EEG findings and clinical severity and quality of life in non-epileptic patients with autism spectrum disorders.

Electroencephalogram (EEG) abnormalities could be seen in up to 60% of non-epileptic children with autism spectrum disorder (ASD). They have been used as biomarkers of ASD severity. The objective of our study is to identify EEG abnormalities in children with different degrees of ASD severity based on the Autism Treatment Evaluation Checklist (ATEC). We also want to assess the quality of life for children with ASD. All of the children underwent at least one hour of sleep-deprived EEG. Forty-five children were enrolled, of whom 42 were male. EEG abnormalities were found in 10 (22.2%) children, predominantly in the bilateral frontal areas. There were no differences in EEG findings among the mild, moderate, and severe ASD groups. The severity of ASD was associated with female sex (p-value = 0.013), ASD with attention deficit hyperactivity disorder (ADHD) (p-value = 0.032), ASD children taking medications (p-value = 0.048), and a lower Pediatric Quality of Life Inventory (PedsQL) (p-value <0.001). Social and emotional domains were the most problematic for health-related quality of life in ASD children, according to parent reports of PedsQL. Further studies with a larger sample size will help to clarify the potential associations between EEG abnormalities and the severity of ASD, as well as the impact on quality of life.

Cardiac dysfunctions in children with drug-resistant epilepsy.

Objective: There were reports of cardiac dysfunction that led to sudden unexpected death in epilepsy (SUDEP) in patients with epilepsy. Early detection of cardiac dysfunction can lead to early management to prevent sudden cardiac death in these patients. The objective of our study is to assess cardiac functions in children with drug-resistant epilepsy (DRE) compared with the normal population by using a standard echocardiogram (SE), tissue Doppler imaging (TDI) and myocardial strain evaluations (MSE). Method: Twenty-seven children who have been diagnosed with DRE based on the International League against Epilepsy (ILAE) were included in the study, along with 27 children whose ages match those of the normal control group. Results: Seventeen children, median age 12 years old, were using more than four anti-seizure medications. Structural brain lesions were the most common cause of epilepsy, 55.6% (15). Generalized tonic-clonic seizures were the most common seizure type, 55.6% (15). Children with DRE had a lower early mitral valve E wave inflow velocity compared with the control group (p < 0.05). They also had lowered early diastolic velocities (e') and myocardial performance index (MPI) when compared with the control group (p < 0.05). There was a statistically significant difference in left ventricular myocardial strain in children with DRE, with an average of -21.1 (IQR -23.5 and -19.4) and control, -25.5 (IQR -27.3 and -24.2). Significance: Children with DRE have an impairment of left ventricular diastolic function and myocardial strain, which could indicate decreased myocardial deformation and contraction compared with controls. These cardiological assessments can be used to evaluate children with DRE for early diagnosis and management of their cardiac dysfunction.

Epstein-Barr virus meningoencephalitis in a young immunocompetent child: A case report.

Epstein-Barr virus (EBV) usually causes mild, asymptomatic, and self-recovered infections in young children. Yet, neurological involvement of this virus has been reported. EBV meningoencephalitis is relatively rare in immunocompetent children. Herein, we describe a case of 2-year-old previously healthy girl presented with high-grade fever and exudative tonsillitis. Her neurological examination showed alteration of consciousness and neck stiffness. A history of generalized tonic-clonic seizures was noted. A diagnosis of EBV meningoencephalitis was definitely confirmed by a positive result for serum viral capsid antigen IgM, and a detection of EBV DNA in cerebrospinal fluid. Her neuroimaging studies demonstrated evidence of leptomeningeal enhancements along bilateral parietal cortical sulci and around the brainstem with a hypodense lesion in the left parietal area - the typical findings of EBV meningoencephalitis. This patient was treated with intravenous corticosteroid without antiviral agents. Her clinical symptoms gradually improved. She was discharged from the hospital on the 19th day of hospitalization without neurological sequelae. Although EBV is not a primary causative agent of meningoencephalitis in immunocompetent children, it should always be considered regardless of the presence or absence of classical infectious mononucleosis symptoms. Early recognition and properly treatment are important for a good prognosis.

Comparison of Clinical Manifestations, Laboratory, Neuroimaging Findings, and Outcomes in Children With Posterior Reversible Encephalopathy Syndrome (PRES) in Children With and Without Renal Disease.

