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Alper's disease is a progressive disease of the nervous system characterized by spasticity, myoclonus and dementia and by jaundice and cirrhosis. This disorder, first described by Alpers in 1931 as "Diffuse progressive degeneration of gray matter of cerebrum," usually begins early in life with convulsions. A continuous seizure (status epilepticus) is often the final event. Alpers diffuse degeneration of gray matter with hepatic cirrhosis is due to more than one cause. Some cases are inherited as autosomal recessive traits with both parents appearing normal but carrying one Alpers gene and each of their children, boys and girls alike, running a 1 in 4 risk of receiving both of the parental Alpers genes and suffering from this disease. Other cases of Alpers disease are due to disorders of oxidative phosphorylation, including mitochondrial DNA depletion syndromes.