myoclonic cerebellar dyssynergia
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)
MONDO:0000114
cerebelloparenchymal disorder
is_a
MONDO:0024237
inherited neurodegenerative disorder
is_a
CPD5
related
Ramsay Hunt cerebellar syndrome
related
Ramsay Hunt syndrome
related
Ramsay Hunt syndrome type 1
related
Ramsay Hunt syndrome type 1 (formerly)
related
cerebelloparenchymal disorder V
related
cerebelloparenchymal disorder type 5
exact
dentate cerebellar ataxia
related
dentatorubral atrophy
related
dyssynergia cerebellaris myoclonica
exact
dyssynergia cerebellaris progressiva
related
myoclonus and ataxia
exact
primary dentatum atrophy
related
progressive myoclonus ataxia
related