Schilder's Disease (myelinoclastic diffuse sclerosis or MDS) is a rare primary demyelinating disorder which has been classified as a variant syndrome within the spectrum of multiple sclerosis.
Features of Schilder's disease:
(1) The patient shows clinical findings associated with increased intracranial pressure that may mimic an intracranial neoplasm (pseudotumor), abscess or other mass lesion.
(2) Imaging studies show a large (at least 3 x 2 cm), sharply demarcated plaque in the subcortical white matter of the cerebral hemisphere. In most cases the lesions are bilateral.
(3) A brain biopsy shows demyelination with a perivascular infiltrate of mononuclear cells (lymphocytes and histiocytes) and microglial proliferation.
(4) Adrenoleukodystrophy and other conditions must be excluded.
(5) Some patients may deteriorate rapidly and die. Some patients may show a positive response to aggressive corticosteroid therapy.
Clincal features may include:
(1) behavioral changes
(2) cognitive deterioration
(3) visual loss
(4) paresis or other motor signs
(5) aphasia
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