in the absence of such treatment. The results of the study gave evidence that neurorehabilitation had produced the more pronounced beneficial influence as regards the correction of motor disturbances even though the disturbances of the somatosensory functions proved to be more resistant to therapy. The data obtained suggest that taking into consideration the afferent deficit has to be mandatory for the purpose of planning the neurorehabilitative treatment of the patients suffering from sensorimotor disturbances associated with the lesions of the central nervous system at the spinal cord level. TMS and SSEP have to be utilized as the tools for the objective evaluation of the effectiveness of the neurorehabilitation process in such patients.

  4. Cord Blood and Transplants

    ... donate their baby’s umbilical cord blood to a public cord blood bank. We have more than 249,000 cord blood ... stored as a cord blood unit at a public cord blood bank for future use. It can then be listed ...

  5. 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

    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

  6. Horner's syndrome in patients admitted to the intensive care unit that have undergone central venous catheterization: a prospective study.

    Butty, Z; Gopwani, J; Mehta, S; Margolin, E

    2016-01-01

    PurposeCentral venous catheterization (CVC) is estimated to be performed in millions of patients per year. Swan-Ganz catheters used for CVC are most often inserted into the internal jugular vein and during this procedure they may come into contact with the sympathetic chain. This study aims to determine the incidence of Horner's syndrome in patients admitted to intensive care unit that have undergone internal jugular CVC insertion during their admission and to determine whether ultrasonography-assisted insertion has decreased the frequency of this complication.Patients and methodsA total of 100 prospective patients admitted to the ICU were examined for the presence of anisocoria and ptosis after undergoing recent CVC. Presence of Horner's syndrome was confirmed by testing with 0.5% apraclonidine and looking for the reversal of anisocoria.ResultsFrequency of Horner's syndrome after CVC was 2% in a sample of 100 prospectively examined patients.ConclusionHorner's syndrome remains a relatively rare but definitive complication of CVC. ICU physicians should be educated about its existence and prevalence and ophthalmologists should inquire about any history of ICU admission necessitating CVC insertion in any patient presenting with Horner's syndrome.

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

    Mike Sharman

    Full Text Available 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

  8. Motor dysfunction of complex regional pain syndrome is related to impaired central processing of proprioceptive information.

    Bank, Paulina J M; Peper, C Lieke E; Marinus, Johan; Beek, Peter J; van Hilten, Jacobus J

    2013-11-01

    Our understanding of proprioceptive deficits in complex regional pain syndrome (CRPS) and its potential contribution to impaired motor function is still limited. To gain more insight into these issues, we evaluated accuracy and precision of joint position sense over a range of flexion-extension angles of the wrist of the affected and unaffected sides in 25 chronic CRPS patients and in 50 healthy controls. The results revealed proprioceptive impairment at both the patients' affected and unaffected sides, characterized predominantly by overestimation of wrist extension angles. Precision of the position estimates was more prominently reduced at the affected side. Importantly, group differences in proprioceptive performance were observed not only for tests at identical percentages of each individual's range of wrist motion but also when controls were tested at wrist angles that corresponded to those of the patient's affected side. More severe motor impairment of the affected side was associated with poorer proprioceptive performance. Based on additional sensory tests, variations in proprioceptive performance over the range of wrist angles, and comparisons between active and passive displacements, the disturbances of proprioceptive performance most likely resulted from altered processing of afferent (and not efferent) information and its subsequent interpretation in the context of a distorted "body schema." The present results point at a significant role for impaired central processing of proprioceptive information in the motor dysfunction of CRPS and suggest that therapeutic strategies aimed at identification of proprioceptive impairments and their restoration may promote the recovery of motor function in CRPS patients. Copyright © 2013 American Pain Society. Published by Elsevier Inc. All rights reserved.

  9. Multicolumn spinal cord stimulation for significant low back pain in failed back surgery syndrome: design of a national, multicentre, randomized, controlled health economics trial (ESTIMET Study).

    Roulaud, M; Durand-Zaleski, I; Ingrand, P; Serrie, A; Diallo, B; Peruzzi, P; Hieu, P D; Voirin, J; Raoul, S; Page, P; Fontaine, D; Lantéri-Minet, M; Blond, S; Buisset, N; Cuny, E; Cadenne, M; Caire, F; Ranoux, D; Mertens, P; Naous, H; Simon, E; Emery, E; Gadan, B; Regis, J; Sol, J-C; Béraud, G; Debiais, F; Durand, G; Guetarni Ging, F; Prévost, A; Brandet, C; Monlezun, O; Delmotte, A; d'Houtaud, S; Bataille, B; Rigoard, P

    2015-03-01

    Many studies have demonstrated the efficacy of spinal cord stimulation (SCS) for chronic neuropathic radicular pain over recent decades, but despite global favourable outcomes in failed back surgery syndrome (FBSS) with leg pain, the back pain component remains poorly controlled by neurostimulation. Technological and scientific progress has led to the development of new SCS leads, comprising a multicolumn design and a greater number of contacts. The efficacy of multicolumn SCS lead configurations for the treatment of the back pain component of FBSS has recently been suggested by pilot studies. However, a randomized controlled trial must be conducted to confirm the efficacy of new generation multicolumn SCS. Évaluation médico-économique de la STImulation MEdullaire mulTi-colonnes (ESTIMET) is a multicentre, randomized study designed to compare the clinical efficacy and health economics aspects of mono- vs. multicolumn SCS lead programming in FBSS patients with radicular pain and significant back pain. FBSS patients with a radicular pain VAS score≥50mm, associated with a significant back pain component were recruited in 14 centres in France and implanted with multicolumn SCS. Before the lead implantation procedure, they were 1:1 randomized to monocolumn SCS (group 1) or multicolumn SCS (group 2). Programming was performed using only one column for group 1 and full use of the 3 columns for group 2. Outcome assessment was performed at baseline (pre-implantation), and 1, 3, 6 and 12months post-implantation. The primary outcome measure was a reduction of the severity of low back pain (bVAS reduction≥50%) at the 6-month visit. Additional outcome measures were changes in global pain, leg pain, paraesthesia coverage mapping, functional capacities, quality of life, neuropsychological aspects, patient satisfaction and healthcare resource consumption. Trial recruitment started in May 2012. As of September 2013, all 14 study centres have been initiated and 112

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

    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.

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

    Kim, Ok Hwa

    2015-01-01

    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.

  12. Spinal Cord Injury 101

    Full Text Available ... spinal cord injury? play_arrow What kind of surgery is common after a spinal cord injury? play_ ... How soon after a spinal cord injury should surgery be performed? play_arrow Is it common to ...

  13. Spinal Cord Injury 101

    Full Text Available ... L Sarah Harrison, OT Anne Bryden, OT The Role of the Social Worker after Spinal Cord Injury ... a spinal cord injury important? play_arrow What role does “compression” play in a spinal cord injury? ...

  14. Spinal Cord Injury 101

    Full Text Available ... Cord Injury Diane M. Rowles, MS, NP How Family Life Changes After Spinal Cord Injury Nancy Rosenberg, ... Children with Spinal Cord Injury Patricia Mucia, RN Family Life After Pediatric Spinal Injury Dawn Sheaffer, MSW ...

  15. Spinal Cord Injury 101

    Full Text Available ... Counseling Blog About Media Donate Spinal Cord Injury Medical Expert Videos Topics menu Topics Spinal Cord Injury ... Jennifer Piatt, PhD David Chen, MD Read Bio Medical Director, Spinal Cord Injury Rehabilitation Program, Rehabilitation Institute ...

  16. Spinal Cord Injury 101

    Full Text Available ... Blog About Media Donate Spinal Cord Injury Medical Expert Videos Topics menu Topics Spinal Cord Injury 101 ... arrow What is the “Spinal Cord Injury Model Systems” program? play_arrow What are the most promising ...

  17. Spinal Cord Injury 101

    Full Text Available ... Topic Resources Peer Counseling Blog About Media Donate Spinal Cord Injury Medical Expert Videos Topics menu Topics Spinal Cord Injury 101 Adult Injuries Spinal Cord Injury 101 David ...

  18. Spinal Cord Injury 101

    Full Text Available ... Topic Resources Peer Counseling Blog About Media Donate Spinal Cord Injury Medical Expert Videos Topics menu Topics Spinal Cord Injury 101 Adult Injuries Spinal Cord Injury 101 ...

  19. Spinal Cord Diseases

    Your spinal cord is a bundle of nerves that runs down the middle of your back. It carries signals back ... of the spine, this can also injure the spinal cord. Other spinal cord problems include Tumors Infections such ...

  20. Spinal cord contusion.

    Ju, Gong; Wang, Jian; Wang, Yazhou; Zhao, Xianghui

    2014-04-15

    Spinal cord injury is a major cause of disability with devastating neurological outcomes and limited 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.

  1. Central venous recanalization in patients with short gut syndrome: restoration of candidacy for intestinal and multivisceral transplantation.

    Lang, Elvira V; Reyes, Jorge; Faintuch, Salomao; Smith, Amy; Abu-Elmagd, Kareem

    2005-09-01

    To assess feasibility and success of venous recanalization in patients with short gut syndrome who have lost their traditional central venous access and required intestinal or multivisceral transplantation. Twelve patients between the ages of 7 and 55 years with short gut syndrome and long-standing total parenteral nutrition (TPN) dependency and/or hypercoagulability were treated. All had extensive chronic central venous occlusions and survival was dependent on restoration of access and planned transplantation. Central venous recanalizations were obtained via sharp needle recanalization techniques, venous reconstructions with stents, and/or extraanatomic access to the central venous system for placement of central venous tunneled catheters. Central venous access was restored in all patients without operative-related mortality. Three major hemodynamic perioperative technical complications were recorded and successfully treated. There were three self-limited early infectious complications. With a mean follow-up of 22 months, eight of the 12 patients were alive with successful small bowel or multivisceral transplantation; six of those became independent of TPN. The remaining four patients died of complications related to TPN (n = 3) or transplantation (n = 1). With a mean follow-up of 20 months, all but two of the recanalized venous accesses were maintained, for a success rate of 83%. Recanalizations of extensive chronic vein occlusions are feasible but associated with high risk. The technique is life-saving for TPN-dependent patients and can restore candidacy for intestinal and multivisceral transplantation. This approach is likely to be increasingly requested because of the current clinical availability of the transplant procedure.

  2. High-sensitivity C-reactive protein to detect metabolic syndrome in a centrally obese population: a cross-sectional analysis

    den Engelsen Corine

    2012-03-01

    Full Text Available Abstract Background People with central obesity have an increased risk for developing the metabolic syndrome, type 2 diabetes and cardiovascular disease. However, a substantial part of obese individuals have no other cardiovascular risk factors, besides their obesity. High sensitivity C-reactive protein (hs-CRP, a marker of systemic inflammation and a predictor of type 2 diabetes and cardiovascular disease, is associated with the metabolic syndrome and its separate components. We evaluated the use of hs-CRP to discriminate between centrally obese people with and without the metabolic syndrome. Methods 1165 people with central obesity but without any previous diagnosis of hypertension, dyslipidemia, diabetes or cardiovascular disease, aged 20-70 years, underwent a physical examination and laboratory assays to determine the presence of the metabolic syndrome (NCEP ATP III criteria. Multivariable linear regression analyses were performed to assess which metabolic syndrome components were independently associated with hs-CRP. A ROC curve was drawn and the area under the curve was calculated to evaluate whether hs-CRP was capable to predict the presence of the metabolic syndrome. Results Median hs-CRP levels were significantly higher in individuals with central obesity with the metabolic syndrome (n = 417; 35.8% compared to individuals with central obesity without the metabolic syndrome (2.2 mg/L (IQR 1.2-4.0 versus 1.7 mg/L (IQR 1.0-3.4; p Conclusions Hs-CRP has limited capacity to predict the presence of the metabolic syndrome in a population with central obesity.

  3. Effects of Laparoscopic Sleeve Gastrectomy on Central Obesity and Metabolic Syndrome in Indian Adults- A Prospective Study.

    Sethi, Pulkit; Thillai, Manoj; Nain, Prabhdeep Singh; Ahuja, Ashish; Vayoth, Sudheer Othiyil; Khurana, Preetika

    2017-01-01

    Increasing incidence of obesity in Indian population has led to an exponential rise in the number of bariatric operations performed annually. Laparoscopic Sleeve Gastrectomy (LSG) has been proposed to cause rapid remission of Type 2 Diabetes Melitus (T2DM) and metabolic syndrome in a weight loss independent manner. To evaluate the effects of LSG on metabolic syndrome and central obesity in morbidly and severely obese Indian adults. Material and Methods: Study was conducted on 91 morbidly obese [Body Mass Index (BMI)>40 kg/m 2 ] and severely obese (BMI>35 kg/m 2 ) individuals who were suffering from diabetes, hypertension or dyslipidemia. The patients were followed up for six months and the trends of glycaemic control, mean blood pressure, lipid profile, weight loss parameters and changes in parameters of central obesity were studied. Weight loss was significant at three months postsurgery and was sustained through six months. There was significant improvement in glycaemic control leading to reduction in need for oral hypoglycaemic agents or insulin in majority of them and even discontinuation of these medications in few patients. Hypertension and dyslipidemia also showed an improving trend through six months postsurgery. There was a significant impact on reduction of central obesity in these patients as marked by significant reduction in waist to hip ratio. LSG produces sustainable weight loss with significant improvement in glycaemic status and control of metabolic syndrome in severe to morbidly obese patients. LSG is also efficacious in reducing central obesity in Indian population which is a major depressive ailment amongst obese individuals.

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

    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.

  5. Spinal Cord Independence Measure, version III: applicability to the UK spinal cord injured population.

    Glass, Clive A; Tesio, Luigi; Itzkovich, Malka; Soni, Bakul M; Silva, Pedro; Mecci, Munawar; Chadwick, Raymond; el Masry, Waghi; Osman, Aheed; Savic, Gordana; Gardner, Brian; Bergström, Ebba; Catz, Amiram

    2009-09-01

    To examine the validity, reliability and usefulness of the Spinal Cord Independence Measure for the UK spinal cord injury population. Multi-centre cohort study. Four UK regional spinal cord injury centres. Eighty-six people with spinal cord injury. Spinal Cord Independence Measure and Functional Independence Measure on admission analysed using inferential statistics, and Rasch analysis of Spinal Cord Independence Measure. Internal consistency, inter-rater reliability, discriminant validity; Spinal Cord Independence Measure subscale match between distribution of item difficulty and patient ability measurements; reliability of patient ability measures; fit of data to Rasch model; unidimensionality of subscales; hierarchical ordering of categories within items; differential item functioning across patient groups. Scale reliability (kappa coefficients range 0.491-0.835; (p Spinal Cord Independence Measure subscales compatible with stringent Rasch requirements; mean infit indices high; distinct strata of abilities identified; most thresholds ordered; item hierarchy stable across clinical groups and centres. Misfit and differences in item hierarchy identified. Difficulties assessing central cord injuries highlighted. Conventional statistical and Rasch analyses justify the use of the Spinal Cord Independence Measure in clinical practice and research in the UK. Cross-cultural validity may be further improved.

  6. A central role of eNOS in the protective effect of wine against metabolic syndrome.

    Leighton, Federico; Miranda-Rottmann, Soledad; Urquiaga, Inés

    2006-01-01

    The positive health effects derived from moderate wine consumption are pleiotropic. They appear as improvements in cardiovascular risk factors such as plasma lipids, haemostatic mechanisms, endothelial function and antioxidant defences. The active principles would be ethanol and mainly polyphenols. Results from our and other laboratories support the unifying hypothesis that the improvements in risk factors after red wine consumption are mediated by endothelial nitric oxide synthase (eNOS). Many genes are involved, but the participation of eNOS would be a constant feature. The metabolic syndrome is a cluster of metabolic risk factors associated with high risk of cardiovascular disease (CVD). The National Cholesterol Education Programmmes Adult Treatment Panel III (NCEPATP III) clinical definition of the metabolic syndrome requires the presence of at least three risk factors, from among abdominal obesity, high plasma triacylglycerols, low plasma HDL, high blood pressure and high fasting plasma glucose. The molecular mechanisms responsible for the metabolic syndrome are not known. Since metabolic syndrome apparently affects 10-30% of the population in the world, research on its pathogenesis and control is needed. The recent finding that eNOS knockout mice present a cluster of cardiovascular risk factors comparable to those of the metabolic syndrome suggests that defects in eNOS function may cause human metabolic syndrome. These mice are hypertensive, insulin resistant and dyslipidemic. Further support for a pathogenic role of eNOS comes from the finding in humans that eNOS polymorphisms associate with insulin resistance and diabetes, with hypertension, with inflammatory and oxidative stress markers and with albuminuria. So, the data sustain the hypothesis that eNOS enhancement should reduce metabolic syndrome incidence and its consequences. Therefore red wine, since it enhances eNOS function, should be considered as a potential tool for the control of metabolic

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

    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. Copyright © 2016 Elsevier Ltd. All rights reserved.

  8. Diagnosis and Management of Combined Central Diabetes Insipidus and Cerebral Salt Wasting Syndrome After Traumatic Brain Injury.

    Wu, Xuehai; Zhou, Xiaolan; Gao, Liang; Wu, Xing; Fei, Li; Mao, Ying; Hu, Jin; Zhou, Liangfu

    2016-04-01

    Combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury (TBI) is rare, is characterized by massive polyuria leading to severe water and electrolyte disturbances, and usually is associated with very high mortality mainly as a result of delayed diagnosis and improper management. We retrospectively reviewed the clinical presentation, management, and outcomes of 11 patients who developed combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury to define distinctive features for timely diagnosis and proper management. The most typical clinical presentation was massive polyuria (10,000 mL/24 hours or >1000 mL/hour) refractory to vasopressin alone but responsive to vasopressin plus cortisone acetate. Other characteristic presentations included low central venous pressure, high brain natriuretic peptide precursor level without cardiac dysfunction, high 24-hour urine sodium excretion and hypovolemia, and much higher urine than serum osmolarity; normal serum sodium level and urine specific gravity can also be present. Timely and adequate infusion of sodium chloride was key in treatment. Of 11 patients, 5 had a good prognosis 3 months later (Extended Glasgow Outcome Scale score ≥6), 1 had an Extended Glasgow Outcome Scale score of 4, 2 died in the hospital of brain hernia, and 3 developed a vegetative state. For combined diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury, massive polyuria is a major typical presentation, and intensive monitoring of fluid and sodium status is key for timely diagnosis. To achieve a favorable outcome, proper sodium chloride supplementation and cortisone acetate and vasopressin coadministration are key. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Personality and Affect When the Central Nervous System is Sensitized: An Analysis of Central Sensitization Syndromes in a Substance Use Disorder Population.

    McKernan, Lindsey C; Finn, Michael T M; Carr, Erika R

    2017-01-01

    Functional somatic syndromes, or more recently termed central sensitivity syndromes (CSS), comprise a significant portion of the chronic pain population. Although it is evident that personality is intricately related to the pain experience, it has not been widely studied. This article examines the impact of CSS on the clinical presentation of individuals presenting to treatment for a substance use disorder (SUD), with an emphasis on personality and emotional functioning. We examined personality profiles of individuals presenting to treatment with SUD between three groups: those with a CSS (n = 30), non-CSS chronic pain (n = 79), and no pain (n = 232). Based on previous research and a psychodynamic conceptualization of CSS, we hypothesized that predictors of the presence of a CSS in this sample would be higher rates of overall anxiety, traumatic stress, perfectionistic traits, and a need for interpersonal closeness. Logistic regression analyses did not support our hypothesis. Exploratory analyses indicated which personality traits most strongly predicted the presence of CSS. We discuss these findings using descriptive psychopathology literature, with recommendations for future research.

  10. Noninvasive ventilatory strategies in the management of a newborn infant and three children with congenital central hypoventilation syndrome.

    Tibballs, James; Henning, Robert D

    2003-12-01

    Four children with congenital central hypoventilation syndrome (CCHS) treated with noninvasive techniques of ventilation are presented. Two infants (one in the newborn period) were treated with nasal mask bilevel positive airway pressure (BiPAP), and then both were transitioned to negative pressure chamber ventilation at several years of age because of possible midface hypoplasia. Tracheostomies were not performed. Two older children were transitioned from mechanical ventilation via tracheostomy to nasal mask BiPAP, and then in one case to negative pressure chamber ventilation, and in the other to phrenic nerve pacing. Their tracheostomies were removed. Copyright 2003 Wiley-Liss, Inc.

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

    Meguro, Kentaro; Nagai, Ryozo; Toyama, Takuji; Adachi, Hitoshi; Ohshima, Shigeru; Taniguchi, Koichi

    2007-01-01

    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% CO 2 and 93% O 2 ). 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

  12. Elsberg syndrome

    Savoldi, Filippo; Kaufmann, Timothy J.; Flanagan, Eoin P.; Toledano, Michel

    2017-01-01

    Objective: Elsberg syndrome (ES) is an established but often unrecognized cause of acute lumbosacral radiculitis with myelitis related to recent herpes virus infection. We defined ES, determined its frequency in patients with cauda equina syndrome (CES) with myelitis, and evaluated its clinical, radiologic, and microbiologic features and outcomes. Methods: We searched the Mayo Clinic medical records for ES and subsequently for combinations of index terms to identify patients with suspected CES and myelitis. Results: Our search yielded 30 patients, 2 diagnosed with ES and an additional 28 with clinical or radiologic evidence of CES retrospectively suspected of having ES. We classified patients in 5 groups according to diagnostic certainty. MRI and EMG confirmed that 2 had only myelitis, 5 only radiculitis, and 16 both. Two had preceding sacral herpes infection and 1 oral herpes simplex. Spinal cord lesions were commonly multiple, discontinuous, not expansile, and centrally or ventrally positioned. Lesions generally spared the distal conus. Nerve root enhancement was occasionally prominent and was smooth rather than nodular. Lymphocytic CSF pleocytosis was common. Thirteen patients (43%) had viral isolation studies, which were commonly delayed; the delay may have accounted for the low rate of viral detection. Acyclovir was administered to 6 patients. Most patients recovered with sequelae; 1 patient experienced encephalomyelitis and died. Conclusion: ES is a definable condition likely responsible for 10% of patients with combined CES and myelitis. Radiologic findings are not entirely specific but may help in differentiating ES from some competing diagnostic considerations. We propose criteria to facilitate diagnosis. PMID:28534040

  13. Combination therapy with lercanidipine and enalapril reduced central blood pressure augmentation in hypertensive patients with metabolic syndrome.

    Ghiadoni, Lorenzo; Bruno, Rosa Maria; Cartoni, Giulia; Stea, Francesco; Magagna, Armando; Virdis, Agostino; Grassi, Davide; Ferri, Claudio; Taddei, Stefano

    2017-05-01

    Arterial stiffness and blood pressure (BP) augmentation are independent predictors of cardiovascular events. In a randomized, open, parallel group study we compared the effect on these parameters of combination therapy with an ACE-inhibitor plus calcium channel blocker or thiazide diuretic in 76 hypertensive patients with metabolic syndrome uncontrolled by ACE-inhibitor monotherapy. After 4weeks run-in with enalapril (ENA, 20mg), patients were randomized to a combination therapy with lercanidipine (LER, 10-20mg) or hydrochlorothiazide (HCT, 12.5-25mg) for 24weeks. Aortic stiffness (carotid to femoral pulse wave velocity, PWV), central BP values and augmentation (augmentation index, AIx) were measured by applanation tonometry. The two groups showed similar office and central BP after run-in. Office (ENA/LER: from 149.1±4.9/94.5±1.5 to 131.7±8.1/82.2±5.3; ENA/HCT: from 150.3±4.7/94.7±2.1 to 133.1±7.1/82.8±5.3mmHg) and central BP (ENA/LER 127.4±17.1/85.2±12.1 to 120.5±13.5/80.0±9.5mmHg; ENA/HCT 121.6±13.4/79.3±9.5mmHg) were similarly reduced after 24weeks. PWV was comparable after run-in and not differently reduced by the two treatments (ENA/LER from 8.6±1.5 to 8.1±1.3m/s, pmetabolic syndrome not controlled by ENA alone. These results indicate a positive effect of the combination of ENA/LER on central BP augmentation, suggesting a potential additive role for cardiovascular protection. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Torakal Ventral Cord Herniation

    Sermin Tok

    2015-11-01

    Full Text Available  Ventral cord herniation is a rare cause of focal myelopathy due to herniation of the thoracic cord through a dural defect.It is also known by a variety of other terms such as spontaneous thoracic cord herniation or idiopathic spinal cord herniation.The key feature is focal distortion and rotation of the cord with no CSF seen between it and the ventral theca.

  15. Spinal cord toxoplasmosis in AIDS

    Carteret, M.; Petit, E.; Granat, O.; Marichez, M.; Gilquin, J.

    1995-01-01

    Toxoplasmosis is the most common brain parasitic infection in acquired immunodeficiency syndrome (AIDS). Spinal cord localizations are still rare (2 cases with cerebral involvement, 2 cases without). A case of both spinal cord and cerebral involvement is reported. Magnetic resonance imaging (MR imaging) was performed because of sensory level (L 1). A focal conus medullaris enlargement was seen, iso intense on T 1 weighted images. This lesion was hyperintense on T 2 weighted sequence, and was homogeneously enhanced after Gadolinium on T 1 weighted images. A medullary oedema was noted. A toxoplasmosis treatment was initiated, without cortico therapy. MR imaging performed one month later (D 30), while important clinical improvements were seen, pointed out normal thickness of conus medullaris, without enhancement after Gadolinium. Disease lesions in AIDS with focal spinal cord processes are reviewed, and diagnostic work-up is discussed. Spinal cord single lesion, associated or not with brain involvements should be treated as a toxoplasmic infection, with MR imaging follow up. This work up should avoid medullary biopsy, still required in case of treatment failure. Cerebral involvements, with multiples lesions can mask medullary localization. (authors). 8 refs., 2 figs

  16. What Is Being Trained? How Divergent Forms of Plasticity Compete To Shape Locomotor Recovery after Spinal Cord Injury.

