Symptomatic West Syndrome, also known as symptomatic infantile spasms, is a severe neurological disorder that affects infants between the age of three and twelve months. It is characterized by a specific type of epileptic seizure called infantile spasms, which are characterized by sudden flexion or extension of the limbs, or the sudden bending forward from the waist. These spasms often occur in clusters and can result in developmental regression or delay.
The term "symptomatic" implies that the syndrome is not a standalone condition but rather a manifestation of an underlying brain abnormality or injury, such as structural abnormalities, genetic disorders, metabolic disorders, brain infections, or brain injuries. These underlying causes are diverse and can vary from individual to individual.
Symptomatic West Syndrome typically exhibits a range of symptoms in addition to the distinctive spasms, including poor feeding habits, delayed motor development, lack of social reciprocity, irritability, and regression in acquired skills. Electroencephalogram (EEG) testing is often used to confirm the diagnosis, as it shows a specific pattern of abnormal brain wave activity called hypsarrhythmia.
Treatment for symptomatic West Syndrome focuses on managing the seizures and addressing the underlying cause. Antiepileptic medications, such as adrenocorticotropic hormone (ACTH) or oral corticosteroids, are commonly prescribed to help control the spasms and improve the child's developmental outcomes. Additionally, early intervention therapies, including physical, occupational, and speech therapy, are often recommended to support the child's overall development.
The prognosis for symptomatic West Syndrome varies depending on the underlying cause, with some cases experiencing significant developmental delays or intellectual disabilities, and others showing improvement with timely intervention and appropriate treatment.
