title: Intravenous immunoglobulin therapy is rarely effective as the initial treatment in West syndrome: A retrospective study of 70 patients.
abstract: OBJECTIVE
  To evaluate factors influencing the efficacy and safety of intravenous immunoglobulins (IVIG) therapy for West syndrome.
  
  
  METHODS
  We investigated seizure outcomes in 70 patients who received IVIG treatment for West Syndrome during the first 3months after the onset of epileptic spasms. IVIG was administered for 3 consecutive days (initial IVIG treatment) at dosages ranging from 100 to 500mg/kg/day. If spasms disappeared within 2weeks of the initial treatment, maintenance IVIG treatment was commenced. We evaluated seizure outcomes at 2weeks (initial evaluation), at 2years (long-term evaluation), and the last visit (last follow-up evaluation) after the initial IVIG treatment. We analyzed dosages of IVIG, age at onset of spasms, treatment lag, and etiologies between responders and non-responders.
  
  
  RESULTS
  Among the patients, 7/70 (10.0%) had cessation of spasms and resolution of hypsarrhythmia at the initial evaluation. Another 6/70 patients (8.6%) were found to have cessation of spasms at the long-term evaluations. The treatment lag in responders was shorter than that in non-responders (P<0.01). There were no significant differences between responders and non-responders in IVIG dosages, age at onset of spasms, and etiologies. Two patients had relapse of partial seizures after cessation of spasms at the last follow-up evaluation. Adverse effects occurred in 2/70 patients.
  
  
  CONCLUSIONS
  The efficacy of IVIG was so low that it should not be considered as first-line treatment in West syndrome. IVIG therapy has a good safety profile and we would recommend it for West syndrome cases with drug resistance, severe complications associated with profound brain damage, severe brain atrophy, and in immunocompromised patients.
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, 2100, Magome, Iwatsuki-ku, Saitama-city, Saitama, 339-8551, Japan; Department of Pediatrics, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan. Electronic address: matsuura.ryuki@pref.saitama.lg.jp.
authors.forename: Ryuki
authors.initials: R
authors.lastname: Matsuura
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, 2100, Magome, Iwatsuki-ku, Saitama-city, Saitama, 339-8551, Japan. Electronic address: hamano.shinichiro@pref.saitama.lg.jp.
authors.forename: Shin-Ichiro
authors.initials: S
authors.lastname: Hamano
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, 2100, Magome, Iwatsuki-ku, Saitama-city, Saitama, 339-8551, Japan; Department of Pediatrics, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan. Electronic address: hirahira.yuko.0621@gmail.com.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hirata
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, 2100, Magome, Iwatsuki-ku, Saitama-city, Saitama, 339-8551, Japan; Department of Pediatrics, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan. Electronic address: sisotti@nifty.com.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Oba
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, 2100, Magome, Iwatsuki-ku, Saitama-city, Saitama, 339-8551, Japan; Department of Pediatrics, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan. Electronic address: kotoko.s@jikei.ac.jp.
authors.forename: Kotoko
authors.initials: K
authors.lastname: Suzuki
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, 2100, Magome, Iwatsuki-ku, Saitama-city, Saitama, 339-8551, Japan; Department of Pediatrics, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan. Electronic address: kenjiro-k@jikei.ac.jp.
authors.forename: Kenjiro
authors.initials: K
authors.lastname: Kikuchi
mesh_terms: D000927:Anticonvulsants / Q000009:adverse effects / Q000627:therapeutic use*; D001921:Brain / Q000000981:diagnostic imaging / Q000187:drug effects / Q000503:physiopathology; D002675:Child, Preschool; D000069279:Drug Resistant Epilepsy / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000009:adverse effects / Q000627:therapeutic use*; D007231:Infant, Newborn; D008297:Male; D019233:Retreatment; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D017211:Treatment Failure
chemical_list: D000927:Anticonvulsants; D016756:Immunoglobulins, Intravenous
keywords: Intravenous immunoglobulin therapy; West syndrome; adverse effect; hypsarrhythmia; outcome; spasms