Transition of Care in Epilepsy.

Transition of care is the planned, coordinated movement from a child and family environment of pediatrics to a patient centered adult care setting. Epilepsy is a common neurological condition. While seizures remit in a proportion of children, in around 50% of children seizures persist into adulthood. Also, with advances in diagnostics and therapeutics, more children with epilepsy survive into adulthood, and need services of adult neurologists. Clinical guidelines from the American Academy of Pediatrics, American College of Family Physicians and American College of Physicians called for "supporting the healthcare transition from adolescence to adulthood", but this occurs in a minority of patients. There are several challenges to implementing transition of care at the level of the patient and family, pediatric and adult neurologist and with systems of care. Transition needs vary based on the type of epilepsy and epilepsy syndrome and presence of co-morbidities. Transition clinics are essential to effective transfer of care, but implementation remains extremely variable, with a variety of clinics or program structures in countries around the world. There is a need to develop multidisciplinary transition clinics, enhance physician education and establish national guidelines for this important process to be put into practice. Further studies are also needed to develop best practices and assess outcomes of well executed transition programs on epilepsy.

Transition of Care in Adolescents with Epilepsy: Perspective of Pediatric Neurologists in India.

This cross-sectional study was designed to determine the current status of transition of care for adolescents with epilepsy to adult neurological services in India and to understand pediatric neurologists' perspectives. After approval from the appropriate Ethics Committee, a pre-designed questionnaire was distributed electronically. Twenty-seven pediatric neurologists from 11 cities across India responded. Pediatric care ended under 15 y for 55.4% responders and at 18 y in another 40.7%. Eighty nine percent introduced the concept of transition or had transition discussions with their patients and parents. Majority of providers did not have a formal plan for transferring the children with epilepsy to an adult neurologist, and very few had transition clinics. Communication with adult neurologists was also variable. Several pediatric neurologists followed patients after transfer for varying periods of time. This study demonstrates increasing awareness regarding the importance of transition of care in this population.

Association of Child Neurology-Indian Epilepsy Society Consensus Document on Parental Counseling of Children with Epilepsy.

When a child is diagnosed with epilepsy, counseling regarding the same is done by the treating doctor. Most parents are frightened and have poor knowledge about epilepsy. Therapeutic advice including drug dosage, administration and side effects takes up the major part of physician's time, thereby neglecting important issues like home seizure management, follow up and others. These lacunae in knowledge require systematic patient and family education. To address these issues, an expert group meeting of pediatric neurologists and epileptologists in India along with social workers/epilepsy educators, legal experts, parents, and teachers was held. The various aspects regarding parental counseling in children with epilepsy were discussed and a consensus document was formulated. Here authors present the group consensus statement on counseling parents and caregivers of children with epilepsy. This document is intended to help physicians and pediatricians counsel the families when a child is diagnosed with epilepsy.

Speech and language delay in children: Prevalence and risk factors.

CONTEXT: Intelligible speech and language is a useful marker for the child's overall development and intellect. Timely identification of delay by primary care physicians can allow early intervention and reduce disability. Data from India on this subject is limited. AIMS: To study the prevalence and risk factors of speech-language delay among children aged 1-12 years. SETTINGS AND DESIGN: A cross sectional study was conducted at the Pediatric outpatient department of a teaching hospital. MATERIALS AND METHODS: Eighty four children (42 children with delayed speech and 42 controls) aged 1-12 years were included. The guardians of these children were requested to answer a questionnaire. History of the child's morbidity pattern and the risk factors for speech delay were recorded. The child's developmental milestones were assessed. STATISTICAL ANALYSIS USED: Data entry was analyzed using SPSS software, version 16. Standard statistical tests were used. A p value of less than 0.05 was taken as statistically significant. RESULTS: Speech and Language delay was found in 42 out of 1658 children who attended the OPD. The risk factors found to be significant were seizure disorder (P=< 0.001)), birth asphyxia (P=0.019), oro-pharyngeal deformity (P=0.012), multilingual family environment (P=< 0.001), family history (P=0.013), low paternal education (P=0.008), low maternal education (P=< 0.001), consanguinity (P=< 0.001) and inadequate stimulation (P=< 0.001). CONCLUSIONS: The prevalence of speech and language delay was 2.53%. and the medical risk factors were birth asphyxia, seizure disorder and oro-pharyngeal deformity. The familial causes were low parental education, consanguinity, positive family history, multilingual environment and inadequate stimulation.

