Initial Electroencephalogram Research Articles

Seizure recurrence following the first unprovoked seizure: Risk factors among children in UAE.

In a retrospective study of paediatric and adolescent patients in Abu Dhabi, UAE, who experienced their first unprovoked seizure between March 2016 and March 2020, with a minimum one-year follow-up, we identified significant risk factors associated with seizure recurrence. Among 317 patients, 96.2% experienced seizure recurrence, with the majority (68.8%) occurring within the first 6-month follow-up period. Notable risk factors for recurrence included focal seizures, symptomatic seizure causes, abnormal initial electroencephalogram (EEG) findings, abnormal brain magnetic resonance imaging results, and the presence of neurological disorders. Interestingly, the type of epileptiform activity in the initial EEG did not predict recurrence risk. Over a 3-year period, the overall recurrence risk was 98.4%, particularly higher in cases with symptomatic seizures compared to idiopathic (genetic) ones. These findings underscore the importance of vigilant monitoring, particularly in the early post-seizure follow-up period, and advocate for initial EEG assessments, especially in cases of remote symptomatic first unprovoked seizures.

Cohort of Phenotype, Genotype, and Outcome of SCN Developmental and Epileptic Encephalopathies from Southern Part of India.

The SCN encephalopathies are one of the rare early childhood intractable epileptic encephalopathies associated with pleomorphic seizures, cognitive decline, motor, and behavioral abnormalities that begin in early infancy. There is a dearth of data on phenotype and genotype of SCN encephalopathies from the Indian subcontinent, hence we are reporting clinical and molecular profile and outcome of SCN developmental and epileptic encephalopathies. This is a retrospective chart review of SCN developmental and epileptic encephalopathies in a tertiary care center, Bangalore, India between January 2015 and March 2020. All children with clinical features of SCN developmental and epileptic encephalopathies and confirmed with pathogenic variants were included. A total of 50 cases of SCN developmental and epileptic encephalopathies were analyzed, 31 of them were male and the mean age of presentation was 7.8 months. Precipitating factors for the first episode of seizure were fever and vaccination accounting for 33 and 8 children, respectively. Forty (80%) children had prolonged seizures and 15 (30%) had epileptic spasms. All children had a normal birth history and normal development before the onset of seizures, which was followed by developmental delay and regression. Thirty (60%) children had behavioral difficulties, notable hyperactivity, and autistic features. Neuroimaging and the initial electroencephalogram (EEG) were normal in all patients. The mean age of abnormal EEG was 14 months. The various subtypes of SCN variants were SCN1A in 31 children followed by SCN2A and SCN9A in eight children each and SCN1B in three children. Frameshift and nonsense mutations were associated with more severe phenotype and poor outcome compared with missense mutations. Thirty-four patients partially responded to treatment and the rest were refractory. The results of genetic testing were used to guide treatment; sodium channel blocking antiepileptic drugs were discontinued in 15 patients and sodium channel blocking agents were started in 3 patients with partial response. Three out of four children on stiripentol had a partial response. The SCN developmental and epileptic encephalopathies can present with epileptic spasms in addition to other types of seizures. Epileptic spasms are more common in nonsense and frameshift mutations. The outcome is poor in children with epileptic spasms compared with those without epileptic spasms. Genetic testing helps to select antiepileptic drugs that lead to seizure reduction.

