Ferrocalcinosis, cerebrovascular


Ferrocalcinosis, cerebrovascular
A condition that was first described in 1930 by T. Fahr and is therefore sometimes called Fahr syndrome, this is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia and the cerebral cortex). Symptoms may include motor function deterioration, dementia, mental retardation, spastic paralysis, dysarthria (poorly articulated speech), spasticity (stiffness of the limbs), ocular (eye) problems, and athetosis (involuntary, writhing movements). Features of Parkinson’s disease such as tremors, rigidity (resistance to imposed movement), a mask-like facial appearance, shuffling gait, and a "pill-rolling" motion of the fingers may also occur in individuals with Fahr’s syndrome. Other symptoms may include dystonia (disordered muscle tone), chorea (involuntary, rapid, jerky movements), and seizures. Onset of the disorder may occur at any time from childhood to adulthood. Fahr syndrome thus involves abnormalities of the neurologic system (cerebral calcification, dementia, spastic paraplegia, athetosis), skull (microcephaly, i.e. an abnormally small head), eyes (glaucoma, optic nerve atrophy, retinitis pigmentosa), and, we would add, a significant hormone problem, namely hypoparathyroidism (the parathyroid gland regulates calcium). The disease is inherited as an autosomal recessive trait in which both parents carry a Fahr gene and each of their children (boys and girls alike) stands a 1 on 4 (25%) risk of receiving both Fahr genes and therefore having this dread disease. There is no cure for Fahr’s syndrome, nor is there a standard course of treatment. Treatment is directed toward minimizing symptoms. The prognosis (outlook) for individuals with Fahr’s syndrome is poor. Progressive neurological deterioration generally results in disability and death. Alternative names for this syndrome include, nonarteriosclerotic cerebral calcification, striopallidodentate calcinosis, and SPD calcinosis.

Medical dictionary. 2011.

Look at other dictionaries:

  • Cerebrovascular ferrocalcinosis — A condition, first described in 1930 by T. Fahr and therefore called Fahr syndrome, that is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia… …   Medical dictionary

  • Síndrome de Fahr — Clasificación y recursos externos OMIM 213600 DiseasesDB 32200 …   Wikipedia Español

  • Calcification, nonarteriosclerotic cerebral — This condition described in 1930 by T. Fahr (and therefore called Fahr syndrome) is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia and the… …   Medical dictionary

  • Cerebral calcification, nonarteriosclerotic — This syndrome described in 1930 by T. Fahr is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia and the cerebral cortex). Symptoms may include… …   Medical dictionary

  • Disease, Fahr — This syndrome described in 1930 by T. Fahr is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia and the cerebral cortex). Symptoms may include… …   Medical dictionary

  • Fahr syndrome — This syndrome described in 1930 by T. Fahr is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia and the cerebral cortex). Symptoms may include… …   Medical dictionary

  • SPD calcinosis — Striopallidodentate (SPD) calcinosis, a condition first described in 1930 by T. Fahr and therefore called Fahr syndrome, is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain …   Medical dictionary

  • Striopallidodentate calcinosis — Striopallidodentate (SPD) calcinosis, a condition first described in 1930 by T. Fahr and therefore called Fahr syndrome, is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain …   Medical dictionary

  • Syndrome, Fahr — This syndrome described in 1930 by T. Fahr is a genetic (inherited) neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain (including the basal ganglia and the cerebral cortex). Symptoms may include… …   Medical dictionary


Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”

We are using cookies for the best presentation of our site. Continuing to use this site, you agree with this.