Short-term nonhormonal and nonsteroid treatment in West syndrome.
OBJECTIVE
West syndrome (WS) is considered an age-dependent epileptic encephalopathy and also a particular type of electrical epileptic status. Short-term hormonal or steroid treatment of WS with good efficacy is reported in the literature. The aim of this retrospective multiinstitutional study was to evaluate the early discontinuation of nonhormonal and nonsteroid treatment for WS.


METHODS
Twenty-two WS cases in which treatment was discontinued after a maximum of 6 months, were collected. Inclusion criteria were the presence of typical EEG hypsarrhythmia (HY) and video-EEG recorded epileptic spasms. Exclusion criteria were the presence of partial seizures or other seizure types before spasm onset. The patients were treated with vigabatrin (VGB) in 19 cases and nitrazepam (NTZ) in three. The dose range was 70-130 mg/kg/day for VGB and 0.7-1.5 mg/kg/day for NTZ. The drug was discontinued if spasms stopped and HY disappeared after a mean treatment period of 5.1 months (range, 3-6 months). All patients underwent repeated and prolonged awake and sleep video-EEG, both before and after drug discontinuation.


RESULTS
Cryptogenic (15) and symptomatic (seven) WS patients were included. All the symptomatic cases had neonatal hypoxic-ischemic encephalopathy. The mean age at spasm onset was 5.5 months (range, 3-7 months; median, 6). The interval between spasm onset and drug administration ranged from 7 to 90 days (mean, 23 days; median, 20). The interval between drug administration and spasm disappearance ranged from 2 to 11 days (mean, 6 days; median, 6 days). The interval between drug administration and HY disappearance ranged from 3 to 30 days (mean, 9 days; median, 10 days). Drugs were stopped progressively over a 30- to 60-day period. Follow-up ranged from 13 to 50 months (mean, 26 months; median, 22 months). None of our cases showed spasm recurrence.


CONCLUSIONS
Our data show that successful nonhormonal and nonsteroid treatment can be shortened to a few months without spasm recurrence in patients with cryptogenic or postanoxic WS.
Capovilla
Giuseppe
G
Department of Child Neuropsychiatry, "C Poma" Hospital, Mantova Department of Neurology, Bambino Gesù Children's Hospital, Rome.
Beccaria
Francesca
F
Montagnini
Alessandra
A
Cusmai
Raffaella
R
Franzoni
Emilio
E
Moscano
Filomena
F
Coppola
Giangennaro
G
Carotenuto
Marco
M
Gobbi
Giuseppe
G
Seri
Stefano
S
Nabbout
Rima
R
Vigevano
Federico
F
Beccaria
Francesco
F
Montagnini
Allesandra
A
Coppola
Giangennero
G
D000927:Anticonvulsants / Q000008:administration & dosage*; D002540:Cerebral Cortex / Q000187:drug effects / Q000503:physiopathology; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004569:Electroencephalography / Q000187:drug effects*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009567:Nitrazepam / Q000008:administration & dosage*; D012189:Retrospective Studies; D055502:Secondary Prevention; D013036:Spasms, Infantile / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D016896:Treatment Outcome; D014741:Video Recording; D020888:Vigabatrin / Q000008:administration & dosage*
D000927:Anticonvulsants; D009567:Nitrazepam; D020888:Vigabatrin