Ramsay Hunt's syndrome I

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A rare form of progressive cerebellar dyssynergia mainly characterised by intention tremor and often associated with convulsions and myoclonic epileptic jerks.

Description

A rare form of progressive cerebellar dyssynergia mainly characterised by intention tremor and often associated with convulsions and myoclonic epileptic jerks. Tremor usually begins locally in one extremity and spreads gradually, eventually involving the entire voluntary motor system. Legs are disturbed less often than arms Other features include unsteady gait, errors in estimating the range, direction, and force of voluntary movements, muscular hypotonia, asthenia, and adiadochokinesia are associated. The cause is uncertain, it may be due to a degeneration of the olivodentatorubral system. Onset usually in early adulthood, the average is 30 years. Mental deterioration occurs but is rare. It has been classified under the spinocerebellar degenerations. Some of the cases are due to mitochondrial abnormalities. Autosomal dominant inheritance with reduced penetrance suggested.

Ramsay Hunt in 1921 described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy. Characteristic features included generalized intention tremors that began as local manifestation in the extremities, especially the arms, and gradually extended to other parts of the voluntary muscular system. In addition to the coarse ataxic tremor that was present only when the muscles were in action and ceased during rest, severe involvement of the finer coordinated movements of the extremities was also present. Associated clinical features, combined with the myoclonus, and asthenia. Hunt also observed this syndrome in association with Friedreich's ataxia. Pathologic study of one case led him to consider that the symptoms were due to primary atrophy of the efferent dentate system of the cerebellum.

THE ARTIST’S PRECEPT
"I would not paint a face
Or rocks or streams or trees –
Mere semblances of things –
But something more than these.
I would not play a tune
Upon the sheng or lute,
Which did not also sing
Meanings that else were mute.
That art is best which gives
To the soul’s range no bound;
Something beside the form,
Something beyond the sound.

We thank James Welters for information submitted.

Bibliography

  • J. R. Hunt:
    Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor. Brain, Oxford, 1914, 37: 247.
  • The role of the carotid arteries in the causation of vascular lesions of the brain, with remarks on certain special features of the symptomatology.
    The American Journal of the Medical Sciences, Philadelphia, 1914, 147: 704-713.
  • Dyssynergia cerebellaris myoclonica- primary atrophy of the dentate system. A contribution to the pathology and symptomatology of the cerebellum.
    Brain, Oxford, 1921, 44: 490-538.

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