Diffuse Cerebral Sclerosis of Schilder

Synonyms

Alper Disease

Alper Syndrome

Alper's Disease

Alper's Syndrome

Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis

Alpers Disease

Alpers Huttenlocher Syndrome

Alpers Progressive Infantile Poliodystrophy

Alpers Syndrome

Alpers' Disease

Alpers' Syndrome

Alpers-Huttenlocher Syndrome

Balo Concentric Sclerosis

Balo's Concentric Sclerosis

Cerebral Sclerosis, Diffuse

Concentric Sclerosis, Balo

Concentric Sclerosis, Balo's

Diffuse Cerebral Scleroses

Diffuse Cerebral Sclerosis

Diffuse Scleroses, Myelinoclastic

Diffuse Sclerosis, Myelinoclastic

Disease, Alpers'

Disease, Schilder

Disease, Schilder's

Encephalitis Periaxialis

Encephalitis Periaxialis Concentrica

Encephalitis Periaxialis Diffusa

Myelinoclastic Diffuse Scleroses

Myelinoclastic Diffuse Sclerosis

Neuronal Degeneration Of Childhood With Liver Disease, Progressive

Poliodystrophia Cerebri

Progressive Neuronal Degeneration of Childhood with Liver Disease

Progressive Sclerosing Poliodystrophies

Progressive Sclerosing Poliodystrophy

Schilder Disease

Schilder's Disease

Schilders Disease

Scleroses, Balo's Concentric

Scleroses, Myelinoclastic Diffuse

Sclerosis, Diffuse Cerebral

Sclerosis, Myelinoclastic Diffuse

Sudanophilic Cerebral Sclerosis

Syndrome, Alpers

Syndrome, Alpers-Huttenlocher

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)