CRITICAL CARE COMPLICATIONS OF LITHIUM TOXICITY

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Lithium is amongst the earliest agents with therapeutic benefit in the treatment of mania and remains employed in the management of bipolar disorder. However, its use is limited by a narrow therapeutic window, with numerous acute and acute-on-chronic toxicities with a spectrum of severity. Here, we discuss the critical care management of two cases of lithium toxicity-associated seizures. CASE PRESENTATION: Case 1:A 54-year-old female with schizophrenia on lithium and quetiapine recently initiated on a course of ibuprofen for sciatica presented with nausea, vomiting, diarrhea, and altered sensorium. Physical exam revealed disorientation to her surroundings with psychomotor slowing and speech latency with tremulousness and a depressed respiratory rate. Labs were notable for acute hypercapnic respiratory failure with concomitant non-anion gap metabolic acidosis and acute kidney injury. Serum lithium levels were elevated to 3.89 mmol/L. Initial electroencephalogram showed epileptiform waves concerning for seizure foci. Urgent dialysis was initiated with transition to continuous renal replacement therapy for lithium clearance. The patient was loaded on levetiracetam. Her metabolic encephalopathy slowly improved, with eventual transfer to the psychiatric ward for further medication titration.Case 2:A 67-year-old female with bipolar disorder on lithium and haloperidol recently discharged after titration of medications in the psychiatric ward presented with altered sensorium. Physical exam was notable for waxing and waning mental status with associated dysarthric speech. Urgent evaluation for stroke found no structural causes of her altered sensorium. Lithium levels returned elevated to 2.410 mmol/L. No renal replacement therapy was pursued after discussion with toxicology given lab value downtrend, but continuous electroencephalogram demonstrated non-convulsive status epilepticus suspicious for chronic lithium toxicity with blood brain barrier penetration. The trachea was intubated and anti-epileptics were initiated including a midazolam drip, levetiracetam load, lacosamide, and fosphenytoin. However, her seizures were refractory with prolonged seizure activity causing persistent global encephalopathy. Tracheostomy and percutaneous endoscopic gastrostomy tubes were placed. The patient remains hospitalized after a six-month course but is now stable off mechanical ventilation. DISCUSSION: Lithium toxicity is an acute medical condition with potentially profound neurologic consequences. The cornerstone of management of lithium toxicity is supportive care. Dialysis for lithium toxicity is recommended based upon current guidelines, with therapy directed by lithium levels, evidence of renal impairment, changes in mentation, and evidence of seizure activity or dysrhythmia [1]. CONCLUSIONS: Lithium is a high-risk medication necessitating regular monitoring, with potential severe neurotoxicity. Reference #1: Decker BS et al. Extracorporeal treatment for lithium poisoning: Systematic review and recommendations from the EXTRIP workgroup. 2015. Clin J Am Soc Nephrol 10: 875-887. DISCLOSURES: Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Pharmaceuticals Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Vapotherm, Inc Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria No relevant relationships by Scott Oh, source=Web Response No relevant relationships by Alexander Yuen, source=Web Response

Encefalitis por virus influenza B en un paciente adulto. Reporte de caso

Neurological manifestations associated with influenza virus infection include encephalitis, encephalopathy, acute necrotizing encephalitis, transverse myelitis, acute disseminated encephalomyelitis, mild encephalitis with reversible splenial syndrome (MERS), and Guillaín Barré syndrome. We report a 16-year-old female who was admitted at our emergency department with seizures, confusion, nystagmus and motor clumsiness five days after an upper a respiratory tract infection. Influenza type B virus infection was confirmed by chain polymerase reaction analysis. The initial electroencephalogram demonstrated a pattern of global slowness without epileptic discharges. One week later, it showed a progression to slow-wave focal bilateral discharges at both temporal and occipital lobes. The patient had a favorable evolution and was discharged 19 days after admission with phenytoin to prevent seizures.

When aphasia is due to aphasic status epilepticus: a diagnostic challenge.

Aphasic status epilepticus is an uncommon entity that should be included in the differential diagnosis of persistent and sudden language disorders. In our study, we describe seven patients admitted with clinical and electroencephalographic diagnosis of aphasic status, who were studied with both neuroimaging and electroencephalogram. The mean age was 65.9years (range of 39-89). Three of the patients had previously been diagnosed of epilepsy. The aphasia was global in six patients. In one case, we found foci of the left hemorrhagic contusions. The initial electroencephalogram (EEG) was not conclusive of status in two patients. In one patient, neuroimaging showed left hemispheric hypoperfusion, compatible with postictal changes. Six out of seven patients required at least two antiepileptic drugs. Three patients died of systemic complications (infectious causes), whereas the other four cases had a complete recovery. Our study highlights that a second EEG study might be necessary to confirm epileptiform activity, when clinical features and other tests suggest an epileptic origin. An early and specific treatment, avoiding or diminishing comorbidities, might significantly improve the prognosis of these patients.

Complex observation of scalp fast (40-150 Hz) oscillations in West syndrome and related disorders with structural brain pathology.

