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1.
Mult Scler ; 14(3): 418-24, 2008 Apr.
Article in English | MEDLINE | ID: mdl-18208888

ABSTRACT

Baló's concentric sclerosis (BCS) is a rare demyelinating disorder usually considered a variant of multiple sclerosis (MS). However, its pathogenesis and its correlation with MS remains unclear and controversial. This report presents seven Hans Chinese subjects diagnosed as BCS on the basis of the pathognomonic MR (magnetic resonance) findings. Upon diagnosis, all the cases displayed good responses to corticosteroids and showed an overall benign prognosis during a follow-up period of 4-13.5 years, although three relapsed later. MR findings suggest that the characteristic concentric lesions of BCS frequently (5/7) coexist with multiple sclerosis-like lesions. During follow-up, the Baló-like lesions may either dissolve over time or transform into an MS-like lesion. Moreover, the Balóand MS-like lesions occurred one after another at the onset and relapse phases of the same patient in two cases. These clinical features suggest that Baló's disease showing benign clinical course and co-existence of multiple sclerosis (MS)-like lesion is not rare among the Chinese, and strengthens the notion that BCS correlates intrinsically with MS.


Subject(s)
Asian People , Diffuse Cerebral Sclerosis of Schilder/complications , Diffuse Cerebral Sclerosis of Schilder/pathology , Multiple Sclerosis/complications , Multiple Sclerosis/pathology , Adult , Brain/pathology , Diffuse Cerebral Sclerosis of Schilder/ethnology , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Multiple Sclerosis/ethnology , Prognosis
2.
J Neurol Neurosurg Psychiatry ; 54(3): 226-9, 1991 Mar.
Article in English | MEDLINE | ID: mdl-2030350

ABSTRACT

Occidental type cerebromuscular dystrophy (OCMD) forms a substantial distinct group within congenital muscular dystrophy (CMD). These patients invariably present with amyotrophy, multiple joint contractures, facial muscle involvement, normal or nearly normal intelligence, leukodystrophic appearance on CT scan, and dystrophic changes in muscle.


Subject(s)
Chromosome Aberrations/genetics , Diffuse Cerebral Sclerosis of Schilder/genetics , Genes, Recessive/genetics , Muscular Dystrophies/genetics , Biopsy , Brain/pathology , Chromosome Aberrations/diagnosis , Chromosome Aberrations/ethnology , Chromosome Aberrations/pathology , Chromosome Disorders , Diffuse Cerebral Sclerosis of Schilder/diagnosis , Diffuse Cerebral Sclerosis of Schilder/ethnology , Diffuse Cerebral Sclerosis of Schilder/pathology , Female , Humans , Infant , Male , Muscles/pathology , Muscular Dystrophies/diagnosis , Muscular Dystrophies/ethnology , Muscular Dystrophies/pathology , Tomography, X-Ray Computed , Turkey
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