Scrub Typhus Associated with Guillain–Barré Syndrome (GBS)

Scrub typhus is a vector-borne disease caused by Orientia tsutsugamushi. Clinical manifestations generally occur due to vasculitis and infammation and can have variable degrees of systemic involvement. Meningoencephalitis and cerebellitis are well-known neurological manifestations of scrub typhus, but the occurrence of Guillain–Barré syndrome is extremely rare. The authors report a 7-y-old boy who developed fever followed by rapidly progressive ascending quadriparesis with arefexia and whose etiological workup revealed positive IgM scrub typhus antibody, as well as, a high OXK titer (1:80). Nerve-conduction studies in all four limbs were suggestive of demyelinating neuropathy. He showed complete recovery after treatment with intravenous immunoglobulin (2 g/kg) and azithromycin.

Epilepsy and Neurodevelopmental Outcomes in a Cohort of West Syndrome Beyond Two Years of Age

Objective To determine epilepsy and neurodevelopmental outcomes beyond 2 y of age and their putative prognostic factors in children with West syndrome (WS). Methods This cross-sectional study was initiated after approval from Institutional Ethics Committee. A follow-up cohort of 114 children (aged?2 y) diagnosed and treated for WS at the authors' center were assessed in-person for epilepsy and neurodevelopmental outcomes using Vineland Social Maturity Scale - Malin’s adaptation for Indian children. Subsequently, age at onset, lead-time-to-treatment, etiology, and response to any of the standard therapies were analyzed as possible predictors of these outcomes. Results Of 114 children (mean age: 55±32 mo, 91 boys), structural etiology was the predominant underlying etiology (79.8%) for WS. At 2 y of age, 64% had ongoing seizures. At the last follow-up, 76% had social quotient<55, and 39% had cerebral palsy (spastic quadriparesis in 21%). An underlying structural etiology was associated with ongoing seizures [OR (95% CI) 3.5 (1.4–9); p=0.008] at 2 y of age and poor developmental outcomes [OR (95% CI): 3.3 (1.3–8.9); p=0.016]. Complete cessation of spasms with the standard therapy was signifcantly associated with better seizure control [OR (95% CI): 5.4 (2.3–13); p<0.001] and neurodevelopmental outcome [OR (95% CI): 5.2 (1.8–14.9); p<0.001]. Conclusion The majority of children with WS have a poor neurodevelopmental outcome and epilepsy control on follow-up. The underlying etiology and response to initial standard therapy for epileptic spasms have a prognostic role in predicting the neurological outcome in these patients on follow-up.

Age at Diagnosis for Autism Spectrum Disorders: Does it Differ by Place of Residence?

Background: Despite steady decline in the age of diagnosis (AOD) at the global level, it has not declined uniformly, and marked disparities are documented by income, education, race, and access to health care. Objectives: The objectives of the study are to examine the urban/rural disparities in the initial age of autism diagnosis and to understand the interplay of the underlying demographic and social factors. Methods: A retrospective case record review of all children who received their initial diagnosis of autism at the Pediatric Psychology Clinic (1997–2018) of a tertiary advanced pediatric center at Chandigarh was conducted. Astructured abstraction data form was used to extract demographic, socioeconomic, and clinical information from the files maintained at the clinic. Results: A total of 1321 case records were examined. The mean AOD was 4.62 years (standard deviation = 2.38) and children from rural communities were diagnosed at 4.87 years, nearly 0.35 years later than urban children (t = 2.47, P = 0.013). Results indicated that 31.1% of the variance in the AOD for children from rural areas was predicted by two variables, namely the number of children in the family and total Childhood Autism Rating Scale (CARS) score (F = 13.62, P = 0.001). For the urban sample, three variables emerged as significant predictors including the number of children in the family, total CARS score, and maternal education and these together explained 20.2% of the variance in the AOD (F = 19.60, P = 0.001). Conclusion: The public health system must be sensitized to the unmet needs of the marginalized socioeconomic groups to access diagnostic and management services in a timely manner.

