. 2021 May 12;62(7):e98–e102. doi: 10.1111/epi.16923

TABLE 1.

Patient characteristics

Patient	Age at diagnosis	CDKL5 pathogenic Variant	Prior ASM^a	ASM at FFA initiation^b	Predominant seizure type, BL (seizure history)^c	CGI	QOLCE		PedsQL
Patient	Age at diagnosis	CDKL5 pathogenic Variant	Prior ASM^a	ASM at FFA initiation^b	Predominant seizure type, BL (seizure history)^c	Post FFA	BL	Post FFA	BL	Post FFA
1	2 months	p.Glu449LeufsX38	Levetiracetam, oxcarbazepine, pentobarbital, topiramate	Diazepam, valproate	TC (AA, MC)	Much Impr	Fair	Very Good	200	425
2	3 months	p.Arg550Ter	Ataluren, cannabidiol, clobazam, levetiracetam, lamotrigine, lorcaserin, valproate	Diazepam, midazolam, perampanel, quetiapine, zonisamide	A (MC, TC)	Slt Worse	Very Good	Fair	500	450
3	6 weeks	p.Glu416ValfsX2	Clonazepam, levetiracetam, topiramate	Clobazam, diazepam, valproate, vigabatrin	TC (A, ES)	Much Impr	Good	Good	650	1100
4	6 weeks	p.His127Tyr	Acetazolamide, cannabidiol, ezogabine, felbamate, lorcaserin, lacosamide, phenytoin, prednisone, primidone, rufinamide, topiramate, vigabatrin	Clonazepam, diazepam, midazolam, valproate	T (A, MC, TC, TA)	Much Impr	Fair	Very Good	450	800
5	5 weeks	p.Arg558ThrfsTer9	Clonazepam, lacosamide, levetiracetam, lorazepam, phenobarbital, phenytoin, vigabatrin	Cannabidiol, clobazam, midazolam, perampanel, valproate	T (A, AA, TA, FWMC, MC, TC)	Slt Impr	Poor	Fair	NC	NC
6	7 months	Xp22.2p22.13	Brivaracetam, clobazam, felbamate, lacosamide, levetiracetam, perampanel, prednisone, topiramate, valproate	Lorazepam, cannabidiol, clonazepam, midazolam	TC (AA)	Much Impr	Poor	Fair	NC	NC
Median (range)	7 (5‐28)	—	6.5 (5‐12)	3.5 (2‐5)	3 (2‐7)	—	—	—	—	—

Seizure types: A, atonic; AA, atypical absence; ES, epileptic spasm; FWMC, focal impaired with motor components; MC, myoclonic; T, tonic; TA, typical absence; TC, tonic‐clonic.

Abbreviations: ASM, antiseizure medication; BL, baseline; CGI, clinical global impression; FFA, fenfluramine; Impr, improved; N/A, not applicable; NC, not calculated (N/A listed too many times); QOLCE, Quality of Life Childhood Epilepsy; Slt, slightly; VNS, vagus nerve stimulation.

^{^a}

Patient 2 was also on ketogenic diet as a prior ASM.

^{^b}

Patient 1 was also on ketogenic diet at FFA initiation; Patient 4 was also on VNS at FFA initiation.

^{^c}

Nonseizure symptoms at baseline in ≥2 patients: constipation (n = 4), hypotonia (n = 3), sleep issues (n = 3), tiredness (n = 3), unsteadiness (n = 3), headaches (n = 2), kidney stones (n = 2), and scoliosis (n = 2).