Myoclonic Cerebellar Dyssynergia (Dyssynergia Cerebellaris Progressiva)

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)
Also Known As:
Dyssynergia Cerebellaris Progressiva; Dentate Cerebellar Ataxia; Dentate Nucleus Syndrome, Ramsay Hunt; Ramsay Hunt Dentate Syndrome; Ataxia, Dentate Cerebellar; Ataxias, Dentate Cerebellar; Atrophies, Dentate Cerebellar; Atrophy, Dentate Cerebellar; Cerebellar Ataxias, Dentate; Cerebellar Atrophy, Dentate; Cerebellar Dyssynergia, Myoclonic; Cerebellar Dyssynergias; Cerebellar Dyssynergias, Myoclonic; Dentate Cerebellar Ataxias; Dentate Cerebellar Atrophies; Dyssynergia, Cerebellar; Dyssynergia, Myoclonic Cerebellar; Dyssynergias, Cerebellar; Dyssynergias, Myoclonic Cerebellar; Myoclonic Cerebellar Dyssynergias; Cerebellar Dyssynergia; Dentate Cerebellar Atrophy; Dyssynergia Cerebellaris Myoclonica; Ramsay Hunt Cerebellar Syndrome
Networked: 8 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1. Spinocerebellar Degenerations (Marinesco Sjogren Syndrome)
2. Myoclonus (Nocturnal Myoclonus)
3. Myoclonic Cerebellar Dyssynergia (Dyssynergia Cerebellaris Progressiva)
4. Machado-Joseph Disease (Spinocerebellar Ataxia Type 3)
5. Myoclonic Epilepsies (Myoclonic Encephalopathy)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Myoclonic Cerebellar Dyssynergia:
1. Nitrazepam (Serenade)IBA
2. Diazepam (Valium)FDA LinkGeneric
3. CeroidIBA
4. Amobarbital (Amsal)IBA
5. Neuraminidase deficiency with beta-galactosidase deficiencyIBA
6. Hunt's syndromeIBA

Therapies and Procedures

1. Electrodes (Electrode)
2. Dilatation and Curettage (D&C)