Dr.Vykuntaraju K.N. is currently working as Assistant Prof of pediatric neurology at Indira Gandhi Institute of Child Health, Bangalore. He completed his under graduation and post graduation in pediatrics from Bangalore Medical College and Research Institute (BMCRI), Bangalore. He completed his DM in Pediatric Neurology from All India Institute of Medical Sciences (AIIMS), New Delhi. He had more than 25 publications in indexed journals and 80 publications in other journals, national and international conferences. He had given talks at various state and national conferences. He is a member of various organizations. His specialist interest is pediatric epilepsy. He is an author and editor of book on cerebral palsy and early stimulation. He started pediatric neurology fellowship first time in Karnataka at Indira Gandhi Institute of Child Health, Bangalore, under Rajiv Gandhi University of Health Sciences. Supervisors: Pediatric Neurology Department- Indira Gandhi Institute of Child Health, Bangalore
Background West's syndrome (WS) is a triad of epileptic spasms (ESs), psychomotor delay, and ... more Background West's syndrome (WS) is a triad of epileptic spasms (ESs), psychomotor delay, and hypsarrhythmia. The treatment of ESs is still controversial. Hence, we designed a randomized controlled trial (RCT) to compare the outcomes in children with WS treated with adrenocorticotropic hormone (ACTH) alone versus ACTH and levetiracetam (LEV). Objectives To compare the treatment outcomes and side effects in children treated with ACTH alone versus ACTH and LEV. Methods This prospective randomized controlled trial was conducted from December 2017 to May 2019 in tertiary care center, Bangaluru. Children from 2 months to 5 years of age, diagnosed with WS were included. Fifty children in each group were analyzed for efficacy and side effects. Results There was no difference in the baseline characteristics in both groups. There was no difference in spasms response at the end of 2 weeks between the groups (88 vs. 82%) with p-value of 0.813. The relapse rates were less in ACTH and LEV gro...
Pathogenic variants in A Disintegrin And Metalloproteinase (ADAM) 22, the postsynaptic cell membr... more Pathogenic variants in A Disintegrin And Metalloproteinase (ADAM) 22, the postsynaptic cell membrane receptor for the glycoprotein leucine-rich repeat glioma-inactivated protein 1 (LGI1), have been recently associated with recessive developmental and epileptic encephalopathy. However, so far, only two affected individuals have been described and many features of this disorder are unknown. We refine the phenotype and report 19 additional individuals harbouring compound heterozygous or homozygous inactivating ADAM22 variants, of whom 18 had clinical data available. Additionally, we provide follow-up data from two previously reported cases. All affected individuals exhibited infantile-onset, treatment-resistant epilepsy. Additional clinical features included moderate to profound global developmental delay/intellectual disability (20/20), hypotonia (12/20) and delayed motor development (19/20). Brain MRI findings included cerebral atrophy (13/20), supported by post-mortem histological e...
Hyperphosphatemic familial tumoral calcinosis (HFTC) presents with varied neurological manifestat... more Hyperphosphatemic familial tumoral calcinosis (HFTC) presents with varied neurological manifestations that have been reported in the literature like facial palsy, vision and hearing impairment, stroke, and headache. In this article, we reported a 12-year-old girl child patient with recurrent facial weakness with bilateral hearing impairment and multiple ulcerative lesions on lower limbs and elbows. On examination, she had lower motor neuron (LMN) facial palsy with conductive hearing loss. The investigations showed hyperphosphatemia (9.3 mg/dL) with normal serum calcium (10.4 mg/dL), alkaline phosphatase (147.9 U/L), parathyroid hormone (23.12 pg/mL), and renal function tests. Elevated serum calcium and phosphorus product (96.72 mg2/mL2) and elevated renal tubular reabsorption of phosphate (TMPxGFR) value (9.16) were noted. Skeletal survey showed hyperostosis in the long bone diaphysis, vertebrae, ribs, pelvic bone, skull, and facial bones with narrowing of cranial ostium, characteri...
Hemiconvulsion-hemiplegia-epilepsy syndrome is an uncommon consequence of prolonged focal febrile... more Hemiconvulsion-hemiplegia-epilepsy syndrome is an uncommon consequence of prolonged focal febrile convulsive seizures in infancy and early childhood. We report two cases of hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and had striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere as documented by magnetic resonance imaging, including extensive diffusion weighted imaging abnormalities in both the cases.
