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DeCS
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Descriptor English:
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Myoclonic Cerebellar Dyssynergia
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Descriptor Spanish:
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Disinergia Cerebelosa Mioclónica
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Descriptor Portuguese:
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Dissinergia Cerebelar Mioclônica
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Synonyms English:
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Ramsay Hunt Cerebellar Syndrome
Cerebellar Dyssynergia
Dentate Cerebellar Atrophy
Dyssynergia Cerebellaris Myoclonica
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Tree Number:
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C10.228.140.252.700.250
C10.228.854.787.500
C10.574.500.825.250
C16.320.400.780.500
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Definition English:
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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) |
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Indexing Annotation English:
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do not confuse X ref RAMSAY HUNT CEREBELLAR SYNDROME with RAMSAY HUNT AURICULAR SYNDROME see HERPES ZOSTER OTICUS or RAMSAY HUNT PARALYSIS SYNDROME see PARKINSONIAN DISORDERS
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History Note English:
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2000(1970); use CEREBELLAR ATAXIA 1970-1990
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Allowable Qualifiers English:
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Record Number:
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22381
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Unique Identifier:
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D002527
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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