Introduction: Wada test is well-known to assess lateralization of memory and language functions, ... more Introduction: Wada test is well-known to assess lateralization of memory and language functions, however super-selective Wada (ss-Wada) to evaluate motor leg function is rare. We present a ss-Wada test within the anterior cerebral artery (ACA) to assess the motor function of the leg. Methods: Retrospective chart review. Results: Comprehensive phase-I/II surgical evaluation revealed an ictal focus around the left post-central gyrus with immediate involvement around left para-central regions. To avoid potential right leg motor dysfunction with the surgery, the patient underwent a ss-Wada procedure. Angiography revealed bilateral ACAs were supplied by the left A1 segment. Super-selective microcatheter injection of amobarbital into the left ACA was performed to avoid cross-filling the contralateral ACA. The ss-Wada test confirmed no right leg motor impairment. Afterward, a craniotomy with direct cortical stimulation confirmed that the left-sided ictal/peri-ictal zone had no clear leg mo...
ObjectiveImprove data‐driven research to inform clinical decision‐making with pediatric epilepsy ... more ObjectiveImprove data‐driven research to inform clinical decision‐making with pediatric epilepsy surgery patients by expanding the Pediatric Epilepsy Research Consortium Epilepsy Surgery (PERC‐Surgery) Workgroup to include neuropsychological data. This article reports on the process and initial success of this effort and characterizes the cognitive functioning of the largest multi‐site pediatric epilepsy surgery cohort in the United States.MethodsPediatric neuropsychologists from 18 institutions completed surveys regarding neuropsychological practice and the impact of involvement in the collaborative. Neuropsychological data were entered through an online database. Descriptive analyses examined the survey responses and cognitive functioning of the cohort. Statistical analyses examined which patients were evaluated and if composite scores differed by domain, demographics, measures used, or epilepsy characteristics.ResultsPositive impact of participation was evident by attendance, sur...
Incidence of epilepsy is highest in the first years of life. 1-3 Drug-resistant epilepsy (DRE) oc... more Incidence of epilepsy is highest in the first years of life. 1-3 Drug-resistant epilepsy (DRE) occurs in one third of all people with epilepsy, with higher than average rates in children <3 years of age, ranging from 35% to 65%, possibly due to specific etiologies presenting in early childhood, such as structural brain abnormalities, early onset neurogenetic and metabolic disorders, and perinatal brain injury, each highly associated with intractability. 4-7 Age at seizure onset of <1 year and preexisting developmental delays predict development of DRE, yet one quarter of children without these characteristics will develop DRE within 1 year of seizure onset. 6 Early recognition and resolution of DRE is essential to optimize developmental
Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance tha... more Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance that they project through the floor of the middle fossa they are secondary. In this case report the authors report on the management of a giant PE extending through the floor of the middle fossa.An 8-month-old boy presented to the authors’ service with a large PE projecting into his neck through a missing left middle fossa floor; the lesion was causing significant meta-, dys-, and hypoplasia of the structures of the anterolateral neck on that side. Surgical goals for this patient included the following: 1) removal of potentially epileptogenic and dysfunctional tissue; 2) preservation of cranial nerves; 3) prevention of cognitive decline or iatrogenic deficit; 4) prevention of CSF leak; 5) reconstruction of skull base; 6) prevention of airway and swallowing compromise; and 7) cosmesis. After a multidisciplinary evaluation with ENT, plastic surgery, and neurology, an operation was performed u...
