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Westphal-Leyden ataxia
Also known as:
Goekay-Tuekel syndrome
Leyden's ataxia
Westphal's ataxia
Associated persons:
Unknown Goekay
Ernst Viktor von Leyden
Unknown Tuekel
Karl Friedrich Otto Westphal
Description:
Acute ataxia with onset in childhood. It presents with vomiting, vertigo, proximal muscle rigidity, and coma. Later symptoms include slow scanning speech; ataxia with convulsive seizures and slow jerky movements of the extremities, mental disorders, faulty memory, and dementia. Sensitivity and motor strength apparently unaffected. Both sexes affected. Death within 10 years. Final stage indistinguishable from Huntington's chorea. Undetermined aetiology. Possibly autosomal dominant inheritance.
Bibliography:
- C. Westphal:
Eigentümliche mit Einschlafen verbundene Anfälle.
Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1877, 7: 631-635.
- E. Leyden:
Ueber acute Ataxie.
Zeitschrift für klinische Medicin, Berlin, 1891, 18: 576.
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