RT Journal Article
T1 [Cryptogenic West syndrome: Clinical profile, response to treatment and prognostic factors].
T2 Síndrome de West criptogénico: perfil clínico, respuesta al tratamiento y factores pronósticos.
A1 Calderón Romero, María
A1 Arce Portillo, Elena
A1 López Lobato, Mercedes
A1 Muñoz Cabello, Beatriz
A1 Blanco Martínez, Bárbara
A1 Madruga Garrido, Marcos
A1 Alonso Luego, Olga
K1 Aetiology
K1 Criptogénico
K1 Cryptogenic
K1 Espasmos infantiles
K1 Etiología
K1 Factores pronósticos
K1 Infantile spasms
K1 Prognostic factors
K1 Síndrome de West
K1 Vigabatrin
K1 Vigabatrina
K1 West syndrome
AB West 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.
YR 2017
FD 2017-12-06
LK http://hdl.handle.net/10668/11890
UL http://hdl.handle.net/10668/11890
LA es
DS RISalud
RD Apr 18, 2025