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Diffuse Cerebral Sclerosis of SchilderLegal notice  


Other terms
Encephalitis Periaxialis,Encephalitis Periaxialis Concentrica,Schilder's Disease,Disease, Schilder's,Schilders Disease,Myelinoclastic Diffuse Sclerosis,Diffuse Scleroses, Myelinoclastic,Diffuse Sclerosis, Myelinoclastic,Myelinoclastic Diffuse Scleroses,Scleroses, Myelinoclastic Diffuse,Sclerosis, Myelinoclastic Diffuse,Poliodystrophia Cerebri,Schilder Disease,Disease, Schilder,Cerebral Sclerosis, Diffuse,Diffuse Cerebral Scleroses,Diffuse Cerebral Sclerosis,Sclerosis, Diffuse Cerebral,Encephalitis Periaxialis Diffusa,Balo Concentric Sclerosis,Concentric Sclerosis, Balo,Balo's Concentric Sclerosis,Concentric Sclerosis, Balo's,Scleroses, Balo's Concentric,Alpers Syndrome,Syndrome, Alpers,Alpers-Huttenlocher Syndrome,Alpers Huttenlocher Syndrome,Syndrome, Alpers-Huttenlocher,Alpers' Syndrome,Alper Syndrome,Alper's Syndrome,Alpers Progressive Infantile Poliodystrophy,Alpers' Disease,Alper Disease,Alper's Disease,Disease, Alpers'

Description
Diffuse Cerebral Sclerosis of Schilder: 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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