First-line treatment for epilepsy is antiepileptic drug and requires an interdisciplinary approac... more First-line treatment for epilepsy is antiepileptic drug and requires an interdisciplinary approach and enduring commitment and adherence from the patient and family for successful outcome. Despite adherence to antiepileptic drugs, refractory epilepsy occurs in approximately 30% of children with epilepsy, and surgical treatment is an important intervention to consider. Surgical management of pediatric epilepsy is highly effective in selected patients with refractory epilepsy; however, an evidence-based protocol, including best methods of presurgical imaging assessments, and neurodevelopmental and/or behavioral health assessments, is not currently available for clinicians. Surgical treatment of epilepsy can be critical to avoid negative outcomes in functional, cognitive, and behavioral health status. Furthermore, it is often the only method to achieve seizure freedom in refractory epilepsy. Although a large literature base can be found for adults with refractory epilepsy undergoing surgical treatment, less is known about how surgical management affects outcomes in children with epilepsy. The purpose of the review was fourfold: (1) to evaluate the available literature regarding presurgical assessment and postsurgical outcomes in children with medically refractory epilepsy, (2) to identify gaps in our knowledge of surgical treatment and its outcomes in children with epilepsy, (3) to pose questions for further research, and (4) to advocate for a more unified presurgical evaluation protocol including earlier referral for surgical candidacy of pediatric patients with refractory epilepsy. Despite its effectiveness, epilepsy surgery remains an underutilized but evidence-based approach that could lead to positive short- and long-term outcomes for children with refractory epilepsy.
This case report describes a toddler with a medical history of biotinidase deficiency who present... more This case report describes a toddler with a medical history of biotinidase deficiency who presented with atypical seizures due to a brain tumor. This is a case report. Electroencephalogram revealed a frontal lobe mass, with magnetic resonance imaging confirmation of a mass extending from the frontal lobe into the genu and anterior corpus callosum. She underwent a near-total resection, and pathology identified a dysembryoplastic neuroepithelial tumor. The patient is now seizure free and clinically doing well. Children with biotinidase deficiency and atypical seizures should receive a full electroencephalogram evaluation, as brain tumors continue to be on the differential for seizures in this patient population.
Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a se... more Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a seizure disorder. Head up tilt test (HUTT) has been used to confirm this diagnosis. There is no data available of its use in children / adolescents from India. To study the usefulness of the HUTT in children and adolescents with suspected NCS. This was a part retrospective and later prospective study set in a tertiary child neurology outpatient department (OPD). Patients with a strong clinical suspicion of syncope were recruited for the study. Clinical and treatment details were either retrieved from the chart or prospectively recorded in later patients. The HUTT was then carried out at baseline and after provocation and the results correlated with the clinical diagnosis. Eighteen children with a mean age of 10.8 years were studied. Eight had precipitating factors. Thirteen had premonitory symptoms. Pallor, temperature change, diaphoresis, headache, tonic / clonic movements, post-ictal confusion and peri-ictal headache were symptoms noticed. Sixteen had a positive HUTT. Seven were on long-term anti-epileptic drugs (AEDs). Two had epileptiform abnormalities on their electroencephalogram (EEG). The diagnosis of syncope is often confused with epilepsy. Head up tilt test has a high sensitivity in the diagnosis of NCS in children / adolescents. It is fairly safe and easy to perform.
Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a se... more Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a seizure disorder. Head up tilt test (HUTT) has been used to confirm this diagnosis. There is no data available of its use in children / adolescents from India. To study the usefulness of the HUTT in children and adolescents with suspected NCS. This was a part retrospective and later prospective study set in a tertiary child neurology outpatient department (OPD). Patients with a strong clinical suspicion of syncope were recruited for the study. Clinical and treatment details were either retrieved from the chart or prospectively recorded in later patients. The HUTT was then carried out at baseline and after provocation and the results correlated with the clinical diagnosis. Eighteen children with a mean age of 10.8 years were studied. Eight had precipitating factors. Thirteen had premonitory symptoms. Pallor, temperature change, diaphoresis, headache, tonic / clonic movements, post-ictal con...
