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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)

Year introduced: 2000(1963)

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Tree Number(s): C10.114.375.112, C10.228.140.400, C10.228.140.695.562.112, C10.314.350.112, C20.

MeSH Unique ID: D002549

Entry Terms:

  • Encephalitis Periaxialis
  • Encephalitis Periaxialis Concentrica
  • Sudanophilic Cerebral Sclerosis
  • Myelinoclastic Diffuse Sclerosis
  • Diffuse Scleroses, Myelinoclastic
  • Diffuse Sclerosis, Myelinoclastic
  • Myelinoclastic Diffuse Scleroses
  • Scleroses, Myelinoclastic Diffuse
  • Sclerosis, Myelinoclastic Diffuse
  • Poliodystrophia Cerebri
  • Schilder Disease
  • Disease, Schilder
  • Schilder's Disease
  • Disease, Schilder's
  • Schilders Disease
  • 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 Disease
  • Alpers Progressive Infantile Poliodystrophy
  • Progressive Sclerosing Poliodystrophy
  • Progressive Sclerosing Poliodystrophies
  • Alpers' Syndrome
  • Alper Syndrome
  • Alper's Syndrome
  • Alpers-Huttenlocher Syndrome
  • Alpers Huttenlocher Syndrome
  • Syndrome, Alpers-Huttenlocher
  • Neuronal Degeneration Of Childhood With Liver Disease, Progressive
  • Progressive Neuronal Degeneration of Childhood with Liver Disease
  • Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
  • Alpers' Disease
  • Alper Disease
  • Alper's Disease
  • Disease, Alpers'

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