1/72. Demyelination of retinal myelinated nerve fibers in Behcet's disease.A 39 year-old Behcet's patient had demyelination of retinal myelinated nerve fibers after recurrent papillitis and vitritis. Oral prednisolone, cyclosporine, and later periocular corticosteroids and oral colchicine were used but demyelination continued over a 5 year-period. A pseudobulbar palsy with urinary incontinence and pyramidal tract signs developed and azathioprine and corticosteroids were used. Demyelination of retinal nerve fibers stopped and while treatment was underway, the central nervous system (CNS) signs were stable. While the ocular pathology of Behcet's can mirror the CNS signs, indeed optic nerve ischemic demyelination may signal the potential for CNS involvement; azathioprine with prednisone may be more effective in the long-term for optic nerve and CNS involvement than cyclosporine with prednisone.- - - - - - - - - - ranking = 1keywords = pseudobulbar, palsy (Clic here for more details about this article) |
2/72. A case of leukoencephalopathy with vanishing white matter.A 6-year old Turkish boy with a recently defined entity: "leukoencephalopathy with vanishing white matter" is described. He was born to consanguinous parents. His psychomotor development was normal till he first presented with fever and generalized tonic-clonic seizures at the age of 2.5, followed by rapid motor and mental deterioration. Decerebrate posture and marked spasticity subsequently developed. The initial MRI examination showed diffuse involvement of white matter, including subcortical U-fibers, with signal intensity parallel to CSF on all sequences. The white matter appeared swollen. The ventricles were slightly enlarged and there was cavum septi pellucidi et vergae. The posterior crus of the internal capsule, external and extreme capsules were affected. Cerebellar hemispheres and vermis showed atrophy. The involvement pattern of brainstem was noteworthy in that pontine tegmentum and cruri cerebri were affected. Follow-up MRI obtained after three years did not show any interval change. brain biopsy showed thinned cortex with relatively preserved cortical layering and neuronal structure. There was rarefaction of the white matter with cystic degeneration. Fibrillary gliosis and increased number of oligodendroglial cells were observed within the cerebral white matter.- - - - - - - - - - ranking = 0.0025892471133305keywords = spastic (Clic here for more details about this article) |
3/72. Accommodation and convergence palsy caused by lesions in the bilateral rostral superior colliculus.PURPOSE: To report a patient who developed accommodation and convergence palsy caused by lesions in the bilateral rostral superior colliculus. DESIGN: Observational case report. methods: A 30-year-old right-handed man experienced sudden onset of diplopia and blurred vision at near vision. RESULTS: The patient showed accommodation and convergence palsy. magnetic resonance imaging revealed lesions located in the bilateral rostral superior colliculus. CONCLUSION: These findings suggest that the rostral superior colliculus is involved in the control of accommodation and vergence eye movements.- - - - - - - - - - ranking = 0.0028803280541854keywords = palsy (Clic here for more details about this article) |
4/72. Episodic coma in a new leukodystrophy.Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.- - - - - - - - - - ranking = 0.0025892471133305keywords = spastic (Clic here for more details about this article) |
5/72. Neuroaxonal leukoencephalopathy with axonal spheroids.A 51-year-old woman with no significant family history exhibited progressive presenile dementia followed by right-sided spastic hemiplegia and died 27 months after the onset of her illness. brain MRI demonstrated a widespread abnormality in the cerebral deep white matter and corpus callosum. Neuropathologically, extensive destruction of axons and myelin and abundant axonal spheroids were found in the deep white matter, preferentially of the frontoparietal areas. The corpus callosum was also severely damaged. The cerebral cortex and subcortical U fibers remained intact. The pathological features were different among the lesions, reflecting the sequential degenerative processes of the white matter. This case is, to our knowledge, the third sporadic case of neuroaxonal leukoencephalopathy with axonal spheroids.- - - - - - - - - - ranking = 0.0025892471133305keywords = spastic (Clic here for more details about this article) |
6/72. Fulminant ascending paralysis as a delayed sequela of diethylene glycol (Sterno) ingestion.The authors report a 57-year-old man who survived 18 days after swallowing an 8-oz. can of Sterno in a suicide attempt. Two days after ingestion, he developed confusion and acute renal failure requiring hemodialysis, followed on day 8 by a delayed but rapidly evolving ascending paralysis. Pathologic examination showed severe demyelination, with lesser axonal damage, of virtually all cranial and peripheral nerves sampled and sparing of central myelin. The diethylene glycol in the Sterno was considered responsible for this intoxication.- - - - - - - - - - ranking = 0.0012541414900235keywords = paralysis (Clic here for more details about this article) |
