Progressive myoclonic ataxia without ragged red fibres: Unverricht-Lundborg disease vs Ramsay Hunt syndrome
| Autor: | A. G. H. Al-Zuhair, R. Khan, R. A. Shakir |
|---|---|
| Rok vydání: | 1992 |
| Předmět: |
Adult
Male Myoclonus congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Pediatrics Ataxia Adolescent Epilepsies Myoclonic Progressive myoclonus epilepsy Diagnosis Differential Consanguinity Epilepsy Degenerative disease medicine Humans Spinocerebellar Degenerations Inclusion Bodies Neurologic Examination Myoclonic Cerebellar Dyssynergia Intellectual impairment Ramsay Hunt syndrome Muscles General Medicine medicine.disease humanities Mitochondria Muscle Surgery Unverricht–Lundborg disease Kuwait Neurology Female Epilepsy Tonic-Clonic Neurology (clinical) medicine.symptom Psychology |
| Zdroj: | Acta Neurologica Scandinavica. 86:470-473 |
| ISSN: | 1600-0404 0001-6314 |
| Popis: | We describe eight patients from three families presenting with myoclonus, ataxia, infrequent seizures and minimal intellectual impairment. All were Arabs from different parts of the Arabian peninsula. The new consensus on terminology, genetic and clinical definition of Baltic myoclonus, Ramsay Hunt syndrome and Unverricht-Lundborg disease suggests that our group are best categorised under the term of progressive myoclonic ataxia of the Unverricht-Lundborg type. Moreover, this report reinforces the existence of this syndrome outside Scandinavia. |
| Databáze: | OpenAIRE |
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