Jaya Shankar Kaushik

Aluminum phosphide (celphos) poisoning in children: A 5-year experience in a tertiary care hospital from northern India.

Background: Aluminum phosphide (ALP) (celphos) is an agricultural pesticide commonly implicated in poisoning. Literature pertaining to the clinical manifestations and treatment outcome of its poisoning among children is limited. Materials and Methods: A retrospective chart review was conducted of the medical records of 30 children aged less than 14 years admitted to pediatric intensive care unit (PICU) of a tertiary care hospital in northern India. Demographic, clinical, and laboratory parameters were recorded. The outcome was categorized into “survivors” and “nonsurvivors.” Results: The Mean (SD) age of the enrolled children [19 males (63.3%)] was 8.55 (3.07) years. Among the 30 children, 14 (46.67%) were nonsurvivors and the rest 16 (53.33%) were survivors. Nonsurvivors had ingested significantly higher doses of ALP (P < 0.001), and showed higher time lag to PICU transfer (P 0.031), presence of abnormal radiological findings on chest skiagram (P = 0.007), and a higher Pediatric Risk of Mortality (PRISM) III score (P < 0.001) at admission. Use of magnesium sulfate was associated significantly with survival [odds ratio (OR) (95% CI): 0.11 (0.02-0.66); P 0.016]. Conclusion: The present study highlights that survival among children with ALP poisoning is predicted by dose of ALP ingestion, time lag to medical attention, and higher PRISM score at admission. Use of magnesium sulfate could be associated with better survival among them.

Legal Provisions, Educational Services and Health Care Across the Lifespan for Autism Spectrum Disorders in India

India is estimated to have over 10 million persons with autism. Rising awareness of autism in India over last decade with ready access to information has led to an increase in prevalence and earlier diagnosis, the creation of services and some policy initiatives. However, there remains a gaping chasm between policy and implementation. The reach and quality of services continues sketchy and uneven, especially in the area of education. The present review discusses existing legal provisions for children and adults with autism in India. It also discusses Governmental efforts and lacunae in existing health care facilities and education services in India. While there are examples of good practice and stories of hope, strong policy initiatives have to support grassroots action to improve the condition of persons with autism in India.

Finger drop sign: Rare presentation of a common disorder.

BACKGROUND Guillain Barre syndrome (GBS) commonly presents with limb weakness and occasional cranial nerve, respiratory or autonomic involvement. Isolated or predominant bilateral finger drop as presenting feature has never been reported in the pediatric age group. CASE A 9-year-old boy presented with deformity of both hands for 7 days and leg pain with difficulty in getting up from floor for 3 days. On examination he had bilateral clawing with subtle hip flexor weakness and hyporeflexia. His nerve conduction study revealed motor axonal neuropathy. His serum lead levels and autoimmune markers were within normal limits. His cerebrospinal fluid examination revealed albuminocytological dissociation. He was diagnosed as GBS and was given intravenous immunoglobulin. He improved completely over next 8 weeks. CONCLUSIONS GBS is one of the commonest causes of acquired neuropathy in the tropics. In resource limited setting, where electrophysiological facilities may not be available, identification of finger drop sign may help in correct management.

Perception of One-Minute Preceptor (OMP) Model as a Teaching Framework among Pediatric Postgraduate Residents: A Feedback Survey

To the Editor: One-Minute preceptor (OMP) model is an efficient teaching framework that includes five micro skills of getting a commitment from the learner about his or her impression of the case, probing for underlying reasoning to explore the learner’s understanding, teaching the general rules pertaining to the case, giving positive feedback and correcting the learner’s errors [1]. OMP model has been well studied in an outpatient setting and inpatient setting with some modifications [2–4]. Three faculty members and three postgraduate residents (5th semester) of Pediatrics department were trained on OMP model by a faculty member well versed with OMP model [1]. Three Pediatric postgraduate residents of the 5th semester were allotted three different clinical cases of children with global developmental delay. Discussion based on OMP model was conducted over 10–15 min for each case. All other pediatric residents of all semester naïve to this model were invited to attend as observers. A total of 23 out of 25 Pediatric residents who attended the session responded to feedback. All respondents (n = 23) believed that OMP ascertains the student’s diagnosis and assesses student’s underlying clinical reasoning. Among 23 respondents, 18(78.3%) strongly felt that OMP assesses students’ fund of knowledge; 20(87.0%) strongly agreed to the point that it teaches student few key points for use in future patient care; 22(95.6%) felt that it provides constructive feedback with recommendations for improvements; 20 (87.0%) agreed that it involves the students in decision-making process. All (n = 23) respondents strongly believed that OMP improves the efficiency and overall effectiveness of the teaching encounter. All respondents (n = 23) wanted this model to be incorporated into Pediatric postgraduate teaching program. OMPmodel of learning encourages students to think about the diagnosis and management, gives them an immediate feedback on their thinking, and helps them rectify their mistakes [5]. Detailed analysis of points in history and examination are strengths of traditional case presentation that often cannot be replaced. Hence, OMP model could be an effective supplement to traditional case presentation in improving analytical skills of Pediatric postgraduate residents. We conclude that OMP was a satisfactory learning experience for Pediatric postgraduate residents.

