Acute demyelinating disease. Classification and non-invasive diagnosis

Acta Neurol Scand. 1992 Dec;86(6):579-85. doi: 10.1111/j.1600-0404.1992.tb05490.x.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Axons / ultrastructure
  • Biopsy
  • Brain / pathology
  • Brain Diseases / diagnosis*
  • Brain Diseases / pathology
  • Demyelinating Diseases / classification
  • Demyelinating Diseases / diagnosis*
  • Demyelinating Diseases / pathology
  • Encephalomyelitis / diagnosis
  • Encephalomyelitis / pathology
  • Female
  • Humans
  • Male
  • Multiple Sclerosis / diagnosis
  • Multiple Sclerosis / pathology
  • Myelin Sheath / pathology
  • Neurologic Examination