Teaching NeuroImage: Glutaredoxin-5-Associated Variant Nonketotic Hyperglycinemia.

A 5-year-old boy presented with subacute motor regression since age 2.5 years. Examination revealed spasticity of bilateral lower extremities, generalized dystonia, and pseudobulbar palsy. Investigations revealed raised plasma lactate (2.5 mmol/L, normal range 0.8-1.5 mmol/L) and no evidence of sideroblastic anemia. Neuroimaging showed cavitating leukoencephalopathy with involvement of long tracts (corticospinal, spinothalamic tracts) and dorsolateral columns of cervicothoracic cord (Figures 1 and 2). A next-generation sequencing test identified a novel homozygous missense variant (c.171C > A, p.Phe57Leu) in exon 1 of the Glutaredoxin-5 (GLRX5) gene.

Nasogastric vs Orogastric Feeding in Stable Preterm (≤32 Weeks) Neonates: A Randomized Open-Label Controlled Trial.

BACKGROUND: Optimal route of tube feeding in preterm babies is not known. OBJECTIVES: To compare the frequency of bradycardia and desatu-ration episodes/hours in hemodynamically stable preterm neo-nates (≤32 wk gestational age) fed by nasogastric vs orogastric route. DESIGN: Randomized controlled trial. PARTICIPANTS: Hemodynamically stable preterm neonates (≤32 wk gestational age) requiring tube feeding. INTERVENTION: Nasogastric vs orogastric tube feeding. PRIMARY OUTCOME: Number of episodes of bradycardia and desaturations/hour. METHODS: Eligible preterm neonates fulfilling the inclusion criteria were enrolled. Each episode of insertion of a nasogastric tube or orogastric tube was labelled as a feeding tube insertion episode (FTIE). FTIE lasted from the time of insertion of tube till the time tube needed to be changed. Reinsertion of the tube in same baby was taken as a fresh FTIE. 160 FTIEs were evaluated during the study period, 80 FTIEs each in babies with gestational age <30 weeks and ≥30 weeks. Number of episodes of bradycardia and desaturation per hour were computed using records in the monitor till the time tube was in situ. RESULTS: The mean episodes of bradycardia and desaturations/hour [mean difference (95% CI) 0.144 (0.067-0.220); P<0.001] were higher in FTIE by nasogastric as compared to the oro-gastric route. CONCLUSION: Orogastric route may be preferable to the nasogastric route in hemodynamically stable preterm neonates.

Association of Child Neurology (AOCN) Consensus Statement on the Diagnosis and Management of Febrile Seizures.

JUSTIFICATION: Febrile seizures are quite common in children but there are controversies in many aspects of their diagnosis and management. METHODS: An expert group consisting of pediatric neurologists and pediatricians was constituted. The modified Delphi method was used to develop consensus on the issues of definitions and investigations. The writing group members reviewed the literature and identified the contentious issues under these subheadings. The questions were framed, pruned, and discussed among the writing group members. The final questions were circulated to all experts during the first round of Delphi consensus. The results of the first round were considered to have arrived at a consensus if more than 75% experts agreed. Contentious issues that reached a 50-75% agreement was discussed further in online meetings and subsequently voting was done over an online platform to arrive at a consensus. Three rounds of Delphi were conducted to arrive at final statements. RESULTS: The expert group arrived at a consensus on 52 statements. These statements pertain to definitions of febrile seizures, role of blood investigations, urine investigations, neuroimaging, electroencephalography (EEG), cerebrospinal fluid analysis and screening for micronutrient deficiency. In addition, role of rescue medications, intermittent anti-seizure medication and continuous prophylaxis, antipyretic medication and micronutrient supplementation have been covered. CONCLUSIONS: This consensus statement addresses various contentious issues pertaining to the diagnosis and management of febrile seizures. Adoption of these statements in office practice will improve and standardize the care of children with this disorder.

Association of Child Neurology (AOCN) - Indian Epilepsy Society (IES) Consensus Guidelines for the Diagnosis and Management of West Syndrome.