A Combination of Moyamoya Pattern and Cerebral Venous Sinus Thrombosis: A Case of Tubercular Vasculopathy

BACKGROUND Moyamoya vasculopathy, arising secondary to tubercular meningitis (TBM) is unusual. There have also been a few reports of cerebral venous sinus thrombosis (CVST) in TBM. A case of TBM, complicated simultaneously by Moyamoya syndrome and CVST, is being presented here. CASE A 1-year-old girl presented with febrile encephalopathy, vomiting, seizures and left hemiparesis. Cerebrospinal fluid analysis was suggestive of TBM. Extensive infarcts were noted in the magnetic resonance imaging, involving right middle cerebral artery (MCA), anterior cerebral artery and the left MCA. Magnetic resonance venogram revealed left transverse venous sinus thrombosis and magnetic resonance angiography showed bilateral moyamoya pattern of arteriopathy. Patient was started on antitubercular therapy and low molecular weight heparin. CONCLUSIONS Early vascular involvement affecting both arterial and venous structures has not hitherto been reported in CNS tuberculosis. Early recognition of secondary complications of CNS tuberculosis is crucial to prevent the morbidity and mortality associated with TBM.

Unusual late neurological complication in a child after an Indian krait bite.

BACKGROUND Neurological manifestations of elapid snakebites include neuromuscular paralysis and cerebrovascular complications. Autonomic manifestations are observed in almost two third of patients following moderate to severe envenomation. PATIENT SUMMARY A 10-year-old boy presented with acute onset flaccid quadriparesis with encephalopathy, cranial neuropathy, and respiratory failure after bite of a common Indian krait. He also had features of autonomic instability in the form of hypertension and variable heart rate. Within 10 days, he was weaned from the ventilator and discharged on multiple oral antihypertensives. Within a week, he returned with focal status epilepticus. MRI of the brain suggested posterior reversible leukoencephalopathy. He recovered completely within 2 days with visual impairments while recovering. Within next 1 month, his antihypertensives were tapered completely. MRI of the brain, repeated after 3 months, confirmed complete resolution. CONCLUSIONS This patient highlights the fact that posterior reversible leukoencephalopathy can be a late complication of Indian krait bite secondary to autonomic instability with systemic hypertension.

Unusual presentation of Sturge-Weber syndrome: Progressive megalencephaly with bilateral cutaneous and cortical involvement

The Sturge Weber syndrome is characterized by developmental delay, seizures in infancy, unilateral cutaneous lesions with ipsilateral leptomeningeal enhancement. We report an unusual presentation of Sturge Weber syndrome with bilateral port wine nevus on the trunk and face along with bilateral cortical involvement in a developmentally normal child with progressive megalencephaly.

Unilateral transient watershed cerebral infarct in a 6-year-old girl with frequently relapsing nephrotic syndrome

Nephrotic syndrome is a prothrombotic state with predisposition to venous sinus thrombosis and arterial vascular stroke. Watershed infarcts in junction of arterial territory develop in hypotensive hypovolemic state. These border zone infarcts are usually bilateral in the absence of unilateral arterial stenosis or microembolism. We report a 6-year-girl of frequently relapsing nephrotic syndrome who developed sudden onset hemiparesis with aphasia. Magnetic resonance (MR) imaging brain revealed unilateral watershed infarct in territory between the major cerebral arterial vessels with evidence of restricted diffusion and normal vessel anatomy on MR angiography. This could possibly reflect asymmetric variant of posterior reversible encephalopathy syndrome that resolved with remission on steroids.

Paroxysmal Kinesigenic Dyskinesia

A 14-year-old boy presented with history of multiple daily episodes of abnormal movements of the body for the last 2 years. The event consisted of sudden onset of uncontrollable, bizarre, twisting movement of both arms and legs lasting for 10-30 seconds with no impairment of consciousness. These events were often precipitated by sudden movement. There was no family history of similar events. His neurological examination, magnetic resonance imaging and electroencephalography were unremarkable.

Peripheral Neuropathy in Thalassemia Major

To the Editor: Neurological complications, including peripheral neuropathy have been described in adolescents and adults with thalassemia major. However, its prevalence in children and the impact of effective chelation on the development of peripheral neuropathy is mostly undetermined. These complications have been attributed to various factors such as iron overload, chronic hypoxia, and drug-induced neurotoxicity with desferrioxamine [1]. This cross-sectional study included children aged 5–15 y with transfusion-dependent thalassemia major. Children on any form of vitamin supplementation (except folic acid) in preceding six months, those with known neuromuscular disease or with a family history of inherited muscle/nerve disease were excluded from the study. Awritten informed consent was obtained, and the study was approved by the Institutional ethical committee. Data were categorized into two groups: effective chelation (Serum ferritin level < 2500 ng/ml) and ineffective chelation (Serum ferritin level > 2500 ng/ml) groups. All patients were subjected to detailed age appropriate neurological examination and detailed nerve conduction study for any clinical evidence of mono or polyneuropathy. Mean (SD) age of enrolled patients (n = 50) was 10.5 (4.5) y with a male predominance [36 (72%)]. Mean (SD) hemoglobin was 8.9 (1.2) g/dl. Majority of children were on deferasirox [30 (60%)]. None of the children had either clinical or electrophysiological evidence of peripheral neuropathy. Electrophysiological parameters including compound muscle action potential (CMAP), sensory nerve action potential (SNAP), and conduction velocity (CV) of the tested motor and sensory nerves were comparable between the two groups of effective and ineffective chelation. Prevalence of electrophysiological peripheral neuropathy ranged from 18.6 to 76% among adolescents and adults with thalassemia major [1–4]. The study findings are consistent with previous studies showing a lack of significant correlation of electrophysiological parameters with serum ferritin levels [2, 5]. Based on this preliminary cross-sectional study with a limited sample size, we conclude that children with beta-thalassemia major did not demonstrate any clinical or electrophysiological evidence of peripheral neuropathy. Peripheral neuropathy shown in thalassemia patients is probably agedependent and does not develop until adolescence. Further research would be of benefit with a larger sample size and age-matched controls.