Abstract
Aims. To build up a coherent shared pathophysiology of NFLE and AP and discuss the underlying functional network. Methods. Reviewing relevant published data we point out common features in semiology of events, relations to macro- and microstructural dynamism of NREM sleep, to cholinergic arousal mechanism and genetic aspects. Results. We propose that pathological arousals accompanied by confused behavior with autonomic signs and/or hypermotor automatisms are expressions of the frontal cholinergic arousal function of different degree, during the condition of depressed cognition by frontodorsal functional loss in NREM sleep. This may happen either if the frontal cortical Ach receptors are mutated in ADNFLE (and probably also in genetically not proved nonlesional cases as well), or without epileptic disorder, in AP, assuming gain in receptor functions in both conditions. This hypothesis incorporates the previous “liberation theory” of Tassinari and the “state dissociation hypothesis” of Bassetti and Terzaghi). We propose that NFLE and IGE represent epileptic disorders of the two antagonistic twin systems in the frontal lobe. NFLE is the epileptic facilitation of the ergotropic frontal arousal system whereas absence epilepsy is the epileptic facilitation of burst-firing working mode of the spindle and delta producing frontal thalamocortical throphotropic sleep system. Significance. The proposed physiopathogenesis conceptualize epilepsies in physiologically meaningful networks.
Highlights
The precise relationship with Cyclic Alternating Pattern (CAP) A phases according to the A1-2-3 subtypes is not quite clear, since the Terzano group reported strong relationship in two studies with A1-2 types: (70–30% [40] and 72–28% [35] compared to A3 type), whereas according to the Zucconi et al [41] study most frequent relationship was found with A2 type
Parasomnias are classically grouped to NREM parasomnias or arousal parasomnias, REM parasomnias, and parasomnias having no sleep state preference
Parasomnias terminated in 74% with NREM sleep or with wakefulness (26%), while Nocturnal Frontal Lobe Epilepsy (NFLE) seizures awakened the patients in 88%
Summary
One of the most characteristic feature of the syndrome is the relation to sleep macro- and micro-structure. The precise relationship with CAP A phases according to the A1-2-3 subtypes is not quite clear, since the Terzano group reported strong relationship in two studies with A1-2 types: (70–30% [40] and 72–28% [35] compared to A3 type), whereas according to the Zucconi et al [41] study most frequent relationship was found with A2 (and A1) type. These microstructural features of sleep of NFLE patients point to a continuous higher level of arousal activity during NREM sleep, besides the overt arousal-related motor seizure elements. Some observation describes an EEG dissociation pattern with anterior delta and posterior alpha activity [37]
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