Successful treatment of epilepsia partialis continua with perampanel: two pediatric cases

Mir, Ali; Almudhry, Montaha; Al-Ghamdi, Fouad; Bashir, Shahid

doi:10.1684/epd.2021.1262

Cited by 6 publications

(2 citation statements)

References 40 publications

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“…Neuronal ceroid lipofuscinosis, POLG-related mitochondrial disease and Menkes have been reported in EPC cases in Saudi Arabia. 24,25 An increasing literature in the past decade found genetic causes to be associated with EPC. Several genetic mutations have been associated with EPC, including mutations in the KCNA2 and SCN1A genes.…”

Section: Brain Biopsymentioning

confidence: 99%

Epilepsia partialis continua: A review

Muthaffar,

Alyazidi

2024

NSJ

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Section: Brain Biopsymentioning

confidence: 99%

Epilepsia partialis continua: A review

Muthaffar,

Alyazidi

2024

NSJ

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“…These findings suggest that PER may be effective for treating myoclonic seizures and symptomatic myoclonus, and it is possible that its indications could be broadened. PER has also been shown to be beneficial for treating status epilepticus [30] and epilepsia partialis continua [31]. Thus, it appears that PER might be emerging as a new broad-spectrum ASM.…”

Section: Efficacy and Safetymentioning

confidence: 99%

A systematic review of the efficacy of perampanel as treatment for myoclonic seizures and symptomatic myoclonus

Mir¹,

Alghamdi²,

Alotaibi³

et al. 2022

Epileptic Disorders

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Epileptic myoclonus or myoclonic seizures can occur in idiopathic generalized epilepsy (IGE) and progressive myoclonus epilepsy (PME). However, symptomatic myoclonus which is stimulus-sensitive and provoked by movement is typically seen in PME and Lance-Adams syndrome. Symptomatic myoclonus is not always associated with epileptiform discharges on the electroencephalogram. Therapeutic interventions such as anti-seizure medications (ASMs), the ketogenic diet and vagus nerve stimulation are not always effective. There is emerging evidence that perampanel (PER), an a-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, may be effective for the treatment of myoclonic seizures and symptomatic myoclonus. We performed a systematic review of the literature to assess the efficacy of PER as treatment for myoclonic seizures and symptomatic myoclonus. Twenty-seven studies with a total sample size of 260 patients were included. The efficacy of PER was analysed separately for myoclonic seizures and symptomatic myoclonus. In the group with myoclonic seizures, 50% responder, 75% responder and seizure freedom rates were reported as 74.3% (101/ 136), 60.3% (82/136) and 57.4% (78/136), respectively, with a follow-up duration of 6-12 months. However, in one post-hoc analysis of data from patients with IGE, the efficacy of PER as treatment for myoclonic seizures during the double-blind phase showed no significant difference compared to placebo. The efficacy of PER for symptomatic myoclonus was reported in a total of 119 patients. Four studies (n=88 patients) reported the efficacy of PER as a decrease in myoclonus score/scale. In the remaining 31 patients, symptomatic myoclonus resolved in three patients, decreased in 21 patients and seven patients showed no improvement. We also analysed the number of patients who were already on levetiracetam (LEV) or valproic acid (VPA) at the time of PER initiation; these data were available for 153 patients. Of these, 56.8% were on LEV and 75.1% were on VPA when PER was initiated. This systematic review suggests that PER may be effective as treatment for drug-resistant myoclonic seizures and symptomatic myoclonus. It may also be effective in patients who have already failed to respond to LEV and VPA. These findings are preliminary yet encouraging. This study has several limitations, particularly given the scarcity of high-quality randomized controlled trials and marked heterogeneity regarding the type and results of the studies. Hence, the findings of this review should be viewed with considerable reservation.

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Truncal Ataxia and Prolonged Coma in an Exploratory Pediatric Perampanel Ingestion

Dean¹,

Passalacqua

Dolcourt

2021

J. Med. Toxicol.

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Introduction Several overdoses of the antiepileptic drug perampanel have been reported in adults, but very few have been reported in children. We report the case of an observed exploratory ingestion of perampanel in a 2-year-old child that resulted in ataxia and prolonged coma. Case Report A previously healthy 2-year-old female patient presented to the emergency department (ED) 30 minutes after the witnessed ingestion of 30 mg of perampanel (2 mg/kg). Within minutes of ingestion, she displayed ataxia and inability to walk. Upon ED presentation, she had normal vital signs but was minimally responsive with physical stimulation. Naloxone was given without response. She was intubated because of profound central nervous system depression and transferred to a pediatric tertiary care facility. She remained intubated with no pharmacological sedation. Physical exam showed a horizontal nystagmus. Detailed neurologic examination of ataxia and coordination was not possible, and she did not demonstrate hyperreflexia, clonus, or rigidity. Her mental status gradually improved, and she was extubated approximately 72 hours after exposure. After extubation, the patient still exhibited truncal ataxia and did not return to baseline until 96 hours post ingestion. Serum drawn approximately 16 hours after exposure showed 870 ng/mL perampanel (ref < 20 ng/mL). She remained hemodynamically stable throughout her hospital course, despite protracted depressed mental status. Discussion Given the severity of our patient's presentation, pediatric patients showing symptoms of perampanel overdose should be triaged to the ED for evaluation in anticipation of a prolonged clinical course with decreased consciousness and hypoventilation.

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Successful treatment of epilepsia partialis continua with perampanel: two pediatric cases

Cited by 6 publications

References 40 publications

Epilepsia partialis continua: A review

Epilepsia partialis continua: A review

A systematic review of the efficacy of perampanel as treatment for myoclonic seizures and symptomatic myoclonus

Truncal Ataxia and Prolonged Coma in an Exploratory Pediatric Perampanel Ingestion

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