Table 2 Clinical characteristics, management, and outcome of patients treated with fenfluramine for status epilepticus
Data | Adult with LGS, current report | Adult with DS, Millet et al., 2021 | Child with DS, Specchio et al., 2020 | Child with DS, Trowbridge et al., 2021 |
|---|---|---|---|---|
Age (y) | 30 | 20 | 8 | 5 |
Sex | Female | Female | Male | Male |
mRS before admission | 3 | 4 | Not reported | Not reported |
Awareness at admission | Stuporous | Stuporous | Stuporous | Not reported |
Diagnosis/etiology | Lennox–Gastaut syndrome, structural etiology | Dravet syndrome, SCN1A frameshift mutation (two base-pair [AT] insertion at 3725–3726) | Dravet syndrome, c.664 C > T (p.R222X) SCN1A variant | Dravet syndrome, c.4162G > T (p.Glu1388Ter) SCN1A variant |
SE semiology | NCSE with frequent tonic seizures | Tonic SE | NCSE with frequent tonic and bilateral tonic–clonic seizures | Recurrent, frequent SE (both NSCE and CSE); FFA start in interval |
STESS score | 3 | 3 | 3 | Not reported |
ASM therapy before admission | LCM 350 mg/d BRV 100 mg/d CZP 0.75 mg/d | LEV 2,500 mg/d VPA 2,000 mg/d CLB 20 mg/d KD | VPA 28.3 mg/kg/d CLB 0.6 mg/kg bw/d | LEV, CZP, CLB, VPA, KD |
Therapy with maximal dosage of ASMs/24 h *co-administered with FFA | 1.) LZP 4 mg/d 2.) VPA 1,500 mg/d 2.) LCM 600 mg/d 2.) BRV 300 mg/d 3.) CLB 15 mg/d 4.) FFA 26 mg/d (0.58 mg/kg bw) | 1.) LZP 4 mg/d 1.) Propofol 100 µg/kg/min 1.) VPA 9,000 mg/d 1.) CLB 30 mg/d 1.) LEV 6,000 mg/d 1.) VNS; KD 2.) MDZ 30 mg/hr 3.) Pentobarbital 2.5 mg/kg/hr 4.) CBD 20 mg/kg bw/d 5.) Felbamate 1,800 mg/d 6.) Ketamine 6 mg/kg/hr 7.) PB 200 mg/d 8.) BRV 300 mg/d 9.) FFA 0.7 mg/kg bw/d | Unclear; VPA and CLB FFA 26 mg/d* (0.52 mg/kg bw/d*) | FFA 0.7 mg/kg bw/d VPA LEV CLB KD |
Duration of SE before FFA | 16 d | 36 d (0.4 mg/kg bw/d) 42 d (0.7 mg/kg bw/d) | Unclear | No further episode of SE since initiation of FFA |
Time to EEG resolution after FFA | 4 d | 7d (post 0.7 mg/kg/d) | 4 d | Not reported |
Concurrent ASMs with FFA | BRV; CLB; LCM, VPA | BRV; VPA; CLB; PB; ketamine; KD, VNS | Not reported | LEV, VPA, CLB, FFA |
Ventilation time | 0 | 55 d | Not reported | Not applicable |
Total length of stay | 37 d | 56 d | Not reported | Not applicable |
mRS score at discharge | 4 | 4 | Not reported | Not applicable |
Disposition | Home | Rehabilitation facility | Not reported | Not applicable |
Follow-up | 2 months | 2 years 3 months | 1 month | 2 years 10 months |
mRS at last follow-up | 3 | Not reported | Not reported | Not reported |
ASMs at follow-up | FFA 17.6 mg/d, BRV 200 mg/d, LCM 400 mg/d | FFA 0.7 mg/kg bw/d; VPA 4,000 mg/d; BRV 200 mg/d; PB 194.4 mg/d; CLB 40 mg/d; VNS; KD | FFA 0.6 mg/kg bw/d; VPA; CLB | LEV, VPA, CLB, FFA |