Autosomal dominant nocturnal frontal lobe epilepsy (ANDFLE) is an idiopathic focal epileptic syndrome with seizures during sleep, in which spasms and twitching of the arms and legs, as well as whistling and screaming, are common and are often mistaken for non-epileptic seizures, such as sleep disorders. Autosomal dominant nocturnal frontal lobe epilepsy was first described in 1994 by Ingrid E. Scheffer et al. and is characterized by strings of nocturnal motor seizures. the causative gene was first identified in 1995 by Steinlein et al. and was the first monogenetic partial epilepsy discovered. It is now thought to be a mutation in the gene encoding the acetylcholine receptor, and four gene variants (CHRNA4, 15q24, CHRNB2, CHRNA2) are known to cause ANDFLE . The age of onset ranged from 2 months to 52 years, with a mean of 11.7 years. 53% had onset before the age of 10 years, and 35% had onset between the ages of 10 and 20 years. All patients had episodes during sleep, 58% shortly after falling asleep, 48% in the early morning hours, 9% reported episodes throughout the night, 30% had episodes during daytime naps, and 58% had a small number of awakening episodes. Seizure frequency: 76% of the patients had a series of seizures, ranging from 4 to 11 per night, with a mean of 7.7 seizures, while 24% of the patients had only one seizure per night. Seizures were brief, lasting from 5 seconds to 5 minutes, with an average of 74 seconds. Seizures begin with gasping, groaning, or uttering single words, often with eyes open, a fearful expression, and staring or upturned eyes. Mouth or hand automatisms are uncommon. The prominent manifestations are somatic motor automatisms, such as sudden sitting up, fierce over-activity, hip forward force, generalized tonic rigidity, clonic shaking and dystonic-like movements; or sudden head lifting, head shaking, head tilting; the upper limbs can be lifted or throwing-like movements, the lower limbs can be overextended, circling, pedaling-like movements, or limb rhythmic-like movements. The patient may crawl around the bed or even fall into the bed and get hurt. Some patients can realize the seizure but can not control it, can hear the external sound but can not respond, and can recall the seizure afterwards. Some patients lose consciousness during the seizure, indicating a secondary generalized seizure. Consciousness returns immediately after the seizure, and there is rarely postictal confusion or postictal headache. The above symptoms suggest that the seizure originates from or involves the supplementary motor area of the frontal lobe (SMA seizure). The severity of the seizures varies from patient to patient within the same family, from early onset of the disease, with episodes occurring every night, leading to severe sleep deprivation, to short episodes in adolescence, which can only be diagnosed by systematic family investigation. The majority of cases are intermittent episodes. Predisposing factors include stress and fatigue, and in women the frequency of attacks may be increased or decreased by menstruation, pregnancy, or menopause. The patient has normal intelligence and a normal neurologic examination and neuroimaging. Diagnosis The diagnosis is confirmed on the basis of clinical presentation, history, and EEG examination. Frequent and brief nocturnal motor seizures characterized by postural or torsional tonus, dystonic-like movements, vocalizations, and somatic automatisms, normal psychomotor development, no evidence of structural brain damage, and a family history of epilepsy and undiagnosed sleep disorders contributes to the diagnosis of ADNFLE. the EEG positivity rate is low, but Video-EEG readily records clinical one-EEG features during the seizure phase. EEG features: background activity and sleep cycles are normal. The sleep process is often frequently interrupted in patients with frequent episodes during sleep. Paroxysmal abnormalities were rarely seen in both waking and sleep EEGs during interictal periods; 84% of patients had no epileptiform activity, 16% had epileptiform activity in one or both frontal, fronto-central, frontotemporal, or temporal regions, and 22% had bilateral or limited slow-wave increases. Video-EEG during the seizure phase showed seizures occurring mainly in the NREM-II phase, with predominantly bifrontal sharp slow-wave activity, rhythmic spikes, rhythmic activity, or arousal-like responses followed by 9-Hz rhythmic activity. In a few patients, there are no clear ictal discharges, or the ictal EEG is masked by a large number of motor artifacts. Differential Diagnosis Autosomal dominant nocturnal frontal lobe epilepsy is often clinically misdiagnosed as being a result of normal sleep behavior, benign nocturnal sleep disorders, nocturnal paroxysmal dystonia, familial movement disorders, or pseudoseizures. Sleep disorders include night terrors, nightmares, and sleepwalking, with night terrors being the most common. The main points of differentiation are the long duration of night terrors, usually 5-10 minutes, the lack of strings of seizures, the seizures are dominated by emotional symptoms such as fear and crying, less motor seizure manifestations, and the seizures usually appear in the NREMlll-1V stage of the first sleep cycle after falling asleep, accompanied by EEG awakening response, without epileptiform electrical activity. The seizure profile of nocturnal paroxysmal dystonia is very similar to that of ADNFLE, with a predominance of brief and cascading dystonic-like movements during sleep, no EEG changes during the seizure period or in the interictal period, good response to carbamazepine treatment, and familial cases consistent with an autosomal mode of inheritance. These events are now considered to be epileptic seizures, and it is hypothesized that the epileptic foci are located in the medial orbitofrontal region, the supplementary motor area, or the temporal lobe, and represent a group of sleep-related partial seizures. Therefore, nocturnal paroxysmal dystonia may be the same type of epileptic lesion as ADNFLE. Familial movement disorders are a group of lesions characterized by stereotyped choreoathetosis or dystonia induced by specific contingencies, and are divided into two categories, both of which are autosomal; one is paroxysmal movement-induced choreoathetosis, with seizures occurring during the daytime, mostly triggered by sudden movements, with a short duration (<20 seconds), and treated with carbamazepine effectively, and the other is nonmovement-induced paroxysmal dystonia, which also The other type is non-motor-induced paroxysmal dystonia, which also occurs upon awakening, and is most often triggered by fatigue, alcohol, coffee, cold, or agitation. The attacks often last for several hours and are not treated with phenytoin sodium or phenobarbital. Treatment Nocturnal frontal lobe epilepsy of autosomal dominant inheritance can be effectively controlled by monotherapy with carbamazepine or phenytoin sodium. Severe cases require treatment with more than one antiepileptic drug, but valproic acid is generally ineffective. Treatment into adulthood is associated with the possibility of recurrence after discontinuation of medication, although seizures have been controlled for a long period of time. The main features of ADNFLE are consistent with the definition of benign partial epilepsy, except that seizures tend to persist into adulthood and require long-term medication, which is inconsistent with most benign partial epilepsies in children.