Publications by authors named "Ramesh Konanki"

32 Publications

The spectrum of acute leukoencephalopathy with restricted diffusion (ALERD): A case series and review of literature.

Eur J Paediatr Neurol 2021 Jun 6;33:86-93. Epub 2021 Jun 6.

Department of Pediatric Neurology, Rainbow Children's Hospital, Hyderabad, Telangana, India. Electronic address:

Introduction: The clinico-etiological spectrum of Acute leukoencephalopathy with restricted diffusion (ALERD) is not well known in Indian population. This is likely to vary between populations and ethnicities.

Methods: We retrospectively reviewed the clinicoetiological spectrum of ALERD at a tertiary care pediatric center, and described the clinical, imaging, etiological spectrum and short-term outcomes.

Results: Eleven out of 78 children with non-traumatic encephalopathy presenting to our center had a final diagnosis of ALERD. The mean age at presentation was 34.9 months (6-80 months) and 63.6% were males. The monophasic course (72.7%) and the diffuse pattern (63.6%) on neuroimaging were predominant in these children. Dengue haemorrhagic fever was the commonest underlying/triggering infection (5 of 11 children). Ten children required mechanical ventilation in view of neurogenic respiratory failure, with mean duration of ventilation of 6.4 days (Range 2-10 days). The duration of hospital stay varied from 11 to 25 days (Mean - 15.3 days). One child (9 %) died, 6 children (54.5 %) had varying degrees of cognitive impairment and 4 (36.3 %) children had a normal outcome. Children with a shorter duration of ventilation seemed to have a better outcome.

Conclusion: Dengue haemorrhagic fever was the commonest cause, and diffuse imaging pattern with monophasic course was the commonest presentation in Indian children with ALERD. The clinical presentation and factors influencing outcome are possibly different from previously described literature.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejpn.2021.05.017DOI Listing
June 2021

Clinical Profile, Yield of Cartridge-based Nucleic Acid Amplification Test (GeneXpert), and Outcome in Children with Tubercular Meningitis.

J Pediatr Neurosci 2020 Jul-Sep;15(3):224-230. Epub 2020 Nov 6.

Department of pediatric Neurology and Neuro-rehabilitation, Rainbow Children's Hospital and Perinatal Centre, Hyderabad, Telangana, India.

Background: GeneXpert MTB/RIF is a test for early, rapid diagnosis of tubercular meningitis (TBM).

Aim: The aim of this article was to study the clinical profile, radiological features, yield of GeneXpert, neurosurgical interventions, and outcome of TBM in children.

Settings And Design: This was a retrospective and prospective observational study.

Materials And Methods: Diagnosis was based on the uniform research definition criteria and was staged according to the British Medical Research Council. Mantoux test, analysis of cerebrospinal fluid (CSF), CSF GeneXpert, and radiological investigations were performed.

Results: Of 36 patients, 50% were aged 1-5 years. Fever (100%), headache (82%), altered sensorium (80%), and vomiting (66%) were common features. Twelve (33%) had contact with active case of tuberculosis; 32 received Bacille Calmette Guarin vaccination. Neurological features included severe deterioration in sensorium (Glasgow Coma Scale < 8) (38%), mild and moderate deficit in sensorium (31%), hemiparesis (41%), and involvement of sixth (25%) and seventh (22%) cranial nerves. Cerebral vision impairment (25%), papilledema (25%), and dystonia (22%) were other findings. CSF GeneXpert was positive in 37% (12/33) patients. Hydrocephalus and basal exudates (75%) were noted on neuro-imaging. Surgical intervention was performed in children with hydrocephalus (13/27). Omayya reservoir was placed in seven children, of which five needed conversion to ventriculoperitoneal (VP) shunt; direct VP shunt was carried out in six (6/13). Good outcome was noted in 78% at discharge. Stage III TBM ( = 0.0001), cerebral infarcts ( = 0.0006), and motor deficits ( = 0.03) were associated with poor outcome. Sequelae included learning difficulties with poor scholastic performance (31.5%).

Conclusion: GeneXpert has high diagnostic specificity, but negative results do not rule out TBM. CSF GeneXpert provided quick results. Placement of Ommaya reservoir in TBM stage II and III with hydrocephalus was not successful. Hydrocephalus was managed conservatively with success (53%).
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/jpn.JPN_92_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7847089PMC
November 2020

Herbal Medicine-Induced Seizures in Children: Single-Center Experience Over 18 Months.

Indian Pediatr 2021 01;58(1):71-73

Department of Neurology, Rainbow Children's Hospital, Banjara Hills, Hyderabad 500 034, Andhra Pradesh, India.

