HOME PRODUCTS SERVICES COMPANY CONTACT FAQ

Myoclonic Cerebellar Dyssynergia (Dyssynergia Cerebellaris Progressiva)

Subscribe to New Research on 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)

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 02/21/1970 - "[The use of "Mogadon" in myoclonic cerebellar dyssynergia. "
2.	Diazepam (Valium)FDA LinkGeneric 08/01/1967 - "Clinical and neurophysiological observations on the effect of Valium in one case of Ramsay-Hunt's "dyssynergia cerebellaris myoclonica"."
3.	CeroidIBA 07/01/1985 - "The authors studied the somatosensory evoked potentials (SEPs) in 16 cases of myoclonic encephalopathies: 8 cases of dyssynergia cerebellaris myoclonica (DCM); 2 cases of dyssynergia cerebellaris progressiva (DCP); 2 cases of Lafora's disease; 1 case of ceroid lipofuscinosis; 3 unclassifiable myoclonic syndromes. "
4.	Amobarbital (Amsal)IBA 03/31/1967 - "[EEG effects of the intravertebral introduction of amytal in a case of myoclonic cerebellar dyssynergia of the Ramsay-Hunt type. "
5.	Neuraminidase deficiency with beta-galactosidase deficiencyIBA 12/01/1995 - "In the patients with spinocerebellar degeneration with denate nucleus lesion (Machado-Joseph disease, DRPLA, MERRF, dyssynergia cerebellaris myoclonica, galactosialidosis), the gradients of Ns' were reduced, although the movements themselves were as fast as normal control. "
6.	Hunt's syndromeIBA 05/01/1975 - "It is concluded that this syndrome belongs to a groups of hereditary ataxias and myoclonus, and differs from myoclonic cerebellar dyssynergia (Ramsay Hunt) and alos from a variety of familial myoclonus and ataxia (Gilbert et al. 1963);"

Therapies and Procedures

1.	Electrodes (Electrode) 03/19/1986 - "Slow negative potential preceding voluntary self-paced middle finger extension, as recorded from scalp electrodes by backward averaging technique, was absent in two patients with dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome); but present in two patients with cerebellar cortical degeneration. "
2.	Dilatation and Curettage (D&C) 02/01/1976 - "The case is described of a woman of 26 suffering (like her mother, a brother and a sister) from a progressively degenerating cerebellar syndrome, at first considered to be hereditary cerebellar ataxia, but which, after action myoclonus appeared, was diagnosed as dyssynergia cerebellaris myoclonica (D.C.M.). "