Possible induction of West syndrome by oxcarbazepine therapy in a patient with complex partial seizures

Veerapandiyan, Aravindhan; Singh, Priyanka; Mikati, Mohamad A.

doi:10.1684/epd.2012.0491

Cited by 10 publications

(6 citation statements)

References 16 publications

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“…Further, until clearer KCNQ2 encephalopathy clinical diagnostic criteria emerge or an established pathogenic variant is detected, concerns that carbamazepine can provoke infantile spasms in susceptible patients is another reason for caution. 33 …”

Section: Discussionmentioning

confidence: 99%

KCNQ2 encephalopathy

Millichap¹,

Park²,

Tsuchida³

et al. 2016

Neurol Genet

206

112

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Objective:To advance the understanding of KCNQ2 encephalopathy genotype–phenotype relationships and to begin to assess the potential of selective KCNQ channel openers as targeted treatments.Methods:We retrospectively studied 23 patients with KCNQ2 encephalopathy, including 11 treated with ezogabine (EZO). We analyzed the genotype–phenotype relationships in these and 70 previously described patients.Results:The mean seizure onset age was 1.8 ± 1.6 (SD) days. Of the 20 EEGs obtained within a week of birth, 11 showed burst suppression. When new seizure types appeared in infancy (15 patients), the most common were epileptic spasms (n = 8). At last follow-up, seizures persisted in 9 patients. Development was delayed in all, severely in 14. The KCNQ2 variants identified introduced amino acid missense changes or, in one instance, a single residue deletion. They were clustered in 4 protein subdomains predicted to poison tetrameric channel functions. EZO use (assessed by the treating physicians and parents) was associated with improvement in seizures and/or development in 3 of the 4 treated before 6 months of age, and 2 of the 7 treated later; no serious side effects were observed.Conclusions:KCNQ2 variants cause neonatal-onset epileptic encephalopathy of widely varying severity. Pathogenic variants in epileptic encephalopathy are clustered in “hot spots” known to be critical for channel activity. For variants causing KCNQ2 channel loss of function, EZO appeared well tolerated and potentially beneficial against refractory seizures when started early. Larger, prospective studies are needed to enable better definition of prognostic categories and more robust testing of novel interventions.Classification of evidence:This study provides Class IV evidence that EZO is effective for refractory seizures in patients with epilepsy due to KCNQ2 encephalopathy.

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Section: Discussionmentioning

confidence: 99%

KCNQ2 encephalopathy

Millichap¹,

Park²,

Tsuchida³

et al. 2016

Neurol Genet

206

112

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“…Epileptic Spasms Without Hypsarrhythmia (ESWH), as for ES in general, are commonly treated with hormonal therapy (Eisermann et al , ; Ishikawa et al , ). OXC and CBZ may induce ES (Mutoh et al , ; Veerapandiyan et al , ). After failure with several AEDs, we administered VGB, also previously reported as a treatment for ESWH, and seizure freedom and cognitive gains were observed.…”

Section: Discussionmentioning

confidence: 99%

“…Epileptic Spasms Without Hypsarrhythmia (ESWH), as for ES in general, are commonly treated with hormonal therapy (Eisermann et al, 2006;Ishikawa et al, 2014). OXC and CBZ may induce ES (Mutoh et al, 1993;Veerapandiyan et al, 2012). After failure L.R.D.…”

Section: Discussionmentioning

confidence: 99%

Epileptic spasms without hypsarrhythmia in infancy and childhood: tonic spasms as a seizure type

