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Disease Diffuse Cerebral Sclerosis of Schilder

Name Diffuse Cerebral Sclerosis of Schilder
Synonyms Alper Disease | Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis | Alper's Disease | Alpers Disease | Alpers' Disease | Alpers Huttenlocher Syndrome | Alpers-Huttenlocher Syndrome | Alpers Progressive Infantile Poliodystrophy | Alper's Syndrome | Alpers Syndrome | Alpers' Syndrome | Alper 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
Definition 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)
Categories Immune system disease | Nervous system disease
MeSH® ID D002549
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Top ↑ Ancestors

1. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Autoimmune Diseases of the Nervous System Has associated chemicals Has associated genes Has associated exposure references Demyelinating Autoimmune Diseases, CNS Has associated chemicals Has associated genes Has associated exposure references Diffuse Cerebral Sclerosis of Schilder Has associated chemicals Has associated genes
2. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Central Nervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Brain Diseases Has associated chemicals Has associated genes Has associated exposure references Diffuse Cerebral Sclerosis of Schilder Has associated chemicals Has associated genes
3. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Central Nervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Brain Diseases Has associated chemicals Has associated genes Has associated exposure references Leukoencephalopathies Has associated chemicals Has associated genes Has associated exposure references Demyelinating Autoimmune Diseases, CNS Has associated chemicals Has associated genes Has associated exposure references Diffuse Cerebral Sclerosis of Schilder Has associated chemicals Has associated genes
4. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Demyelinating Diseases Has associated chemicals Has associated genes Has associated exposure references Demyelinating Autoimmune Diseases, CNS Has associated chemicals Has associated genes Has associated exposure references Diffuse Cerebral Sclerosis of Schilder Has associated chemicals Has associated genes
5. DiseasesImmune System Diseases Has associated chemicals Has associated genes Has associated exposure references Autoimmune Diseases Has associated chemicals Has associated genes Has associated exposure references Autoimmune Diseases of the Nervous System Has associated chemicals Has associated genes Has associated exposure references Demyelinating Autoimmune Diseases, CNS Has associated chemicals Has associated genes Has associated exposure references Diffuse Cerebral Sclerosis of Schilder Has associated chemicals Has associated genes

Top ↑ Descendants

Diffuse Cerebral Sclerosis of Schilder Has associated chemicals Has associated genes
  MITOCHONDRIAL DNA DEPLETION SYNDROME 4A (ALPERS TYPE) Has associated chemicals Has associated genes
  Pelizaeus-Merzbacher like brain sclerosis
  Pelizaeus-Merzbacher-like disease, autosomal recessive, 2