The SCN encephalopathies are one of the rare early childhood intractable epileptic encephalopathi... more The SCN encephalopathies are one of the rare early childhood intractable epileptic encephalopathies associated with pleomorphic seizures, cognitive decline, motor, and behavioral abnormalities that begin in early infancy. There is a dearth of data on phenotype and genotype of SCN encephalopathies from the Indian subcontinent, hence we are reporting clinical and molecular profile and outcome of SCN developmental and epileptic encephalopathies. This is a retrospective chart review of SCN developmental and epileptic encephalopathies in a tertiary care center, Bangalore, India between January 2015 and March 2020. All children with clinical features of SCN developmental and epileptic encephalopathies and confirmed with pathogenic variants were included. A total of 50 cases of SCN developmental and epileptic encephalopathies were analyzed, 31 of them were male and the mean age of presentation was 7.8 months. Precipitating factors for the first episode of seizure were fever and vaccination...
Annals of Indian Academy of Neurology ¦ Volume 24 ¦ Issue 5 ¦ September-October 2021 799 Influenz... more Annals of Indian Academy of Neurology ¦ Volume 24 ¦ Issue 5 ¦ September-October 2021 799 Influenza‐associated MOG antibody‐positive longitudinally extensive transverse myelitis: A case report. BMC Neurol 2014;14:224. 4. Wynford‐Thomas R, Jacob A, Tomassini V. Neurological update: MOG antibody disease. J Neurol 2018;266:1280‐6. 5. Girija AS, Rafeeque M, Abdurehman KP. Neurological complications of chickenpox. Ann Indian Acad Neurol 2007;10:240‐6. 6. Lee YJ. Acute disseminated encephalomyelitis in children: Differential diagnosis from multiple sclerosis on the basis of clinical course. Korean J Pediatr 2011;54:234‐40. 7. Wang Q, Cai L, Wang X. Acute disseminated encephalomyelitis following varicella‐zoster virus infection: Case report of effective treated both in clinical symptom and neuroimaging. Brain Behav 2019;9:e01374. 8. Hennes E, Baumann M, Schanda K, Anlar B, Bajer‐Kornek B, This is an open access journal, and articles are distributed under the terms of the Creative Commons
The study attempts to characterize the clinical, demographic, risk factors, electroencephalograph... more The study attempts to characterize the clinical, demographic, risk factors, electroencephalographical, and neuroimaging features of hot-water epilepsy (HWE) in children. This is a hospital-based observational study in the pediatric neurology clinic and who met the clinical definition of hot-water epilepsy were studied from January 2017 to October 2018. Clinical history, demographic data, and examination findings were recorded in a pre-structured proforma. Electroencephalography (EEG) and neuroimaging were carried out. A total of 68 children with male to female ratio of 2.4:1 were studied. The most common age of onset of seizures was between 1 and 5 y. Focal seizures with impaired awareness were the most common semiology (48.5%). Abnormal EEG was detected in 13.2% and abnormal neuroimaging in 4.4% which consisted of incidental abnormalities. Nonreflex seizures occurred in 35.3% of the children with HWE and the risk factors associated with this were not statistically significant. Clobazam before taking bath helped to achieve seizure control in 85.7% of the children. Hot-water epilepsy should be suspected in children who develop seizures following a hot-water bath. The most common age of onset is 1–5 y. EEG and neuroimaging are normal in the majority of cases. Nonreflex seizures occurred in 35.3% of the children.
Biotinidase deficiency is a treatable neurometabolic disorder. It usually presents during the fir... more Biotinidase deficiency is a treatable neurometabolic disorder. It usually presents during the first year of life with seizures, ataxia, hypotonia, vision and hearing disturbance, alopecia, and skin rashes. It can have various neuroimaging findings but demyelinating leukoencephalopathy is an unusual finding in children with biotinidase deficiency that can cause diagnostic challenge as it can radiologically mimic perinatal hypoxic–ischemic encephalopathy or other leukodystrophies. It reverses with early diagnosis and treatment with biotin supplementation and the outcome is rewarding.