Approximately 15% of patients with Sturge-Weber syndrome have bilateral intracranial involvement ... more Approximately 15% of patients with Sturge-Weber syndrome have bilateral intracranial involvement and the prognosis of these patients is believed to be particularly unfavorable. We reviewed the clinical and neuroimaging features of patients with Sturge-Weber syndrome and bilateral intracranial involvement. Seizure variables, presence of hemiparesis and degree of developmental impairment at last follow-up were compared to imaging abnormalities. Out of 110 Sturge-Weber syndrome patients studied, 14 had bilateral brain involvement, which always showed an asymmetric pattern on glucose metabolism positron emission tomography. Although most patients had frequent seizures initially, associated with frontal hypometabolism on positron emission tomography, six patients (43%) had achieved good seizure-control during follow-up. Bilateral frontal hypometabolism was associated with severe developmental impairment; two children with bitemporal hypometabolism showed autistic features. Hemiparesis was associated with superior frontal (motor cortex) hypometabolism. Three patients had resective surgery, resulting in improved seizure control and/or developmental outcome. The severity of neurological complications and clinical course depend highly on the extent of cortical dysfunction in bilateral Sturge-Weber syndrome; bilateral frontal and temporal hypometabolism is associated with poor developmental outcome. Nevertheless, good seizure control and only mild/moderate developmental impairment can be achieved in about half of bilateral Sturge-Weber syndrome patients, with or without resective surgery.
Extrapontine and central pontine myelinolysis (EPM/CPM) are rare events in pediatric neurology bu... more Extrapontine and central pontine myelinolysis (EPM/CPM) are rare events in pediatric neurology but can have devastating consequences. They are most commonly associated with rapid correction of hyponatremia but have been reported in other situations as well. This condition is relatively more common in adult neurology, not surprisingly, as alcoholism and associated malnutrition are often predisposing conditions. There have been few case reports in children with regards to this. We describe a 4-year old who presented with focal neurological deficits in the setting of diabetic ketoacidosis and the ensuing underlying osmotic imbalances. The patient made a remarkable recovery with no deficits of note-cognitive or motor. To our knowledge this is the youngest case reported so far of EPM in a child with diabetic ketoacidosis. The history of the condition, early animal experiments, clinicopathologic correlates, previous case reports and other scenarios in which this unusual event can occur are discussed--though the exact pathogenesis of this condition still remains unclear. We hope to bring to the attention of clinicians caring for children in the acute care setting, the importance of gradual correction of serum osmolality to reduce morbidity and mortality.
Introduction: Wada test is well-known to assess lateralization of memory and language functions, ... more Introduction: Wada test is well-known to assess lateralization of memory and language functions, however super-selective Wada (ss-Wada) to evaluate motor leg function is rare. We present a ss-Wada test within the anterior cerebral artery (ACA) to assess the motor function of the leg. Methods: Retrospective chart review. Results: Comprehensive phase-I/II surgical evaluation revealed an ictal focus around the left post-central gyrus with immediate involvement around left para-central regions. To avoid potential right leg motor dysfunction with the surgery, the patient underwent a ss-Wada procedure. Angiography revealed bilateral ACAs were supplied by the left A1 segment. Super-selective microcatheter injection of amobarbital into the left ACA was performed to avoid cross-filling the contralateral ACA. The ss-Wada test confirmed no right leg motor impairment. Afterward, a craniotomy with direct cortical stimulation confirmed that the left-sided ictal/peri-ictal zone had no clear leg mo...
ObjectiveImprove data‐driven research to inform clinical decision‐making with pediatric epilepsy ... more ObjectiveImprove data‐driven research to inform clinical decision‐making with pediatric epilepsy surgery patients by expanding the Pediatric Epilepsy Research Consortium Epilepsy Surgery (PERC‐Surgery) Workgroup to include neuropsychological data. This article reports on the process and initial success of this effort and characterizes the cognitive functioning of the largest multi‐site pediatric epilepsy surgery cohort in the United States.MethodsPediatric neuropsychologists from 18 institutions completed surveys regarding neuropsychological practice and the impact of involvement in the collaborative. Neuropsychological data were entered through an online database. Descriptive analyses examined the survey responses and cognitive functioning of the cohort. Statistical analyses examined which patients were evaluated and if composite scores differed by domain, demographics, measures used, or epilepsy characteristics.ResultsPositive impact of participation was evident by attendance, sur...
