New-onset Seizures Research Articles

Introducing the “Temporal Thumb Sign” in Pediatric Patients With New-Onset Idiopathic Seizures With and Without Elevated Cerebrospinal Fluid Opening Pressure

BackgroundTemporal lobe changes, such as anterior temporal lobe meningoceles or encephaloceles, have been documented as possible epileptogenic foci in a subset of pediatric patients with seizures. In our study, we aim to analyze a different structural change in the temporal lobe, remodeling of the posterior temporal skull base by the inferior temporal gyrus called the “temporal thumb sign” (TTS), in pediatric patients presenting with new-onset seizures with or without elevated opening pressure (OP), patients presenting with confirmed diagnosis of idiopathic intracranial hypertension (IIH) without seizure presentation, and healthy controls. MethodsMagnetic resonance imaging scans of 163 pediatric patients were studied retrospectively for the presence of TTS. We analyzed the scans of 43 patients with elevated OP and confirmed IIH, 40 patients with elevated OP and new-onset idiopathic seizures, 40 patients with normal OP and new-onset idiopathic seizures, and 40 age- and sex-matched healthy controls. ResultsThe TTS was detected most frequently in patients with elevated OP and seizures at 72.5% compared with patients with IIH with no seizures and patients with normal OP and seizures (32.6% and 27.5%, respectively). The TTS had a frequency of 12.5% in the control group. The TTS had the highest combination of specificity and sensitivity (72.5% and 72.5%) in patients with seizures and elevated OP compared with patients with seizures and normal OP (P value < 0.001). ConclusionsOur results suggest the Kamali “temporal thumb sign” is a novel imaging feature that may be used as a sensitive and specific imaging finding associated with seizures and elevated OP in the pediatric population.

Pearls & Oy-sters: Status Epilepticus and Cerebral Edema From Hyperammonemia Due to Disseminated Ureaplasma and Mycoplasma Species.

Nonhepatic hyperammonemia syndrome is a rare cause of neurologic dysfunction and cerebral edema and has most commonly been reported in posttransplant patients. Only recently has opportunistic infection with Ureaplasma species and Mycoplasma hominis been found to be key to the pathogenesis. We describe the cases of 3 immunosuppressed patients who developed hyperammonemia syndrome with new-onset refractory status epilepticus and diffuse cerebral edema. PCR was positive for M hominis in 1 patient and Ureaplasma parvum in the other 2. Despite early diagnostic suspicion and aggressive management with empirical antibiotics, seizure control, hypertonic saline, and ammonia elimination, none of our patients survived this life-threatening infection. Nonhepatic hyperammonemia and new-onset seizures can be presenting features of disseminated Ureaplasma species and M hominis infections in posttransplant patients. Immunosuppression in the absence of organ transplantation is likely sufficient to trigger this entity, as was the case in our third patient. When suspected, empiric combination antibiotics should be used due to high likelihood of resistance. The diagnostic test of choice is PCR. Patients with hyperammonemia syndrome associated with these infections typically have a poor prognosis. Early recognition and aggressive multimodal interventions may be key to ameliorating the high mortality and severe neurologic sequelae from this entity.

Cerebral amyloid angiopathy-related inflammation with posterior reversible encephalopathy syndrome-like presentation: a case report

BackgroundCerebral amyloid angiopathy-related inflammation (CAA-RI), which presents with acute or subacute cognitive or functional decline, focal or multifocal neurologic deficits, new onset of seizures, or a combination of seizures and neurologic deficits, shares clinical and radiologic similarities with posterior reversible encephalopathy syndrome (PRES). Differential diagnosis is critical because the treatment principle for these 2 conditions differs greatly. Here, we present a case of PRES-like CAA-RI and the strategy used to discriminate between the 2 conditions.Case presentationA patient with probable CAA-RI was first thought to suffer from PRES. Initial high-dose methylprednisolone therapy caused rapid improvement of the neurologic symptoms but abrupt discontinuation of corticosteroids resulted in clinical relapse and deterioration. Subsequent reinitiation of high-dose methylprednisolone followed by tapering off of oral prednisone led to clinical and radiologic recovery at the 3-month follow-up.ConclusionsWe suggest that in cases where it is difficult to distinguish between CAA-RI and PRES solely based on magnetic resonance imaging, a good response to corticosteroids and an apolipoprotein E (ApoE) ε4/ε4 genotype are critical for establishing a diagnosis of CAA-RI. If there is clinical deterioration, sudden withdrawal of high-dose corticosteroid during the active phase of CAA-RI should be avoided.

