Methsuximide for Intractable Childhood Seizures

PEDIATRICS ◽  
1991 ◽  
Vol 87 (2) ◽  
pp. 186-189
Author(s):  
Michael B. Tennison ◽  
Robert S. Greenwood ◽  
Michael V. Miles
Keyword(s):  
Adverse Effects ◽  
Seizure Frequency ◽  
Intractable Epilepsy ◽  
Intractable Seizures

Methsuximide was added to the therapeutic regimens of 25 children with intractable epilepsy. In 15 patients the drug was well tolerated and resulted in a 50% or greater reduction in seizure frequency. No serious or irreversible adverse effects were seen. Methsuximide is frequently overlooked and may be an effective adjunctive antiepileptic for children with intractable seizures.

scholarly journals Patient-assessed satisfaction and outcome after microsurgical resection of cavernomas causing epilepsy

2010 ◽  
Vol 29 (3) ◽  
pp. E16 ◽  
Author(s):  
Jamie J. Van Gompel ◽  
W. Richard Marsh ◽  
Fredric B. Meyer ◽  
Gregory A. Worrell
Keyword(s):  
Seizure Frequency ◽  
Intractable Epilepsy ◽  
Survey Study ◽  
Intractable Seizures ◽  
Select Group ◽  
Age At Surgery ◽  
Survey Results ◽  
Microsurgical Resection

Object Microsurgical resection of supratentorial cavernomas associated with intractable epilepsy is performed frequently. Despite its common occurrence, little is known about patient perceptions of microsurgical resection for cavernomas. This survey study was performed to investigate patient perceived outcome after surgery for cavernomas associated with intractable epilepsy. Methods The authors' surgical database was searched for cavernoma resection performed between 1971 and July of 2006. Of the initial 173 patients identified, 102 met criteria for medically intractable seizures. These 102 patients were then mailed a survey to determine follow-up and patient satisfaction. Thirty-nine surveys were returned as undeliverable, and 30 (48%) of the remaining 63 patients responded. Results The average age at surgery for patients responding to this survey was 40 ± 16 years compared with 35 ± 15 years for all 102 patients. At prolonged follow-up, 87% of patients reported being seizure-free. Of those with seizures, 2 (7%) reported being nearly seizure-free (rare disabling seizures), 2 (7%) believed they had a worthwhile improvement in seizure frequency, and no patient (0%) in this series believed they did not have a worthwhile improvement in seizure frequency. Ninety percent of responders stated they definitely, and 10% probably, would have surgery again. No patient responded that they probably or definitely would not have epilepsy surgery. Mean clinical follow-up was 36 ± 8 months and survey follow-up was 97 ± 13 months for these 30 patients. Use of the mail-in survey increased follow-up length 2.7 times longer compared with clinical follow-up. Conclusions It is clear from this select group of survey responders that patients undergoing surgery for cavernomas associated with medically intractable epilepsy are happy they underwent surgery (100%) and had excellent surgical outcomes (87% seizure-free) at prolonged follow-up of 97 ± 13 months. These survey results support that microsurgical resection for cavernomas is highly effective and significantly improves these patients' quality of life.

scholarly journals Add-on Trial of Clobazam in Intractable Adult Epilepsy with Plasma Level Correlations

1990 ◽  
Vol 17 (3) ◽  
pp. 311-316 ◽  
Author(s):  
A. Guberman ◽  
M. Couture ◽  
K. Blaschuk ◽  
A. Sherwin
Keyword(s):  
Plasma Level ◽  
Adverse Effects ◽  
Seizure Frequency ◽  
Plasma Levels ◽  
Active Metabolite ◽  
Intractable Epilepsy ◽  
Adult Patients ◽  
Toxic Responses

ABSTRACT:Clobazam, a novel benzodiazepine, was used as an add-on agent in 47 adult patients with intractable epilepsy and a variety of seizure types. A >75% reduction in seizure frequency was observed in 42% (18) of the patients and a >50% reduction in 65% (29) of the patients which was sustained over a mean follow-up period of 13.3 (±5.6) months. Nine patients had to discontinue the drug due to minor adverse effects or increased seizures. Possible tolerance developed in 6 patients. Plasma levels of the active metabolite N-desmethylclobazam were linearly related to dosage and appeared to correlate with both therapeutic and toxic responses. Clobazam appears to be a safe and effective add-on antiepileptic for a wide variety of seizure types in intractable epilepsy.