    Huie, J Russell; Morioka, Kazuhito; Haefeli, Jenny; Ferguson, Adam R

    2017-05-15

    Spinal cord injury (SCI) is a devastating syndrome that produces dysfunction in motor and sensory systems, manifesting as chronic paralysis, sensory changes, and pain disorders. The multi-faceted and heterogeneous nature of SCI has made effective rehabilitative strategies challenging. Work over the last 40 years has aimed to overcome these obstacles by harnessing the intrinsic plasticity of the spinal cord to improve functional locomotor recovery. Intensive training after SCI facilitates lower extremity function and has shown promise as a tool for retraining the spinal cord by engaging innate locomotor circuitry in the lumbar cord. As new training paradigms evolve, the importance of appropriate afferent input has emerged as a requirement for adaptive plasticity. The integration of kinematic, sensory, and loading force information must be closely monitored and carefully manipulated to optimize training outcomes. Inappropriate peripheral input may produce lasting maladaptive sensory and motor effects, such as central pain and spasticity. Thus, it is important to closely consider the type of afferent input the injured spinal cord receives. Here we review preclinical and clinical input parameters fostering adaptive plasticity, as well as those producing maladaptive plasticity that may undermine neurorehabilitative efforts. We differentiate between passive (hindlimb unloading [HU], limb immobilization) and active (peripheral nociception) forms of aberrant input. Furthermore, we discuss the timing of initiating exposure to afferent input after SCI for promoting functional locomotor recovery. We conclude by presenting a candidate rapid synaptic mechanism for maladaptive plasticity after SCI, offering a pharmacological target for restoring the capacity for adaptive spinal plasticity in real time.

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

    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.

  18. Duloxetine in patients with central neuropathic pain caused by spinal cord injury or stroke: a randomized, double-blind, placebo-controlled trial

    Vranken, J. H.; Hollmann, M. W.; van der Vegt, M. H.; Kruis, M. R.; Heesen, M.; Vos, K.; Pijl, A. J.; Dijkgraaf, M. G. W.

    2011-01-01

    The mechanisms underlying central neuropathic pain are poorly understood. Pain inhibitory mechanisms including sertononergic and norepinephrine systems may be dysfunctional. In this randomized, double-blinded, placebo-controlled trial we evaluated the effects of duloxetine on pain relief

  19. Spinal Cord Injury 101

    Full Text Available ... About Media Donate Spinal Cord Injury Medical Expert Videos ... Home Kim Eberhardt Muir, MS Coping with a New Injury Robin Dorman, PsyD Sex and Fertility After Spinal Cord Injury Diane M. ...

  20. Spinal Cord Injury 101

    Full Text Available ... of Spinal Cord Injury Rehabilitation Kristine Cichowski, MS Occupational Therapy after Spinal Cord Injury Katie Powell, OT ... does not provide medical advice, recommend or endorse health care products or services, or control the information ...

  1. Spinal Cord Dysfunction (SCD)

    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...

  2. Spinal Cord Injury 101

    Full Text Available ... Injury Facts and Figures Care and Treatment After SCI Spinal Cord Injury Rehabilitation Pediatric Spinal Cord Injuries Video Library SCI Medical Experts People Living with SCI Personal Experiences ...

  3. Spinal Cord Injury 101

    Full Text Available ... of spinal cord injuries? play_arrow What does stem-cell research on animals tell us? play_arrow When can we expect stem-cell treatments to become available for spinal cord injuries? ...

  4. Spinal Cord Injury 101

    Full Text Available ... spinal cord injuries? play_arrow What does stem-cell research on animals tell us? play_arrow When can we expect stem-cell treatments to become available for spinal cord injuries? ...

  5. Spinal Cord Injury 101

    Full Text Available ... Spinal Cord Injuries Video Library SCI Medical Experts People Living with SCI Personal Experiences by Topic Resources ... Spinal Cord Injuries Video Library SCI Medical Experts People Living with SCI Personal Experiences by Topic Resources ...

  6. Spinal Cord Injury 101

    Full Text Available ... play_arrow What are the chances of regaining feeling and mobility after a spinal cord injury? play_arrow How long does it usually take for feeling and movement to return after a spinal cord ...

  7. Spinal Cord Injury 101

    Full Text Available ... RN Pediatric Injuries Pediatric Spinal Cord Injury 101 Lawrence Vogel, MD The Basics of Pediatric SCI Rehabilitation ... Rogers, PT Recreational Therapy after Spinal Cord Injury Jennifer Piatt, PhD David Chen, MD Read Bio Medical ...

  8. Spinal Cord Injury 101

    Full Text Available ... Disabilities Photography by Rona Talcott Website by Mobile Marketing LLC Understanding Spinal Cord Injury About Us Expert Videos Contact Us Personal Experience Videos Blog Videos By Topic Media Resources Donate to support families facing spinal cord ...

  9. Spinal cord stimulation

    ... this page: //medlineplus.gov/ency/article/007560.htm Spinal cord stimulation To use the sharing features on this page, please enable JavaScript. Spinal cord stimulation is a treatment for pain that uses ...

  10. Spinal Cord Injury 101

    Full Text Available ... Spinal Cord Injury 101 Lawrence Vogel, MD The Basics of Pediatric SCI Rehabilitation Sara Klaas, MSW Transitions for Children with Spinal Cord Injury Patricia Mucia, RN Family Life After Pediatric Spinal Injury Dawn Sheaffer, MSW Rehabilitation ...

  11. Spinal Cord Injury 101

    Full Text Available ... Spinal Cord Injury 101 David Chen, MD Preventing Pressure Sores Mary Zeigler, MS Transition from Hospital to ... a spinal cord injury? play_arrow Why are high-dose steroids often used right after an injury? ...

  12. Central Sensitization Syndrome and the Initial Evaluation of a Patient with Fibromyalgia: A Review

    Kevin C. Fleming

    2015-04-01

    Full Text Available In both primary care and consultative practices, patients presenting with fibromyalgia (FM often have other medically unexplained somatic symptoms and are ultimately diagnosed as having central sensitization (CS. Central sensitization encompasses many disorders where the central nervous system amplifies sensory input across many organ systems and results in myriad symptoms. A pragmatic approach to evaluate FM and related symptoms, including a focused review of medical records, interviewing techniques, and observations, is offered here, giving valuable tools for identifying and addressing the most relevant symptoms. At the time of the clinical evaluation, early consideration of CS may improve the efficiency of the visit, reduce excessive testing, and help in discerning between typical and atypical cases so as to avoid an inaccurate diagnosis. Discussion of pain and neurophysiology and sensitization often proves helpful.

  13. A rare association of central hypothyroidism and adrenal insufficiency in a boy with Williams-Beuren syndrome

    Devi Dayal

    2017-03-01

    Full Text Available Primary hypothyroidism related to morphological and volumetric abnormalities of the thyroid gland is one of the commonest of several endocrine dysfunctions in Williams-Beuren syndrome (WBS. We report a 10-month-old boy with WBS who presented with central hypothyroidism. During the neonatal period, he had prolonged jaundice, feeding difficulties and episodes of colic that continued during early infancy. Additionally, there was slowing of growth and mild developmental delay. He underwent surgical repair for supravalvular aortic stenosis at 6 months of age. An evaluation done to exclude cortisol deficiency before initiating levothyroxine lead to the detection of secondary adrenal insufficiency, unreported previously in WBS. In addition, insulin-like growth factor-1 (IGF-1 and IGF-binding protein-3 levels were low. This report of hypopituitarism in WBS indicates a need for complete evaluation of pituitary dysfunction in children with WBS.

  14. Hypocretin Deficiency Associated with Narcolepsy Type 1 and Central Hypoventilation Syndrome in Neurosarcoidosis of the Hypothalamus.

    Mayo, Mary Catherine; Deng, Jane C; Albores, Jeffrey; Zeidler, Michelle; Harper, Ronald M; Avidan, Alon Y

    2015-09-15

    We report a case of a 53-year-old man presenting with depressed alertness and severe excessive sleepiness in the setting of neurosarcoidosis. Neuroimaging demonstrated hypothalamic destruction due to sarcoidosis with a CSF hypocretin level of 0 pg/mL. The patient also experienced respiratory depression that presumably resulted from hypocretin-mediated hypothalamic dysfunction as a result of extensive diencephalic injury. This is a novel case, demonstrating both hypocretin deficiency syndrome, as well as respiratory dysfunction from destruction of hypocretin neurons and extensive destruction of key diencephalic structures secondary to the underlying neurosarcoidosis. © 2015 American Academy of Sleep Medicine.

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

    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. PHOX2B mutation-confirmed congenital central hypoventilation syndrome in a Chinese family: presentation from newborn to adulthood.

    Lee, Peilin; Su, Yi-Ning; Yu, Chong-Jen; Yang, Pan-Chyr; Wu, Huey-Dong

    2009-02-01

    Congenital central hypoventilation syndrome (CCHS) is characterized by compromised chemoreflexes resulting in sleep hypoventilation. We report a Chinese family with paired-like homeobox 2B (PHOX2B) mutation-confirmed CCHS, with a clinical spectrum from newborn to adulthood, to increase awareness of its various manifestations. After identifying central hypoventilation in an adult man (index case), clinical evaluation was performed on the complete family, which consisted of the parents, five siblings, and five offspring. Pulmonary function tests, overnight polysomnography, arterial blood gas measurements, hypercapnia ventilatory response, and PHOX2B gene mutation screening were performed on living family members. Brain MRI, 24-h Holter monitoring, and echocardiography were performed on members with clinically diagnosed central hypoventilation. The index patient and four offspring manifested clinical features of central hypoventilation. The index patients had hypoxia and hypercapnia while awake, polycythemia, and hematocrit levels of 70%. The first and fourth children had frequent cyanotic spells, and both died of respiratory failure. The second and third children remained asymptomatic until adulthood, when they experienced impaired hypercapnic ventilatory response. The third child had nocturnal hypoventilation with nadir pulse oximetric saturation of 59%. Adult-onset CCHS with PHOX2B gene mutation of the + 5 alanine expansions were confirmed in the index patient and the second and third children. The index patient and the third child received ventilator support system bilevel positive airway pressure treatment, which improved the hypoxemia, hypercapnia, and polycythemia without altering their chemosensitivity. Transmission of late-onset CCHS is autosomal-dominant. Genetic screening of family members of CCHS probands allows for early diagnosis and treatment.

  17. [Combination of acupuncture, cupping and medicine for treatment of fibromyalgia syndrome: a multi-central randomized controlled trial].

    Jang, Zhen-Ya; Li, Chang-Du; Qiu, Ling; Guo, Jun-Hua; He, Ling-Na; Yue, Yang; Li, Fang-Ze; Qin, Wen-Yi

    2010-04-01

    To evaluate the clinical effect of combination of acupuncture, cupping and medicine for treatment of fibromyalgia syndrome. By using multi-central randomized controlled method, 186 cases were randomly divided into an acupuncture combined with cupping and western medicine group (group A), an acupuncture combined with cupping group (group B) and a western medicine group (group C) and treated continuously for 4 weeks. The treatment of acupuncture combined with cupping was produced by acupuncture at five mental points and moving cupping on the Hechelu of the back, once evrey other day, thrice each week, and the western medicine therapy by oral administration of Amitriptyline, once each day. The scores of McGill Pain Questionnaire (MPQ), the amount of tenderness point and the time of producing effect were compared and the therapeutic effects were assessed with the Hamilton Depression Scale (HAMD). The cured and markedly effective rate was 65.0% (39/60) in the group A, which was superior to 15.9% (10/63) in the group B and 16.1% (9/56) in the group C (both P cupping and medicine on fibromyalgia syndrome is superior to that of the simple acupuncture combined with cupping or the simple medicine.

  18. JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

    Hoeve, Hans L J; Brooks, Alice S; Smit, Liesbeth S

    2015-07-01

    We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  19. Spinal Cord Injury 101

    Full Text Available ... the spinal cord work? play_arrow Why is the level of a spinal cord injury important? play_arrow What role does “compression” play in a spinal cord injury? play_arrow Why are high-dose steroids often used right after an injury? play_arrow What is meant ...

  20. Spinal Cord Injury 101

    Full Text Available ... arrow What is the “Spinal Cord Injury Model Systems” program? play_arrow What are the most promising new treatments for spinal cord injuries? play_arrow What are the latest developments in the use of electrical stimulation for spinal cord injuries? play_arrow ...

  1. Spinal Cord Injuries

    ... 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 ...

  2. Spinal Cord Injury 101

    Full Text Available ... injury? play_arrow How does the spinal cord work? play_arrow Why is the level of a spinal cord injury important? play_arrow What role does “compression” play in a spinal cord injury? play_arrow Why are high-dose steroids often used right after an injury? play_arrow What is meant ...

  3. Spinal Cord Injury 101

    Full Text Available ... Abuse and Spinal Cord Injury Allen Heinemann, PhD How Peer Counseling Works Julie Gassaway, MS, RN Pediatric Injuries Pediatric Spinal ... What is a spinal cord injury? play_arrow How does the spinal cord work? play_arrow Why is the level of a ...

  4. Re-irradiation of metastatic spinal cord compression: A feasibility study by volumetric-modulated arc radiotherapy for in-field recurrence creating a dosimetric hole on the central canal

    Mancosu, Pietro; Navarria, Piera; Bignardi, Mario; Cozzi, Luca; Fogliata, Antonella; Lattuada, Paola; Santoro, Armando; Urso, Gaetano; Vigorito, Sabrina; Scorsetti, Marta

    2010-01-01

    When local recurrences arise within an irradiated region involving metastatic spinal cord compression, the dose limit to the spinal cord reduces the chance to re-treat the patient by 3D-conformational RT technique. The possibility of using volumetric modulated arc RT by RapidArc was evaluated for dose sparing at spinal cord level and preserving target coverage. A clinically satisfactory PTV coverage and dose sparing to the spinal cord were obtained. An upcoming trial on patients will provide clinical outcomes.

  5. Polyalanine expansion and frameshift mutations of the paired-like homeobox gene PHOX2B in congenital central hypoventilation syndrome.

    Amiel, Jeanne; Laudier, Béatrice; Attié-Bitach, Tania; Trang, Ha; de Pontual, Loïc; Gener, Blanca; Trochet, Delphine; Etchevers, Heather; Ray, Pierre; Simonneau, Michel; Vekemans, Michel; Munnich, Arnold; Gaultier, Claude; Lyonnet, Stanislas

    2003-04-01

    Congenital central hypoventilation syndrome (CCHS or Ondine's curse; OMIM 209880) is a life-threatening disorder involving an impaired ventilatory response to hypercarbia and hypoxemia. This core phenotype is associated with lower-penetrance anomalies of the autonomic nervous system (ANS) including Hirschsprung disease and tumors of neural-crest derivatives such as ganglioneuromas and neuroblastomas. In mice, the development of ANS reflex circuits is dependent on the paired-like homeobox gene Phox2b. Thus, we regarded its human ortholog, PHOX2B, as a candidate gene in CCHS. We found heterozygous de novo mutations in PHOX2B in 18 of 29 individuals with CCHS. Most mutations consisted of 5-9 alanine expansions within a 20-residue polyalanine tract probably resulting from non-homologous recombination. We show that PHOX2B is expressed in both the central and the peripheral ANS during human embryonic development. Our data support an essential role of PHOX2B in the normal patterning of the autonomous ventilation system and, more generally, of the ANS in humans.

  6. Elucidating the mechanism of posterior reversible encephalopathy syndrome: a case of transient blindness after central venous catheterization.

    Rao, Neal M; Raychev, Radoslav; Kim, Doojin; Liebeskind, David S

    2012-11-01

    Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by reversible symptoms including headache, visual disturbances, focal neurological deficits, altered mentation, and seizures. It has been associated with circumstances that may affect the cerebrovascular system, such as hypertension, eclampsia, and immunosuppression with calcineurin inhibitors. The underlying etiology of PRES has remained unclear; however, cerebrovascular autoregulatory dysfunction, hyperperfusion, and endothelial activation have been implicated. We describe a case of a young patient with lung transplant, who presented with headache, acute binocular blindness, and seizure immediately after infusion of saline through a peripherally inserted central catheter line, which inadvertently terminated cephalad in the left internal jugular vein, near the jugular foramen. Subsequent brain magnetic resonance imaging revealed vasogenic edematous lesions in a pattern consistent with PRES--a diagnosis supported by his constellation of symptoms, history of lung transplantation on tacrolimus immunosuppression, and relative hypertension. This is the first reported case describing the development of PRES after the insertion of a peripherally inserted central catheter line. The development of PRES in a typical high-risk patient immediately after cerebral venous outflow obstruction implicates the role of the cerebral venous system and provides potential insight into the mechanism of this disorder that remains of unclear pathogenesis.

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

    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

  8. Uric acid in metabolic syndrome: From an innocent bystander to a central player

    Kanbay, Mehmet; Jensen, Thomas; Solak, Yalcin; Le, Myphuong; Roncal-Jimenez, Carlos; Rivard, Chris; Lanaspa, Miguel A.; Nakagawa, Takahiko; Johnson, Richard J.

    2016-01-01

    Uric acid, once viewed as an inert metabolic end-product of purine metabolism, has been recently incriminated in a number of chronic disease states, including hypertension, metabolic syndrome, diabetes, non-alcoholic fatty liver disease, and chronic kidney disease. Several experimental and clinical studies support a role for uric acid as a contributory causal factor in these conditions. Here we discuss some of the major mechanisms linking uric acid to metabolic and cardiovascular diseases. At this time the key to understanding the importance of uric acid in these diseases will be the conduct of large clinical trials in which the effect of lowering uric acid on hard clinical outcomes is assessed. Elevated uric acid may turn out to be one of the more important remediable risk factors for metabolic and cardiovascular diseases. PMID:26703429

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

    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.

  10. Are there endogenous stem cells in the spinal cord?

    Ferrucci, Michela; Ryskalin, Larisa; Busceti, Carla L; Gaglione, Anderson; Biagioni, Francesca; Fornai, Francesco

    2017-12-01

    Neural progenitor cells (NPC) represent the stem-like niche of the central nervous system that maintains a regenerative potential also in the adult life. Despite NPC in the brain are well documented, the presence of NPC in the spinal cord has been controversial for a long time. This is due to a scarce activity of NPC within spinal cord, which also makes difficult their identification. The present review recapitulates the main experimental studies, which provided evidence for the occurrence of NPC within spinal cord, with a special emphasis on spinal cord injury and amyotrophic lateral sclerosis. By using experimental models, here we analyse the site-specificity, the phenotype and the main triggers of spinal cord NPC. Moreover, data are reported on the effect of specific neurogenic stimuli on these spinal cord NPC in an effort to comprehend the endogenous neurogenic potential of this stem cell niche.

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

    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.

  12. Contributions of the cerebellum to disturbed central processing of visceral stimuli in irritable bowel syndrome.

    Rosenberger, Christina; Thürling, Markus; Forsting, Michael; Elsenbruch, Sigrid; Timmann, Dagmar; Gizewski, Elke R

    2013-04-01

    There is evidence to support that the cerebellum contributes to the neural processing of both emotions and painful stimuli. This could be particularly relevant in conditions associated with chronic abdominal pain, such as the irritable bowel syndrome (IBS), which are often also characterized by affective disturbances. We aimed to test the hypothesis that in IBS, symptoms of anxiety and depression modulate brain activation during visceral stimulation within the cerebellum. We reanalyzed a previous data set from N = 15 female IBS patients and N = 12 healthy women with a specific focus on the cerebellum using advanced normalization methods. Rectal distension-induced brain activation was measured with functional magnetic resonance imaging using non-painful and painful rectal distensions. Symptoms of anxiety and depression, assessed with the Hospital Anxiety and Depression scale, were correlated with cerebellar activation within IBS patients. Within IBS, depression scores were associated with non-painful distension-induced activation in the right cerebellum primarily in Crus II and lobule VIIIb, and additionally in Crus I. Depression scores were also associated with painful distension-induced activation predominantly in vermal lobule V with some extension to the intermediate cerebellum. Anxiety scores correlated significantly with non-painful induced activation in Crus II. Symptoms of anxiety and depression, which are frequently found in chronic pain conditions like IBS, modulate activation during visceral sensory signals not only in cortical and subcortical brain areas but also in the cerebellum.

  13. Polycystic ovary syndrome in Central Australia: Diagnosis and screening of cardiometabolic risk and emotional wellbeing

    Ellis, Emma; Gibson-Helm, Melanie; Boyle, Jacqueline A

    2018-04-01

    Polycystic ovary syndrome (PCOS) is a common condition that affects fertility, body image and emotional wellbeing in women, as well as significantly increasing a woman’s likelihood of developing type 2 diabetes mellitus (T2DM) and other cardiovascular disease risk factors. The objective of this study was to assess how management of PCOS in an Aboriginal primary care setting aligns with national standards for diagnosis and screening of cardiometabolic risk and emotional wellbeing. We conducted a retrospective clinical audit of 63 women who had PCOS listed as a diagnosis in their clinical record. Most women (95%) were correctly diagnosed, the most common trigger being menstrual irregularity (83%). Screening for cardiometabolic complications and emotional wellbeing as recommended by the national guideline was applied inconsistently, including 38% of eligible women not being screened for T2DM in the previous 12 months, and no woman being formally screened for emotional wellbeing. Discussion of lifestyle management was nearly universal; most women (75%) were referred to a dietician, although a third did not attend their appointment. Some components of recommended PCOS care were provided at high levels, including correct application of diagnostic criteria. However, PCOS management and screening for complications are being applied inconsistently in a population with high levels of cardiometabolic and emotional wellbeing risk.

  14. Successful treatment of central venous catheter induced superior vena cava syndrome with ultrasound accelerated catheter-directed thrombolysis.

    Dumantepe, Mert; Tarhan, Arif; Ozler, Azmi

    2013-06-01

    Superior vena cava (SVC) syndrome results from obstruction of flow through the vessel either by external compression or thrombosis. External compression by intrathoracic neoplasms is the most common etiology, especially lung cancer and lymphoma. Thrombosis is becoming increasingly common due to the use of indwelling catheters and implantable central venous access devices. Most patients are unresponsive to anticoagulation alone which appears to be effective only in the mildest cases. However, recent advances in catheter-based interventions have led to the development of a variety of minimally invasive endovascular strategies to remove venous thrombus and accepted as an important first-line treatment given its high overall success rate and low morbidity as compared with medical and surgical treatments. Ultrasound accelerated catheter-directed thrombolysis (UACDT) has been developed to rapidly and completely resolve the existing thrombus. This technique integrates high frequency, low intensity ultrasound (US) with standard CDT in order to accelerate clot dissolution, reducing treatment time and the incidence of thrombolysis-related complications. An US wave enhances drug permeation through thrombus by disaggregating the fibrin matrix, exposing additional plasminogen receptor sites to the thrombolytic agent. The US energy affects thrombus in the entire venous segment, increasing the probability of complete thrombus clearing. We report the case of a 56-year-old man who presented with a 5 days history of SVC syndrome symptoms who had been receiving chemotherapy for colon cancer through a right subclavian vein port catheter. The patient successfully treated with UACDT with EkoSonic(®) Mach4e Endovascular device with an overnight infusion. © 2013 Wiley Periodicals, Inc. Copyright © 2013 Wiley Periodicals, Inc.

  15. A rare case of concomitant sicca keratopathy and ipsilateral central facial palsy in Wallenberg’s dorsolateral medullary syndrome

    De Bruyn, Deborah

    2017-03-01

    Full Text Available Objective: To describe a patient with a right-sided supranuclear facial palsy and concomitant sicca keratopathy of the right eye following right-sided dorsolateral medullary infarction. Methods: Our patient underwent a complete ophthalmologic and neurologic examination including biomicroscopy, fundus examination, cranial nerve examination, Shirmer I test, and magnetic resonance imaging of the brain.Results: A 61-year-old woman presented in emergency with a central facial nerve palsy on the right side and truncal ataxia. Neurologic assessment revealed a concurrent dysphagia, dysarthria, hypoesthesia of the right face, and weakness of the right upper limb. Magnetic resonance imaging of the brain showed an old left-sided cerebellar infarction, but a recent ischemic infarction at the level of the right dorsolateral medulla oblongata was the cause of our patient’s current problems. One month after diagnosis of the right-sided dorsolateral medullary syndrome, there were complaints of ocular irritation and a diminished visual acuity in the right eye. Biomicroscopy showed a sicca keratopathy with nearly complete absence of tear secretion on the Shirmer I test, but with normal eye closure and preserved corneal reflexes and sensitivity.Conclusion: A dorsolateral medullary syndrome can have a variable expression in symptomatology. Our case is special because of the combination of an ipsilateral supranuclear facial palsy with normal upper facial muscle function together with an ipsilateral sicca keratopathy as a result of a nearly absent tear secretion. We hypothesized that the mechanism underlying the patient’s sicca keratopathy ipsilateral to the supranuclear facial palsy involved the superior salivatory nucleus, which is situated in the caudal pons inferiorly of the motor facial nucleus and is most probably affected by a superior extension of the infarcted area in the right medulla oblongata.

  16. Vascular anatomy of the spinal cord

    Thron, A.K.

    1988-01-01

    The book summarizes the anatomic guidelines of external blood supply to the spinal cord. The basic principles of arterial supply and venous drainage are illustrated by explicit schemes for quick orientation. In the first part of the book, systematic radiologic-anatomic investigations of the superficial and deep vessels of all segments of the spinal cord are introduced. The microvascular morphology is portrayed by numerous microradiographic sections in all three dimensions without overshadowing. The three-dimensional representation of the vascular architecture illustrates elementary outlines and details of arterial territories, anastomotic cross-linking as well as the capillary system, particularly the hitherto unknown structure of the medullary venous system with its functionally important anastomoses and varying regional structures. These often now radiologic-anatomic findings are discussed as to their functional and pathophysiologic impact and constitute the basic on which to improve one's understanding of vascular syndromes of the spinal cord

  17. Sensory and Motor Responses to Spinal Cord Injury

    Yezierski, Robert P

    1999-01-01

    The goal of Dr. Yezierski's research was to gain a better understanding of the anatomical, neurochemical and functional changes that occur within the central nervous system following spinal cord injury...

  18. Spinal cord magnetic resonance imaging in suspected multiple sclerosis

    Lycklama a Nijeholt, G.J.; Bergers, E.; Castelijns, J.A.; Barkhof, F.; Uitdehaag, B.M.J.; Polman, C.H.

