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Aim: Survey of treatment practices and adherence to pediatric status epilepticus (PSE) management guidelines in India. Methods: This eSurvey was conducted over 35 days (15th October to 20th November 2023) and included questions related to hospital setting; antiseizure medications (ASMs); ancillary treatment; facilities available; etiology; and adherence to PSE management guidelines. Results: A total of 170 respondents participated, majority of them were working in tertiary level hospitals (94.1%) as pediatric intensivists (56.5%) and pediatricians (19.4%), and were in clinical practice for 2-10 years (46.5%). Majority use intravenous (IV) midazolam and levetiracetam as first- and second-line ASMs (67.1 and 51.2%, respectively). In cases with refractory status epilepticus (RSE), the most commonly used ASM is midazolam infusion (92.4%). For super-refractory status epilepticus (SRSE), the commonly used third-line ASMs include midazolam infusion (34.1%), thiopentone infusion (26.5%), high dose phenobarbitone (18.2%), and ketamine infusion (15.3%). Overall, in cases with SRSE, 44.7% respondents use ketamine infusion, 42.5% use add-on oral topiramate, and 34.7% use high-dose phenobarbitone (1-3 mg/kg/hour) infusion. Most respondents targeted both clinical and EEG seizure control (48.8%). Ancillary treatment used for SRSE included IV pyridoxine (57.1%), methylprednisolone (45.3%), IVIG (42.4%), ketogenic diet (40.6%), and second-line immunomodulation (33.5%). Most common causes were febrile SE, viral encephalitis, and febrile illness-related epilepsy syndrome (60.6%, 52.4%, and 37.1%, respectively). Facilities available included pediatric intensive care units (PICU) (97.1%), mechanical ventilation (98.2%), pediatric neurologist (68.8%), MRI brain (86.5%), EEG (69.4%), and viral PCR (58.2%). The compliance with guidelines for timing of initiation of ASM ranged from 63.5 to 88.8%. Conclusion: Intravenous midazolam bolus/es, levetiracetam, and midazolam infusion are commonly used first-, second-, and third-line ASMs, respectively. There were wide variations in use of ASMs for RSE and SRSE, ancillary treatment, and compliance to PSE management guidelines. [ABSTRACT FROM AUTHOR]

Acute neuromuscular disorders are a group of neurological emergencies characterized by rapid onset of motor weakness commonly involving the respiratory and bulbar muscles. These can be acute or an exacerbation of the chronic neuromuscular disease. An accurate and timely diagnosis allows for the timely institution of specific therapies, improving long-term functional outcomes. Landry--Guillain--Barre syndrome (LGBS), acute transverse myelitis, compressive myelopathy, traumatic spinal lesions, and anterior horn cell myelitis (due to viral infections) are some of the common causes of acute motor weakness in children. LGBS is the most common among all. The diagnosis relies on clinical presentation, signs, cerebrospinal fluid analysis, electrophysiological studies, and neuroimaging of the spine. Once compressive or traumatic spinal lesions are suspected, immediate neuroimaging of the spine is essential to plan early neurosurgical intervention. Pediatric intensive care unit (PICU) admission is often required for supportive management and immunomodulation. Supportive management typically includes endotracheal intubation to protect the airway, mechanical ventilation (MV) to support breathing, monitoring, management of autonomic dysfunction, control of pain, physiotherapy, adequate nutrition, prevention of pressure sores, bladder--bowel care, psychological support, and planning of long-term care. Autonomic dysfunction can cause hemodynamic instability, arrhythmias, sudden cardiac arrest, and death, requiring close monitoring and management in PICU. Tracheostomy is often needed in children who continue to have respiratory paralysis and are predicted to require long-term term mechanical ventilation. The outcome generally depends on the primary disease and long-term care. [ABSTRACT FROM AUTHOR]

Byline: Shrimanth. Siddegowda, Renu. Suthar, Pratibha. Singhi, Chirag. Ahuja, Niranjan. Khandelwal, Naveen. Sankhyan Background: We aimed to study the radiological and epilepsy outcomes of children with multiple neurocysticercosis (NCC) and [...]

