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hereditary ataxia

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Hereditary ataxia
One of a group of hereditary degenerative diseases of the spinal cord or cerebellum. These diseases cause tremor, spasm, and wasting of muscle.
Mentioned in: Movement Disorders

hereditary ataxia,
one of a group of inherited degenerative diseases of the spinal cord, cerebellum, and often other parts of the nervous system, characterized by tremor, spasm, muscle wasting, skeletal change, and sensory disturbances resulting in impaired motor activity. Kinds of hereditary ataxia include ataxia-telangiectasia syndrome and Friedreich's ataxia.

ataxia [ah-tak´se-ah]
failure of muscular coordination; irregularity of muscular action. adj., adj atac´tic, atax´ic.
cerebellar ataxia ataxia due to disease of the cerebellum.
Friedreich's ataxia see friedreich's ataxia.
frontal ataxia disturbance of equilibrium associated with tumor of the frontal lobe.
hereditary ataxia Friedreich's ataxia.
hysterical ataxia ataxia recognizable as a conversion symptom; see also astasia-abasia.
locomotor ataxia tabes dorsalis.
sensory ataxia ataxia due to loss of proprioception (joint position sense), resulting in poorly judged movements and becoming aggravated when the eyes are closed.
ataxia-telangiectasia a severe, autosomal recessive, progressive ataxia, associated with telangiectasias (dilation of small blood vessels) in the skin and eyes; immunodeficiency with frequent infections of the respiratory tract from sinuses to lungs; and abnormal eye movements. Called also Louis-Bar's syndrome.

ataxia
failure of muscular coordination; irregularity of muscular action.

cerebellar ataxia
ataxia characterized by defects in rate, range, force and direction of movement of limbs. There is a broad based stance, inability to maintain the head in the proper position so that it oscillates, there is hypermetria or hypometria, direction cannot be maintained and the animal falls easily, often in an exaggerated way.
congenital ataxia
cerebellar ataxia due to viral infection of the fetus, e.g. bovine virus diarrhea and feline panleukopenia, or to inheritance, e.g. in cattle. See also cerebellar atrophy.
copper-related ataxia
see copper nutritional deficiency.
enzootic ataxia
see enzootic ataxia.
equine sensory ataxia
see enzootic equine incoordination.
familial convulsions and ataxia of cattle
see familial convulsions and ataxia of cattle.
feline ataxia
see feline panleukopenia.
foal ataxia
see enzootic equine incoordination.
frontal ataxia
disturbance of equilibrium occurring in cases of tumor of the frontal lobe.
hereditary ataxia
see hereditary ataxia.
hound ataxia
a degenerative myelopathy of Foxhounds, Harrier hounds and Beagles. Affected dogs show increasing hindleg incoordination. A dietary cause is suspected.
locomotor ataxia
tabes dorsalis.
otarid ataxia
a syndrome in pinnipeds caused by nutritional deficiency of thiamin or enterotoxemia or hypoglycemia. Signs include heelwalking (elevation and curling of the rear toes) followed by running staggers, ataxia and violent falling.
progressive ataxia
an inherited disease of cattle in which hind limb ataxia commences at 6 months to 3 years of age and worsens over 1 to 2 more years to the point of recumbency. It is a myelin disorder with eosinophilic plaques in the cerebellar medula and peduncles. Called also progressive sensory ataxia of Charolais cattle.
sensorimotor ataxia
caused by moderate spinal cord lesions, manifested by weakness of movement, scuffing of toes, incomplete limb extension, knuckling, wobbly gait, easy falling, difficult rising.
sensory ataxia
ataxia due to loss of proprioception (joint position sense), resulting in poorly judged movements and becoming aggravated when the eyes are blindfolded.
spinal ataxia
see equine protozoal myeloencephalitis.
vestibular ataxia
a loss of balance with preservation of strength. If unilateral, the abnormality is asymmetrical; if bilateral, it is symmetrical.

hereditary
transmissible or transmitted from parent to offspring; genetically determined.

hereditary ataxia
occurs in smooth-haired Fox terriers and Jack Russell terriers and is progressive over a long period, commencing at 2 to 4 months of age; characterized by symmetrical demyelination within the spinal cord.
hereditary collagen dysplasia
a group of diseases which occur in most species and are characterized by looseness and stretchability of the skin, often accompanied by laxity of the joints and occasionally by absence of the tooth enamel. The inherited defect is one of deficient collagen synthesis. See also dermatosparaxis, ehlers-danlos syndrome. Called also hyperelastosis cutis, rubber-puppy syndrome.
hereditary defect
anatomical or functional defect conditioned in its appearance by inherited factors.
hereditary melanoma
benign, heritable, cutaneous tumor of pigs.
hereditary thrombopathias
an inherited bleeding tendency in which the platelets do not function properly. Recorded in cattle and dogs.


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Carl's association with NAF started when his wife was diagnosed with an unknown form of hereditary ataxia and became involved with a clinical study at NIH.
Vlaamse Liga voor Ataxie van Friedreich en andere ertelijke ataxiein European Federation of Hereditary Ataxias (Euro-Ataxia) Kleine Kuipersstraat 27 Brugge B-8000, Belgium 32 50 333043 ?
The two regularly contributed original poems and personal stories related to their hereditary ataxia for publication in Generations.
 
 
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