Magnusson et al reported an increase in psychiatric symptoms in families with ADNFLE (see Phenotype Correlations by Gene).ДЇamilial variation. Picard et al found below-normal general intellect in five (45%) of 11 subjects with special difficulty in executive tasks and concluded that cognitive dysfunction is an integral part of ADNFLE caused by a heterozygous pathogenic variant in the nicotinic receptor (see Phenotype Correlations by Gene). Clinical neurologic examination is normal and intellect is usually preserved however, in some individuals neuropsychological assessment reveals reduced intellect, cognitive deficits, or psychiatric comorbidity. Interictal sleep EEG may show infrequent epileptiform discharges.Д¬ognitive findings. Some of the seizures reported are paroxysmal dystonia similar to those during sleep, and others are generalized tonic-clonic seizures, generalized atonic seizures, and focal seizures with impairment of consciousness or awareness. Note: A minority of individuals experience daytime seizures, typically during a period of poor seizure control. Aura may be nonspecific or may consist of numbness in one limb, fear, a shiver, vertigo, or a feeling of falling or being pushed. Some individuals experience an aura preceding the seizure during sleep and are aware of the onset. A sense of difficulty breathing and hyperventilation may occur, as well as vocalization, clonic features, urinary incontinence, and secondary generalization. Retained awareness during seizures is common and may cause affected individuals to fear falling asleep. The reported frequency ranges from one to 20 attacks each night, a mean of 20 seizures per month about 60% of the affected individuals reported more than 15 seizures per month. The three distinct sub-classifications of seizure types based on clinical features of the seizures (semiology) and their duration are 'paroxysmal arousals,' 'paroxysmal dystonia,' and 'episodic wandering.'Д®pisodic wandering is somnambulic agitated behavior arising from NREM sleep. The hyperkinetic manifestations may appear bizarre, sometimes with ambulation, bicycling movements, ballism (flinging or throwing arm movements), and pelvic thrusting movements. The seizures are often stereotyped and brief (5 seconds to 5 minutes) they vary from simple arousals from sleep to dramatic hyperkinetic events with tonic or dystonic features. The affected individual often goes back to sleep rapidly after a seizure, only to be awakened by another event. Seizures may occur in any stage of sleep, although typically in clusters in non-REM (NREM) sleep, most commonly in stage 2 sleep.

The history may be obtained from the affected individual and witnesses, and supplemented if necessary by video-EEG monitoring. Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is characterized by clusters of nocturnal motor seizures with a range of manifestations.

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