Many common household herbal preparations may have seizurogenic ingredients. We report 15 children with seizures following exposure to such compounds: oral ingestion of liquid preparation in 13, and local application of balm and Eucalyptus oil ingestion in one each. All children, except one, had generalized seizures. This study highlights the need to address this history during evaluation of first seizure, and increase awareness of seizurogenic potential of such preparations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7840423PMC
January 2021

Persistent Craniopharyngeal Canal: A Rare Cause for Recurrent Meningitis in Pediatric Population.

Ann Indian Acad Neurol 2020 Jul-Aug;23(4):545-548. Epub 2019 Mar 5.

Department of Pediatric Hemato-Oncology, Rainbow Children's Hospital, Hyderabad, Telangana, India.

We present the case of a 5-year-old girl who had six episodes of meningitis. She also had panhypopituitarism and was found to have a persistent craniopharyngeal canal (CPC) as the cause of her recurrent meningitis. Role of neuroradiology and a high index of suspicion by the clinical team are highlighted here. Persistent CPC is a rare cause of recurrent meningitis. We discuss the approach to the child with recurrent meningitis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_411_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7657271PMC
March 2019

Mitochondrial carbonic anhydrase VA deficiency in three Indian infants manifesting early metabolic crisis.

Brain Dev 2020 Aug 4;42(7):534-538. Epub 2020 May 4.

Division of Neonatology, Rainbow Children's Hospital, Hyderabad, India.

Background: Hyperammonemia and hyperlactatemia in neonates and young children with non-specific biochemical markers poses a diagnostic challenge. An accurate diagnosis is essential for effective management.

Case Reports: We present three infants from unrelated families, one with infantile and two with neonatal hyperammonemic encephalopathy, hypoglycaemia, and hyperlactatemia. The underlying cause was confirmed following whole exome sequencing as biochemical markers were not conclusive of a definite diagnosis.

Conclusion: The combination of hyperammonemic encephalopathy, hyperlactatemia and hypoglycemia in neonates and infants should prompt physicians to suspect Carbonic anhydrase VA deficiency. Majority of these children can have a favourable long-term outcome with symptomatic treatment.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.braindev.2020.04.007DOI Listing
August 2020

KCNT1-related epilepsy: An international multicenter cohort of 27 pediatric cases.

Epilepsia 2020 04 13;61(4):679-692. Epub 2020 Mar 13.

Neurology Division, Department of Pediatrics, Lady Harding Medical College and Kalawati Saran Children's Hospital, New Delhi, India.

Objective: Through international collaboration, we evaluated the phenotypic aspects of a multiethnic cohort of KCNT1-related epilepsy and explored genotype-phenotype correlations associated with frequently encountered variants.

Methods: A cross-sectional analysis of children harboring pathogenic or likely pathogenic KCNT1 variants was completed. Children with one of the two more common recurrent KCNT1 variants were compared with the rest of the cohort for the presence of particular characteristics.

Results: Twenty-seven children (15 males, mean age = 40.8 months) were included. Seizure onset ranged from 1 day to 6 months, and half (48.1%) exhibited developmental plateauing upon onset. Two-thirds had epilepsy of infancy with migrating focal seizures (EIMFS), and focal tonic seizures were common (48.1%). The most frequent recurrent KCNT1 variants were c.2800G>A; p.Ala934Thr (n = 5) and c.862G>A; p.Gly288Ser (n = 4). De novo variants were found in 96% of tested parents (23/24). Sixty percent had abnormal magnetic resonance imaging (MRI) findings. Delayed myelination, thin corpus callosum, and brain atrophy were the most common. One child had gray-white matter interface indistinctness, suggesting a malformation of cortical development. Several antiepileptic drugs (mean = 7.4/patient) were tried, with no consistent response to any one agent. Eleven tried quinidine; 45% had marked (>50% seizure reduction) or some improvement (25%-50% seizure reduction). Seven used cannabidiol; 71% experienced marked or some improvement. Fourteen tried diet therapies; 57% had marked or some improvement. When comparing the recurrent variants to the rest of the cohort with respect to developmental trajectory, presence of EIMFS, >500 seizures/mo, abnormal MRI, and treatment response, there were no statistically significant differences. Four patients died (15%), none of sudden unexpected death in epilepsy.

Significance: Our cohort reinforces common aspects of this highly pleiotropic entity. EIMFS manifesting with refractory tonic seizures was the most common. Cannabidiol, diet therapy, and quinidine seem to offer the best chances of seizure reduction, although evidence-based practice is still unavailable.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/epi.16480DOI Listing
April 2020

Association of Child Neurology-Indian Epilepsy Society Consensus Document on Parental Counseling of Children with Epilepsy.

Indian J Pediatr 2019 07 8;86(7):608-616. Epub 2019 Jun 8.

