Preferred Label : myoclonic cerebellar dyssynergia;
MeSH definition : 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);
MeSH synonym : dentate cerebellar ataxias; dentate cerebellar atrophy; atrophies, dentate cerebellar; cerebellar atrophy, dentate; ramsay hunt dentate syndrome; cerebellar dyssynergias, myoclonic; dyssynergias, myoclonic cerebellar; dentate cerebellar ataxia; ataxia, dentate cerebellar; dentate cerebellar atrophies; dentate nucleus syndrome, ramsay hunt; dyssynergia cerebellaris myoclonica; dyssynergia, myoclonic cerebellar; myoclonic cerebellar dyssynergias; ramsay hunt cerebellar syndrome; ataxias, dentate cerebellar; cerebellar dyssynergias; dyssynergia, cerebellar; dyssynergias, cerebellar; atrophy, dentate cerebellar; cerebellar ataxias, dentate; cerebellar dyssynergia; cerebellar dyssynergia, myoclonic; dyssynergia cerebellaris progressiva;
Related MeSH term : cerebelloparenchymal disorder V; Spinodentate Atrophy; Dyssynergia Cerebellaris Myoclonica Of Hunt;
MeSH annotation : 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; 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;
Origin ID : D002527;
UMLS CUI : C0007761;
Allowable qualifiers
Automatic exact mappings (from CISMeF team)
DO Cross reference
Manual NTBT mappings (CISMeF)
Record concept(s)
Related MeSH Supplementary Concept(s)
Semantic type(s)
UMLS correspondences (same concept)
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)