The epileptology of alternating hemiplegia of childhood

Neurology ◽  
2019 ◽  
Vol 93 (13) ◽  
pp. e1248-e1259 ◽  
Author(s):  
Julie Uchitel ◽  
Ashley Helseth ◽  
Lyndsey Prange ◽  
Melissa McLean ◽  
Ryan Ghusayni ◽  
...  
Keyword(s):  
Epileptic Seizures ◽  
Current Data ◽  
Drug Resistant ◽  
Alternating Hemiplegia Of Childhood ◽  
Generalized Seizures ◽  
Eeg Changes ◽  
Patients With Epilepsy ◽  
Multiple Episodes ◽  
Video Eeg

ObjectiveTo report our experience and investigate 5 original hypotheses: (1) multiple types of epileptic seizures occur in alternating hemiplegia of childhood (AHC), and these can be the initial presentation; (2) epileptiform abnormalities often appear well after clinical seizures; (3) nonepileptic reduced awareness spells (RAS) occur frequently; (4) epilepsy is commonly drug resistant but may respond to vagal nerve stimulation (VNS); and (5) status epilepticus (SE) is common and is usually refractory and recurrent.MethodsWe analyzed a cohort of 51 consecutive patients with AHC.ResultsThirty-two of 51 patients had epilepsy: 18 focal seizures, frontal more frequently than temporal, and then posterior. Eleven had primary generalized seizures (tonic-clonic, myoclonic, and/or absence). Epileptic seizures preceded other AHC paroxysmal events in 8 (lag 5.63 ± 6.55 months; p = 0.0365). In 7 of 32, initial EEGs were normal, with the first epileptiform EEG lagging behind by 3.53 ± 4.65 years (p = 0.0484). RAS occurred equally in patients with epilepsy (16 of 32) and patients without epilepsy (10 of 19, p = 1.0). Twenty-eight patients had video-EEG; captured RAS showed no concomitant EEG changes. Nineteen patients (59%) were drug resistant. VNS resulted in >50% reduction in seizures in 5 of 6 (p < 0.04). Twelve patients (38%) had SE (9 of 12 multiple episodes), refractory/superrefractory in all (p < 0.001), and 4 of 12 had regression after SE.ConclusionsEpilepsy in AHC can be focal or generalized. Epileptic seizures may be the first paroxysmal symptom. EEG may become epileptiform only on follow-up. Epilepsy, although frequently drug resistant, can respond to VNS. RAS are frequent and nonepileptic. SE often recurs and is usually refractory/superrefractory. Our observations are consistent with current data on AHC-ATP1A3 pathophysiology.

scholarly journals Evaluation of ubiquitin C-terminal hydrolase-L1 enzyme levels in patients with epilepsy

2020 ◽  
Vol 78 (7) ◽  
pp. 424-429
Author(s):  
Ibrahim Halil YASAK ◽  
Mustafa YILMAZ ◽  
Murat GÖNEN ◽  
Metin ATESCELIK ◽  
Mehtap GURGER ◽  
...  
Keyword(s):  
Healthy Volunteers ◽  
Epileptic Seizure ◽  
Epileptic Seizures ◽  
Healthy Individuals ◽  
Roc Curve Analysis ◽  
Remission Period ◽  
Significant Difference ◽  
Epileptic Attack ◽  
Patients With Epilepsy

ABSTRACT Objective: Ubiquitin C-terminal Hydrolase-L1 (UCH-L1) enzyme levels were investigated in patients with epilepsy, epileptic seizure, remission period, and healthy individuals. Methods: Three main groups were evaluated, including epileptic seizure, patients with epilepsy in the non-seizure period, and healthy volunteers. The patients having a seizure in the Emergency department or brought by a postictal confusion were included in the epileptic attack group. The patients having a seizure attack or presenting to the Neurology outpatient department for follow up were included in the non-seizure (remission period) group. Results: The UCH-L1 enzyme levels of 160 patients with epilepsy (80 patients with epileptic attack and 80 patients with epilepsy in the non-seizure period) and 100 healthy volunteers were compared. Whereas the UCH-L1 enzyme levels were 8.30 (IQR=6.57‒11.40) ng/mL in all patients with epilepsy, they were detected as 3.90 (IQR=3.31‒7.22) ng/mL in healthy volunteers, and significantly increased in numbers for those with epilepsy (p<0.001). However, whereas the UCH-L1 levels were 8.50 (IQR=6.93‒11.16) ng/mL in the patients with epileptic seizures, they were 8.10 (IQR=6.22‒11.93) ng/mL in the non-seizure period, and no significant difference was detected (p=0.6123). When the UCH-L1 cut-off value was taken as 4.34 mg/mL in Receiver Operating Characteristic (ROC) Curve analysis, the sensitivity and specificity detected were 93.75 and 66.00%, respectively (AUG=0.801; p<0.0001; 95%CI 0.747‒0.848) for patients with epilepsy. Conclusion: Even though UCH-L1 levels significantly increased more in patients with epilepsy than in healthy individuals, there was no difference between epileptic seizure and non-seizure periods.