Yoga in Children with Epilepsy: A Randomized Controlled Trial.

CONTEXT: Majority of epilepsy begins in childhood. Twenty to thirty percent of patients may not respond to antiepileptic drugs. Yoga as a complementary therapy has been found to be beneficial in adults, but has not yet been studied in children with epilepsy. AIM: To study the effect of yoga on seizure and electroencephalogram (EEG) outcome in children with epilepsy. SETTING AND DESIGN: A randomized controlled trial was conducted in the pediatric neurology outpatient department of a tertiary care teaching hospital. MATERIALS AND METHODS: Twenty children aged 8-12 years with an unequivocal diagnosis of epilepsy on regular antiepileptic drugs were enrolled. Yoga therapy was provided to 10 children (study group) and 10 children formed the control group. Yoga therapy was given as 10 sessions of 1h each. We compared seizure frequency and EEG at baseline, 3, and 6 months. Statistical analysis was carried out using standard statistical tests. A P value of <0.05 was considered significant. RESULTS: No children had seizures at the end of 3 and 6 months in the study group. In the control group, at 3 and 6 months, four and three children, respectively, had seizures. Eight children each in both the groups had an abnormal EEG at enrollment. At the end of 6 months, one EEG in the study group and seven in the control group were abnormal (P = 0.020). CONCLUSION: Yoga as an additional therapy in children with epilepsy leads to seizure freedom and significant improvement in EEG at 6 months.

Congenital Chikungunya--A Cause of Neonatal Hyperpigmentation.

A 12-day-old neonate presented with ill-defined dark pigmentation over the centrofacial area with flagellate pigmentation on the trunk and patchy pigmentation on the extremities. The mother had a history of fever starting a week before delivery and continuing for 3 days in the postpartum period. Together these led to consideration of a possible diagnosis of congenital chikungunya, which was confirmed according to the immunoglobulin M antibodies to chikungunya in the mother and child. The rare occurrence of cutaneous pigmentation was the only clue to the retrospective diagnosis of neonatal chikungunya. Chikungunya is an emerging viral disease that can be transmitted maternally during pregnancy and in the peripartum period. It can be added to the list of viral infections that can lead to fetal demise or, when present during labor and delivery, can cause neonatal disease with cutaneous signs.

Isolated intramuscular cysticercosis: a case report.

Human cysticercosis is caused by Cysticercus cellulosae, larvae of a tapeworm, Taenia solium. Cysticercosis can involve any tissue in the body; the most common affected sites are central nervous system, subcutaneous tissue, eyes, and muscles. A few cases of isolated intramuscular cysticercosis without any other tissue involvement have been reported in pediatric population. Here, we report a case of intramuscular cysticercosis diagnosed by ultrasonography in a 5.5 year-old boy who presented with the swellings over the calf and the scapular region, without any associated neurological or ocular involvement. The patient responded well to the course of steroids and Albendazole with complete resolution of both the swellings.

Haemophagocytic lymphohistiocytosis: a cause of unresponsive malaria in a 5-year-old girl.

A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytopenia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglyceridaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone.

Haemophagocytic lymphohistiocytosis: a cause of unresponsive malaria in a 5-year-old girl.

A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytopenia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglyceridaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone.

Hemophagocytic lymphohistiocytosis associated with cytomegalovirus infection in an immunocompetent infant: a diagnostic and therapeutic challenge!

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that results from inappropriate activation of the immune system. Many viral agents are known to trigger HLH but cytomegalovirus (CMV) associated HLH is rarely described. We report a case of CMV related HLH in a 3½ month old immunocompetent male infant who presented with fever, respiratory distress and hepatosplenomegaly. He had fulminant sepsis like course in the hospital as he continued to have hectic fever spikes, progressive pneumonia, increasing hepatosplenomegaly and multiple episodes of generalized convulsions. Investigations revealed bicytopenia, biochemical hepatitis, hyperferritinemia and hypofibrinogenemia. CMV IgM serology was reactive in both infant and mother. Diagnosis of CMV-HLH was made as per HLH 2004 diagnostic protocol. Infant was successfully treated with intravenous ganciclovir along with dexamethasone and etoposide.

Recent advances in neonatal seizures.

