Quinine in the Treatment of Malignant Migrating Partial Seizures in Infancy: A Case Report

The syndrome of malignant migrating partial seizures in infancy was first described by Coppola and colleagues in 1995. The International League Against Epilepsy defines this form of epilepsy as a seizure onset in the first 6 months of life, occurrence of almost continuous migrating polymorphous focal seizures, combined with multifocal ictal EEG discharges, and progressive deterioration of psychomotor development. Most cases are pharmacoresistant and have poor outcomes. A lot of publications described the trial of several medications such as Stiripentol, Rufinamide, Cannabidiol, and finally Ketogenic diet, to control the refractory devastating seizures. We describe a 13-month-old girl with malignant migrating partial seizures in infancy who was started on Quinine for the control of her refractory seizures after the trial of multiple antiepileptic medications that failed to control her seizures, including Clonazepam, Carbamazepine, Phenobarbitone, Phyntion, Midazolam, Valproate, Perampanel & Ketogenic diet, all were tried by different combination at different times. Finally, as malignant migrating partial seizures in infancy are sometimes linked to K channelopathy, a trial of Quinine was given in a dose of 30mg/kg/d. Patients showed an excellent response with control of clinical & electrographic seizures. Now she is seizure-free for five months and undergoing physiotherapy. She started rolling over but doesn't have much improvement in motor milestones, is not following or cooing, and is unable to say clear words. Keywords: MMPSI – malignant migrating partial seizures in infancy- Quinine – Intractable epilepsy- CPLANE-1 gene defect

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Usefulness of ketogenic diet in a girl with migrating partial seizures in infancy

Brain and Development ◽

10.1016/j.braindev.2015.12.012 ◽

2016 ◽

Vol 38 (6) ◽

pp. 601-604 ◽

Cited By ~ 10

Author(s):

Tatsuo Mori ◽

Katsumi Imai ◽

Taikan Oboshi ◽

Yuh Fujiwara ◽

Saoko Takeshita ◽

...

Keyword(s):

Ketogenic Diet ◽

Partial Seizures ◽

Migrating Partial Seizures

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P.036 Targeted molecular therapy with quinidine for seizures in a neonate with KCNT1 mutation leads to poor response

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2017.121 ◽

2017 ◽

Vol 44 (S2) ◽

pp. S22-S23

Author(s):

JM Smith ◽

M Patel ◽

Q Xu ◽

S Uliel-Sibony ◽

M Demos ◽

...

Keyword(s):

Poor Response ◽

Epileptic Encephalopathy ◽

Molecular Therapy ◽

K Channel ◽

Eeg Monitoring ◽

Partial Seizures ◽

Targeted Molecular Therapy ◽

Target Dose ◽

Electrographic Seizures ◽

Migrating Partial Seizures

Background: The KCNT1 gene encodes subunits of the Na+-activated K+ channel, widely expressed in the CNS. Mutations of this gene have been implicated in Malignant Migrating Partial Seizures of Infancy (MMPSI). This early-onset epileptic encephalopathy represents a challenge due to pharmacoresistance. The channel-specific mutation represents the potential for targeted pharmacotherapy. Quinidine is a partial antagonist of the KCNT1 encoded channel; patients with MMPSI have been reported to have responded to doses ranging 34.4/kg/d - 60mg/kg/d. We present a case of MMPSI with a KCNTI mutation (c.G1283A:p.R428Q) trialled on quinidine. Methods: Following ineffective trials of 6 anti-seizure medications, this patient was trialled on oral quinidine. This patient was titrated up to a dose of 52mg/kg/d. Twenty-four hour EEG monitoring prior to quinidine therapy, and at target dose were compared. Results: Prior to initiation of quinidine, this patient experienced 22 electrographic seizures over 24 hours. At target dose, this patient experienced greater than 70 seizures over 24 hours. Conclusions: Quinidine has previously been reported to be effective in patients with MMPSI with the same and different mutations. We report the second case of a patient with MMPSI and KCNT1 mutation R428Q with poor clinical response to quinidine.

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Ketogenic Diet Therapy for Intractable Epilepsy in Infantile Alexander Disease: A Small Case Series and Analyses of Astroglial Chemokines and Proinflammatory Cytokines

Epilepsy Research ◽

10.1016/j.eplepsyres.2020.106519 ◽

2021 ◽

Vol 170 ◽

pp. 106519

Author(s):

Shu Hamada ◽

Takeo Kato ◽

Kengo Kora ◽

Tatsuya Kawaguchi ◽

Tenshin Okubo ◽

...

Keyword(s):

Ketogenic Diet ◽

Proinflammatory Cytokines ◽

Case Series ◽

Intractable Epilepsy ◽

Diet Therapy ◽

Alexander Disease ◽

Small Case Series

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Malignant Migrating Partial Seizures of Infancy (Coppola-Dulac Syndrome)

Epilepsy - Advances in Diagnosis and Therapy ◽

10.5772/intechopen.82838 ◽

2019 ◽

Author(s):

Alexey Kholin

Keyword(s):

Partial Seizures ◽

Migrating Partial Seizures

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Migrating partial seizures in infancy and 47XYY syndrome: Cause or coincidence?

Epilepsy & Behavior Case Reports ◽

10.1016/j.ebcr.2014.02.003 ◽

2014 ◽

Vol 2 ◽

pp. 43-45 ◽

Cited By ~ 4

Author(s):

Rajesh Shankar Iyer ◽

Thanikasalam ◽

Mugundhan Krishnan

Keyword(s):

Partial Seizures ◽

Migrating Partial Seizures

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Predictors of Postoperative Seizure Recurrence: A Longitudinal Study of Temporal and Extratemporal Resections

Epilepsy Research and Treatment ◽

10.1155/2016/7982494 ◽

2016 ◽

Vol 2016 ◽

pp. 1-7 ◽

Cited By ~ 3

Author(s):

Hai Chen ◽

Pradeep N. Modur ◽

Niravkumar Barot ◽

Paul C. Van Ness ◽

Mark A. Agostini ◽

...

Keyword(s):

Univariate Analysis ◽

Intractable Epilepsy ◽

Seizure Recurrence ◽

Term Outcome ◽

Long Term Outcome ◽

Longitudinal Outcome ◽

Ictal Eeg ◽

Multiple Variables ◽

Postoperative Seizure

Objective. We investigated the longitudinal outcome of resective epilepsy surgery to identify the predictors of seizure recurrence. Materials and Methods. We retrospectively analyzed patients who underwent resections for intractable epilepsy over a period of 7 years. Multiple variables were investigated as potential predictors of seizure recurrence. The time to first postoperative seizure was evaluated using survival analysis and univariate analysis at annual intervals. Results. Among 70 patients, 54 (77%) had temporal and 16 (23%) had extratemporal resections. At last follow-up (mean 48 months; range 24–87 months), the outcome was Engel class I in 84% (n=59) of patients. Seizure recurrence followed two patterns: recurrence was “early” (within 2 years) in 82% of patients, of whom 83% continued to have seizures despite optimum medical therapy; recurrence was “late” (after 2 years) in 18%, of whom 25% continued to have seizures subsequently. Among the variables of interest, only resection site and ictal EEG remained as independent predictors of seizure recurrence over the long term (p<0.05). Extratemporal resection and discordance between ictal EEG and resection area were associated with 4.2-fold and 5.6-fold higher risk of seizure recurrence, respectively. Conclusions. Extratemporal epilepsy and uncertainty in ictal EEG localization are independent predictors of unfavorable outcome. Seizure recurrence within two years of surgery indicates poor long-term outcome.

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