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)

Also Known As:

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

Nervous System Diseases: 14737
- Demyelinating Diseases: 9340
  - CNS Demyelinating Autoimmune Diseases
    - Diffuse Cerebral Sclerosis of Schilder: 121
- Central Nervous System Diseases: 4371
  - Brain Diseases: 16141
    - Leukoencephalopathies: 1594
      - CNS Demyelinating Autoimmune Diseases
        Diffuse Cerebral Sclerosis of Schilder: 121
    - Diffuse Cerebral Sclerosis of Schilder: 121
- Autoimmune Diseases of the Nervous System: 1576
  - CNS Demyelinating Autoimmune Diseases
    - Diffuse Cerebral Sclerosis of Schilder: 121
Immune System Diseases: 4430
- Autoimmune Diseases: 35458
  - Autoimmune Diseases of the Nervous System: 1576
    - CNS Demyelinating Autoimmune Diseases
      - Diffuse Cerebral Sclerosis of Schilder: 121

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Disease

Drug/Important Bio-Agent (IBA)