Holmes Syndrome
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
- ↑ 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 .
- ↑ GM Holmes: A form of familial degeneration of the cerebellum. In: Brain. Oxford 1907, 30, pp. 466-489.
- ↑ Who named it