Full Outline of Unresponsiveness score and the Glasgow Coma Scale in prediction of pediatric coma / 世界急诊医学杂志（英文）

@#BACKGROUND: This study was done to compare the admission Full Outline of Unresponsiveness (FOUR) score and Glasgow Coma Scale (GCS) as predictors of outcome in children with impaired consciousness. METHODS: In this observational study, children (5–12 years) with impaired consciousness of <7 days were included. Children with traumatic brain injury, on sedatives or neuromuscular blockade; with pre-existing cerebral palsy, mental retardation, degenerative brain disease, vision/hearing impairment; and seizure within last 1 hour were excluded. Primary outcomes: comparison of area under curve (AUC) of receiver operating characteristic (ROC) curve for in-hospital mortality. Secondary outcomes: comparison of AUC of ROC curve for mortality and poor outcome on Pediatric Overall Performance Category Scale at 3 months. RESULTS: Of the 63 children, 20 died during hospital stay. AUC for in-hospital mortality for GCS was 0.83 (CI 0.7 to 0.9) and FOUR score was 0.8 (CI 0.7 to 0.9) [difference between areas –0.0250 (95%CI 0.0192 to 0.0692), Z statistic 1.109, P=0.2674]. AUC for mortality at 3 months for GCS was 0.78 (CI 0.67 to 0.90) and FOUR score was 0.74 (CI 0.62 to 0.87) (P=0.1102) and AUC for poor functional outcome for GCS was 0.82 (CI 0.72 to 0.93) and FOUR score was 0.79 (CI 0.68 to 0.9) (P=0.2377), which were also comparable. Inter-rater reliability for GCS was 0.96 and for FOUR score 0.98. CONCLUSION: FOUR score was as good as GCS in prediction of in-hospital and 3-month mortality and functional outcome at 3 months. FOUR score had a good inter-rater reliability.

Neurocysticercosis in children.

Neurocysticercosis (NCC) is a common cause of seizures and neurologic disease. Although there may be variable presentations depending on the stage and location of cysts in the nervous system, most children (> 80%) present with seizures particularly partial seizures. About a third of cases have headache and vomiting. Diagnosis is made by either CT or MRI. Single enhancing lesions are the commonest visualization of a scolex confirms the diagnosis. Some cases have multiple cysts with a characterstic starry-sky appearance. Management involves use of anticonvulsants for seizures and steroids for cerebral edema. The use of cysticidal therapy continues to be debated. Controlled studies have shown that cysticidal therapy helps in increased and faster resolution of CT lesions. Improvement in long - term seizure control has not yet been proven. Children with single lesions have a good outcome and seizure recurrence rate is low. Children with multiple lesions have recurrent seizures. Extraparenchymal NCC has a guarded prognosis but it is rare in children. In endemic areas NCC must be considered in the differential diagnosis of seizures and various other neurological disorders.

Impact of parent and teacher concordance on diagnosing attention deficit hyperactivity disorder and its sub-types.

OBJECTIVE: This study examines the extent to which parents and teachers agree on the diagnosis of Attention Deficit Hyperactivity Disorder (ADHD) and its subtypes, as per the DSM IV criteria. It assesses whether the extent of agreement between informants improves by making the ADHD criteria more flexible. METHODS: Parents and teachers of 119 clinic-referred sample of children (mean age=8.4 years, S.D= 2.48) with disruptive behavioral symptoms completed the Vanderbilt Attention Deficit Hyperactivity Disorder Diagnostic Parent and Teacher Rating Scales, respectively. Concordance of parent and teacher reports for the presence or absence of diagnosis of ADHD and type of ADHD was examined by percent agreement and the kappa statistics. RESULTS: Of the 119 children referred for disruptive behavior disorders, 96 (80.6%) met criteria for any type of ADHD according to the parents' report; and only 68 (57.1%) met criteria according to the teachers' report. Parent and teacher agreement for the diagnosis of any type of ADHD was only 52% (k= .11, n.s); and the agreement regarding diagnosis of sub-type was even poorer. Making the criteria more flexible vis-à-vis impairment or number of symptoms did not improve agreement between the informants. CONCLUSION: Clear guidelines are needed to reconcile the differences between informants in order to promote uniform diagnostic practices among clinicians working with children having ADHD.