Neonatal seizures are the most important indicators of underlying brain injury. Seizures in a neonate are different from seizures in older children in many aspects including clinical presentation and etiology. The neonatal brain is immature and tends to have a decreased seizure threshold. Neonatal seizures are classified, based on their presentation as, clinical seizures, electroclinical seizures and electroencephalographic seizures; based on the pathophysiology as epileptic and nonepileptic seizures; and also on the basis of the etiology. Hypoxic ischemic encephalopathy is the leading cause of neonatal seizures, followed by intracranial hemorrhage, metabolic causes such as hypoglycemia and hypocalcemia, intracranial infections and strokes. Neonatal epilepsy syndromes are rare. Electroencephalography (EEG) is the gold standard for diagnosis. Amplitude integrated EEG (aEEG) is also used for continuous monitoring. The approach to management consists of initial stabilization of the neonate followed by treatment of potentially correctable injurious processes such as hypocalcemia, hypoglycemia and electrolyte disturbances, etiology specific therapies and antiepileptic drug (AED) therapy. Phenobarbital remains the first line AED therapy. Pharmacokinetic data on newer drugs is limited. Prognosis depends on the etiology, seizure type, neurological examination at discharge and EEG. Long term neurodevelopmental follow up is essential for babies with neonatal seizures.

Consensus guidelines on management of childhood convulsive status epilepticus.

JUSTIFICATION: Status epilepticus has a wide etiological spectrum, and significant morbidity and mortality. Management using a pre-determined uniform protocol leads to better outcomes. Multiple protocols for management of childhood status epilepticus are available, without much consensus. PROCESS: A 'Multi-disciplinary Consensus Development Workshop on Management of Status Epilepticus in Children in India' was organized. The invited experts included Pediatricians, Pediatric neurologists, Neurologists, Epileptologists, and Pediatric intensive care specialists from India, with experience in the relevant field. Experts had previously been divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed consensus on the topic. During the meeting, each group presented their recommendations, which were deliberated upon by the house and a consensus was reached on various issues; the document was finalized after incorporating suggestions of experts on the draft document. OBJECTIVE: To provide consensus guidelines on evaluation and management of convulsive status epilepticus in children in India (excluding neonatal and super-refractory status epilepticus). RECOMMENDATIONS: Each institution should use a pre-determined protocol for management of status epilepticus; pre-hospital management and early stabilization is the key to a satisfactory outcome of status epilepticus. Pharmacotherapy should not be delayed for any investigations; the initial management should consist of a parenteral benzodiazepine by any route feasible. Subsequent management has been detailed. The group also felt the need for more epidemiological research on status epilepticus from India, and identified certain research areas for the purpose.

Concurrent malaria and dengue Fever: a need for rapid diagnostic methods.

Malaria and dengue fever are endemic in the South-East Asian region including India. Both the illnesses share similar symptomatology, but differ in certain respects such as different- causative organisms and mosquito vector with diverse habitat. Hence, concurrent malaria and dengue fever in the same patient is said to be unusual. There have been cases of concurrent malaria and dengue, but they are scarce from highly endemic region like ours. Here, we describe three unusual cases of Plasmodium vivax and dengue co-infection diagnosed by use of rapid diagnostic tests. Early diagnosis and timely intervention is crucial in managing such patients.

Maternal ingestion of diclofenac leading to renal failure in newborns.

BACKGROUND: The use of non-steroidal anti-inflammatory drugs, such as indomethacin, ibuprofen, and nimesulide, during pregnancy has been reported to cause nephrotoxicity in the fetus. However, neonatal renal failure following antenatal exposure to diclofenac has not been reported in the literature. We report three cases of neonatal renal failure, including a pair of twins, following ingestion of diclofenac by the mother during pregnancy. CASE-DIAGNOSIS/TREATMENT: Cases 1 and 2 involved a pair of twins born to a mother with oligohydramnios. The first twin had nonoliguric renal failure with incomplete recovery at day 17 of life. The second twin developed anuria and hyperkalemia on day 2 of life, for which peritoneal dialysis was initiated. After 20 days of peritoneal dialysis, the second twin remained oligo-anuric, developed peritonitis, and died. Case 3 involved a female infant born to a primigravida with severe oligohydramnios. The baby developed oliguria and renal failure after birth, which was managed conservatively. Creatinine normalized by day 15 of life and remained normal at 1 year of age. Ultrasonography in the first week of life showed that all three infants had normal-sized kidneys. Both mothers had been administered diclofenac during pregnancy. CONCLUSIONS: In utero exposure to diclofenac may be associated with neonatal renal failure that may be transient or irreversible. We recommend that the use of diclofenac during pregnancy be avoided.