It is named for James Ramsay Hunt who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921. (wikipedia.org)
A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. (jefferson.edu)
Epilepsy1
Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment It has also been alternatively called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome. (wikipedia.org)
Ataxia1
Onset of symptoms usually occurs in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks. (wikipedia.org)
Brainstem1
Ramsay Hunt syndrome type 1 is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei and has been associated with a wide range of diseases, including Lafora disease, dentatorubropallidoluysian atrophy, and celiac disease. (wikipedia.org)
Jerks3
A very rare and progressive cerebellar dyssynergia with intention tremor first localized to one extremity, convulsions, and myoclonic epileptic jerks. (mhmedical.com)
CASE PRESENTATION: We present the case of a patient with severe myoclonic jerks and mild dystonia since childhood. (bvsalud.org)
At first neurological visit at the age of 46 years old, she presented brief myoclonic jerks predominating in the upper limbs and neck, mild at rest and elicited by action, posture and tactile stimulus. (bvsalud.org)
Atrophy1
Ramsay Hunt syndrome type 1 is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei and has been associated with a wide range of diseases, including Lafora disease, dentatorubropallidoluysian atrophy, and celiac disease. (wikipedia.org)
Intention tremor1
Dyssynergia and intention tremor of the extremities become prominent features with age. (medscape.com)
Spinal cord1
Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (nih.gov)
Disorders2
Treatment of cerebellar disorders depends on the cause. (medlineplus.gov)
RESULTS: EOA associated gene mutations cause a spectrum of disorders, including myoclonic and epileptic phenotypes. (bvsalud.org)
Manifestations1
Slow initiation and performance of all voluntary activity and muscular hypotonia are characteristic and are also manifestations of cerebellar symptomatology. (medscape.com)