Outcome of West Syndrome: A Critical Review
| Autor: | Deepa Gautam, Sudhir Adhikari |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Pediatrics
medicine.medical_specialty business.industry Seizure types West Syndrome medicine.disease Hypsarrhythmia Vigabatrin Review article 03 medical and health sciences Tuberous sclerosis Epilepsy 0302 clinical medicine 030225 pediatrics Pediatrics Perinatology and Child Health Etiology Medicine Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery medicine.drug |
| Zdroj: | Journal of Pediatric Epilepsy. :001-006 |
| ISSN: | 2146-4588 2146-457X |
| DOI: | 10.1055/s-0040-1708809 |
| Popis: | West syndrome is a genetically heterogeneous electro-clinical syndrome starting in early infancy. Short-term goal of therapy is spasm control and with standard hormonal or vigabatrin treatment, spasms can be controlled in 60 to 80% of patients in 2 weeks to 3 months period. Hormonal treatment with oral steroid is an alternative therapy to injectable adrenocorticotropin hormone, especially in low resource areas. Vigabatrin is preferred in tuberous sclerosis patients. Long-term aim of treatment is sustained remission of seizures and better neurodevelopmental outcome. About 50 to 70% of children are spasm free for prolonged duration, but epilepsy with multiple seizure types including Lennox–Gastaut syndrome is evident in 20 to 40% of children in long-term follow-up. Though hypsarrhythmia is helpful for the diagnosis, prognostic role of the resolution of electroencephalographic abnormalities is still uncertain. Seizures can be controlled in 40 to 60% of the patients, but only 12 to 40% children have normal neurodevelopmental outcome and a third of children are left with severe disabilities. Children with unknown etiology and normal development at spasm onset have better clinical outcome. Young age at onset, nonstandard therapy have less favorable outcome. Surgery is helpful for spasm control in patients with structural lesions and refractory spasms even in the absence of structural lesions. |
| Databáze: | OpenAIRE |
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