JUSTIFICATION: West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use. PROCESS: An expert group consisting of pediatric neurologists and epileptologists was constituted. Experts were divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed a consensus. The evidence was reviewed, and for areas where the evidence was not certain, the Delphi consensus method was adopted. The final guidelines were circulated to all experts for approval. RECOMMENDATIONS: Diagnosis should be based on clinical recognition (history/home video recordings) of spasms and presence of hypsarrhythmia or its variants on electroencephalography. A magnetic resonance imaging of the brain is the preferred neuroimaging modality. Other investigations such as genetic and metabolic testing should be planned as per clinico-radiological findings. Hormonal therapy (adrenocorticotropic hormone or oral steroids) should be preferred for cases other than tuberous sclerosis complex and vigabatrin should be the first choice for tuberous sclerosis complex. Both ACTH and high dose prednisolone have reasonably similar efficacy and adverse effect profile for West syndrome. The choice depends on the preference of the treating physician and the family, based on factors of cost, availability of infrastructure and personnel for daily intramuscular injections, and monitoring side effects. Second line treatment options include anti-epileptic drugs (vigabatrin, sodium valproate, topiramate, zonisamide, nitrazepam and clobazam), ketogenic diet and epilepsy surgery.

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.

Association of Child Neurology-Indian Epilepsy Society Consensus Document on Social and Legal Aspects of Childhood Epilepsy (SOLACE).

Epilepsy is a chronic neurological disorder which affects not only the health of the affected child, but also has an economic, psychological and emotional impact on the family as a whole. In the transition from Person with Disability (PWD) act (1995) to Rights of Persons with Disabilities act (RPWD act) (2016), which covers all aspects of life of a person with any disability, epilepsy has been excluded from the list of disorders, resulting in a loss of many of the benefits that were earlier available to persons with epilepsy, causing concern to all caregivers of persons with epilepsy. Additionally, physicians/ pediatricians/ neurologists are not really aware of the benefits that are available to persons with epilepsy, especially children. 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 implication of epilepsy being dropped as a disability, was discussed, and most of the experts concurred that epilepsy should be considered as a disability, depending of the type of seizures or the epilepsy syndrome. Also, the current status of income tax benefits, child care benefits, travel concession, schooling and health insurance for children with epilepsy in India were also discussed. The importance of creating awareness on these issues was stressed on. Here authors present the group consensus statement on these legal and social aspects about the care of children with epilepsy.

Recent advances in febrile seizures.

Febrile seizures are the most common seizures of childhood. A family history of febrile seizures is common, and the disorder is genetically heterogenous. While guidelines are available for management of simple febrile seizures, the management of complex febrile seizures is individualised. After a febrile seizure, it is important to rule out CNS infection and the decision to perform a lumbar puncture should be based on the clinical condition of the child. Neuroimaging and EEG are not required immediately in workup for simple or complex febrile seizures. Recurrence of febrile seizures may be managed at home by the parents with benzodiazepines. If the recurrences are multiple or prolonged and parents are unable to give home treatment, intermittent benzodiazepine prophylaxis may be given. Continuous antiepileptic prophylaxis may be given only to the children where intermittent prophylaxis has failed. Febrile seizures are also associated with increased risk of epilepsy, but this cannot be prevented by any form of treatment. There is also an increased risk of mesial temporal sclerosis, but whether this is an effect or cause of febrile seizures is as yet unclear. There is no increase in neurological handicaps or mortality following febrile seizures.

An easy method of attachment to an impacted canine.

BACKGROUND: Since many years, various bonding attachments have been used as a mode of traction for surgically exposed impacted teeth. It has always been a challenge to select an attachment considering predictability of the bonded attachment, mucogingival and periodontal conditions of the overlying tissues, and additional inventory requirement. METHODS: A 0.010-in. stainless steel ligature wire with eyelets at one end and spiral twisted at the other end was made and used as an attachment to guide surgically exposed impacted canine. RESULTS: Orthodontic guided eruption of an impacted canine of a 16-year-old patient using this simple attachment with 1-year follow-up illustrates adequate amount of attached gingiva. CONCLUSIONS: Ligature wire attachment is a simple inexpensive attachment that can be custom made without any need for additional inventory, besides being more comfortable to patients.

ISOLATED LEVOCARDIA WITH SITUS INVERSUS (A Case Report).

A case of isolated levocardia with situs inversus is reported. The child had a normally formed spleen, which is uncommon in such cases.