Department of Pediatric Neurology, Madhukar Rainbow Children's Hospital, Malviya Nagar, Delhi, 110017, India.

When a child is diagnosed with epilepsy, counseling regarding the same is done by the treating doctor. Most parents are frightened and have poor knowledge about epilepsy. Therapeutic advice including drug dosage, administration and side effects takes up the major part of physician's time, thereby neglecting important issues like home seizure management, follow up and others. These lacunae in knowledge require systematic patient and family education. To address these issues, an expert group meeting of pediatric neurologists and epileptologists in India along with social workers/epilepsy educators, legal experts, parents, and teachers was held. The various aspects regarding parental counseling in children with epilepsy were discussed and a consensus document was formulated. Here authors present the group consensus statement on counseling parents and caregivers of children with epilepsy. This document is intended to help physicians and pediatricians counsel the families when a child is diagnosed with epilepsy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-019-02946-zDOI Listing
July 2019

Immunodeficiency, Motor Delay, and Hypouricemia Caused by a Novel Mutation of Purine Nucleoside Phosphorylase Gene in an Indian Infant.

Ann Indian Acad Neurol 2019 Apr-Jun;22(2):231-233

Department of Clinical Genetics, MedGenome Laboratory, Bengaluru, Karnataka, India.

We describe an 11-month-old boy who presented with recurrent respiratory infections from 6 months of age. His elder sister died at 10 months with severe septicemia and meningitis. The boy had a mild motor delay. Investigations revealed T cell deficiency and very low serum uric acid suggestive of purine nucleoside phosphorylase (PNP) deficiency - a rare variant of severe combined immunodeficiency disease. A novel homozygous missense mutation of c.597C>G(p. S199R) of exon 5 on PNP gene confirmed the diagnosis. We suggest that uric acid should be a part of investigation profile for unidentified motor delay, as recurrent infections can be late presentation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_430_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6472228PMC
April 2019

Ictal FDGPET and SPECT in hemifacial seizures due to cerebellar epilepsy-Case report.

Neurol India 2019 Jan-Feb;67(1):169-172

Department of Neurosurgery, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India.

The role of cerebellum in seizure generation is debatable. Semiology and electroencephalography (EEG) findings are non-specific and sometimes misleading, posing further difficulty in proving the epileptogenicity in pre-surgical workup. We report two cases of cerebellar lesions who presented with hemifacial seizures since the neonatal period and were refractory to antiepileptic drugs (AEDs). Both inter-ictal and ictal EEGs were non-contributory. Magnetic resonance imaging (MRI) showed a lesion in the cerebellum, in proximity to cerebellar peduncle in both the patients. (18) F-fluorodeoxyglucose-positron emission tomography (FDG-PET) and ictal single photon emission computed tomography (SPECT) showed focal hypermetabolism and hyperperfusion respectively, corresponding to the lesion on MRI in both the cases. Intraoperative electrocorticography showed rhythmic spikes confirming the epileptogenic nature of the lesion. Both patients were operated with a favorable surgical outcome. Histopathology was suggestive of a ganglioglioma in one child and a low-grade glioma in the other. Both cases illustrate that FDG-PET and SPECT can act as surrogate markers for invasive recordings to prove the epileptogenicity of cerebellar lesions, especially in resource limited settings.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0028-3886.253622DOI Listing
December 2019

Meta-analysis Evaluating Efficacy and Safety of Levetiracetam for the Management of Seizures in Children: Pediatric Neurologist's Viewpoint.

Authors:
Ramesh Konanki

Indian Pediatr 2018 11;55(11):992

Consultant Pediatric Neurologist, Rainbow Hospital for Women and Children, Hyderabad, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
November 2018

Case Reports: Survival from Rabies: Case Series from India.

Am J Trop Med Hyg 2019 01;100(1):165-169

Department of Pediatrics and Pediatric Intensive Care Unit, Seth G.S. Medical College and KEM Hospital, Mumbai, India.

Rabies, a zoonotic viral encephalitis, continues to be a serious public health problem in India and several other countries in Asia and Africa. Survival is rarely reported in rabies, which is considered to be almost universally fatal. We report the clinical and radiological findings of eight patients with laboratory-confirmed rabies who survived the illness. With the exception of one patient who recovered with mild sequelae, all survivors had poor functional outcomes. The reported survival from rabies in recent years may reflect an increased awareness of the disease and greater access to better critical care facilities in rabies-endemic countries. Nonetheless, there is an urgent need to focus on preventive strategies to reduce the burden of this dreadful disease in rabies-endemic countries.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4269/ajtmh.18-0711DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335910PMC
January 2019

Neurodevelopmental disorders in children aged 2-9 years: Population-based burden estimates across five regions in India.

