scholarly journals Autoimmune epilepsy due to N-methyl-d-aspartate receptor antibodies in a child: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Jithangi Wanigasinghe ◽  
Thashi Chang
Keyword(s):  
Central Nervous System Infection ◽  
Poor Response ◽  
Seizure Freedom ◽  
Structural Abnormality ◽  
Long Term Outcome ◽  
Methyl Prednisolone ◽  
Aspartate Receptor ◽  
Autoimmune Epilepsy ◽  
Receptor Antibodies

Abstract Introduction Seizures of autoimmune etiology may occur independent of or predate syndromes of encephalitis. We report a child with “pure” autoimmune epilepsy followed up for 7 years to highlight long-term effects of this epilepsy and the importance of early initiation and appropriate escalation of immunosuppression to achieve a good long-term outcome. Case presentation A previously healthy 5-year-old Sri Lankan boy presented with acute, frequent, brief focal seizures of temporal-lobe semiology without clinical and investigatory findings suggestive of central nervous system infection, tumor, structural abnormality, or metabolic causes. His epilepsy showed poor response to increasing doses and combinations of antiseizure medications. Further investigations detected N-methyl-d-aspartate receptor antibodies in serum, but not cerebrospinal fluid. Treatment with intravenous methyl prednisolone and maintenance on mycophenolate resulted in a rapid reduction, with seizure freedom achieved within 5–6 weeks. He relapsed when immunotherapy and anti seizure medications were reduced after seizure freedom for 24 months. This, and subsequent relapses, showed poor response to modification of anti-seizure medications, but treatment with immunotherapy (methyl prednisolone and rituximab) achieved complete seizure freedom. At 7-years of follow-up, he remains free of seizure for over 3 years, and has average academic performance and satisfactory quality of life. Conclusions Autoimmune epilepsy is a recognized independent entity. Diagnostic criteria have been suggested for its early recognition and confirmation of diagnosis. Early diagnosis and initiation of immunosuppression, with prompt escalation of treatment when necessary, remains key to good patient outcome.

scholarly journals Sleep-related hypermotor epilepsy

Neurology ◽  
2016 ◽  
Vol 88 (1) ◽  
pp. 70-77 ◽  
Author(s):  
Laura Licchetta ◽  
Francesca Bisulli ◽  
Luca Vignatelli ◽  
Corrado Zenesini ◽  
Lidia Di Vito ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Cox Regression ◽  
Univariate Analysis ◽  
Age At Onset ◽  
Hazard Ratio ◽  
Seizure Freedom ◽  
Brain Disorder ◽  
Long Term Outcome

Objective:To assess the long-term outcome of sleep-related hypermotor epilepsy (SHE).Methods:We retrospectively reconstructed a representative cohort of patients diagnosed with SHE according to international diagnostic criteria, sleep-related seizures ≥75% and follow-up ≥5 years. Terminal remission (TR) was defined as a period of ≥5 consecutive years of seizure freedom at the last follow-up. We used Kaplan-Meier estimates to calculate the cumulative time-dependent probability of TR and to generate survival curves. Univariate and multivariate Cox regression analyses were performed.Results:We included 139 patients with a 16-year median follow-up (2,414 person-years). The mean age at onset was 13 ± 10 years. SHE was sporadic in 86% of cases and familial in 14%; 16% of patients had underlying brain abnormalities. Forty-five percent of patients had at least 1 seizure in wakefulness lifetime and 55% had seizures only in sleep (typical SHE). At the last assessment, 31 patients achieved TR (TR group, 22.3%), while 108 (NTR group, 77.7%) still had seizures or had been in remission for <5 years. The cumulative TR rate was 20.4%, 23.5%, and 28.4% by 10, 20, and 30 years from inclusion. At univariate analysis, any underlying brain disorder (any combination of intellectual disability, perinatal insult, pathologic neurologic examination, and brain structural abnormalities) and seizures in wakefulness were more frequent among the NTR group (p = 0.028; p = 0.043). Absence of any underlying brain disorder (hazard ratio 4.21, 95% confidence interval 1.26–14.05, p = 0.020) and typical SHE (hazard ratio 2.76, 95% confidence interval 1.31–5.85, p = 0.008) were associated with TR.Conclusions:Our data show a poor prognosis of SHE after a long-term follow-up. Its outcome is primarily a function of the underlying etiology.