scholarly journals Efficacy of Vagal Nerve Stimulation for Drug-Resistant Epilepsy: Is it the Stimulation or Medication?

2017 ◽  
Vol 44 (5) ◽  
pp. 532-537 ◽  
Author(s):  
Jaylynn Arcand ◽  
Karen Waterhouse ◽  
Lizbeth Hernandez-Ronquillo ◽  
Aleksander Vitali ◽  
Jose F. Tellez-Zenteno
Keyword(s):  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Drug Resistant ◽  
Idiopathic Generalized Epilepsy ◽  
Drug Resistant Epilepsy ◽  
Generalized Epilepsy ◽  
Patients With Epilepsy ◽  
Seizure Classification

AbstractBackground: Vagus nerve stimulation (VNS) therapy has been widely recognized as an alternative for the treatment of drug-resistant epilepsy, although modification of antiepileptic drugs (AEDs) during VNS treatment could explain the improvement in patients. Methods: We retrospectively assessed the efficacy of VNS in 30 adult patients with epilepsy treated with >6 months of follow-up. The criteria for implantation were the following: (1) not a candidate for resective epilepsy surgery, (2) drug-resistant epilepsy, (3) impairment of quality of life, (4) no other option of treatment, and (5) patients with idiopathic generalized epilepsy who fail to be controlled with appropriate AEDs. We assessed sociodemographics, seizure etiology, seizure classification, and AEDs used during treatment with VNS. We assessed adverse effects and efficacy. Responder rate was defined as >50% seizure improvement from baseline. Results: Thirty patients (females, 18; males, 12; age, 35.1±13.3 years) were included. After 6, 12, 24, and 36 months of follow-up, the response rates were: 13/30 (43%), 13/27 (48%), 9/22 (41%), and 8/16 (50%), respectively; none was seizure free. Fifty-seven percent, 33%, 59%, and 81% of patients had changes of medication type or dose at 6, 12, 24, and 36 months respectively. In the majority of patients, the change of medication consisted of an increase in the dose of AEDs. Conclusions: Our study shows that VNS is an effective therapy, although significant changes in medications were done along with the therapy; therefore, the real effect of VNS could be controversial.

scholarly journals Management of patients with single and rare epileptic seizures

2020 ◽  
Vol 15 (2) ◽  
pp. 12-16
Author(s):  
A. S. Kotov
Keyword(s):  
Magnetic Resonance ◽  
Epileptic Seizures ◽  
Resonance Imaging ◽  
Medical Problems ◽  
Unprovoked Seizure ◽  
Life Study ◽  
History Of ◽  
Patients With Epilepsy ◽  
The Brain

Objective: to study the structure of the disease and develop tactics of treatment of patients with single and rare epileptic seizures. Materials and methods. 1200 patients with epilepsy were examined, 103 patients were identified who had no more than 3 seizures throughout their life. Study included evaluation of anamnesis, clinical and neurological examination, routine electroencephalography and/or videoelectroencephalographic monitoring, magnetic resonance imaging of the brain. Results. Relapse of seizures in individuals with a history of follow-up occurred in 32 % of cases; epileptogenic changes in magnetic resonance image and, especially, anatomical and electro-clinical correlation of the epilepsy focus were associated with the risk of relapse. Conclusions. The decision on drug therapy after the first unprovoked seizure should be based on the ratio of the risk of repeated seizures and the risk of side effects. This solution should be individual and take into account not only medical problems, but also the preferences of the patient and his family members.

