Adverse Drug Reaction Classification System

ADR Ontology
ADR Term Alpers' disease
ADR ID BADD_A08657
ADR Hierarchy
03      Congenital, familial and genetic disorders
03.18      Cytoplasmic disorders congenital
03.18.01      Genetic mitochondrial abnormalities NEC
03.18.01.002      Alpers' disease
17      Nervous system disorders
17.19      Congenital and peripartum neurological conditions
17.19.03      Congenital and peripartum cerebral disorders
17.19.03.004      Alpers' 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) [MeSH]
MedDRA Code 10062943
MeSH ID D002549; D002549; D002549
ADR Severity Grade (FAERS)
ADR Severity Grade (CTCAE) Not Available
Synonym
Alpers' disease | Poliodystrophia cerebri | Diffuse Cerebral Sclerosis of Schilder | 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'
ADR Related Proteins
Protein Name UniProt AC TTD Target ID
Not AvailableNot AvailableNot Available
Drugs Leading to the ADR
Drug IDDrug NameADR Frequency (FAERS)ADR Severity Grade (FAERS)
BADD_D02329Valproic acid0.000173%
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