Population | Etiology | Treatment | Add-on therapy | Outcome | |
---|---|---|---|---|---|
Shoemaker et al. [65] | N = 3 term and preterm | N = 1 PAIS N = 2 PHVD | N = 2 PHB and PHE (dose: N/A) N = 1 PHE and OXC (dose: N/A) | N = 3 LEV as 3rd line (dose: N/A) | LEV’s administration resulted in seizure control in all three patients. |
Tanriverdi et al. [66] | N = 1 term | SWS | PHB (20 mg/kg) | PHE as 2nd line (20 mg/kg) LEV as 3rd line (20 mg/kg) | Seizure control was achieved after LEV intravenous infusion. |
Hmaimess et al. [67] | N = 1 Term | KCNT1 mutation | PHB (dose: N/A) | PHT, LTG, CLZ LEV (10–30 mg/kg) | LEV’s introduction resulted in dramatic decrease in seizure activity by the eighth day of treatment. |
Ledet et al. [68] | N = 1 term | LLA | PHB (20 mg/kg) | LEV (40 mg/kg) | The patient was seizure-free on PHB and maintained seizure freedom on LEV that minimally interfered with her other ongoing treatments. |
Li Jiang et al. [69] | N = 9 term | STXBP1mutations | PHB (dose: N/A) | 5 patients did not respond and were tried on several AEDs (TPM, NZP, LEV, VPA, VIT B6, PDN, ACTH, KD) | 44.4% of cases (4/9) in our study showed apparent responses to LEV. |
Dilena et al. [70] | N = 1 term | SCN2A | PHB (dose: N/A) | LEV + PYR as 2nd line PHE as 3rd line (12–18 mg/kg/day) | Seizure freedom was reached on PHE, first, and maintained on oral CBZ. |
Bonhorst et al. [71] | N = 1 term | KCNQ2 | PHB (20 mg/kg) + VIT B6 (30 mg/kg/d) | MDZ c.i. as 2nd line (0.25 mg/kg/h) TPM as 3rd line (2 mg/kg/day) LID (6 mg/kg), then switched to PHE and, later CBZ (dose: N/A) | Seizure freedom was reached on LID; the patient developed methemoglobinemia as side-effect and seizure freedom was maintained with PHE, first, and oral CBZ, later. |
Numis et al. [72] | N = 3 term and late preterm | KCNQ2 encephalopathy | PHB (dose: N/A) | LEV, TPM, VGB, CLZ, KD failed. CBZ was initiated at 3, 4 and 13 months (dose: N/A) | 2/3 patients responded to CBZ and were seizure free at 30 months though developed severe psychomotor delay, quadriplegia, axial hypotonia with appendicular hypertonia, and a tendency to opisthotonos. |
Spagnoli et al. [73] | N = 1 term | EIMFS due to KCNQ2 mutations | PHB (dose N/A) | PYR, LEV, PHE, MDZ, TPM, NTZ CBZ (dose: N/A) | After multiple AEDs failure, seizure ceased after 3 weeks from CBZ’s introduction. Patient was seizure free at nine months. |
Blumkin et al. [74] | N = 1 Term | KNCQ2 | PHB (dose: N/A) | TPM, LEV, VPA, LTG, PYR, folinic acid CBZ (50 mg/kg) | Seizure control was initially achieved with TPM. Seizures reoccurred after 3 weeks and did not respond to several AEDs until CBZ. |
Buttle et al. [75] | N = 1 term | KCNQ2 | PHB (dose: N/A) | LEV, LRZ, CLZ, PYR LID (2–4 mg/kg/h) CBZ (40 mg/kg) | After several AEDs failed, seizure freedom was reached on LID and maintained at a 13 months follow-up on oral CBZ. |
Soldovieri et al. [76] | N = 1 term | KCNQ2 mutation (Kv7.2 subunit) | PHB (dose: N/A) | PYR, LEV, PHE, TPM, OXC | Partial response to an association of PHB, PHE, TPM. At 5 months he was switched to OXC and maintained seizure freedom until 14 months. The patient developed severe DD. |