scholarly journals Drug Resistant Epilepsy Among Patients Attended The Neurosciences Hospital

2019 ◽  
Vol 13 (1) ◽  
pp. 108-115
Author(s):  
Nael Husain Zaer
Keyword(s):  
Drug Resistance ◽  
Medical History ◽  
Refractory Epilepsy ◽  
Seizure Freedom ◽  
Drug Resistant ◽  
Past Medical History ◽  
Number Of Patients ◽  
Drug Resistant Epilepsy ◽  
Patients With Epilepsy

Background: Drug resistant epilepsy is defined as failure of adequate trials of two tolerated, appropriately chosen and used antiepileptic drug schedules to achieve sustained seizure freedom. Up to 30% of patients referred to clinics with a diagnosis of pharmaco-resistant epilepsy may have been misdiagnosed, and many can be helped by optimizing their treatment.Pseudoresistance, in which seizures persist because the underlying disorder has not been adequately or appropriately treated, must be ruled out or corrected before drug treatment can be considered to have failed. Objectives: The objectives of this study were to determine the causes of drug failure in patients with epilepsy and to differentiate between drug resistant epilepsy and pseudoresistant epilepsy. Type of the study: This is a retrospective study. Method: It is conducted in Baghdad governorate at the epilepsy clinic in the neurosciences hospital during the period from the 1st of February through July 2013. Two hundred patients with refractory epilepsy were involved. These patients attended the epilepsy clinic during 2011 and 2012. The data was collected from the files of the patients including age, gender, weight, history of presenting illness, type of seizure, drugs used, duration of disease, EEG and imaging findings, compliance and follow up. Results: Drug resistance epilepsy constituted a prevalence of 24% (128) as the total number of patients with epilepsy attending the hospital during the same period was 527.The mean age of patients with refractory epilepsy was 25 years. Male were 56.5% (113/200) and urban residents were 70.5% (141/200). The study revealed that 64% (128/200) of refractory epilepsy was attributed to drug resistance; while the remaining proportion was pseudoresistance 36% (72/200). The main cause of pseudoresistance was poor compliance 36.1% (26/72).The most common type of seizure in the sampled patients was generalized tonic clonic seizures in 51.5% (103/200).Compliance was found to be statistically associated with abnormal EEG finding, past medical history (hypertension, cardiac diseases, encephalitis, diabetes mellitus and any significant history) and quality of follow up. The follow-up was found to be statistically associated with the family history, past medical history( encephalitis and hypertension) and compliance of patient. Conclusion:A considerable number of patientsdiagnosed as cases of drug resistant epilepsy had another explanation causing drug failure.The study recommends the application of consensus definition for drug resistant epilepsy and periodic evaluation of patients with drug resistant epilepsy to exclude pseudoresistance.

scholarly journals “Ictal” Bradyarrhythmias in Patients with Drug-Resistant Epilepsy: Results of Long-Term Heart Rhythm Monitoring

Kardiologiia ◽  
2021 ◽  
Vol 60 (12) ◽  
pp. 90-96
Author(s):  
S. E. Serdyuk ◽  
K. V. Davtyan ◽  
S. G. Burd ◽  
E. S. Mishina ◽  
O. M. Drapkina ◽  
...  
Keyword(s):  
Heart Rhythm ◽  
Epileptic Seizures ◽  
Channel Blockers ◽  
Drug Resistant ◽  
Disorder Of Consciousness ◽  
Functional Changes ◽  
Long Duration ◽  
Drug Resistant Epilepsy ◽  
Patients With Epilepsy

Aim      To determine the type and incidence of ictal bradyarrhythmias in patients with drug-resistant types of epilepsy by long-term electrocardiogram (ECG) monitoring.Material and methods  Subcutaneous ECG monitors programed for recording pauses >3 sec and episodes of bradycardia ≤45 bpm were implanted in 193 patients with persistent epileptic seizures without organic pathology of the myocardium. Recording was activated by the patient/family at the onset of epileptic seizure. The follow-up period was 36 months with visits to the clinic every three months.Results For 36 months of monitoring, 6494 ECG fragments were recorded. Ictal bradycardia was observed in 6.7 % of patients, including ictal asystole in 2.6 % of patients. Episodes of bradycardia and asystole during epileptic seizures were transient and developed significantly more frequently in men, patients with long duration of the disease, bilateral tonic-clonic or focal seizures with disorder of consciousness, during sleep, on the background of treatment with several antiepileptic agents, mostly from the group of potassium channel blockers.Conclusion      Bradyarrhythmias accompanying epileptic seizures are transient and reproducible from seizure to seizure. They reflect functional changes in the myocardium and do not determine the life prediction for patients with epilepsy without organic pathology of the heart.

Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas

Neurosurgery ◽  
2000 ◽  
Vol 47 (6) ◽  
pp. 1343-1352 ◽  
Author(s):  
Jean Régis ◽  
Fabrice Bartolomei ◽  
Bertrand de Toffol ◽  
Pierre Genton ◽  
Tatsuya Kobayashi ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Pathological Condition ◽  
Gamma Knife Surgery ◽  
Clear Correlation ◽  
Drug Resistant ◽  
Hypothalamic Hamartomas ◽  
Generalized Seizures ◽  
Successful Group ◽  
Drug Resistant Epilepsy

ABSTRACT OBJECTIVE Drug-resistant epilepsy associated with hypothalamic hamartomas (HHs) can be cured by microsurgical resection of the lesions. Morbidity and mortality rates for microsurgery in this area are significant. Gamma knife surgery (GKS) is less invasive and seems to be well adapted for this indication. METHODS To evaluate the safety and efficacy of GKS to treat this uncommon pathological condition, we organized a multicenter retrospective study. Ten patients were treated in seven different centers. The follow-up periods were more than 12 months for eight patients, with a median follow-up period of 28 months (mean, 35 mo; range, 12–71 mo). All patients had severe drug-resistant epilepsy, including frequent gelastic and generalized tonic or tonicoclonic attacks. The median age was 13.5 years (range, 1–32 yr; mean, 14 yr) at the time of GKS. Three patients experienced precocious puberty. All patients had sessile HHs. The median marginal dose was 15.25 Gy (range, 12–20 Gy). Two patients were treated two times (at 19 and 49 mo) because of insufficient efficacy. RESULTS All patients exhibited improvement. Four patients were seizure-free, one experienced rare nocturnal seizures, one experienced some rare partial seizures but no more generalized attacks, and two exhibited only improvement, with reductions in the frequency of seizures but persistence of some rare generalized seizures. Two patients, now seizure-free, were considered to exhibit insufficient improvement after the first GKS procedure and were treated a second time. A clear correlation between efficacy and dose was observed in this series. The marginal dose was more than 17 Gy for all patients in the successful group and less than 13 Gy for all patients in the “improved” group. No side effects were reported, except for poikilothermia in one patient. Behavior was clearly improved for two patients (with only slight improvements in their epilepsy). Complete coverage of the HHs did not seem to be mandatory, because the dosimetry spared a significant part of the lesions for two patients in the successful group. CONCLUSION We report the first series demonstrating that GKS can be a safe and effective treatment for epilepsy related to HHs. We advocate marginal doses greater than or equal to 17 Gy and partial dose-planning when necessary, for avoidance of critical surrounding structures.

scholarly journals The effect of epilepsy control on psychogenic non epileptic seizures

2021 ◽  
Author(s):  
Muaz.A.Ibrahim ◽  
Tasneem.M.F ◽  
Khabab Abbasher Hussien Mohamed Ahmed ◽  
Abdallah M. Abdallah ◽  
Mohammed Eltahier Abdalla Omer ◽  
...  
Keyword(s):  
Epileptic Seizure ◽  
Focal Epilepsy ◽  
Neurological Diseases ◽  
Epileptic Seizures ◽  
Abnormal Behavior ◽  
Detailed History ◽  
Follow Up Care ◽  
History Of ◽  
Patients With Epilepsy

