A cluster-randomized trial comparing home-based primary health care and usual clinic care for epilepsy in a resource-limited country.

OBJECTIVE: To ascertain whether home-based care with community and primary healthcare workers' support improves adherence to antiseizure medications, seizure control, and quality of life over routine clinic-based care in community samples of people with epilepsy in a resource-poor country. METHODS: Participants included consenting individuals with active epilepsy identified in a population survey in impoverished communities. The intervention included antiseizure medication provision, adherence reinforcement and epilepsy self- and stigma management guidance provided by a primary health care-equivalent worker. We compared the intervention group to a routine clinic-based care group in a cluster-randomized trial lasting 24 months. The primary outcome was antiseizure medication adherence, appraised from monthly pill counts. Seizure outcomes were assessed by monthly seizure aggregates and time to first seizure and impact by the Personal Impact of Epilepsy scale. RESULTS: Enrolment began on September 25, 2017 and was complete by July 24, 2018. Twenty-four clusters, each comprising ten people with epilepsy, were randomized to either home- or clinic-care. Home-care recipients were more likely to have used up their monthly-dispensed epilepsy medicine stock (regression coefficient: 0.585; 95% confidence intervals, 0.289-0.881; P = 0.001) and had fewer seizures (regression coefficient: -2.060; 95%CI, -3.335 to -0.785; P = 0.002). More people from clinic-care (n = 44; 37%) than home-care (n = 23; 19%) exited the trial (P = 0.003). The time to first seizure, adverse effects and the personal impact of epilepsy were similar in the two arms. SIGNIFICANCE: Home care for epilepsy compared to clinic care in resource-limited communities improves medication adherence and seizure outcomes and reduces the secondary epilepsy treatment gap.

Osteomalacic Myopathy in Children and Adolescents with Vitamin-D Deficiency.

BACKGROUND: Osteomalacic myopathy secondary to vitamin-D deficiency is an under-recognized cause of muscle weakness in children and adolescents. AIM: To describe a cohort of children and adolescents with osteomalacic myopathy. SETTINGS AND DESIGN: Pediatric neurology unit of a tertiary care hospital. METHODS AND MATERIAL: Charts of children and adolescents with osteomalacic myopathy were retrospectively reviewed for demographics, clinical presentation, laboratory investigations, and treatment response. Diagnosis of vitamin-D deficiency was made on the basis of a combination of clinical, biochemical, and radiographic findings. Response to treatment with vitamin-D confirmed vitamin-D deficiency as the cause of myopathic symptoms. RESULTS: Twenty-six children-15 girls and 11 boys aged between 20 months and 19 years-with osteomalacic myopathy were identified. Fifteen (58%) children were between 10 years and 19 years of age. Twenty-one (81%) children presented with myopathic symptoms of progressive walking difficulty, with eventual loss of ambulation in six. Four children came to attention through hypocalcemic seizures. One nonambulatory child with cerebral palsy presented with loss of previously attained ability to roll over and sit. All children had proximal muscle weakness on examination. Fifteen (58%) children had clinical signs of rickets. All the children who underwent biochemical (n = 24) and radiographic (n = 16) investigations had results consistent with vitamin-D deficiency. Only in one child, the diagnosis of osteomalacic myopathy was made on the basis of clinical findings. Response to vitamin D was uniformly good. CONCLUSIONS: Vitamin-D deficiency should be considered in the differential diagnosis of proximal myopathy in children and adolescents.

Clinical characteristics of epilepsy in resource-limited communities in Punjab, Northwest India.

