Quality of life at 6 years in children treated for West syndrome with hormonal therapy.

INTRODUCTION: West syndrome is a severe epileptic encephalopathy occurring in infancy. Majority of affected children suffer from poor epilepsy control in later life and are dependent on care-givers for daily living. There is no previous study evaluating the Quality of Life (QOL) in children suffered from WS. METHOD: A prospective cohort study was performed at six years in a group of children with West syndrome, followed up in the Sri Lanka Infantile Spasm Study (SLISS). The quality of life was evaluated using Sri Lankan Health-Related Quality-of-Life Index for school children (SLHRQ-S), an age-specific, primary caregiver proxy-rated, validated questionnaire for Sri Lankan children with epilepsy. Information on epilepsy, medication, and daily activities was obtained from the parents. RESULTS: Fifty parents of initial 97 children treated for WS participated. The majority had no ongoing epilepsy (56%) at time of evaluation. The mean QOL was 67.22 (SD 15.68). Mean QOL scores for individual domains showed that physical domain was the worst affected (58.51 (SD = 22.11)). Psychological and social function domains were 68.73 (SD = 17.74) and 75.2 (SD = 14.87), respectively. Male sex (0.02), using multiple anti-seizure medications (0.00) and lower ILAE epilepsy control scale (0.02) were significantly associated with a poor quality of life. Age at onset, delay in treatment, and early spasm control were among the factors that did not influence quality of life. CONCLUSION: Despite having control of their epilepsy in the majority, these children suffered from poor quality of life. The greater impact on the physical domain possibly is related to the effect of underlying pathologies.

Herpes Simplex Virus-2 Encephalitis Complicated with Multiple Cranial Neuritis and Dysautonomia.

Introduction: Herpes simplex encephalitis (HSE) is mainly caused by herpes simplex virus-1 infection (HSV-1). Herpes simplex virus-2 (HSV-2) infection is rare except in neonates or the immune-compromised. Cranial neuritis is rarely reported in association with HSE. This case study in an eleven-month-old followed by a literature review on cranial neuritis in HSE in children is presented due to the rarity of both situations. Case Report: An eleven-month old otherwise healthy infant presented with encephalitis due to HSV-2 infection which was complicated with dysautonomia manifesting as blood pressure fluctuations and tachycardia, and cranial neuritis manifesting as unilateral ptosis and palatal palsy. The clinical presentation of brain stem encephalitis was confirmed by the Magnetic Resonance Imaging findings of hyperintense foci and contrast enhancement in the medulla oblongata. Following treatment with acyclovir, he made a complete recovery. He did not have any clinical or laboratorial evidence suggestive of immune deficiency. Conclusion: HSV-2 infection can occur beyond the neonatal age group even in the absence of immune compromise. The brainstem encephalitis manifesting as cranial neuritis and autonomic dysfunction made a complete recovery.

Experience during COVID-19 lockdown and self-managing strategies among caregivers of children with epilepsy: A study from low middle income country.

PURPOSE: Abrupt halt of service provision due to pandemic state of COVID-19, significantly affected care of patients with chronic diseases like epilepsy; its impact being greater on caregivers of vulnerable groups such as children with epilepsy. We performed this study to describe difficulties posed by the lockdown to caregivers of children with epilepsy in a low-middle income country and describe their responses and self-management strategies to overcome difficulties and prepare for a recurrence. METHOD: A cross-sectional all-island survey was carried out at paediatric neurology centers in Sri Lanka. Data was gathered via a face-to-face interview after the lockdown period. Parental stress level was evaluated using a self-rating Stress Assessment Questionnaire. RESULTS: Caregivers of 140 children with epilepsy from seven centers served by paediatric neurologists were interviewed. Mean duration of epilepsy was 7.9 years(SD 4). Majority were on one (52.1 %) or two (20 %) anti-seizure medications regularly. The pandemic did not affect epilepsy control in majority (87.3 %), however, signficant proportion faced difficulties over regular reviews and presecription refills. Despite difficluties, 87.1 % of parents maintained dispensing anti-seizure medications to their child regularly. Caregivers demonstrated healthy self-management strategies such as awareness on medications and access methods to healthcare during lockdown and remained confident of accessability to services. Stress was experienced in < 5%. CONCLUSION: Lockdown status for COVID-19 did not significantly affect the control of epilepsy in children though it posed difficulties for regular reviews and obtaining medications. Self-management strategies will help caregivers to adopt to new-normal status and potential future outbreaks.