spinocerebellar ataxia

spinocerebellar ataxia
spinocerebellar ataxia n any of a group of inherited neurodegenerative disorders that are characterized by cerebellar dysfunction manifested esp. by progressive ataxia

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any of a group of hereditary disorders, some of autosomal dominant and others of autosomal recessive inheritance, characterized by progressive degeneration of the cerebellum, brainstem, spinal cord, peripheral nerves, and sometimes other regions of the brain, with neuronal loss from affected areas and secondary degeneration of white matter tracts. The autosomal dominant disorders are triplet repeat disorders; in most, expansion of a CAG triplet repeat leads to large polyglutamine tracts in the affected protein, although the disorders differ in many other details, such as the gene affected and the threshold for clinical disease.

Medical dictionary. 2011.

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  • Spinocerebellar ataxia — (SCA) is a genetic disease with multiple types, each of which could be considered a disease in its own right. ymptomsSpinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait… …   Wikipedia

  • Spinocerebellar ataxia type-13 — (SCA13) is a rare autosomal dominant disorder, which, like other types of SCA, is characterized by dysarthria, nystagmus, and ataxia of gait, stance and the limbs due to cerebellar dysfunction. Patients with SCA13 also tend to present with… …   Wikipedia

  • Spinocerebellar Ataxia Type-3 — Spinocerebellar Ataxia Type 3, also known as Machado Joseph Disease (MJD), is an extremely rare hereditary ataxia, which means a lack of muscle control. This is caused by degeneration of cells in the hindbrain. [Machado Joseph Disease Fact Sheet …   Wikipedia

  • Spinocerebellar ataxia type-6 — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 12339 ICD10 = ICD10|G|11|2|g|10 ICD9 = ICD9|334.9 ICDO = OMIM = 183086 MedlinePlus = eMedicineSubj = neuro eMedicineTopic = 556 Spinocerebellar ataxia type 6 (SCA6) is a rare, late onset,… …   Wikipedia

  • Ataxia (disambiguation) — Ataxia can mean: *Ataxia, is unsteady and clumsy motion of the limbs or trunk due to a failure of the gross coordination of muscle movements. This includes cerebellar , sensory , and vestibular ataxia. The word ataxia is also part of the name of… …   Wikipedia

  • Ataxia — For other uses, see Ataxia (disambiguation). Ataxia (from Greek α [used as a negative prefix] + τάξις [order], meaning lack of order ) is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a …   Wikipedia

  • Ataxia — Wobbliness. Ataxia is incoordination and unsteadiness due to the brain’s failure to regulate the body’s posture and regulate the strength and direction of limb movements. Ataxia is usually a consequence of disease in the brain,… …   Medical dictionary

  • ataxia — noun inability to coordinate voluntary muscle movements; unsteady movements and staggering gait • Syn: ↑ataxy, ↑dyssynergia, ↑motor ataxia • Derivationally related forms: ↑ataxic, ↑atactic • Hypernyms: ↑ …   Useful english dictionary

  • spinocerebellar disorder — noun any of several congenital disorders marked by degeneration of the cerebellum and spinal cord resulting in spasticity and ataxia • Hypernyms: ↑birth defect, ↑congenital anomaly, ↑congenital defect, ↑congenital disorder, ↑congenital… …   Useful english dictionary

  • spinocerebellar degeneration — any of a group of inherited disorders of the cerebellum and corticospinal tracts in the brain. They are characterized by spasticity of the limbs and cerebellar ataxia …   Medical dictionary

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