    2000-01-01

    We examined the value of spinal cord magnetic resonance imaging (MRI) in the diagnostic work-up of multiple sclerosis (MS). Forty patients suspected of having MS were examined within 24 months after the start of symptoms. Disability was assessed, and symptoms were categorized as either brain or spinal cord. Work-up further included cerebrospinal fluid analysis and standard proton-density, T2-, and T1-weighted gadolinium-enhanced brain and spinal cord MRI. Patients were categorized as either clinically definite MS (n = 13), laboratory-supported definite MS (n = 14), or clinically probable MS (n = 4); four patients had clinically probable MS, and in nine MS was suspected. Spinal cord abnormalities were found in 35 of 40 patients (87.5 %), consisting of focal lesions in 31, only diffuse abnormalities in two, and both in two. Asymptomatic spinal cord lesions occurred in six patients. All patients with diffuse spinal cord abnormality had clear spinal cord symptoms and a primary progressive disease course. In clinically definite MS, the inclusion of spinal imaging increased the sensitivity of MRI to 100 %. Seven patients without a definite diagnosis had clinically isolated syndromes involving the spinal cord. Brain MRI was inconclusive, while all had focal spinal cord lesions which explained symptoms and ruled out other causes. Two other patients had atypical brain abnormalities suggesting ischemic/vascular disease. No spinal cord abnormalities were found, and during follow-up MS was ruled out. Spinal cord abnormalities are common in suspected MS, and may occur asymptomatic. Although diagnostic classification is seldom changed, spinal cord imaging increases diagnostic sensitivity of MRI in patients with suspected MS. In addition, patients with primary progressive MS may possibly be earlier diagnosed. Finally, differentiation with atypical lesions may be improved. (orig.)

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

    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

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

    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

  1. Identification of Subgroups of Women with Carpal Tunnel Syndrome with Central Sensitization.

    Fernández-de-Las-Peñas, César; Fernández-Muñoz, Juan J; Navarro-Pardo, Esperanza; da-Silva-Pocinho, Ricardo F; Ambite-Quesada, Silvia; Pareja, Juan A

    2016-09-01

    Identification of subjects with different sensitization mechanisms can help to identify better therapeutic strategies for carpal tunnel syndrome (CTS). The aim of the current study was to identify subgroups of women with CTS with different levels of sensitization. A total of 223 women with CTS were recruited. Self-reported variables included pain intensity, function, disability, and depression. Pressure pain thresholds (PPT) were assessed bilaterally over median, ulnar, and radial nerves, C5-C6 joint, carpal tunnel, and tibialis anterior to assess widespread pressure pain hyperalgesia. Heat (HPT) and cold (CPT) pain thresholds were also bilaterally assessed over the carpal tunnel and the thenar eminence to determine thermal pain hyperalgesia. Pinch grip force between the thumb and the remaining fingers was calculated to determine motor assessment. Subgroups were determined according to the status on a previous clinical prediction rule: PPT over the affected C5-C6 joint 66 points. The ANOVA showed that women within group 1 (positive rule, n = 60) exhibited bilateral widespread pressure hyperalgesia (P < 0.001) and bilateral thermal thresholds (P < 0.001) than those within group 2 (negative rule, n = 162). Women in group 1 also exhibited higher depression than those in group 2 (P = 0.023). No differences in self-reported variables were observed. This study showed that a clinical prediction rule originally developed for identifying women with CTS who are likely to respond favorably to manual physical therapy was able to identify women exhibiting higher widespread pressure hyper-sensitivity and thermal hyperalgesia. This subgroup of women with CTS exhibiting higher sensitization may need specific therapeutic programs. © 2016 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  2. Neuromyelitis optica presenting with horner syndrome: A case report and review of literature.

    Uludağ, İrem Fatma; Sarıteke, Alp; Öcek, Levent; Zorlu, Yaşar; Şener, Ufuk; Tokuçoğlu, Figen; Uludağ, Burhanettin

    2017-05-01

    Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. We describe a 19 years old woman with left Horner syndrome (HS), who was diagnosed as NMO with characteristic longitudinally extensive myelitis and positive serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Our report describes one of the very rare ocular motor symptoms in NMO patients. Copyright © 2017 Elsevier B.V. All rights reserved.

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

    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.

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

    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.

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

    Trotot, P.M.; Sansonetti, P.J.; Levillain, R.; Cabanis, E.A.; Lavayssiere, R.; Sandoz-Tronca, C.

    1988-01-01

    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 [fr

  6. Radiation tolerance of the cervical spinal cord

    McCunniff, A.J.; Liang, M.J.

    1989-01-01

    The incidence of permanent injury to the spinal cord as a complication of radiation therapy generally correlates positively with total radiation dosage. However, several reports in the literature have indicated that fraction size is also an important factor in the development or nondevelopment of late injuries in normal tissue. To determine the effect of fraction size on the incidence of radiation-induced spinal cord injuries, we reviewed 144 cases of head and neck cancer treated at our institution between 1971 and 1980 with radiation greater than 5600 cGy to a portion of the cervical spinal cord. Most of these patients received greater than or equal to 6000 cGy, with fraction sizes ranging from 133 cGy to 200 cGy. Fifty-three of the 144 patients have been followed up for 2 years or more. Nearly half of these (26 patients) received greater than 6000 cGy with fraction sizes of 133 cGy to 180 cGy. Only 1 of the 53 (1.9%) has sustained permanent spinal cord injury; 20 months after completion of radiation treatments he developed Brown-Sequard syndrome. Our experience suggests that radiation injuries to the spinal cord correlate not only with total radiation dosage, but also with fraction size; low fraction sizes appear to decrease the incidence of such injuries

  7. Spinal Cord Injury 101

    Full Text Available ... Disabilities Photography by Rona Talcott Website by Mobile Marketing LLC Understanding Spinal Cord Injury About ... Your email address * This iframe contains the logic required to ...

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

    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.

  9. MR findings of central nervous system involvement in acquired immunodeficiency syndrome patient : a report of two cases

    Hong, Hye Suk; Kim, Dong Ik; Lee, Byeong Hee; Jeong, Sun Yang

    1996-01-01

    Central nervous system (CNS) manifestations in acquired immunodeficiency syndrome (AIDS) patients are an early and common feature. The spectrum of AIDS-related CNS diseases are encephalitis caused by the human immunodeficiency virus(HIV) itself, opportunistic infection, infarct and malignancy. We experienced two cases of CNS involvement in AIDS and they were serologically diagnosed as HIV encephalitis and CNS toxoplasmosis, respectively. In the case of the HIV encephalitis patient, brain MRI showed a non-enhancing lesion with high signal intensity on T2WI and low signal on T1WI and there was no mass effect on the right frontal lobe, periventricular white matter, splenium of the corpus callosum or bilateral basal ganglia. In the other case of CNS toxoplasmosis, MR showed multiple nodular and rim enhanced mass lesions in the right basal ganglia, thalamus and periventricular white matter, which were of low signal intensity on T1WI and of high intensity on T2WI. We thus report the related MRI findings

  10. Common genetic variants near the Brittle Cornea Syndrome locus ZNF469 influence the blinding disease risk factor central corneal thickness.

    Yi Lu

    2010-05-01

    Full Text Available Central corneal thickness (CCT, one of the most highly heritable human traits (h(2 typically>0.9, is important for the diagnosis of glaucoma and a potential risk factor for glaucoma susceptibility. We conducted genome-wide association studies in five cohorts from Australia and the United Kingdom (total N = 5058. Three cohorts were based on individually genotyped twin collections, with the remaining two cohorts genotyped on pooled samples from singletons with extreme trait values. The pooled sample findings were validated by individual genotyping the pooled samples together with additional samples also within extreme quantiles. We describe methods for efficient combined analysis of the results from these different study designs. We have identified and replicated quantitative trait loci on chromosomes 13 and 16 for association with CCT. The locus on chromosome 13 (nearest gene FOXO1 had an overall meta-analysis p-value for all the individually genotyped samples of 4.6x10(-10. The locus on chromosome 16 was associated with CCT with p = 8.95x10(-11. The nearest gene to the associated chromosome 16 SNPs was ZNF469, a locus recently implicated in Brittle Cornea Syndrome (BCS, a very rare disorder characterized by abnormal thin corneas. Our findings suggest that in addition to rare variants in ZNF469 underlying CCT variation in BCS patients, more common variants near this gene may contribute to CCT variation in the general population.

  11. Comparative care and outcomes for acute coronary syndromes in Central and Eastern European Transitional countries: A review of the literature.

    Smith, Fraser G D; Brogan, Richard A; Alabas, Oras; Laut, Kristina G; Quinn, Tom; Bugiardini, Raffaele; Gale, Chris P

    2015-12-01

    The purpose of this review was to compare quality of care and outcomes following acute coronary syndrome (ACS) in Central and Eastern European Transitional (CEET) countries. This was a review of original ACS articles in CEET countries from PubMed, ISI Web of Science, Medline and Embase databases published in English from November 2003 to February 2014. Seventeen manuscripts fulfilled the search criteria. Of 19 CEET countries studied, there were no published ACS management or outcome data for four countries. In-hospital mortality for patients with acute myocardial infarction (AMI) ranged from 6.3% in the Czech Republic to 15.3% in Latvia. In-hospital mortality for ST-elevation myocardial infarction (STEMI) ranged from 3.0% in Poland to 20.7% in Romania. For STEMI, primary percutaneous coronary intervention (PCI) ranged from 1.0% to over 92.0%, fibrinolytic therapy from 0.0% to 49.6%, and no reperfusion therapy from 7.0% to 63.0%. Many CEET countries do not have published ACS care and outcomes data. Of those that do, there is evidence for substantial geographical variation in early mortality. Wide variation in emergency reperfusion strategies for STEMI suggests that acute cardiac care is likely to be modifiable and if addressed could reduce mortality from ACS in CEET countries. The collection of ACS care and outcomes data across Europe must be prioritised. © The European Society of Cardiology 2014.

  12. A new frailty syndrome: central obesity and frailty in older adults with the human immunodeficiency virus.

    Shah, Krupa; Hilton, Tiffany N; Myers, Lauren; Pinto, Jonathan F; Luque, Amneris E; Hall, William J

    2012-03-01

    To evaluate the relationships between body composition and physical frailty in community-dwelling older adults with the human immunodeficiency virus (HIV) (HOA). Cross-sectional. Academic hospital-based infectious disease clinic in Rochester, New York. Forty community-dwelling HOA aged 50 and older undergoing antiretroviral therapy who were able to ambulate without assistive devices with a mean age of 58, a mean BMI of 29.0 kg/m(2), mean CD4 count of 569 cells/mL, and a mean duration since HIV diagnosis of 17 years; 28% were female and 57% Caucasian. Subjective and objective measures of functional status were evaluated using the Physical Performance Test (PPT), the graded treadmill test, knee strength, gait speed, balance, and the Functional Status Questionnaire (FSQ). Body composition was evaluated using dual-energy X-ray absorptiometry (DXA) and magnetic resonance imaging (MRI). Sixty percent (25/40) of the participants met standard criteria for physical frailty. Frail (FR) and nonfrail (NF) participants were comparable in age, sex, CD4 count, and viral load. FR HOA had greater impairments in PPT, peak oxygen uptake, FSQ, walking speed, balance, and muscle quality than NF HOA. FR HOA had a greater body mass index (BMI), fat mass, and truncal fat with lipodystrophy. Moreover, PPT score was inversely related to trunk fat (correlation coefficient (r) = -0.34; P = .04) and ratio of intermuscular fat to total fat (r = -0.60; P = .02) after adjusting for covariates. HOA represent an emerging cohort of older adults who frequently experience frailty at a much younger age than the general older population. Central obesity and fat redistribution are important predictors of frailty in community-dwelling HOA. These findings suggest that physical frailty in HOA may be amenable to lifestyle interventions, especially exercise and diet therapy. © 2012, Copyright the Authors Journal compilation © 2012, The American Geriatrics Society.

  13. 22q11.2q13 duplication including SOX10 causes sex-reversal and peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease.

    Falah, Nadia; Posey, Jennifer E; Thorson, Willa; Benke, Paul; Tekin, Mustafa; Tarshish, Brocha; Lupski, James R; Harel, Tamar

    2017-04-01

    Diagnosis of genetic syndromes may be difficult when specific components of a disorder manifest at a later age. We present a follow up of a previous report [Seeherunvong et al., (2004); AJMGA 127: 149-151], of an individual with 22q duplication and sex-reversal syndrome. The subject's phenotype evolved to include peripheral and central demyelination, Waardenburg syndrome type IV, and Hirschsprung disease (PCWH; MIM 609136). DNA microarray analysis defined the duplication at 22q11.2q13, including SOX10. Sequencing of the coding region of SOX10 did not reveal any mutations. Our data suggest that SOX10 duplication can cause disorders of sex development and PCWH, supporting the hypothesis that SOX10 toxic gain of function rather than dominant negative activity underlies PCWH. © 2017 Wiley Periodicals, Inc.

  14. An algorithmic programming approach for back pain symptoms in failed back surgery syndrome using spinal cord stimulation with a multicolumn surgically implanted epidural lead: a multicenter international prospective study.

    Rigoard, Philippe; Jacques, Line; Delmotte, Alexandre; Poon, Katherine; Munson, Russell; Monlezun, Olivier; Roulaud, Manuel; Prevost, Audrey; Guetarni, Farid; Bataille, Benoit; Kumar, Krishna

    2015-03-01

    Many studies have demonstrated the efficacy and the medical/economic value of epidural spinal cord stimulation for the treatment of "failed back surgery syndrome" (FBSS). However, the back pain component of FBSS has been recalcitrant. Recent clinical trials have suggested that multicolumn surgically implanted leads combined with enhanced programming capabilities in the newer implantable pulse generators demonstrate the ability to treat the back pain component of FBSS. The objective of our present international multicentre study is to prospectively evaluate these findings in a larger population. We conducted a prospective, nonrandomized, observational study on 76 patients with refractory FBSS, consecutively implanted with multicolumn spinal cord stimulation (SCS) between 2008 and 2011 in three neurosurgical pain management centers (Poitiers, France; Montréal, Canada; and Regina, Canada). The primary objective of this study was to prospectively analyze the effect of multicolumn lead programming on paresthesia coverage for the back pain region in these patients. The secondary objective was to assess the analgesic efficacy of this technique on the global and back pain components. Paresthesia could be induced in the lower extremities in the majority of patients with at least one of the configurations tested. Bilateral low back paresthesia was induced in 53.5% of patients, while unilateral low back paresthesia was induced in 78.9% of patients. Multicolumn configurations were statistically more effective than monocolumn configurations for all anatomic regions studied. At 6 months, 75.4% of patients receiving multicolumn stimulation (n = 57) obtained at least a 30% improvement of the back pain VAS score, while 42.1% of patients obtained at least a 50% improvement of the back pain VAS score. This study confirms the hypothesis that multicolumn SCS should be considered as an important tool in the treatment of radicular and axial pain in FBSS patients. The efficacy of this

  15. Remyelination of the injured spinal cord

    Sasaki, Masanori; Li, Bingcang; Lankford, Karen L.; Radtke, Christine; Kocsis, Jeffery D.

    2008-01-01

    Contusive spinal cord injury (SCI) can result in necrosis of the spinal cord, but often long white matter tracts outside of the central necrotic core are demyelinated. One experimental strategy to improve functional outcome following SCI is to transplant myelin-forming cells to remyelinate these axons and improve conduction. This review focuses on transplantation studies using olfactory ensheathing cell (OEC) to improve functional outcome in experimental models of SCI and demyelination. The biology of the OEC, and recent experimental research and clinical studies using OECs as a potential cell therapy candidate are discussed. PMID:17618995

  16. Predictors of pain relief following spinal cord stimulation in chronic back and leg pain and failed back surgery syndrome: a systematic review and meta-regression analysis.

    Taylor, Rod S; Desai, Mehul J; Rigoard, Philippe; Taylor, Rebecca J

    2014-07-01

    We sought to assess the extent to which pain relief in chronic back and leg pain (CBLP) following spinal cord stimulation (SCS) is influenced by patient-related factors, including pain location, and technology factors. A number of electronic databases were searched with citation searching of included papers and recent systematic reviews. All study designs were included. The primary outcome was pain relief following SCS, we also sought pain score (pre- and post-SCS). Multiple predictive factors were examined: location of pain, history of back surgery, initial level of pain, litigation/worker's compensation, age, gender, duration of pain, duration of follow-up, publication year, continent of data collection, study design, quality score, method of SCS lead implant, and type of SCS lead. Between-study association in predictive factors and pain relief were assessed by meta-regression. Seventy-four studies (N = 3,025 patients with CBLP) met the inclusion criteria; 63 reported data to allow inclusion in a quantitative analysis. Evidence of substantial statistical heterogeneity (P regression analysis showed no predictive patient or technology factors. SCS was effective in reducing pain irrespective of the location of CBLP. This review supports SCS as an effective pain relieving treatment for CBLP with predominant leg pain with or without a prior history of back surgery. Randomized controlled trials need to confirm the effectiveness and cost-effectiveness of SCS in the CLBP population with predominant low back pain. © 2013 The Authors Pain Practice Published by Wiley Periodicals, Inc. on behalf of World Institute of Pain.

  17. Predictors of Pain Relief Following Spinal Cord Stimulation in Chronic Back and Leg Pain and Failed Back Surgery Syndrome: A Systematic Review and Meta-Regression Analysis

    Taylor, Rod S; Desai, Mehul J; Rigoard, Philippe; Taylor, Rebecca J

    2014-01-01

    We sought to assess the extent to which pain relief in chronic back and leg pain (CBLP) following spinal cord stimulation (SCS) is influenced by patient-related factors, including pain location, and technology factors. A number of electronic databases were searched with citation searching of included papers and recent systematic reviews. All study designs were included. The primary outcome was pain relief following SCS, we also sought pain score (pre- and post-SCS). Multiple predictive factors were examined: location of pain, history of back surgery, initial level of pain, litigation/worker's compensation, age, gender, duration of pain, duration of follow-up, publication year, continent of data collection, study design, quality score, method of SCS lead implant, and type of SCS lead. Between-study association in predictive factors and pain relief were assessed by meta-regression. Seventy-four studies (N = 3,025 patients with CBLP) met the inclusion criteria; 63 reported data to allow inclusion in a quantitative analysis. Evidence of substantial statistical heterogeneity (P pain relief following SCS was noted. The mean level of pain relief across studies was 58% (95% CI: 53% to 64%, random effects) at an average follow-up of 24 months. Multivariable meta-regression analysis showed no predictive patient or technology factors. SCS was effective in reducing pain irrespective of the location of CBLP. This review supports SCS as an effective pain relieving treatment for CBLP with predominant leg pain with or without a prior history of back surgery. Randomized controlled trials need to confirm the effectiveness and cost-effectiveness of SCS in the CLBP population with predominant low back pain. PMID:23834386

  18. Spinal Cord Injury 101

    Full Text Available ... spinal cord injuries? play_arrow What is “Braingate” research? play_arrow How would stem-cell therapies work ... cord injuries? play_arrow What does stem-cell research on animals tell us? play_arrow When can ...

  19. Spinal Cord Injury 101

    Full Text Available ... Cord Injury Allen Heinemann, PhD How Peer Counseling Works Julie Gassaway, MS, RN Pediatric Injuries Pediatric Spinal ... injury? play_arrow How does the spinal cord work? play_arrow Why is the level of a ...

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  1. Spinal Cord Injury 101

    Full Text Available ... Spinal Cord Injury Guy W. Fried, MD Substance Abuse and Spinal Cord Injury Allen Heinemann, PhD How ... arrow Why are high-dose steroids often used right after an injury? play_arrow What is meant ...

  2. The prevalence of the metabolic syndrome did not increase in Mexico City between 1990-1992 and 1997-1999 despite more central obesity.

    Lorenzo, Carlos; Williams, Ken; Gonzalez-Villalpando, Clicerio; Haffner, Steven M

    2005-10-01

    Trends in the metabolic syndrome might follow trends in obesity. We examined this hypothesis in the Mexico City Diabetes Study (MCDS), a study that showed rising trends in obesity, and the effect of the metabolic syndrome on the risk of coronary heart disease (CHD). Designed as a population-based study, the MCDS enrolled subjects in 1990-1992 (n = 2,282). Follow-up visits were held in 1993-1995 (n = 1,764) and 1997-1999 (n = 1,754). We used the revised metabolic syndrome definition of the National Cholesterol Education Program and the Framingham equations to estimate the 10-year CHD risk. In men, the age-adjusted prevalence of the metabolic syndrome was 38.9% in 1990-1992, 43.4% in 1993-1995, and 39.9% in 1997-1999; in women, the prevalences were 65.4, 65.7, and 59.9%, respectively. The prevalence did not change in men (P = 0.349) between 1990-1992 and 1997-1999, but decreased in women (P metabolic syndrome nor CHD risk has increased in Mexico City. Lower blood pressure and triglyceride values appear to have counteracted increases in central obesity and fasting glucose.

  3. Genetics Home Reference: Meckel syndrome

    ... when a structure called the neural tube, a layer of cells that ultimately develops into the brain and spinal cord, fails to close completely during the first few weeks of embryonic development. Meckel syndrome can also cause problems with ...

  4. Primary multifocal gliosarcoma of the spinal cord

    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.

  5. The Cerebral Cost of Breathing: An fMRI Case-Study in Congenital Central Hypoventilation Syndrome

    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

  6. Sonographic findings of normal newborn spinal cord

    Park, Chan Sup; Kim, Dong Gyu

    1988-01-01

    The authors performed spinal cord ultrasonography of 21 healthy newborn infants in Gyeongsang National University Hospital. Normal spinal cord revealed low echogenecity at that of cerebrospinal fluid and was demarcated by intense reflections from its dorsal and ventral surfaces. The central canal was routinely seen as a thin linear reflection in the center of the cord. The nerve roots making up the cauda equina formed a poorly defined collection of intense linear echoes extending from the conus. On real time image, the normal spinal cord exhibited rather slow and rhythmical anteroposterior movement within the subarachnoid fluid. A distinct and rapid vascular pulsation of the spinal cord was usually recognizable. The approximate level of vertebral bodies was determined as follows; most ventrally located vertebral body was thought to be L5 and S1 was seen slightly posterior to the L5 directed inferoposteriorly. According to the above criteria terminal portions of spinal cord were seen around the L2 body in 5 MHz and pointed termination of conus medullaris was clearly seen at L2-3 junction and in upper body of L3 by 7.5 MHz. So it would be better to examine by 5 MHz for spatial orientation and then by 7.5 MHz for more accurate examination. High-resolution, real-time ultrasonography was a safe, rapid screening technique for evaluation of the spinal cord in infants. Additional applications of spinal sonography may be possible in the evaluation of neonatal syringohydromyelia and meningocele as well as intraspinal cyst localization for possible percutaneous puncture by ultrasound guidance

  7. Radiation effects in brain and spinal cord

    Franke, H.D.; Lierse, W.

    1978-01-01

    Radiation sensitivity of both the brain and spinal cord in prenatal and postnatal stages, in infancy and adult age is represented also in consideration of a combined treatment with methotrexate. In adults, application of important doses of high-energy radiation increases the risk of injurious effects to the central nervous system. If the spinal cord is involved, more than 60% of the radiolesions have a progredient course ending with death. The pathogenesis and disposing factors are referred to, and the incidence of radiation necrosis with regard to age and sex, the degrees of injury and their frequence within different ranges of dosage are analyzed on the basis of data from universal literature. An examination of 'tolerance doses' for the spinal cord is made by means of Strandquist-diagrams and of the Ellis-formula. The slopes of regression lines are reported for various 'degrees of response' in skin, brain and spinal cord following radiation therapy. In the Strandquist-diagram, slopes of regression lines are dependent on the 'degree of response', flattening if skin and spinal cord are affected by radiation in the same degree, necroses having the same slope for both the organs. (orig./MG) [de

  8. Regional differences in radiosensitivity across the rat cervical spinal cord

    Bijl, Hendrik P.; Luijk, Peter van; Coppes, Rob P.; Schippers, Jacobus M.; Konings, Antonius W.T.; Kogel, Albert J. van der

    2005-01-01

    Purpose: To study regional differences in radiosensitivity within the rat cervical spinal cord. Methods and materials: Three types of inhomogeneous dose distributions were applied to compare the radiosensitivity of the lateral and central parts of the rat cervical spinal cord. The left lateral half of the spinal cord was irradiated with two grazing proton beams, each with a different penumbra (20-80% isodoses): lateral wide (penumbra = 1.1 mm) and lateral tight (penumbra = 0.8 mm). In the third experiment, the midline of the cord was irradiated with a narrow proton beam with a penumbra of 0.8 mm. The irradiated spinal cord length (CT-2) was 20 mm in all experiments. The animals were irradiated with variable single doses of unmodulated protons (150 MeV) with the shoot-through method, whereby the plateau of the depth-dose profile is used rather than the Bragg peak. The endpoint for estimating isoeffective dose (ED 50 ) values was paralysis of fore and/or hind limbs within 210 days after irradiation. Histology of the spinal cords was performed to assess the radiation-induced tissue damage. Results: High-precision proton irradiation of the lateral or the central part of the spinal cord resulted in a shift of dose-response curves to higher dose values compared with the homogeneously irradiated cervical cord to the same 20-mm length. The ED 50 values were 28.9 Gy and 33.4 Gy for the lateral wide and lateral tight irradiations, respectively, and as high as 71.9 Gy for the central beam experiment, compared with 20.4 Gy for the homogeneously irradiated 20-mm length of cervical cord. Histologic analysis of the spinal cords showed that the paralysis was due to white matter necrosis. The radiosensitivity was inhomogeneously distributed across the spinal cord, with a much more radioresistant central white matter (ED 50 = 71.9 Gy) compared with lateral white matter (ED 50 values = 28.9 Gy and 33.4 Gy). The gray matter did not show any noticeable lesions, such as necrosis or

  9. Efficacy of transverse tripolar spinal cord stimulator for the relief of chronic low back pain from failed back surgery.