SummaryWe investigated the relationship between the scalp distribution of fast (40–150 Hz) oscillations (FOs) and epileptogenic lesions in West syndrome (WS) and related disorders. Subjects were 9 pediatric patients with surgically confirmed structural epileptogenic pathology (age at initial electroencephalogram [EEG] recording: mean 7.1 months, range 1–22 months). The diagnosis was WS in 7 patients, Ohtahara syndrome in 1, and a transitional state from Ohtahara syndrome to WS in the other. In the scalp EEG data of these patients, we conservatively detected FOs, and then examined the distribution of FOs. In five patients, the scalp distribution of FOs was consistent and concordant with the lateralization of cerebral pathology. In another patient, FOs were consistently dominant over the healthy cerebral hemisphere, and the EEG was relatively low in amplitude over the pathological atrophic hemisphere. In the remaining 3 patients, the dominance of FOs was inconsistent and, in 2 of these patients, the epileptogenic hemisphere was reduced in volume, which may result from atrophy or hypoplasia. The correspondence between the scalp distribution of FOs and the epileptogenic lesion should be studied, taking the type of lesion into account. The factors affecting scalp FOs remain to be elucidated.

Time interval providing highest yield for initial EEG in patients with new onset seizures

PurposeTiming providing highest yield of initial electroencephalogram (EEG) after new onset unprovoked seizures, is an important and practical issue both in diagnosis and patient care. Current guidelines suggest routine EEG is the standard of care in work-up. However, yield of the initial EEG and exact timeframe in which to perform remain unclear and standardization in both adult and pediatric populations is still lacking. Our objective was to determine a concrete timeframe to perform initial EEG in patients with new onset unprovoked seizures and provide greater yield of EEG benefits in patient management. MethodThis is a retrospective chart review study of both pediatric and adult patients identified from EEG Database over 1999–2014 located at Kaleida Health at Buffalo using keyword “new onset seizure”. ResultsThe percentage of EEGs revealing epileptiform discharges is much higher if EEGs are performed within 12hours after the seizure onset compared to studies done beyond this timeframe (53.1% versus 23.9%). There was no significant difference in the presence of epileptiform discharges if the study is done 12hours after the seizure onset. Odds ratio was 3.599 (the presence of epileptiform discharges) with a Confidence Interval of 1.691–7.660 (p=0.001) within first 12hours of initial event. ConclusionThis study demonstrated statistical significance in yield of epileptiform discharges by performing EEG within the first 12hours of seizure onset, in both adult and pediatric populations, which should be considered for clinical practice and patient care.

Temporal current-source of spikes suggests initial treatment failure in childhood absence epilepsy

PurposeIn addition to the frontal lobe, the temporal lobe may also be involved in typical absence seizures. However, few studies have addressed the relationship between this involvement and drug responsiveness in childhood absence epilepsy (CAE). In this study, we observed the current-source distribution (CSD) of generalized spike-and-wave discharges (GSWDs) and investigated the relationship between temporal lobe involvement in the CSD and responsiveness to initial antiepileptic drug (AED) in CAE. MethodSeventeen consecutive patients with CAE were retrospectively enrolled in the study. Patients were divided into an initial-response group and an initial-failure group, according to their responsiveness to the initial AED treatment. For each patient, the spike peak CSD of an averaged GSWD was obtained from the initial electroencephalogram. We compared the incidence of temporal involvement in the CSD between the two groups. We also compared clinical variables, including age of onset, gender, type and dose of first AED, time to cessation of clinical seizures, and seizure-free status. ResultsThe initial-response and initial-failure groups contained 12 and five patients, respectively. Temporal lobe involvement was more frequent (80% vs. 17%, p=0.03), and time to cessation of clinical seizures was more prolonged (median 2.5 months vs. 8 months, p<0.01) in the initial-failure than in the initial-response group. None of the other variables studied differed between groups. ConclusionInitial AED failure was associated with temporal involvement in the CSD of CAE patients. This electrophysiological information may be helpful in clinical practice by estimating the efficacy of initial AED treatment in AED-naïve CAE patients in advance.

