Sumeet R. Dhawan

Knowledge, Attitude and Practice (KAP) Study of Pediatricians on Infantile Spasms

ObjectiveTo investigate the knowledge, attitude, and practice of Infantile Spasms among pediatricians.MethodsA survey was carried out among pediatricians serving in Punjab, Haryana, Chandigarh, Himachal Pradesh and Delhi. The survey was done by Survey Monkey Software through emails by using a structured questionnaire between July 2016 and December 2017.ResultsA total of 236 pediatricians responded to the survey. Most of the respondents (95.5%) correctly considered Infantile Spasms as a seizure type. The most preferred investigation was Electroencephalogram by 91.8% pediatricians; however, only 57.7% considered it to decide the treatment. Perinatal asphyxia was the most recognized etiology (60.7% pediatricians). For treatment of Infantile Spasms, 66.8% follow Nelson textbook of Pediatrics. Adrenocorticotropic hormone was the most preferred first choice drug by 40% pediatricians. Alternate anti-epileptic drug was considered by 60.9% pediatricians when there is no clinical response. Only 24% pediatricians considered treatment response as a complete cessation of spasms. Majority (90%) of pediatricians felt that there is a necessity for increased awareness and 62% pediatricians felt that available information was insufficient.ConclusionsA substantial number of pediatricians lack precise knowledge on evidence-based practice of Infantile Spasms. In developing countries, where pediatricians provide the initial management of Infantile Spasms, there is need to empower them and develop simplified national guidelines/consensus statement for management of Infantile Spasms.

Predictors of Neurological Outcome of Tuberculous Meningitis in Childhood: A Prospective Cohort Study From a Developing Country.

Aims: To assess the long-term outcome of childhood tuberculous meningitis treated with modern 4-drug antitubercular regimens and to determine predictors of survival and morbidity. Methods: In this single-center prospective cohort, outcome of children with tuberculous meningitis treated with standard regimens was assessed at 6 months and 12 months after discharge using the Pediatric Cerebral Performance Category Scale. Results: Of 130 children, 38 died in hospital and 34 were either severely disabled or comatose/vegetative at discharge. At 6 and 12 months, 87% of the survivors were either normal (n = 62) or mildly disabled (n = 17, on the Pediatric Cerebral Performance Category scale). On multivariate analysis, the factors associated with poor outcome at 12 months were stage III at admission (adjusted odds ratio 4.4, 95% confidence interval, 1.7-11.2, P = .002) and presence of infarcts on neuroimaging (adjusted odds ratio 2.6, 95% confidence interval, 1.1-6.6, P = .037). Conclusions: Despite the high in-hospital mortality, in resource-constraint settings, the survivors showed remarkable improvement, with two-thirds returning to a normal functional status at 6 months’ follow-up.

ANCA-associated Vasculitis Presenting as Severe Pulmonary Hypertension and Right Heart Failure

Microscopic polyangiitis (MPA) is a small vessel multisystemic disorder characterised by necrotising small vessel vasculitis without any immune deposits. Kidney and lung are the predominant organs affected in MPA. Skin, gastrointestinal and neurological findings are also described. Isolated pulmonary manifestations are rare. The authors describe a two-year girl who presented with right heart failure and was subsequently diagnosed as ANCA-associated vasculitis. This case report is intended to sensitise pediatricians to consider systemic vasculitis with pulmonary hemorrhage in children with pulmonary hypertension even in the absence of severe pallor.

Multiple Intracranial Aneurysms in HIV Infection

Neurological findings in HIV are common and include cognitive impairment, microcephaly, nonspecific white matter lesions and seizures. Cerebral vasculopathy and stroke are uncommon and may be due to primary HIV vasculopathy or opportunistic infections such as tuberculosis and cryptococcal meningitis. The authors describe a 7-y-old boy who presented with severe headache and was detected to have aneurysmal bleed due to intracranial aneurysm.

Isolated Congenital Absence of Cranial Nerves: Report of Two Cases

&NA; Isolated cranial nerve absence is a rare condition that can be diagnosed using high‐resolution cranial nerve magnetic resonance (MR) imaging. Thorough clinical examination with proper knowledge of the course of cranial nerves may help diagnose this rare condition. We describe two cases, one each of, isolated congenital absence of the third and seventh cranial nerve with their clinical presentation. High‐resolution T2‐weighted MR imaging was done in both patients which revealed absence of cisternal segment of the right‐sided third nerve and cisternal with canalicular segment of the right‐sided facial nerve.