    Buvanendran, Asokumar; Lubenow, Timothy J

    2008-01-01

    Failed back surgery syndrome is a common clinical entity for which spinal cord stimulation has been found to be an effective mode of analgesia, but with variable success rates. To determine if focal stimulation of the dorsal columns with a transverse tripolar lead might achieve deeper penetration of the electrical stimulus into the spinal cord and therefore provide greater analgesia to the back. Case report. We describe a 42-year-old female with failed back surgery syndrome that had greater back pain than leg pain. The tripolar lead configuration was achieved by placing percutaneously an octapolar lead in the spinal midline followed by 2 adjacent quadripolar leads, advanced to the T7-T10 vertebral bodies. Tripolar stimulation pattern resulted in more than 70% pain relief in this patient during the screening trial, while stimulation of one or 2 electrodes only provided 20% pain relief. After implantation of a permanent tripolar electrode system with a single rechargeable battery, the pain relief was maintained for one year. This is case report describing a case of a patient with chronic low back pain with a diagnosis of failed back surgery syndrome in which transverse tripolar stimulation using an octapolar and 2 quadripolar leads appeared to be beneficial. The transverse tripolar system consists of a central cathode surrounded by anodes, using 3 leads. This arrangement may contribute to maximum dorsal column stimulation with minimal dorsal root stimulation and provide analgesia to the lower back.

  10. Distribution of Wfs1 protein in the central nervous system of the mouse and its relation to clinical symptoms of the Wolfram syndrome

    Luuk, H.; Koks, S.; Plaas, M.

    2008-01-01

    enrichment of Wf1 protein in the central extended amygdala and ventral striatum. Prominent Wfs1 expression was seen in the hippocampal CA1 region, parasubiculum, superficial part of the second and third layers of the prefrontal cortex and proisocortical areas, hypothalamic magnocellular neurosecretory system......, alveus, fimbria, dorsal hippocampal commissure; subiculum, and to a lesser extent in the central sublenticular extended amygdala, compact part of substantia nigra, and ventral tegmental area. The neuroanatomical findings suggest that the lack of Wf1 protein function can be related to several neurological...... and psychiatric symptoms found in Wolfram syndrome. Enrichment of Wfs1 protein in the central extended amygdala suggests a role in the modulation of anxiety and fear Udgivelsesdato: 2008/8/20...

  11. Transplantation of spinal cord-derived neural stem cells for ALS: Analysis of phase 1 and 2 trials.

    Glass, Jonathan D; Hertzberg, Vicki S; Boulis, Nicholas M; Riley, Jonathan; Federici, Thais; Polak, Meraida; Bordeau, Jane; Fournier, Christina; Johe, Karl; Hazel, Tom; Cudkowicz, Merit; Atassi, Nazem; Borges, Lawrence F; Rutkove, Seward B; Duell, Jayna; Patil, Parag G; Goutman, Stephen A; Feldman, Eva L

    2016-07-26

    To test the safety of spinal cord transplantation of human stem cells in patients with amyotrophic lateral sclerosis (ALS) with escalating doses and expansion of the trial to multiple clinical centers. This open-label trial included 15 participants at 3 academic centers divided into 5 treatment groups receiving increasing doses of stem cells by increasing numbers of cells/injection and increasing numbers of injections. All participants received bilateral injections into the cervical spinal cord (C3-C5). The final group received injections into both the lumbar (L2-L4) and cervical cord through 2 separate surgical procedures. Participants were assessed for adverse events and progression of disease, as measured by the ALS Functional Rating Scale-Revised, forced vital capacity, and quantitative measures of strength. Statistical analysis focused on the slopes of decline of these phase 2 trial participants alone or in combination with the phase 1 participants (previously reported), comparing these groups to 3 separate historical control groups. Adverse events were mostly related to transient pain associated with surgery and to side effects of immunosuppressant medications. There was one incident of acute postoperative deterioration in neurologic function and another incident of a central pain syndrome. We could not discern differences in surgical outcomes between surgeons. Comparisons of the slopes of decline with the 3 separate historical control groups showed no differences in mean rates of progression. Intraspinal transplantation of human spinal cord-derived neural stem cells can be safely accomplished at high doses, including successive lumbar and cervical procedures. The procedure can be expanded safely to multiple surgical centers. This study provides Class IV evidence that for patients with ALS, spinal cord transplantation of human stem cells can be safely accomplished and does not accelerate the progression of the disease. This study lacks the precision to

  12. Trauma: Spinal Cord Injury.

    Eckert, Matthew J; Martin, Matthew J

    2017-10-01

    Injuries to the spinal column and spinal cord frequently occur after high-energy mechanisms of injury, or with lower-energy mechanisms, in select patient populations like the elderly. A focused yet complete neurologic examination during the initial evaluation will guide subsequent diagnostic procedures and early supportive measures to help prevent further injury. For patients with injury to bone and/or ligaments, the initial focus should be spinal immobilization and prevention of inducing injury to the spinal cord. Spinal cord injury is associated with numerous life-threatening complications during the acute and long-term phases of care that all acute care surgeons must recognize. Published by Elsevier Inc.

  13. Repositioning and Leaving In Situ the Central Venous Catheter During Percutaneous Treatment of Associated Superior Vena Cava Syndrome: A Report of Eight Cases

    Stockx, Luc; Raat, Henricus; Donck, Jan; Wilms, Guy; Marchal, Guy

    1999-01-01

    Purpose: To describe a combined procedure of repositioning and leaving in situ a central venous catheter followed by immediate percutaneous treatment of associated superior vena cava syndrome (SVCS). Methods: Eight patients are presented who have central venous catheter-associated SVCS (n = 6 Hickman catheters, n = 2 Port-a-cath) caused by central vein stenosis (n = 4) or concomitant thrombosis (n = 4). With the use of a vascular snare introduced via the transcubital or transjugular approach, the tip of the central venous catheter could be engaged, and repositioned after deployment of a stent in the innominate or superior vena cava. Results: In all patients it was technically feasible to reposition the central venous catheter and treat the SVCS at the same time. In one patient flipping of the Hickman catheter in its original position provoked dislocation of the released Palmaz stent, which could be positioned in the right common iliac vein. Conclusion: Repositioning of a central venous catheter just before and after stent deployment in SVCS is technically feasible and a better alternative than preprocedural removal of the vascular access

  14. Central sensitivity syndromes: a new paradigm and group nosology for fibromyalgia and overlapping conditions, and the related issue of disease versus illness.

    Yunus, Muhammad B

    2008-06-01

    To discuss the current terminologies used for fibromyalgia syndrome (FMS) and related overlapping conditions, to examine if central sensitivity syndromes (CSS) is the appropriate nosology for these disorders, and to explore the issue of disease versus illness. A literature search was performed through PubMed, Web of Science, and ScienceDirect using a number of keywords, eg, functional somatic syndromes, somatoform disorders, medically unexplained symptoms, organic and nonorganic, and diseases and illness. Relevant articles were then reviewed and representative ones cited. Terminologies currently used for CSS conditions predominantly represent a psychosocial construct and are inappropriate. On the other hand, CSS seems to be the logical nosology based on a biopsychosocial model. Such terms as "medically unexplained symptoms," "somatization," "somatization disorder," and "functional somatic syndromes" in the context of CSS should be abandoned. Given current scientific knowledge, the concept of disease-illness dualism has no rational basis and impedes proper patient-physician communication, resulting in poor patient care. The concept of CSS is likely to promote research, education, and proper patient management. CSS seems to be a useful paradigm and an appropriate terminology for FMS and related conditions. The disease-illness, as well as organic/non-organic dichotomy, should be rejected.

  15. Sleep disordered breathing in spinal cord injury: A systematic review.

    Chiodo, Anthony E; Sitrin, Robert G; Bauman, Kristy A

    2016-07-01

    Spinal cord injury commonly results in neuromuscular weakness that impacts respiratory function. This would be expected to be associated with an increased likelihood of sleep-disordered breathing. (1) Understand the incidence and prevalence of sleep disordered breathing in spinal cord injury. (2) Understand the relationship between injury and patient characteristics and the incidence of sleep disordered breathing in spinal cord injury. (3) Distinguish between obstructive sleep apnea and central sleep apnea incidence in spinal cord injury. (4) Clarify the relationship between sleep disordered breathing and stroke, myocardial infarction, metabolic dysfunction, injuries, autonomic dysreflexia and spasticity incidence in persons with spinal cord injury. (5) Understand treatment tolerance and outcome in persons with spinal cord injury and sleep disordered breathing. Extensive database search including PubMed, Cochrane Library, CINAHL and Web of Science. Given the current literature limitations, sleep disordered breathing as currently defined is high in patients with spinal cord injury, approaching 60% in motor complete persons with tetraplegia. Central apnea is more common in patients with tetraplegia than in patients with paraplegia. Early formal sleep study in patients with acute complete tetraplegia is recommended. In patients with incomplete tetraplegia and with paraplegia, the incidence of sleep-disordered breathing is significantly higher than the general population. With the lack of correlation between symptoms and SDB, formal study would be reasonable. There is insufficient evidence in the literature on the impact of treatment on morbidity, mortality and quality of life outcomes.

  16. Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

    Sethi Prahlad

    2011-01-01

    Full Text Available Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome.

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  1. Spinal cord compression in b-thalassemia: follow-up after radiotherapy

    Silvana Fahel da Fonseca

    Full Text Available CONTEXT: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including b-thalassemia. CASE REPORT: We report the case of a patient with intermediate b-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. DISCUSSION: 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.

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

    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)

  3. Spinal cord compression in β-thalassemia: follow-up after radiotherapy

    Fonseca, Silvana Fahel da; Figueiredo, Maria Stella; Cancado, Rodolfo Delfini; Nakadakare, Fernando; Segreto, Roberto; Kerbauy, Jose

    1998-01-01

    Spinal cord compression due to extramedullary hematopoiesis is a well-described bu rare syndrome encountered in several hematologic disorders, including β-thalassemia. We report a case of a patient with intermediate β-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)

  4. Obesity, Metabolic Syndrome, and Musculoskeletal Disease: Common Inflammatory Pathways Suggest a Central Role for Loss of Muscle Integrity

    Kelsey H. Collins

    2018-02-01

    Full Text Available Inflammation can arise in response to a variety of stimuli, including infectious agents, tissue injury, autoimmune diseases, and obesity. Some of these responses are acute and resolve, while others become chronic and exert a sustained impact on the host, systemically, or locally. Obesity is now recognized as a chronic low-grade, systemic inflammatory state that predisposes to other chronic conditions including metabolic syndrome (MetS. Although obesity has received considerable attention regarding its pathophysiological link to chronic cardiovascular conditions and type 2 diabetes, the musculoskeletal (MSK complications (i.e., muscle, bone, tendon, and joints that result from obesity-associated metabolic disturbances are less frequently interrogated. As musculoskeletal diseases can lead to the worsening of MetS, this underscores the imminent need to understand the cause and effect relations between the two, and the convergence between inflammatory pathways that contribute to MSK damage. Muscle mass is a key predictor of longevity in older adults, and obesity-induced sarcopenia is a significant risk factor for adverse health outcomes. Muscle is highly plastic, undergoes regular remodeling, and is responsible for the majority of total body glucose utilization, which when impaired leads to insulin resistance. Furthermore, impaired muscle integrity, defined as persistent muscle loss, intramuscular lipid accumulation, or connective tissue deposition, is a hallmark of metabolic dysfunction. In fact, many common inflammatory pathways have been implicated in the pathogenesis of the interrelated tissues of the musculoskeletal system (e.g., tendinopathy, osteoporosis, and osteoarthritis. Despite these similarities, these diseases are rarely evaluated in a comprehensive manner. The aim of this review is to summarize the common pathways that lead to musculoskeletal damage and disease that result from and contribute to MetS. We propose the overarching

  5. Obesity, Metabolic Syndrome, and Musculoskeletal Disease: Common Inflammatory Pathways Suggest a Central Role for Loss of Muscle Integrity.

    Collins, Kelsey H; Herzog, Walter; MacDonald, Graham Z; Reimer, Raylene A; Rios, Jaqueline L; Smith, Ian C; Zernicke, Ronald F; Hart, David A

    2018-01-01

    Inflammation can arise in response to a variety of stimuli, including infectious agents, tissue injury, autoimmune diseases, and obesity. Some of these responses are acute and resolve, while others become chronic and exert a sustained impact on the host, systemically, or locally. Obesity is now recognized as a chronic low-grade, systemic inflammatory state that predisposes to other chronic conditions including metabolic syndrome (MetS). Although obesity has received considerable attention regarding its pathophysiological link to chronic cardiovascular conditions and type 2 diabetes, the musculoskeletal (MSK) complications (i.e., muscle, bone, tendon, and joints) that result from obesity-associated metabolic disturbances are less frequently interrogated. As musculoskeletal diseases can lead to the worsening of MetS, this underscores the imminent need to understand the cause and effect relations between the two, and the convergence between inflammatory pathways that contribute to MSK damage. Muscle mass is a key predictor of longevity in older adults, and obesity-induced sarcopenia is a significant risk factor for adverse health outcomes. Muscle is highly plastic, undergoes regular remodeling, and is responsible for the majority of total body glucose utilization, which when impaired leads to insulin resistance. Furthermore, impaired muscle integrity, defined as persistent muscle loss, intramuscular lipid accumulation, or connective tissue deposition, is a hallmark of metabolic dysfunction. In fact, many common inflammatory pathways have been implicated in the pathogenesis of the interrelated tissues of the musculoskeletal system (e.g., tendinopathy, osteoporosis, and osteoarthritis). Despite these similarities, these diseases are rarely evaluated in a comprehensive manner. The aim of this review is to summarize the common pathways that lead to musculoskeletal damage and disease that result from and contribute to MetS. We propose the overarching hypothesis that there

  6. Haematopoietic transplants combining a single unrelated cord blood unit and mobilized haematopoietic stem cells from an adult HLA-mismatched third party donor. Comparable results to transplants from HLA-identical related donors in adults with acute leukaemia and myelodysplastic syndromes.

    Sebrango, Ana; Vicuña, Isabel; de Laiglesia, Almudena; Millán, Isabel; Bautista, Guiomar; Martín-Donaire, Trinidad; Regidor, Carmen; Cabrera, Rafael; Fernandez, Manuel N

    2010-06-01

    We describe results of the strategy, developed by our group, of co-infusion of mobilized haematopoietic stem cells as a support for single-unit unrelated cord blood transplant (dual CB/TPD-MHSC transplants) for treatment of haematological malignancies in adults, and a comparative analysis of results obtained using this strategy and transplants performed with mobilized haematopoietic stem cells from related HLA-identical donors (RTD) for treatment of adults with acute leukaemia and myelodysplastic syndromes. Our data show that the dual CB/TPD-MHSC transplant strategy results in periods of post-transplant neutropenia, final rates of full donor chimerism and transplant-related mortality rates comparable to those of the RTD. Final survival outcomes are comparable in adults transplanted because of acute leukaemia, with different incidences of the complications that most influence these: a higher incidence of infections related to late recovery of protective immunity dependent on T cell functions, and a lower incidence of serious acute graft-versus-host disease and relapses. Recent advances in cord blood transplant techniques allow allogeneic haematopoietic stem cell transplantation (HSCT) to be a viable option for almost every patient who may benefit from this therapeutic approach. Development of innovative strategies to improve the post-transplant recovery of T cells function is currently the main challenge to further improving the possibilities of unrelated cord blood transplantation. Copyright © 2010 Elsevier Ltd. All rights reserved.

  7. BING-NEEL SYNDROME: ILLUSTRATIVE CASES AND COMPREHENSIVE REVIEW OF THE LITERATURE

    Marzia Varettoni

    2017-10-01

    Full Text Available The Bing-Neel syndrome is a rare neurological complication of Waldenström’s Macroglobulinemia which results from a direct involvement of central nervous system by malignant lymphoplasmacytic cells. The clinical suspicion of Bing-Neel syndrome may be difficult because neurologic symptoms are heterogeneous, non specific and sometimes underhand. A definitive diagnosis of Bing-Neel syndrome can be confidently made using brain and spinal cord magnetic resonance imaging as well as histopathology and/or cerebrospinal fluid analysis to confirm the neoplastic infiltration of central nervous system. The detection in the cerebrospinal fluid of patients with Bing-Neel syndrome of the MYD88 (L265P somatic mutation, which is highly recurrent in Waldenström’s Macroglobulinemia, revealed useful for the diagnosis and monitoring of central nervous system involvement. Despite recommendations recently published, there is still no clear consensus on treatment of Bing-Neel syndrome, which includes systemic immunochemotherapy, intrathecal chemotherapy and brain irradiation as possible options. Ibrutinib, a Bruton kinase inhibitor highly active in patients with Waldenström’s Macroglobulinemia, has been recently added to the therapeutic armamentarium of Bing-Neel syndrome due to its ability to pass the blood-brain barrier. However, prospective clinical trials are eagerly awaited with the aim to define the optimal treatment strategy.  Here we describe four illustrative cases of Bing-Neel syndrome diagnosed and treated at our Institution and review the literature on this topic.

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

    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.

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

    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.

  10. Central vestibular syndrome in a red fox (Vulpes vulpes) with presumptive right caudal cerebral artery ischemic infarct and prevalent midbrain involvement.

    Ricciardi, Mario; Gernone, Floriana; Simone, Antonio De; Giannuzzi, Pasquale

    2017-01-01

    A wild young male red fox ( Vulpes vulpes ) was found in the mountainous hinterland of Rome (Italy) with a heavily depressed mental status and unresponsive to the surrounding environment. Neurological examination revealed depression, left circling, right head tilt, ventromedial positional strabismus and decreased postural reactions on the left side. Neurological abnormalities were suggestive of central vestibular syndrome. Two consecutive MRIs performed with 30 days interval were compatible with lacunar ischemic infarct in the territory of right caudal cerebral artery and its collateral branches. The lesion epicentre was in the right periaqueductal portion of the rostral mesencephalic tegmentum. Neuroanatomical and neurophysiological correlation between lesion localization and clinical presentation are discussed.

  11. Central vestibular syndrome in a red fox (Vulpes vulpes with presumptive right caudal cerebral artery ischemic infarct and prevalent midbrain involvement

    Mario Ricciardi

    2017-06-01

    Full Text Available A wild young male red fox (Vulpes vulpes was found in the mountainous hinterland of Rome (Italy with a heavily depressed mental status and unresponsive to the surrounding environment. Neurological examination revealed depression, left circling, right head tilt, ventromedial positional strabismus and decreased postural reactions on the left side. Neurological abnormalities were suggestive of central vestibular syndrome. Two consecutive MRIs performed with 30 days interval were compatible with lacunar ischemic infarct in the territory of right caudal cerebral artery and its collateral branches. The lesion epicentre was in the right periaqueductal portion of the rostral mesencephalic tegmentum. Neuroanatomical and neurophysiological correlation between lesion localization and clinical presentation are discussed.

  12. Magnetic resonance imaging of experimental spinal cord injury

    Nakamura, Tsutomu; Kumano, Kouichi; Kadoya, Satoru

    1989-01-01

    Correlation between pathological findings and magnetic resonance imaging (MRI) of experimental cord injury were investigated. Cord injuries were made on ten Wistar rats weighing 80-170 gm by epidural compression of the thoracic cord with a Biemer cerebral vascular clip for 5-20 seconds. Several hours after the procedure animals were examined by spin echo axial MR images with a pulse sequence of TR/TE=1000/36 msec. MR studies were repeated on 4 animals 3-7 days after the initial examination. Immediately after the latest MRI examination animals were sacrificed and fixed with 10% formalin. Three micron thickness paraffin sections stained with hematoxylin and eosin were evaluated under a microscope. The pathological finding was hemorrhagic necrosis with edema of various severity depending on duration of clip application. The hemorrhagic necrosis was observed either unilaterally or bilaterally to the cord. MR findings of the cord were of high intensity in five animals which were severely injured, while central low intensity of the injured cord appeared in three mildly injured animals. Of the remaining two animals which had mild injury, one showed unilateral high intensity, while no definitive change was demonstrated in the other. The high intensity in the MRI suggested edema associated with hemorrhagic necrosis rather than hemorrhage. The central low intensity appearing in the mildly injured cord might be hemorrhage in the gray matter. It is concluded that MRI was useful to diagnose not only the level and severity but also the pathological process in the injured cord, and thus to estimate the prognosis of the cord injuries. (author)

  13. Is "Delayed Umbilical Cord Clamping" Beneficial for Premature Newborns?

    Amir-Mohammad Armanian

    2017-05-01

    Full Text Available Background: The appropriate moment for clamping the umbilical cord is controversial. Immediate cord clamping (ICC is an item of active management of the third stage of labor (AMTSL. Unclamped umbilical cord may cause inconvenience in preterm neonates because they commonly need some levels of emergent services. Some studies revealed delayed cord clamping (DCC of preterm neonates results in better health conditions like lower rates of respiratory distress syndrome (RDS, less morbidities in labor room and lower risk of postpartum hemorrhage. The aim of the present study was to determine the effect of delayed umbilical cord clamping on premature neonatal outcomes. Materials and Methods: In this single‑center randomized control trial study, sixty premature neonates (gestational age ≤ 34 weeks were randomly assigned to ICC (cord clamped at 5–10 seconds or DCC (30–45 seconds groups and followed up in neonatal intensive care unit (NICU. Primary outcomes were 1st and 5th minute Apgar score, average of level of hematocrit after birth, intra ventricle hemorrhage and need some levels of resuscitation. Results: Differences in demographic characteristics were not statistically significant. After birth, neonates who had delayed clamping had significantly higher mean hematocrit after at 4-hour of birth (49.58+5.15gr/dl vs. 46.58+5.40gr/dlin DCC vs. ICC groups, respectively (P=0.031. Delayed cord clamping reduced the duration of need to nasal continues positive airway pressure (NCPAP (86.7% and 60.0% in ICC and DCC groups, respectively, P= 0.039. Attractively, the results showed lower incidence of clinical sepsis in delayed cord clamping neonates (53.3% vs. 23.3% in ICC and DCC groups, respectively, P=0.033. Conclusion: Prematurity complications might decrease by delay umbilical cord clamping which improve the hematocrit, duration of need to NCPAP and incidence of clinical sepsis. Furthermore, DCC may have no negative impact on neonatal resuscitation.

  14. Food Insecurity and Its Association With Central Obesity and Other Markers of Metabolic Syndrome Among Persons Aged 12 to 18 Years in the United States.

    Holben, David H; Taylor, Christopher A

    2015-09-01

    Food insecurity is a preventable health threat and may precipitate central obesity and metabolic syndrome in children and adolescents in the United States. To examine (1) health by household food security status; and (2) differences and prevalence of central obesity among persons aged 12 to 18 years in the United States. The National Health and Nutrition Examination Survey was administered to a cross-sectional sample of persons aged 12 to 18 years in 1999 to 2006. Controlling for age, race/ethnicity, and sex differences in mean obesity and chronic disease factors across levels of food insecurity (analysis of covariance [Bonferroni post hoc] and ORs [logistic regression analyses]) were examined, as were differences in the rates of risk factors (χ(2) statistics). A total of 7435 participants were analyzed. Those from marginally food secure (n=751) and low-food secure (n=1206) (population size estimate, 26,714,182) households were significantly more likely than their high-food secure counterparts (n=4831) to be overweight (P=.036) (OR, 1.44), and those from marginally food secure households were 1.3-times more likely to be obese (P=.036). Nearly 25% of respondents from marginally food secure, low-food secure, and very low-food secure (n=647) households reported central obesity (P=.002), which was 1.4 to 1.5 times more likely than those from high-food secure households. Participants from high-food secure households had significantly higher mean high-density lipoprotein values (P=.019). Risk factors indicative of metabolic syndrome were present in 3.1%. Household food insecurity was associated with an increased likelihood of being overweight and having central obesity. Limitations included the use of cross-sectional data and some self-reported data and the inability to control for all moderating variables in obesity and overall health status.

  15. Cranial nerve vascular compression syndromes of the trigeminal, facial and vago-glossopharyngeal nerves: comparative anatomical study of the central myelin portion and transitional zone; correlations with incidences of corresponding hyperactive dysfunctional syndromes.

    Guclu, Bulent; Sindou, Marc; Meyronet, David; Streichenberger, Nathalie; Simon, Emile; Mertens, Patrick

    2011-12-01

    The aim of this study was to evaluate the anatomy of the central myelin portion and the central myelin-peripheral myelin transitional zone of the trigeminal, facial, glossopharyngeal and vagus nerves from fresh cadavers. The aim was also to investigate the relationship between the length and volume of the central myelin portion of these nerves with the incidences of the corresponding cranial dysfunctional syndromes caused by their compression to provide some more insights for a better understanding of mechanisms. The trigeminal, facial, glossopharyngeal and vagus nerves from six fresh cadavers were examined. The length of these nerves from the brainstem to the foramen that they exit were measured. Longitudinal sections were stained and photographed to make measurements. The diameters of the nerves where they exit/enter from/to brainstem, the diameters where the transitional zone begins, the distances to the most distal part of transitional zone from brainstem and depths of the transitional zones were measured. Most importantly, the volume of the central myelin portion of the nerves was calculated. Correlation between length and volume of the central myelin portion of these nerves and the incidences of the corresponding hyperactive dysfunctional syndromes as reported in the literature were studied. The distance of the most distal part of the transitional zone from the brainstem was 4.19  ±  0.81 mm for the trigeminal nerve, 2.86  ±  1.19 mm for the facial nerve, 1.51  ±  0.39 mm for the glossopharyngeal nerve, and 1.63  ±  1.15 mm for the vagus nerve. The volume of central myelin portion was 24.54  ±  9.82 mm(3) in trigeminal nerve; 4.43  ±  2.55 mm(3) in facial nerve; 1.55  ±  1.08 mm(3) in glossopharyngeal nerve; 2.56  ±  1.32 mm(3) in vagus nerve. Correlations (p  nerves and incidences of the corresponding diseases. At present it is rather well-established that primary trigeminal neuralgia, hemifacial spasm and vago

  16. Pregnancy Complications: Umbilical Cord Abnormalities

    ... Umbilical cord abnormalities Umbilical cord abnormalities Now playing: E-mail to a friend Please fill in all fields. ... blood supply) to the baby. The two arteries transport waste from the baby to the placenta (where ...