X-Linked Lissencephaly With Abnormal Genitalia as a Tangential Migration Disorder Causing Intractable Epilepsy: Proposal for a New Term, “Interneuronopathy”

2004 ◽  
Vol 19 (3) ◽  
pp. 392-397
Author(s):  
Mitsuhiro Kato ◽  
William B. Dobyns
Keyword(s):  
Intractable Epilepsy ◽  
Brain Magnetic Resonance Imaging ◽  
Homeobox Gene ◽  
Tangential Migration ◽  
Intractable Seizures ◽  
Migration Disorder ◽  
Male Patients ◽  
Paired Class ◽  
Clonic Convulsions ◽  
The Brain

X-linked lissencephaly with abnormal genitalia is the first human disorder in which deficient tangential migration in the brain has been demonstrated. Male patients with X-linked lissencephaly with abnormal genitalia show intractable seizures, especially clonic convulsions or myoclonus from the first day of life, but neither infantile spasms nor hypsarrhythmia on electroencephalograms so far. Brain magnetic resonance imaging shows anterior pachygyria and posterior agyria with a mildly thick cortex, agenesis of the corpus callosum, and dysplastic basal ganglia. ARX, a paired-class homeobox gene with four polyalanine sequences, is a responsible gene for X-linked lissencephaly with abnormal genitalia. The brain of Arx knockout mice shows aberrant tangential migration and differentiation of γ-aminobutyric acid (GABA)ergic interneurons. In human X-linked lissencephaly with abnormal genitalia, a neuropathologic study has suggested a loss of interneurons. Meanwhile, polyalanine expansion of ARX causes symptomatic or nonsymptomatic West's syndrome and nonsyndromic mental retardation. The striking epileptogenicity of X-linked lissencephaly with abnormal genitalia and West's syndrome associated with ARX mutations is considered to be caused by a disorder of interneurons involving a tangential migration disorder. We propose “interneuronopathy” as a term for this. ( J Child Neurol 2005;20:392—397).

Potassium Bromide in the Treatment of Pediatric Refractory Epilepsy

2019 ◽  
Vol 34 (10) ◽  
pp. 582-585 ◽  
Author(s):  
Kazuo Kodama ◽  
Taku Omata ◽  
Yoshimi Watanabe ◽  
Hiromi Aoyama ◽  
Yuzo Tanabe
Keyword(s):  
Adverse Effects ◽  
Seizure Frequency ◽  
Refractory Epilepsy ◽  
Focal Epilepsy ◽  
Dravet Syndrome ◽  
Generalized Seizures ◽  
Clonic Seizures ◽  
Moderate Effect ◽  
Generalized Epilepsy ◽  
Free Status

Objective: We evaluated potassium bromide’s (KBr’s) efficacy and tolerability for pediatric refractory epilepsy. Methods: We retrospectively reviewed the records of 42 patients treated with KBr in our hospital between 2008 and 2016 (age: 4 months to 19 years; mean: 6.2 years). Thirteen of them had 2 seizure types. The treatment durations ranged from 1 month to 6 years (mean: 15.0 months). Results: KBr had an excellent effect (seizure-free status) in 3 patients (7.1%), a moderate effect (>50% reduction in seizure frequency from the pretreatment baseline) in 21 patients (50.0%), and no effect (<50% reduction in seizure frequency from the pretreatment baseline) in 18 patients (42.9%). The effective daily doses ranged from 20 to 80 mg/kg (mean: 50.0 mg/kg). KBr was effective in 59.1% patients with generalized epilepsy (n = 22), 55.6% patients with focal epilepsy (n = 18), and both patients with Dravet syndrome. An excellent or moderate effect was found in 72.2% patients with tonic seizures (n = 18), 66.6% patients with generalized tonic-clonic seizures (n = 6), 75.0% patients with secondary generalized seizures (n = 4), 46.2% patients with focal seizures (n = 13), and 20% patients with infantile spasms (n = 10) but no patients with myoclonic seizures (n = 2). Adverse effects including drowsiness, excitement, and rashes were reported in 13 patients (31.0%). Conclusions: These findings suggest that KBr is particularly effective for tonic seizures, generalized tonic-clonic seizures, and secondary generalized seizures. Although the adverse effects need further attention, KBr should be considered for pediatric refractory epilepsy.