Unexplained Transaminitis and Hyperactivity: Do Not Forget the Musculoskeletal Examination

To the Editor: An eleven-year old boy presented with recurrent abdominal pain, hyperactivity and aggressive behavior. His anthropometry, general and systemic evaluation were within normal limits. His investigations (prior to referral) revealed transaminitis (alanine aminotransferase of 2021 U/L and aspartate aminotransferase of 931 U/L). Without further thought and this symptom complex, Wilson’s disease was initially considered. His evaluation for chronic liver disease (viral hepatitis, ceruloplasmin, serum copper, autoimmune antibodies, MRI brain, slit lamp for Kayser–Fleischer rings) were unremarkable. The child was referred to our center to find out the etiology of this unexplained transaminitis. A review history revealed difficulty in running and examination revealed pseudohypertrophy of calf and deltoid (Fig. 1) and mild proximal weakness. With a clinical possibility of Becker’s muscle dystrophy (BMD), creatine phosphokinase (CPK) was evaluated and was elevated 6747 U/L. 79-exon multiplex PCR of dystrophin gene confirmed deletion of exon 2 and 5, confirming the diagnosis of BMD. The case is important as it highlights the dwindling art of the clinical examination. It is not uncommon to see that with ease of availability of investigations, the physicians tend to get lost in the clinical diagnosis even before comprehensive clinical assessment. Had the clinical examination been complete, it would not have taken longer to recognize that transaminitis could be of muscle origin. Transaminitis is common in muscle disease, seen in 97.5% cases with Duchene muscular dystrophy (DMD) and all cases of BMD. The degree (median) of transaminitis is more in DMD compared to BMD (6.55 times vs. 2.91 in BMD). Mild transaminitis of 1.65 times may also be seen in limb girdle dystrophy [1]. So, to a certain extent, degree of transaminitis may help in differentiating muscle dystrophies. It is not uncommon to see such children being subjected to liver biopsies [2]. In one series, 36% and 32% children of BMD had attention deficit hyperkinetic disorder and learning disabilities [3]. So, in a child with behavioral abnormalities and/or learning difficulties, who has physical difficulties in coping with other children, a possibility of muscle disease should be evaluated. We suggest that all children with unexplained transaminitis should be asked history of proximal muscle weakness symptoms and evaluated clinically for pseudohypertrophy and subsequently by CPK-total which may save exorbitant cumbersome tests for liver disease. * Lokesh Saini drlokeshsaini@gmail.com

Recurrent Cerebrospinal Fluid Pleocytosis

To the Editor: An 11 y boy presented with severe continuous headache for the last 72 h. He has history of recurrent headaches for the last 2 y. Headache would occu r 3–5 t imes pe r mon th and l a s t 1–3 h . Intermittently these were associated with photophobia, phonophobia, vomiting, retro-orbital pain and transient paresthesias of hand. Cerebrospinal fluid (CSF) evaluat ion had shown lymphocyt ic pleocytosis (Table 1). He had been treated with antibiotics for meningitis during the first two episodes. Detailed evaluation for chronic meningitis (tuberculosis, cryptococcus meningitis, herpes polymerase chain reaction, cytomegalovirus, acanthamoeba, fungal culture, HIV ELISA and malignant cytology) was unremarkable. Contrast magnetic resonance imaging, angiography and venography were normal. He was managed as a case of headache and neurological deficits with CSF lymphocytosis (HaNDL). He was treated with anti-emetics and oral rizatriptian (no antibiotics) for the acute episode, and subsequently started on flunarizine prophylaxis. The child is asymptomatic for the last 6 mo. The case aims to sensitize the pediatricians about non-infectious etiology of CSF pleocytosis in a child with severe headache. Acute headaches in children can be due to sinusitis, migraine, meningitis, intracranial hemorrhage or brain tumors [1]. A normal neuroimaging excludes hemorrhage and tumors, however the clinical distinction between the other etiologies can be challenging. The location of pain and character of pain in sinusitis, migraine and meningitis is commonly fronto-temporal and squeezing or pounding in nature [1]. Due to its seriousness, cerebrospinal fluid analysis is usually done as in the index case to exclude meningitis. Since all the evaluations showed pleocytosis, a possibility of recurrent and chronic meningitis was suspected. However, fever was notably absent during all the episodes and infective markers like C-reactive protein (CRP) and procalcitonin were repeatedly negative. This led to a suspicion of non-infectious etiology like HaNDL which is characterized by a prodrome of viral illness followed by severe headache (lasting 1 h-1 wk) and neurological deficits [2]. The deficits in the index case were only in form of paresthesias which may be due to sluggish blood circulation in the corresponding area of cortex [3]. To conclude, the knowledge of this syndrome is important to limit unnecessary evaluation (multiple costly invasive and noninvasive investigations) and treatment of chronic meningitis with multiple antibiotics in a child with recurrent headaches associated with CSF pleocytosis.

