Drug-Resistant Myoclonic Epilepsy Revealing Juvenile Huntington's Disease: A Case Report
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Published:2018-03
Issue:01
Volume:07
Page:021-023
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ISSN:2146-457X
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Container-title:Journal of Pediatric Epilepsy
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language:en
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Short-container-title:J Pediatr Epilepsy
Author:
Feki Fatma1,
Triki Chahnez1,
Amara Nesrine1
Affiliation:
1. Department of Child Neurology, Sfax University Hospital, Sfax, Tunisia
Abstract
AbstractJuvenile Huntington's disease (JHD) shares many general clinical features with the adult form. One important difference is that JHD patients experience more epileptic manifestations, sometimes difficult to control. We describe an atypical clinical picture of a genetically confirmed JHD patient diagnosed during evaluation for a progressive myoclonic epilepsy. A female patient with a family history of psychiatric disorders developed recurrent drug-resistant myoclonic seizures at the age of 6 years, followed by extrapyramidal symptoms (rigidity and dystonia). Cognitive impairment, akinetic rigidity syndrome, and dystonia were noticed at the age of 10 years. Epileptiform abnormalities were noted in ictal electroencephalography. Magnetic resonance imaging showed brain atrophy. Genetic testing for HD confirmed the diagnosis. JHD can initially manifest as myoclonic epilepsy. A DNA testing should be performed if clinical history is suggestive.
Publisher
Georg Thieme Verlag KG
Subject
Clinical Neurology,Pediatrics, Perinatology, and Child Health