PLoS Med 2018 07 24;15(7):e1002615. Epub 2018 Jul 24.

Department of Neurology, Paras Hospital, Gurugram, Haryana, India.

Background: Neurodevelopmental disorders (NDDs) compromise the development and attainment of full social and economic potential at individual, family, community, and country levels. Paucity of data on NDDs slows down policy and programmatic action in most developing countries despite perceived high burden.

Methods And Findings: We assessed 3,964 children (with almost equal number of boys and girls distributed in 2-<6 and 6-9 year age categories) identified from five geographically diverse populations in India using cluster sampling technique (probability proportionate to population size). These were from the North-Central, i.e., Palwal (N = 998; all rural, 16.4% non-Hindu, 25.3% from scheduled caste/tribe [SC-ST] [these are considered underserved communities who are eligible for affirmative action]); North, i.e., Kangra (N = 997; 91.6% rural, 3.7% non-Hindu, 25.3% SC-ST); East, i.e., Dhenkanal (N = 981; 89.8% rural, 1.2% non-Hindu, 38.0% SC-ST); South, i.e., Hyderabad (N = 495; all urban, 25.7% non-Hindu, 27.3% SC-ST) and West, i.e., North Goa (N = 493; 68.0% rural, 11.4% non-Hindu, 18.5% SC-ST). All children were assessed for vision impairment (VI), epilepsy (Epi), neuromotor impairments including cerebral palsy (NMI-CP), hearing impairment (HI), speech and language disorders, autism spectrum disorders (ASDs), and intellectual disability (ID). Furthermore, 6-9-year-old children were also assessed for attention deficit hyperactivity disorder (ADHD) and learning disorders (LDs). We standardized sample characteristics as per Census of India 2011 to arrive at district level and all-sites-pooled estimates. Site-specific prevalence of any of seven NDDs in 2-<6 year olds ranged from 2.9% (95% CI 1.6-5.5) to 18.7% (95% CI 14.7-23.6), and for any of nine NDDs in the 6-9-year-old children, from 6.5% (95% CI 4.6-9.1) to 18.5% (95% CI 15.3-22.3). Two or more NDDs were present in 0.4% (95% CI 0.1-1.7) to 4.3% (95% CI 2.2-8.2) in the younger age category and 0.7% (95% CI 0.2-2.0) to 5.3% (95% CI 3.3-8.2) in the older age category. All-site-pooled estimates for NDDs were 9.2% (95% CI 7.5-11.2) and 13.6% (95% CI 11.3-16.2) in children of 2-<6 and 6-9 year age categories, respectively, without significant difference according to gender, rural/urban residence, or religion; almost one-fifth of these children had more than one NDD. The pooled estimates for prevalence increased by up to three percentage points when these were adjusted for national rates of stunting or low birth weight (LBW). HI, ID, speech and language disorders, Epi, and LDs were the common NDDs across sites. Upon risk modelling, noninstitutional delivery, history of perinatal asphyxia, neonatal illness, postnatal neurological/brain infections, stunting, LBW/prematurity, and older age category (6-9 year) were significantly associated with NDDs. The study sample was underrepresentative of stunting and LBW and had a 15.6% refusal. These factors could be contributing to underestimation of the true NDD burden in our population.

Conclusions: The study identifies NDDs in children aged 2-9 years as a significant public health burden for India. HI was higher than and ASD prevalence comparable to the published global literature. Most risk factors of NDDs were modifiable and amenable to public health interventions.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1371/journal.pmed.1002615DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6057634PMC
July 2018

Comparison of telephone with face to face consultation for follow up of Neurocysticercosis.

Epilepsy Res 2018 09 12;145:110-115. Epub 2018 Jun 12.

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. Electronic address:

Objectives: There is significant scarcity of specialists to provide care for children with epilepsy in many parts of the world. Telemedicine is a potential future option. This study was planned to estimate the diagnostic accuracy of telephone consultation to identify Critical Clinical Events (breakthrough seizures, drug non-compliance, drug adverse events, features of raised intracranial pressure, and other disease-related events),compared to the Face-to-Face consultation (gold standard), in children with Neurocysticercosis (NCC) and symptomatic seizures, following the completion of cysticidal therapy.

Methods: Children aged 2-15 years attending a tertiary health care facility with a diagnosis of NCC and symptomatic seizures were enrolled after completion of the cysticidal therapy. The parents were contacted by a Pediatric Neurology Resident on Telephone before the scheduled hospital visit. Subsequently, all the children were seen directly in hospital the next day by another Pediatric Neurology Resident. The information was noted on a structured questionnaire. The diagnostic accuracy of telephone consultation for identifying the Critical Clinical Events was estimated using Face-to-Face consultation as the gold standard.