BACKGROUND: To demonstrate and compare the clinical manifestations, laboratory findings, and neuroimaging findings of posterior reversible encephalopathy syndrome (PRES) in children with and without underlying renal disease. METHODS: The study included 23 children with a diagnosis of PRES from January 2009 to March 2019. All data, including clinical manifestations, laboratory findings, underlying medical illness, and neuroimaging results, were obtained. RESULTS: Sixteen had underlying renal disease. The median age of PRES onset was 10.3 years in children with renal disease and 9.8 years in children without renal disease. Higher blood pressure at the baseline, on admission, and at the onset of PRES was found in the renal disease group more than in the nonrenal disease group (P < 0.05). Seizures were likely seen in the renal disease group compared with the nonrenal disease group (P = 0.03). Generalized tonic-clonic seizures were the most common seizure type in both groups. An initial CT scan revealed vasogenic edema in 75% of the renal group and 85.7% of the nonrenal group. During a long-term follow-up, all children recovered without significant neurological deficits or subsequent epilepsy. CONCLUSIONS: Hypertension and higher baseline blood pressure are more common in children with renal disease who develop PRES compared with nonrenal disease. Seizures are more common in the renal disease group. A computed tomographic (CT) scan can help with PRES diagnosis when magnetic resonance imaging is not available. All children with PRES recovered without significant neurological deficits or subsequent epilepsy.

Comparison of Arterial Ischemic and Hemorrhagic Pediatric Stroke in Etiology, Risk Factors, Clinical Manifestations, and Prognosis.

BACKGROUND: Stroke is relatively rare in children but has a significant impact on long-term morbidity and mortality. There are limited data regarding the etiology, clinical manifestation, and prognosis of arterial ischemic stroke (AIS) and hemorrhagic stroke (HS) in children. OBJECTIVE: The aim of this study is to identify and compare etiology, risk factors, clinical manifestations, and prognostic outcomes between arterial ischemic and hemorrhagic pediatric stroke. METHODS: We retrospectively reviewed all hospital medical records and pediatric neurology database of 83 children who were first diagnosed with AIS and HS at the Pediatric Department, Chiang Mai University Hospital, Chiang Mai, Thailand between January 1, 2009, and December 31, 2018. All children were from 1 month to 18 years old. RESULTS: Fifty-one AIS (56%) and 32 (35.2%) HS were identified. The median age of onset was 6.9 years for AIS and 5.3 years for HS. Moyamoya disease/syndrome was the most common cause in AIS (21.6%). Rupture of cerebral arteriovenous malformation was the most common cause in HS (21.9%). More than one-third (39%) of children had multiple risk factors associated with stroke. Iron deficiency anemia was commonly found in children with AIS (39.2%). The majority of clinical presentations were hemiparesis (80.4%) for AIS and alteration of consciousness (68.8%) for HS. The median time to diagnosis exceeded 6 hours in both AIS and HS. The overall mortality rate of acute stroke was 5.1 per 100 person-years (95% confidence interval [CI], 2.9-9). The mortality rate was higher in HS compared with that in AIS with statistical significance (16.6; 95% CI, 8.9-30.8 vs 1.1%; 95% CI, 0.3-4.6 per 100 person-years). Thirty children (36.1%) developed epilepsy during the follow-up (median duration, 26 months). Recurrent stroke occurred in 1 child with AIS and 1 child with HS. CONCLUSIONS: Moyamoya disease/syndrome and arteriovenous malformation rapture are the most common cause of AIS and HS, respectively. Iron deficiency anemia was commonly found in childhood AIS. The time to diagnosis in both AIS and HS was delayed. The mortality rate in HS was higher than in AIS. Neurological deficits are seen in 70% of childhood AIS during the follow-up. One-third of the children in our study developed epilepsy, which generally responds to a single antiseizure medication. The recurrence rate of childhood stroke was low compared with adult stroke.

Risk factors of epilepsy in children with complex febrile seizures: A retrospective cohort study.

