Calderón Romero, MaríaArce Portillo, ElenaLópez Lobato, MercedesMuñoz Cabello, BeatrizBlanco Martínez, BárbaraMadruga Garrido, MarcosAlonso Luego, Olga2023-01-252023-01-252017-12-06http://hdl.handle.net/10668/11890West syndrome (WS) is an age-dependent epileptic encephalopathy in which the prognosis varies according to the, not always identified, underlying origin. To 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. The 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. The 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. The 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.esAttribution-NonCommercial-NoDerivatives 4.0 Internationalhttp://creativecommons.org/licenses/by-nc-nd/4.0/AetiologyCriptogénicoCryptogenicEspasmos infantilesEtiologíaFactores pronósticosInfantile spasmsPrognostic factorsSíndrome de WestVigabatrinVigabatrinaWest syndromeFemaleHumansInfantMalePrognosisRetrospective StudiesSpasms, InfantileTreatment Outcomeresearch article29223473open access10.1016/j.anpedi.2017.10.0122341-2879https://doi.org/10.1016/j.anpedi.2017.10.012