Schilder's myelinoclastic diffuse sclerosis.
We report here a case of Schilder's myelinoclastic diffuse sclerosis in a 14-year-old girl with sudden bilateral visual loss. Computed tomographic scan showed two large symmetrical lesions in the occipital lobes and a smaller hypodense area in the frontal lobe. Cerebrospinal fluid examination revealed increased immunoglobulin G fraction with the presence of oligoclonal bands. Ultrastructural study of a biopsy specimen disclosed a demyelinating disorder with no cytoplasmic inclusions. Steroid treatment was followed by a dramatic response, with almost complete visual recovery and shrinkage of the lesions.
Lana-Peixoto
M A
MA
Department of Neurology, Federal University of Minas Gerais Medical School, Belo Horizonte, Brazil.
dos Santos
E C
EC
D000293:Adolescent; D003907:Dexamethasone / Q000627:therapeutic use*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D009778:Occipital Lobe / Q000000981:diagnostic imaging* / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D014786:Vision Disorders / Q000209:etiology* / Q000503:physiopathology
D003907:Dexamethasone