AIM: To analyse the effects of felbamate in refractory infantile spasms/West syndrome. METHOD: We conducted a 10-year retrospective study of infants (including all infants younger than 18mo) treated with felbamate for electroencephalography-recorded epileptic spasms persisting after first-line treatment. RESULTS: In total, 29 infants (17 males, 12 females) were included in the study. Felbamate was initiated at a mean age of 13.8 months (range 4.5-66mo) after sequential administration or combination of vigabatrin and oral steroids; a ketogenic diet was implemented in 23 infants. Eight infants became spasm-free at a mean dose of 34.6mg/kg/day felbamate (range 26-45mg/kg/day). Mean duration of felbamate use was 19 months (range 1-67mo) for the 19 infants whose treatment was terminated. No severe side effects were observed. Reversible neutropenia led to withdrawal of felbamate in six patients. One spasm-free patient demonstrated recurrence when felbamate was withdrawn. INTERPRETATION: N-methyl-d-aspartate receptors with felbamate controlled epileptic spasms in eight infants resistant to first-line treatment should be targeted.
AIM: To analyse the effects of felbamate in refractory infantile spasms/West syndrome. METHOD: We conducted a 10-year retrospective study of infants (including all infants younger than 18mo) treated with felbamate for electroencephalography-recorded epileptic spasms persisting after first-line treatment. RESULTS: In total, 29 infants (17 males, 12 females) were included in the study. Felbamate was initiated at a mean age of 13.8 months (range 4.5-66mo) after sequential administration or combination of vigabatrin and oral steroids; a ketogenic diet was implemented in 23 infants. Eight infants became spasm-free at a mean dose of 34.6mg/kg/day felbamate (range 26-45mg/kg/day). Mean duration of felbamate use was 19 months (range 1-67mo) for the 19 infants whose treatment was terminated. No severe side effects were observed. Reversible neutropenia led to withdrawal of felbamate in six patients. One spasm-free patient demonstrated recurrence when felbamate was withdrawn. INTERPRETATION: N-methyl-d-aspartate receptors with felbamate controlled epileptic spasms in eight infants resistant to first-line treatment should be targeted.
Authors: Bobby G Ng; Erik A Eklund; Sergey A Shiryaev; Yin Y Dong; Mary-Alice Abbott; Carla Asteggiano; Michael J Bamshad; Eileen Barr; Jonathan A Bernstein; Shabeed Chelakkadan; John Christodoulou; Wendy K Chung; Michael A Ciliberto; Janice Cousin; Fiona Gardiner; Suman Ghosh; William D Graf; Stephanie Grunewald; Katherine Hammond; Natalie S Hauser; George E Hoganson; Kimberly M Houck; Jennefer N Kohler; Eva Morava; Austin A Larson; Pengfei Liu; Sujana Madathil; Colleen McCormack; Naomi J L Meeks; Rebecca Miller; Kristin G Monaghan; Deborah A Nickerson; Timothy Blake Palculict; Gabriela Magali Papazoglu; Beth A Pletcher; Ingrid E Scheffer; Andrea Beatriz Schenone; Rhonda E Schnur; Yue Si; Leah J Rowe; Alvaro H Serrano Russi; Rossana Sanchez Russo; Farouq Thabet; Allysa Tuite; María Mercedes Villanueva; Raymond Y Wang; Richard I Webster; Dorcas Wilson; Alice Zalan; Lynne A Wolfe; Jill A Rosenfeld; Lindsay Rhodes; Hudson H Freeze Journal: J Inherit Metab Dis Date: 2020-08-05 Impact factor: 4.982