  17. Arteriovenous malformations of the cervical spinal cord

    Nagasawa, Shiro; Yoshida, Shinzo; Ishikawa, Masatsune; Yonekawa, Yasuhiro; Handa, Hajime

    1984-01-01

    Arteriovenous malformation (AVM) of the cervical spinal cord has been known to constitute 5-13% of all spinal AVMs. In contrast to the AVMs located in thoracic or thoraco-lumbar regions, cervical AVM has several characteristic features such as preponderance in younger generation, high incidence of subarachnoid hemorrhage, intramedullary location of the nidus usually fed by the anterior spinal arterial system. We reported three cases of cervical AVMs, which located intramedullary at the levels of C 4 -C 6 , C 1 -C 4 and C 1 -C 2 , respectively. Although selective angiography (vertebral artery, thyrocervical artery, costocervical artery) was essential for the diagnosis of these lesions, computerized tomographic (CT) study with both intrathecal injection of metrizamide and intravenous infusion of contrast material (dynamic and static study) was found to be extremely advantageous in detecting the topography of AVMs in the concerned horizontal planes of the spinal cord. Removal of AVM was given up in one case because of its possible involvement of the anterior spinal artery and central artery shown by CT scan. Removal of AVMs were performed in other two cases. A lateral approach was tried in one case with the AVM located in C 1 -C 2 level, in which CT scan revealed not only an intramedullary but the associated extramedullary AVM in ventrolateral surface of the spinal cord. This operative approach was found to involve less bone removal and markedly reduce spinal cord manipulation necessary to deal with ventrally situated high cervical lesions, compared with a posterior approach with laminectomy. (author)

  18. The peripheral and central mechanisms of transition of acute to chronic pain and the possible role of cyclooxygenase-2 inhibition in the prevention of pain syndrome chronization

    O. S. Davydov

    2016-01-01

    Full Text Available Chronic pain syndromes as a cause of suffering, short-term or persistent disability, and social losses greatly worsen quality of life. The mechanisms leading to the occurrence and maintenance of chronic pain are traditionally of interest for in-depth study since each of them is potentially a target for pharmacotherapy. Peripheral and central sensitizations, as well as disinhibition make different contributions to the development of chronic pain. The fact that cyclooxygenase-2 (COX-2 inhibitors may affect at both the peripheral and central, spinal levels, by modulating such a phenomenon as central sensitization, has been recently discussed. There are theoretical prerequisites for a discussion of this action of COX-2 inhibitors; however, clinical findings supporting this hypothesis have been scarce so far. In this connection, of interest is the clinical trial published in 2016, which may suggest to a high degree of accuracy that some analgesic effect of the selective COX-2 inhibitor etoricoxib is realized through the central mechanisms of pain modulation. 

  19. Epstein-Barr virus-associated primary central nervous system lymphoma in a child with the acquired immunodeficiency syndrome. A case report and review of the literature.

    Rodriguez, M M; Delgado, P I; Petito, C K

    1997-12-01

    A 34-month-old black boy who had contracted acquired immunodeficiency syndrome from his mother presented with fever, vomiting, and cough. He was cachectic, hypertonic, and developmentally delayed. A brain computed tomography scan revealed masses in the left frontal horn, subependymal, and periventricular regions; secondary edema; and hydrocephalus. The differential diagnosis was cerebral lymphoma versus toxoplasmosis. The patient had disseminated Mycobacterium avium-intracellulare infection, lymphoid interstitial pneumonitis, as well as Pseudomonas and Klebsiella pneumonia. He died of respiratory insufficiency 53 days after admission. The autopsy confirmed a primary cerebral B-cell lymphoma, large cell type, which was positive for Epstein-Barr virus, latent phase, by in situ hybridization. Primary central nervous system lymphomas are rare in children, in contrast to adults. To our knowledge, only five well-documented cases of primary cerebral lymphomas in infants and children with acquired immunodeficiency syndrome have been reported previously. The current study shows that these childhood lymphomas are associated with and presumably caused by Epstein-Barr virus and thus have a pathogenesis similar to that of primary central nervous system lymphomas in adults.

  20. Radiation nephritis causing nephrotic syndrome

    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.

  1. Localization of Brain Natriuretic Peptide Immunoreactivity in Rat Spinal Cord

    Essam M Abdelalim

    2016-12-01

    Full Text Available Brain natriuretic peptide (BNP exerts its functions through natriuretic peptide receptors. Recently, BNP has been shown to be involved in a wide range of functions. Previous studies reported BNP expression in the sensory afferent fibers in the dorsal horn of the spinal cord. However, BNP expression and function in the neurons of the central nervous system are still controversial. Therefore, in this study, we investigated BNP expression in the rat spinal cord in detail using RT-PCR and immunohistochemistry. RT-PCR analysis showed that BNP mRNA was present in the spinal cord and DRG. BNP immunoreactivity was observed in different structures of the spinal cord, including the neuronal cell bodies and neuronal processes. BNP immunoreactivity was observed in the dorsal horn of the spinal cord and in the neurons of the intermediate column and ventral horn. Double-immunolabeling showed a high level of BNP expression in the afferent fibers (laminae I-II labeled with calcitonin gene-related peptide (CGRP, suggesting BNP involvement in sensory function. In addition, BNP was co-localized with CGRP and choline acetyltransferase in the motor neurons of the ventral horn. Together, these results indicate that BNP is expressed in sensory and motor systems of the spinal cord, suggesting its involvement in several biological actions on sensory and motor neurons via its binding to NPR-A and/or NPR-B in the DRG and spinal cord.

  2. Prevalence of central sleep apnea during continous positive airway pressure (CPAP) titration in subjects with obstructive sleep apnea syndrome at an altitude of 2640 m.

    Bazurto Zapata, Maria Angelica; Martinez-Guzman, William; Vargas-Ramirez, Leslie; Herrera, Karen; Gonzalez-Garcia, Mauricio

    2015-03-01

    The occurrence of central apneas when applying positive pressure (CPAP) to patients with obstructive sleep apnea syndrome (OSAS) is called complex sleep apnea (CompSA). This causes poor adherence to CPAP and persistence of symptoms. In Bogota, a city located at an altitude of 2640 m above sea level, chronic hypoxemia can generate certain instability of the respiratory system during sleep which could increase the presence of central apnea. The aim was to establish the prevalence of central apnea (central apnea index >5/h) in adults with moderate or severe OSAS during CPAP titration, and the factors associated with this. Patients over 18 years old with OSAS were referred to the Fundacion Neumologica Colombiana Sleep Center, from January 2008 to June 2010. Polysomnogram (PSG) for CPAP titration was performed according to the American Academy of Sleep Medicine criteria. The prevalence was calculated and the clinical and baseline PSG factors associated with the CompSA were analyzed. We included 988 patients, 58% men. CompSA prevalence was 11.6%. Factors associated with CompSA were: central apneas in the baseline PSG (OR: 5.34 [3.49-8.16]), history of heart failure (OR: 2.53 [1.58-4.07]), and male sex (OR: 1.68 [1.06-2.69]). The prevalence of complex sleep apnea in Bogota (11.6%) was intermediate compared to the reported in lower altitudes. The factors associated with the development of CompSA were male sex, heart failure, and the presence of central apnea in the baseline PSG. Copyright © 2014 Elsevier B.V. All rights reserved.

  3. Increased central immunoreactive beta-endorphin content in patients with Wernicke-Korsakoff syndrome and in alcoholics.

    Summers, J A; Pullan, P T; Kril, J J; Harper, C G

    1991-01-01

    beta-endorphin, adrenocorticotrophin, and alpha-melanocyte stimulating hormone were measured by radioimmunoassay in three areas of human brain at necropsy in seven subjects with Wernicke-Korsakoff syndrome and in 52 controls. Thiamin concentration in six brain areas was also measured. Mamillary body beta-endorphin concentrations were significantly increased in those with the syndrome compared with controls, and those controls with high alcohol intake showed increased mamillary body beta-endorphin compared with controls with low alcohol intake. Brain thiamin concentration was similar in both groups, with the exception of the brainstem, where it was reduced in subjects with Wernicke-Korsakoff syndrome. Thalamic beta-endorphin in controls was inversely correlated with thiamin in frontal white matter, frontal cortex, parietal white matter and parietal cortex, while beta-endorphin in the hypothalamus of patients was inversely correlated with thiamin in frontal cortex, parietal white matter, thalamus and brainstem. These results suggest that there is a disturbance of the endorphinergic system in Wernicke-Korsakoff syndrome which may be related to alcohol intake. PMID:1650797

  4. Increase in central striatal dopamine transporters in patients with Shwachman-Diamond syndrome: additional evidence of a brain phenotype

    Booij, Jan; Reneman, Liesbeth; Alders, Marielle; Kuijpers, Taco W.

    2013-01-01

    Patients with Shwachman-Diamond syndrome (SDS) do not only experience well-described physical features like skeletal abnormalities and hematological dysfunctions, but recent studies also suggested attention and working memory deficits in SDS. Indeed, a recent structural magnetic resonance imaging

  5. Spinal cord swelling and candidiasis

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

    1982-01-01

    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.)

  6. Spinal cord swelling and candidiasis

    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.

  7. Hospital- and community-based interventions enhancing (re) employment for people with spinal cord injury : a systematic review

    Roels, E. H.; Aertgeerts, B.; Ramaekers, D.; Peers, K.

    Study design: Systematic Review. Objectives: To investigate the effect of interventions enhancing (re) employment following spinal cord injury (SCI). Setting: Studies from multiple countries were included. Methods: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials (CENTRAL), CINAHL,

  8. Spinal Cord Stimulation With Hybrid Lead Relieves Pain in Low Back and Legs

    de Vos, Cecilia Cecilia Clementine; Levy, R.M.; Dijkstra, Cindy; Lenders, Mathieu W.P.M.; Holsheimer, J.

    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

  9. AAPT Diagnostic Criteria for Central Neuropathic Pain

    Widerstrom-Noga, Eva; Loeser, John D.; Jensen, Troels Staehelin

    2017-01-01

    Central neuropathic pain, which is pain caused by a lesion or disease of the central somatosensory nervous system, is a serious consequence of spinal cord injury, stroke, multiple sclerosis, and other conditions affecting the central nervous system. A collaborative effort between the Analgesic....... This article focuses on central neuropathic pain associated with spinal cord injury, stroke, and multiple sclerosis, but the AAPT framework can be extended to central pain due to other causes such as traumatic brain injury. The classification of central neuropathic pain is organized according to the AAPT...

  10. Fixed cord in spinal stenosis

    Levy, L.M.; Wang, H.; Francomano, C.; Hurko, O.; Carson, B.; Heffez, D.S.; DiChiro, G.; Bryan, R.N.

    1990-01-01

    This paper evaluates patients with cervical spinal canal compromise due to congenital anomalies (achondroplasia, Chiari malformation) and degenerative diseases using MR cord motion and cerebrospinal fluid (CSF) flow studies. Pulsatile longitudinal motion of the cervical cord was determined by means of cardiac-gated velocity phase contrast methods, including cine. Pathology included dwarfism (n = 15), Chiari malformation (n = 10), spondylosis (n = 10), and acute cord compression (n = 9). Symptomatic cases of congenital cervical stenosis had decreased cord motion, although CSF flow was not always significantly compromised. Postoperative cases demonstrated good cord and CSF motion, unless compression or obstruction was present

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

    Ridder, D.M.D.; Lenders, M.W.P.M.; de Vos, Cecilia Cecilia Clementine; 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

  12. Vocal Cord Paralysis

    ... Viral infections. Some viral infections, such as Lyme disease, Epstein-Barr and herpes, can cause inflammation and damage directly to the nerves in the larynx. Neurological conditions. If you have certain ... disease, you may experience vocal cord paralysis. Risk factors ...

  13. Spinal Cord Injury 101

    Full Text Available ... Home Kim Eberhardt Muir, MS Coping with a New Injury Robin Dorman, PsyD Sex and Fertility After ... program? play_arrow What are the most promising new treatments for spinal cord injuries? play_arrow What ...

  14. Spinal Cord Injury 101

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

  15. Spinal Cord Injury 101

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

  16. Spinal Cord Injury 101

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

  17. Spinal Cord Injury 101

    Full Text Available ... Anne Bryden, OT The Role of the Social Worker after Spinal Cord Injury Patti Rogers, SW Marguerite David, ... injuries. The website does not provide medical advice, recommend or endorse health care products or services, or control the information ...

  18. A case of central nervous system vasculitis related to an episode of Guillain-Barrè syndrome

    Sinardi, Daniele; Spada, Antonella; Marino, Antonella; Mondello, Epifanio

    2000-01-01

    The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune Guillain-Barrè syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next mo...

  19. Postmortem diffusion MRI of the entire human spinal cord at microscopic resolution

    Evan Calabrese

    Full Text Available The human spinal cord is a central nervous system structure that plays an important role in normal motor and sensory function, and can be affected by many debilitating neurologic diseases. Due to its clinical importance, the spinal cord is frequently the subject of imaging research. Common methods for visualizing spinal cord anatomy and pathology include histology and magnetic resonance imaging (MRI, both of which have unique benefits and drawbacks. Postmortem microscopic resolution MRI of fixed specimens, sometimes referred to as magnetic resonance microscopy (MRM, combines many of the benefits inherent to both techniques. However, the elongated shape of the human spinal cord, along with hardware and scan time limitations, have restricted previous microscopic resolution MRI studies (both in vivo and ex vivo to small sections of the cord. Here we present the first MRM dataset of the entire postmortem human spinal cord. These data include 50 μm isotropic resolution anatomic image data and 100 μm isotropic resolution diffusion data, made possible by a 280 h long multi-segment acquisition and automated image segment composition. We demonstrate the use of these data for spinal cord lesion detection, automated volumetric gray matter segmentation, and quantitative spinal cord morphometry including estimates of cross sectional dimensions and gray matter fraction throughout the length of the cord. Keywords: Spinal cord, Magnetic resonance microscopy, Tractography, Human, Gray matter

  20. Scrub typhus complicated by acute respiratory distress syndrome and multiorgan failure; an unrecognized alarming entity in central India: A report of two cases

    Amrish Saxena

    2014-01-01

    Full Text Available Scrub typhus is an acute infectious illness, distributed throughout the Asia Pacific rim. In India, it has been reported from northern, eastern, and southern India. However, cases of scrub typhus have not been well-documented from Vidarbha, an eastern region of Maharashtra state in central India. We report two cases of complicated scrub typhus from Vidarbha region. These cases admitted in unconscious state with 8-10 days history of fever, body ache, cough, and progressive breathlessness. The diagnosis in both cases was based on presence of eschar, a positive Weil-Felix test, and a positive rapid diagnostic test (immunochromatographic assay. Both cases were complicated by acute respiratory distress syndrome (ARDS and multiorgan failure. Both of them presented in their 2 nd week of illness and died during the hospital course in spite of intensive supportive care. The main cause of mortality was delayed referral leading to delay in diagnosis and treatment.

  1. A patient with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and severe hypoganglionosis associated with a novel SOX10 mutation.

    Akutsu, Yuko; Shirai, Kentaro; Takei, Akira; Goto, Yudai; Aoyama, Tomohiro; Watanabe, Akimitu; Imamura, Masatoshi; Enokizono, Takashi; Ohto, Tatsuyuki; Hori, Tetsuo; Suzuki, Keiko; Hayashi, Masaharu; Masumoto, Kouji; Inoue, Ken

    2018-05-01

    In this report, we present the case of a female infant with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease (PCWH) associated with a novel frameshift mutation (c.842dupT) in exon 5, the last exon of SOX10. She had severe hypoganglionosis in the small intestine and entire colon, and suffered from frequent enterocolitis. The persistence of ganglion cells made both the diagnosis and treatment difficult in the neonatal period. She also showed hypopigmentation of the irises, hair and skin, bilateral sensorineural deafness with hypoplastic inner year, severe demyelinating neuropathy with hypotonia, and diffuse brain hypomyelination. The p.Ser282GlnfsTer12 mutation presumably escapes from nonsense-mediated decay and may generate a dominant-negative effect. We suggest that hypoganglionosis can be a variant intestinal manifestation associated with PCWH and that hypoganglionosis and aganglionosis may share the same pathoetiological mechanism mediated by SOX10 mutations. © 2018 Wiley Periodicals, Inc.

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

    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.

  3. Crooked Calf Syndrome: Managing Lupines on Rangelands of the Channel Scablands of East-Central Washington State

    “Crooked calf syndrome”, the contracture-type skeletal defects and cleft palate caused by velvet lupine (Lupinus leucophyllus) on the channel Scablands of east-central Washington State are the same as those defects induced by Conium maculatum (poison-hemlock) and Nicotiana spp. (wild tobacco) in rum...

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

    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.

  5. Variations in the formation of the human caudal spinal cord.

    Saraga-Babić, M; Sapunar, D; Wartiovaara, J

    1995-01-01

    Collection of 15 human embryos between 4-8 developmental weeks was used to histologically investigate variations in the development of the caudal part of the spinal cord and the neighboring axial organs (notochord and vertebral column). In the 4-week embryo, two types of neurulation were parallelly observed along the anteroposterior body axis: primary in the areas cranial to the neuroporus caudalis and secondary in the more caudal tail regions. In the 5-week embryos, both parts of the neural tube fused, forming only one continuous lumen in the developing spinal cord. In the three examined embryos we found anomalous pattern of spinal cord formation. Caudal parts of these spinal cords displayed division of their central canal into two or three separate lumina, each surrounded by neuroepithelial layer. In the caudal area of the spinal cord, derived by secondary neurulation, formation of separate lumina was neither connected to any anomalous notochord or vertebral column formation, nor the appearance of any major axial disturbances. We suggest that development of the caudal part of the spinal cord differs from its cranial region not only in the type of neurulation, but also in the destiny of its derivatives and possible modes of abnormality formation.

  6. MRI and clinical symptoms in chronic cervical cord injury

    Soeda, Shuichi; Maruiwa, Hirofumi; Yokoi, Masahiro; Saitoh, Seiya; Yamauchi, Kenji.

    1992-01-01

    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.)

  7. Acute spinal cord injuries

    Takahashi, M.; Izunaga, H.; Sato, R.; Shinzato, I.; Korogi, Y.; Yamashita, Y.

    1991-01-01

    This paper reports on sequential MR images and neurologic findings that were correlated in 40 acute spinal cord injuries. Within 1 week after injury, frequent initial MR changes appeared isointense on both T1- and T2-weighted images and isointense on T1- and hyperintense on T2-weighted images. After 2 months, hypointensity appeared on T1-weighted images and hyperintensity persisted or appeared on T2-weighted images. Clinical improvements were observed in patients with isointensity on both T1- and T2-weighted images at the initial examination. A larger area of hyperintensity on subsequent T2-weighted images was correlated with no neurologic improvement. MR findings were good indicators of the spinal cord injury

  8. International Spinal Cord Injury

    Dvorak, M F; Itshayek, E; Fehlings, M G

    2015-01-01

    STUDY DESIGN: Survey of expert opinion, feedback and final consensus. OBJECTIVE: To describe the development and the variables included in the International Spinal Cord Injury (SCI) Spinal Interventions and Surgical Procedures Basic Data set. SETTING: International working group. METHODS......: A committee of experts was established to select and define data elements. The data set was then disseminated to the appropriate committees and organizations for comments. All suggested revisions were considered and both the International Spinal Cord Society and the American Spinal Injury Association endorsed...... spinal intervention and procedure is coded (variables 1 through 7) and the spinal segment level is described (variables 8 and 9). Sample clinical cases were developed to illustrate how to complete it. CONCLUSION: The International SCI Spinal Interventions and Surgical Procedures Basic Data Set...

  9. Spinal Cord Doses in Palliative Lung Radiotherapy Schedules

    Ffrrcsi, F.H.; Parton, C.

    2006-01-01

    Aim: We aim to check the safety of the standard palliative radiotherapy techniques by using the Linear quadratic model for a careful estimation of the doses received by the spinal cord, in all standard palliative lung radiotherapy fields and fractionation. Material and Methods: All patients surveyed at this prospective audit were treated with palliative chest radio-therapy for lung cancer over a period from January to June 2005 by different clinical oncology specialists within the department. Radiotherapy field criteria were recorded and compared with the recommended limits of the MRC trial protocols for the dose and fractionation prescribed. Doses delivered to structures off the field central axis were estimated using a standard CT scan of the chest. Dose estimates were made using an SLPLAN planning system. As unexpected spinal cord toxicity has been reported after hypo fractionated chest radiotherapy, a sagittal view was used to calculate the isodoses along the length of the spinal cord that could lie within the RT field. Equivalent dose estimates are made using the Linear Quadratic Equivalent Dose formula (LQED). The relative radiation sensitivity of spinal cord for myelopathy (the a/b dose) cord has been estimated as a/b = 1 Gy. Results: 17 Gy in 2 fraction and 39 Gy in 13 fraction protocols have spinal cord equivalent doses (using the linear-quadratic model) that lie within the conventional safe limits of 50 Gy in 25 fractions for the 100% isodose. However when the dosimetry is modelled for a 6 MV 100 cm isocentric linac in 3 dimensions, and altered separations and air space inhomogeneity are considered, the D-Max doses consistently fall above this limit on our 3 model patients. Conclusion: The 17 Gy in 2 fraction and 39 Gy in 13 fraction protocol would risk spinal cord damage if the radio therapist was unaware of the potential spinal cord doses. Alterative doses are suggested below 15.5 Gy/ 2 fractions (7 days apart) would be most acceptable

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

    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......-free group. The rostral-caudal extent of the lesion measured by MRI did not differ between the two patient groups, and there were no statistically significant differences in any of the predefined areas of interest on the axial plane images. This study suggests that neuronal hyperexcitability plays a key role...... 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....

  11. Value of MRI and DTI as Biomarkers for Classifying Acute Spinal Cord Injury

    2014-10-29

    Datasets were checked for goodness of fit as part of the quality assurance process. Whole cord FA, MD, LD (λ1) and TD (λ23) were calculated for...discussion 1658. 10. Marino RJ, Ditunno JF,Jr, Donovan WH, Maynard F ,Jr. Neurologic recovery after traumatic spinal cord injury: Data from the model spinal...Jan;75(1):67-72. 17. Foo D, Subrahmanyan TS, Rossier AB. Post-traumatic acute anterior spinal cord syndrome. Paraplegia. 1981;19(4):201-5. 18

  12. Asthma mimic: Case report and literature review of vocal cord nodule associated with wheezing.

    Kashif, Muhammad; Singh, Tushi; Aslam, Ahsan; Khaja, Misbahuddin

    2017-01-01

    Asthma is a heterogeneous disease, usually characterized by chronic airway inflammation. Various clinical conditions can mimic asthma, such as foreign body aspiration, subglottic stenosis, congestive heart failure, diffuse panbronchiolitis, aortic arch anomalies, reactive airway dysfunction syndrome, chronic obstructive pulmonary disease, retrosternal goiter, vocal cord tumors, other airway tumors, and vocal cord dysfunction. Upper airway obstruction can be a life-threatening emergency. Here, we present the case of a 58-year-old female with recurrent hospital visits for wheezing and exacerbations of asthma, who was later found to have a vocal cord nodule confirmed to be squamous cell carcinoma, which was mimicking like asthma.

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

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

    2010-01-01

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

  14. Central sensitization does not identify patients with carpal tunnel syndrome who are likely to achieve short-term success with physical therapy.

    Fernández-de-Las-Peñas, César; Cleland, Joshua A; Ortega-Santiago, Ricardo; de-la-Llave-Rincon, Ana Isabel; Martínez-Perez, Almudena; Pareja, Juan A

    2010-11-01

    The aim of the current study was to identify whether hyperexcitability of the central nervous system is a prognostic factor for individuals with carpal tunnel syndrome (CTS) likely to experience rapid and clinical self-reported improvement following a physical therapy program including soft tissue mobilization and nerve slider neurodynamic interventions. Women presenting with clinical and electrophysiological findings of CTS were involved in a prospective single-arm trial. Participants underwent a standardized examination and then a physical therapy session. The physical therapy sessions included both soft tissue mobilization directed at the anatomical sites of potential median nerve entrapment and a passive nerve slider neurodynamic technique targeted to the median nerve. Pressure pain thresholds (PPT) over the median, radial and ulnar nerves, C5-C6 zygapophyseal joint, carpal tunnel and tibialis anterior muscle were assessed bilaterally. Additionally, thermal detection and pain thresholds were measured over the carpal tunnel and thenar eminence bilaterally to evaluate central nervous system excitability. Subjects were classified as responders (having achieved a successful outcome) or non-responders based on self-perceived recovery. Variables were entered into a stepwise logistic regression model to determine the most accurate variables for determining prognosis. Data from 72 women were included in the analysis, of which 35 experienced a successful outcome (48.6%). Three variables including PPT over the C5-C6 joint affected side 66 points were identified. If 2 out of 3 variables were present (LR + 14.8), the likelihood of success increased from 48.6 to 93.3%. We identified 3 factors that may be associated with a rapid clinical response to both soft tissue mobilization and nerve slider neurodynamic techniques targeted to the median nerve in women presenting with CTS. Our results support that widespread central sensitization may not be present in women with CTS who

  15. The Pathology of Wobbly Hedgehog Syndrome.

    Díaz-Delgado, Josué; Whitley, Derick B; Storts, Ralph W; Heatley, Jill J; Hoppes, Sharman; Porter, Brian F

    2018-01-01

    Wobbly hedgehog syndrome (WHS) is a leading cause of neurologic disease in African pygmy hedgehogs (APHs; Atelerix albiventris). This study describes the signalment, clinical signs, gross, microscopic, and ultrastructural lesions of WHS in a cohort of 12 pet APHs. Microscopically, lesions consisted of status spongiosus of the white matter, typically bilateral and symmetrical, with myelin degeneration and loss that was accompanied by neuronal/axonal degeneration plus reactive microgliosis and mild, focal astrocytosis and astrogliosis. Lesions were most severe in the cerebellum and medulla oblongata, as well as cervical and thoracic spinal cord. Less affected areas were the corona radiata, corpus callosum, corpus striatum, internal capsule, and the mesencephalon. Ultrastructurally, the lesions consisted of splitting of the myelin sheath at the intraperiod line with subsequent focal expansion, resulting in status spongiosus, disruption, dilatation, rhexis, and phagocytosis. Based on these results, WHS is best described as a "spongy myelinopathy" with widespread central nervous system involvement.

