1/107. Connatal pelizaeus-merzbacher disease associated with the jimpy(msd) mice mutation.In a patient with connatal pelizaeus-merzbacher disease with the same mutation in the proteolipid protein gene as in jimpy(msd) mice the immunohistochemical study of the brain demonstrated deficiencies of myelin and proteolipid protein despite good expression of myelin basic protein. The mechanism of myelination is partly disturbed by the mutation; therefore jimpy(msd) mice can be used as a suitable model for further studies in connatal pelizaeus-merzbacher disease.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/107. MRI features of Balo's concentric sclerosis.We report MRI findings in a 56-year-old woman with Balo's concentric sclerosis (BCS) who initially presented with a progressive hemiparesis. MRI showed two lesions with a concentric pattern in the left frontoparietal region and a laminated, arcuate pattern in the right frontal region. These patterns were best seen in post-contrast images and were consistent with BCS. In addition, there were several small cerebral multiple sclerosis-like plaques. The clinical symptoms improved and the MR findings regressed after corticosteroid therapy. The patient had completely recovered 12 months later, except for mild right hand numbness. MRI showed further regression of the lesions, but the concentric pattern was still present. This case demonstrated that BCS can run a benign prolonged course and may persist for a long time. Concentric or laminated contrast enhancement in the acute phase may suggest that bands of demyelination in BCS occur synchronously rather than successively.- - - - - - - - - - ranking = 37241.972802145keywords = sclerosis, multiple sclerosis, ms (Clic here for more details about this article) |
3/107. Balo's concentric sclerosis: value of magnetic resonance imaging in diagnosis.We report two cases of Balo's concentric sclerosis that demonstrate the typical magnetic resonance imaging (MRI) findings of concentric rings of demyelination involving the superficial and deep white matter and sparing the cortex. In both cases biopsy was not performed as MRI findings and multi-mode evoked potential studies were consistent with demyelinating illness. The theories regarding the pathogenesis of this peculiar appearance are briefly reviewed.- - - - - - - - - - ranking = 29659.065644417keywords = sclerosis (Clic here for more details about this article) |
4/107. Evidence that Alpers-Huttenlocher syndrome could be a mitochondrial disease.We report an 11-year-old boy with a slight developmental delay and epilepsy. After he was placed on valproate, he developed hepatic failure and increasing neurologic symptoms, including epilepsia partialis continua, and died. autopsy findings in liver and cerebrum were consistent with progressive neuronal degeneration of childhood with liver disease, also called Alpers-Huttenlocher syndrome. Ragged red fibers and cytochrome c oxidase negative fibers were present in muscle. These results suggest that Alpers-Huttenlocher syndrome, at least in some patients, is a mitochondrial disease.- - - - - - - - - - ranking = 0.16666666666667keywords = ms (Clic here for more details about this article) |
5/107. Recurrent myelinoclastic diffuse sclerosis: a case report of a child with Schilder's variant of multiple sclerosis.Myelinoclastic diffuse sclerosis (MDS, Schilder's disease) is a rare CNS demyelinating disorder affecting mainly children and usually presenting as an intracranial mass lesion. We report the first case of recurrent intracranial MDS where the third episode of demyelination involved the cervical spinal cord. This may represent a subset of the disease, which should be considered as Schilder's variant (childhood form) of multiple sclerosis.- - - - - - - - - - ranking = 67572.768099721keywords = sclerosis, multiple sclerosis (Clic here for more details about this article) |
6/107. Pathognomonic MR imaging findings in Balo concentric sclerosis.Irregular, concentric zones of increased signal on T2-weighted cranial MR imaging studies may strongly suggest Balo concentric sclerosis (BCS), a rare but recognized variant of multiple sclerosis. Differentiating BCS from multiple sclerosis or neoplasm can be difficult clinically, but MR imaging findings noted in this case may be pathognomonic.- - - - - - - - - - ranking = 44824.546626539keywords = sclerosis, multiple sclerosis (Clic here for more details about this article) |
7/107. Fulminant course in a case of diffuse myelinoclastic encephalitis-- a case report.We report on a 10-year old previously healthy boy who exhibited a fulminant and nearly monophasic clinical course of demyelinating encephalitis with relapsing intracranial hypertension syndrome. Histologic examination of a diagnostic brain biopsy revealed an inflammatory demyelinating process with perivascular T lymphocytic infiltration and axonal damage reminiscent of multiple sclerosis-like lesions. In the brain, the dna of human Herpes virus 6 (HHV6) was detectable. Eleven months after the initial symptoms and on maintainance with oral steroids, MRI showed demyelination of both hemispheres as well as demyelination of the brain stem and wallerian degeneration. The boy exhibited a severe neurologic defect syndrome. The clinical and radiological course is unusual because of the asymmetric progression of the encephalitis and the extensive confluent lesions without demarcated border or enhancement of the rim after injection of gadolinium. The clinical course showed no definite steroid response. The pathogenetic relevance of HHV6 remains elusive. Although single patients with HHV6-associated encephalomyelitis have been reported, HHV6 dna is occasionally detected in brains of healthy individuals.- - - - - - - - - - ranking = 7582.9071577275keywords = sclerosis, multiple sclerosis, ms (Clic here for more details about this article) |
8/107. Balo's concentric sclerosis: clinical and radiologic features of five cases.Balo's concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis. Five BCS cases were diagnosed antemortem based on their typical concentric mass patterns on MR images and based on clinical and CSF findings. Histopathologic investigation was also performed in one case. Our case report supports the concept that BCS may be a self-limited disease that is not always fatal. Characteristic MR imaging findings may allow antemortem diagnosis of BCS when performed at the onset of the disease.- - - - - - - - - - ranking = 37241.806135478keywords = sclerosis, multiple sclerosis (Clic here for more details about this article) |
9/107. Rasmussen's syndrome associated with chronic brain stem encephalitis.We report a 3-year-old boy with a clinical picture of Rasmussen's encephalitis who had clinical, radiological and pathological evidence of brain stem involvement resulting in death. In addition, there were unexpected neuropathological findings of severe bilateral mesial temporal sclerosis. We discuss the novel finding of brain stem involvement in this condition and the association with mesial temporal sclerosis.- - - - - - - - - - ranking = 11863.626257767keywords = sclerosis (Clic here for more details about this article) |
10/107. Balo's concentric sclerosis: surviving normal myelin in a patient with a relapsing-remitting dinical course.Balo's concentric sclerosis is a demyelinating disorder in which bands of demyelination alternate with concentric bands of myelin preservation. The pathogenesis of the lesion is unknown. Previous reports using modern histopathologic techniques have shown the bands of myelin preservation to be comprised of remyelinated or partially demyelinated myelin. Here we report a case of Balo's concentric sclerosis in a 24-year-old East Indian patient with a previous history of relapsing-remitting multiple sclerosis (MS). Pathologically, the bands of myelin preservation showed myelin sheaths of normal thickness, with focal areas of demyelination. The findings, taken together with those of previously reported cases, suggest that Balo's concentric sclerosis is a variant of MS, and the concentric lesion may be an intermediary form in evolution of a chronic active MS plaque. The pathogenesis of this concentric lesion may be explained by periodic suppression of demyelination in the rapidly expanding border, allowing remyelination or only transient incomplete demyelination to occur.- - - - - - - - - - ranking = 49105.432393245keywords = sclerosis, multiple sclerosis (Clic here for more details about this article) |
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