Successful treatment of epilepsia partialis continua due to Rassmussen encephalitis with perampanel

Göde, Katja; Großmann, Annette; Rösche, Johannes

doi:10.1515/joepi-2016-0003

Cited by 6 publications

(6 citation statements)

References 10 publications

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“…Perampanel has been effective in treating epilepsia partialis continua and seizures due to Rasmussen encephalitis . The patient had a history of temporal lobe resection and perioperative intracerebral hemorrhage, and had been treated with various combination therapies, usually of at least 4 AEDs at any 1 time.…”

Section: Perampanel In Clinical Practicementioning

confidence: 99%

Perampanel: Does it have broad‐spectrum potential?

Potschka

Trinka

2018

Epilepsia

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This article reviews the profile of perampanel, a novel noncompetitive α-amino-3-hydroxyl-5-methyl-4-isoxazole-propionate (AMPA) receptor antagonist, and its role as a potential broad-spectrum antiepileptic drug in the treatment of epilepsy. For this narrative review, data were collected using specified search criteria. Articles reporting the evidence for perampanel's efficacy from preclinical models, phase 3 clinical studies, observational studies, and descriptive evidence were included. AMPA receptors play a key role in mediating the action of glutamate at the excitatory synapse. Preclinical research showed the AMPA receptor blockade to constitute a promising target for antiepileptic drug therapy. In animal models, perampanel proved to be protective against seizures and reduce seizure severity and duration. Four phase-3 randomized controlled trials (3 in patients with focal seizures and one in primary generalized tonic-clonic seizures in idiopathic generalized epilepsy) have been completed. In focal (partial) onset seizures, perampanel (4, 8, and 12 mg) significantly reduced seizure frequency per 28 days (23.3%-28.8% vs 12.8%; P < .01) and responder rates (≥50% reduction in seizures) (28.5%-35.3% vs 19.3%; P < .05) compared with placebo. In primary generalized tonic-clonic seizures, perampanel 8 mg resulted in greater reduction in seizure frequency per 28 days (-76.5% vs -38.4%; P < .0001) and responder rate (64.2% vs 39.5%; P = .0019) than placebo. The efficacy, safety, and tolerability of perampanel have been reproduced in real-world clinical practice, and the agent has been shown to be effective in other epilepsy syndromes. Perampanel is a potentially broad-spectrum antiepileptic drug with a novel mechanism of action that may be a useful addition for patients with epilepsy with various seizure types. The availability of novel antiepileptic drugs for epilepsy treatment enables more individualized treatment for these patients.

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Section: Perampanel In Clinical Practicementioning

confidence: 99%

Perampanel: Does it have broad‐spectrum potential?

Potschka

Trinka

2018

Epilepsia

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“…However, perampanel has been reported effective against refractory Rasmussen encephalitis and Rasmussen-like encephalitis, which are other conditions in which autoantibodies such as anti-AMPAR antibody and anti-NMDAR antibody are detected, though it is uncertain whether its effects are primary or secondary. [16–18] From the above evidence, a possible explanation for the effectiveness of perampanel here is that anti-NMDAR antibody produced due to the primary anti-NMDAR encephalitis cross-linked with NMDAR in the postsynaptic membrane. This caused internalization of NMDAR, and consequently a decrease in the number of receptors as well as an increase in AMPAR.…”