Primary intraventricular synovial sarcoma of the brain with recurrence - case presentation

BackgroundWe report a case of recurrent primary intraventricular synovial sarcoma of the brain with no extracranial primary, initially reported as a haemangiopericytoma. We believe this is the first reported case of primary intraventricular synovial sarcoma at this site.Case presentationA 27-year-old male presented to hospital with a new onset of seizures. Imaging revealed a left ventricular trigone mass with surrounding oedema. He underwent a left occipito-temporal craniotomy and resection with the histology reported as haemangiopericytoma. Resection was followed by adjuvant radiation treatment. Seven years later follow-up imaging revealed a 4 mm contrast enhancing lesion in the previous surgical bed. The patient underwent resection. Histological analysis of the recurrence revealed a spindle cell tumour with a SS18 gene rearrangement consistent with synovial sarcoma. Retrospective fluorescent in-situ hybridisation analysis of original histology also revealed a SS18 gene rearrangement consistent with a diagnosis of synovial sarcoma.ConclusionSynovial sarcoma should be included as part of the differential diagnosis for patients presenting with intraventricular spindle cell tumours in the brain.

Stroke Characteristics as Predictors of New-onset Seizure in Patients with Acute Ischemic Stroke.

The current guidelines on diagnosis and management of new-onset seizures in stroke are not well defined, especially in the Indian setting. Our study aims at providing insight into the hospital prevalence risk of new-onset seizures following ischemic stroke and to correlate seizure risk with the characteristics of stroke and other clinical parameters. A total of 127 patients were analyzed for the study where we assessed the clinical severity and the imaging severity of stroke using the National Institute of Health Stroke Scale (NIHSS) score and Alberta Stroke Program Early CT (ASPECT) score, respectively. Seizure-related variables including semiology, timing, and details of antiepileptic drugs (AEDs) were assessed under the domain of early and late poststroke seizures (PSSs). All patients were followed for 6 months for the seizure recurrence and change in Barthel index. In statistical analysis, quantitative variables were compared using the independent t-test/Mann-Whitney U test, and qualitative variables were correlated using Chi-square test/Fisher's exact test. Univariate and multivariate logistic regression was used to find out the significant risk factors of acute symptomatic seizure. The mean age of the study population was 59.72 years (±14.77), with a male predominance (60.63%). About 78.74% of the cases had an NIHSS score more than or equal to 6.24% had posterior circulation strokes and the rest had anterior circulation strokes. A cortical location of infarct was observed in 62.2% of cases and a subcortical location in 61.4% of cases. The prevalence of early PSSs observed in our study was 10.6%. Of those, 80% had generalized seizures, 13.3% had focal seizures, and 6.67% had focal seizures with secondary generalizations. No patient in the study group had late-onset seizures. Total leukocyte count, serum protein levels, serum uric acid levels, and erythrocyte sedimentation rate (ESR) values were associated with early seizures (p&lt;0.05). Patients with early seizures were found to have a longer hospital stay (8 vs 6 days with p&lt;0.05). In the Trial of Org 10,172 in Acute Stroke Treatment (TOAST) etiological classification, an acute stroke of undetermined etiology was found to have a significant association with the occurrence of early seizure in both univariate and multivariate analysis [p = 0.030; odds ratio (OR) 4.735 (1.160-22.576)]. There was no difference in change in the Barthel index among the two groups. There was no recurrence of seizures in those who defaulted for AED and one patient had a seizure even on AED. Prophylactic AEDs in stroke patients based on stroke characteristics could not be ascertained, but the sample size was small. Knowing the fact that antiepileptics cause sedation and increase the chance of aspiration, continuing AEDs in patients who develop acute symptomatic seizures should be judged judiciously.

De Novo Seizures, Obsessive Compulsive Symptoms and Neutropenias in Patients on Clozapine: A Retrospective Cohort Study.

