Acute demyelinating disease. Classification and non-invasive diagnosis.
Five young patients are described with biopsy-proven acute demyelinating disease. Two cases are classified as Schilder's disease, a particular childhood form of multiple sclerosis (MS) with atypical clinical manifestation, normal or atypical CSF-findings and large bilateral lesions in magnetic resonance imaging (MRI). Two further cases presented with a fulminant bout of MS with typical clinical picture and CSF-findings; they are classified as Marburg's disease. The last case was an acute second bout of classical MS. The biopsy seemed to be justified in 4 cases, but unnecessary in the last case. Localized proton magnetic resonance spectroscopy (MRS) performed in one case allowed us to examine the focal cerebral abnormalities directly and non-invasively. The spectra revealed a pattern typical of acute demyelination suggesting potential for a replacement of biopsy in the future.
Poser
S
S
Department of Neurology and Neuropathology, Göttingen University, Germany.
Lüer
W
W
Bruhn
H
H
Frahm
J
J
Brück
Y
Y
Felgenhauer
K
K
D000328:Adult; D001369:Axons / Q000648:ultrastructure; D001706:Biopsy; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000175:diagnosis* / Q000473:pathology; D003711:Demyelinating Diseases / Q000145:classification / Q000175:diagnosis* / Q000473:pathology; D004679:Encephalomyelitis / Q000175:diagnosis / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000473:pathology; D009186:Myelin Sheath / Q000473:pathology; D009460:Neurologic Examination