Stroke following a bicycle injury.

Stroke is not an uncommon problem in children. However, stroke following intraoral trauma is rare. A high index of suspicion of neurological complications following apparently asymptomatic oral trauma helps to identify this peculiar form of childhood stroke.

Predictors of long term neurological outcome in bacterial meningitis.

OBJECTIVE: To study the long-term neurological and developmental outcome and the clinical and laboratory predictors of sequelae in children with acute bacterial meningitis (ABM). METHODS: Detailed clinical and demographic data was retrieved from the medical records of study children. Subsequently they were followed up for a minimum of 12 months after discharge for development, neurological and hearing assessment. All sequelae were identified and divided into minor or major. For analysis data was divided into 2 groups those with sequelae and without sequelae at follow-up. Statistical analysis was done using SPSS version 10.00 and Epi Info version 2000. RESULTS: 61 boys and 19 girls, a mean age of 31.4 +/= 41.9 months at the time of ABM, were included in the study. Of these 62.5% children were infants. Mean age at follow-up was 58.6 +/= 47.2 months. Sequelae were observed in 32 (40%) children (8 (10%) minor and 24 (30%) major). Mean social quotient at follow-up was 92.8 +/= 32.6. Developmentally 22 (37.9%) children were normal and 20 (34.5%) had global delay. Seizures (P=0.015), cranial nerve palsy (P=0.0065), abnormal deep tendon reflexes (P=0.002), Glasgow coma scale score (GCS) < 8 (P = 0.044) at admission, a CSF culture positive for bacteria and abnormal findings on ultrasonography or computed tomography of head at admission had significant association with sequelae at follow-up. All children (7/7) who had infarct on CT scan (P=0.001) and 12 (80%) of 15 patients who had hydrocephalus (OR - 9.0, 95% CI - 2.03-45.6, P=0.001) diagnosed on CT scan developed severe sequelae. On multiple regressions GCS score <8, presence of cranial nerve palsy and abnormal deep tendon reflexes were independent predictors of sequelae. CONCLUSION: Neurological and audiological sequelae and global developmental delay may be seen in about one third of survivors of bacterial meningitis. GCS score <8, presence of infarct or cranial nerve palsy, or hydrocephalous on CT/ ultrasound at admission may help in identification of children most likely to need long term follow up and rehabilitation.

Patterns of development in young children with autism.

OBJECTIVE: To determine the extent to which the developmental profile of children less than 4 years can help in distinguishing children with autism from children with developmental delay. METHODS: Subjects were 32 children with autism as per the DSM IV criteria and 32 children with developmental delay matched on chronological and academic age. The Developmental Profile II was used to assess the developmental functioning in five domains including physical, social, self help, academic, and communication. RESULTS: The two groups showed significantly different developmental profiles and these differences were accounted for mainly by significantly lower social skills and superior motor skills in the autistic group as compared to the developmentally delayed group. CONCLUSION: Developmental Profile II may help in distinguishing young children with autistic disorder from non-autistic children with comparable developmental delays.

Predictive value of electroencephalography and computed tomography in childhood non-traumatic coma.