Results: A total of 1145 potential events were evaluated. Of these, the face-to-face consultation identified 56 events that would need hospital visit for detailed evaluation (breakthrough seizures in 19, drug non-compliance in 15, adverse drug events in 11, features of raised intracranial pressure in 8, and other disease-related events in 3), and 1089 events that did not require hospital consultation. The sensitivity, specificity, positive and negative predictive values of telephone consultation were 89.28% (78.12-95.96), 97.61% (96.52-98.43), 65.79% (54.01-76.30), and 99.43% (98.78-99.79) respectively. The likelihood ratios when telephone consultation was positive and negative were 37.3 and 0.11 respectively.

Significance: Telephone consultation is an acceptable mode of follow-up for children with mild Neurocysticercosis and symptomatic seizures after completion of cysticidal therapy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.eplepsyres.2018.06.005DOI Listing
September 2018

Development of All India Institute of Medical Sciences-Modified International Clinical Epidemiology Network Diagnostic Instrument for Neuromotor Impairments in Children Aged 1 Month to 18 Years.

Front Public Health 2017 21;5:313. Epub 2017 Nov 21.

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Introduction: There is shortage of specialists for the diagnosis of children with neuromotor impairments (NMIs), especially in resource limited settings. Existing International Clinical Epidemiology Network (INCLEN) instrument for diagnosing NMI have been validated for children aged 2-9 years. The current study modified the same including wider symptomatology and age group (1 month to 18 years).

Methods: The Modified INCLEN diagnostic tool (INDT) was developed by a team of experts by modifying the existing tool to widen the age range (1 month to 18 years) and include broader symptomatology (inclusion of milestones from the first 2 years of life and better elucidation of cerebellar and extrapyramidal features) in a tertiary care teaching hospital of North India between January and April 2015. A trained medical graduate applied the candidate tool, which was followed by gold standard evaluation by a Pediatric Neurologist (both blinded to each other).

Results: A total of 197 children (102 with NMI and 95 without NMI) were enrolled for the study. The sensitivity, specificity, positive and negative predictive values, positive and negative likelihood ratio of the modified NMI tool were 90.4% (82.6-95.5), 95.5% (88.7-98.7), 95.5% (88.9-98.7), 90.3% (82.4-95.5), 19.9 (12.1-32.6), and 0.13 (0.08-0.12), respectively.

Conclusion: The All India Institute of Medical Sciences modified INDT NMI tool is a simple and structured instrument covering a wider symptomatology in the 1 month to 18 years age group with acceptable diagnostic accuracy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fpubh.2017.00313DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5702309PMC
November 2017

Development and validation of AIIMS modified INCLEN diagnostic instrument for epilepsy in children aged 1 month-18 years.

Epilepsy Res 2017 02 25;130:64-68. Epub 2017 Jan 25.

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Objectives: There is shortage of specialists for the diagnosis of children with epilepsy, especially in resource limited settings. Existing INCLEN (International Clinical Epidemiology Network) instrument was validated for children aged 2-9 years. The current study validated modifications of the same including wider symptomatology and age group.

Methods: The Modified INCLEN tool was validated by a team of experts by modifying the existing tools (2-9 years) to widen the age range from 1 month to 18 years and include broader symptomatology in a tertiary care teaching hospital of North India between January and June 2015. A qualified medical graduate applied the candidate tool which was followed by gold standard evaluation by a Pediatric Neurologist (both blinded to each other).

Results: A total of 197 children {128 boys (65%) and 69 girls (35%)}, with a mean age of 72.08 (±50.96) months, completed the study. The sensitivity, specificity, positive and negative predictive value, positive and negative likelihood ratio of the modified epilepsy tool were 91.5% (84.5-96.1), 88.6% (80.0-93.5), 89.7% (81.9-95.3), 90.8% (83.7-95.7), 8 (6.6-9.8) and 0.09 (0.07-0.12) respectively.

Significance: The new modified diagnostic instruments for epilepsy is simple, structured and valid instruments covering 1month to 18 years for use in resource limited settings with acceptable diagnostic accuracy. All seizure semiologies as well as common seizure mimics like breath-holding spells are included in the tool. It also provides for identification of acute symptomatic and febrile seizures.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.eplepsyres.2017.01.008DOI Listing
February 2017

Clinical profile and outcome of refractory convulsive status epilepticus in older children from a developing country.

Seizure 2016 Mar 1;36:31-35. Epub 2016 Feb 1.

Department of Neurology, Krishna Institute of Medical Sciences, Hyderabad, India.

Purpose: The current study evaluates the etiology, clinical course and outcome of refractory convulsive status epilepticus (CSE) in older children.

Methods: Retrospective analysis of data of 73 children with CSE, aged ≥2 and ≤12 years was performed. Odds ratios were calculated between variables for clinical course and outcome. Mortality of the group was analyzed using survival analysis.

