Diffuse Cerebral Sclerosis of Schilder (Alpers' Disease)

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47 relevant articles (0 outcomes, 4 trials/studies) found for this Disease

Description: 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' Syndrome; Encephalitis Periaxialis Concentrica; Encephalitis Periaxialis Diffusa; Alper Disease; Alper Syndrome; Alper's Disease; Alper's 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; Scleroses, Balo's Concentric; Scleroses, Myelinoclastic Diffuse; Sclerosis, Diffuse Cerebral; Sclerosis, Myelinoclastic Diffuse; Syndrome, Alpers; Alpers Syndrome; Cerebral Sclerosis, Diffuse; Encephalitis Periaxialis; Poliodystrophia Cerebri; Schilder Disease

Disease Context: Research Results

Nervous System Diseases: 3406

Demyelinating Diseases: 3164

CNS Demyelinating Autoimmune Diseases

Diffuse Cerebral Sclerosis of Schilder: 47

Central Nervous System Diseases: 929

Brain Diseases: 1117

Diffuse Cerebral Sclerosis of Schilder: 47

Autoimmune Diseases of the Nervous System: 16

CNS Demyelinating Autoimmune Diseases

Diffuse Cerebral Sclerosis of Schilder: 47

Immune System Diseases: 1232

Autoimmune Diseases: 11535

Autoimmune Diseases of the Nervous System: 16

CNS Demyelinating Autoimmune Diseases

Diffuse Cerebral Sclerosis of Schilder: 47

Related Diseases

1.	Multiple Sclerosis
2.	Neoplasms (Cancer)
3.	Progressive Multifocal Leukoencephalopathy
4.	Infection
5.	Edema
40 Diseases and 23 more articles analyzed in the Research Interface, order at left...

Experts

1.	Nguyen, Khue V: 2 articles (09/2005 - 05/2004)
2.	Naviaux, Robert K: 2 articles (09/2005 - 05/2004)
3.	Chiras, J: 1 article (04/2005)
4.	Guillevin, R: 1 article (04/2005)
5.	Tourbah, A: 1 article (04/2005)
6.	Papeix, C: 1 article (04/2005)
7.	Pierrot-Deseilligny, C: 1 article (04/2005)
8.	Bruneteau, G: 1 article (04/2005)
9.	Hansikova, H: 1 article (08/2004)
10.	Blahova, K: 1 article (08/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Diffuse Cerebral Sclerosis of Schilder:

1.	Pyruvate CarboxylaseIBA 01/01/1977 - "The authors describe an anatomical familial case of progressive infantile cerebral poliodystrophy (Alpers disease), in which the study of enzyme kinetics of hepatic pyruvate carboxylase revealed an abnormal graph reflecting a loss of activity of the enzyme with low concentrations of substrate, This is a new feature in the literature on Alpers disease, and possibly indicates one of the pathogenic mechanisms responsible in this disorder which remains mysterious, although its pathology has been clearly described." 06/01/1975 - "Proceedings: Infantile cerebral poliodystrophy (Alper's disease) in an infant with hyperlactacidemia and hepatic pyruvate carboxylase]" Get all the results and reference details, order at left...
2.	Formaldehyde (Formol)IBA 10/01/1987 - "We carried out magnetic resonance imaging (MRI) studies on human brains which had been fixed in formalin solution for over 2 years and had been proven neuropathologically to be cases of multiple sclerosis (MS), progressive multifocal leukoencephalopathy (PML), and Balo's concentric sclerosis (Balo)" Get all the results and reference details, order at left...
3.	tebufenozide (Mimic)IBA 10/01/1994 - "Neuroimaging studies also may mimic brain tumor or abscess; however, the absence of significant edema, the irregular and incomplete ring enhancement, the discrepancy between size of the lesions and the associated mass effect, and the absence of other lesions elsewhere in the brain may help differentiate Schilder's disease from neoplasm, infection, and other demyelinating lesions" Get all the results and reference details, order at left...
4.	Mitochondrial DNA (mtDNA)IBA 08/15/2008 - "Twenty-one had Alpers syndrome, the commonest severe POLG1 autosomal recessive phenotype, comprising hepatoencephalopathy and often mtDNA depletion" 06/01/2008 - "Alpers syndrome is a fatal disorder due to mutations in the POLG gene encoding the catalytic subunit of mitochondrial DNA polymerase gamma (Pol gamma) involved in mitochondrial DNA (mtDNA) replication" 06/01/2008 - "The W748S POLG1 mutation seems to lead to tissue-specific, partial mtDNA depletion in patients with juvenile-onset Alpers syndrome" 04/01/2008 - "Alpers syndrome is a fatal neurogenetic disorder caused by the mutations in POLG1 gene encoding the mitochondrial DNA polymerase gamma (polgamma)" 11/01/2007 - "We show here that recessive mutations of the close functional partner of the polymerase, the Twinkle helicase, can also manifest as early encephalopathy with liver involvement, a phenotype reminiscent of Alpers syndrome, and are a new genetic cause underlying tissue-specific mtDNA depletion." Get all the results and reference details, order at left...
5.	CholesterolIBA 05/01/1976 - "No significant differences in [14C]cholesterol rates of uptake or release were observed between control and Schilder's disease fibroblasts" 03/01/1976 - "Letter: Schilder's disease: cholesterol metabolism in cultured fibroblasts." 03/01/1974 - "Schilder's disease: abnormal cholesterol retention and accumulation in cultivated fibroblasts." Get all the results and reference details, order at left...
6.	Pyruvic Acid (Pyruvate)IBA 09/01/1984 - "We will present 8 children with progressive infantile or juvenile poliodystrophy (Alpers' disease), associated with a defect in pyruvate metabolism" 12/01/1981 - "Progressive infantile poliodystrophy (Alpers' disease) is associated with abnormalities in pyruvate metabolism or in cell mitochondria" Get all the results and reference details, order at left...
7.	Valproic Acid (Valproate, Semisodium)FDA LinkGeneric 05/01/1997 - "We report 2 siblings with Alpers disease who were discordant for exposure to valproate (VPA)" 05/01/1997 - "Valproate-induced liver failure in one of two siblings with Alpers disease." 08/01/2000 - "We suggest that this is a late-onset Huttenlocher variant of Alpers' syndrome, where fulminant liver failure can be triggered by valproic acid, and orthotopic liver transplantation can subsequently trigger a fatal neurologic deterioration" Get all the results and reference details, order at left...
8.	Protons (Proton)IBA 04/01/2005 - "Contribution of proton magnetic resonance spectroscopy to the diagnosis of Balo's concentric sclerosis]" 06/01/1997 - "Balo's concentric sclerosis: a clinical case study of brain MRI, biopsy, and proton magnetic resonance spectroscopic findings." Get all the results and reference details, order at left...
9.	ParaffinIBA 10/01/1994 - "Although frozen sections of these lesions are often interpreted as astrocytoma, the inflammatory, primarily histiocytic, nature of Schilder's disease is more easily recognized in paraffin-embedded material" 01/01/1990 - "Using a monoclonal antibody to an inner mitochondrial membrane antigen and light microscopic immunohistochemistry, we investigated the distribution of increased immunostaining (mitochondrial anomalies, MA) on paraffin sections from 21 brains with infantile spongy dystrophy (Leigh's disease, 8; Canavan's disease, 4; Alpers' syndrome, 2; mixed spongy dystrophy, 7)" Get all the results and reference details, order at left...
10.	Fatty Acids (Saturated Fatty Acids)IBA 02/01/1986 - "Defective peroxisomal beta-oxidation is manifested in Schilder's disease (adrenoleukodystrophy) characterized by accumulation of very long fatty acids" 06/01/1971 - "Fatty acid composition of cholesterol esters in brains of patients with Schilder's disease, G M1 -gangliosidosis and Tay-Sachs disease, and its possible relationship to the -position fatty acids of lecithin." Get all the results and reference details, order at left...
31 Drugs and Important Bio-Agents and 46 more articles analyzed in the Research Interface, order at left...

Therapies and Procedures

1.	Plasmapheresis 01/01/1997 - "Serial magnetic resonance imaging (MRI) study of a patient with Balo's concentric sclerosis treated with immunoadsorption plasmapheresis." 09/01/1984 - "Correct diagnosis of Schilder's disease opens the possibility of tentatives of treatment with immunosuppression, plasmapheresis and lymphocytopheresis." Get all the results and reference details, order at left...
2.	Liver Transplantation 08/01/2000 - "We suggest that this is a late-onset Huttenlocher variant of Alpers' syndrome, where fulminant liver failure can be triggered by valproic acid, and orthotopic liver transplantation can subsequently trigger a fatal neurologic deterioration" Get all the results and reference details, order at left...
2 Therapies and Procedures and 3 more articles analyzed in the Research Interface, order at left...

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