scholarly journals Efficacy of limited hippocampal radiofrequency thermocoagulation for mesial temporal lobe epilepsy

2019 ◽  
Vol 131 (3) ◽  
pp. 781-789 ◽  
Author(s):  
Ching-Yi Lee ◽  
Han-Tao Li ◽  
Tony Wu ◽  
Mei-Yun Cheng ◽  
Siew-Na Lim ◽  
...  
Keyword(s):  
Adverse Effects ◽  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Seizure Frequency ◽  
Seizure Control ◽  
Mesial Temporal Lobe Epilepsy ◽  
Temporary Increase ◽  
Drug Resistant ◽  
Radiofrequency Thermocoagulation ◽  
Mesial Temporal Lobe

OBJECTIVERadiofrequency thermocoagulation (RFTC), which has been developed for drug-resistant epilepsy patients, involves less brain tissue loss due to surgery, fewer surgical adverse effects, and generally good seizure control. This study demonstrates the effectiveness of RFTC performed at limited hippocampal locations.METHODSDaily seizure diaries were prospectively maintained for at least 6 months by 9 patients (ages 30–59 years) with drug-resistant mesial temporal lobe epilepsy (MTLE) before treatment with RFTC. The limited target for stereotactic RFTC was chosen based on intraoperative electroencephalography (EEG) recording and was initially tested with a Radionics electrode at a low temperature, 45°C, for 60 seconds. The therapeutic RFTC heating parameters were 78°C–80°C for 90 seconds. All patients who received the RFTC treatment underwent both MRI and EEG recording immediately postoperatively and at the 3-month follow-up. Monthly outpatient clinic visits were arranged over 6 months to document seizure frequency and severity to clarify the changes noted in imaging studies and EEG patterns.RESULTSTwo patients were excluded from our analysis because one had undergone multiple seizure surgeries and the other had a poor recording of seizure frequency, before the RFTC surgery. Five and two patients underwent left-sided and right-sided RFTC, respectively. None of the patients had generalized tonic-clonic attacks postoperatively, and no adverse effects or complications occurred. According to MRI data, the effect of coagulation was limited to less than 1.0 cm in diameter and perifocal edema was also in limited range. The seizure frequency within 6 months decreased postoperatively with a mean reduction in seizures of 78% (range 36%–100%). Only two patients had a temporary increase in seizure frequency within 2 weeks of the surgery, and over 50% of all patients showed a decrease in average seizure frequency.CONCLUSIONSThe study results confirm that limited RFTC provides a more effective surgery with similar seizure control but fewer complications than resective surgery for drug-resistant MTLE patients.

Nicotine: A Targeted Therapy for Epilepsy Due to nAChR Gene Variants

2020 ◽  
pp. 088307382097485
Author(s):  
Jordana Fox ◽  
Drew M. Thodeson ◽  
Alison M. Dolce
Keyword(s):  
Targeted Therapy ◽  
Autosomal Dominant ◽  
Medical Center ◽  
Nicotine Patch ◽  
Intractable Epilepsy ◽  
Experimental Therapy ◽  
Therapeutic Effects ◽  
Gene Variants ◽  
Nicotine Treatment ◽  
Intractable Seizures

Objective: Genetic variants of the neuronal nicotinic acetylcholine receptor (nAChR) cause autosomal dominant sleep-related hypermotor epilepsy. Approximately 30% of autosomal dominant sleep-related hypermotor epilepsy patients are medically intractable. In preclinical models, pathogenic nAChR variants cause a gain of function mutation with sensitivity to acetylcholine antagonists and agonists. Nicotine modifies the activity of nAChRs and can be used as targeted therapy. Methods: We reviewed next-generation sequencing epilepsy panels from a single laboratory (GeneDx) from patients at Children’s Medical Center Dallas between 2011 and 2015 and identified patients with nAChR variants. Retrospective review of records included variant details, medical history, neuroimaging findings, and treatment history. Results: Twenty-one patients were identified. Four patients were prescribed nicotine patches for intractable seizures. Three of 4 patients had a clinical response, with >50% seizure reduction. Conclusions: Treatment with a nicotine patch can be an effective therapy in epilepsy patients with nAChR gene variants. We propose consideration of transdermal nicotine treatment in intractable epilepsy with known nAChR variants as an experimental therapy. Further clinical trials are needed to fully define therapeutic effects.