Results: Thirty three (45.2%) children progressed to refractory status epilepticus (RSE). The most common etiology for CSE was acute symptomatic in 44 (60.3%) of which 37 had presumed CNS infections. The odds of progressing to RSE were higher in children with acute symptomatic etiology (OR 2.62; CI - 95%; 0.99-7.14; p=0.041). Progression to RSE increased the chances of severe sepsis by six times (OR 6.08; CI - 95%; 1.19-31.02; p=0.036) and acidosis by nearly 15 times (OR 14.77; CI - 95%; 1.19-31.02; p=0.020). Overall mortality was 13.7%, higher in RSE (21.2% vs.7.5%). Amongst the 63 surviving children followed for 1 year from discharge, progression to RSE increased the odds of disability by seven times (OR 7.08; CI 29.31; p=0.004).

Conclusion: Acute symptomatic etiology was the commonest cause of CSE among older children from developing country and increased the odds of progressing to RSE. RSE was significantly associated with disability at 1 year from discharge.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2016.01.014DOI Listing
March 2016

Teaching NeuroImages: Fetal deep medullary vein thrombosis presenting as progressive intracerebral hemorrhage.

Neurology 2015 Jul;85(1):e5-6

From the Departments of Neurology (R.K., N.S., L.L.) and Fetal Medicine (C.R.), Rainbow Hospital for Women and Children; and the Department of Neuroradiology (D.R.V.), Mediciti Hospitals, Hyderabad, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1212/WNL.0000000000001719DOI Listing
July 2015

Consensus guidelines on management of childhood convulsive status epilepticus.

Indian Pediatr 2014 Dec;51(12):975-90

Departments of Pediatrics, *Maulana Azad Medical College and #Lady Hardinge Medical College, New Delhi; @Division of Pediatric Neurology, Department of Pediatrics, PGIMER, Chandigarh; ^Rainbow Hospital for Women and Children, Hyderabad; $Department of Pediatrics, KLE University's JN Medical College, Belgaum; °Department of Pediatrics and Neonatology,KJ Somaiya Medical College, Hospital and Research Centre, Mumbai; India. Correspondence to: Prof Satinder Aneja, Convener, Multi-disciplinary Group on Management of Status Epilepticus in Children in India; Director-Professor, Department of Pediatrics, Lady Hardinge Medical College, New Delhi 110 001, India.

Justification: Status epilepticus has a wide etiological spectrum, and significant morbidity and mortality. Management using a pre-determined uniform protocol leads to better outcomes. Multiple protocols for management of childhood status epilepticus are available, without much consensus.

Process: A 'Multi-disciplinary Consensus Development Workshop on Management of Status Epilepticus in Children in India' was organized. The invited experts included Pediatricians, Pediatric neurologists, Neurologists, Epileptologists, and Pediatric intensive care specialists from India, with experience in the relevant field. Experts had previously been divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed consensus on the topic. During the meeting, each group presented their recommendations, which were deliberated upon by the house and a consensus was reached on various issues; the document was finalized after incorporating suggestions of experts on the draft document.

Objective: To provide consensus guidelines on evaluation and management of convulsive status epilepticus in children in India (excluding neonatal and super-refractory status epilepticus).

Recommendations: Each institution should use a pre-determined protocol for management of status epilepticus; pre-hospital management and early stabilization is the key to a satisfactory outcome of status epilepticus. Pharmacotherapy should not be delayed for any investigations; the initial management should consist of a parenteral benzodiazepine by any route feasible. Subsequent management has been detailed. The group also felt the need for more epidemiological research on status epilepticus from India, and identified certain research areas for the purpose.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s13312-014-0543-4DOI Listing
December 2014

INCLEN Diagnostic Tool for Epilepsy (INDT-EPI) for primary care physicians: development and validation.

Indian Pediatr 2014 Jul;51(7):539-43

Inclen Trust International, New Delhi, India. Correspondence to: Dr Narendra K Arora, Executive Director, The Inclen Trust, International, F1-5, Okhla Industrial Area, Phase-1, New Delhi, India.

Objective: To evaluate the diagnostic accuracy of a new diagnostic instrument for epilepsy - INCLEN Diagnostic Tool for Epilepsy (INDT-EPI) - with evaluation by expert pediatric neurologists.

Study Design: Evaluation of diagnostic test.

Setting: Tertiary care pediatric referral centers in India.

Methods: Children aged 2-9 years, enrolled by systematic random sampling at pediatric neurology out-patient clinics of three tertiary care centers were independently evaluated in a blinded manner by primary care physicians trained to administer the test, and by teams of two pediatric neurologists.

