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Diffuse Cerebral Sclerosis of Schilder (Alpers' Disease)

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 Progressive Infantile Poliodystrophy; Alpers' Syndrome; Alpers-Huttenlocher Syndrome; Encephalitis Periaxialis Concentrica; Encephalitis Periaxialis Diffusa; Progressive Neuronal Degeneration of Childhood with Liver Disease; Progressive Sclerosing Poliodystrophy; 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
Networked: 96 relevant articles (2 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Status Epilepticus (Complex Partial Status Epilepticus)
2. Mitochondrial Diseases (Mitochondrial Disease)
3. Leigh Disease (Leigh's Disease)
4. Multiple Sclerosis
5. Neoplasms (Cancer)

Experts

1. Naviaux, Robert K: 6 articles (03/2013 - 05/2004)
2. Copeland, William C: 5 articles (03/2013 - 12/2005)
3. Zeviani, Massimo: 4 articles (08/2008 - 06/2007)
4. Poulton, Joanna: 3 articles (06/2013 - 06/2007)
5. Horvath, Rita: 3 articles (12/2012 - 05/2009)
6. Chinnery, Patrick F: 3 articles (12/2012 - 05/2009)
7. Rahman, Shamima: 3 articles (05/2012 - 02/2009)
8. Wong, Lee-Jun C: 3 articles (02/2012 - 04/2008)
9. Suomalainen, Anu: 3 articles (11/2011 - 06/2007)
10. Longley, Matthew J: 3 articles (08/2010 - 12/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Diffuse Cerebral Sclerosis of Schilder:
1. Mitochondrial DNA (mtDNA)IBA
2. Pyruvate CarboxylaseIBA
3. Formaldehyde (Formol)FDA Link
4. tebufenozide (Mimic)IBA
5. Valproic Acid (Valproate, Semisodium)FDA LinkGeneric
6. Protons (Proton)IBA
7. Lactic AcidFDA LinkGeneric
8. CholesterolIBA
9. Mitochondrial encephalopathyIBA
10. DNA polymerase gammaIBA

Therapies and Procedures

1. Hemispherectomy
2. Ketogenic Diet
3. Plasmapheresis
4. Liver Transplantation