Clozapine has well-documented inter-ethnic variations in pharmacokinetics. There is a paucity of data about clozapine use and associated adverse events such as seizures, obsessive compulsive symptoms, neutropenia, and agranulocytosis, from India. This retrospective cohort study followed up 228 patients initiated on clozapine in a tertiary care referral center in India for an average of 10 years. We calculated incidence rates of new-onset seizures, new-onset obsessive compulsive symptoms, agranulocytosis, and neutropenia. We collected data on doses of clozapine used and serum assays and calculated concentration-to-dose (C/D) ratios. We also collected relevant clinical details about clozapine-induced seizures. In the sample, 16.8% had new-onset seizures, 12.3% had new-onset OC symptoms, 2.7% had neutropenia, and 0.9% had agranulocytosis. The mean C/D ratio was 2.09 (SD = 1.8). Almost half (46.3%) of available serum assays were in the supra-therapeutic range. Seizures were associated with a higher clozapine dose at one year (OR = 1.003; 95%CI = 1.000-1.006; P value = 0.045) and the presence of positive psychotic symptoms at one year (OR = 4.214; 95%CI = 1.894-9.373; P < 0.001). Compared to existing literature, Indians have a higher rate of clozapine- related seizures and need lower doses to reach therapeutic serum levels.

QLTI-15. VENOUS SINUS THROMBOSIS AFTER BRAIN TUMOR RESECTION

Abstract Post-operative venous sinus thrombosis (POVST) is an uncommon complication from a craniotomy for brain tumor resection. There are few data in how to treat POVST, including the use of anticoagulation and follow-up. It has been found that POVST is more common in dural-based lesions especially located on or near a sinus, however treatment has not been standardized. We retrospectively reviewed our POVST cases between January 2018 to December 2020 for brain tumor resection, whether we chose to start anticoagulation or not, and the eventual outcome. We also performed a literature review on the topic to compare findings and management. We identified 14 cases of POVST; 8 of the cases were for extra-axial masses. Of the 14, 6 POVST had clot involving the superior sagittal sinus (SSS), with 3 being discharged with a direct oral anticoagulant (DOAC) and one being discharged on warfarin. With transverse sinus involvement, 6 cases were noted (with no SSS clot), with 2 started on a DOAC and 2 being started on ASA 81mg. In the sigmoid sinus there were 2 cases and did not receive anticoagulation. Of the 14 total cases, 1 was symptomatic (new-onset seizures) necessitating surgical recanalization and no other cases had symptoms attributed to the POVST. Two patients died from unrelated reasons prior to receiving any outpatient imaging, and all other patients had recanalization of their involved sinuses within 2-5 months. There were no complications in patients receiving anticoagulation. Our literature review didn't show a standardized method in POVST treatment. There is also disagreement in what anticoagulation should be used, and if it is necessary. Based on our retrospective analysis and literature review, if the clot is symptomatic, involves the SSS, or propagates, we recommend anticoagulation. Outside the SSS, it should be determined on a case-by-case basis, but anticoagulation may not be necessary.

RBIO-03. A MULTI-INSTITUTIONAL EXPERIENCE OF GAMMATILE BRACHYTHERAPY AS TREATMENT FOR RECURRENT GLIOBLASTOMAS

Abstract BACKGROUND Recurrent glioblastoma remains a disease without established standard of care. Here, we report the safety profile and survival outcome of recurrent glioblastoma patients treated with maximal safe resection followed by intraoperative placement of GammaTile (GT), a collagen tile-embedded Cs-131 brachytherapy platform. METHODS The study included isocitrate Dehydrogenase (IDH) wild-type glioblastoma patients who suffered tissue confirmed recurrence after concurrent temozolomide-radiation therapy. The series included consecutively patients treated at the University of Minnesota, Barrow Neurological Institute, Vidant Health, and Emory University Hospital. Survival results were stratified by methyl-guanine methyl transferase (MGMT) promoter methylation status RESULTS In total 48 patients were treated between 2019 and 2022 in this multi-institutional experience. The median days of hospitalization following the procedure was two (range: 1-15), with 72.5% of the GT implanted patients discharged home before postoperative day three. In terms of post-operative course, two patients suffered new-onset seizure post-surgery (4.0%). There was one (2%) 30-day mortality from intracranial hemorrhage secondary to heparinization for an ischemic limb. There were 13% readmission within 30 days, including hydrocephalus (n=2), urinary tract infection (n=1), deep venous thrombosis (n=1), failure to thrive (n=1), and infected wound (n=1). Of note, the patient with the infected wound had suffered a previous wound infection prior to the GT implantation and the pathogenic organism for both infections were the same (S. aureus). With a median follow-up of 250 days, twelve-month local control was 82%. Median overall survival (OS) from the time of diagnosis was 19 and 33 months for the MGMT unmethylated and methylated tumors, respectively. Select patients (n = 6) treated with adjuvant ketogenic diet in addition to GT and subsequent chemotherapy exhibited survival beyond expectation (&amp;gt; 14 mo, median survival not reached). CONCLUSION This multi-institutional experience supports further investigation of GT brachytherapy as a treatment for recurrent glioblastomas.