P84 – 2801: Two novel, disease-causing mutations in the ACSF3 gene in a patient with combined malonic and methylmalonic aciduria

Background Combined malonic and methylmalonic aciduria (CMAMMA) is a rare inborn error of metabolism with elevated excretion of malonic acid (MA) and methylmalonic acid (MMA) in urine commonly caused by mutations in the MLYCD gene leading to malonyl-CoA decarboxylase (MCD) deficiency. Clinical features are metabolic acidosis, seizures, axial hypotonia, developmental delay, failure to thrive, microcephaly, cardiomyopathy, elevated transaminases and gastrointestinal symptoms. Benign clinical courses and asymptomatic patients summarized as non-classical phenotype with mostly normal MCD activity are described. In these patients mutations in the ACSF3 gene encoding a methylmalonyl-CoA and malonyl-CoA synthetase are frequently detected. Case report We describe a 3-year-old boy diagnosed with CMAMMA at the age of 26 months. After an uneventful pregnancy a boy of unrelated Afghan parents was born at term with a birth weight of 2955 g. At three weeks he presented with two generalized seizures and metabolic acidosis. Plasma lactat level was normal. Initially moderately elevated levels of MMA were found in urine. Plasma amino acids, carnitine levels, acylcarnitine profile, homocysteine, folate, vitamin B12, liver and renal function parameters were normal. Initial electroencephalogram (EEG) was pathologic and anticonvulsive therapy with phenobarbital was initiated. No further seizures occurred, EEG normalized at the age of three months and antiepileptic medication was stopped. Besides microcephaly the development is age-appropriate up to now. Asymptomatic cholelithiasis persisted despite therapy with ursodeoxycholic acid. Echocardiography was normal. Genetic analysis revealed two novel, disease-causing mutations in the ACSF3 gene and led to the diagnosis auf CMAMMA. Excretion of MMA in urine decreased spontaneously. A mild protein restricted diet was started. A younger sister also presented generalized seizure and elevated MMA in urine at the age of four weeks. Her genetic analysis is in progress. Conclusion We found two novel mutations in the ACSF3 gene which we assume to cause a non-classic CMAMMA phenotype.

The butterfly pattern in a patient with chromosome 7 deletion.

The butterfly pattern in a patient with chromosome 7 deletion.

39.: Rapidly progressive dementia and protein 14-3-3 in cerebrospinal fluid: Not always Creutzfeldt-Jakob disease

This study aimed to illustrate difficulties of accurately diagnosing a rapidly progressive dementia syndrome and the unspecific nature of presence of 14-3-3 protein in the cerebrospinal fluid. An 84-year-old gentleman was transferred from a private rehabilitation hospital after a surgical repair of neck of femur fracture in a state of persistent drowsiness intermixed with lucid intervals with visual hallucinations. Initial electroencephalogram showed triphasic delta waves that did not change with gradual escalation of antiepileptic medication. MRI and bloods were unremarkable. His cerebrospinal fluid revealed normal proteins and protein 14-3-3, done through the Australian Creutzfeldt-Jakob Disease Registry in Melbourne, was positive. He was given a presumptive diagnosis of Creutzfeldt-Jakob disease and his family was counselled appropriately. He passed away in a nursing home 2 months later. Subsequent brain biopsy revealed no spongiform changes but intracytoplasmic Lewy bodies in neurons of frontal, temporal and parietal cortices. Despite widely held notion, positive 14-3-3 test is not diagnostic of Creutzfeldt-Jakob disease in a patient with rapidly progressive dementia.

Compound heterozygous BRAT1 mutations cause familial Ohtahara syndrome with hypertonia and microcephaly.

Homozygous frameshift BRAT1 mutations were found in patients with lethal neonatal rigidity and multifocal seizure syndrome (MIM# 614498). Here, we report on two siblings with compound heterozygous mutations in BRAT1. They had intractable seizures from neonatal period, dysmorphic features and hypertonia. Progressive microcephaly was also observed. Initial electroencephalogram showed a suppression-burst pattern, leading to a diagnosis of Ohtahara syndrome. They both died from pneumonia at 1 year and 3 months, respectively. Whole-exome sequencing of one patient revealed a compound heterozygous BRAT1 mutations (c.176T>C (p.Leu59Pro) and c.962_963del (p.Leu321Profs*81)). We are unable to obtain DNA from another patient. The p.Leu59Pro mutation occurred at an evolutionarily conserved amino acid in a CIDE-N (N-terminal of an cell death-inducing DFF45-like effector) domain, which has a regulatory role in the DNA fragmentation pathway of apoptosis. Our results further support that mutations of BRAT1 could lead to epileptic encephalopathy.

