Myoclonic and Major Seizures in Early Adult Huntington's Chorea: Case-Report and Electro-Clinical Findings

Kereshi, Stjepan; Schlagenhauff, Reinhold E.; Richardson, Kathryn S.

doi:10.1177/155005948001100202

Cited by 11 publications

(7 citation statements)

References 5 publications

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“…In case of myoclonus impacting the functional capacity of the patients, treatment with sodium valproate or clonazepam, used alone or in combination, and in escalating doses, is recommended (Grade C) (25–32). Levetiracetam is a therapeutic alternative for the same indication.…”

Section: Resultsmentioning

confidence: 99%

International Guidelines for the Treatment of Huntington's Disease

et al. 2019

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The European Huntington's Disease Network (EHDN) commissioned an international task force to provide global evidence-based recommendations for everyday clinical practice for treatment of Huntington's disease (HD). The objectives of such guidelines are to standardize pharmacological, surgical and non-pharmacological treatment regimen and improve care and quality of life of patients. A formalized consensus method, adapted from the French Health Authority recommendations was used. First, national committees (French and English Experts) reviewed all studies published between 1965 and 2015 included dealing with HD symptoms classified in motor, cognitive, psychiatric, and somatic categories. Quality grades were attributed to these studies based on levels of scientific evidence. Provisional recommendations were formulated based on the strength and the accumulation of scientific evidence available. When evidence was not available, recommendations were framed based on professional agreement. A European Steering committee supervised the writing of the final recommendations through a consensus process involving two rounds of online questionnaire completion with international multidisciplinary HD health professionals. Patients' associations were invited to review the guidelines including the HD symptoms. Two hundred and nineteen statements were retained in the final guidelines. We suggest to use this adapted method associating evidence base–medicine and expert consensus to other rare diseases.

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Section: Resultsmentioning

confidence: 99%

International Guidelines for the Treatment of Huntington's Disease

et al. 2019

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“…Initial clinical trials with valproate proved disappointing and did not show any clinical benefit in HD [135][136]. Later, some studies have indicated beneficial effects of valproate in myoclonic hyperkinesias in HD patients either as a monotherapy [137] or as a combination therapy with haloperidol [138]. These studies demonstrate improved motor performance in subjects after valproate treatment.…”

Section: Role Of Hdac Inhibitors In Huntington's Disease Patients -Clmentioning

confidence: 94%

Transcriptional dysregulation in Huntington’s disease: The role of histone deacetylases

Sharma

Taliyan

2015

Pharmacological Research

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“…Epilepsy is especially frequent in the Westphal-variant, however, it may also occur in adults with HD [226]. Several HD patients with epilepsy and myoclonus responded to treatment with valproate [227][228][229] or clonazepam [230]. Urinary incontinency is a major, widely ignored problem of late-stage HD [231] and is usually caused by a detrusor hyperreflexia.…”

Section: B Treatment Of Other Neurological Featuresmentioning

confidence: 99%