Introduction: Comorbid conditions such as intellectual disability (ID), visual disability, epilep... more Introduction: Comorbid conditions such as intellectual disability (ID), visual disability, epilepsy, and hearing impairment are associated with cerebral palsy (CP). For many children with cerebral palsy, it is these cooccurring conditions that may often have the greatest impact on the child and family from varying perspective. Aims: The aim of the current study was to provide data on the frequency and type of comorbidities in children with cerebral palsy and the burden of comorbidities among each motor subtypes of CP. In addition, we evaluated the causative agent of motor subtypes of CP. Methods: Two hundred consecutive children with cerebral palsy attending the pediatric neurology outpatient department with an age group from 3 months to 18 years were enrolled in the study. Information on neurologic subtype classified according to the topographic distribution of the motor impairment on neurologic examination and the presence of comorbidities: ID, visual impairment, hearing impairment, and coexisting afebrile seizures, was obtained. Demographic factors were also noted. Results: The mean age of the children was 55 months, with 120 boys and 80 girls. ID was seen in 91% (182/200) of children. Active afebrile seizure disorder was noted in 40% (80/200), severe auditory impairment was noted in 4% (8/200), and cortical visual impairment was noted in 19.5% (39/200) of children. Comorbidities were most frequent in children with spastic and dyskinetic cerebral palsy. The most common type of seizures was focal seizures in 47 individuals (23.5% of all the individuals), followed by generalized seizures in 23 individuals (11.5% of all the individuals) and myoclonic seizures in 11 individuals (5.5% of all the individuals). Conclusion: ID and seizures are more frequently associated comorbidities with CP. Bilateral spastic and dyskinetic CP are more likely to be associated with comorbidities.
Objective: To study the etiology and clinical profile of non-traumatic coma in children at tertia... more Objective: To study the etiology and clinical profile of non-traumatic coma in children at tertiary care center and to determine the predictors of outcome. Methods: One hundred and four consecutive children between 2 mo-12 yr were studied. The clinical signs at admission; vital signs, Glasgow coma scale, respiratory pattern, papillary reflex, extra-ocular movements, fundus picture and motor deficits were recorded. Etiology of coma was determined by clinical history, examination and relevant investigations. Their progress was monitored clinically, biochemically and with multi-system monitors. Outcome was recorded as survived or died. Results: Etiology of coma in 65% cases was intracranial infections; other causes were metabolic (20%). Sixty-seven percent recovered completely, 16% had residual neurodeficits, 16% died. Survival was better in children with intracranial infections (13%) as compared to metabolic coma (33%). On multivariate logistic regression, bradycardia, hypoten- sion, abnormal respiratory pattern (especially, ataxic type), duration of coma more than 48 h, Glasgow coma scale < 7 at ad- mission, unequal and non-reactive pupils, papilledema, abnormal extra-ocular movements, motor deficits, signs of meningitis correlated with mortality. Requirement of ventilatory support and abnormal computerized tomography findings correlated with mortality. Conclusions: Intracranial infections were the most common cause of non-traumatic coma in children; the most common cause of death being metabolic coma. Simple clinical signs and relevant investigations served as prognostic indicators of outcome.
Background West's syndrome (WS) is a triad of epileptic spasms (ESs), psychomotor delay, and ... more Background West's syndrome (WS) is a triad of epileptic spasms (ESs), psychomotor delay, and hypsarrhythmia. The treatment of ESs is still controversial. Hence, we designed a randomized controlled trial (RCT) to compare the outcomes in children with WS treated with adrenocorticotropic hormone (ACTH) alone versus ACTH and levetiracetam (LEV). Objectives To compare the treatment outcomes and side effects in children treated with ACTH alone versus ACTH and LEV. Methods This prospective randomized controlled trial was conducted from December 2017 to May 2019 in tertiary care center, Bangaluru. Children from 2 months to 5 years of age, diagnosed with WS were included. Fifty children in each group were analyzed for efficacy and side effects. Results There was no difference in the baseline characteristics in both groups. There was no difference in spasms response at the end of 2 weeks between the groups (88 vs. 82%) with p-value of 0.813. The relapse rates were less in ACTH and LEV gro...
Pathogenic variants in A Disintegrin And Metalloproteinase (ADAM) 22, the postsynaptic cell membr... more Pathogenic variants in A Disintegrin And Metalloproteinase (ADAM) 22, the postsynaptic cell membrane receptor for the glycoprotein leucine-rich repeat glioma-inactivated protein 1 (LGI1), have been recently associated with recessive developmental and epileptic encephalopathy. However, so far, only two affected individuals have been described and many features of this disorder are unknown. We refine the phenotype and report 19 additional individuals harbouring compound heterozygous or homozygous inactivating ADAM22 variants, of whom 18 had clinical data available. Additionally, we provide follow-up data from two previously reported cases. All affected individuals exhibited infantile-onset, treatment-resistant epilepsy. Additional clinical features included moderate to profound global developmental delay/intellectual disability (20/20), hypotonia (12/20) and delayed motor development (19/20). Brain MRI findings included cerebral atrophy (13/20), supported by post-mortem histological e...