Etiology of new-onset seizures in adult patients of different age groups presenting to the emergency department in North India and their outcomes.

Seizures are one of the most common neurological emergencies encountered in both urban and rural India. There is only limited research work on the etiology of new-onset seizures in adult patients of different age groups presenting to the emergency department, especially from the Indian sub-continent. A new-onset seizure can be the first presentation of stroke, or it may be a symptom of brain infections, metabolic abnormality, brain tumor, systemic disease, or an early phase of epilepsy, which needs scrutiny and appropriate management. A dedicated study of the underlying etiology of new-onset seizures among different age groups and their incidence and prevalence can help in the prognostication and clinical management of these patients. This was a prospective observational cross-sectional study conducted in the Emergency Medical Out-patient Department and emergency medical ward of the Post-graduate Institute of medical education and research, Chandigarh. In our research, males out-numbered females. The most common seizure type recorded in our study was generalized tonic-clonic. In the younger age group between 13 and 35 years, infective etiologies were dominant. In the middle age group between 36 and 55 years, cerebrovascular accidents were the dominant etiology, followed by infective causes and metabolic causes. In the older age group above 55 years, the most dominating etiology found was cerebrovascular accident. Almost 72% had abnormal brain imaging. The most common abnormality found was ischemic infarcts. The second most common abnormality detected was a meningeal enhancement. A small percentage of patients had an intra-cranial bleed, and a very small percentage had a subarachnoid hemorrhage. In younger patients, infections such as tubercular and pyogenic meningitis and cerebral malaria are the most common causes of new-onset seizures, followed by malignancy and metabolic causes, in descending order. In the middle age group, stroke is the most common etiology, followed by central nervous system (CNS) infections and metabolic causes, in descending order. In elderly patients, stroke is the leading etiology for new-onset seizures. Physicians working in rural and remote areas routinely face challenges in managing patients with new-onset seizures. Knowledge of different etiologies in different age groups will equip them to make informed decisions regarding investigations and treatment of patients with new-onset seizures. It also encourages them to aggressively search for CNS infections, especially in younger patients.

PSAT183 Severe Hypocalcemia Precipitated by COVID-19 Infection in a Patient with Albright Hereditary Osteodystrophy and Pseudohypoparathyroidism

Abstract Background Hypocalcemia is a common endocrine manifestation of COVID 19 infection. We present a rare case of severe hypocalcemia in a patient with a history of Pseudohypoparathyroidism (PHP) type 1A after COVID-19 infection. PHP is a genetic disorder that presents with hypocalcemia, hyperphosphatemia, and secondary hyperparathyroidism due to marked target-organ unresponsiveness to parathyroid hormone (PTH). PHP type 1A also has a characteristic somatic phenotype known as Albright Hereditary Osteodystrophy (AHO), including short stature, brachydactyly, shortened metatarsals, and often reduced intelligence. Case Presentation This is a 53-year-old female with PHP type 1A, diabetes mellitus type 2, hypothyroidism, vitamin D deficiency, mild intellectual disability, and hypertension. She presented with new onset of seizures. She was also found to be positive for COVID-19 infection. On Review of systems, she had diarrhea but denied perioral or extremity paresthesia, palpitations, muscle spasms and bone pain. She reported compliance with calcitriol and calcium supplements at home. She had short stature, round face, short neck, obesity, brachydactyly (short digits of right 4th and 5th fingers) and shortened left 4th metacarpal. Labs revealed corrected serum calcium from baseline of 7.8 mg/dL to 4.4 mg/dL, ionized calcium 2.9 mg/dL, vitamin D 12.8ng/ml, PTH 142pg/ml and phosphorus 6.2mg/dl. EKG showed T wave inversions and prolonged QT interval. She did not have tetany, carpopedal spasm, paresthesia, Chvostek's sign, or Trousseau's sign. After receiving a total of 11 grams of elemental calcium, calcitriol, and ergocalciferol, her corrected calcium level improved to 7.4 mg/dL and EKG normalized. She was discharged home with calcium carbonate 1,250 mg twice a day and calcitriol 0.5 mcg daily. Discussion PHP type 1A is an autosomal dominant mutation of the stimulatory G protein alpha subunit (G alpha or GNAS) derived from the mother due to genomic imprinting which leads to PTH resistance in the kidneys and parathyroid glands. Our patient had a long-standing history of mild hypocalcemia with calcium and calcitriol supplements. Sudden worsening of hypocalcemia was likely related poor calcium and calcitriol absorption induced by COVID-19 infection. Patient with chronic hypocalcemia may not present with typical hypocalcemia signs or symptoms due to their tolerance. Conclusion Clinicians should be aware of the risk of developing severe hypocalcemia in patients who are calcium dependent or vitamin D deficient, malnourished during critical illness, or have COVID-19 infection which may decrease serum albumin and reduce total serum calcium level. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