  16. [Complex regional pain syndrome (CRPS) : An update].

    Dimova, V; Birklein, F

    2018-04-17

    The acute phase of complex regional pain syndrome (CRPS) is pathophysiologically characterized by an activation of the immune system and its associated inflammatory response. During the course of CRPS, central nervous symptoms like mechanical hyperalgesia, loss of sensation, and body perception disorders develop. Psychological factors such as pain-related anxiety and traumatic events might have a negative effect on the treatment outcome. While the visible inflammatory symptoms improve, the pain often persists. A stage adapted, targeted treatment could improve the prognosis. Effective multidisciplinary treatment includes the following: pharmacotherapy with steroids, bisphosphonates, or dimethylsulfoxide cream (acute phase), and antineuropathic analgesics (all phases); physiotherapy and behavioral therapy for pain-related anxiety and avoidance of movement; and interventional treatment like spinal cord or dorsal root ganglion stimulation if noninvasive options failed.

  17. Is early cord clamping, delayed cord clamping or cord milking best?

    Vatansever, Binay; Demirel, Gamze; Ciler Eren, Elif; Erel, Ozcan; Neselioglu, Salim; Karavar, Hande Nur; Gundogdu, Semra; Ulfer, Gozde; Bahadir, Selcen; Tastekin, Ayhan

    2018-04-01

    To compare the antioxidant status of three cord clamping procedures (early clamping, delayed clamping and milking) by analyzing the thiol-disulfide balance. This randomized controlled study enrolled 189 term infants who were divided into three groups according to the cord clamping procedure: early clamping, delayed clamping and milking. Blood samples were collected from the umbilical arteries immediately after clamping, and the thiol/disulfide homeostasis was analyzed. The native and total thiol levels were significantly (p total thiol ratio was significantly (p = .026) lower in the delayed cord clamping and milking groups compared with the early clamping groups. Early cord clamping causes the production of more disulfide bonds and lower thiol levels, indicating that oxidation reactions are increased in the early cord clamping procedure compared with the delayed cord clamping and milking procedures. The oxidant capacity is greater with early cord clamping than with delayed clamping or cord milking. Delayed cord clamping or milking are beneficial in neonatal care, and we suggest that they be performed routinely in all deliveries.

  18. Spinal cord injury below-level neuropathic pain relief with dorsal root entry zone microcoagulation performed caudal to level of complete spinal cord transection.

    Falci, Scott; Indeck, Charlotte; Barnkow, Dave

    2018-06-01

    OBJECTIVE Surgically created lesions of the spinal cord dorsal root entry zone (DREZ) to relieve central pain after spinal cord injury (SCI) have historically been performed at and cephalad to, but not below, the level of SCI. This study was initiated to investigate the validity of 3 proposed concepts regarding the DREZ in SCI central pain: 1) The spinal cord DREZ caudal to the level of SCI can be a primary generator of SCI below-level central pain. 2) Neuronal transmission from a DREZ that generates SCI below-level central pain to brain pain centers can be primarily through sympathetic nervous system (SNS) pathways. 3) Perceived SCI below-level central pain follows a unique somatotopic map of DREZ pain-generators. METHODS Three unique patients with both intractable SCI below-level central pain and complete spinal cord transection at the level of SCI were identified. All 3 patients had previously undergone surgical intervention to their spinal cords-only cephalad to the level of spinal cord transection-with either DREZ microcoagulation or cyst shunting, in failed attempts to relieve their SCI below-level central pain. Subsequent to these surgeries, DREZ lesioning of the spinal cord solely caudal to the level of complete spinal cord transection was performed using electrical intramedullary guidance. The follow-up period ranged from 1 1/2 to 11 years. RESULTS All 3 patients in this study had complete or near-complete relief of all below-level neuropathic pain. The analyzed electrical data confirmed and enhanced a previously proposed somatotopic map of SCI below-level DREZ pain generators. CONCLUSIONS The results of this study support the following hypotheses. 1) The spinal cord DREZ caudal to the level of SCI can be a primary generator of SCI below-level central pain. 2) Neuronal transmission from a DREZ that generates SCI below-level central pain to brain pain centers can be primarily through SNS pathways. 3) Perceived SCI below-level central pain follows a unique

  19. C-Peptide Is a Sensitive Indicator for the Diagnosis of Metabolic Syndrome in Subjects from Central Mexico.

    Gonzalez-Mejia, M Elba; Porchia, Leonardo M; Torres-Rasgado, Enrique; Ruiz-Vivanco, Guadalupe; Pulido-Pérez, Patricia; Báez-Duarte, Blanca G; Pérez-Fuentes, Ricardo

    2016-05-01

    Metabolic Syndrome (MetS) is associated with elevated risk for developing diabetes and cardiovascular disease. A key component of MetS is the development of insulin resistance (IR). The homeostatic model assessment (HOMA) model can determine IR by using insulin or C-peptide concentrations; however, the efficiency of insulin and C-peptide to determine MetS has not been compared. The aim of the study was to compare the efficiency of C-peptide and insulin to determine MetS in Mexicans. Anthropometrics, glucose, insulin, C-peptide, triglycerides, and high-density lipoproteins were determined in 156 nonpregnant females and 114 males. Subjects were separated into normal or positive for MetS. IR was determined by the HOMA2 calculator using insulin or C-peptide. Correlations were calculated using the Spearman correlation coefficient (ρ). Differences between correlations were determined by calculating Steiger's Z. The sensitivity was determined by the area under receiver operating characteristics curve (AUC) analysis. Independent of the MetS definition [Adult Treatment Panel III (ATP III), International Diabetes Federation (IDF), or World Health Organization (WHO)], C-peptide and insulin were significantly higher in MetS subjects (P indicator of MetS. Since C-peptide has recently emerged as a biomolecule with significant importance for inflammatory diseases, monitoring C-peptide levels will aid clinicians in preventing MetS.

  20. Pain in Down's Syndrome

    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

  1. PARANEOPLASTIC DAMAGE TO THE CENTRAL AND PERIPHERAL NERVOUS SYSTEM IN BREAST CANCER: A CASE REPORT

    E. S. Koroleva

    2017-01-01

    Full Text Available Paraneoplastic neurological syndrome involves the concurrent development of cancer and neurologicaldiseases. Breast cancer is the second most common cancer associated with paraneoplastic damage to the central and peripheral nervous system. Autoimmune genesis of the disease is characterized by the presence of highly specific onconeural antibodies, which selectively affect neurons in the brain cord, spinal cord and spinal ganglia, and cause the onset of neurological symptoms within 2 years before cancer is detected. Six well-characterized onconeural antibodies detected in the blood serum of breast cancer patients can be used for the laboratory diagnosis of paraneoplastic neurological syndrome. Of them, anti-Hu, anti-CV2 and anti-amphiphysin antibodies cause polyneuropathy most often. Anti-Yo antibody is usually associated with cerebellar degeneration. Multiple neuronal autoantibodies can be simultaneously detected in a patient. Removal of the tumor may lead to stabilization and even partial regression of the neurological symptoms in 70 % of patients. Therefore, the surgical treatment of cancer should consider not only the tumor extension, but also the severity and progression of neurological deficit. We present a case of paraneoplastic cerebellar degeneration and paraneoplastic polyneuropathy in a 50-year-old woman with the neurological symptoms appeared 5 months before breast infiltrating ductal carcinoma was detected. The current approaches to the diagnosis of paraneoplastic neurological syndrome, as well as feasibility of radical removal of the tumor due to progression of neurological deficit were discussed.

  2. Sleep apnea syndrome after irradiation of the neck

    Herlihy, J.P.; Whitlock, W.L.; Dietrich, R.A.; Shaw, T.

    1989-01-01

    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

  3. Cutting the Cord-2

    2004-01-01

    This animation shows the view from the rear hazard avoidance cameras on the Mars Exploration Rover Spirit as the rover turns 45 degrees clockwise. This maneuver is the first step in a 3-point turn that will rotate the rover 115 degrees to face west. The rover must make this turn before rolling off the lander because airbags are blocking it from exiting from the front lander petal. Before this crucial turn took place, engineers instructed the rover to cut the final cord linking it to the lander. The turn took around 30 minutes to complete.

  4. Cutting the Cord

    2004-01-01

    This animation shows the view from the front hazard avoidance cameras on the Mars Exploration Rover Spirit as the rover turns 45 degrees clockwise. This maneuver is the first step in a 3-point turn that will rotate the rover 115 degrees to face west. The rover must make this turn before rolling off the lander because airbags are blocking it from exiting off the front lander petal. Before this crucial turn could take place, engineers instructed the rover to cut the final cord linking it to the lander. The turn took around 30 minutes to complete.

  5. Evaluation of best corrected visual acuity and central macular thickness after intravitreal dexamethasone implant injections in patients with Irvine-Gass syndrome: A retrospective study of six cases.

    Keilani, Chafik; Halalchi, Aziz; Wakpi Djeugue, Désiré; Regis, Anne; Abada, Samir

    2016-10-01

    Irvine-Gass syndrome is a macular edema (ME) that specifically occurs after cataract surgery. Its incidence varies from 0.2-2%. The purpose of this study is to evaluate the effectiveness of intravitreal dexamethasone implant injections in patients with Irvine-Gass syndrome. Patients with ME secondary to cataract surgery who underwent intravitreal injections of dexamethasone implant between December 2011 to October 2014 at François-Quesnay hospital (Mantes-la-Jolie, France) were retrospectively reviewed. The patients were followed for at least 10 months. All the patients were handled by intravitreal injection of dexamethasone in the eye of study among which some resisted to a preliminary treatment by non-steroidal anti-inflammatory drug (NSAID) and acetazolamide. The patients were examined each month. The patients were again handled by intravitreal injection of dexamethasone if they presented a recurrence. The primary endpoint of the study was determined on best corrected visual acuity (BCVA) using early diabetic retinopathy study (ETDRS) scale and central macular thickness (CMT) [μm] using optical coherence tomography (OCT) 3 and 6 months after the first injection. Secondary endpoints were the number of recurrences, the number of injections, the duration average before the first recurrence, the BCVA 10 months after the first injection and the tolerance. Six eyes of six patients were studied. At baseline, the mean (standard deviation [SD]) of the BCVA was 59.8±11. Three months after the first injection, the mean (SD) of the BCVA showed a statistically significant increase to 72.2±8.6 (P=0.03). Six months after the first injection, the mean (SD) of the BCVA showed a statistically significant increase to 72±11.8 (P=0.03). Concerning the CMT, the mean (SD) was 495.6±135.2 before treatment. Three months after the first injection, the mean (SD) of the CMT showed a statistically significant decrease to 268.6±57.8 (P=0.03). Six months after the first injection, the

  6. Guillain–Barre syndrome: Demographics, clinical profile & seasonal variation in a tertiary care centre of central India

    Shrivastava, Manisha; Nehal, Shah; Seema, Navaid

    2017-01-01

    Background & objectives: Guillain–Barre syndrome (GBS) is an autoimmune disease and a recognized cause of generalized progressive paralysis worldwide. The present study was aimed to document the clinical findings, demographics and seasonal variations amongst the patients with GBS during the hospital stay. Methods: A retrospective analysis of 66 referred cases diagnosed as GBS was conducted. Medical records and the data related to age, sex, antecedent illness, duration of symptoms before admission, muscle power graded by the Medical Research Council scale, functional scores, details of Intensive Care Unit complications and need for ventilation were obtained. The patients were divided into four seasonal groups: S1 (spring, February to April), S2 (summer, May to July), S3 (rainy, August to October) and S4 (winter, November to January) and parameters were studied. Results: The mean age of the patients was 40.69 yr. Forty one (62.1%) patients had a history of preceding illness. Forty nine (74.2%) patients showed quadriparesis as most common complaint. Thirty three (50%) patients were of acute inflammatory demyelinating polyneuropathy (AIDP) variant. The highest number of GBS cases (60%) was found in S1 and S2. The maximum duration of hospital stay was observed in S3 group (mean 23 days). Interpretation & conclusions: GBS seems to affect all age groups with male preponderance. Most common antecedent event and presenting feature were flu-like illness and quadriparesis, respectively. AIDP was the most common variant. Most cases occurred from February to July (S1 and S2 group) (maximum in July) with preceding influenza and diarrhoea and maximum duration of hospital stay was observed in S3 group. Prospective studies with follow up of GBS patients need to be done to confirm findings. PMID:28639596

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

    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.

  8. Association of MEP1A gene variants with insulin metabolism in central European women with polycystic ovary syndrome.

    Lam, Uyen D P; Lerchbaum, Elisabeth; Schweighofer, Natascha; Trummer, Olivia; Eberhard, Katharina; Genser, Bernd; Pieber, Thomas R; Obermayer-Pietsch, Barbara

    2014-03-10

    Polycystic ovary syndrome (PCOS) shows not only hyperandrogenemia, hirsutism and fertility problems, but also metabolic disturbances including obesity, cardiovascular events and type-2 diabetes. Accumulating evidence suggests some degree of inflammation associated with prominent aspects of PCOS. We aimed to investigate the association of genetic variants 3'UTR rs17468190 (G/T) of the inflammation-associated gene MEP1A (GenBank ID: NM_005588.2) with metabolic disturbances in PCOS and healthy control women. Genetic variants rs17468190 (G/T) of MEP1A gene were analyzed in 576 PCOS women and 206 controls by using the Taqman fluorogenic 5'-exonuclease assay. This polymorphism was tested for association with anthropometric, metabolic, hormonal, and functional parameters of PCOS. There was a borderline significant difference in genotype distribution between PCOS and control women (p=0.046). In overweight/obese PCOS patients, the variants rs17468190 (G/T) in the MEP1A gene are associated with glucose and insulin metabolism. In a dominant model, the GG genotype of the MEP1A gene was more strongly associated with insulin metabolism in overweight/obese PCOS women (body mass index, BMI>25 kg/m(2)), than in GT+TT genotypes. The MEP1A GG-carriers showed a significantly increased homeostatic model assessment - insulin resistance (HOMA-IR) (p=0.003), elevation of fasting insulin (p=0.004) and stimulated insulin (30 min, pdisease modification in PCOS. It might contribute to the abnormalities of glucose metabolism and insulin sensitivity and serve as a diagnostic or therapeutic target gene for PCOS. Copyright © 2014 Elsevier B.V. All rights reserved.

  9. Vocal cord dysfunction in children.

    Noyes, Blakeslee E; Kemp, James S

    2007-06-01

    Vocal cord dysfunction is characterised by paradoxical vocal cord adduction that occurs during inspiration, resulting in symptoms of dyspnoea, wheeze, chest or throat tightness and cough. Although the condition is well described in children and adults, confusion with asthma often triggers the use of an aggressive treatment regimen directed against asthma. The laryngoscopic demonstration of vocal cord adduction during inspiration has been considered the gold standard for the diagnosis of vocal cord dysfunction, but historical factors and pulmonary function findings may provide adequate clues to the correct diagnosis. Speech therapy, and in some cases psychological counselling, is often beneficial in this disorder. The natural course and prognosis of vocal cord dysfunction are still not well described in adults or children.

  10. Spinal cord compression due to metastases

    Azevedo, C.M. de; Matushita, J.P.K.; Silva, M.A.F. da; Koch, H.A.

    1986-01-01

    A study of 20 patients with medullary compression syndrome due to lesions not related to the central nervous system is presented. Plain films of the spine and myelography are made to determine the level of osseous involvement, the level of the spinal block and to planning radiotherapy. (Author) [pt

  11. Spinal cord electrophysiology II: extracellular suction electrode fabrication.

    Garudadri, Suresh; Gallarda, Benjamin; Pfaff, Samuel; Alaynick, William

    2011-02-20

    Development of neural circuitries and locomotion can be studied using neonatal rodent spinal cord central pattern generator (CPG) behavior. We demonstrate a method to fabricate suction electrodes that are used to examine CPG activity, or fictive locomotion, in dissected rodent spinal cords. The rodent spinal cords are placed in artificial cerebrospinal fluid and the ventral roots are drawn into the suction electrode. The electrode is constructed by modifying a commercially available suction electrode. A heavier silver wire is used instead of the standard wire given by the commercially available electrode. The glass tip on the commercial electrode is replaced with a plastic tip for increased durability. We prepare hand drawn electrodes and electrodes made from specific sizes of tubing, allowing consistency and reproducibility. Data is collected using an amplifier and neurogram acquisition software. Recordings are performed on an air table within a Faraday cage to prevent mechanical and electrical interference, respectively.

  12. Spontaneous axonal regeneration in rodent spinal cord after ischemic injury

    von Euler, Mia; Janson, A M; Larsen, Jytte Overgaard

    2002-01-01

    cells, while other fibers were unmyelinated. Immunohistochemistry demonstrated that some of the regenerated fibers were tyrosine hydroxylase- or serotonin-immunoreactive, indicating a central origin. These findings suggest that there is a considerable amount of spontaneous regeneration after spinal cord......Here we present evidence for spontaneous and long-lasting regeneration of CNS axons after spinal cord lesions in adult rats. The length of 200 kD neurofilament (NF)-immunolabeled axons was estimated after photochemically induced ischemic spinal cord lesions using a stereological tool. The total...... length of all NF-immunolabeled axons within the lesion cavities was increased 6- to 10-fold at 5, 10, and 15 wk post-lesion compared with 1 wk post-surgery. In ultrastructural studies we found the putatively regenerating axons within the lesion to be associated either with oligodendrocytes or Schwann...

  13. 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

    Janaina Vall; Violante Augusta Batista Braga

    2005-01-01

    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 ...

  14. Gorlin-goltz syndrome

    Ahmed, N.; Salman, M.; Mansoor, M.A.

    2007-01-01

    Multiple jaw cysts are a characteristic manifestation of basal cell nevus (Gorlin) syndrome. Gorlin-Goltz syndrome is characterized by symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. This patient showed recurrent jaw and maxillary cysts, for which he was followed for 2 years. (author)

  15. The acute radiation syndrome

    Souhami Filho, L.

    1985-01-01

    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.) [pt

  16. Alterations in cardiac autonomic control in spinal cord injury.

    Biering-Sørensen, Fin; Biering-Sørensen, Tor; Liu, Nan; Malmqvist, Lasse; Wecht, Jill Maria; Krassioukov, Andrei

    2018-01-01

    A spinal cord injury (SCI) interferes with the autonomic nervous system (ANS). The effect on the cardiovascular system will depend on the extent of damage to the spinal/central component of ANS. The cardiac changes are caused by loss of supraspinal sympathetic control and relatively increased parasympathetic cardiac control. Decreases in sympathetic activity result in heart rate and the arterial blood pressure changes, and may cause arrhythmias, in particular bradycardia, with the risk of cardiac arrest in those with cervical or high thoracic injuries. The objective of this review is to give an update of the current knowledge related to the alterations in cardiac autonomic control following SCI. With this purpose the review includes the following subheadings: 2. Neuro-anatomical plasticity and cardiac control 2.1 Autonomic nervous system and the heart 2.2 Alteration in autonomic control of the heart following spinal cord injury 3. Spinal shock and neurogenic shock 3.1 Pathophysiology of spinal shock 3.2 Pathophysiology of neurogenic shock 4. Autonomic dysreflexia 4.1 Pathophysiology of autonomic dysreflexia 4.2 Diagnosis of autonomic dysreflexia 5. Heart rate/electrocardiography following spinal cord injury 5.1 Acute phase 5.2 Chronic phase 6. Heart rate variability 6.1 Time domain analysis 6.2 Frequency domain analysis 6.3 QT-variability index 6.4 Nonlinear (fractal) indexes 7. Echocardiography 7.1 Changes in cardiac structure following spinal cord injury 7.2 Changes in cardiac function following spinal cord injury 8. International spinal cord injury cardiovascular basic data set and international standards to document the remaining autonomic function in spinal cord injury. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. Axonal regeneration in zebrafish spinal cord

    Hui, Subhra Prakash

    2018-01-01

    Abstract In the present review we discuss two interrelated events—axonal damage and repair—known to occur after spinal cord injury (SCI) in the zebrafish. Adult zebrafish are capable of regenerating axonal tracts and can restore full functionality after SCI. Unlike fish, axon regeneration in the adult mammalian central nervous system is extremely limited. As a consequence of an injury there is very little repair of disengaged axons and therefore functional deficit persists after SCI in adult mammals. In contrast, peripheral nervous system axons readily regenerate following injury and hence allow functional recovery both in mammals and fish. A better mechanistic understanding of these three scenarios could provide a more comprehensive insight into the success or failure of axonal regeneration after SCI. This review summarizes the present understanding of the cellular and molecular basis of axonal regeneration, in both the peripheral nervous system and the central nervous system, and large scale gene expression analysis is used to focus on different events during regeneration. The discovery and identification of genes involved in zebrafish spinal cord regeneration and subsequent functional experimentation will provide more insight into the endogenous mechanism of myelination and remyelination. Furthermore, precise knowledge of the mechanism underlying the extraordinary axonal regeneration process in zebrafish will also allow us to unravel the potential therapeutic strategies to be implemented for enhancing regrowth and remyelination of axons in mammals. PMID:29721326

  18. Nogo-A expression dynamically varies after spinal cord injury

    Jian-wei Wang

    2015-01-01

    Full Text Available The mechanism involved in neural regeneration after spinal cord injury is unclear. The myelin-derived protein Nogo-A, which is specific to the central nervous system, has been identified to negatively affect the cytoskeleton and growth program of axotomized neurons. Studies have shown that Nogo-A exerts immediate and chronic inhibitory effects on neurite outgrowth. In vivo, inhibitors of Nogo-A have been shown to lead to a marked enhancement of regenerative axon extension. We established a spinal cord injury model in rats using a free-falling weight drop device to subsequently investigate Nogo-A expression. Nogo-A mRNA and protein expression and immunoreactivity were detected in spinal cord tissue using real-time quantitative PCR, immunohistochemistry and western blot analysis. At 24 hours after spinal cord injury, Nogo-A protein and mRNA expression was low in the injured group compared with control and sham-operated groups. The levels then continued to drop further and were at their lowest at 3 days, rapidly rose to a peak after 7 days, and then gradually declined again after 14 days. These changes were observed at both the mRNA and protein level. The transient decrease observed early after injury followed by high levels for a few days indicates Nogo-A expression is time dependent. This may contribute to the lack of regeneration in the central nervous system after spinal cord injury. The dynamic variation of Nogo-A should be taken into account in the treatment of spinal cord injury.

  19. Botulinum toxin's axonal transport from periphery to the spinal cord.

    Matak, Ivica; Riederer, Peter; Lacković, Zdravko

    2012-07-01

    Axonal transport of enzymatically active botulinum toxin A (BTX-A) from periphery to the CNS has been described in facial and trigeminal nerve, leading to cleavage of synaptosomal-associated protein 25 (SNAP-25) in central nuclei. Aim of present study was to examine the existence of axonal transport of peripherally applied BTX-A to spinal cord via sciatic nerve. We employed BTX-A-cleaved SNAP-25 immunohistochemistry of lumbar spinal cord after intramuscular and subcutaneous hind limb injections, and intraneural BTX-A sciatic nerve injections. Truncated SNAP-25 in ipsilateral spinal cord ventral horns and dorsal horns appeared after single peripheral BTX-A administrations, even at low intramuscular dose applied (5 U/kg). Cleaved SNAP-25 appearance in the spinal cord after BTX-A injection into the sciatic nerve was prevented by proximal intrasciatic injection of colchicine (5 mM, 2 μl). Cleaved SNAP-25 in ventral horn, using choline-acetyltransferase (ChAT) double labeling, was localized within cholinergic neurons. These results extend the recent findings on BTX-A retrograde axonal transport in facial and trigeminal nerve. Appearance of truncated SNAP-25 in spinal cord following low-dose peripheral BTX-A suggest that the axonal transport of BTX-A occurs commonly following peripheral application. Copyright © 2012 Elsevier Ltd. All rights reserved.

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

    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.

  1. Scissors stab wound to the cervical spinal cord at the craniocervical junction.

    Zhang, Xiao-Yong; Yang, Ying-Ming

    2016-06-01

    Stab wounds resulting in spinal cord injury of the craniocervical junction are rare. A scissors stab wound to the cervical spinal cord has been reported only once in the literature. This paper aimed to report a case of Brown-Séquard-plus syndrome in an 8-year-old boy secondary to a scissors stab wound at the craniocervical junction. Case report and review of the literature. Case report of an 8-year-old boy accidentally stabbed in the neck by scissors, which were thrown as a dart. The case study of an 8-year-old boy who was hospitalized because of a scissors stab wound at the craniocervical junction. The patient developed Brown-Séquard-plus syndrome on the left side of the body. Magnetic resonance imaging revealed a laceration of the spinal cord at the craniocervical junction with cerebrospinal fluid leakage. Careful cleansing and interrupted sutures of the wounds were performed to prevent cerebrospinal fluid leakage. Rehabilitation therapy was performed 2 days later. A follow-up examination revealed complete recovery of the neurologic deficit 8 months post-injury. Treatment of scissors stab wounds to the cervical spinal cord, whether conservative management or thorough surgical exploration, should be individualized based on history, examination, and imaging. As shown in this case report, despite conservative management, complete recovery, which was unexpected, was attributed to the initial mild laceration of the spinal cord and ipsilateral spinal cord functional compensation. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. Central arterial stiffness and diastolic dysfunction are associated with insulin resistance and abdominal obesity in young women but polycystic ovary syndrome does not confer additional risk.