OBJECTIVES: To describe clinical characteristics of a community-based epilepsy cohort from resource-limited communities in Punjab, Northwest India. METHODS: The cohort was gathered following a two-stage screening survey. We cross-sectionally examined and followed up the cohort for one year. A panel of neurologists assigned seizure types, syndromes, and putative etiologies and categorized drug responsiveness. RESULTS: The cohort of 240 included 161 (67.1%) men, 109 (45.4%) illiterates and 149 (62.1%) unemployed. Current age was >18 years in 155 (64.6%) but age at epilepsy onset was <18 years in 173 (72.1%). Epilepsies due to structural and metabolic causes were diagnosed in 99 (41.3%), but syndromic assignments were not possible in 97 (40.4%). After one year, drug-resistant epilepsy was established in 74 (30.8%). Perinatal events (n = 35; 14.6%) followed by CNS infections (n = 32; 13.3%) and traumatic brain injury (n = 12; 5.0%) were common risk factors. Most of those with CNS infections (n = 19; 63.3%), perinatal antecedents (n = 23; 76.7%), and other acquired risk factors (n = 27; 90.0%) presented with epilepsy due to structural and metabolic causes. Perinatal events were the putative etiology for nearly 40.7% of generalized epilepsies due to structural and metabolic causes and 28.2% of all epilepsies with onset <10 years. SIGNIFICANCE: Existing classifications schemes should be better suited to field conditions in resource-limited communities in low- and middle-income countries. The finding of drug-resistant epilepsy in nearly at least a third in a community-based sample underscores an unmet need for enhancing services for this segment within healthcare systems. Perinatal events, CNS infections, and head injury account for a third of all epilepsies and hence preventative interventions focusing on these epilepsy risk factors should be stepped up.

Epilepsy knowledge, attitudes, and practices among primary healthcare providers in an Indian district.

INTRODUCTION: Scaling up the involvement of primary care providers in epilepsy management in low- and middle-income countries (LMICs) requires an understanding of their epilepsy knowledge, attitudes, and practices (KAP). AIM: The aim of the study was to document levels of knowledge about, attitudes towards, and practices regarding epilepsy among different ranks of primary healthcare providers in a North-Western Indian district. METHODS: The survey included government medical officers (MOs), auxiliary nurse midwives (ANMs), and accredited social health activists (ASHAs). They were administered a specially designed KAP questionnaire. Responses were analyzed according to rank. RESULTS: The survey showed that nearly 10% of ANMs and almost a fifth of ASHAs had never heard about epilepsy. A quarter of MOs and over two-thirds of ANMs and ASHAs had never provided care to someone with epilepsy. There were significant differences in the levels of knowledge between the three groups of workers. CONCLUSIONS: Closing the huge gaps in KAP by educating primary care and community health workers about epilepsy should be a priority before engaging them in the epilepsy care delivery.

A home-based, primary-care model for epilepsy care in India: Basis and design.

OBJECTIVES: A cluster-randomized trial of home-based care using primary-care resources for people with epilepsy has been set up to optimize epilepsy care in resource-limited communities in low- and middle-income countries. The primary aim is to determine whether treatment adherence to antiepileptic drugs is better with home-based care or with routine clinic-based care. The secondary aims are to compare the effects of the two care pathways on seizure control and quality of life. METHODS: The home-based intervention comprises epilepsy medication provision, adherence reinforcement, and epilepsy self-management and stigma management guidance provided by an auxiliary nurse-midwife equivalent. The experimental group will be compared to a routine clinic-based care group using a cluster-randomized design in which the unit of analysis is a cluster of 10 people with epilepsy residing in an area cared for by a single accredited government grass-roots health care worker. The primary outcome is treatment adherence as measured by monthly tablet counts supplemented by two self-completed questionnaires. The secondary outcomes include monthly seizure frequency, time to first seizure (in days) after enrollment, proportion of patients experiencing seizure freedom for the duration of the study, and quality of life measured by the "Personal Impact of Epilepsy Scale," all assessed by an independent study nurse. RESULTS: The screening phase and neurologic evaluations and randomizations have been recently completed and follow-up is underway. SIGNIFICANCE: The results of the trial are likely to have substantial bearing on the development of governmental policies and strategies to provide coverage and care for patients with epilepsy in resource-limited countries.

Paediatric brainstem: A comprehensive review of pathologies on MR imaging.