Hyperphosphatemic familial tumoral calcinosis (HFTC) presents with varied neurological manifestat... more Hyperphosphatemic familial tumoral calcinosis (HFTC) presents with varied neurological manifestations that have been reported in the literature like facial palsy, vision and hearing impairment, stroke, and headache. In this article, we reported a 12-year-old girl child patient with recurrent facial weakness with bilateral hearing impairment and multiple ulcerative lesions on lower limbs and elbows. On examination, she had lower motor neuron (LMN) facial palsy with conductive hearing loss. The investigations showed hyperphosphatemia (9.3 mg/dL) with normal serum calcium (10.4 mg/dL), alkaline phosphatase (147.9 U/L), parathyroid hormone (23.12 pg/mL), and renal function tests. Elevated serum calcium and phosphorus product (96.72 mg2/mL2) and elevated renal tubular reabsorption of phosphate (TMPxGFR) value (9.16) were noted. Skeletal survey showed hyperostosis in the long bone diaphysis, vertebrae, ribs, pelvic bone, skull, and facial bones with narrowing of cranial ostium, characteri...
Hemiconvulsion-hemiplegia-epilepsy syndrome is an uncommon consequence of prolonged focal febrile... more Hemiconvulsion-hemiplegia-epilepsy syndrome is an uncommon consequence of prolonged focal febrile convulsive seizures in infancy and early childhood. We report two cases of hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and had striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere as documented by magnetic resonance imaging, including extensive diffusion weighted imaging abnormalities in both the cases.
The SCN encephalopathies are one of the rare early childhood intractable epileptic encephalopathi... more The SCN encephalopathies are one of the rare early childhood intractable epileptic encephalopathies associated with pleomorphic seizures, cognitive decline, motor, and behavioral abnormalities that begin in early infancy. There is a dearth of data on phenotype and genotype of SCN encephalopathies from the Indian subcontinent, hence we are reporting clinical and molecular profile and outcome of SCN developmental and epileptic encephalopathies. This is a retrospective chart review of SCN developmental and epileptic encephalopathies in a tertiary care center, Bangalore, India between January 2015 and March 2020. All children with clinical features of SCN developmental and epileptic encephalopathies and confirmed with pathogenic variants were included. A total of 50 cases of SCN developmental and epileptic encephalopathies were analyzed, 31 of them were male and the mean age of presentation was 7.8 months. Precipitating factors for the first episode of seizure were fever and vaccination...
Annals of Indian Academy of Neurology ¦ Volume 24 ¦ Issue 5 ¦ September-October 2021 799 Influenz... more Annals of Indian Academy of Neurology ¦ Volume 24 ¦ Issue 5 ¦ September-October 2021 799 Influenza‐associated MOG antibody‐positive longitudinally extensive transverse myelitis: A case report. BMC Neurol 2014;14:224. 4. Wynford‐Thomas R, Jacob A, Tomassini V. Neurological update: MOG antibody disease. J Neurol 2018;266:1280‐6. 5. Girija AS, Rafeeque M, Abdurehman KP. Neurological complications of chickenpox. Ann Indian Acad Neurol 2007;10:240‐6. 6. Lee YJ. Acute disseminated encephalomyelitis in children: Differential diagnosis from multiple sclerosis on the basis of clinical course. Korean J Pediatr 2011;54:234‐40. 7. Wang Q, Cai L, Wang X. Acute disseminated encephalomyelitis following varicella‐zoster virus infection: Case report of effective treated both in clinical symptom and neuroimaging. Brain Behav 2019;9:e01374. 8. Hennes E, Baumann M, Schanda K, Anlar B, Bajer‐Kornek B, This is an open access journal, and articles are distributed under the terms of the Creative Commons
The study attempts to characterize the clinical, demographic, risk factors, electroencephalograph... more The study attempts to characterize the clinical, demographic, risk factors, electroencephalographical, and neuroimaging features of hot-water epilepsy (HWE) in children. This is a hospital-based observational study in the pediatric neurology clinic and who met the clinical definition of hot-water epilepsy were studied from January 2017 to October 2018. Clinical history, demographic data, and examination findings were recorded in a pre-structured proforma. Electroencephalography (EEG) and neuroimaging were carried out. A total of 68 children with male to female ratio of 2.4:1 were studied. The most common age of onset of seizures was between 1 and 5 y. Focal seizures with impaired awareness were the most common semiology (48.5%). Abnormal EEG was detected in 13.2% and abnormal neuroimaging in 4.4% which consisted of incidental abnormalities. Nonreflex seizures occurred in 35.3% of the children with HWE and the risk factors associated with this were not statistically significant. Clobazam before taking bath helped to achieve seizure control in 85.7% of the children. Hot-water epilepsy should be suspected in children who develop seizures following a hot-water bath. The most common age of onset is 1–5 y. EEG and neuroimaging are normal in the majority of cases. Nonreflex seizures occurred in 35.3% of the children.
