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Therapy in Sleep-Related Hypermotor Epilepsy (SHE)

  • Sleep Disorders (A Iranzo, Section Editor)
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Abstract

Purpose of review

The purpose of this review is to summarize and discuss current options and new advances in the treatment of sleep-related hypermotor epilepsy (SHE), focusing on pharmacological and surgical treatments.

Recent findings

Carbamazepine (CBZ) has traditionally been regarded as the first-line treatment option in SHE patients. In patients showing an unsatisfactory response to monotherapy, topiramate (TPM), lacosamide (LCM) and acetazolamide (ACZ) could be reasonable add-on strategies. The increasing understanding of the role of neuronal nicotinic acetylcholine receptor (nAChR) in SHE pathophysiology has led to the evaluation of compounds able to modulate this receptor system, including nicotine patches and fenofibrate. Despite polytherapy with two or more antiepileptic drugs (AEDs), about one-third of SHE patients suffer from drug-resistant seizures. In selected drug-resistant patients, epilepsy surgery is a therapeutic approach that offers high probability of recovery, with up to two-third of patients becoming seizure-free after resection of the epileptogenic zone.

Summary

An evidence-based approach from randomized placebo-controlled trials in SHE patients is lacking, and current treatment recommendations are based only on case reports and small series.

Furthermore, most of these case reports and case series involve patients with a known genetic defect, which only accounts for a small proportion of SHE patients. Therefore, a prospective study in a large cohort of sporadic SHE patients is necessary in order to provide clinicians with an evidence-based treatment for this rare form of epilepsy. An early and effective anti-epileptic treatment is mandatory for SHE patients, in order to prevent the risk of increasing seizure frequency throughout the disease course with relevant impact on patients’ cognitive profile and daytime performances.

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Author notes

  1. Gian Maria Asioli and Simone Rossi contributed equally to this work.

Authors and Affiliations

  1. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy

    Gian Maria Asioli MD, Simone Rossi MD, Francesca Bisulli MD, PhD, Laura Licchetta MD, PhD, Paolo Tinuper MD & Federica Provini MD, PhD

  2. IRCCS, Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Francesca Bisulli MD, PhD, Laura Licchetta MD, PhD, Paolo Tinuper MD & Federica Provini MD, PhD

  3. Department of Biomedical and Neuromotor Sciences, IRCCS, Istituto delle Scienze Neurologiche, Via Altura, 3, 40123, Bologna, Italy

    Federica Provini MD, PhD

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  1. Gian Maria Asioli MD
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  2. Simone Rossi MD
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  3. Francesca Bisulli MD, PhD
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  4. Laura Licchetta MD, PhD
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  5. Paolo Tinuper MD
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  6. Federica Provini MD, PhD
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Correspondence to Federica Provini MD, PhD.

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Conflict of Interest

Dr. Asioli declares that he has no conflict of interest. Dr. Rossi declares that he has no conflict of interest. Dr. Bisulli declares that she has no conflict of interest. Dr. Licchetta declares that she has no conflict of interest. Dr. Tinuper reports non-financial support and other from EISAI, non-financial support and other from LIVANOVA, non-financial support and other from UCB, non-financial support and other from GW, outside the submitted work. Dr. Provini reports personal fees from Vanda Pharmaceutical, personal fees from Sanofy, personal fees from Zambon, personal fees from Fidia, personal fees from Bial, personal fees from Eisai Japan, personal fees from Italfarmaco, outside the submitted work.

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Asioli, G.M., Rossi, S., Bisulli, F. et al. Therapy in Sleep-Related Hypermotor Epilepsy (SHE). Curr Treat Options Neurol 22, 1 (2020). https://doi.org/10.1007/s11940-020-0610-1

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