Disease Information for Familial Basal ganglion calcification (Fahr's syndrome)

Fahr"s syndrome rare hereditary autosomal dominant neurologic disorder; onset of basal ganglion syndrome progressive begins in 40s to 50s; characterized by slow progession of dementia, seizures, regression mentation, all with typical parkinsonism like syndrome; head xrays or ct confirm in part; cortex is involved and onset as early as childhood/adolescence described;----------------------------------- Fahr"s Disease; Cerebrovascular Ferrocalcinosis;

Idiopathic Basal Ganglia Calcification (IBGC); Nonarteriosclerotic Cerebral Calcifications;SPD Calcinosis

Striopallidodentate Calcinosis; Fahr"s Disease is a rare degenerative neurological disorder characterized by the presence of abnormal calcium deposits (calcifications) and associated cell loss in certain areas of the brain (eg, basal ganglia); The condition is often referred to as idiopathic basal ganglia calcification or IBGC because there is no apparent explanation for such calcification in these brain regions (idiopathic); Associated symptoms include progressive deterioration of cognitive abilities (dementia) and loss of acquired motor skills; As the condition progresses, paralysis may develop that is associated with increased muscle stiffness (rigidity) and restricted movements (spastic paralysis); Additional abnormalities may include relatively slow, involuntary, continual writhing movements (athetosis) or chorea, a related condition characterized by irregular, rapid, jerky movements; In some affected individuals, there may also be gradual deterioration of the nerve fibers that transmit impulses from the retinas to the brain (optic atrophy), a condition associated with partial or near complete visual impairment; Fahr"s Disease is often familial; Familial Fahr"s Disease may be transmitted as an autosomal recessive trait or, in other affected families (kindreds), may have autosomal dominant inheritance; In other instances, the condition appears to occur randomly for unknown reasons (sporadically); Some experts suggest that the condition may sometimes result from an unidentified infection during pregnancy affecting the developing fetus (intrauterine infection);--------[NORD 2005]--------

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