Outcomes: A 13-item questionnaire administered by trained primary care physicians (candidate test) and comprehensive subject evaluation by pediatric neurologists (gold standard).

Results: There were 240 children with epilepsy and 274 without epilepsy. The candidate test for epilepsy had sensitivity and specificity of 85.8% and 95.3%; positive and negative predictive values of 94.0% and 88.5%; and positive and negative likelihood ratios of 18.25 and 0.15, respectively.

Conclusions: The INDT-EPI has high validity to identify children with epilepsy when used by primary care physicians.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s13312-014-0443-7DOI Listing
July 2014

Profile of prothrombotic factors in Indian children with ischemic stroke.

J Clin Neurosci 2014 Aug 27;21(8):1315-8. Epub 2014 Jan 27.

Department of Cardiac Biochemistry, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

This study was undertaken in view of paucity of data regarding the profile of prothrombotic factors in children with ischemic stroke. Sixty-four children with ischemic stroke were prospectively evaluated for prothrombotic factors over a 2 year period. The blood samples were analyzed for protein C (PC), protein S (PS), activated protein C resistance (APCR), factor V Leiden (FVL), anti-thrombin-III (AT-III), lipoprotein (a) [Lp(a)], lupus anticoagulant (LA), anti-cardiolipin antibodies (aCL) immunoglobulin (Ig) M and IgG, homocysteine, and methylenetetrahydrofolate reductase (MTHFR) at least 3 months after the onset of stroke. At least one prothrombotic factor was identified in 45.3% children (29/64). These included hyperhomocysteinemia (11/64), PC deficiency (9/64), aCL (8/64), PS deficiency (5/64), APCR (3/64), AT-III deficiency (2/64) and LA (1/64). Multiple factors were coexistent in 17.2% (11/64). The prevalence of PC deficiency, PS deficiency and co-existence of multiple abnormalities observed were similar to the published literature. Elevated Lp(a) and APCR were less prevalent. FVL and MTHFR were not seen in any of the study children. Forty-five percent of children had at least one prothrombotic abnormality. Hyperhomocysteinemia, PC deficiency, aCL and PS deficiency were the most frequent prothrombotic abnormalities.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jocn.2013.10.030DOI Listing
August 2014

Childhood Guillain-Barré syndrome subtypes in northern India.

J Clin Neurosci 2014 Mar 13;21(3):427-30. Epub 2013 Oct 13.

Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar East, New Delhi, DL 110029, India. Electronic address:

This prospective cross-sectional study was conducted at a tertiary care research centre in North India to describe the frequency and clinical characteristics of subtypes of childhood Guillain-Barré syndrome. Among the 68 children enrolled, 65 were finally diagnosed with Guillain-Barré syndrome (median age, 60 months); 45 (69%) were boys. The most common subtype was acute motor axonal neuropathy in 27 patients (41.5%, 95% confidence interval [CI] 29-54), followed by acute inflammatory demyelinating polyneuropathy in 15 (23%, 95% CI 13.5-35), and acute motor sensory axonal neuropathy in three (4.6%, 95% CI 1-13). Twelve patients (18.5%, 95% CI 10-30) had inexcitable nerves, and eight (12.4%, 95% CI 5.5-23) were unclassifiable. Those with acute inflammatory demyelinating polyneuropathy were more likely to have had a preceding upper respiratory tract infection. The acute motor axonal neuropathy subtype peaked in incidence during the winter and monsoon months.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jocn.2013.04.030DOI Listing
March 2014

Mineralizing angiopathy with infantile basal ganglia stroke after minor trauma.

Dev Med Child Neurol 2014 Jan 17;56(1):78-84. Epub 2013 Sep 17.

Department of Pediatric Neurology, Rainbow Hospital for Women and Children, Hyderabad, India.

Aims: The objective of this study was to describe a cohort of infants with basal ganglia stroke associated with mineralization in the lenticulostriate arteries and their clinical outcomes.

Method: Subcortical strokes occurring in infants during the study period were categorized as arterial ischaemic, venous, or haemorrhagic. A cohort of infants with basal ganglia infarcts and associated mineralization of lenticulostriate arteries were identified. This group was analysed for possible aetiological factors, clinical course, and recurrence rate of the stroke.

Results: Of 23 infants with basal ganglia arterial ischaemic stroke, 22 (16 males, six females; mean age 11mo [±SD 4.8mo]) were found to have lenticulostriate artery mineralization. Twenty infants presented with hemiparesis and two presented with recurrent episodes of hemidystonia. Eighteen infants had a history of minor trauma before onset of stroke. No other predisposing factors were identified in this cohort. There were no demonstrable causes for vascular and soft tissue calcification. The mean follow-up was 11 months, during which five infants experienced stroke recurrence. Of the 17 infants who did not experience a recurrent stroke, eight exhibited complete neurological recovery, and nine had mild residual hemiparesis.