PMON317 Rapid Progression of Adrenal insufficiency in Child with Autoimmune Polyendocrine Syndrome Type 1

Abstract Background Autoimmune Polyendocrine Syndrome type 1 (APS-1) is an autosomal recessive rare disease resulting from mutations in the autoimmune regulator gene (AIRE) on chromosome 21q22.3. APS-1 was first described as a syndrome in 1946 as a triad of mucocutaneous candidiasis, primary hypoparathyroidism, and adrenal insufficiency. With an AIRE gene mutation, the immune system will have a T-regulatory cell defect, which leads to a loss of immune tolerance. This will increase the risk of autoimmune disease in the body's organs such as the pancreas, thyroid, adrenal gland, liver, intestine, skin, and parathyroid gland. Clinical case A 6-year-old male was admitted to the pediatric floor for new-onset seizures and tetany. Initial laboratory assessment was concerning for severe hypocalcemia associated with low parathyroid hormone (PTH) and hyponatremia. Blood workup showed: low serum sodium 130 mmol/L (136-144), potassium 4.1 mmol/L(3.4-4.7), carbon dioxide 16 mmol/L; ionized calcium 0.67 mmol/L (1.19-1.41), phosphorus 7.4mg/dL (4.0-7.0), PTH 9.0pg/mL (12.0-88.0). Calcium replacement and calcitriol were initiated with a resolution of symptoms. Further investigations were done for the adrenal gland given his low sodium level. He underwent an ACTH stimulation test that he passed, with a cortisol baseline of 39mcg/dl with a peak of 48mcg/dl. ACTH level was high at 120pg/mL (5-27), and a repeat in 4 weeks was normal at 11pg/ml. His renin activity was elevated at 71ng/ml/hr (1.5-3.5). Renin activity was repeated and was elevated again at 34ng/ml/hr, prompting adrenal antibody testing, which revealed an elevated titer 1: 10 (&amp;lt;1: 10). Due to concern for APS-1, genetic testing was obtained and revealed a pathological variant of AIRE of c.892G&amp;gt;A and c.967_979del consistent with APS-1. He was started on 0.1mg Florinef and was later followed routinely at an outpatient endocrinology clinic. Fifteen months after diagnosis, he had a repeat ACTH stimulation test, and his baseline cortisol was 9.1mcg/dl with a peak at 60 minutes of 9.6mcg/dl (&amp;lt;18). ACTH level was elevated at 425pg/ml at this time. He was started on hydrocortisone replacement. Of note, investigation of siblings revealed a 10-month-old sister with abnormal AIRE mutation and a 17-month-old sister with unexplained death. Conclusion APS-1 can affect multiple organs, and cases can significantly vary in age and the number of affected organs at presentation. The patient presented with atypical presentation as he had hypocalcemia and hypoparathyroidism as an initial abnormality. Typically, adrenal insufficiency appears in the second decade of life but, the patient has it at the time of diagnosis, and it rapidly progresses. Family history plays an essential role in investigating autoimmune disease and warrants further assessment for autoimmune markers. While typically a disease that progresses insidiously into adulthood, cases like ours demonstrate rapid autoimmune disease progression. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Predictors of abnormal electroencephalogram and neuroimaging in children presenting to the emergency department with new-onset afebrile seizures

