Infancy is an age group with a high incidence of epilepsy, mostly of symptomatic or cryptogenic origin, and in 1989 the ILAE classification of epilepsy and epileptic syndromes included only one idiopathic epileptic syndrome with infantile onset, namely benign myoclonic epilepsy of infancy. In the subsequent almost 10 years, there has been further understanding of idiopathic epilepsy with infantile onset, and new cases continue to be reported. Two new benign infantile epilepsy syndromes, familial and nonfamilial benign partial epilepsy in infants, have been defined in the recently proposed new classification scheme for epilepsy syndromes. I. Benign partial epilepsy of infants Benign partial epilepsy of infants was first reported by Fukuyama in 1963. Many authors have since described the seizure symptomatology and EEG characteristics of similar cases, most of which have been reported from Asian countries. 1. Etiology: The disease is idiopathic epilepsy of infancy and may be related to a genetic susceptibility to convulsions. Children often have a family history of benign convulsions, such as febrile convulsions or benign infantile convulsions. No other etiology can be found. Clinical and EEG manifestations: The age of onset is between 3 and 20 months, with most children starting within 1 year of age. There is no difference in the incidence between males and females. The children had normal psychomotor development before the onset of the disease, and there were no organic lesions or neurological abnormalities. Neuroimaging and laboratory tests were normal. The main features of benign infantile convulsions are based on the results of Video-EEG monitoring and follow-up of a group of 12 children reported by Watanabe et al. Seizures can occur during wakefulness or sleep. Convulsive symptoms include isotropic deviation of both eyes, turning of the head or trunk to one side, and mild clonic movements involving the eye muscles, face, and limbs. There may be a mild increase in muscle tone. Crying during sleep seizures is often the initial manifestation, followed by mild convulsive symptoms. Half of the patients have simple automatisms in the later part of the seizure, such as smacking, chewing, salivation, swallowing and other oral feeding automatisms, or purposeless movements of the limbs (somatic automatisms), but there is a lack of complex fine or semi-purposeful automatisms. Seizures last 30 to 200 seconds, often occur in consecutive series, and may be followed by generalized seizures. Seizures occur 1 to 10 times in a day, are continuous for 1 to 3 days, and can recur within a few weeks. No persistent status epilepticus has been reported. The interictal EEG is normal. The EEG during the seizure period can be seen as restricted low-voltage fast waves or repetitive 0 rhythm discharges with increasing amplitude and decreasing frequency, followed by theta or delta waves with interspersed spikes or sharp waves. The initial site of paroxysmal discharges during the seizure period is mostly in the temporal region, with a few seen in the frontal, parietal or occipital regions, which may gradually or rapidly spread to other regions. (2) Benign familial infantile convulsions Vigevane et al. reported a total of 17 families in succession. There is a history of benign infantile convulsions in first or second degree relatives and the mode of inheritance is autosomal dominant. However, the disorder may also be associated with other genetic defects, suggesting genetic heterogeneity. The seizures are characterized by motor arrest, sluggishness, head and eye deflection to one side, increased generalized muscle tone or cyanosis, followed by clonic jerking of one limb, which may spread to bilateral synchronous or asynchronous clonic movements. The EEG is normal during the interictal period. Lateral slow waves and spikes may be recorded in the parieto-occipital region between consecutive series of seizures. The recruitment rhythm of EEG during seizures starts from the central occipital region on one side and spreads to one hemisphere and the whole brain successively. 3. Diagnosis and differential diagnosis: It is difficult to determine the state of consciousness during infantile seizures, making it difficult to clinically identify generalized or partial seizures, simple or complex partial seizures. In most cases, the type of seizure is determined based on EEG recordings or video EEG monitoring during the seizure period. The main basis for diagnosis is recurrent partial seizures over a short period of time, absence of other etiology leading to convulsive seizures, normal interictal EEG, discharges of local origin during seizures, normal psychomotor development before and after onset, easy seizure control, and good long-term prognosis. The disease should be differentiated from convulsive seizures caused by transient metabolic disorders in infancy such as hypocalcemia and hypoglycemia, and related conditions need to be excluded. It should also be differentiated from various non-epileptic events in infants. Treatment and prognosis: Since most children have continuous and recurrent seizures at the onset, and it is difficult to determine the long-term benign prognosis at an early stage, early administration of antiepileptic drugs is generally recommended. Valproic acid, carbamazepine, or phenobarbital may be used orally. The majority of children do not have recurrences after drug administration, but a few have recurrences within a few months, which are controlled with appropriate dose increases. No cases of drug resistance have been reported. The dose was reduced and discontinued after one and a half to two and a half years of administration and no recurrence was observed after discontinuation of the drug. The development was normal after long-term follow-up. Benign myoclonic epilepsy in infants Benign myoclonic epilepsy in infants was first reported by Dravet et al. in 1981. In recent years, another type of benign myoclonic epilepsy with infantile onset, in which seizures are triggered by sound or tactile stimuli, has been reported, called infantile reflex myoclonic epilepsy.