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Familial Idiopathic Basal Ganglia Calcification

Abstract

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NORD is very grateful to Dr Joao Ricardo Mendes de Oliveira, MD, PhD, Department of Neuropsychiatry, Federal University of Pernambuco, Brazil, for assistance in the preparation of this report.

Synonyms of Familial Idiopathic Basal Ganglia Calcification

  • cerebrovascular ferrocalcinosis
  • Fahr disease, formerly
  • FIBGC
  • striopallidodentate calcinosis

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Familial idiopathic basal ganglia calcification (FIBGC) is a rare neurological disorder characterized by the presence of abnormal calcium deposits (calcifications) of unknown cause. Associated symptoms include progressive deterioration of cognitive abilities (dementia), loss of contact with reality (psychosis), mood swings 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.

Familial Idiopathic Basal Ganglia Calcification Resources

Organizations:

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