McNally et al. [77] | N = 1 term | SCN8A | PHB (20 mg/kg) + LEV (20–60 mg/kg) | OXC (up to 80 mg/kg) + PHE (20 mg/kg) + LTG (2 mg/kg/day) | The association of three sodium channel blockers (OXC + PHE + LTG) reduced seizures’ frequency. |
Okumura et al. [78] | N = 1 term | 2q21-q31deletion (SCN1A cluster) | PHB (dose: 20 mg/kg) | LEV as 2nd line (40 mg/kg) VPA as 3rd line (50 mg/kg) | PHB and LEV failed to control seizures; VPA reduced seizures’ frequency. |
Riesgo et al. [79] | N = 3 preterm and term | N = 1 NAS N = 1 fetal distress N = 1 PVL | PHB (dose: N/A) | TPM (0.5–8 mg/kg/d) after several other AEDs failed (PHB, PHE, CLZ, VPA, MDZ) | Seizure cessation in all three after TPM’s administration. |
Sirsi et al. [80] | N = 3 term | N = 1 HIE N = 1 meningitis N = 1 EIEE | PHB (dose: N/A) | PHE as 2nd line (dose: N/A) MDZ as 3rd line (up to 0.2 mcg/kg/h) | Seizure control within 6–72 h after MDZ’s introduction. One patient developed hypotension, that responded to inotropic support. |
Steinberg et al. [81] | N = 2 preterm | N = 1 IVHN = 1 PVL | PHB (20 mg/kg) | PHE as 2nd line (20 mg/kg) Rectal VPA as 3rd line (20–30 mg/kg) | Seizure control was achieved and maintained on a 12 months follow-up on VPA. |
Tarocco et al. [82] | N = 1 late preterm | Pierre-Robin, polymicrogyria, lissencephaly | PHB (dose: N/A) | PHE, MDZ, LEV, PPF KTM (2 mg/kg + c.i. of 10 mcg/kg/min) | Immediate complete clinical and electrographic response was obtained after KTM introduction; after 15 days SE relapsed and the patient died. |
Baxter et al. [83] | N = 3 term | N = 2 EIEE N = 1 Aicardi-Goutieres | PHB (dose: N/A) | PYR, CLZ, VPA VGB (40 mg/kg/d) | 2/3 patients showed full response to VGB. |
Wolf et al. [84] | N = 1 term | Incontinentia pigmenti | PHB (35 mg/kg) | LRZ as 2nd line (0.2 mg/kg) PHE as 3rd line (20 mg/kg) Dexamethasone (0.25 mg/kg/d) | Rapid improvement and clinical seizures termination followed the initiation of CCS therapy. |
Shevell et al. [85] | N = 1 term | BFNE | PHB (10 mg/kg) | / | Patient presented no more seizures, was discharged home on oral PHB, suspended at five months of life |
Lee et al. [86] | N = 1 term | KCNQ2 | PHB (6 mg/kg/day) | PHE as 2nd line (8 mg/kg/day) VGB (50 mg/kg/day) | VGB reduced seizures; Once treatment with Vigabatrin was administered seizures reduced to one per day until day 24 of post-natal life, time at which the last seizure was recorded. |
Sato et al. [87] | N = 2 late preterm and term | HIE | PHB (10 mg/kg) | / | Both patients temporarily controlled seizures on PHB. One relapsed and developed severe DD. |
Sillanpää et al. [88] | N = 1 term | Feeding epilepsy | PHB (60 mg/day) + chlorpromazine (9 mg/day) | The patient was seizure-free since day 14 of PHB. | Only few cases of neonatal feeding seizures are described. In this case the patient was seizure-free on PHB, after a six days combination-therapy with chlorpromazine. |
Tramonte et al. [89] | N = 1 term | Temporal lobe hemorrhage | PHB (dose: N/A) | / | After PHB’s administration no more autonomic seizures (apnea, desaturations) were noticed. |