BackgroundElectroencephalogram and neuroimaging in pediatric patients with new-onset afebrile seizures are performed to detect any underlying pathological severe condition that may require emergent neuro-intervention and guide prognosis. This study aims to determine the predictors of abnormal EEG and neuroimaging in children presenting to the emergency department with new-onset afebrile seizures.MethodsThis single-center cross-sectional study was conducted at a tertiary care hospital in Karachi, Pakistan, from July 01, 2019, to June 30, 2021. All patients aged one month to 18 years who presented with new-onset afebrile seizures were included. Demographic and clinical data were recorded, including age, gender, seizure type, duration of seizure, associated signs and symptoms, and disposition. Multivariable regression analysis was applied to determine the predictors of abnormal EEG and CT scan or MRI findings.ResultsOut of 201 participants, most patients were in the infantile age group (41.3%), with an equal gender distribution. The most common type of seizure was generalized onset 152 (75.6%). EEG was performed on a total of 126 patients (62.7%) and out of these patients, 67 patients (53.1%) had abnormal findings. In a multivariable analysis, the age group of 5 to 10 years and seizure duration of more than 5 min were significantly associated with higher odds of abnormal EEG findings. In contrast, only the focal onset of seizure was significantly associated with higher odds of abnormal neuroimaging findings.ConclusionThe study emphasizes the need for a protocol regarding the performance of EEG and neuroimaging in children presenting to the ED with new-onset afebrile seizures that would aid emergency physicians in the direction of appropriate management, thus ensuring a better quality of patient care and outcomes.

Intraosseous meningioma, a rare presentation of a common brain tumor: illustrative case.

BACKGROUNDThe subset of extradural meningiomas arising from bone is called primary intraosseous meningioma. The present article outlines the clinical presentation, investigation, surgical management, and clinical outcomes of a challenging case of intraosseous meningioma.OBSERVATIONSA 27-year-old male presented with new-onset seizure of 3 years’ duration and growing painless hard swelling involving the left frontoparietal bone, the medial aspect of the left orbit, the nasal bone, and the temporal bone. Brain computed tomography showed a diffuse intradiploic space osseous lesion with a ground-glass appearance. The patient underwent bifrontal craniotomy and tumor excision. The histopathological sections showed the presence of classic whorls of meningothelial cells around reactive bony tissue suggestive of intraosseous meningioma. Postoperatively, the patient was neurologically intact, and he was discharged in a stable condition. The outcome of this case was satisfactory.LESSONSThe treatment of primary intraosseous meningioma is wide surgical excision together with the involved dura and any intradural tumor. The location of the tumor determines the approach and the amount of resection, keeping in mind preservation of function. The use of adjuvant therapy is reserved for cases of tumor recurrence or an atypical type.

Role of Electroencephalogram (EEG) and Magnetic Resonance Imaging (MRI) Findings in Early Recognition and Diagnosis of Neuronal Ceroid Lipofuscinosis Type 2 Disease.

Neuronal ceroid lipofuscinosis type 2 (CLN2) disease is a very rare neurodegenerative lysosomal storage disorder. Progression is rapid and irreversible, making early diagnosis crucial for timely treatment. A group of pediatric neurologists and neuroradiologists with expertise in CLN2 convened to discuss early electroencephalogram (EEG) and magnetic resonance imaging (MRI) findings in CLN2 diagnosis. Of 18 CLN2 cases, 16 (88.9%) had background slowing and 16 (88.9%) had epileptiform discharges on initial EEG. Seven of 17 (41.2%) patients who received intermittent low-frequency photic stimulation had a photoparoxysmal response. Initial MRIs showed subtle cerebellar (n = 14, 77.8%) or cerebral (n = 9, 50.0%) atrophy, white matter abnormalities (n = 11, 61.1%), and basal ganglia T2 hypointensity (n = 6, 33.3%), which became more apparent on follow-up MRI. The recognition of even subtle cerebellar atrophy and white matter signal changes in children aged 2-5 years who present with language delay, new-onset seizures, and an EEG with epileptiform discharges and background slowing should prompt investigation for CLN2. Because these early signs are not unique to CLN2, genetic testing is essential early in the diagnostic journey.

Causes of New-Onset Seizures and Their Treatment in Children With Non-CNS Malignancies: A Retrospective Study in a Tertiary Care Center