Interpretation: Acute basal ganglia stroke after minor trauma associated with mineralization of lenticulostriate arteries in infants is a distinct clinicoradiological entity. Investigations for prothrombotic states and vasculopathies are normal. Although neurological outcomes in most children are good, trauma is a risk factor for recurrence of stroke.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/dmcn.12275DOI Listing
January 2014

Midbrain neurocysticercal granuloma appearing as "face of panda".

Neurology 2013 May;80(21):1999

Rainbow Children's Hospital and Perinatal Centre, Hyderabad, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1212/WNL.0b013e318293e357DOI Listing
May 2013

Vein of Galen aneurysmal dilatation in a 14-month-old boy.

Pediatr Neurol 2012 Jul;47(1):71-3

Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pediatrneurol.2012.04.001DOI Listing
July 2012

Infantile-onset Alexander disease: a genetically proven case with mild clinical course in a 6-year-old Indian boy.

J Child Neurol 2013 Mar 7;28(3):396-8. Epub 2012 May 7.

Department of Pediatrics, Division of Pediatric Neurology, All India Institute of Medical Sciences, New Delhi, India.

Alexander disease is an autosomal dominant leukoencephalopathy characterized by developmental delay, macrocephaly, and characteristic neuroimaging abnormalities predominantly involving frontal lobes. We report a 6-year-old Indian boy with infantile-onset Alexander disease, who has an unusually mild clinical course and a de novo p.Leu359Val mutation in the glial fibrillary acidic protein gene.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0883073812444313DOI Listing
March 2013

Maternal Music Exposure during Pregnancy Influences Neonatal Behaviour: An Open-Label Randomized Controlled Trial.

Int J Pediatr 2012 14;2012:901812. Epub 2012 Feb 14.

Division of Neurology, Cincinnati Children's Hospital Medical Centre, MLC 2015, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.

Objective. This study evaluated the effect of antenatal music exposure to primigravida healthy mothers on the behaviour of their term appropriate-for-date newborns assessed using Brazelton Neonatal Behavioral Assessment Scale (BNBAS). Methods. This was a single-centre, randomized, open-label controlled trial. Primigravida mothers aged 19-29 years, free of chronic medical diseases or significant deafness, with singleton pregnancy, with a gestation of 20 weeks or less, were randomized to listen to a pre-recorded music cassette for approximately 1 hour/day in addition to standard antenatal care (intervention arm) or standard care only (control arm). Perinatal factors with adverse effect on neonatal behaviour were deemed as protocol violations. Outcome measure included scores on 7 clusters of BNBAS. Primary analysis was per protocol. The trial is registered with ClinicalTrials.gov (NCT01278329). Results. One hundred and twenty-six newborns in the music group and 134 in the control group were subjected to BNBAS assessment. The infants of mothers exposed to music during pregnancy performed significantly better on 5 of the 7 BNBAS clusters. The maximal beneficial effect was seen with respect to orientation (ES 1.13, 95% CI 0.82-1.44, P < 0.0001) and habituation (ES 1.05, 95% CI 0.53-1.57, P = 0.0001). Conclusion. Prenatal music exposure to mother significantly and favourably influences neonatal behaviour.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2012/901812DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3299264PMC
August 2012

Child neurology: epilepsy of infancy with migrating focal seizures.

Neurology 2011 Jul;77(4):e21-4

Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1212/WNL.0b013e3182267b4fDOI Listing
July 2011

Limb-girdle myasthenia gravis in a 10-year-old girl: a case report.

J Child Neurol 2011 Nov 5;26(11):1434-7. Epub 2011 Jul 5.

Division of Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

A 10-year-old girl presented with progressive proximal limb muscle weakness without facial, ocular, or bulbar muscle involvement. There was no fatigability or diurnal fluctuation in symptoms. Her weakness worsened with febrile illnesses and recovered with accruing disabilities over a few weeks. Serum creatine kinase levels and muscle biopsy were normal. A significant decrement on repetitive nerve stimulation test and positive response to therapeutic neostigmine challenge test confirmed the diagnosis of limb-girdle myasthenia. She responded well to corticosteroids and thymectomy, demonstrating a likely autoimmune etiology. This case highlights the long-term fluctuations in a case of myasthenia gravis and the need for a high index of suspicion for myasthenia in children presenting with unexplained muscle weakness, even in the absence of typical features such as fatigability, diurnal fluctuation, and oculobulbar weakness.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0883073811410059DOI Listing
November 2011

Cognitive decline and limb weakness in a 10 year old boy.

J Clin Neurosci 2010 Dec;17(12):1542, 1609

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
December 2010
-->