Biotinidase deficiency is a treatable neurometabolic disorder. It usually presents during the fir... more Biotinidase deficiency is a treatable neurometabolic disorder. It usually presents during the first year of life with seizures, ataxia, hypotonia, vision and hearing disturbance, alopecia, and skin rashes. It can have various neuroimaging findings but demyelinating leukoencephalopathy is an unusual finding in children with biotinidase deficiency that can cause diagnostic challenge as it can radiologically mimic perinatal hypoxic–ischemic encephalopathy or other leukodystrophies. It reverses with early diagnosis and treatment with biotin supplementation and the outcome is rewarding.
Introduction: Comorbid conditions such as intellectual disability (ID), visual disability, epilep... more Introduction: Comorbid conditions such as intellectual disability (ID), visual disability, epilepsy, and hearing impairment are associated with cerebral palsy (CP). For many children with cerebral palsy, it is these cooccurring conditions that may often have the greatest impact on the child and family from varying perspective. Aims: The aim of the current study was to provide data on the frequency and type of comorbidities in children with cerebral palsy and the burden of comorbidities among each motor subtypes of CP. In addition, we evaluated the causative agent of motor subtypes of CP. Methods: Two hundred consecutive children with cerebral palsy attending the pediatric neurology outpatient department with an age group from 3 months to 18 years were enrolled in the study. Information on neurologic subtype classified according to the topographic distribution of the motor impairment on neurologic examination and the presence of comorbidities: ID, visual impairment, hearing impairment, and coexisting afebrile seizures, was obtained. Demographic factors were also noted. Results: The mean age of the children was 55 months, with 120 boys and 80 girls. ID was seen in 91% (182/200) of children. Active afebrile seizure disorder was noted in 40% (80/200), severe auditory impairment was noted in 4% (8/200), and cortical visual impairment was noted in 19.5% (39/200) of children. Comorbidities were most frequent in children with spastic and dyskinetic cerebral palsy. The most common type of seizures was focal seizures in 47 individuals (23.5% of all the individuals), followed by generalized seizures in 23 individuals (11.5% of all the individuals) and myoclonic seizures in 11 individuals (5.5% of all the individuals). Conclusion: ID and seizures are more frequently associated comorbidities with CP. Bilateral spastic and dyskinetic CP are more likely to be associated with comorbidities.
Objective: To study the etiology and clinical profile of non-traumatic coma in children at tertia... more Objective: To study the etiology and clinical profile of non-traumatic coma in children at tertiary care center and to determine the predictors of outcome. Methods: One hundred and four consecutive children between 2 mo-12 yr were studied. The clinical signs at admission; vital signs, Glasgow coma scale, respiratory pattern, papillary reflex, extra-ocular movements, fundus picture and motor deficits were recorded. Etiology of coma was determined by clinical history, examination and relevant investigations. Their progress was monitored clinically, biochemically and with multi-system monitors. Outcome was recorded as survived or died. Results: Etiology of coma in 65% cases was intracranial infections; other causes were metabolic (20%). Sixty-seven percent recovered completely, 16% had residual neurodeficits, 16% died. Survival was better in children with intracranial infections (13%) as compared to metabolic coma (33%). On multivariate logistic regression, bradycardia, hypoten- sion, abnormal respiratory pattern (especially, ataxic type), duration of coma more than 48 h, Glasgow coma scale < 7 at ad- mission, unequal and non-reactive pupils, papilledema, abnormal extra-ocular movements, motor deficits, signs of meningitis correlated with mortality. Requirement of ventilatory support and abnormal computerized tomography findings correlated with mortality. Conclusions: Intracranial infections were the most common cause of non-traumatic coma in children; the most common cause of death being metabolic coma. Simple clinical signs and relevant investigations served as prognostic indicators of outcome.
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Papers by Dr.Vykunta Raju K.N