scholarly journals P.018 Forced normalization after vagal nerve stimulation in a case of intractable Lennox-Gastaut syndrome

2018 ◽  
Vol 45 (s2) ◽  
pp. S20-S20
Author(s):  
A Denton ◽  
A Vitali ◽  
K Waterhouse ◽  
J Tellez-Zenteno
Keyword(s):  
Vagus Nerve ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Psychiatric Symptoms ◽  
Intractable Epilepsy ◽  
Complex Case ◽  
Drop Attacks ◽  
Intractable Seizures ◽  
Forced Normalization ◽  
Clonic Seizures

Background: Forced normalization is the development of psychiatric symptoms in a patient experiencing remission of intractable seizures. The mechanism of this phenomenon is unknown. We present a complex case of Lennox Gastaut syndrome that experiences forced normalization after vagus nerve stimulation (VNS). Methods: This case details a 31-year-old male with seizures since early childhood. The patient has intractable epilepsy and failed AEDs, VNS, and a partial callosotomy. Results: The patient was in remission from 2-12 years old, when seizures returned at a frequency of 2-5 per day. He has multiple types of seizures including drop attacks, absences, and tonic-clonic seizures. Patient experienced status epilepticus multiple times. Twelve AEDs were failed before VNS was started in 2010, which helped curb the severity of seizures and the potential for clusters. Forced normalization developed over the course of treatment with VNS. The patient behavior was characterized by aggression, paranoia, and hallucinations. VNS was turned off late in 2010 and then re-started in January of 2011. Patient proceeded to cycle between several days of seizures without psychiatric symptoms and several days of psychosis without seizures. Conclusions: Vagus nerve stimulation gave way to forced normalization, characterized here as aggressive behaviour and psychosis. Forced normalization is seen commonly after epilepsy surgery, but rarely following VNS.

P6-1 Effect of vagus nerve stimulation on EEG and seizure frequency in children with intractable epilepsy

2010 ◽  
Vol 121 ◽  
pp. S134
Author(s):  
B. Unay ◽  
E. Erdogan ◽  
S. Vurucu ◽  
Z. Gokcil ◽  
N. Bulakbasi ◽  
...  
Keyword(s):  
Vagus Nerve ◽  
Seizure Frequency ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Intractable Epilepsy

Surgical Treatment of Intractable Epilepsy Associated with Schizencephaly

Neurosurgery ◽  
1991 ◽  
Vol 29 (3) ◽  
pp. 421-429 ◽  
Author(s):  
Richard Leblanc ◽  
Donatella Tampieri ◽  
Yves Robitaille ◽  
William Feindel ◽  
Frederick Andermann
Keyword(s):  
Magnetic Resonance Imaging ◽  
Surgical Treatment ◽  
Magnetic Resonance ◽  
Intractable Epilepsy ◽  
Epileptic Activity ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Intractable Seizures ◽  
Generalized Seizures ◽  
Assessment And Treatment

Abstract With the advent of magnetic resonance imaging, there has been an increased recognition of schizencephaly during life, especially in epileptic patients. We report our experience with the assessment and treatment of three patients with medically intractable seizures associated with this condition. The three men were aged 24 to 37 years. Two had delayed developmental milestones and hemiparesis or hemiplegia. One had normal development and a normal neurological examination. Seizures began between the ages of 15 and 19 years and lasted for 5 to 22 years before surgery. All had partial simple or generalized seizures with predominant electroencephalographic and electrocorticographic epileptic activity localized to temporal and frontal lobes on the side of the lesion. Neuropsychological assessment indicated widespread dysfunction maximal at the areas of predominant electroencephalographic abnormality. Magnetic resonance imaging demonstrated anterior parasagittal, parietal, and Rolandic cerebral clefts, with ventricular diverticuli, gray matter heterotopia, polymicrogyria, and a true agenesis of the corpus callosum in individual patients. The patients underwent temporal (one patient) and frontotemporal (two patients) lobectomies without additional neurological deficits or neuropsychological deterioration. Postoperative follow-up showed reduction in seizure frequency. We conclude that the surgical treatment of epilepsy is well tolerated in such patients, and their seizures can be alleviated by resection of epileptogenic areas.

Sign in / Sign up

Export Citation Format

Share Document