Presentation of an Anti-NMDAR-Encephalitis with Focal Motor Seizures and Focal Movement Disorder

Objective: This article aims to compare the clinical features of an uncommon manifestation of an anti-N-methyl d-aspartate (NMDAR) antibody encephalitis with other reported cases Method: A case report of a 2 + 7/12-year-old girl Result: We report a case of a 2 + 7/12 years old girl who presented with series of right focal tonic seizures without alteration of consciousness, later on following isolated perioral twisting and dyskinesia of the right hand without impaired consciousness. No additional symptoms of an anti-NMDAR-encephalitis appeared. Initial electroencephalograms (EEGs) were normal, developing a continuous deceleration of the left hemisphere within 3 weeks. Cerebral magnetic resonance imaging and cerebrospinal fluid (CSF) were normal. NMDAR antibodies were highly positive with 1:2,000 in serum, 1:64 in CSF. We gave three pulses of intravenous methylprednisolone within 3 months. Last follow-up 3/13 with good outcome and decreased NMDAR antibody titers. The EEG still showed a discrete intermittend deceleration, the right hand still showed a discrete athetosis. Neoplasm screening was negative. Interpretation: 1. The age of onset of an anti-NMDAR antibody encephalitis can be very early in childhood (40% of patients in literature are younger than 18 years, the youngest reported patient is 23 months old). 2. Psychological/mental alterations can be absent (although 87.5% of patients in the literature start with psychiatric alterations). 3. The symptoms of an anti-NMDAR antibody encephalitis such as movement disorders and motor seizures can be localized strictly unilateral-similar to Rasmussen encephalitis (so far reported in one 11 years old girl with anti-NMDAR antibody encephalitis). 4. The treatment of anti-NMDAR antibody encephalitis spans a longer unproved period of time. Rarely, monotherapy with intravenous methylprednisolone is sufficient—often an add-on therapy with rituximab is necessary. Currently, there does not exist any standard for childhood and adolescence treatment.

Analysis of clinical predictors of intractable childhood epilepsy

Objective To identify clinical predictive factors in intractable childhood epilepsy, to provide some evidence for its early diagnosis. Methods Six hundred and twenty-four children with newly diagnosed epilepsy were retrospectively identified in Xinhua Hospital Affiliated to Shanghai Jiaotong University Medical School(1993-2009). The epileptic children were divided into a drug-responsive epilepsy group(n=584) and an intractable epilepsy group(n=40). Clinical data of patients were retrospectively analyzed, including the gender, age, pathogen, inducement, number of pre-treatment seizures, seizure type, seizure type change, seizure duration, family history, previous history, physical examination, brain CT and initial electroencephalogram(EEG) findings.Single factor analysis and Logistic regression analysis were made in 2 groups. Results Strong univariate associations suggested that some factors could increase the risks of intractable epilepsy: symptomatic or cryptogenic epilepsy, mental retardation, type of infantile spasm, positive neurological examination and large absolute number of pre-treatment seizures, and changes in seizure types in the course of the disease.With multiple Logistic regression, independent predictors of intractability were symptomatic or cryptogenic etiology(OR=3.61), large absolute number of pre-treatment seizures and changes in seizure types in the course of the disease(OR=4.76). Conclusions It's necessary to be wary of intractable epilepsy and to adjust therapy accordingly when seizures were uncontrollable and accompanied by one or more conditions such as symptomatic or cryptogenic epilepsy, large absolute number of pre-treatment seizures, changes in seizure types in the course of the disease. Key words: Child; Intractable epilepsy; Predictors

