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Byline: Ram. Prabhu, Arushi. Saini, Renu. Suthar, Sameer. Vyas, Prahbhjot. Malhi, Savita. Attri, Lokesh. Saini, Jitendra. Sahu, Naveen. Sankhyan Objectives: To study the clinical spectrum of inherited gray matter degenerative [...]

Byline: Ahibhushan. Sonbhadra, Bandi. Chaithanya Reddy, Arushi. Saini, Kara. Tiewsoh, Pradip. Paria, Shivan. Kesavan, Renu. Suthar, Lesa. Dawman, Savita. Attri Background: Peripheral neuropathy in chronic kidney disease (CKD) is the [...]

Byline: Pawan. Kumar, Balamurugan. Nagarajan, Arushi. Saini, Debajyoti. Chatterjee, Sameer. Vyas, Gnanamani. Senguttuvan, Harpreet. Singh, Shushanta. Sahoo, Renu. Suthar Sir, Juvenile xanthogranuloma (JXG) is a benign disorder that presents in [...]

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]

Objectives: The profile of seizures in neurocutaneous syndromes is variable. We aimed to define the characteristics of epilepsy in children with neurocutaneous syndromes. Materials and Methods: Cross-sectional study over 18 months at a tertiary care pediatric hospital, including children with neurocutaneous syndromes aged between 1 and 15 years, using the 2017-International League Against Epilepsy classification. Results: In 119 children with neurocutaneous syndromes, 94 (79%) had epilepsy. In eight children with neurofibromatosis one with epilepsy, 5 (62.5%) had generalized motor tonic-clonic seizures, 1 (12.5%) had generalized motor epileptic spasms, 1 (12.5%) had generalized motor automatism, and 1 (12.5%) had a focal seizure. In 69 children with tuberous sclerosis complex with epilepsy, 30 (43.5%) had generalized motor epileptic spasms, 23 (33.3%) had focal seizures, and nine (13.0%) had generalized motor tonic-clonic seizures. In 14 children with Sturge-Weber syndrome with epilepsy, 13 (92.8%) had focal seizures, and 1 (7.2%) had generalized motor tonic seizures. Statistically significant associations were found between epilepsy and intellectual disability (P = 0.02) and behavioral problems (P = 0.00). Conclusion: Profiling seizures in children with neurocutaneous syndromes are paramount in devising target-specific treatments as the epileptogenesis in each syndrome differs in the molecular pathways leading to the hyperexcitability state. Further multicentric studies are required to unravel better insights into the epilepsy profile of neurocutaneous syndromes. [ABSTRACT FROM AUTHOR]

Byline: Balamurugan. Nagarajan, Pawan. Kumar, K. Sudeep, Renu. Suthar, Manisha. Biswal, Jayashree. Muralidharan, Naveen. Sankhyan, Vivek. Lal Sir, Scrub typhus is an important tropical infection in India. Seizures, altered sensorium, [...]

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]

Purpose: Duchenne muscular dystrophy (DMD) is X-linked progressive neuromuscular disorder of childhood onset which leads to loss of ambulation, followed by respiratory and cardiac muscle weakness. Physiotherapy is integral to the multidisciplinary care plan for boys with DMD. During the COVID-19 pandemic, Tele-medicine was increasingly used to provide healthcare to patients. The study aimed to assess the efficacy and feasibility of Tele-rehabilitation among ambulatory boys with DMD. Materials and Methods: Ambulatory boys aged 5 to 12 years with DMD were recruited using purposive sampling. The Tele-rehabilitation program was designed by a neuromuscular team and provided by a physiotherapist through telephonic video calls twice a month for three months. A compliance diary was provided to fill out at home after each session. The outcome was measured with a change in Medical Research Council (MRC) sum score and gower's times at 12 weeks using paired t- test. Results: 92 boys with DMD were enrolled and 66 (71%) boys completed the program and had follow-up at 12 weeks. The baseline MRC sum score was 38.2±3.7, and the gower's time was 9.2±6.7 seconds. At 12 weeks MRC sum score was 38.8±4.2, and gower's time was 8.59±7.7 seconds (p-value- 0.03 and p-value- 0.001), respectively. The Vigno's and Brook's scores didn't show a significant change. Compliance of >50% to the Tele-rehabilitation exercise programs was present in 89% of subjects. Conclusion: A short-term Tele-rehabilitation program was feasible with a high compliance rate and efficacious in motor strengthening among boys with DMD. [ABSTRACT FROM AUTHOR]

Byline: Renu. Suthar, Indar. Sharawat, Katja. Eggermann, Hansashree. Padmanabha, Arushi. Saini, Bhavneet. Bharti, Ingo. Kurth, Pratibha. Singhi, Naveen. Sankhyan Objectives: Hereditary sensory and autonomic neuropathy (HSAN) is a group of [...]

Byline: Renu. Suthar, Chandana. Bhagwat, Pradip. Paria, Divya. Aggarwal, Namita. Kumar, Debajyoti. Chatterjee, Arushi. Saini, Suresh. Angurana, Naveen. Sankhyan Sir, Spinal muscular atrophy (SMA) is the most common autosomal recessive [...]

Byline: Surjeet. Kumar, Azhar. Navid, Raman. Sharma, Renu. Suthar, Sameer. Vyas, Suresh. Angurana Dear Editor Dengue is a common arboviral infection in India and it classically presents with fever, arthralgia, [...]

Byline: Bhanudeep. Singanamalla, Arushi. Saini, Savita. Attri, Renu. Suthar, Kanya. Mukhopadhyay Inborn errors of metabolism (IEM) are a heterogenous group of disorders in which the body cannot metabolize the food [...]

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 [...]

Byline: Renu. Suthar, Shivan. Kesavan, Indar. Sharawat, Manisha. Malviya, Titiksha. Sirari, Bhupendra. Sihag, Arushi. Saini, Vishalavath. Jyothi, Naveen. Sankhyan Background: X-linked dystrophinopathies have a wide spectrum of manifestation. The most [...]