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Schilder's disease

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A rare, progressive and invariably fatal disease of the central nervous system characterized by adrenal atrophy and diffuse cerebral demyelination. The major clinical features are progressive dementia, spasticity, cortical blindness, deafness, hemiplegia, quadruplegia, and pseudobulbar palsy. Onset in late childhood. Most patiens die within a few months after onset.

Recent advances in neurochemistry has made it apparent that Schilder disease belongs to a heterogenous group of disorders which includes Krabbe disease, sudanophilic cerebral sclerosis, metachromatic leukodystrophy and adrenoleukodystrophy. Schilder's disease does not exist as a distinct entity and it can be foreseen that the eponym will eventually be relegated to oblivion.

In 1925 Flatau described Schilder's disease in detail and proposed the name encephaloleukopathia scleroticans progressiva instead of encephalitis periaxialis diffusa.


  • P. F. Schilder:
    Zur Kenntnis der sogenannten diffusen Sklerose (über Encephalitis periaxialis diffusa).
    Zeitschrift für die gesamte Neurologie und Psychiatrie, 1912, 10 Orig.: 1-60. Zur Frage der Encephalitis periaxialis diffusa (sogenannte diffuse Sklerose).
    Zeitschrift für die gesamte Neurologie und Psychiatrie, 1913, 15: 359.
  • E. Flatau:
    Encephaloleucopathia scleroticans progressiva. Neurologia Polska, L’Encephale (Journal de neurologie et de psychiatre), 7, 1925, 475-499.

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