TY - JOUR T1 - Cryptogenic West syndrome: Clinical profile, response to treatment and prognostic factors JO - Anales de Pediatría (English Edition) T2 - AU - Calderón Romero,María AU - Arce Portillo,Elena AU - López Lobato,Mercedes AU - Muñoz Cabello,Beatriz AU - Blanco Martínez,Bárbara AU - Madruga Garrido,Marcos AU - Alonso Luego,Olga SN - 23412879 M3 - 10.1016/j.anpede.2017.10.006 DO - 10.1016/j.anpede.2017.10.006 UR - https://www.analesdepediatria.org/en-cryptogenic-west-syndrome-clinical-profile-articulo-S2341287918301352 AB - IntroductionWest syndrome (WS) is an age-dependent epileptic encephalopathy in which the prognosis varies according to the, not always identified, underlying origin. ObjectivesTo define the profile of cryptogenic (a least studied isolated sub-group) WS, in Spain. To study its outcome, response to different treatments, and to establish prognostic factors. Patients and methodsThe study included a review of the medical records of 16 patients diagnosed with cryptogenic WS during the period, 2000–2015. The mean follow-up time was 6.6 years, with a minimum of 2 years. ResultsThe large majority (11/16) were male. The mean age at onset was 6 months, and 6/16 had a family history of idiopathic epilepsy. The first line treatment with vigabatrin had an electrical–clinical response in 5/16 patients, with the remaining cases responding to adrenocorticotropic hormone (ACTH). Almost half (44%) of the patients progressed to other types of epilepsy, with no difference between those treated with vigabatrin or ACTH. A greater number of adverse effects were obtained with ACTH, with no retinal involvement being observed with vigabatrin. The aetiological cause was found in 2/16. Being female, late onset, and early control of the hypsarrhythmia, were factors of a good prognosis. ConclusionsThe overall prognosis of cryptogenic WS was more serious than expected. Although the incidence of Lennox–Gastaut syndrome was low, the progression to focal epilepsy was the most common, with it appearing within the first 2 years of the diagnosis. The initial response to vigabatrin was lower than expected, but the long-term result was comparable to ACTH. ER -