Autism and Dietary Therapy

We report the history of a child with autism and epilepsy who, after limited response to other interventions following her regression into autism, was placed on a gluten-free, casein-free diet, after which she showed marked improvement in autistic and medical symptoms. Subsequently, following pubertal onset of seizures and after failing to achieve full seizure control pharmacologically she was advanced to a ketogenic diet that was customized to continue the gluten-free, casein-free regimen. On this diet, while still continuing on anticonvulsants, she showed significant improvement in seizure activity. This gluten-free casein-free ketogenic diet used medium-chain triglycerides rather than butter and cream as its primary source of fat. Medium-chain triglycerides are known to be highly ketogenic, and this allowed the use of a lower ratio (1.5:1) leaving more calories available for consumption of vegetables with their associated health benefits. Secondary benefits included resolution of morbid obesity and improvement of cognitive and behavioral features. Over the course of several years following her initial diagnosis, the child's Childhood Autism Rating Scale score decreased from 49 to 17, representing a change from severe autism to nonautistic, and her intelligence quotient increased 70 points. The initial electroencephalogram after seizure onset showed lengthy 3Hz spike-wave activity; 14 months after the initiation of the diet the child was essentially seizure free and the electroencephalogram showed only occasional 1-1.5 second spike-wave activity without clinical accompaniments.

A Case of Central Nervous System Infection Due to West Nile Virus Lineage-1 in Ankara Province, Turkey

West Nile virus (WNV) is a mosquito-borne Flavivirus (family Flaviviridae), maintained in an enzootic cycle between birds as amplifying hosts and mosquito vectors. While WNV exposure in humans frequently remain subclinical, a febrile illness called West Nile fever occurs in about 20% and neuroinvasive disease in less than 1% of the affected individuals. For the last two decades, WNV has caused outbreaks of severe neuroinvasive disease in humans and horses in Europe, the Mediterranean Basin and emerged in the American continent. Although, previous serosurveillance reports have revealed human WNV exposure in various regions in Turkey; well-characterized clinical cases have only been reported after 2009-2010. In this report, a case of WNV encephalitis caused by a Lineage 1 virus strain and identified in Ankara province, Central Anatolia, Turkey, was presented. An 87 year-old woman with a history of hypertension and a recent febrile episode was admitted to Hacettepe University Hospital in late May 2012, with altered consciousness, myoclonic jerks in facial muscles and left extremity. Hyponatremia and increased alanine and aspartate aminotransferase levels were noted in blood analyses. Initial electroencephalogram (EEG) demonstrated diffuse slow waves. Areas of restricted diffusion in right dorsal thalamus was observed in cranial magnetic resonance imaging (MRI). Despite supportive therapy, the patient's neurological condition worsened. Follow-up EEG displayed paroxysmal lateralizing epileptiform discharges (PLEDs) in the right hemisphere and T2-hyperintense lesions in the right temporoparietal cortex, insula and thalamus with components of cytotoxic and vasogenic edema were observed in MRI. A cerebrospinal fluid (CSF)-serum pair was evaluated to identify potential causes of encephalitis. CSF biochemical and microscopic findings were within normal limits except for decreased glucose levels. Bacterial, mycobacterial and fungal cultures, antigen assays and polymerase chain reaction (PCR) employed for Herpes simplex virus types 1 and 2 were negative. Commercial and in house assays for WNV, tick-borne encephalitis virus, Toscana virus (TOSV) antibodies revealed TOSV IgG in serum. Previously described nested PCRs targeting WNV envelope glycoprotein and phlebovirus consensus sequences demonstrated WNV positive results in serum and CSF, which were further characterized via cycle sequencing of amplicons as WNV Lineage 1 Clade 1a. Four serum samples obtained within 23 days after the diagnosis were negative for viral RNA and specific antibodies via commercial assays and WNV plaque reduction neutralization assay. During follow-up with supportive therapy and anti-epileptics, the patient's general and neurological condition improved mildly and control EEG and MRI demonstrated regression of previous lesions. However, the patient passed away on the 10th week of hospital admission due to nosocomial infections. These findings confirmed the inital data which indicated the circulation of WNV Lineage 1 strains in Central Anatolia, Turkey. WNV seroconversion may be delayed or absent in elderly individuals without overt diseases associated with immunosuppression. Thus investigation of WNV RNA together with the specific serological tests may help the accurate diagnosis of suspected cases.

