Schilder type multiple sclerosis

Schilder disease, (also known as diffuse myelinoclastic sclerosis or diffuse cerebral sclerosis) is considered a variant of multiple sclerosis (MS), and represents an extremely rare progressive demyelinating process that begins in childhood 1,4.

The disease is extremely rare with less than 10 cases reported in the literature that fulfill the original Posner diagnostic criteria 1.

Symptoms include aphasia, balance instability, dementia, headache, incontinence, personality changes, seizures, tremors, visual disturbances and weakness 1.

Proposed by Posner in 1985, all six criteria must be met 2

  • 1-2 large plaques: should be in each brain hemisphere predominantly in centrum semiovale. Lesions are greater than 2 cm in 2/3 dimensions 3
  • no additional lesions identified
  • no peripheral nervous system abnormality
  • normal adrenal function
  • normal serum long chain fatty acids 
  • pathologic/histologic changes consistent with subacute/chronic myelinoclastic diffuse sclerosis 

Schilder type multiple sclerosis has 1-2 large demyelinating plaques, which are hyperintense on T2 weighted images, in each cerebral hemisphere, predominantly in the centrum semiovale. Lesions typically are greater than 2cm in diameter 3.

Note that the diagnosis cannot be made on imaging findings alone.

Patients have a poor prognosis. Current recommendations are high dose corticosteroids and supportive care 3.

Shilder disease is named after Paul Ferdinand Schilder (1886-1940), the Austrian psychiatrist and researcher who first described the disease in 1912.

Appearances, clinical presentation and histological findings are similar to tumefactive demyelinating lesions, and it has been proposed that in fact Schilder type multiple sclerosis actually merely represents the paediatric version of tumefactive demyelinating lesions 4.

Appearances are also similar to Marburg variant of multiple sclerosis, although the brain stem is usually spared, and response to steroids is more common 4

 

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Article information

rID: 12275
Synonyms or Alternate Spellings:
  • Schilder type (MS variant)
  • Multiple sclerosis Schilder type
  • Multiple sclerosis Schilder variant
  • Diffuse cerebral sclerosis
  • Diffuse myelinoclastic sclerosis

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