Abstract Background: Epilepsy is one of the most common neurological diseases. The coexistence of epilepsy and PNES poses a major challenge to neurologists and psychiatrists in the treatment and follow-up care of patients. Methods: 33 Sudanese patients with epilepsy and concomitant psychogenic non epileptic seizure where included in this study and full detailed history of both epilepsy and PNES was obtained. The study was conducted in Daoud charity clinic during the period from May to July 2018Results : Out of 33 patients 19 were males and 14 were females. Seven patients had focal epilepsy, 23 had generalized tonic clonic epilepsy (GTC), 2 had GTC with atypical absent seizure, and one had GTC with myoclonus.Six patients experienced an attack of PNES after more than 1 year of being free of epilepsy, while 9 patients had PNES attack between 3 months to 1 year of the last attack of epilepsy, and 18 patients developed both PNES and epilepsy within less than 3 months.The pattern of presentation of PNES was bizarre movements in 13 patients, abnormal behavior in 9 patients, while 11 patients experienced both bizarre movement and abnormal behavior note that all those 11 patients had GTC epilepsy. Conclusion: Experiencing psychogenic non epileptic seizure (PNES) in patients with coexisting PNES and epilepsy is not affected by the epilepsy free period

Neurologist, 11 years’ experience, USA

2020 ◽  
pp. 63-67
Author(s):  
Markus Reuber ◽  
Gregg H. Rawlings ◽  
Steven C. Schachter
Keyword(s):  
Epileptiform Activity ◽  
Epileptic Seizures ◽  
Clinical Value ◽  
Interictal Eeg ◽  
Eeg Recordings ◽  
Patients With Epilepsy ◽  
Present Possibility ◽  
Epilepsy Monitoring

This chapter focuses on the clinical value of continued vigilance and neurologic follow-up after video-EEG (VEEG) confirmation of the diagnosis of Psychogenic Non-Epileptic Seizures (PNES), even when long-term EEG recordings over two separate admissions to an epilepsy monitoring unit have not captured any epileptiform activity. It has been shown that 19% of patients with epilepsy will not have interictal epileptiform abnormality during an admission for long-term VEEG monitoring. In particular, patients with extratemporal lobe epilepsies who have deep/mesial seizure foci and those with well-controlled epilepsies will be likely not to have epileptiform interictal EEG activity. In consideration of these observations, it has been advised that patients with PNES and non-epileptiform long-term EEG recordings should be followed by a neurologist for at least six months after discontinuation of antiepileptic drugs (AED). This consideration is due to the small but ever-present possibility of coexisting epilepsy as well as the observation that the risk of breakthrough epileptic seizures is highest during the initial six months after discontinuation of AEDs.

scholarly journals Patients with epilepsy and patients with psychogenic non-epileptic seizures: Video-EEG, clinical and neuropsychological evaluation

Seizure ◽  
2011 ◽  
Vol 20 (9) ◽  
pp. 706-710 ◽  
Author(s):  
Katherine Turner ◽  
Ada Piazzini ◽  
Valentina Chiesa ◽  
Valentina Barbieri ◽  
Aglaia Vignoli ◽  
...  
Keyword(s):  
Epileptic Seizures ◽  
Neuropsychological Evaluation ◽  
Patients With Epilepsy ◽  
Video Eeg

scholarly journals Probable causal link between epilepsy and sleep apnea: case report

2007 ◽  
Vol 65 (1) ◽  
pp. 164-166 ◽  
Author(s):  
Michele Dominici ◽  
Fernando Pompeu Filho ◽  
Marleide da Mota Gomes
Keyword(s):  
Sleep Apnea ◽  
Sleep Apnea Syndrome ◽  
Epileptic Seizures ◽  
Causal Link ◽  
Daytime Somnolence ◽  
Respiratory Events ◽  
Co Morbidity ◽  
Epileptic Patients ◽  
Patients With Epilepsy ◽  
Video Eeg

Patients with epilepsy were reported to have concomitant sleep apnea, but it has been rarely linked to the epilepsy itself. We present a case of a 28-year-old, obese man with secondary medically resistant partial complex epilepsy due to a brain trauma, with progressive snoring, and sleep agitation, apneas, and important daytime somnolence. It was noticed in the polysomnographic study that he had several sleep respiratory events, probably due both to the epileptic seizures and the sleep apnea syndrome as a co-morbidity. Apnea and epilepsy will be discussed. A careful video-EEG-polysomnography study is important in evaluating refractory epileptic patients and/or epileptic patients with snoring.

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