BackgroundSeizures occur in up to 13% of children with non-central nervous system (CNS) malignancies, but little is known about their causes and optimal diagnostic and therapeutic approaches. Here we sought to determine etiologies and clinical trajectories of new-onset seizures in this patient population. MethodsA retrospective chart review over a 10-year period was conducted at the American University of Beirut Medical Center to identify children with non-CNS malignancies and at least one new-onset seizure. Data were collected on the underlying malignancy, seizure etiology, clinical course, treatments, electroencephalograms, and brain imaging. ResultsNew-onset seizures occurred in 56 children (2-year median follow-up), most commonly in the context of acute lymphoblastic leukemia, lymphomas, and sarcomas. In 19 children, the first seizure consisted of status epilepticus. The most common etiologies were cerebrovascular accidents, posterior reversible encephalopathy syndrome, and metastasis. Forty-nine patients received anti-seizure medications (ASMs). Withdrawal of ASMs was successful in 19 children with normal initial or follow-up brain imaging but failed in three patients with persistent brain lesions. The remaining children, all of whom except two had structural brain abnormalities, received chronic ASMs and remained seizure free for a median period of 2 years at the last follow-up in survivors. ConclusionsNot only are seizures in children with non-CNS cancers often indicative of a serious brain insult, but they can also be challenging in the form of status epilepticus. An urgent diagnostic evaluation is therefore needed to expedite treatment, which should be tailored to the chronicity of the underlying cause.

Clinicoepidemiological profile and outcome among children with acute encephalitis syndrome from Central India

Background: Acute encephalitis syndrome (AES) is defined as the acute onset of fever and a change in mental status and/or new onset of seizures (excluding simple febrile seizures) in a person of any age at any time of the year. Aims and Objectives: The objectives of the study were as follows: (a) To study clinicoepidemiological profile and outcome among children with AES admitted at M.Y.H. Hospital and MGM Medical College, Indore, M.P., and (b) to study prognostic factors associated with bad outcomes. Materials and Methods: This prospective observational study was conducted over 12 months (July 2019–August 2020) at the pediatric department of our hospital. Inclusion criteria: Inpatient children aged 6 months–14 years meeting the case definitions of acute encephalitis syndrome. Exclusion criteria: Simple febrile seizures. Results: Out of 50 AES cases, majority were between 1 and 5 years of age (40%). There were more males 31 (62%) than 19 (38%) females. Most of the cases were reported during the monsoon period 29 (58%) followed by post-monsoon 13 (26%) and pre-monsoon 8 (16%). Out of 50 cases, all had fever and altered sensorium, 37 (74%) had convulsions, 14 (28%) had vomiting, and 9 (18%) had headache. Out of 50 cases, 42 (84%) had viral etiology including 7 (14%) of dengue encephalitis and 1 (2%) of case of human immunodeficiency virus encephalitis. Only 5 (10%) cases had bacterial etiology. Those who needed inotropes and mechanical ventilation showed significant mortality. Conclusion: The peak of AES cases occurred during the monsoon period. A higher proportion of such cases had viral etiology on cerebrospinal fluid analysis. Use of inotropes and mechanical ventilation was identified to be associated with significant mortality.

Adenovirus causing acute encephalitis in an immunocompetent elderly female - The first case reported in literature

Encephalitis is a leading cause of morbidity and mortality in elderly patients. Prognostic factors independently associated with an adverse clinical outcome are age ≥65, fever, Glasgow Coma Scale score &lt;13, and seizures. The most common viruses causing encephalitis are herpes simplex virus, varicella-zoster, human immunodeficiency virus, Epstein–Bar virus, cytomegalovirus, Japanese encephalitis, and arboviruses. We hereby present a case of a 55-year-old female who presented with a new-onset seizure, fever, and altered behavior. She was clinically diagnosed with encephalitis. Her diagnostic lumbar puncture was suggestive of lymphocytic pleocytosis, red blood cells, and raised protein with normal glucose. She had normal magnetic resonance imaging of the brain; however, cerebrospinal fluid demonstrated the presence of adenovirus DNA by real-time polymerase chain reaction. She was managed conservatively; however, the course was complicated by drug-induced acute kidney injury. Our patient had a favorable outcome. To the best of our knowledge, this is the first case of adenovirus encephalitis in an elderly immunocompetent female. Adenovirus is a rare cause of encephalitis in adults; however, high index of suspicion and timely management with available antivirals can lead to successful outcomes.

Who Is at Risk? A Critical Case of Japanese Encephalitis.