Incidence of epilepsy is highest in the first years of life. 1-3 Drug-resistant epilepsy (DRE) oc... more Incidence of epilepsy is highest in the first years of life. 1-3 Drug-resistant epilepsy (DRE) occurs in one third of all people with epilepsy, with higher than average rates in children <3 years of age, ranging from 35% to 65%, possibly due to specific etiologies presenting in early childhood, such as structural brain abnormalities, early onset neurogenetic and metabolic disorders, and perinatal brain injury, each highly associated with intractability. 4-7 Age at seizure onset of <1 year and preexisting developmental delays predict development of DRE, yet one quarter of children without these characteristics will develop DRE within 1 year of seizure onset. 6 Early recognition and resolution of DRE is essential to optimize developmental
Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance tha... more Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance that they project through the floor of the middle fossa they are secondary. In this case report the authors report on the management of a giant PE extending through the floor of the middle fossa.An 8-month-old boy presented to the authors’ service with a large PE projecting into his neck through a missing left middle fossa floor; the lesion was causing significant meta-, dys-, and hypoplasia of the structures of the anterolateral neck on that side. Surgical goals for this patient included the following: 1) removal of potentially epileptogenic and dysfunctional tissue; 2) preservation of cranial nerves; 3) prevention of cognitive decline or iatrogenic deficit; 4) prevention of CSF leak; 5) reconstruction of skull base; 6) prevention of airway and swallowing compromise; and 7) cosmesis. After a multidisciplinary evaluation with ENT, plastic surgery, and neurology, an operation was performed u...
First-line treatment for epilepsy is antiepileptic drug and requires an interdisciplinary approac... more First-line treatment for epilepsy is antiepileptic drug and requires an interdisciplinary approach and enduring commitment and adherence from the patient and family for successful outcome. Despite adherence to antiepileptic drugs, refractory epilepsy occurs in approximately 30% of children with epilepsy, and surgical treatment is an important intervention to consider. Surgical management of pediatric epilepsy is highly effective in selected patients with refractory epilepsy; however, an evidence-based protocol, including best methods of presurgical imaging assessments, and neurodevelopmental and/or behavioral health assessments, is not currently available for clinicians. Surgical treatment of epilepsy can be critical to avoid negative outcomes in functional, cognitive, and behavioral health status. Furthermore, it is often the only method to achieve seizure freedom in refractory epilepsy. Although a large literature base can be found for adults with refractory epilepsy undergoing surgical treatment, less is known about how surgical management affects outcomes in children with epilepsy. The purpose of the review was fourfold: (1) to evaluate the available literature regarding presurgical assessment and postsurgical outcomes in children with medically refractory epilepsy, (2) to identify gaps in our knowledge of surgical treatment and its outcomes in children with epilepsy, (3) to pose questions for further research, and (4) to advocate for a more unified presurgical evaluation protocol including earlier referral for surgical candidacy of pediatric patients with refractory epilepsy. Despite its effectiveness, epilepsy surgery remains an underutilized but evidence-based approach that could lead to positive short- and long-term outcomes for children with refractory epilepsy.
This case report describes a toddler with a medical history of biotinidase deficiency who present... more This case report describes a toddler with a medical history of biotinidase deficiency who presented with atypical seizures due to a brain tumor. This is a case report. Electroencephalogram revealed a frontal lobe mass, with magnetic resonance imaging confirmation of a mass extending from the frontal lobe into the genu and anterior corpus callosum. She underwent a near-total resection, and pathology identified a dysembryoplastic neuroepithelial tumor. The patient is now seizure free and clinically doing well. Children with biotinidase deficiency and atypical seizures should receive a full electroencephalogram evaluation, as brain tumors continue to be on the differential for seizures in this patient population.
Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a se... more Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a seizure disorder. Head up tilt test (HUTT) has been used to confirm this diagnosis. There is no data available of its use in children / adolescents from India. To study the usefulness of the HUTT in children and adolescents with suspected NCS. This was a part retrospective and later prospective study set in a tertiary child neurology outpatient department (OPD). Patients with a strong clinical suspicion of syncope were recruited for the study. Clinical and treatment details were either retrieved from the chart or prospectively recorded in later patients. The HUTT was then carried out at baseline and after provocation and the results correlated with the clinical diagnosis. Eighteen children with a mean age of 10.8 years were studied. Eight had precipitating factors. Thirteen had premonitory symptoms. Pallor, temperature change, diaphoresis, headache, tonic / clonic movements, post-ictal confusion and peri-ictal headache were symptoms noticed. Sixteen had a positive HUTT. Seven were on long-term anti-epileptic drugs (AEDs). Two had epileptiform abnormalities on their electroencephalogram (EEG). The diagnosis of syncope is often confused with epilepsy. Head up tilt test has a high sensitivity in the diagnosis of NCS in children / adolescents. It is fairly safe and easy to perform.
Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a se... more Neurocardiogenic syncope (NCS) is a common paroxysmal disorder that is often misdiagnosed as a seizure disorder. Head up tilt test (HUTT) has been used to confirm this diagnosis. There is no data available of its use in children / adolescents from India. To study the usefulness of the HUTT in children and adolescents with suspected NCS. This was a part retrospective and later prospective study set in a tertiary child neurology outpatient department (OPD). Patients with a strong clinical suspicion of syncope were recruited for the study. Clinical and treatment details were either retrieved from the chart or prospectively recorded in later patients. The HUTT was then carried out at baseline and after provocation and the results correlated with the clinical diagnosis. Eighteen children with a mean age of 10.8 years were studied. Eight had precipitating factors. Thirteen had premonitory symptoms. Pallor, temperature change, diaphoresis, headache, tonic / clonic movements, post-ictal con...
Approximately 15% of patients with Sturge-Weber syndrome have bilateral intracranial involvement ... more Approximately 15% of patients with Sturge-Weber syndrome have bilateral intracranial involvement and the prognosis of these patients is believed to be particularly unfavorable. We reviewed the clinical and neuroimaging features of patients with Sturge-Weber syndrome and bilateral intracranial involvement. Seizure variables, presence of hemiparesis and degree of developmental impairment at last follow-up were compared to imaging abnormalities. Out of 110 Sturge-Weber syndrome patients studied, 14 had bilateral brain involvement, which always showed an asymmetric pattern on glucose metabolism positron emission tomography. Although most patients had frequent seizures initially, associated with frontal hypometabolism on positron emission tomography, six patients (43%) had achieved good seizure-control during follow-up. Bilateral frontal hypometabolism was associated with severe developmental impairment; two children with bitemporal hypometabolism showed autistic features. Hemiparesis was associated with superior frontal (motor cortex) hypometabolism. Three patients had resective surgery, resulting in improved seizure control and/or developmental outcome. The severity of neurological complications and clinical course depend highly on the extent of cortical dysfunction in bilateral Sturge-Weber syndrome; bilateral frontal and temporal hypometabolism is associated with poor developmental outcome. Nevertheless, good seizure control and only mild/moderate developmental impairment can be achieved in about half of bilateral Sturge-Weber syndrome patients, with or without resective surgery.
Extrapontine and central pontine myelinolysis (EPM/CPM) are rare events in pediatric neurology bu... more Extrapontine and central pontine myelinolysis (EPM/CPM) are rare events in pediatric neurology but can have devastating consequences. They are most commonly associated with rapid correction of hyponatremia but have been reported in other situations as well. This condition is relatively more common in adult neurology, not surprisingly, as alcoholism and associated malnutrition are often predisposing conditions. There have been few case reports in children with regards to this. We describe a 4-year old who presented with focal neurological deficits in the setting of diabetic ketoacidosis and the ensuing underlying osmotic imbalances. The patient made a remarkable recovery with no deficits of note-cognitive or motor. To our knowledge this is the youngest case reported so far of EPM in a child with diabetic ketoacidosis. The history of the condition, early animal experiments, clinicopathologic correlates, previous case reports and other scenarios in which this unusual event can occur are discussed--though the exact pathogenesis of this condition still remains unclear. We hope to bring to the attention of clinicians caring for children in the acute care setting, the importance of gradual correction of serum osmolality to reduce morbidity and mortality.
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Papers by Samir Karia