The brainstem is a midline structure formed by the midbrain, pons and medulla and is a home for various vital neurological centres of the human body. A diverse spectrum of disease entities can involve the brainstem, which includes infections, metabolic disorders, demyelination, vascular conditions, neurodegenerative disorders and tumours. Brainstem involvement can be primary or secondary, i.e., as part of systemic disorders. Due to the overlapping clinical presentation and symptomatology, imaging plays a decisive role in the detection, localisation and characterisation of brainstem pathologies. Magnetic resonance imaging (MRI) is the modality of choice and the use of advanced MR techniques such as diffusion-weighted imaging and spectroscopy can be especially helpful in providing a tenable diagnoses. This article is a compilation of the MR imaging manifestations of a spectrum of common and uncommon brainstem pathologies that can be encountered in the paediatric age group. Teaching Points • The paediatric brainstem can be afflicted by many pathologies that may overlap clinico-radiologically. • MRI is the best modality for the localisation and diagnosis of brainstem pathologies. • Diffusion-weighted imaging is useful in the diagnosis of vascular and metabolic disorders. • Occasionally, demyelination and neoplasms can be indistinguishable on imaging.

Infantile tremor syndrome -- down but not out.

Retrospective chart review of 21 infants with infantile tremor syndrome for vitamin B12 deficiency showed low serum vitamin B12 levels in 8/16 (50%). Of the eight infants with normal levels, six had received vitamin B12 before referral. Macrocytosis and low maternal serum B12 was found in 12 and seven cases each. Treatment with vitamin B12 alone produced rapid recovery.

Treatment of cyanotic breath-holding spells with oral theophylline in a 10-year-old boy.

Cyanotic breath-holding spells are generally benign and resolve spontaneously by 4 to 5 years of age. Treatment with iron and other drugs has been employed in selected cases with very frequent and severe episodes. We describe a 10-year-old boy with recent-onset cyanotic breath-holding spells that were activity limiting. He was unable to participate in physical activities with his peers as any argument or emotional upset provoked these spells. Treatment with oral iron and piracetam was ineffective. However, treatment with oral theophylline produced dramatic amelioration of symptoms, and he was once again able to participate in play activities with his peers. We believe that general central nervous system stimulant and respirogenic effects of theophylline were instrumental in control of symptoms in our child.

A report of Joubert syndrome in an infant, with literature review.

Joubert syndrome (JS) is a rare autosomal recessive disorder with key finding of cerebellar vermis hypoplasia with a complex brainstem malformation that comprises the molar tooth sign on axial magnetic resonance images. This syndrome is difficult to diagnose clinically because of its variable phenotype. The exact diagnosis is often not made for several years after birth. This report shows that with the availability of magnetic resonance imaging (MRI), especially in developing countries like India, it is quite feasible to make an early diagnosis which may positively affect the subsequent management and outcome. We present a case of JS in a 7-month-old girl who presented to the pediatric outpatient clinic with developmental delay and abnormal eye movements. MRI showed molar tooth configuration of superior cerebellar peduncles, the fourth ventricle shaped like a bat wing and hypoplasia of the vermis which resulted in median approach of the two cerebellar hemispheres.

Magnetic resonance imaging findings in Reye syndrome: case report and review of the literature.

Magnetic resonance imaging findings in Reye syndrome have been reported only infrequently. A previously well 8-year-old boy presented with repeated episodes of vomiting and abdominal pain followed by altered sensorium and tonic spasms. This occurred 5 days after upper respiratory tract infection. His laboratory data revealed elevated liver enzymes, prolonged prothrombin time, and high blood ammonia levels. Magnetic resonance imaging of the brain done on the day of admission revealed diffuse cerebral edema and signal alterations in brainstem, bilateral thalami, medial temporal lobes, parasagittal cortex, and cerebellar and subcortical white matter. Diffusion restriction was seen in thalami, midbrain, cerebellar white matter, subcortical white matter, and parasaggital cortex in the watershed territory. The patient made a full recovery. Follow-up magnetic resonance imaging after a week revealed complete resolution of all except thalamic lesions. Although diffusion restriction in thalami and midbrain has been reported previously, this is the first report indicating diffusion restriction in subcortical white matter and the parasagittal cortex.

Sleep study abnormalities in children with attention deficit hyperactivity disorder.

