Dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome): a condition unrelated to mitochondrial encephalomyopathies.
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Dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome): a condition unrelated to mitochondrial encephalomyopathies.
Thirteen patients with dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome) had full clinical and neurophysiological study as well as muscle biopsy. The patients had action myoclonus, generalised epileptic seizures, and mild cerebellar syndrome. The disease was inherited in an autosomal recessive pattern in five patients, and occurred as isolated cases in the remaining eight patients. The...
متن کاملFacial paralysis due to Ramsay Hunt syndrome - A rare condition.
Ramsay Hunt syndrome (or herpes zoster oticus) is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion occurs. Usually, there are auricular vesicles and symptoms and signs such otalgia and peripheral facial paralysis. In addition, rarely, a rash around the mouth can be seen. Immunodeficient patients are more susceptible...
متن کاملMyoclonus and Ataxia Occuring in a Family.
[n 1914 J. Ramsay Hunt described the syndrome he called 'dyssynergia cerebellaris progressiva'. He regarded this condition with its well-defined cerebellar symptoms as an 'organic disease caused by degeneration of certain special structures of the cerebellar mechanism'. In 1921 at an annual meeting of the American Neurological Association, he presented a further six cases which combined the sym...
متن کاملRamsay Hunt syndrome
The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explain...
متن کاملAtypical Ramsay Hunt syndrome.
Ramsay Hunt syndrome (RHS) is the reactivation of herpes zoster in the geniculate ganglion and typically presents the triad of ipsilateral peripheral type facial paralysis, ear pain, and erythematous vesicles in the external auditory canal and auricle. However, some unusual variants may occur. Here we present a patient of atypical RHS with uncommonly extensive dermatomal involvement of cranial ...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 1989
ISSN: 0022-3050
DOI: 10.1136/jnnp.52.2.262