Marchi

Seraphim

Corso

et al. 2015

Epileptic Disorders

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Epileptic spasms were defined by the International League Against Epilepsy Task Force on Classification and Terminology in 2001 as a specific seizure type. Epileptic spasms without hypsarrhythmia have been described in some series of patients, occurring either in infancy or childhood. More prolonged epileptic spasms without hypsarrhythmia were previously defined as a different seizure type, and referred to as "tonic spasm seizures". Here, we present a 5-year-old boy who started having epileptic spasms without hypsarrhythmia at 8 months of age, effectively treated with oxcarbazepine. With the withdrawal of medication, epileptic spasms returned. Video-EEG monitoring revealed high-voltage slow waves superimposed by low-voltage fast activity, followed by an electrodecremental phase and a burst of asymmetric fast activity, time-locked to clinical tonic spasm seizures. Brain MRI showed left temporal atrophy with temporal pole grey/white matter junction blurring and ictal PET-CT showed left basal frontal hypermetabolism. Seizures were refractory to several AEDs and vigabatrin was introduced with seizure cessation. Despite efforts to classify epileptic spasms, these are still considered as part of the group of unknown seizure types. In some cases, a focal origin has been suggested, leading to the term "periodic spasms" and "focal spasms". In this case, epileptic spasms without hypsarrhythmia, associated with tonic spasms, may be a variant of focal spasms and might be considered as an epileptic syndrome. [Published with video sequence]A cluster of three tonic spasms, characterized by sudden flexion of the head and proximal muscles, lasting 5, 4.3 and 1 second, respectively. The spasms were accompanied simultaneously by upward deviation of the eyes and oral automatisms at the end.Key words for the video research on www.epilepticdisorders.com

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“…The EEG showed bi‐frontocentral spikes with normal posterior dominant rhythm for age, indicating regional irritation involving the frontal lobes. One may also speculate that the use of a sodium channel blocker (SCB), such as oxcarbazepine (OXC), before the onset of ES (although we are unable to verify the timing of initiation) may have had a role in ES onset (Veerapandiyan et al ., ).…”

Section: Discussionmentioning

confidence: 97%

Epileptic spasms in paediatric post‐traumatic epilepsy at a tertiary referral centre

Park

Chugani

2017

Epileptic Disorders

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Aim. To recognize epileptic spasms (ES) as a seizure type after traumatic brain injury (TBI), accidental or non‐accidental, in infants and children. In the process, we aim to gain some insight into the mechanisms of epileptogenesis in ES. Methods. A retrospective electronic chart review was performed at the Children's Hospital of Michigan from 2002 to 2012. Electronic charts of 321 patients were reviewed for evidence of post‐traumatic epilepsy. Various clinical variables were collected including age at TBI, mechanism of trauma, severity of brain injury, electroencephalography/neuroimaging data, and seizure semiology. Results. Six (12.8%) of the 47 patients diagnosed with post‐traumatic epilepsy (PTE) had ES. Epileptic spasms occurred between two months to two years after TBI. All patients with ES had multiple irritative zones, manifesting as multifocal epileptiform discharges, unilateral or bilateral. Cognitive delay and epileptic encephalopathy were seen in all six patients, five of whom were free of spasms after treatment with vigabatrin or adrenocorticotropic hormone. Conclusion. The risk of PTE is 47/321(14.6%) and the specific risk of ES after TBI is 6/321 (1.8%). The risk of ES appears to be high if the age at which severe TBI occurred was during infancy. Non‐accidental head trauma is a risk factor of epileptic spasms. While posttraumatic epilepsy (not ES) may start 10 years after the head injury, ES starts within two years, according to our small cohort. The pathophysiology of ES is unknown, however, our data support a combination of previously proposed models in which the primary dysfunction is a focal or diffuse cortical abnormality, coupled with its abnormal interaction with the subcortical structures and brainstem at a critical maturation stage.

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Possible induction of West syndrome by oxcarbazepine therapy in a patient with complex partial seizures

Cited by 10 publications

References 16 publications

KCNQ2 encephalopathy

KCNQ2 encephalopathy

Epileptic spasms without hypsarrhythmia in infancy and childhood: tonic spasms as a seizure type

Epileptic spasms in paediatric post‐traumatic epilepsy at a tertiary referral centre

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