Diseases

Diffuse Cerebral Sclerosis of Schilder

diffuse cerebral sclerosis of schilder

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Description: NCI2016_02D:A rare congenital demyelinating disorder affecting the central nervous system. It is characterized by a demyelinating destructive lesion affecting an entire brain lobe or hemisphere. Signs and symptoms include dementia, cortical deafness and blindness, pseudobulbar palsy, and hemiplegia.|MSH2017_2016_08_12: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)

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