Definition of Cerebral calcification, nonarteriosclerotic
Cerebral calcification, nonarteriosclerotic definition - medical term This syndrome desc ribed
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 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 a significant hormone problem, namely hypoparathyroidism (underactivity of the
parathyroid gland which 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 the 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,
Fahr disease, striopallidodentate calcinosis, SPD calcinosis, and cerebrovascular
ferrocalcinosis.
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