Hyperkinetic seizures

Ana Carolina Coan MD PhD (

Dr. Coan of the University of Campinas - UNICAMP has no relevant financial relationships to disclose.

)
Fernando Cendes MD PhD (Dr. Cendes of the University of Campinas - UNICAMP has no relevant financial relationships to disclose.)
Jerome Engel Jr MD PhD, editor. (

Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, received honorariums from Cerebel for advisory committee membership.

)
Originally released June 10, 2014; last updated January 18, 2021; expires January 18, 2024

Overview

In this article, the authors present recent data and discussion about the causes, syndromes, diagnosis, and management of hyperkinetic seizures.

Key points

 

• Hyperkinetic seizures are characterized by motor seizures involving predominantly proximal limb or axial muscles in irregular sequential ballistic movements.

 

• The origin of hyperkinetic seizures is more commonly localized in the mesial frontal or orbitofrontal regions.

 

• Seizures often occur during sleep and recur repeatedly, with intervals of a few seconds.

 

• Exclusively nocturnal hyperkinetic seizures are the typical manifestations in autosomal dominant nocturnal frontal lobe epilepsy.

 

• Sleep‐related hyperkinetic epilepsy (SHE), previously named nocturnal frontal lobe epilepsy (NFLE), is a focal epilepsy characterized by a wide spectrum of seizures occurring predominantly during sleep, including hyperkinetic seizures.

Historical note and terminology

According to proposed terminology by the International League Against Epilepsy (ILAE), hyperkinetic seizures are characterized by motor seizures involving “predominantly proximal limb or axial muscles producing irregular sequential ballistic movements, such as pedaling, pelvic thrusting, thrashing, rocking movements or by an increase of ongoing movements or inappropriately rapid performance of a movement” (Blume et al 2001).

Epileptic seizures with prominent motor components have been described in the literature for over 50 years. In 1986, Lugaresi and colleagues proposed the term “nocturnal paroxysmal dystonia” to describe a syndrome of sleep-related motor attacks (Lugaresi et al 1986). Lüders and colleagues first introduced the term “hypermotor seizure” in a proposal for a semiologic seizure classification (Lüders et al 1993). In 2001, the ILAE Task Force on Classification and Terminology proposed the term “hyperkinetic seizure” to describe this type of event (Blume et al 2001), but the operational classification of seizure types by ILAE proposes that the term “hypermotor” should replace “hyperkinetic” (Fisher et al 2017).

Sleep‐related hyperkinetic epilepsy (SHE), previously named nocturnal frontal lobe epilepsy (NFLE), is a focal epilepsy characterized by a wide spectrum of seizures occurring predominantly during sleep (Gibbs et al 2019; Loddo et al 2020). The diagnosis of sleep‐related hyperkinetic epilepsy is based on the clinical history and video-EEG documentation of seizures ((Rheims et al 2008; Loddo et al 2020). Sleep‐related hyperkinetic epilepsy includes hyperkinetic seizures associated with asymmetric‐tonic attacks, dystonic postures, paroxysmal arousals, and epileptic nocturnal wandering (Provini et al 1999; Tinuper et al 2016; Loddo et al 2020).

The accurate prevalence of hyperkinetic seizures is not known. In one cohort, this seizure type was reported in 12% of patients (Manford et al 1996).

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