Section: Discussionmentioning

confidence: 93%

Efficacy of perampanel for anti-N-methyl-D-aspartate receptor encephalitis

Akiyama

Sasaki²,

Hasegawa³

2019

Medicine

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Rationale:We report this 1st case because perampanel may be effective against the seizures and abnormal behavior that occur in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Patient concerns:The patient was a healthy 26-year-old woman who suddenly developed seizures and exhibited abnormal behavior.Diagnoses:NMDAR encephalitis was diagnosed based on positive NMDAR antibody on cerebrospinal fluid analysis.Interventions:Treatment with anticonvulsants and sedatives was started immediately, along with steroid pulse therapy and plasmapheresis, but these measures did not adequately control the repeated seizures and abnormal behavior. However, with the addition of oral perampanel, an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, the seizures and abnormal behavior promptly disappeared.Outcomes:The patient was transferred to the rehabilitation hospital and returned to her job.Lessons:It appears that perampanel rapidly eliminated these clinical features by inhibiting inflow of abnormal glutamic acid and attenuating nerve hyperexcitability by acting as a selective and noncompetitive antagonist of AMPA receptors that had increased in the postsynaptic membrane due to anti-NMDAR encephalitis. To the best of our knowledge, there are no other reports showing that perampanel was effective against anti-NMDAR encephalitis. This case suggests that perampanel may be effective against the seizures and abnormal behavior that occur in anti-NMDAR encephalitis.

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“…To our knowledge, there are as yet only two reports in the literature demonstrating successful treatment of EPC with perampanel in adult patients (table 2). [6,7,11] Focal resection Focal cortical dysplasia [12] Sturge-Weber syndrome [40] Subpial dissection Rasmussen encephalitis [40] Neurostimulation Chronic cortical stimulation Unknown Old ischemic insult [19] rTMS Non-specific [20] tDCS POLG-related mitochondrial disease Autoimmune epilepsy [21] [41] Vagus nerve stimulation Rasmussen encephalitis Chronic inflammatory encephalitis [18] AEDs Perampanel Meningioma [42] Rasmussen encephalitis [43] Levetiracetam Oligodendroglioma [22] ICH [23] Non-specific [10] Topiramate Non-specific [24] Lacosamide Subdural hematoma [25] Primidone Unknown [26] Immunotherapy Immunomodulatory treatments Rasmussen encephalitis [13] Others Botulinum toxin Rasmussen encephalitis [14] Anaplastic astrocytoma [16] Metabolic correction Non-ketotic hyperglycemia [15] [17] AEDs: antiepileptic drugs; ICH: intracerebral hemorrhage; rTMS: transcranial magnetic stimulation; tDCS: transcranial direct current stimulation. In this article, we report the successful treatment of EPC in two pediatric patients, one with NCL and the other with POLG-related mitochondrial disease.…”

Section: Discussionmentioning

confidence: 99%

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

Mir¹,

Almudhry²,

Al-Ghamdi³

et al. 2021

Epileptic Disorders

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Epilepsia partialis continua (EPC) is a form of focal motor status epilepticus, associated with multiple etiologies. Etiology-specific treatments, such as hemispherotomy for Rasmussen encephalitis, lesionectomy for focal cortical dysplasia, and metabolic correction for non-ketotic hyperglycemia, have proven to be efficacious in treating EPC, but, in general, EPC is difficult to treat and often drug-resistant, and there is little evidence to guide therapy. We report the successful treatment of EPC with perampanel in two pediatric patients. The first patient was a 12-yearold boy with neuronal ceroid lipofuscinosis (NCL) who started to have EPC around the age of 10 years, characterized by left hemifacial myoclonic twitches and hemibody jerks that were almost continuous throughout the day and disappeared during sleep. He had failed several antiepileptic drugs (AEDs). The EPC stopped within three days of initiating perampanel. The second patient was a six-year-old boy with POLG-related mitochondrial disease who presented to the emergency room with continuous jerky movements of the right arm and face after a trivial head injury. After failing several AEDs, including a midazolam drip, the EPC was controlled with perampanel. Both patients showed dramatic improvement and continue to show sustained efficacy after around five months of follow-up. Based on our observations, perampanel, which has a unique mechanism of action, appears to be a promising therapeutic option for treating EPC.

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Successful treatment of epilepsia partialis continua due to Rassmussen encephalitis with perampanel

Cited by 6 publications

References 10 publications

Perampanel: Does it have broad‐spectrum potential?

Perampanel: Does it have broad‐spectrum potential?

Efficacy of perampanel for anti-N-methyl-D-aspartate receptor encephalitis

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

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