Drug treatment of infantile spasms

Infantile spasms, also known as West’s syndrome, was first reported by the British physician West in the Lancet in 1841 (Infantilespasms, IS), and is the most common age-dependent epileptic encephalopathy of infancy. There are 20,000 to 50,000 new cases worldwide each year, and the onset is concentrated within the first year of life, with a particular prevalence in the first 4 to 8 months of life. The clinical presentation is characterized by a characteristic cascade of spastic seizures, delayed or regressed psychomotor development, and a triad of peak dysrhythmic changes in the EEG (high amplitude multifocal spikes and slow epileptic waves interspersed with a background of disorganized EEG waves). The disease is a refractory form of epilepsy G, which is difficult to treat and has a poor prognosis. Infantile spasms can be caused by a variety of etiologies, including prenatal, perinatal, and postnatal factors, metabolic diseases, developmental malformations of the brain, neurocutaneous syndromes (mainly tuberous sclerosis), central nervous system infections, and cerebrovascular diseases. In the clinical treatment process, most of them are classified into symptomatic and cryptogenic according to the etiology, head imaging changes and treatment effect, and symptomatic is the main cause. The control rate of cryptogenic is better than symptomatic. The treatment of infantile spasms includes drug combination therapy, ketogenic diet and surgical treatment. Currently, ketogenic diet and surgical treatment are rarely used in clinical practice due to limited experience and technology, and pharmacological treatment is still the first choice for this disease. In this article, we only describe the most commonly used drugs for the treatment of IS, and summarize the treatment mechanisms, common dosing regimens, and common adverse effects of various drugs that are currently used. In recent years, with the development of epileptology and neuropharmacology, the drug treatment for this disease has been significantly improved, but there is still no unified optimal treatment plan worldwide, and there are some differences in the treatment plans in Europe, America, Japan and China. Common treatment options: 1. First-line drugs: China and the United States use adrenocorticotropic hormone (ACTH) as the first-line drug for infantile spasms, Japan uses zonisamide as the first-line drug, and Europe uses aminoglutethimide as the first-line drug; 2. Combination drugs: topiramate, valproic acid, chloronitrostim, nitrostim; 3. Other drugs added to the treatment: levetiracetam, gangliosides, high-dose vitamin B6, high-dose gammaglobulin, lamotrigine, etc. During the course of drug treatment, the program should be adjusted according to the spasm control, EEG monitoring and drug side effects.