7/72. adult onset pigmentary orthochromatic leukodystrophy with ovarian dysgenesis.Pigmentary type of orthochromatic leukodystrophy (POLD) is an adult-onset leukodystrophy, characterized pathologically by the presence of glial and microglial cytoplasmic pigment inclusions. The complete phenotype, genotype and pathogenetic mechanisms in POLD have not been elucidated. We followed for 18 years a woman with autopsy-proven POLD, who presented with 'frontal' dementia and spasticity. Her further course was marked by progressive mutism, apraxia and seizures. Her sister had died of the same disease after a much more rapidly progressing course. These sisters had primary infertility with pathologic evidence of streak ovaries. Diagnosis was confirmed in both cases by post-mortem examination. POLD is a rare cause of adult-onset leukodystrophy presenting with dementia. Ovarian dysgenesis is extremely rare in the absence of demonstrable chromosomal abnormalities and extends the clinical spectrum of POLD.- - - - - - - - - - ranking = 0.0025892471133305keywords = spastic (Clic here for more details about this article) |
8/72. Early peripheral nervous system manifestations of infantile Krabbe disease.Early infantile Krabbe disease is relatively frequent in the Muslim-Arab population in israel. It can be easily diagnosed when it presents with the classic clinical picture characterized by central nervous system manifestations of spasticity, irritability, motor regression and seizures associated with a positive family history. We studied eight children diagnosed with Krabbe disease. In two of these children (25%), peripheral neuropathy was the single initial symptom and the only neurologic finding noted for a period of months. In these patients, diagnosis of Krabbe's disease was delayed and established only 9-11 months after the initial symptoms. In two other children with "classical picture" Krabbe disease, areflexia was noted on admission. The occurrence of peripheral neuropathy as an initial symptom in early infantile Krabbe disease may be underestimated. Krabbe disease should be considered in the differential diagnosis of early infantile peripheral neuropathy. early diagnosis of affected children might be important for genetic counseling for families at risk.- - - - - - - - - - ranking = 0.0025892471133305keywords = spastic (Clic here for more details about this article) |
9/72. Self-inflicted tourniquet paralysis mimicking acute demyelinating polyneuropathy.Tourniquet paralysis is an uncommon complication of surgery, and self-inflicted tourniquet paralysis has never been documented to our knowledge. We report a patient with bilateral self-induced tourniquet paralysis of the lower extremities, whose symptoms were initially attributed to an acute demyelinating sensorimotor polyneuropathy based on clinical presentation and electrodiagnostic study. After investigations failed to reveal a cause, he was found to have placed tourniquets on his legs because of a rare obsession with limb amputation known as apotemnophilia. Significant spontaneous partial resolution of clinical symptoms was noted after 6 weeks. Electrophysiologic evidence of segmental demyelination of multiple motor nerves localized to the same region may help to distinguish this condition from other forms of acute demyelinating polyneuropathy.- - - - - - - - - - ranking = 0.0017557980860328keywords = paralysis (Clic here for more details about this article) |
10/72. Demyelinating disease in monoclonal gammopathy of undetermined significance.We describe herein the case of a 57 year old man who, over the last five years, has presented ataxic and spastic gait on the right side, a reduction in fine motor movement of the fingers mainly on the right side, superficial right side brachiocrural hypoesthesia and a marked dysarthria associated with internuclear ophthalmoplegia. The neurological picture, after an initial progressive worsening which lasted some months, remained relatively stable over the years. Repeated magnetic resonance imaging (MRI) of the brain and spinal cord documented the presence of demyelinating plaques spread in the white matter of the periventricular region and the semioval centres, and a right side paramedian plaque at the C4-C5 level, none of which were in the active phase. oligoclonal bands were revealed in the cerebrospinal fluid (CSF). Monoclonal IgM/lambda gammopathy with anti-myelin and anti-nucleo reactivity, found with serum immunofixation, were confirmed several times in successive annual controls, not associated to myeloproliferative pathology. The lack of progression in the clinical picture would seem to contradict the diagnosis of late multiple sclerosis. The presence of antibody activity against the myelin might support the hypothesis of a pathogenetic role of the immunoglobulins at the onset of the demyelinating disease in this patient. However, in the end, there is the possibility of casual association with a poorly functioning immune system connected to age.- - - - - - - - - - ranking = 0.0057413383638679keywords = dysarthria, spastic (Clic here for more details about this article) |
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