Table 2 Clinical features of patients with congenital long QT syndrome comorbiding with epilepsy
References | Patient no. | Age | Sex | Family history | Seizure classification | Age at seizure onset | ASMs | EEGs | ECGs | CT/MRI | Genetic testing | Follow-up |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
Anderson JH, et al. [20] | 1 | 16 | F | SUDEP | Intractable partial epilepsy | 14 | OxcarbazepineLamotrigine Zonisamide Levetiracetam | Right temporal epileptiform discharges | Abnormal T-wave | Abnormal right mesial hippocampus | KCNH2: Ala 57 Pro (p. A57P) | Seizure free after surgery Lamotrigine alone; β-blocker therapy |
Kazuhiro T, et al. [21] | 1 | 10 | F | No | Focal secondary GTCS | 10 | valproic Carbamazepine | Spikes in the posterior lobe | QT prolongation | Normal | SCN5A: E1784K | Seizure-free; mexiletine hydrochloride Placed ILR |
González A, et al. [22] | 1 (proband) | 11 | F | Epilepsy cardiac arrhythmia and | Focal epilepsy | 10 | Lamotrigine | Spike waves of left temporal/ frontal/ occipital region | QT prolongation | Normal | KCNQ1; p.R192Cfs ∗91 | Seizure-free for 3 years |
1 (proband’s father) | 45 | M | SUDEP | Focal secondary GTCS | 13 | Valproate Carbamazepine | Focal slow wave in frontotemporal regions | QT prolongation | Normal | KCNQ1: Q530X | Seizure-free for 15 years | |
Zhou Y, et al. [23] | 1 (proband) | 25 | F | Epilepsy cardiac arrhythmia and SUDEP | GTCS | 7 | Levetiracetam, Valproate Clonazepam | Interictal: left-temporal spikes Ictal: low-wave sharp waves and spikes, mingled with EMG artifacts | QT prolongation | Normal | KCNH2: Arg 744* | Seizure-free for 3 years |
1 (proband’s ma ther) | 50 | F | GTCS | 18 | Oxcarbazepine Valproate Carbamazepine | NA | QT prolongation | NA | KCNH2: Arg 744* | Seizure-free Palpitations and dizziness occasionally occurred | ||
Partemi S, et al. [24] | 1 (proband) | 18 | F | Epilepsy and SUDEP | GTCS | 13 | Levetiracetam | Generalized | QT prolongation | Normal | KCNH2: | SUDEP |
1 (proband’sister) | 18 | F | GTCS | 15 | Levetiracetam | spike-wave and spike-slow wave complexes | Abnormal T-wave | c.246T > C | Seizure-free for 2 years; ICD | |||
Bagnall RD, et al. [25] | 11 | NA | NA | Epilepsy and cardiac arrhythmia and SUDEP | Mesial temporal lobe epilepsy; Dravet syndrome; JME; absence seizures GTCS | 10.3 ± 8.2 (0–34) | NA | Five was +, six were NA | QT prolongation | Hippocampal sclerosis (1) focal cortical dysplasia (1) normal (3) NA (6) | KCNH2 (3) KCNQ1(1) SCN2A (2) ANK2(3) AKAP9(2) | SUDEP |
Johnson JN, et al. [26] | 98/343 | NA | NA | Epilepsy and cardiac arrhythmia and SUDEP | NA | 27 ± 18 | NA | NA | QT prolongation | NA | KCNQ1 KCNH2 SCN5A | NA |
Zarroli K, et al. [27] | 1 | 26 | M | Epilepsy and cardiac arrhythmia and SUDEP | Focal secondary GTCS | NA | levetiracetam | C4 sharp-wave | QT prolongation bradycardia | Prominence in right temporal ventricular horn | KCNH2 c.2775dupG (P926AfsX14) | Seizure-free ICD |
Tu E, et al. [28] | 17 | NA | NA | NA | Frequent seizure | NA | One or two types | NA | LQTS | NA | KCNQ1(2) KCNH2(5) SCN5A (10) | SUDEPs |
Anderson JH, et al. [29] | 10 | NA | NA | NA | Seizures/seizure-like episodes | NA | NA | Generalized or mutifocal sharp waves | LQTS | Normal | KCNQ1(2) KCNH2(7) SCN5A (1) | NA |