OBJECTIVES: To study value of electroencephalogram (EEG) and computed tomography (CT Scan) in predicting outcome of non-traumatic coma in children. METHODS: 100 consecutive children, between 2 months to 12 years, with non-traumatic coma, (Glasgow Coma Scale score < 8). Demographic and clinical data was recorded at admission. EEG and CT scan were done within 24 hours of admission. Etiologic diagnosis was assigned on basis of clinical data and relevant laboratory investigations. The outcome was recorded as survived and died. Among survivors it was graded as no disability, or mild, moderate, or severe disability. Odds ratio and/or relative risk (RR) with 95% confidence interval (C I) were calculated. RESULTS: EEG could be done in 60 patients (43 survived; 7 were normal, 8 had mild, 17 moderate and 11 severe disability) CT scan in 93 patients (60 survived; 11 were normal, 14 had mild, 21 moderate and 14 severe disability). A normal/borderline EEG was associated with good outcome (P = 0.001); 11 of 12 survived and of survivors 55% had no or mild disability. Electrocerebral silence on EEG was a predictor of death (OR = 44 -sub .95% Cl - 1.5-7372; P = 0.01). An abnormal EEG was associated with significant increase in risk of disability among survivors (RR = 2.6, 95% Cl = 1.2-5.4, P = 0.03). Among CT abnormalities intracranial bleed suggested increased risk of death (RR = 2.1; 95% Cl - 0.8-5.3; P = 0.058), while, hydrocephalus was associated with better survival (RR = 0.7; 95% CI - 0.5 to 0.96; P = 0.029). However, hydrocephalus when compared with other abnormal CT scan findings, was associated with higher risk of moderate and severe disability among survivors (P = 0.046) CONCLUSION: A normal CT scan and EEG, and some of the specific findings could be helpful in predicting outcome in children with non-traumatic coma. EEG and CT scan should be done at admission in all patients with non-traumatic coma if feasible.

Non traumatic coma.

OBJECTIVE: To study the etiology and clinical profile of non-traumatic coma in children and to determine the clinical signs predictive of outcome. METHODS: 100 consecutive cases of non-traumatic coma between 2 months to 12 years. Clinical signs studied were temperature, pulse, heart rate, blood pressure, coma severity by Glasgow coma scale (GCS), respiratory pattern, pupillary and corneal reflex, extra ocular movements, motor patterns, seizure types and fundus picture. These were recoded at admission and after 48 hours of hospital stay. Etiology of coma was determined on basis of clinical history, examination and relevant laboratory investigations by the treating physician. The outcome was recorded as survived or died, and among those who survived as normal, mild, moderate, or severe disability. Chi-square test and logistic regression analysis were done to determine predictors of outcome. RESULTS: Etiology of coma in 60% cases was CNS infection (tubercular meningitis-19, encephalitis-18, bacterial meningitis-16, others-7); other causes were toxic-metabolic conditions (19%), status epilepticus (10%), intracranial bleed (7%), and miscellaneous (4%). 65 children survived, 11 were normal, 14 had mild disability, 21 had moderate disability and 14 were severely disabled and dependent. Survival was significantly better in patients with CNS infection (63%) as compared to those with toxic-metabolic causes (27%) and intracranial bleed (43%, P < 0.05). On bivariate analysis age < or = 3 years, poor pulse volume, abnormal respiratory pattern and apnoea, abnormal pupillary size and reaction, abnormal extra ocular movements, absent corneal reflex, abnormal motor muscle tone at admission or 48 hours correlated significantly with mortality. Survival was better with increasing GCS (Spearman rho = .32, P < 0.001). On logistic regression age < 3 years, poor pulse volume, absent extraocular movements and papilloedema at admission and 48 hours after admission were independent significant predictors of death. CONCLUSION: CNS infections were the most common cause of non-traumatic coma in childhood. Simple clinical signs were good predictors of outcome.

Correlates of quality of life with epilepsy.

