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ataxia
(redirected from congenital ataxia)

   Also found in: Dictionary/thesaurus, Encyclopedia, Wikipedia 0.01 sec.
ataxia /atax·ia/ (ah-tak´se-ah) failure of muscular coordination; irregularity of muscular action.atac´ticatax´ic
Bruns' frontal ataxia  a disturbance of equilibrium and gait due to a lesion in the frontal lobe, characterized by assumption of a broad-based gait with the feet flat on the ground and a tendency to retropulsion.
Friedreich's ataxia  hereditary sclerosis of the dorsal and lateral columns of the spine, usually beginning in childhood or youth; it is attended with ataxia, speech impairment, scoliosis, peculiar movements, paralysis, and often hypertrophic cardiomyopathy.
locomotor ataxia  tabes dorsalis.
motor ataxia  inability to control the coordinate movements of the muscles.
sensory ataxia  ataxia due to loss of proprioception (joint position sensation) between the motor cortex and peripheral nerves, resulting in poorly judged movements, the incoordination becoming aggravated when the eyes are closed.
ataxia-telangiectasia  a severe autosomal recessive progressive cerebellar ataxia, associated with oculocutaneous telangiectasia, abnormal eye movements, sinopulmonary disease, and immunodeficiency.

a·tax·i·a (-tks-) or a·tax·y (-tks)
n.
Loss of the ability to coordinate muscular movement. Also called dyssynergia, incoordination.

Ataxia
The inability to control voluntary muscle movement, most frequently resulting from disorders in the brain or spinal cord.

ataxia
[ətak′sē·ə]
Etymology: Gk, without order
an impaired ability to coordinate movement, often characterized by a staggering gait and postural imbalance. It can have many causes, including lesions in the spinal cord or cerebellum that may be the sequelae of birth trauma, congenital disorder, infection, degenerative disorder, neoplasm, toxic substance stroke, or head injury. See also hereditary ataxia. ataxial, ataxic, adj.

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 (·taksˑ·ē·),
n postural imbalance and a staggering ambulatory style.

ataxia (ātak´sē),
n a muscular incoordination characterized by irregular muscle activity.
ataxia, locomotor,

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.

ataxia 
An inability to coordinate muscular activity during voluntary movements.

ataxia
Neurology Muscle incoordination and gait unsteadiness due to cerebellar dysfunction, and compromise in regulating limb movement. See Cerebellar gait, Friedreich's ataxia, Hereditary cerebellar ataxia, Spinocerebellar ataxia Vox populi Wobbling.

Patient discussion about ataxia.

Q. Can I inherit ataxia to my children? I have Ataxia. Does this mean that all my kids will have it too?

A. The hereditary ataxias are genetic, which means they are caused by a defect in a certain gene that is present from the start of a person's life. There are both dominant and recessive ataxias. If it's a dominant ataxia then each child of a parent with an autosomal dominant ataxia gene has a 50/50 chance of whether they will inherit the ataxia gene or not. If it's recessive then it takes a "double dose" of the ataxia gene to result in disease symptoms. Both parents must be carriers of the disease gene in order for it to pass on. Each child of parents who are both carriers of a recessive disease has a 25% chance of inheriting two ataxia genes so will develop the disease, a 50% chance of inheriting just one of the ataxia genes and, therefore, be a carrier and a 25% chance of inheriting no ataxia gene and be completely free of ataxia.
Therefore, it depends which ataxia gene you have, if it's dominant or recessive and whether your spouse is a carrier too.

Read more or ask a question about ataxia


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By this approach we may identify a novel patient with a genomic rearrangement in 8q, confirming that the gene we found interrupted is responsible for cerebellar hypoplasia and MR, or more probably identify new regions important in the pathogenesis of congenital ataxias.
There are also so-called congenital ataxias, that is, children are born with abnormalities that become apparent later.
In this project, we proposed to generate a mouse model for a gene that was found to be mutated in some forms of congenital ataxia, the type of ataxia that is seen in newborns and associated with a disorder in development of the cerebellum.
 
 
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