This study aimed to determine the seropositivity of myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and aquaporin-4 antibodies (AQP4-Ab) and outcomes in children with acquired demyelinating syndromes (ADSs). Children (6 months–15 years) with suspected ADS were enrolled and tested for MOG-Ab and AQP4-Ab prospectively over 18 months at a tertiary care hospital in North India. Children with proven nonimmune-mediated neurological disorders were enrolled as controls. Of 79 children with suspected ADS, 66 were enrolled. Among the enrolled children with ADS, acute demyelinating encephalomyelitis (ADEM) (25) was the most common first clinical event followed by optic neuritis (ON) (20) and transverse myelitis (TM) (19; one child had ON and TM simultaneously [neuromyelitis optica spectrum disorders [NMOSDs]]), while two children had clinically isolated syndrome (CIS) apart from ON and TM. Fourteen (21.2%, confidence interval [CI] 11.3–31.1) tested positive for one antibody (12 [18.1%; 95% CI 10.5–25.5%] for MOG-Ab and 2 [3%; 95% CI 0–7.2%] for AQP4-Ab). None of the 62 controls tested positive for any antibody. The final diagnosis in those with the monophasic ADS was ADEM (21), ON (13), TM (16), and other CIS (1) while that in children with recurrent events was multiphasic disseminated encephalomyelitis (MDEM) (2), NMOSD (3), ADEM-ON (4), recurrent ON (4), and MS (2). Among those with the first event, 4/51 (7.8%; 95% CI 0.5–15.2%) were MOG-Ab positive and 2 AQP4-Ab positive, whereas 8/15 (53.3% [95% CI 28.1–78.6%]) with recurrent events (MDEM [2], ADEM-ON [4], recurrent ON [1], and recurrent TM [1]) were MOG-Ab positive. Hence, MOG-Abs are the most common antibodies detected in one in five children with pediatric ADS, especially in relapsing disease. AQP4-Abs are rare in children with ADS. [ABSTRACT FROM AUTHOR]

Background: Peripheral neuropathy in chronic kidney disease (CKD) is the most common neurological complication. We aimed to look at the prevalence and patterns of neuropathy in children with CKD. Methods: This cross-sectional study was conducted over 1 year in children with CKD, stage III and above. Nerve conduction studies (NCS) were performed as per standard protocols using surface electrodes on the muscles and by supramaximal stimulation of the corresponding nerves. Presence of electrophysiological abnormalities in the absence of clinical symptoms or signs was considered as subclinical neuropathy. Results: Nearly 45 children were evaluated. The majority were males (n = 39, 86.7%). The mean age was 7.9 ± 3 years (range 2--14). The mean estimated glomerular filtration rate (GFR) at enrolment was 23.3 ± 14.6 mL/min/1.73 m2 (range 5--67). The majority of children were in stage III (n = 19, 42%), followed by stages V (n = 15, 33%) and IV (n = 11, 25%). There was no evidence of clinical neuropathy; 13 children (29%) showed subclinical neuropathy. All the nerves had an axonal pattern of involvement. Motor polyneuropathy was most common type of peripheral neuropathy. The commonest nerves involved were tibial and common peroneal nerves. There were no biochemical or clinical predictors of neuropathy in our cohort. Conclusion: The prevalence of subclinical neuropathy is high in children with CKD, stage III and above. Axonal motor polyneuropathy is the predominant pattern. Electrophysiological assessment of nerve function should be routinely done in children with advanced stages of CKD to prevent chronic complications. [ABSTRACT FROM AUTHOR]

