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... > Nervous System Diseases > Central Nervous System Diseases > Brain Diseases > Cerebellar Diseases > Spinocerebellar Degenerations > Myoclonic Cerebellar Dyssynergia
Nervous System Diseases > Central Nervous System Diseases > Spinal Cord Diseases > Spinocerebellar Degenerations > Myoclonic Cerebellar Dyssynergia
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Heredodegenerative Disorders, Nervous System > Spinocerebellar Degenerations > Myoclonic Cerebellar Dyssynergia
Nervous System Diseases > Neurodegenerative Diseases > Heredodegenerative Disorders, Nervous System > Spinocerebellar Degenerations > Myoclonic Cerebellar Dyssynergia

Terme préférentiel

Myoclonic Cerebellar Dyssynergia  

Type

  • mesh:Descriptor

Définition

  • A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal SEIZURES, spasticity, and DYSKINESIAS. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (From Joynt, Clinical Neurology, 1991, Ch37, pp60-1)

Concept générique

Synonyme(s)

  • Cerebellar Dyssynergia
  • Dentate Cerebellar Ataxia
  • Dentate Cerebellar Atrophy
  • Dentate Nucleus Syndrome, Ramsay Hunt
  • Dyssynergia Cerebellaris Myoclonica
  • Dyssynergia Cerebellaris Progressiva
  • Ramsay Hunt Cerebellar Syndrome
  • Ramsay Hunt Dentate Syndrome

Qualificatif(s) autorisé(s)

Traductions

URI

http://data.loterre.fr/ark:/67375/JVR-S38P0XRH-K

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RDF/XML TURTLE JSON-LD Date de création 01/01/1999, dernière modification le 03/07/2012