RESUMEN
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.
Asunto(s)
Pueblo Asiatico , Esclerosis Cerebral Difusa de Schilder/complicaciones , Esclerosis Cerebral Difusa de Schilder/patología , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/patología , Adulto , Encéfalo/patología , Esclerosis Cerebral Difusa de Schilder/etnología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Esclerosis Múltiple/etnología , PronósticoRESUMEN
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.