Background: X-linked dystrophinopathies have a wide spectrum of manifestation. The most common forms are severe Duchenne muscular dystrophy (DMD) and Becker's muscular dystrophy (BMD). However, less common manifestations are isolated cardiomyopathy, myalgia, cramps, rhabdomyolysis, hyperCKemia, and manifest female carriers. Materials and Methods: This case series is a part of an ongoing long-term prospective cohort of children with DMD and BMD from the year 2013. The clinical details are maintained in the clinic files and standard management protocols are followed. For this case series, clinical details were collected from the clinic files and recorded on a case record proforma. Details of cardiology, radiology, and genetic investigations were collected. Results: We report cases of classical DMD, BMD, manifest female carrier with proximal pelvic girdle weakness, a female carrier with isolated dilated cardiomyopathy, and infantile-onset asymptomatic hyperCKemia. We also report less common but notable clinical presentations of DMD, autism, intellectual disability, epilepsy, and asymptomatic transaminitis. Conclusions: It is important for clinicians to be aware of these less common clinical presentations for prompt diagnosis, and to avoid unnecessary investigations. Here, we report the clinical spectrum of dystrophinopathies seen in pediatric neuromuscular clinic and emphasize the variability and expanding knowledge about different manifestations of dystrophinopathies. [ABSTRACT FROM AUTHOR]

Background: Internationally approved electrodiagnostic criteria for Guillain Barre syndrome lack in children. We intended to compare the diagnostic accuracy of the currently available five electrophysiological criteria for childhood Guillain Barre Syndrome (GBS) at the time of sentinel assessment. Methodology: In this single-center study, data of children diagnosed with GBS between January 2013 to December 2017 were retrieved. Patient charts were reviewed for clinical features, electrophysiological recordings. The electrodiagnostic results (4 motor nerves and two sensory nerves in upper limbs and lower limbs) were reanalyzed and were classified based on Dutch group; Ho; Hadden; Hughes and Rajabally criteria for GBS. Results: During this study period, of the 205 children with clinical features of GBS, 15 children had incomplete electrophysiological data, and four children were excluded due to missing data. The mean age of onset of the 186 children enrolled was 77 months; the median duration from symptom onset to electrodiagnostic evaluation was seven days; pure motor and motor-sensory form of GBS was seen in 71 and 115 children. Based on the Hadden criteria, a demyelinating pattern was noted in 57 children; axonal in 37; Inexcitable in 84 and Equivocal in 8 children. The sensitivity of the various criteria ranged from 71% to 100% for demyelination, 97% to 100% for axonal. The degree of agreement using Hadden and Rajabally criteria for Equivocal subtypes was 0.93. Conclusions: The Rajabally criteria showed the best sensitivity, specificity and diagnostic accuracy for electrodiagnosis of GBS in children when compared against Hadden criteria. [ABSTRACT FROM AUTHOR]

Background: Calcified neurocysticercosis (NCC) predisposes patients to an enduring state of epilepsy. The predictors for calcification in parenchymal neurocysticercosis are not well defined.Method: In this prospective cohort study, consecutive children with single-lesion parenchymal NCC were enrolled and followed up for one year. All patients were investigated with brain 3 T-MRI and electroimmunotransfer blot (EITB). Clinical follow-ups were performed every 3 months. Radiology was repeated at the 6-month and one-year follow-ups. The proportion of calcified lesions at one year and the predictors of calcification were studied.Result: During the study period from June 2013 to December 2015, 93 children with single lesion parenchymal NCC were enrolled. At presentation, 90 % of the lesions were in the colloidal stage, and 71 % of the lesions had moderate to severe perilesional oedema. All children had 6 months of follow-up, and 86 (92.5 %) had one year of follow-up. Seizure recurrence was present in 13 (14 %) children. Follow-up radiology at one year showed lesion resolution in 51 (59 %) lesions and calcification in 28 (32.5 %) lesions. Children with calcified lesions during follow-up had a higher odds of seizure recurrences {OR, 95 % CI 3.6(2.3-5.6)}. The presence at baseline of diffusion restriction {OR, 95% CI 2.9 (1.01-8.8)}, scolex or wall calcification in the T2 Star weighted angiography MRI images {OR, 95% CI 3.7 (1.7-8.2)} and >10 mm size of the lesion {OR, 95 % CI 2.4 (1.2-5.01)} predicted lesion calcification.Conclusion: Children with calcification of the parenchymal NCC lesions have a higher risk for seizure recurrence during follow-up. The presence of diffusion restriction, calcified nidus in the colloidal nodular stage, and >10 mm size of the lesion at baseline predicted calcification of the lesion during follow-up. [ABSTRACT FROM AUTHOR]