Levetiracetam Induced Neuropsychiatric Manifestation in a 5-year-old Boy

To the Editor: A five-year-old developmentally normal boy presentedwith six episodes of seizures (Generalized tonic-clonic) for last 6 mo. He was initially started on phenytoin but later switched over to levetiracetam (30mg/kg) because of poor seizure control. Two weeks after starting of levetiracetam, the child had a change in behavior like poor attention, poor concentration span and decreased interaction with others. Three weeks later, he had intermittent episodes of crying and shouting and hitting other family members without any reason. He also had suicidal tendencies in the form of attempts to cut his wrist with a knife and selfstrangulation with a belt. Evaluation for vasculitis (complete blood counts and anti-nuclear antibody), autoimmune encephalitis, worsening epilepsy and other structural etiologies were unremarkable (normal awake and sleep electroencephalography and MRI brain with MR-angiography). Clinical diagnosis of genetic generalised epilepsy was considered. A possibility of levetiracetam induced neuropsychiatric side-effects was considered and levetiracetam was switched over to topiramate. Within few days, he had improvement in interaction with parents, sleep, and behavior. Improvement in symptoms following the withdrawal of levetiracetam suggested a probable causality. He was discharged after 1 wk, and he remained symptom-free over the next 3 mo. Behavioral and cognitive impairments are common in children with epilepsy. These symptoms can be epilepsy itself or due to treatment emergent side-effects of anti-epileptic drugs. Twenty to 30 % of patients with epilepsy have associated mood disorders, and 2–7% may have frank psychosis [1]. Behavioral adverse effects are common with levetiracetam and are seen in 5– 34% of children [2]. Most of these are mild and seldom need discontinuation of levetiracetam. Common adverse effects include aggression, excessive sleepiness, hyperactivity and depression [2, 3]. Rapid titration and prior intellectual/learning disability were associated with higher incidence of adverse effects. Severe adverse effects like severe depression, psychosis, and suicidal tendencies are rare [4, 5]. The suicidal tendencies may be explained by bouts of rage and aggression in the index child, which may lead to self-harm. Concomitant use of pyridoxine has been described to ameliorate behavioral side-effects of levetiracetam by decreasing hyperactivity. To conclude, the possibility of severe behavioral abnormalities should be borne in mind even with commonly used and seemingly safe antiepileptic drugs.

Fulminant Early Onset Subacute Sclerosing Panencephalitis.

To the Editor: A 27-mo-old boy presented with increasing frequency of recurrent drop attacks for 6 wk. He had become bedridden for the past 3 wk. The child had a history of measles like illness at the age of 1 y, despite having received a single dose of measles vaccine at 9 mo. On examination, he was bedridden; unable to speak meaningfully; unable to recognize caregivers; or understand any commands. He had continuous involuntary choreiform movements, and periodic myoclonic jerks. His growth parameters were normal. Cranial nerve examination revealed absent visual fixation, normal fundus, preserved pupillary reflexes, and only a startle response to sound. Motor examination revealed generalized hypotonia and brisk deep tendon reflexes. On investigations, EEG revealed a slow background for age and periodic large amplitude slow wave complexes. MRI brain showed bilateral T2/FLAIR white matter hyperintensities in bilateral deep peritrigonal areas (Fig. 1). Cerebrospinal fluid and serum anti-measles antibody (IgG) was positive in titre of 1:625. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was concluded. The child was started on valproate and clonazepam for myoclonus. Isoprinosine and interferons could not be started in view of financial constraints. At last follow up six months after discharge the child was in vegetative state. SSPE is a disorder characterised by rapid neurological deterioration caused by a mutated measles virus. The onset in this child before the age of 3 y was unusual. SSPE usually has a latency of 5 to 15 y [1]. Neonatal, perinatal or intrapartum measles in mother may cause rapidly progressive SSPE [2]. However, no such history of measles in the mother was there in the index case. The rapid progression as in the index case leading to a vegetative state in less than 3 mo is seen in 10 % of the cases. Such a course is more likely with measles at an early age [3]. The early MRI changes in SSPE consist of parietooccipital white matter involvement and deep white matter

Necrotizing Fasciitis of Scalp and Neck in Neonates

A term 3 kg hospital born neonate presented on day 9 of life with continuous high grade fever since day 2 of life. Baby developed progressive erythema and swelling of scalp, face and periorbital area with small scattered black patches for 5 days (Fig.1A). Complete blood count showed haemoglobin of 13.5 g/dL, total leukocyte count of 26,200/mm3 and platelet count of 71,000/mm3. Coagulogram, cerebrospinal fluid (CSF) analysis, HIV ELISA, blood culture, immunoglobulin profile, T and B-lymphocyte subsets and nitro-blue tetrazolium tests were in normal range. Contrast CT scan showed diffuse skin and subcutaneous thickening of face and neck. Baby was managed with vancomycin, meropenem and clindamycin. He developed clinical deterioration and multiple areas of gangrenous spots surrounded by inflamed edematous skin over right half of face and scalp. Ultrasonography of neck was suggestive of pyomyositis and NF. Figure 1: A) Showing cellulitis of face, scalp and neck with areas of cutaneous skin necrosis of case 1. B) showed areas of granulation tissue over the scalp and neck after antibiotic therapy.