Ohtahara syndrome, also known as Ohtahara syndrome, was first reported by the Japanese scholar Ohtahara in 1977. Prior to that, the syndrome was mostly diagnosed as infantile spasms, but the clinical and EEG features and prognosis of Ohtahara syndrome are indeed different from those of infantile spasms, so the new classification of epilepsy syndromes in 2001 includes it as a separate syndrome. The majority have severe congenital or perinatal brain injury, and neuroimaging often reveals relatively large structural abnormalities, such as significant congenital brain developmental abnormalities, Acardi syndrome, cerebral penetrance malformations, and Leighs encephalopathy. A small number are of cryptogenic etiology. Since burst a suppression pattern is also seen in normal preterm infants and during deep anesthesia, it is speculated that the pathophysiological alterations of this disease may be related to diffuse or multifocal brain injury, especially gray matter lesions causing abnormal formation and connectivity of neuronal loops. Clinical symptoms: Otawara syndrome has the following features: very early onset, in the first months of life, the main type of convulsion is tonic-spasticity, it often presents in clusters rather than episodically, and partial motor seizures can occur. Fulminant-inhibitory EEG is consistently observed during this period both awake and asleep. despite multiple etiologies, neuroimaging usually reveals gross structural abnormalities caused by prenatal brain dysplasia in affected children. The prognosis is poor and includes early death or significant psychomotor impairment and refractory convulsions. Some cases may progress to West syndrome and further to Lennox-Gastaut syndrome. The disease develops in the neonatal period and early infancy, with more than half of the children having an onset within 1 month of age. The main type of convulsions is tonic spasm with or without clusters, which can be single or continuous, often manifested as an “eprosthotonic” posture, with extreme head bowing, foot reaching forward, body tensing, stopping for about 10s and reappearing at intervals of 9-15s. -The episodes are extremely frequent and can occur 10-20 times a day, with tens of jerks each time. Most cases occur not only in the waking state but also during sleep. In addition to tonic spasms, partial convulsions are observed in some cases, but myoclonic seizures are rarely seen. The progression of the disease type is clearly characterized: from Otahara syndrome to West syndrome (at 3-6 months of age in many cases in mid-infancy) and from West syndrome to Lennox-Gastnat syndrome. Otawara syndrome type 1 shows evolution from a continuous burst-inhibitory pattern to a peak rhythm disturbance that can then change to widespread slow spiking waves with a poor prognosis. type 2 evolves from burst-inhibitory waves to focal spiking waves with a slightly better prognosis than type 1. Diagnosis and differential diagnosis: Otawara syndrome, an early infantile epileptic encephalopathy with inhibitory burst wave EEG changes, is a type of age-dependent epileptic encephalopathy, which is characterized by onset within 3 months of age, especially in the neonatal period, and children with asymmetric congenital structural brain abnormalities. The diagnostic criteria are as follows: (1) age of onset is newborn and small infant; (2) frequent and uncontrollable tonic and/or tonic spastic seizures; (3) EEG in the form of burst-inhibitory; (4) severe psychomotor disorder; (5) multiple etiologies; and (6) can be transformed into infantile spasms. Among them, ①-④ are the necessary conditions for diagnosis; ⑤ and ⑥ are the reference conditions. Treatment and prognosis: The treatment of Otawara syndrome is the same as that of infantile spasms. Most respond poorly to medication and seizures are difficult to control. The prognosis is worse than that of infantile spasms, with some children dying in infancy. Most survivors evolve clinical and EEG features of infantile spasms at 3 to 6 months of age with severe mental retardation, cerebral palsy, and other neurological problems.