The study objective was to describe polysomnographic findings in children with attention deficit hyperactivity disorder (ADHD) with diverse sleep problems. Polysomnographic data were retrospectively analyzed for 33 children (age 3-16 years) with ADHD who had sleep studies performed for diverse sleep complaints. Eight patients (24%) had obstructive sleep apnea, 10 (30%) had periodic limb movements of sleep, 8 (24%) had upper airway resistance syndrome, and 5 (15%) had obstructive hypoventilation. The ADHD group showed decreased sleep efficiency, increased arousal index, increased wake after sleep onset, decreased oxygen saturation nadir, and increased snoring, compared with control subjects. Compared with ADHD children without sleep disordered breathing, those who had sleep disordered breathing were significantly more obese and had more sleep architectural abnormalities (including increased sleep latency, increased rapid eye movement latency, increased wake after sleep onset, and increased arousal index with more oxygen desaturations), although total sleep time and sleep efficiency were not significantly different. Sleep disordered breathing and periodic limb movements of sleep appear to be common among children with ADHD who have symptoms of disturbed sleep.

Spectrum of polysomnographic abnormalities in children with epilepsy.

This study sought to evaluate polysomnographic abnormalities in a cohort of 40 children with epilepsy who underwent a sleep study because of various sleep complaints. Retrospective analyses included polysomnographic variables, antiepileptic drugs, type of epilepsy, and seizure control. The subgroup with epilepsy and obstructive sleep apnea syndrome was compared with 11 children who manifested uncomplicated obstructive sleep apnea syndrome. Thirty-three patients (83%) exhibited snoring (42.5%), sleep-disordered breathing (obstructive hypoventilation, 12.5%; obstructive sleep apnea, 20%; and upper-airway resistance syndrome, 7.5%), or periodic limb movements of sleep (10%). Children with poor seizure control demonstrated significantly lower sleep efficiency, a higher arousal index, and a higher percentage of rapid-eye-movement sleep compared with children who were seizure-free or exhibited good seizure control. Patients with epilepsy and obstructive sleep apnea had significantly a higher body mass index, longer sleep latency, a higher arousal index, and a lower apnea-hypopnea index, but significantly more severe desaturation compared with patients with uncomplicated obstructive sleep apnea. A significant proportion of children with epilepsy referred for polysomnography with diverse sleep problems manifest sleep-disordered breathing, including obstructive sleep apnea syndrome.

Intravenous levetiracetam in children with epilepsy.

Intravenous levetiracetam recently became available for use in patients aged >16 years. There are few data about its safety and efficacy in children. We retrospectively analyzed data from children treated with intravenous levetiracetam. Ten patients (6 female, 4 male), aged 3 weeks to 19 years, were treated with intravenous levetiracetam at a mean dose of 50.5 mg/kg/day for a mean duration of 4.9 days. Four patients received intravenous levetiracetam for acute repetitive seizures/status epilepticus, and three as replacement for oral levetiracetam because administration of oral levetiracetam was temporarily infeasible. One patient each received intravenous levetiracetam for seizure prophylaxis during brain biopsy, as maintenance treatment after acute seizures, and as substitute for sodium valproate. Three of four patients with acute repetitive seizures/status epilepticus became seizure-free; the fourth patient had a partial reduction in seizure frequency. All three patients who received intravenous levetiracetam as substitute for oral levetiracetam tolerated the switch well. The other three patients were seizure-free on intravenous levetiracetam. No serious adverse effects were observed, and all patients completed treatment with intravenous levetiracetam for the intended period. Intravenous levetiracetam may be effective in various clinical situations requiring intravenous administration of an antiepileptic drug.

Recurrent nocturnal tongue biting in a child with hereditary chin trembling.

A 13-month-old boy presented with repeated episodes of tongue biting during sleep. On evaluation, he was found to have hereditary chin trembling, a rare autosomal dominant condition characterized by continuous or intermittent tremulous activity of the mentalis muscle. This is the first report of this kind from India. The tongue biting appeared to be the result of parasomnia. Treatment with clonazepam was very effective; it completely ameliorated the symptom of tongue biting but had no effect on chin trembling.