An S wave in ECG lead V6 predicts poor response to cardiac resynchronization therapy and long-term outcome

Heart Rhythm ◽  
2020 ◽  
Vol 17 (2) ◽  
pp. 265-272
Author(s):  
Zeyu Jiang ◽  
Yuanhao Qiu ◽  
Zhiyong Qian ◽  
Yao Wang ◽  
Yongwei Zhao ◽  
...  
Keyword(s):  
Cardiac Resynchronization Therapy ◽  
Poor Response ◽  
Cardiac Resynchronization ◽  
Resynchronization Therapy ◽  
S Wave ◽  
Term Outcome ◽  
Long Term Outcome

Abstract and Posters of the XXXIV Annual Meeting of The Association for European Paediatric Cardiology, Sofia, 19-22 May, 1999

1999 ◽  
Vol 9 (S2) ◽  
pp. 1-38
Keyword(s):  
Gestational Age ◽  
Heart Defects ◽  
Neonatal Morbidity ◽  
In Utero ◽  
Structural Abnormality ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Congenital Complete Heart Block ◽  
Few Data

Introduction: Only few data are available in literature regarding the long term outcome of the newborns with isolated congenital complete heart block (CHB). The aim of the study was to describe neonatal morbidity, incidence of minor heart defects, mortality and the risks of poor outcome in the patients with CHB diagnosed in utero or as a newborn.Patients and methods: Since 1950, CHB whithout structural abnormality has been diagnosed in 152 children aged between 0-15 years in five tertiary centers in Finland. Diagnosis was made in utero or postnatally in 91 children with a median gestational age of 29.3 weeks. Maternal connective tissue disease was evident in 90% of the cases. At birth the median gestational age was 37.1 weeks (range 29–41) and the median birthweight 2969 g (range 905–4370 g). Sixty infants of 91 (66%) were girls and 7 of 91 (8%) were twins. Mean follow-up time was 10.6 years (range 0–47.5 years).Results: Fetal heart rate at diagnosis was 57 beats/min (median), and after birth 56 beats/min. Delivery route was cesarean section in 57% of cases. Insidence of neonatal morbidity was 58%; hydrops 27%, cardiac failure 46%, symptoms of neonatal lupus 18%, symptomatic PDA 16%, RDS 8%, NEC 7%, 1VH 6% and pulmonary hypertension 4%. There were 6 perinatal deaths (7%; one in utero and 5 postnatally). Pacing as a newborn was indicated in 48 of 90 cases (53%); 36 received pacemaker (PM) at older ages.

Anterior temporal lobectomy with amygdalohippocampectomy for mesial temporal sclerosis: predictors of long-term seizure control

2013 ◽  
Vol 119 (2) ◽  
pp. 261-272 ◽  
Author(s):  
Robert E. Elliott ◽  
Robert J. Bollo ◽  
Jonathan L. Berliner ◽  
Alyson Silverberg ◽  
Chad Carlson ◽  
...  
Keyword(s):  
Statistical Power ◽  
Temporal Lobectomy ◽  
Binary Logistic Regression Analysis ◽  
Seizure Freedom ◽  
Mesial Temporal Sclerosis ◽  
Anterior Temporal Lobectomy ◽  
Long Term Outcome ◽  
The Impact

Object In this paper the authors' goal was to identify preoperative variables that predict long-term seizure freedom among patients with mesial temporal sclerosis (MTS) after single-stage anterior temporal lobectomy and amygdalohippocampectomy (ATL-AH). Methods The authors retrospectively reviewed 116 consecutive patients (66 females, mean age at surgery 40.7 years) with refractory seizures and pathologically confirmed MTS who underwent ATL-AH with at least 2 years of follow-up. All patients underwent preoperative MRI and video-electroencephalography (EEG); 106 patients (91.4%) underwent Wada testing and 107 patients (92.2%) had neuropsychological evaluations. The authors assessed the concordance of these 4 studies (defined as test consistent with the side of eventual surgery) and analyzed the impact of preoperative variables on seizure freedom. Results The median follow-up after surgery was 6.7 years (mean 6.9 years). Overall, 103 patients (89%) were seizure free, and 109 patients (94%) had Engel Class I or II outcome. Concordant findings were highest for video-EEG (100%), PET (100%), MRI (99.0%), and Wada testing (90.4%) and lowest for SPECT (84.6%) and neuropsychological testing (82.5%). Using binary logistic regression analysis (seizure free or not) and Cox proportional hazard analysis (seizure-free survival), less disparity in the Wada memory scores between the ipsilateral and contralateral sides was associated with persistent seizures. Conclusions Seizure freedom of nearly 90% can be achieved with ATL-AH in properly selected patients with MTS and concordant preoperative studies. The low number of poor outcomes and exclusion of multistage patients limit the statistical power to determine preoperative variables that predict failure. Strong Wada memory lateralization was associated with excellent long-term outcome and adds important localization information to structural and neurophysiological data in predicting outcome after ATL-AH for MTS.

