Morvan syndrome and related disorders associated with CASPR2 antibodies
Jan. 23, 2023
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In this article, the authors present recent data and discussion about the causes, syndromes, diagnosis, and management of hyperkinetic seizures.
• 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.
• 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.
• Exclusively or predominantly nocturnal hyperkinetic seizures are the typical manifestations in autosomal dominant sleep-related hypermotor epilepsy (ADSHE), previously named autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE), which is associated with mutations in the neuronal nicotinic acetylcholine receptor subunits.
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” (05; 12).
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 (24). Lüders and colleagues first introduced the term “hypermotor seizure” in a proposal for a semiologic seizure classification (23). In 2001, the ILAE Task Force on Classification and Terminology proposed the term “hyperkinetic seizure” to describe this type of event (05), and it was chosen for the 2017 classification (12).
Sleep‐related hypermotor epilepsy (SHE), previously named nocturnal frontal lobe epilepsy (NFLE), is a focal epilepsy characterized by a wide spectrum of seizures occurring predominantly during sleep (14; 21). The diagnosis of sleep‐related hypermotor epilepsy (SHE) is based on the clinical history and video-EEG documentation of seizures (39; 21). Sleep‐related hypermotor epilepsy includes hyperkinetic seizures associated with asymmetric‐tonic attacks, dystonic postures, paroxysmal arousals, and epileptic nocturnal wandering (38; 47; 21).
The accurate prevalence of hyperkinetic seizures is not known. In one cohort, this seizure type was reported in 12% of patients (26).
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