Holmes Syndrome

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The Holmes syndrome or Holmes degeneration is a hereditary disorder characterized by cerebellar-related ataxia due to cerebellar - degeneration . It is counted among the ADCA .

Synonyms are:

  • Systematized primarily progressive cerebellar degeneration
  • Cerebello-olive atrophy

The name refers to the first description from 1907 by the British neurologist Gordon Morgan Holmes (1876-1965).

distribution

The inheritance is autosomal dominant .

Clinical manifestations

Diagnostic criteria are:

  • Manifestation in middle to later adulthood
  • Ataxia beginning on the legs and spreading towards the head
  • Speech disorders
  • Holding and intent tremors
  • Decreased muscle tone
  • Later, nystagmus

Individual evidence

  1. a b c B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7th edition. Urban & Schwarzenberg, 1990, ISBN 3-541-01727-9 .
  2. GM Holmes: A form of familial degeneration of the cerebellum. In: Brain. Oxford 1907, 30, pp. 466-489.
  3. Who named it