Epilepsy Surgery of the Temporal Lobe in Pediatric Population: A Retrospective Analysis

Neurosurgery ◽  
2011 ◽  
Vol 70 (3) ◽  
pp. 684-692 ◽  
Author(s):  
Miguel Angel Lopez-Gonzalez ◽  
Jorge Alvaro Gonzalez-Martinez ◽  
Lara Jehi ◽  
Prakash Kotagal ◽  
Ann Warbel ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Epilepsy Surgery ◽  
Pediatric Patients ◽  
Hippocampal Sclerosis ◽  
Pediatric Population ◽  
Cleveland Clinic ◽  
Seizure Freedom ◽  
Complication Rates ◽  
Long Term Outcome

Abstract Background: There is still some reluctance to refer pediatric patients for epilepsy surgery, despite evidence of success in retrospective series. Objective: To describe surgical experience and long-term outcome in pediatric temporal lobe epilepsy (TLE) at a single institution. Methods: Retrospective review of pediatric (&lt;18-years-old) TLE patients who underwent surgery between November 1996 and December 2006 at Cleveland Clinic Epilepsy Center. Cox proportional hazard modeling was used to assess outcome predictors. Results: One hundred thirty pediatric patients met study criteria. Mean time between seizure onset and surgery was 6.3 years. Invasive evaluation was used in 32 patients (24.5%). Hippocampal sclerosis was present in 70 patients (53.8%), either alone or associated in dual pathology. The complication rate was 7%. The seizure-freedom rates at 1, 2, 5, and 12 years were 76%, 72%, 54%, and 41%, respectively (Kaplan-Meier). With the use of the Engel outcome classification, 98 (75.3%) patients were class I, 11 (8.5%) class II, 9 (7%) class III, and 12 (9.2%) were class IV at last follow-up. Only 4 (3.1%) patients underwent reoperations. Antiepileptic drugs (AEDs) were discontinued in 36 patients (28.3%) in a mean period of 18 months (SD ± 17 months; range, 6-102 months). Although left-sided resection, lower number of preoperative AED trials (≤4), and tumor pathology correlated with favorable seizure outcomes, extensive surgical resection remained the only significant outcome predictor after multivariate analysis (P = .007; HR = 0.13 [95% confidence interval 0.007–0.64]). Conclusion: Careful selection of surgical candidates by multidisciplinary evaluations is required. Long-term seizure control is achieved successfully with acceptable low complication rates.

Long-term outcome after emergency resection of hypothalamic hamartomas for status gelasticus

2011 ◽  
Vol 30 (2) ◽  
pp. E5 ◽  
Author(s):  
Sandipan Pati ◽  
Reena G Rastogi ◽  
Adib A. Abla ◽  
Harold L. Rekate ◽  
Yu-Tze Ng
Keyword(s):  
Endoscopic Resection ◽  
Emergency Surgery ◽  
Seizure Freedom ◽  
Gelastic Seizures ◽  
Long Term Outcome ◽  
Hypothalamic Hamartomas ◽  
Long Term Follow Up ◽  
Emergency Resection

Object Gelastic seizures are epileptic events characterized by bouts of laughter. They are rare and mostly associated with hypothalamic hamartomas (HHs). Status gelasticus, a rare form of status epilepticus, is defined as a prolonged cluster of gelastic seizures (> 20–30 minutes) without necessarily involving loss of awareness between seizures. Emergency resection of the hamartoma is highly effective in these situations and should be considered as early as possible. The authors retrospectively reviewed their surgical cases to document the success, complications, and long-term follow-up after emergency resection of HHs for status gelasticus. Methods The authors report on a retrospective case series from a single tertiary care center. Three patients who presented with status gelasticus underwent emergency resection of HHs. Demographic details, seizure history, medical treatment, and postoperative follow-up data were evaluated. Long-term follow-up (minimum 2 years) data were obtained either from the last clinic visit notes or via telephone and e-mail contacts. The institutional review board at St. Joseph's Hospital approved this study. Results In the last 7 years, of 157 patients who underwent HH resection, the resection was performed on an emergency basis for status gelasticus in 3 cases. At emergency surgery, these 3 patients ranged in age from 9 months to 3.5 years. All of the patients were boys. Delalande and Fohlen Type II, III, and IV lesions were present in the 3 patients. Surgical approaches for resection of HH included an orbitozygomatic, transcallosal anterior interforniceal approach and endoscopic resection. Status gelasticus was terminated following emergency surgery in all cases, and 1 patient was seizure free. Postsurgical complications included, in 1 case, a small right thalamic infarct with mild transient left hemiparesis, which completely resolved within 2 days. Within 2 years of their original surgery, 2 patients underwent further elective surgeries (endoscopic resection and radiosurgery for persistent symptomatic seizures). Follow-up since their most recent surgery ranged from 8 months to 2 years. Two patients were seizure free and 1 patient had greater than 50% reduction in seizures. Conclusions Status gelasticus associated with HHs can be successfully terminated by emergency resection of the HH. Long-term follow-up in the present series suggests good seizure freedom results or at least greater than 50% reduction in seizures, although repeat operations were necessary.

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