    Rees, E; Coulson, R; Dunstan, F; Evans, W D; Blundell, H L; Luzio, S D; Dunseath, G; Halcox, J P; Fraser, A G; Rees, D A

    2014-09-01

    Are arterial stiffness, carotid intima-media thickness and diastolic dysfunction increased in young women with polycystic ovary syndrome (PCOS) independently of the effects of obesity? Insulin resistance and central obesity are associated with subclinical cardiovascular dysfunction in young women, but a diagnosis of PCOS does not appear to confer additional risk at this age. Some studies have shown that young women with PCOS may have increased measures of cardiovascular risk, including arterial stiffness, carotid intima-media thickness and myocardial dysfunction. However, it is difficult to establish how much of this risk is due to PCOS per se and how much is due to obesity and insulin resistance, which are common in PCOS and themselves associated with greater vascular risk. This cross-sectional study comprised 84 women with PCOS and 95 healthy volunteers, aged 16-45 years. The study was conducted in a university hospital. Subjects underwent a comprehensive assessment of body composition (including computed tomography (CT) assessment of visceral fat; VF), measurements of arterial stiffness (aortic pulse wave velocity; aPWV), common carotid intima-media thickness (ccIMT), diastolic function (longitudinal tissue velocity; e':a') and endocrinological measures. A sample size of 80 in each group gave 80% power for detecting a difference of 0.45 m/s in aPWV or a difference of 0.25 in e':a'. After adjustment for age and body mass index (BMI), PCOS subjects had a greater insulin response (insulin area under the curve-IAUC) following glucose challenge (adjusted difference [AD] 35 900 pmol min/l, P insulin resistance were only partly attenuated by adjusting for logVF. There was no significant relationship between aPWV or e':a' and either testosterone or adiponectin. The study recruited young women meeting the Rotterdam criteria for PCOS diagnosis; hence our findings may not be generalizable to older patients or those meeting other definitions of the syndrome. Biochemical

  3. Neurogenic bladder in Hunter's syndrome.

    Koyama, K; Moda, Y; Sone, A; Tanaka, H; Hino, Y

    1994-01-01

    We encountered a rare patient with Hunter's syndrome who exhibited urinary retention as a result of a neurogenic bladder, uninhibited detrusor contractions, and detrusor-sphincter dyssynergia. Neurological findings were consistent with cervical myelopathy and cervical MR imaging showed very narrow segments at the cord level C2-4. We speculate that this Hunter's syndrome patient has cervical myelopathy and that this neurological dysfunction causes the neurogenic bladder. PMID:8014981

  4. Serial changes of magnetic resonance imaging of spinal cord lesions in multiple sclerosis

    Sugimura, Kimiya; Sekimoto, Yoichi; Koike, Yasuo; Takahashi, Akira

    1987-01-01

    Serial changes of magnetic resonance imaging (MRI) of spinal cord lesions in multiple sclerosis (MS) were demonstrated. Two inpatients of MS were followed-up for 8 and 5 months respectively. The first case was a 38-year-old housewife with lesions in upper cervical cord, medulla oblongata and visual nerve. The second case was a 45-year-old man with middle thracic spinal cord and brain stem lesions. Both cases were successfully induced into the remission by peroral prednisolone therapy. In the first case, in early stage of the disease, low signal (in IR method) and high signal (in T 2 -weighted SE method) intensities with enlarged lower dorsal medulla were demonstrated. The second MRI in this case specially in horizontal sections revealed round high intensity lesions (in T 2 -weighted SE) with clear margins, which appeared to push away the normal spinal cord tissue. In the third MRI, T 2 weighted SE revealed localized narrowing in C 2 and C 3 cervical cord, and even no signal lesions in IR method were shown in the central of the spinal cord. The forth and fifth MRI, however, showed almost normally recovered spinal cord and medulla oblongata. In the second case, the first MRI revealed high intensity lesions in the middle thracic spinal cord in T 2 weight SE, and moreover, the spinal cord looked very enlarged. In IR method, localized patchy low intensity lesions were seen in the enlarged spinal cord, but in this case, the MRI demonstrated that localized patchy high intensity lesions without cord swelling in SE remained long after the clinically complete recovery of the disease. (author)

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

    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.

  6. Epidural tumour (calcified fibroma) as cause of a 'Cervical Syndrome'

    Piotrowski, W.P.

    1983-01-01

    A calcified fibroma caused a so called cervical syndrome not responding to medical treatment. In the computerized tomography a compression of the cervical spinal cord could be demonstrated. From this the indication for the operation was given. (Author)

  7. Epidural tumour (calcified fibroma) as cause of a 'Cervical Syndrome'

    Piotrowski, W P

    1983-01-01

    A calcified fibroma caused a so called Cervical Syndrome not responding to medical treatment. In the computerized tomography a compression of the cervical spinal cord could be demonstrated. From this the indication for the operation was given.

  8. Triple A (Allgrove) syndrome: an unusual association with syringomyelia

    2013-01-01

    Triple A (Allgrove) syndrome was first described by Allgrove in 1978 in two pairs of siblings. Since then, about 100 cases have been reported, all of them displaying an autosomal recessive pattern of inheritance. Clinical picture is characterized by achalasia, alacrimia and ACTH-resistant adrenal failure. A progressive neurological syndrome including central, peripheral and autonomic nervous system impairment, and mild mental retardation is often associated. The triple A syndrome gene, designated AAAS, is localized on chromosome 12q13. It consists of 16 exons, encoding for a 546 aminoacid protein called ALADIN (Alacrimia-Achalasia-aDrenal Insufficiency Neurologic disorder). We report on a 13 year-old boy presenting with Addison’s disease, dysphagia, muscle weakness, excessive fatigue and recent onset gait ataxia. The analysis of the AAAS gene revealed a homozygous missense mutation in exon 12. It was a T > G transversion at nucleotide position 1224, resulting in a change of leucine at amino acid position 381 into arginine (Leu381Arg or L381R). Brain appearance was found normal at magnetic resonance imaging (MRI) and functional spectroscopy analysis showed normal levels of the main metabolites. Spine MRI showed a cystic cavity within the spinal cord (syringomyelia), localized between the sixth cervical vertebra and the first thoracic vertebra. Cerebellar tonsils descended 7 mm caudal to foramen magnum, consistently with a mild type 1 Chiari malformation. Mild posterior inter-vertebral disk protrusions were evident between T9 and T10 and between L4 and L5. To our knowledge, this is the first report describing type 1 Chiari malformation and multiple spinal cord abnormalities in a patient with Allgrove syndrome. PMID:23800107

  9. Morphometric changes in the spinal cord during prenatal life: a stereological study in sheep.

    Sadeghinezhad, Javad; Zadsar, Narges; Hasanzadeh, Beal

    2018-03-01

    This study describes the volumetric changes in the spinal cord during prenatal life in sheep using quantitative stereological methods. Twenty healthy sheep fetuses were included in the present study, divided into four groups representing 9-11, 12-14, 15-17, and 18-20 weeks of gestation. In each group, the spinal cord was dissected out and sampled according to the unbiased systematic random sampling method then used for stereological estimations. The total volume of spinal cord, volume of gray matter (GM), volume of white matter (WM), ratio of GM volume to WM volume, and volume of central canal (CC) were estimated in the whole spinal cord and its various regions using Cavalieri's principle. The total volume of the spinal cord increased 8 times from week 9 to week 20. The cervical region showed the greatest (9.7 times) and the sacral region the least (6.3 times) volumetric change. The CC volume of the whole spinal cord increased 5.8 times from week 9 to week 20. The cervical region developed faster (8.2 times) and the thoracic region slower (4.4 times) than the total spinal cord. During development, the volume ratio of GM to WM decreased from lower toward upper regions. The greatest volume changes occurred mostly in weeks 9-11 and 12-14. The cervical region showed the greatest volume changes in comparison with other regions of the spinal cord.

  10. Propitious Therapeutic Modulators to Prevent Blood-Spinal Cord Barrier Disruption in Spinal Cord Injury.

    Kumar, Hemant; Ropper, Alexander E; Lee, Soo-Hong; Han, Inbo

    2017-07-01

    The blood-spinal cord barrier (BSCB) is a specialized protective barrier that regulates the movement of molecules between blood vessels and the spinal cord parenchyma. Analogous to the blood-brain barrier (BBB), the BSCB plays a crucial role in maintaining the homeostasis and internal environmental stability of the central nervous system (CNS). After spinal cord injury (SCI), BSCB disruption leads to inflammatory cell invasion such as neutrophils and macrophages, contributing to permanent neurological disability. In this review, we focus on the major proteins mediating the BSCB disruption or BSCB repair after SCI. This review is composed of three parts. Section 1. SCI and the BSCB of the review describes critical events involved in the pathophysiology of SCI and their correlation with BSCB integrity/disruption. Section 2. Major proteins involved in BSCB disruption in SCI focuses on the actions of matrix metalloproteinases (MMPs), tumor necrosis factor alpha (TNF-α), heme oxygenase-1 (HO-1), angiopoietins (Angs), bradykinin, nitric oxide (NO), and endothelins (ETs) in BSCB disruption and repair. Section 3. Therapeutic approaches discusses the major therapeutic compounds utilized to date for the prevention of BSCB disruption in animal model of SCI through modulation of several proteins.

  11. High-field MR imaging of tethered cord

    Sigal, R.; Bicetre, L.; Blass, C.; Doyon, D.; Pariente, D.

    1986-01-01

    MR imaging examinations of 12 patients with tethered cord syndrome have been performed on a 1.5-T MR imaging unit. Patients ranged in age from 3 to 60 years. MR findings were compared with those of myelography and metrizamide CT in all cases. Five patients underwent surgical control and postoperative MR imaging. Sagittal and axial sections were obtained using a spin-echo multisection, multiecho technique. T1-axial weighted images (SE 600/25) were sufficient to locate the position of the tip of the conus. They also allowed identification of extraspinal and intraspinal lipomas and clear-cut demarcation form associated tethered cord. Drawbacks of MR imaging were lack of precise depiction of the bone structures and the fact that clear identification of abnormal roots was problematic. The craniovertebral junction was always checked; two asymptomatic Chiari malformations were visualized. This study leads the authors to conclude that MR imaging should be used as the examination of first choice in the management of tethered cord syndrome

  12. Pulpwood production in the North Central Region, 1988.

    Ronald L. Hackett; W. Brad Smith

    1990-01-01

    Lake States pulpwood production climbed to a record 8.1 million cords. Central States pulpwood production dropped 1% from 1987's production of 403 thousand cords. Pulpwood production is shown by county and species group for Michigan, Minnesota, Wisconsin, Iowa, Missouri, Illinois, and Indiana.

  13. Spinal cord: motor neuron diseases.

    Rezania, Kourosh; Roos, Raymond P

    2013-02-01

    Spinal cord motor neuron diseases affect lower motor neurons in the ventral horn. This article focuses on the most common spinal cord motor neuron disease, amyotrophic lateral sclerosis, which also affects upper motor neurons. Also discussed are other motor neuron diseases that only affect the lower motor neurons. Despite the identification of several genes associated with familial amyotrophic lateral sclerosis, the pathogenesis of this complex disease remains elusive. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Structural and Functional Substitution of Deleted Primary Sensory Neurons by New Growth from Intrinsic Spinal Cord Nerve Cells: An Alternative Concept in Reconstruction of Spinal Cord Circuits

    Nicholas D. James

    2017-07-01

    Full Text Available In a recent clinical report, return of the tendon stretch reflex was demonstrated after spinal cord surgery in a case of total traumatic brachial plexus avulsion injury. Peripheral nerve grafts had been implanted into the spinal cord to reconnect to the peripheral nerves for motor and sensory function. The dorsal root ganglia (DRG containing the primary sensory nerve cells had been surgically removed in order for secondary or spinal cord sensory neurons to extend into the periphery and replace the deleted DRG neurons. The present experimental study uses a rat injury model first to corroborate the clinical finding of a re-established spinal reflex arch, and second, to elucidate some of the potential mechanisms underlying these findings by means of morphological, immunohistochemical, and electrophysiological assessments. Our findings indicate that, after spinal cord surgery, the central nervous system sensory system could replace the traumatically detached original peripheral sensory connections through new neurite growth from dendrites.

  15. Surgical reconstruction of spinal cord circuit provides functional return in humans

    Thomas Carlstedt

    2017-01-01

    Full Text Available This mini review describes the current surgical strategy for restoring function after traumatic spinal nerve root avulsion in brachial or lumbosacral plexus injury in man. As this lesion is a spinal cord or central nervous injury functional return depends on spinal cord nerve cell growth within the central nervous system. Basic science, clinical research and human application has demonstrated good and useful motor function after ventral root avulsion followed by spinal cord reimplantation. Recently, sensory return could be demonstrated following spinal cord surgery bypassing the injured primary sensory neuron. Experimental data showed that most of the recovery depended on new growth reinnervating peripheral receptors. Restored sensory function and the return of spinal reflex was demonstrated by electrophysiology and functional magnetic resonance imaging of human cortex. This spinal cord surgery is a unique treatment of central nervous system injury resulting in useful functional return. Further improvements will not depend on surgical improvements. Adjuvant therapy aiming at ameliorating the activity in retinoic acid elements in dorsal root ganglion neurons could be a new therapeutic avenue in restoring spinal cord circuits after nerve root avulsion injury.

  16. MR microscopy of the cervical spinal cord

    Carvlin, M.J.; Asato, R.; Hackney, D.B.; Kassab, E.A.; Muraki, A.S.; Joseph, P.M.; Fielding, R.M.; Hennessy, M.J.

    1988-01-01

    High-resolution magnetic resonance (MR) imaging was performed on ten fresh cadaver cervical spinal cords in order to identify internal features of the spinal cord and to distinguish anatomy from artifact. Axial, sagittal, and coronal long repetition time (TR), long echo time (TE) and short TR, short TE spin-echo, gradient-echo, and inversion-recovery images were acquired at 1.5 T (Siemens), 1.9T, and 4.7T (Varian/Sisco) with an inplane resolution of 0.05-1mm. The dorsal and ventral horns of the gray matter as well as the lateral and posterior funiculi of the white matter were distinctly resolved from truncation artifacts in sagittal and axial images. In short TR, short TE, long TR, long TE spin-echo and gradient-echo (TR, 35 msec; TE, 7 msec; flip angle, 10 0 -90 0 ) images, the central gray matter demonstrated higher signal intensity than the white matter. These findings are in contradistinction to the image contrast typically observed in brain. High-resolution MR imaging techniques capable of demonstrating this anatomy in vivo are being developed

  17. SPINAL CORD- A CADAVERIC STUDY

    Vijayamma K. N

    2018-01-01

    Full Text Available BACKGROUND Spinal cord is situated within the vertebral canal extending from the lower end of the medulla oblongata at the upper border of first cervical vertebra. In early foetal life, it extends throughout the length of the vertebral canal, and at the time of birth, it reaches the level of third lumbar vertebra. In adult, it ends at the lower border of first lumbar vertebra and thereafter continued as filum terminale, which gets attached to tip of coccyx. Spinal cord is covered by three protective membranes called spinal meninges, diameter, arachnoid and pia mater. The diameter and arachnoid mater extent up to second sacral vertebra and the pia mater forms filum terminale and extend at the tip of coccyx. MATERIALS AND METHODS Forty spinal cord cadaveric specimen were studied by dissection method after exposing the vertebral canal. The roots of spinal nerve were sectioned on both sides and the cord is released along with its coverings. The dura and arachnoid mater were incised longitudinally and the subarachnoid space, blood vessels, nerve roots, ligament denticulata, cervical and lumbar enlargements were observed. The blood vessels including radicular arteries were also studied photographed. RESULTS The spinal cord is a highly vascular structure situated within the vertebral canal, covered by diameter, arachnoid mater and pia mater. Spinal dura is thicker anteriorly than posteriorly. The pia mater forms linea splendens, which extend along the whole length of the cord in front of the anterior median fissure. The average length of the cord is 38 cm. The length and breadth of cervical enlargement was more compared to lumbar enlargement. The number of rootlets in both dorsal and ventral roots accounts more in cervical compared to other regions of the cord. The ligament denticulata is a thin transparent bands of pia mater attached on either sides of the cord between the dorsal and ventral roots of spinal nerves. The tooth like extensions are well

  18. Cord Blood Chimerism And Relapse After Haplo-Cord Transplantation

    van Besien, Koen; Koshy, Nebu; Gergis, Usama; Mayer, Sebastian; Cushing, Melissa; Rennert, Hannah; Slotky, Ronit; Mark, Tomer; Pearse, Roger; Rossi, Adriana; Phillips, Adrienne; Vasovic, Liljana; Ferrante, Rosanna; Hsu, Michael; Shore, Tsiporah

    2018-01-01

    Haplo-cord stem cell transplantation combines the infusion of CD34 selected hematopoietic progenitors from a haplo-identical donor with an umbilical cord blood graft from an unrelated donor and allows faster count recovery, with low rates of disease recurrence and chronic GVHD. But the contribution of the umbilical cord blood graft to long-term transplant outcome remains unclear. We analyzed 39 recipients of haplo-cord transplants with AML and MDS, engrafted and in remission at 2 months. Median age was 66 (18-72) and all had intermediate, high, or very high risk disease. Less than 20% UCB chimerism in the CD33 lineage was associated with an increased rate of disease recurrence (54% vs 11% Pdisease recurrence (46% vs 12%, P=0.007) Persistent haplo-chimerism in the CD3 lineage was associated with an increased rate of disease recurrence (40% vs 15%, P=0.009) Chimerism did not predict for treatment related mortality. The cumulative incidence of acute GVHD by day 100 was 43%. The cumulative incidence of moderate/severe chronic GVHD was only 5%. Engraftment of the umbilical cord blood grafts provides powerful GVL effects which protect against disease recurrence and is associated with low risk of chronic GVHD. Engraftment of CD34 selected haplo-identical cells can lead to rapid development of circulating T-cells, but when these cells dominate, GVL-effects are limited and rates of disease recurrence are high. PMID:27333804

  19. Median nerve conduction velocity and central conduction time measured with somatosensory evoked potentials in thyroxine-treated infants with Down syndrome

    van Trotsenburg, A. S. Paul; Smit, Bert J.; Koelman, Johannes H. T. M.; Dekker-van der Sloot, Marijke; Ridder, Jeannette C. D.; Tijssen, Jan G. P.; de Vijlder, Jan J. M.; Vulsma, Thomas

    2006-01-01

    OBJECTIVE: The aim of this study was to determine whether thyroxine treatment would improve nerve conduction in infants with Down syndrome. METHODS: A single-center, nationwide, randomized, double-blind, clinical trial was performed. Neonates with Down syndrome were assigned randomly to thyroxine (N

  20. Expression of Lymphatic Markers in the Adult Rat Spinal Cord.

    Kaser-Eichberger, Alexandra; Schroedl, Falk; Bieler, Lara; Trost, Andrea; Bogner, Barbara; Runge, Christian; Tempfer, Herbert; Zaunmair, Pia; Kreutzer, Christina; Traweger, Andreas; Reitsamer, Herbert A; Couillard-Despres, Sebastien

    2016-01-01

    Under physiological conditions, lymphatic vessels are thought to be absent from the central nervous system (CNS), although they are widely distributed within the rest of the body. Recent work in the eye, i.e., another organ regarded as alymphatic, revealed numerous cells expressing lymphatic markers. As the latter can be involved in the response to pathological conditions, we addressed the presence of cells expressing lymphatic markers within the spinal cord by immunohistochemistry. Spinal cord of young adult Fisher rats was scrutinized for the co-expression of the lymphatic markers PROX1 and LYVE-1 with the cell type markers Iba1, CD68, PGP9.5, OLIG2. Rat skin served as positive control for the lymphatic markers. PROX1-immunoreactivity was detected in many nuclei throughout the spinal cord white and gray matter. These nuclei showed no association with LYVE-1. Expression of LYVE-1 could only be detected in cells at the spinal cord surface and in cells closely associated with blood vessels. These cells were found to co-express Iba1, a macrophage and microglia marker. Further, double labeling experiments using CD68, another marker found in microglia and macrophages, also displayed co-localization in the Iba1+ cells located at the spinal cord surface and those apposed to blood vessels. On the other hand, PROX1-expressing cells found in the parenchyma were lacking Iba1 or PGP9.5, but a significant fraction of those cells showed co-expression of the oligodendrocyte lineage marker OLIG2. Intriguingly, following spinal cord injury, LYVE-1-expressing cells assembled and reorganized into putative pre-vessel structures. As expected, the rat skin used as positive controls revealed classical lymphatic vessels, displaying PROX1+ nuclei surrounded by LYVE-1-immunoreactivity. Classical lymphatics were not detected in adult rat spinal cord. Nevertheless, numerous cells expressing either LYVE-1 or PROX1 were identified. Based on their localization and overlapping expression with

  1. Descending pain modulation in irritable bowel syndrome (IBS): a systematic review and meta-analysis.

    Chakiath, Rosemary J; Siddall, Philip J; Kellow, John E; Hush, Julia M; Jones, Mike P; Marcuzzi, Anna; Wrigley, Paul J

    2015-12-10

    Irritable bowel syndrome (IBS) is a common functional gastrointestinal disorder. While abdominal pain is a dominant symptom of IBS, many sufferers also report widespread hypersensitivity and present with other chronic pain conditions. The presence of widespread hypersensitivity and extra-intestinal pain conditions suggests central nervous dysfunction. While central nervous system dysfunction may involve the spinal cord (central sensitisation) and brain, this review will focus on one brain mechanism, descending pain modulation. We will conduct a comprehensive search for the articles indexed in the databases Ovid MEDLINE, Ovid Embase, Ovid PsycINFO and Cochrane Central Register of Controlled Trial (CENTRAL) from their inception to August 2015, that report on any aspect of descending pain modulation in irritable bowel syndrome. Two independent reviewers will screen studies for eligibility, assess risk of bias and extract relevant data. Results will be tabulated and, if possible, a meta-analysis will be carried out. The systematic review outlined in this protocol aims to summarise current knowledge regarding descending pain modulation in IBS. PROSPERO CRD42015024284.

  2. Suicide in a spinal cord injured population

    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)....

  3. Pattern Recognition of the Multiple Sclerosis Syndrome

    Stewart, Renee; Healey, Kathleen M.

    2017-01-01

    During recent decades, the autoimmune disease neuromyelitis optica spectrum disorder (NMOSD), once broadly classified under the umbrella of multiple sclerosis (MS), has been extended to include autoimmune inflammatory conditions of the central nervous system (CNS), which are now diagnosable with serum serological tests. These antibody-mediated inflammatory diseases of the CNS share a clinical presentation to MS. A number of practical learning points emerge in this review, which is geared toward the pattern recognition of optic neuritis, transverse myelitis, brainstem/cerebellar and hemispheric tumefactive demyelinating lesion (TDL)-associated MS, aquaporin-4-antibody and myelin oligodendrocyte glycoprotein (MOG)-antibody NMOSD, overlap syndrome, and some yet-to-be-defined/classified demyelinating disease, all unspecifically labeled under MS syndrome. The goal of this review is to increase clinicians’ awareness of the clinical nuances of the autoimmune conditions for MS and NMSOD, and to highlight highly suggestive patterns of clinical, paraclinical or imaging presentations in order to improve differentiation. With overlay in clinical manifestations between MS and NMOSD, magnetic resonance imaging (MRI) of the brain, orbits and spinal cord, serology, and most importantly, high index of suspicion based on pattern recognition, will help lead to the final diagnosis. PMID:29064441

  4. Radiation treatment of spinal cord neoplasms

    Smirnov, R.V.

    1982-01-01

    Results of radiation treatment of spinal cord neoplasms are presented. The results of combined (surgical and radiation) treatment of tumors are studied. On the whole it is noted that radiation treatment of initial spinal cord tumours is not practised on a large scale because of low radiostability of spinal cord

  5. Traumatic spinal cord injury in MR imaging

    Bronarski, J.; Wozniak, E.

    1993-01-01

    Spinal cord injuries in tetraplegics were briefly discussed on the basis of MR imaging. It was found that severe cervical spine trauma usually results in concussion - the complete transection of the cord is rare. A case of 19 years old male with total cord transection confirmed by MR imaging is described. (author)

  6. CORD PROLAPSE, ASSOCIATED FACTORS AND FETAL OUTCOME

    Several factors predispose to cord prolapse, amongst which are breech presentation, abnormal lie and presentation, hydramnios and long cord [2-3, 5-7]. Perinatal mortality is the most feared complication and often seen in up to 91% of cases [8-9]. Little is known about the pattern of umbilical cord prolapse in Cameroon as ...

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

    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.

  8. MRI in Lyme disease of the spinal cord

    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.)

  9. MRI in Lyme disease of the spinal cord

    Mantienne, C.; Catalaa, I.; Sevely, A.; Cognard, C.; Manelfe, C.; Albucher, J.F.

    2001-01-01

    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.)

  10. Lymphangioma of the spermatic cord

    Campos, Antônio Carlos Ligocki; Costa, Marco Aurélio Raeder da; Salvalaggio, Paolo Rogério de Oliveira; Torres, Luiz Fernando Bleggi; Coelho, Júlio Cézar Uili

    1998-01-01

    We describe a case of a 22-year-old man that had been submitted to a left herniorraphy 11 years previously to the present admission. He returned to our hospital with another mass in the same side of the groin. At operation, several small cysts linked to the spermatic cord were demonstrated. At this time, an histological exam demonstrated the presence of conective tissue. The final histology report confirmed the diagnosis of lymphangioma of the spermatic cord in the groin region. The patient w...

  11. Comparison of standard, prone and cine MRI in the evaluation of tethered cord

    Singh, Sukhjinder; Kline-Fath, Beth; Racadio, Judy M.; Bierbrauer, Karin; Salisbury, Shelia; Macaluso, Maurizio; Jackson, Elizabeth C.; Egelhoff, John C.

    2012-01-01

    Tethered cord syndrome (TCS) is defined by abnormal traction on the spinal cord that confines its movement. Surgical cord release usually stops neurological deterioration; therefore, early and accurate neuroradiological diagnosis is important. Supine MRI is the imaging modality of choice, but prone MRI and cine MRI can demonstrate cord movement. We compared the diagnostic accuracies of standard MRI, prone MRI and cine MRI in patients with clinical suspicion of TCS and evaluated inter-reader reliability for MR imaging. Children who underwent MRI for suspicion of TCS were retrospectively identified. Supine, prone and cine MRI studies were re-read by two pediatric neuroradiologists. Conus level, filum appearance and cord movement were documented. Thirteen of 49 children had tethered cord documented at surgery. Conus level had the highest diagnostic accuracy (sensitivity 69-77%, specificity 94%, positive predictive value 82-83%, negative predictive value 89-92%, correct diagnosis 88-90%) and highest between-reader concordance (98%). Prone and cine MRI did not add to the accuracy of the supine imaging. Conus level provides the highest diagnostic accuracy and inter-reader reliability in TCS. Until a larger series is evaluated, it remains questionable whether prone or cine MRI provides enough additional diagnostic information to warrant routine use. (orig.)