Background and Purpose: Haemophilus influenzae type b (Hib) infection occurs mostly in children and is transmitted from person to person through the respiratory pathway. Hib strain is associated with meningitis or encephalitis. It is not an uncommon infection, particularly, in the developing world. This prospective cohort study was done with the aim of describing imaging findings in patients with Hib meningoencephalitis. Materials and Methods: In a prospective cohort study, consecutive children admitted in the pediatric emergency unit with acute febrile encephalopathy were enrolled. The clinical details, CSF analysis, and microbiological and serological investigations were recorded on a case record proforma. Children with confirmed Hib meningoencephalitis were included in this study. Clinicoradiological features were assessed. Results: A total of 16 patients with acute febrile encephalopathy, in whom CSF latex agglutination, CSF culture, or CSF multiplex PCR were positive for H. influenzae were included in this study. All these children were investigated with magnetic resonance imaging (MRI) brain. Important imaging findings were meningitis, predominantly around frontoparietal lobes (43%), cerebritis (28%), ventriculitis (14%), and subdural collections (21.5%). One patient had features consistent with acute disseminated encephalomyelitis (ADEM) while four patients had normal MRI scan. Conclusions: H. influenzae is still a common cause of meningitis in infants and children in the developing world. We have tried to study the most common MRI features associated with Hib infection to help radiologists alert the treating clinicians to further investigate these patients for appropriate prognostication. [ABSTRACT FROM AUTHOR]

Background and Purpose: Epstein–Barr virus (EBV) meningoencephalitis can have variable and nonspecific brain magnetic resonance imaging (MRI) findings in children. This study was done with the purpose of describing brain MRI findings in children with EBV meningoencephalitis. Materials and Methods: The study included 45 pediatric patients that presented with variable neurological symptoms and were found to have EBV meningoencephalitis based on positive EBV deoxyribonucleic acid (DNA) in the cerebrospinal fluid. All these patients had undergone brain MRI. Clinical and radiological features were evaluated. Results: Fever was a presenting feature in all cases. Signs of meningitis and raised intracranial pressure (ICP) were seen in 24 (53.3%) cases, encephalopathy in 15 (33.3%), and seizures were present in 33 (73.3%). MRI was abnormal in 29 (64.4%) patients. The cortical/subcortical pattern was diagnosed in 9 (20%) cases, white matter involvement in 7 (15.5%), basal ganglia in 5 (11.1%), thalamic involvement in 4 (8.8%), brain stem involvement in 2 (6.2%), substantia nigra involvement in 2 (4.4%), and cerebellar involvement in 2 (4.4%). Diffusion restriction was present in 11 (24.4%) cases and susceptibility changes in 7 (15.5%). Meningeal enhancement was present in 10 (22.2%) cases. In addition, brain abscess and subdural effusion/empyema were present in 1 (2.2%) case each. Conclusion: Pediatric EBV meningoencephalitis has varied clinicoradiological spectrum and there is no specific MRI pattern to characterize the meningoencephalitis on imaging. Common MRI findings include cortical-subcortical involvement, white matter changes, basal ganglia, and thalamic involvement. [ABSTRACT FROM AUTHOR]

To study the cognitive profile and scholastic performance of children with parenchymal neurocysticercosis. A total of 500 children with a diagnosis of neurocysticercosis and epilepsy registered in our pediatric neurocysticercosis clinic between January 1996 and December 2002 were enrolled. Patients were evaluated for their scholastic performance using their school grades. Cognitive assessment was done using Parental interview and the “Draw-a-Man” test. Poor scholastic performance was seen in 22.2% (111) children. Draw-a-Man test was done in 148 children; 18.2% (27/148) had scores equivalent to IQ <70. Intermittent headache, behavior problems, and poor memory were reported in 40% (201) children. Multiple lesions, lower socioeconomic status, and calcified lesions on follow-up were associated with academic underachievement (P < .05). About a fourth of children with neurocysticercosis had cognitive impairment during follow-up. This was mostly seen in children from lower socioeconomic status and in those with multiple-lesion neurocysticercosis. [ABSTRACT FROM AUTHOR]