OBJECTIVE: To examine the quality of life of children with epilepsy and to identify the demographic, disease related, and behavioral and emotional functioning variables in the prediction of quality of life of children with epilepsy. METHOD: Forty three children aged 4 to 15 years (Mean=10.3 years) with epilepsy were recruited from the outpatient services of the Department of Pediatrics, of a tertiary care teaching hospital in North India. Quality of life was measured by Impact of Epilepsy Schedule, a 39 items parent reported questionnaire and child's emotional and behavioral functioning at home was assessed by the Childhood Psychopathology Measurement Schedule. RESULTS: Majority of the parents expressed major concerns regarding seizures, treatment by anticonvulsants, present and future problems for the child and problems in parenting. Nearly 40% of the children had psychopathology scores in the clinically significant maladjustment range. Step-wise multiple regression analysis revealed that the psychopathology scores and mother's education accounted for 39% of the variance in the quality of life scores. CONCLUSION: Children with epilepsy have a relatively compromised quality of life and focusing simply on control of seizures may not address the full range of child's emotional and behavioral difficulties.

Botulinum toxin in children with cerebral palsy.

Botulinum toxin is a neurotoxin that blocks the synaptic release of acetylcholine from cholinergic nerve terminals mainly at the neuromuscular junction, resulting in irreversible loss of motor end plates. It is being widely tried as a targeted antispasticity treatment in children with cerebral palsy. A number of studies have shown that it reduces spasticity and increases the range of motion and is particularly useful in cases with dynamic contractures. However improvement in function has not been convincingly demonstrated. It is an expensive mode of therapy and the injections need to be repeated after 3-6 months. Whereas Botulinum toxin can be a valuable adjunct in select cases, it should not be projected as a panacea for children with spastic cerebral palsy.

Cerebral palsy-management.

Cerebral Palsy encompasses a heterogeneous group of non-progressive motor disorders caused by injury to the developing brain. Management is best done in a multidisciplinary set up under one roof. Comprehensive assessment of the child to evaluate functional ability and associated problems is followed by an individualized plan of management with long term goals and short term objectives. Participation of the family is pivotal to ensure proper habilitation of the child. A home-based management plan is advocated. Considerable experience, sensitivity and understanding are needed both for breaking the news and counselling the parents of a child with cerebral palsy.

Self induced photosensitive epilepsy.

The authors report a case of a 12-year-old girl who had rare self induced photosensitive epilepsy. She used to move her right hand over the right eye while simultaneously rubbing the forehead since the age of 8. During these episodes she was lost in herself. Lately these episodes were followed by brief spell of unconsciousness. The EEG examination, in its third attempt, revealed bilateral multiple symmetric spikes on photic stimulation. She admitted that she often induced the episode herself and derived pleasure out of it. She responded well to Sodium valproate.

Attention deficit hyperactivity disorder.

Attention deficit hyperactivity disorder (ADHD) is one of the most commonly diagnosed behavior disorder of childhood. In recent years, increasing number of preschoolers appear to be manifesting the core symptoms of ADHD. Diagnosis of ADHD in very young children is difficult as high activity level, impulsivity and short attention span are to some extent age appropriate characteristics of normal pre-school children. Concerns both about over-diagnosis and under-diagnosis have been expressed in the literature. Management emphasizing parental counseling, behavior management strategies, and appropriate pharmacotherapy is recommended.

Status epilepticus: emergency management.

Status Epilepticus (SE) is a medical emergency and requires prompt and aggressive treatment. Stabilization of airway, breathing and circulation and expeditious termination of seizures are immediate goals. Intravenous benzodiazepines-diazepam, midazolam or lorazepam and phenytoin are the first line drugs recommended for termination of seizures. Diazepam (or midazolam), thiopental and propofol infusion are useful for control of Refractory SE (RSE). Newer drugs are being investigated for use in SE. We prefer diazepam infusion. In children the mortality from SE ranges from 3-10% and the morbidity is twice. Mortality and morbidity are highest with SE associated with CNS infections, which is the most important cause of SE in our country. The outcome depends on the underlying etiology, age, rapidity of SE and adequacy of care. Adherence to a time-framed protocol in the emergency department helps in improving the final outcome.