BACKGROUND: Febrile seizures are the most common seizures in children. Children with complex febrile seizures have a higher risk of subsequent epilepsy compared with children with simple febrile seizures. Multiple risks factors for epilepsy, including focal status epilepticus, family history of epilepsy, neurodevelopmental abnormalities and abnormal electroencephalogram findings, have been found with inconsistent results. The aim of this study is to identify risk factors for developing epilepsy in children with complex febrile seizures. METHODS: The study included 248 children aged 3-72-months, diagnosed with complex febrile seizures at Chiang Mai University Hospital. Demographic data, seizure characteristics, electroencephalogram and neuroimaging findings were identified, and assessed to establish whether they were risk factors for subsequent epilepsy. RESULTS: Fifty-five patients (22.1%) had subsequent epilepsy. Using Cox regression-survival analysis, factors associated with epilepsy were prolonged seizures >15 min (P = 0.006; Hazard Ratio (HR): 2.475; 95% Confidence Interval (CI): 1.294-4.735), developmental delay (P = 0.019; HR: 4.476; 95% CI: 2.280-15.646), epileptiform discharges on electroencephalogram (P = 0.023; HR: 1.391; 95%CI: 1.174-1.876), and abnormal neuroimaging (computed tomography or magnetic resonance imaging; P = 0.028; HR: 1.355; 95% CI: 1.034-1.776). Age at onset, peak febrile temperature, duration between the onset of fever and the occurrence of seizure, recurrent seizures within 24 h, focal seizures, abnormal neurological exams and family history of febrile seizure or epilepsy were not associated with increased risk of subsequent epilepsy in this study. CONCLUSIONS: Risk factors associated with increased risk of epilepsy in children with complex febrile seizures are prolonged seizures or febrile status epilepticus, developmental delay, electroencephalogram epileptiform discharges, and abnormal neuroimaging. Their presence would merit close clinical monitoring.

Pattern of Brain Injury Predicts Long-Term Epilepsy Following Neonatal Encephalopathy.

OBJECTIVE: To determine if patterns of hypoxic-ischemic brain injury on magnetic resonance imaging (MRI) in term newborns predict subsequent childhood epilepsy. METHODS: This retrospective cohort study includes term newborns with encephalopathy (n = 181) born between 2004-2012 and admitted to British Columbia Children's Hospital. MRI was performed between 3 and 5 days of age. The predominant patterns of hypoxic-ischemic injury were classified as Normal, Watershed, Basal Nuclei, Total, and Focal-Multifocal. Lesions in hippocampus, motor and occipital cortex were noted. RESULTS: Of 181 newborns, 166 (92%) survived the neonatal period, and 132 (80%) had follow-up with a median duration of 61 months (IQR: 28-95). Twenty-three children (17%) developed epilepsy. A higher proportion with Watershed, Basal Nuclei, or Total patterns developed epilepsy (P < .001). Injury to motor cortex, hippocampus, and occipital lobe (P < .01) were independent risk factors for epilepsy. In the adjusting logistic model, Watershed (odds ratio = 16.0, 95% CI [1.3, 197.2], P = .03) and Basal Nuclei injury (odds ratio = 19.4, 95% CI [1.9, 196.3], P = .01) remained independent risk factors. Therapeutic hypothermia did not alter these associations. Severity of brain injury and recurrent neonatal seizures are other clinical risk factors. SIGNIFICANCE: In term newborns with hypoxic-ischemic encephalopathy, the predominant pattern of Watershed and Basal Nuclei injury are valuable predictors for development of epilepsy in later childhood.

Manifestations of Tuberous Sclerosis Complex: The Experience of a Provincial Clinic.

BACKGROUND: Tuberous sclerosis complex (TSC) is a neurocutaneous disorder with a wide spectrum of manifestations. Recent consensus recommendations stress the importance of multidisciplinary management of children with TSC. The objective of this study was to examine the manifestations of TSC at a large referral centre to determine the care needs of this population. METHODS: A retrospective, systematic chart review was performed of children with TSC managed at British Columbia Children's Hospital. Patients were identified through epilepsy and clinical neurophysiology databases. RESULTS: The study population comprised 81 patients, born between 1987 and 2014, who were a median of 10 years (range, 0.2-23.2) at most recent follow-up. Epilepsy occurred in 91% of patients, including 32% with a history of infantile spasms. Nineteen patients underwent epilepsy surgery, nine (47%) of whom were seizure-free at most recent follow-up. Overall, 61% of epilepsy patients had been seizure-free for at least 1 year at the time of last follow-up. Neuropsychiatric disorders were diagnosed in 49% of children, with autism (25%), attention deficit hyperactivity order (19%) and anxiety (16%) being the most common. Cardiac rhabdomyomata occurred in 35% of children and renal angiomyolipomas were seen in 43%. A total of 91% had skin manifestations. CONCLUSION: This study outlines the multisystem manifestations of TSC, observed through a large pediatric referral center. Epilepsy and neuropsychiatric disorders are the major source of morbidity in this age group and provide many challenges to the treating clinician. Because a subset of the study population is still quite young, the prevalence of neuropsychiatric disorders is likely underestimated.