Mitochondrial Depletion Causes Neonatal-Onset Leigh Syndrome, Myopathy, and Renal Tubulopathy

The authors describe a newborn with postnatal myopathy who subsequently developed feeding difficulties, ophthalmoplegia, ptosis, encephalopathy, and seizures. She became ventilator dependent after sudden apnea. The myopathy was without ragged red fibers in the muscle biopsy. An electron transport chain study showed a markedly generalized low level of enzyme activity, particularly in complexes I, I + III, and IV. An initial electroencephalogram finding was normal; subsequent electroencephalograms showed suppression bursts. The mitochondrial copy number in skeletal muscle was 2% of normal.

Nested Case-Control Study of Children Presenting with First Afebrile Seizure at KKH: Seizure Recurrence and Comorbidities

Introduction:First afebrile seizures are common in Singapore (incidence 24 per 100,000 person-years). We determined the seizure recurrence risk after first afebrile seizure, the risk factors at presentation and evaluated the quality of life of these children.Methods:Nested case-control study of children presenting with their first afebrile seizure from 2002 to 2004. Risk factors were measured at initial presentation. This follow-up study administered standardised telephone questionnaires to patients/caregivers seven years later. Subjects with only one afebrile seizure (1AS) were compared to those with recurrent afebrile seizures (RAS) for birth history, risk factors, developmental delay, school status, initial electroencephalogram (EEG), neuroimaging and initial seizure episode using Fisher's exact test. Quality of life was screened with a questionnaire.Results:Of 103 eligible subjects, 54 responded with seizures recurring in 21 (38.9%). Initial seizure frequency was yearly at 57.9%. The RAS group had more risk factors ( P=0.013) and greater frequency of abnormal initial development ( P &lt;0.005), abnormal neurological examination in this population ( P=0.033) and abnormal current development ( P=0.028). Quality of life differed in behavioural changes ( P=0.022) and parental relationship with spouses ( P=0.004). EEG and neuroimaging were not predictive. Number of initial seizure episodes showed a significant difference between 1AS and RAS groups ( P=0.037).Conclusion:Abnormal development and neurological examination are risk factors for seizure recurrence after the first afebrile seizure. Children with more than four seizures during the initial seizure presentation may be at an increased risk of seizure recurrence. Recurrent seizures affect quality of life of both the affected child and the family.

The onset of acute oxcarbazepine toxicity related to prescription of clarithromycin in a child with refractory epilepsy

The onset of acute oxcarbazepine toxicity related to prescription of clarithromycin in a child with refractory epilepsy

Analysis of status epilepticus related presumed encephalitis in children

Objective Encephalitis is an acute infection of brain parenchyma characterized clinically by fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity. Here we report an analysis of status epilepticus (SE) related presumed encephalitis in a series of children. Methods We retrospectively reviewed cases of SE related presumed encephalitis treated in the pediatric intensive care unit, between February 2002 and June 2006. Factors evaluated included age, sex, clinical symptoms, seizure type, presence of SE or refractory status epilepticus (RSE), initial electroencephalogram (EEG) finding, neuroimaging study, cerebrospinal fluid (CSF) and outcome. Results There were 46 patients (19 girls and 27 boys), aged 8 months to 16 years. Twenty (43.4%) of 46 children developed RSE. The major clinical symptoms included fever (100%), upper respiratory symptoms (56.5%) and altered level of consciousness (45.6%). The initial seizure type was categorized as focal (23.9%), generalized (34.8%), primary focal and secondary generalized (41.3%). Initial EEG revealed a focal (30.8%), or multifocal (19.2%) epileptiform discharge in the SE group and a focal (5%), or multifocal (70%) or generalized (25%) epileptiform discharge in the RSE group. The time of follow-up for this study was 6 months to 51 months. In the SE group, 4 died, 16 developed epilepsy and/or neurologic deficits, and 6 returned to baseline. In the RSE group, 6 died, 13 developed epilepsy and/or neurologic deficits, and none returned to baseline. All survivors were discharged on antiepileptic medications. Conclusions Our data indicated that children of SE related presumed encephalitis had a high mortality and morbidity. Outcome was related to multifocal or generalized abnormalities of the initial EEG and presence of RSE.