PracticeUpdate Neurology June 2019

EDITOR’S PICKS 10

Clinical Features of Sleep-Related Hypermotor Epilepsy in Relation to the Seizure-Onset Zone Epilepsia Take-home message • Even though sleep-related seizures are thought to be related to what was previously known as nocturnal frontal lobe epilepsy and is now called sleep-related hypermotor epilepsy (SHE), seizures with a frontal lobe-like semiology can also arise outside the frontal lobe in a third of cases. The authors argue that distinguishing a frontal from an extrafrontal onset is thus challenging based on currently known studies. A study that would describe the clinical features of both frontal- and extrafrontal-onset SHE would be helpful in surgical planning for refractory patients. From 91 frontal SHE and 44 extrafrontal SHE patients, the authors classified seizure semiology according to four semiology patterns (SPs): elementary motor signs (SP1); unnatural hypermotor movements (SP2); integrated hypermotor movements (SP3); and gestural behaviors with high emotional content (SP4). Frontal and extrafrontal SHE shared many features (such as young age at onset, high seizure-frequency rate, high rate of scalp electroencephalography and magnetic resonance imaging abnormalities, similar histopathologic substrates, and good postsurgical outcomes) in temporal SHE, elementary motor signs (SP1) were rare, and gestural behaviors with high emotional manifestations (SP4) were common, whereas, in operculo-insular and posterior SHE, SP4 was absent. • Postictal confusion is significantly more common in temporal SHE. Early non-motor manifestations were common (eg, cephalic, sensory, pain) and may play a role in localizing the seizure-onset zone. Omar Iqbal Khan MD Abstract

four semiology patterns (SPs): elementary motor signs (SP1), unnatural hypermotor movements (SP2), integrated hypermotor movements (SP3), and gestural behaviors with high emotional con- tent (SP4). Early nonmotor manifestations were also assessed. " Postictal confusion is significantly more common in temporal SHE. " RESULTS Our case series consisted of 91 frontal SHE and 44 extrafrontal SHE cases. Frontal and extrafrontal SHE shared many features such as young age at onset, high seizure-frequency rate, high rate of scalp electroencephalography (EEG) and magnetic resonance imaging (MRI) abnor- malities, similar histopathologic substrates, and good postsurgical outcome. Within the frontal lobe, SPs were organized in a posteroanterior gradient (SP1-4) with respect to the SOZ. In tem- poral SHE, SP1 was rare and SP3-4 frequent, whereas in operculoinsular and posterior SHE, SP4 was absent. Nonmotor manifestations were frequent (70%) and some could provide valuable localizing information. SIGNIFICANCE Our study shows that the presence of certain SP and nonmotor manifestations may provide helpful information to localize seizure onset in patients with SHE. Clinical Features of Sleep-Related Hypermotor Epilepsy in Relation to the Seizure-Onset Zone: A Review of 135 Surgically Treated Cases. Epilepsia 2019 Apr 01;60(4)707-717, SA Gibbs,

of both frontal and extrafrontal SHE, focusing on ictal semiologic patterns in order to increase diagnostic accuracy. METHODS We retrospectively analyzed the clini- cal features of patients with drug-resistant SHE seen in our center for epilepsy surgery. Patients were divided into frontal and extrafrontal SHE (temporal, operculoinsular, and posterior SHE). We classified seizure semiology according to

OBJECTIVES Sleep-related hypermotor epilepsy (SHE), formerly nocturnal frontal lobe epilepsy, is characterized by abrupt and typically sleep-re- lated seizures with motor patterns of variable complexity and duration. They seizures arise more frequently in the frontal lobe than in the extrafrontal regions but identifying the seizure onset-zone (SOZ) may be challenging. In this study, we aimed to describe the clinical features

P Proserpio, S Francione, et al. www.practiceupdate.com/c/81308

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