Concept information
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Genetic Diseases, Inborn
Heredodegenerative Disorders, Nervous System
Spinocerebellar Degenerations
Nervous System Diseases
Neurodegenerative Diseases
Heredodegenerative Disorders, Nervous System
Spinocerebellar Degenerations
Preferred term
Myoclonic Cerebellar Dyssynergia
Type
-
Topical Descriptor
Broader concept
Entry terms
- Ataxia, Dentate Cerebellar
- Ataxias, Dentate Cerebellar
- Atrophies, Dentate Cerebellar
- Atrophy, Dentate Cerebellar
- Cerebellar Ataxias, Dentate
- Cerebellar Atrophy, Dentate
- Cerebellar Dyssynergia
- Cerebellar Dyssynergias
- Dentate Cerebellar Ataxia
- Dentate Cerebellar Ataxias
- Dentate Cerebellar Atrophies
- Dentate Cerebellar Atrophy
- Dentate Nucleus Syndrome, Ramsay Hunt
- Dyssynergia, Cerebellar
- Dyssynergia Cerebellaris Myoclonica
- Dyssynergia Cerebellaris Progressiva
- Dyssynergias, Cerebellar
- Ramsay Hunt Cerebellar Syndrome
- Ramsay Hunt Dentate Syndrome
Note
- do not confuse entry term RAMSAY HUNT CEREBELLAR SYNDROME with RAMSAY HUNT AURICULAR SYNDROME see HERPES ZOSTER OTICUS or RAMSAY HUNT PARALYSIS SYNDROME see PARKINSONIAN DISORDERS
Scope note
- 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)
History note
- 2000(1970); use CEREBELLAR ATAXIA 1970-1990
In other languages
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Finnish
-
myoklooninen pikkuaivodyssynergia
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myoklooninen serebellaarinen dyssynergia
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pikkuaivodyssynergia
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pikkuaivojen dyssynergia
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serebellaarinen dyssynergia
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Swedish
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Ramsey Hunt cerebellärt syndrom
URI
http://www.yso.fi/onto/mesh/D002527
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