Japanese encephalitis (JE) is critical epidemic encephalitis caused by the JE virus (JEV) in Southeast Asia. The World Health Organization defined "acute encephalitis syndrome" (AES) as an acute onset of fever with a change of mental status and/or new-onset seizure, mainly for the surveillance of JE. The key clues for the diagnosis include the patient age group of unvaccinated era or waning vaccine-induced immunity and the history of possible mosquito bites in epidemic areas. We report a 47-year old man who is in an unvaccinated era with potential waning immunity. The patient presented with fever and altered mental status for 2 days. He was speechless and could not follow commands. The patient had gone camping in the countryside a week before the visit. At the emergency department, neck stiffness was noted. There was a leukocytosis with a left shift by blood cell count. The brain computed tomography was essentially normal. The cerebrospinal fluid (CSF) sample via lumbar puncture showed leukocytosis, a high protein level, and a low sugar level in comparison to serum tests. Further antibody test of CSF confirmed the diagnosis. Magnetic resonance imaging (MRI) of the brain revealed a high signal in the right thalamus and a mildly swollen left caudate nucleus 4 days after admission. He was extubated and finally discharged with partial dependency on activities of daily living. This case reminds us of the JE in AES. Emergency physicians should be aware of the suspicious case of unvaccinated age or waning immunity and possible mosquito bites in epidemic areas. The role of MRI on JE was also discussed in this article.

COVID-19-Induced Seizures: A Meta-Analysis of Case Series and Retrospective Cohorts.

The adverse events and complications of coronavirus disease 2019 (COVID-19) continue to challenge the medical profession despite the worldwide vaccination against the severe acute respiratory coronavirus 2 (SARS-CoV-2), the causative agent of COVID-19. Other than typical respiratory manifestations, COVID-19 also presents a wide range of neurological manifestations. This article underlines the pooled incidence of COVID-19-induced seizures in patients with epilepsy and without epilepsy. Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocols, we conducted a bibliographical search, and an initial search revealed 1,375 articles. In total, 21 articles were included in the final analysis by following the inclusion criteria. A total of 11,526 patients from 21 published articles that met the predetermined search criteria were included. The median age of the patients was 61.9 years, of whom 51.5% were males. A total of 255 patients presented with seizures as the first manifestation of COVID-19 with a prevalence of 2.2% (95% confidence interval = 0.05-0.24, p < 0.01) (I2 = 97%), of which 71 patients had previously been diagnosed with epilepsy. Among patients with epilepsy, 49 patients had seizures as an initial presentation of SARA-CoV-2 with an incidence of 72% (0.54-0.85, p = 0.1) (I2 = 34). Although the incidence of COVID-19-induced seizures is not high compared to other neurological manifestations, seizure incidence in epileptic patients with COVID-19 is remarkably high. New-onset seizures in any patient should be considered a presentation of COVID-19 in the absence of other causative factors.

Factors Associated With New-Onset Seizures Following Stereotactic Radiosurgery for Newly Diagnosed Brain Metastases

PurposeStereotactic radiosurgery (SRS) is a highly effective therapy for newly diagnosed brain metastases. Prophylactic antiepileptic drugs are no longer routinely used in current SRS practice, owing to a perceived low overall frequency of new-onset seizures and potential side effects of medications. It is nonetheless desirable to prevent unwanted side effects following SRS. Risk factors for new-onset seizures after SRS have not been well established. As such, we aimed to characterize variables associated with increased seizure risk. Methods and MaterialsPatients treated with SRS for newly diagnosed brain metastases between 2013 and 2016 were retrospectively reviewed at a single institution. Data on baseline demographics, radiation parameters, and clinical courses were collected. ResultsThe cohort consisted of 305 patients treated with SRS without prior seizure history. Median age and baseline Karnofsky Performance Scale score were 64 years (interquartile range, 55-70) and 80 (interquartile range, 80-90), respectively. Twenty-six (8.5%) patients developed new-onset seizures within 3 months of SRS. There was no association between new-onset seizures and median baseline Karnofsky Performance Scale score, prior resection, or prior whole brain radiation therapy. There were significant differences in the combined total irradiated volume (12.5 vs 3.7 cm3, P < .001), maximum single lesion volume (8.8 vs 2.8 cm3, P = .003), lesion diameter (3.2 vs 2.0 cm, P = .003), and number of lesions treated (3 vs 1, P = .018) between patients with and without new-onset seizures, respectively. On multivariate logistic regression, total irradiated volume (odds ratio, 1.09 for every 1-cm1 increase in total volume; confidence interval, 1.02-1.17; P = .016) and pre-SRS neurologic symptoms (odds ratio, 3.08; 95% confidence interval, 1.19-7.99; P = .020) were both significantly correlated with odds of seizures following SRS. ConclusionsOur data suggest that larger total treatment volume and the presence of focal neurologic deficits at presentation are associated with new-onset seizures within 3 months of SRS. High-risk patients undergoing SRS may benefit from counseling or prophylactic antiseizure therapy.