Background Acute transvers myelitis (ATM) is a rare and disabling condition in childhood. There are only few reports of clinical profile, prognosis and predictors of ATM from developing countries. Objective To study the clinical profile of children with ATM and predictors of its outcome. Method Retrospective analysis of children <12 years of age diagnosed with ATM over a period of 6 years from a tertiary care institute. Results Thirty six children (21 boys, median age-7.5 years) were diagnosed with ATM. Weakness was symmetrical at onset in 27 (75%) children with progression over a median of 2 days (IQR 1–5 days). Severe weakness at onset with lower limb power ≤1/5 on MRC scale was present in 27 (75%), a sensory level in 25(69.4%) and bladder dysfunction in 31(86.1%) children. MRI showed longitudinal extensive myelitis (LETM) in 27 (75%) children and the thoracic cord was most commonly affected [18 (50%)]. On a median follow up of 35 months (range IQR 11–57 months); 15 (41.7%) were non ambulatory or required assistance to walk. Severe weakness at onset with power ≤1 on MRC scale, spinal shock, respiratory muscle weakness, mechanical ventilation, greater mean time to diagnosis and treatment was associated with bad outcome. ATM was a monophasic illness in all, except in 3 children; all with neuromyelitis optica spectrum disorder. Progression to multiple sclerosis was not seen in any child in our cohort. Conclusion In this series of childhood ATM from North India, the disease was severe, monophasic and involved long segments (≥3) of cord in majority. Nearly half the children remain dependent on follow up. Delayed diagnosis and delayed initiation of steroid therapy was associated with poor outcome. [ABSTRACT FROM AUTHOR]

Aim Kawasaki disease ( KD) has been associated with abnormal lipid profiles. The latter, in turn, have been linked to changes in subcutaneous fat. In this comparative cross-sectional study we have quantified distribution of subcutaneous fat during follow-up of a cohort of North Indian children with KD. Method We compared 35 KD children (at least 2 years after disease) and 33 healthy controls. Study parameters included weight, height and skinfold thickness ( SFT) over biceps, triceps, midaxillary, subscapular, medial calf and suprailiac areas. Waist and hip circumferences were also recorded. All parameters were measured four times at 6-monthly intervals using standardized techniques. Serum lipids were assayed in the study group. Results Study children were enrolled 3.7 ± 2.5 years after KD and mean age at enrolment was 8.26 ± 3.65 years. Suprailiac SFT measured higher in boys with KD ( P ≤ 0.05). Biceps SFT was higher in the study group, but the difference was not significant. Other SFT were not affected. Waist and hip circumference was higher in the study group than controls ( P ≤ 0.05). Waist/hip circumference ratio was not affected. Serum low-density lipoprotein cholesterol (LDL-C) and triglycerides were higher in the study group as compared to historical controls (95.60 ± 36.12 and 129.40 ± 64.62 mg/dL vs. 80.10 ± 2.20 and 91.1 ± 29.85; P ≤ 0.05). Total cholesterol and high-density lipoprotein cholesterol levels remained unaffected. Conclusion Children with KD (especially boys) had increased subcutaneous fat deposition in the suprailiac region and waist, during follow-up. Serum LDL-C and triglycerides were elevated. KD children may have a tendency to develop central obesity. Further studies, with longer follow-up, would be necessary to show whether this has implications for development of coronary artery disease later in life. [ABSTRACT FROM AUTHOR]

The article focuses on the neurological issues related to COVID‑19 pandemic. It mentions that children with SARS‑CoV‑2 infection tend to have a milder COVID‑19 disease with lower mortality. It also mentions that neurological complications seen in COVID‑19 may be related to sepsis, hypoxia, or due to direct neurotropism of coronaviruses.