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Schilder's Disease

Medline NLM definition: A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73). Year introduced: 2000(1963)

Shilder's Disease:  Patient UK

Schilder's Disease information page:  National Institute of Neurological Disorders and Stroke (US site)

PubMed search on Diffuse Cerebral Sclerosis of Schilder

(Schilders Disease is not a MeSH term, but it is associated with the MeSH term Diffuse Cerebral Sclerosis of Schilder)

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