  12. Comparison of standard, prone and cine MRI in the evaluation of tethered cord

    Singh, Sukhjinder [Cohen Children' s Medical Center, Department of Radiology, New Hyde Park, NY (United States); Kline-Fath, Beth; Racadio, Judy M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Bierbrauer, Karin [Cincinnati Children' s Hospital Medical Center, Department of Neurosurgery, Cincinnati, OH (United States); Salisbury, Shelia; Macaluso, Maurizio [Cincinnati Children' s Hospital Medical Center, Division of Biostatistics and Epidemiology, Cincinnati, OH (United States); Jackson, Elizabeth C. [Cincinnati Children' s Hospital Medical Center, Department of Pediatrics, Division of Nephrology, Cincinnati, OH (United States); Egelhoff, John C. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Phoenix Children' s Hospital, Department of Radiology, Phoenix, AZ (United States)

    2012-06-15

    Tethered cord syndrome (TCS) is defined by abnormal traction on the spinal cord that confines its movement. Surgical cord release usually stops neurological deterioration; therefore, early and accurate neuroradiological diagnosis is important. Supine MRI is the imaging modality of choice, but prone MRI and cine MRI can demonstrate cord movement. We compared the diagnostic accuracies of standard MRI, prone MRI and cine MRI in patients with clinical suspicion of TCS and evaluated inter-reader reliability for MR imaging. Children who underwent MRI for suspicion of TCS were retrospectively identified. Supine, prone and cine MRI studies were re-read by two pediatric neuroradiologists. Conus level, filum appearance and cord movement were documented. Thirteen of 49 children had tethered cord documented at surgery. Conus level had the highest diagnostic accuracy (sensitivity 69-77%, specificity 94%, positive predictive value 82-83%, negative predictive value 89-92%, correct diagnosis 88-90%) and highest between-reader concordance (98%). Prone and cine MRI did not add to the accuracy of the supine imaging. Conus level provides the highest diagnostic accuracy and inter-reader reliability in TCS. Until a larger series is evaluated, it remains questionable whether prone or cine MRI provides enough additional diagnostic information to warrant routine use. (orig.)

  13. 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

    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.

  14. Electromechanical gait training with functional electrical stimulation: case studies in spinal cord injury.

    Hesse, S; Werner, C; Bardeleben, A

    2004-06-01

    Single case studies. To describe the technique of intensive locomotor training on an electromechanical gait trainer (GT) combined with functional electrical stimulation (FES). Neurological Rehabilitation Clinic, Berlin, Germany. Four spinal cord-injured (SCI) patients, one tetraparetic, two paraparetic, and one patient with an incomplete cauda syndrome, more than 3 months postinjury, who were unable to walk at all, or with two therapists. They received 25 min of locomotor training on the GT plus FES daily for 5 weeks in addition to the regular therapy. The patients tolerated the programme well, and therapists rated the programme less strenuous compared to manually assisted treadmill training. Gait ability improved in all four patients; three patients could walk independently on the floor with the help of technical aids, and one required the help of one therapist after therapy; gait speed and endurance more than doubled, and the gastrocnemius activity increased in the patients with a central paresis. This combined technique allows intensive locomotor therapy in SCI subjects with reduced effort from the therapists. The patients' improved walking ability confirmed the potential of locomotor therapy in SCI subjects.

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

    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...

  16. MR imaging and spinal cord injury

    Azar-Kia, B.; Fine, M.; Naheedy, M.; Elias, D.

    1987-01-01

    MR imaging has significantly improved diagnostic capability of spinal cord injuries. Other available diagnostic modalities such as plain films, myelography, CT, and post-CT myelography have failed to consistently show the secific evidence of spinal cord injuries and their true extent. The authors are presenting our experiences with MR imaging in spinal column injury. They have found MR imaging to be the procedure of choice for prognostic evaluation of spinal cord trauma. They are showing examples of recent and old spinal cord injury such as hematomyelia, myelomalacia, transection, spinal cord edema, and cavitation

  17. Distribution of elements in human spinal cord

    Yukawa, Masae; Kobayashi, T.; Qiu, Y.; Kameda, N.; Ito, Y.; Otomo, E.

    1992-01-01

    The distribution of elements in human spinal cord was investigated on unfixed frozen cord material using PIXE technique. Distribution of Cu, Zn and Fe were not uniform in the cross section of the spinal cord and concentrations of these elements were higher in the anterior gray horn than in the other areas, while K and Cl distributed uniformly. The content of K changed along the spinal cord from the cervical to the lumbar level. These findings are discussed in relation to current understanding of the physiology of the spinal cord. (author)

  18. Physiological Activity of Spinal Cord in Children: An 18F-FDG PET-CT Study.

    Taralli, Silvia; Leccisotti, Lucia; Mattoli, Maria Vittoria; Castaldi, Paola; de Waure, Chiara; Mancuso, Agostino; Rufini, Vittoria

    2015-06-01

    Retrospective study. To evaluate, in a pediatric population, F-Fluoro-deoxy-glucose (F-FDG) metabolic activity of normal spinal cord and to assess the correlation with demographic, clinical, and environmental variables. F-FDG uptake of normal spinal cord is variable in children. The knowledge of physiological metabolism of spinal cord is essential to distinguish normal from pathological findings by positron emission tomography-computed tomography (PET-CT). We retrospectively evaluated F-FDG positron emission tomography-computed tomography scans from a total of 167 pediatric patients (97 males; 3.9-18.9 yr) divided into 4 age groups (0-4.9 yr, 5-9.9 yr, 10-14.9 yr, and 15-18.9 yr), excluding those submitted to previous or recent therapeutic procedures influencing spinal cord metabolism or with central nervous system diseases. Spinal cord was divided into 3 levels (C1-C7; D1-D6; and D7-L1), and maximum standardized uptake value (SUVmax) of each cord level was measured. Correlations between SUVmax and spinal cord level, age, body weight, sex, type of disease, and season were statistically assessed. Median SUVmax was similar and significantly (P spinal cord levels. A positive and significant association between SUVmax and body weight, female sex, and Hodgkin lymphoma was found. No significant association with season was observed. By multivariate analysis, only weight and female sex remained significant. Knowledge of physiological F-FDG spinal cord activity in children is essential for a correct interpretation of positron emission tomography-computed tomography, especially in oncologic pediatric patients to avoid potential pitfalls. N/A.

  19. Cytoarchitecture of the spinal cord of the postnatal (P4) mouse.

    Sengul, Gulgun; Puchalski, Ralph B; Watson, Charles

    2012-05-01

    Interpretation of the new wealth of gene expression and molecular mechanisms in the developing mouse spinal cord requires an accurate anatomical base on which data can be mapped. Therefore, we have assembled a spinal cord atlas of the P4 mouse to facilitate direct comparison with the adult specimens and to contribute to studies of the development of the mouse spinal cord. This study presents the anatomy of the spinal cord of the P4 C57Bl/6J mouse using Nissl and acetyl cholinesterase-stained sections. It includes a detailed map of the laminar organization of selected spinal cord segments and a description of named cell groups of the spinal cord such as the central cervical (CeCv), lateral spinal nucleus, lateral cervical, and dorsal nuclei. The motor neuron groups have also been identified according to the muscle groups they are likely to supply. General features of Rexed's laminae of the P4 spinal cord showed similarities to that of the adult (P56). However, certain differences were observed with regard to the extent of laminae and location of certain cell groups, such as the dorsal nucleus having a more dispersed structure and a more ventral and medial position or the CeCv being located in the medial part of lamina 5 in contrast to the adult where it is located in lamina 7. Motor neuron pools appeared to be more tightly packed in the P4 spinal cord. The dorsal horn was relatively larger and there was more white matter in the P56 spinal cord. Copyright © 2012 Wiley Periodicals, Inc.

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

    Ferguson, Adam R.; Huie, J. Russell; Crown, Eric D.; Baumbauer, Kyle M.; Hook, Michelle A.; Garraway, Sandra M.; Lee, Kuan H.; Hoy, Kevin C.; Grau, James W.

    2012-01-01

    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. A 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 in response to various noxious inputs, 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 below the level of SCI. The literature demonstrates that activity-dependent plasticity within the spinal cord must be carefully tuned to promote adaptive spinal training. Prior work from our group has shown that 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 SCI. We review these basic phenomena, how these findings relate to the broader spinal plasticity literature, discuss the cellular and molecular mechanisms, and finally discuss implications of these and other findings for improved rehabilitative therapies after SCI. PMID

  1. Spinal cord infarction: MR imaging and clinical features in 16 cases

    Weidauer, Stefan; Lanfermann, Heinrich; Zanella, Friedhelm E.; Nichtweiss, Michael

    2002-01-01

    Spinal cord infarctions are rare and due to heterogeneous etiologies. The aim of the study was to analyze the MR imaging findings and evaluate their correlations with clinical symptoms in ischemic spinal cord lesions. MR images and clinical features of 16 patients (11 male, 5 female) with typical sudden onset of neurological deficits caused by spinal cord ischemia were evaluated. MR imaging was performed within 2 h to 14 days after the initial neurological symptoms. Eight patients had follow-up examinations including contrast-enhanced MR imaging. MR abnormalities were best demonstrated on sagittal T2-weighted images, with ''pencil-like'' hyperintensities (16/16) and cord enlargement (9/16). Axial T2-weighted images showed bilateral (13/16) and unilateral (3/16) hyperintensities according, in 15 patients, to anterior spinal artery (ASA) territory, with three of them located particularly in the spinal sulcal artery territory. In one patient only the posterior spinal artery (PSA) territory was involved. Spinal cord was affected at the cervical level (especially C2-C3) in seven patients, at the upper thoracic level (T3-T5) in two patients and at the thoracolumbar region including the conus medullaris (T10-L1) in seven patients. Presumed etiologies were vascular surgery (3 patients), infrarenal aortic aneurysm (1 patient), bilateral vertebral artery dissection (1 patient), hypotension (1 patient), spine operation (1 patient), excessive cocaine misuse (1 patient) and cardioembolic vertebral artery occlusion (1 patient); six of seven patients with unclear etiologies had vascular risk factors such as hypertension, diabetes and cigarette smoking. MR imaging is therefore useful in detecting spinal cord infarction, with axial T2-weighted images showing hyperintensities in the ASA territory in 15 of 16 patients. Contrary to the presumed spinal cord watershed at the lower cervical and upper thoracic level, and despite numerous central arteries in the cervical cord, our data

  2. Activated microglia in the spinal cord underlies diabetic neuropathic pain.

    Wang, Dongmei; Couture, Réjean; Hong, Yanguo

    2014-04-05

    Diabetes mellitus is an increasingly common chronic medical condition. Approximately 30% of diabetic patients develop neuropathic pain, manifested as spontaneous pain, hyperalgesia and allodynia. Hyperglycemia induces metabolic changes in peripheral tissues and enhances oxidative stress in nerve fibers. The damages and subsequent reactive inflammation affect structural properties of Schwann cells and axons leading to the release of neuropoietic mediators, such as pro-inflammatory cytokines and pro-nociceptive mediators. Therefore, diabetic neuropathic pain (DNP) shares some histological features and underlying mechanisms with traumatic neuropathy. DNP displays, however, other distinct features; for instance, sensory input to the spinal cord decreases rather than increasing in diabetic patients. Consequently, development of central sensitization in DNP involves mechanisms that are distinct from traumatic neuropathic pain. In DNP, the contribution of spinal cord microglia activation to central sensitization and pain processes is emerging as a new concept. Besides inflammation in the periphery, hyperglycemia and the resulting production of reactive oxygen species affect the local microenvironment in the spinal cord. All these alterations could trigger resting and sessile microglia to the activated phenotype. In turn, microglia synthesize and release pro-inflammatory cytokines and neuroactive molecules capable of inducing hyperactivity of spinal nociceptive neurons. Hence, it is imperative to elucidate glial mechanisms underlying DNP for the development of effective therapeutic agents. The present review highlights the recent developments regarding the contribution of spinal microglia as compelling target for the treatment of DNP. Copyright © 2014 Elsevier B.V. All rights reserved.

  3. Serotonin syndrome

    Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

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

    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.

  5. Spinal cord injury at birth

    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...

  6. Churg Strauss syndrome

    Lopez Rengifo, Diana Milena; Contreras Zuniga, Eduardo; Osio, Luis Fernando

    2007-01-01

    The Churg-Strauss syndrome, also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. The most common organ involved is the lung, followed by the skin. The Churg-Strauss syndrome, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems

  7. The stress ulcer syndrome

    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

  8. Work-associated irritable larynx syndrome.

    Anderson, Jennifer A

    2015-04-01

    The purpose of this study is to review the relevant literature concerning work-associated irritable larynx syndrome (WILS), a hyperkinetic laryngeal disorder associated with occupational irritant exposure. Clinical symptoms are variable and include dysphonia, cough, dyspnoea and globus pharyngeus. WILS is a clinical diagnosis and can be difficult to differentiate from asthma. Treatment options for WILS include medical and behavioural therapy. Laryngeal-centred upper airway symptoms secondary to airborne irritants have been documented in the literature under a variety of diagnostic labels, including WILS, vocal cord dysfunction (VCD), laryngeal hypersensitivity and laryngeal neuropathy and many others. The underlying pathophysiology is as yet poorly understood; however, the clinical scenario suggests a multifactorial nature to the disorder. More recent literature indicates that central neuronal plasticity, inflammatory processes and psychological factors are all likely contributors. Possible mechanisms for WILS include central neuronal network plasticity after noxious exposure and/or viral infection, inflammation (i.e. reflux disease) and intrinsic patient factors such a psychological state. Treatment is individualized and frequently includes one or more of the following: environmental changes in the workplace, GERD therapy, behavioural/speech therapy, psychotherapy counselling and neural modifiers.

  9. Apert Syndrome: A Case Report

    Masoud Gharib

    2012-10-01

    Full Text Available Apert syndrome is a genetic defect which was first described by Eugene Apert in 1906. it's incidence is approximately one in 50000 births. This syndrome is many abnormalities in your body and Central Nervous System. rehabilitation can increase children and their parent's quality of life.We report a case of Apert syndrome and his occupational therapy program.

  10. Outcomes in Treatment for Intradural Spinal Cord Ependymomas

    Volpp, P. Brian; Han, Khanh; Kagan, A. Robert; Tome, Michael

    2007-01-01

    Purpose: Spinal cord ependymomas are rare tumors, accounting for <2% of all primary central nervous system tumors. This study assessed the treatment outcomes for patients diagnosed with spinal cord ependymomas within the Southern California Kaiser Permanente system. Methods and Materials: We studied 23 patients treated with surgery with or without external beam radiotherapy (EBRT). The local and distant control rates and overall survival rates were determined. Results: The overall local control, overall recurrence, and 9-year overall survival rate was 96%, 17.4%, and 63.9%, respectively. Conclusions: The results of our study indicate that en bloc gross total resection should be the initial treatment, with radiotherapy reserved primarily for postoperative cases with unfavorable characteristics such as residual tumor, anaplastic histologic features, or piecemeal resection. Excellent local control and overall survival rates can be achieved using modern microsurgical techniques, with or without local radiotherapy

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

    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.

  12. Cord Blood Banking for Potential Future Transplantation.

    Shearer, William T; Lubin, Bertram H; Cairo, Mitchell S; Notarangelo, Luigi D

    2017-11-01

    This policy statement is intended to provide information to guide pediatricians, obstetricians, and other medical specialists and health care providers in responding to parents' questions about cord blood donation and banking as well as the types (public versus private) and quality of cord blood banks. Cord blood is an excellent source of stem cells for hematopoietic stem cell transplantation in children with some fatal diseases. Cord blood transplantation offers another method of definitive therapy for infants, children, and adults with certain hematologic malignancies, hemoglobinopathies, severe forms of T-lymphocyte and other immunodeficiencies, and metabolic diseases. The development of universal screening for severe immunodeficiency assay in a growing number of states is likely to increase the number of cord blood transplants. Both public and private cord blood banks worldwide hold hundreds of thousands of cord blood units designated for the treatment of fatal or debilitating illnesses. The procurement, characterization, and cryopreservation of cord blood is free for families who choose public banking. However, the family cost for private banking is significant and not covered by insurance, and the unit may never be used. Quality-assessment reviews by several national and international accrediting bodies show private cord blood banks to be underused for treatment, less regulated for quality control, and more expensive for the family than public cord blood banks. There is an unquestionable need to study the use of cord blood banking to make new and important alternative means of reconstituting the hematopoietic blood system in patients with malignancies and blood disorders and possibly regenerating tissue systems in the future. Recommendations regarding appropriate ethical and operational standards (including informed consent policies, financial disclosures, and conflict-of-interest policies) are provided for physicians, institutions, and organizations that

  13. Central nervous system radiation injury in small animal models

    Kogel, A.J. van der

    1991-01-01

    Experimental studies on radiation injury in the central nervous system have been carried out in many species ranging from mouse to monkey. This review is restricted to studies in rodents irradiated with low linear energy transfer (LET) radiation. In this paper, the various rodent models of brain and spinal cord injury are described with particular emphasis on the pathology of different types of lesions and theories of their pathogenesis. Many of the initial studies were limited to relatively high single doses, but in later work more clinically relevant fractionated irradiation schemes were employed. This has led to the recognition of various types of early and late delayed injury that are analogous to the syndromes observed in humans. Two main pathways have been suggested for the pathogenesis, one involving predominantly the progressive loss of glial cells and the other involving vascular injury. The relative importance of both mechanisms will be discussed with respect to treatment conditions and to dose level in particular. An hypothesis is presented concerning the possible role of different cell types in the development of specific syndromes

  14. Elsberg syndrome: A rarely recognized cause of cauda equina syndrome and lower thoracic myelitis.

    Savoldi, Filippo; Kaufmann, Timothy J; Flanagan, Eoin P; Toledano, Michel; Weinshenker, Brian G

    2017-07-01

    Elsberg syndrome (ES) is an established but often unrecognized cause of acute lumbosacral radiculitis with myelitis related to recent herpes virus infection. We defined ES, determined its frequency in patients with cauda equina syndrome (CES) with myelitis, and evaluated its clinical, radiologic, and microbiologic features and outcomes. We searched the Mayo Clinic medical records for ES and subsequently for combinations of index terms to identify patients with suspected CES and myelitis. Our search yielded 30 patients, 2 diagnosed with ES and an additional 28 with clinical or radiologic evidence of CES retrospectively suspected of having ES. We classified patients in 5 groups according to diagnostic certainty. MRI and EMG confirmed that 2 had only myelitis, 5 only radiculitis, and 16 both. Two had preceding sacral herpes infection and 1 oral herpes simplex. Spinal cord lesions were commonly multiple, discontinuous, not expansile, and centrally or ventrally positioned. Lesions generally spared the distal conus. Nerve root enhancement was occasionally prominent and was smooth rather than nodular. Lymphocytic CSF pleocytosis was common. Thirteen patients (43%) had viral isolation studies, which were commonly delayed; the delay may have accounted for the low rate of viral detection. Acyclovir was administered to 6 patients. Most patients recovered with sequelae; 1 patient experienced encephalomyelitis and died. ES is a definable condition likely responsible for 10% of patients with combined CES and myelitis. Radiologic findings are not entirely specific but may help in differentiating ES from some competing diagnostic considerations. We propose criteria to facilitate diagnosis.

  15. The role of CO2 and central chemoreception in the control of breathing in the fetus and the neonate

    Darnall, Robert A.

    2010-01-01

    Central chemoreception is active early in development and likely drives fetal breathing movements, which are influenced by a combination of behavioral state and powerful inhibition. In the premature human infant and newborn rat ventilation increases in response to CO2; in the rat the sensitivity of the response increases steadily after ~P12. The premature human infant is more vulnerable to instability than the newborn rat and exhibits periodic breathing that is augmented by hypoxia and eliminated by breathing oxygen or CO2 or the administration of respiratory stimulants. The sites of central chemoreception active in the fetus are not known, but may involve the parafacial respiratory group which may be a precursor to the adult RTN. The fetal and neonatal rat brainstem spinal-cord preparations promise to provide important information about central chemoreception in the developing rodent and will increase our understanding of important clinical problems, including The Sudden Infant Death Syndrome, Congenital Central Hypoventilation Syndrome, and periodic breathing and apnea of prematurity. PMID:20399912

  16. Placental cord insertion and birthweight discordance in twin pregnancies: results of the national prospective ESPRiT Study.

    Kent, Etaoin M; Breathnach, Fionnuala M; Gillan, John E; McAuliffe, Fionnuala M; Geary, Michael P; Daly, Sean; Higgins, John R; Dornan, James; Morrison, John J; Burke, Gerard; Higgins, Shane; Carroll, Stephen; Dicker, Patrick; Manning, Fiona; Malone, Fergal D

    2011-10-01

    The purpose of this study was to evaluate the impact of noncentral placental cord insertion on birthweight discordance in twins. We performed a multicenter, prospective trial of twin pregnancies. Placental cord insertion was documented as central, marginal, or velamentous according to a defined protocol. Association of the placental cord insertion site with chorionicity, birthweight discordance, and growth restriction were assessed. Eight hundred sixteen twin pairs were evaluated; 165 pairs were monochorionic, and 651 pairs were dichorionic. Monochorionic twins had higher rates of marginal (P = .0068) and velamentous (P < .0001) placental cord insertion. Noncentral placental cord insertion was more frequent in smaller twins of discordant pairs than control pairs (29.8% vs 19.1%; P = .004). Velamentous placental cord insertion in monochorionic twins was associated significantly with birthweight discordance (odds ratio, 3.5; 95% confidence interval, 1.3-9.4) and growth restriction (odds ratio, 4; 95% confidence interval, 1.1-14.3). Noncentral placental cord insertion contributes to birthweight discordance in monochorionic twin pregnancies. Sonographic delineation of placental cord insertion may be of value in antenatal assessment of twin pregnancies. Copyright © 2011 Mosby, Inc. All rights reserved.

  17. New allergies after cord blood transplantation.

    Vaughan, Leigh Ann; Vu, Mary; Sengsayadeth, Salyka; Lucid, Catherine; Clifton, Carey; McCarty, Karen; Hagaman, David; Domm, Jennifer; Kassim, Adetola; Chinratanalab, Wichai; Goodman, Stacey; Greer, John; Frangoul, Haydar; Engelhardt, Brian G; Jagasia, Madan; Savani, Bipin N

    2013-10-01

    Umbilical cord blood transplantation (CBT) is an effective treatment for benign and malignant diseases. Late effects of CBT are not well described in the literature. In the present study, we present our experience of new-onset allergies in long-term survivors after CBT. After an initial patient had a severe peanut allergic reaction after CBT, all CBT patients were prospectively followed for new allergy development. Fifty patients received CBT between March 2006 and June 2011. The median follow-up after CBT was 447 days (range, 12-2022). At the time of analysis, 30 patients were alive, with 3-year survival of 55.5%; median follow-up of surviving patients was 910 days (range, 68-2022). The allergic syndrome developed in five patients, with the cumulative incidence of new allergies at 2 years of 18.4% (95% confidence interval, 10.8-26). The median time to onset of new allergy after transplantation was 298 days (range, 250-809). Allergy development has been linked to a delayed maturation of the immune system in several studies. We present the first case series of patients who had new allergies after CBT. Further study of this novel complication as well as counseling of patients after CBT would be important. Copyright © 2013 International Society for Cellular Therapy. Published by Elsevier Inc. All rights reserved.

  18. Spinal cord stimulation: Current applications for treatment of chronic pain.

    Vannemreddy, Prasad; Slavin, Konstantin V

    2011-01-01

    Spinal cord stimulation (SCS) is thought to relieve chronic intractable pain by stimulating nerve fibers in the spinal cord. The resulting impulses in the fibers may inhibit the conduction of pain signals to the brain, according to the pain gate theory proposed by Melzack and Wall in 1965 and the sensation of pain is thus blocked. Although SCS may reduce pain, it will not eliminate it. After a period of concern about safety and efficacy, SCS is now regaining popularity among pain specialists for the treatment of chronic pain. The sympatholytic effect of SCS is one of its most interesting therapeutic properties. This effect is considered responsible for the effectiveness of SCS in peripheral ischemia, and at least some cases of complex regional pain syndrome. The sympatholytic effect has also been considered part of the management of other chronic pain states such as failed back surgery syndrome, phantom pain, diabetic neuropathy, and postherpetic neuralgia. In general, SCS is part of an overall treatment strategy and is used only after the more conservative treatments have failed. The concept of SCS has evolved rapidly following the technological advances that have produced leads with multiple contact electrodes and battery systems. The current prevalence of patients with chronic pain requiring treatment other than conventional medical management has significantly increased and so has been the need for SCS. With the cost benefit analysis showing significant support for SCS, it may be appropriate to offer this as an effective alternative treatment for these patients.

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

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

    2016-10-01

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

  20. Spinal Meninges and Their Role in Spinal Cord Injury: A Neuroanatomical Review.

    Grassner, Lukas; Grillhösl, Andreas; Griessenauer, Christoph J; Thomé, Claudius; Bühren, Volker; Strowitzki, Martin; Winkler, Peter A

    2018-02-01

    Current recommendations support early surgical decompression and blood pressure augmentation after traumatic spinal cord injury (SCI). Elevated intraspinal pressure (ISP), however, has probably been underestimated in the pathophysiology of SCI. Recent studies provide some evidence that ISP measurements and durotomy may be beneficial for individuals suffering from SCI. Compression of the spinal cord against the meninges in SCI patients causes a "compartment-like" syndrome. In such cases, intentional durotomy with augmentative duroplasty to reduce ISP and improve spinal cord perfusion pressure (SCPP) may be indicated. Prior to performing these procedures routinely, profound knowledge of the spinal meninges is essential. Here, we provide an in-depth review of relevant literature along with neuroanatomical illustrations and imaging correlates.