Progressive myoclonic ataxia without ragged red fibres: Unverricht-Lundborg disease vs Ramsay Hunt syndrome

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...

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Bibliographic Details
Published inActa neurologica Scandinavica Vol. 86; no. 5; p. 470
Main Authors Shakir, R A, Khan, R A, al-Zuhair, A G
Format Journal Article
LanguageEnglish
Published Denmark 01.11.1992
Subjects
Adolescent
Adult
Consanguinity
Diagnosis, Differential
Epilepsies, Myoclonic - diagnosis
Epilepsies, Myoclonic - genetics
Epilepsy, Tonic-Clonic - diagnosis
Epilepsy, Tonic-Clonic - genetics
Female
Humans
Inclusion Bodies - ultrastructure
Kuwait
Male
Mitochondria, Muscle - ultrastructure
Muscles - pathology
Myoclonic Cerebellar Dyssynergia - diagnosis
Myoclonic Cerebellar Dyssynergia - genetics
Myoclonus - diagnosis
Myoclonus - genetics
Neurologic Examination
Spinocerebellar Degenerations - diagnosis
Spinocerebellar Degenerations - genetics
Kuwait
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Summary: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.
ISSN:0001-6314
DOI:10.1111/j.1600-0404.1992.tb05126.x