From nocturnal frontal lobe epilepsy to sleep-related hypermotor epilepsy: a 35-year diagnostic challenge. Diagnostic criteria with different level of certainty are also provided. Major points of agreement emerged on the relationship of the seizures with sleep and not with the circadian pattern of seizure occurrence and on the possible extrafrontal origin of hypermotor seizures. The paper summarized the results of the Consensus Conference held in Bologna, Italy in 2014 on SHE during which the Nocturnal Frontal Lobe Epilepsy was renamed Sleep-Related Hypermotor Epilepsy (SHE). Definition and diagnostic criteria of sleep-related hypermotor epilepsy. Tinuper P, Bisulli F, Cross JH, Hesdorffer D, Kahane P, Nobili L, et al.Nocturnal paroxysmal dystonia with short-lasting attacks: three cases with evidence for an epileptic frontal lobe origin of seizures. Tinuper P, Cerullo A, Cirignotta F, Cortelli P, Lugaresi E, Montagna P. Hypnogenic paroxysmal dystonia : epileptic seizure or a new syndrome ? Sleep. Papers of particular interest, published recently, have been highlighted as: 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. 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. SummaryĪn 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.įurthermore, 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. 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. ![]() Despite polytherapy with two or more antiepileptic drugs (AEDs), about one-third of SHE patients suffer from drug-resistant seizures. 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. In patients showing an unsatisfactory response to monotherapy, topiramate (TPM), lacosamide (LCM) and acetazolamide (ACZ) could be reasonable add-on strategies. Recent findingsĬarbamazepine (CBZ) has traditionally been regarded as the first-line treatment option in SHE patients. 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.
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