Update on Pharmacological Treatment of Progressive Myoclonus Epilepsies

Ferlazzo, Edoardo; Trenité, Dorothee Kasteleijn‐Nolst; Haan, Gerrit-Jan de; Nitschké, Felix; Ahonen, Saija; Gasparini, Sara; Minassian, Berge A.

doi:10.2174/1381612823666170809114654

Cited by 24 publications

(19 citation statements)

References 76 publications

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“…• Patients with focal lesions 25 (eg focal cortical dysplasia, tuberous sclerosis complex) • Tonic-clonic seizures in IGE 12 • PME 26 • Brain-tumor-related epilepsy 27…”

Section: Perampanel As Early Adjunctive Therapymentioning

confidence: 99%

Optimal Use of Perampanel in Asian Patients with Epilepsy: Expert Opinion

Chinvarun¹,

Huang²,

Wu³

et al. 2021

TCRM

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Perampanel is a once-daily, first-in-class AMPA receptor antagonist approved for the treatment of epilepsy and exhibits broad-spectrum efficacy in a range of seizure types when used as both monotherapy and adjunctive therapy. Clinical studies and real-world evidence have demonstrated the advantages of initiating perampanel at low doses and utilizing a slow titration strategy. Initiating perampanel at an early stage has also been shown to be associated with better patient outcomes. However, the optimal use and place of perampanel in clinical practice has not yet been clearly defined for the Asian patient population. Use of perampanel in clinical practice varies markedly across the Asia region because of variation in knowledge, attitudes, and practice. There is currently no specific guidance on best practices for prescribing perampanel in Asian patients or how to optimize treatment strategies to maximize adherence. A group of epilepsy experts attended a virtual meeting in September 2020 to discuss their experience with using perampanel in the Asian practice setting, including their views regarding appropriate patient populations, optimal starting and maintenance doses, optimal titration regimens, key barriers to adherence, and prevention and management of adverse events. This article summarizes key clinical and realworld evidence for perampanel and consolidates the experts' opinions on optimization of perampanel prescribing and adherence in real-world practice, providing practical strategies for clinicians to implement to improve outcomes for people with epilepsy in Asia.

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“…• Patients with focal lesions 25 (eg focal cortical dysplasia, tuberous sclerosis complex) • Tonic-clonic seizures in IGE 12 • PME 26 • Brain-tumor-related epilepsy 27…”

Section: Perampanel As Early Adjunctive Therapymentioning

confidence: 99%

Optimal Use of Perampanel in Asian Patients with Epilepsy: Expert Opinion

Chinvarun¹,

Huang²,

Wu³

et al. 2021

TCRM

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“…A series of eight Huntington disease patients with predominantly myoclonic movements showed a dosedependent reduction in functional disability [19]. Valproic acid is often regarded as a first-line therapy in progressive myoclonic epilepsies [20].…”

Section: Treatmentmentioning

confidence: 99%

Physiology-Based Treatment of Myoclonus

Pena

Caviness

2020

Neurotherapeutics

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Myoclonus can cause significant disability for patients. Myoclonus has a strikingly diverse array of underlying etiologies, clinical presentations, and pathophysiological mechanisms. Treatment of myoclonus is vital to improving the quality of life of patients with these disorders. The optimal treatment strategy for myoclonus is best determined based upon careful evaluation and consideration of the underlying etiology and neurophysiological classification. Electrophysiological testing including EEG (electroencephalogram) and EMG (electromyogram) data is helpful in determining the neurophysiological classification of myoclonus. The neurophysiological subtypes of myoclonus include cortical, cortical-subcortical, subcortical-nonsegmental, segmental, and peripheral. Levetiracetam, valproic acid, and clonazepam are often used to treat cortical myoclonus. In cortical-subcortical myoclonus, treatment of myoclonic seizures is prioritized, valproic acid being the mainstay of therapy. Subcortical-nonsegmental myoclonus may be treated with clonazepam, though numerous agents have been used depending on the etiology. Segmental and peripheral myoclonus are often resistant to treatment, but anticonvulsants and botulinum toxin injections may be of utility depending upon the case. Pharmacological treatments are often hampered by scarce evidence-based knowledge, adverse effects, and variable efficacy of medications.

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“…Sodium valproate is generally considered to be first-line pharmacological therapy, although should be avoided in patients with MERFF. Drugs such as carbamazepine, phenytoin, and vigabatrin are known to aggravate myoclonus and seizures, whilst lamotrigine can have an unpredictable effect [ 14 ]. Levetiracetam, clonazepam, zonisamide, brivaracetam and perampanel can be useful adjuncts.…”

Section: Discussionmentioning

confidence: 99%

Moyamoya and progressive myoclonic epilepsy secondary to CLN6 bi-allelic mutations – A previously unreported association

Talbot

Singh

Puvirajasinghe

et al. 2020

Epilepsy & Behavior Reports

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The neuronal ceroid lipofuscinoses (NCL) are a collection of lysosomal storage diseases characterised by the accumulation of characteristic inclusions containing lipofuscin in various tissues of the body and are one of the causes of progressive myoclonic epilepsy. Mutations in at least thirteen genes have been identified as causes of NCL, which can present as infantile, late-infantile, juvenile or adult forms. CLN6 codes for an endoplasmic reticulum transmembrane protein of unknown function. Homozygous and compound heterozygous mutations of the gene are associated with both late-infantile (LINCL) and adult onset (ANCL) forms of NCL, including Kufs disease, comprising ANCL without associated visual loss. Moyamoya, a rare vasculopathy of the circle of Willis, has been reported in conjunction with a number of inflammatory and other diseases, as well as a handful of lysosomal storage diseases. To our knowledge, this is the first reported case of Moyamoya in the context of the neuronal ceroid lipofuscinoses or a CLN6 -related disease.

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Update on Pharmacological Treatment of Progressive Myoclonus Epilepsies

Cited by 24 publications

References 76 publications

Optimal Use of Perampanel in Asian Patients with Epilepsy: Expert Opinion

Optimal Use of Perampanel in Asian Patients with Epilepsy: Expert Opinion

Physiology-Based Treatment of Myoclonus

Moyamoya and progressive myoclonic epilepsy secondary to CLN6 bi-allelic mutations – A previously unreported association

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