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 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.
- Cerebral cortex
Cerebral cortex: A thin mantle of gray matter about the size of a formal dinner napkin covering the surface of each cerebral hemisphere. The cerebral cortex is crumpled and folded, forming numerous convolutions (gyri) and crevices (sulci). It is made up of six layers of nerve cells and the nerve pathways that connect them. The […]
- Cerebral edema
Cerebral edema: Accumulation of excessive fluid in the substance of the brain. The brain is especially susceptible to injury from edema, because it is located within a confined space and cannot expand. Also known as brain edema, brain swelling, swelling of the brain, and wet brain.
- Cerebral fornix
Cerebral fornix: An arching fibrous band in the brain that connects the two lobes of the cerebrum. There are two such bands, each of which is an arched tract of nerves.
- Cerebral hemispheres
Cerebral hemispheres: The two halves of the cerebrum, the largest part of the brain.
- Cerebral herniation
Cerebral herniation: The abnormal protrusion of brain tissue through an opening when there is increased intracranial pressure (when the brain is under increased pressure). The increased pressure may be due to a number of causes including inflammation of the brain (as in meningitis), a tumor, hemorrhage, and edema (swelling of the brain). The tonsils of […]