A hereditary disease resembling LOCOMOTOR ATAXIA, and due to degenerative changes in nerve tracts and nerve cells of the spinal cord and the brain. It occurs usually in children, or at any rate before the 20th year of life, and affects often several brothers and sisters. Its chief symptoms are unsteadiness of gait, with loss of the knee jerks, followed later by diffculties of speech, tremors of the hands, head and eyes, deformity of the feet, and curvature of the spine. There is often associated heart disease. The sufferer gets gradually worse, but may live, with increasing disability, for 20–30 years.
Loss of coordination, though the power necessary to make the movements is still present. Thus an ataxic person may have a good grip in each hand but be unable to do any ?ne movements with the ?ngers; or, if the ataxia be in the legs, the person throws these about a great deal in walking while still being able to lift the legs and take steps quite well. This is due to a sensory defect or to disease of the cerebellum. (See FRIEDREICH’S ATAXIA; LOCOMOTOR ATAXIA.)... ataxia
Uncoordinated movements, including an unsteady gait, caused by damage to or disease of the cerebellum (see BRAIN). Brain tumours, MULTIPLE SCLEROSIS (MS) and stroke can result in ataxia – as can excessive consumption of alcohol, and degeneration of the cerebellum as a result of an inherited disease. A?ected victims may have slurred speech, hand tremors and nystagmus (see under EYE, DISORDERS OF).... cerebellar ataxia
A very rare inherited disease in which degeneration of nerve fibres in the spinal cord causes loss of coordinated movement and balance. Once symptoms have developed, the disease becomes progressively more severe. Treatment can help with the symptoms but cannot alter the course of the disease.... friedreich’s ataxia
an inherited (autosomal *recessive) neurological disorder. *Ataxia is usually noted early in life, and a key feature is the presence of dilated blood vessels visible in the sclerae of the eyes and on the cheeks and ears. Other symptoms may include slow slurred speech, abnormal eye movements, skin lesions, and immune deficiency. Affected individuals may develop malignant disease. A raised level of *alpha-fetoprotein is found in the blood.... ataxia telangiectasia
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