This case report presents a 23-year-old male diagnosed with Charcot-Marie-Tooth (CMT) disease, who exhibited additional neurological symptoms suggestive of leukodystrophy. The patient experienced recurrent episodes of slurred speech, imbalance, and a recent tonic-clonic seizure, prompting admission. Neurological examination and imaging revealed bilateral white matter changes, raising suspicion of leukoencephalopathy. Further investigations confirmed a nonsense mutation c.64C>T (p.Arg22*) in the gap junction beta 1 (GJB1) gene. This case underscores the complexity of Charcot-Marie-Tooth disease type 1 (CMTX1) with atypical central nervous system (CNS) manifestations, highlighting the importance of comprehensive diagnostic evaluations and a multidisciplinary approach to management.

Listeria cerebritis is a rare yet serious central nervous system infection, which can present with leptomeningeal enhancement, abscess, and seizures. An adult patient with a history of metastatic melanoma presented with left-sided weakness, later identified as postictal Todd\'s paralysis due to focal motor seizures. Further diagnostic workup revealed a leptomeningeal abscess in the setting of listeria cerebritis. The patient\'s condition improved after treatment with a prolonged course of ampicillin, gentamicin, and linezolid over eight weeks. Leptomeningeal disease in patients with cancer history is often thought to be metastatic disease but infections, such as listeria, should be considered even if cerebrospinal fluid is bland. Treatment of listeria may need to be prolonged in patients who are immunocompromised.

Dandy-Walker malformation or syndrome is a rare congenital deformity in which the cerebellar vermis is hypoplastic and upwardly rotated, the fourth ventricle enlarged, and the posterior fossa cystically dilated. It represents the most common type of posterior fossa malformations that are usually diagnosed before the age of one year old. We present a seven-year-old boy with a history of neonatal hypotonia and delayed walking, who presented with speech and language difficulties. His physical examination and cognitive tests were unremarkable. The patient\'s brain magnetic resonance imaging showed a partial defect of the inferior part of the cerebellar vermis and communication between a normal-sized cisterna magna and the fourth ventricle. There were no other coexisting central nervous system or systemic anomalies. This isolated inferior vermian hypoplasia was compatible with an uncommon variant of the Dandy-Walker syndrome. The aim of this report is to provide insight into the importance of implementing a pediatrician-psychiatrist collaboration in the clinical decision-making process of such developmental delay cases. What makes the present case further interesting are the new-onset unprovoked seizures that developed and recurred in the setting of such isolated and less severe posterior fossa anomaly, raising both diagnostic and therapeutic challenges.

BACKGROUND: This study aimed to determine whether the administration of antiepileptic drugs (AED) alters the likelihood of detecting epileptiform abnormalities in electroencephalographies (EEG) performed early after a first epileptic seizure.
METHODS: We performed a retrospective, observational study including patients with a first seizure attended at our centre\'s emergency department between July 2014 and November 2019. We collected clinical data, as well as technical data on the acquisition and interpretation of the EEG performed within the first 72 hours after the seizure, and the factors related with seizure recurrence.
RESULTS: We recruited 155 patients with a mean (SD) age of 48.6 (22.5) years; 61.3% were men. Regarding seizure type, 51% presented tonic-clonic seizures of unknown onset and 12% presented focal to bilateral tonic-clonic seizures. Thirty-nine patients (25.2%) received AED treatment before the EEG was performed: 33 received a non-benzodiazepine AED and 6 received a benzodiazepine. Epileptiform abnormalities were observed in 29.7% of patients. Previous administration of AEDs was not significantly associated with the probability of detecting interictal epileptiform abnormalities (P = .25) or with the risk of recurrence within 6 months (P = .63).
CONCLUSIONS: Administration of AEDs before an early EEG following a first seizure does not decrease the likelihood of detecting epileptiform abnormalities. These findings suggest that starting AED treatment immediately in patients with a high risk of early recurrence does not imply a reduction in the diagnostic accuracy of the test.

Herpes simplex encephalitis is a rare disease presentation that is usually characterized by its temporal involvement and positive cerebrospinal fluid (CSF) polymerase chain reaction (PCR) for the herpes simplex virus (HSV). HSV PCR has a sensitivity of 96% and specificity of 99%. Even when the test is negative, if clinical suspicion is high, acyclovir therapy should be continued with a repeated PCR within a week. In this case, we report a 75-year-old female patient who presented with signs of hypertensive emergency with rapid deterioration to seizure-like activity on electroencephalogram (EEG) and signs of temporal encephalitis on magnetic resonance imaging (MRI). The patient did not respond to the initial regimen of antibiotics but did show significant clinical response to acyclovir though she had a negative CSF PCR for HSV ten days after the start of her neurological symptoms. In this case, we argue that alternative methods of diagnosis should be considered in cases of acute encephalitis. Our patient had negative PCR but her computerized tomography (CT), EEG, and MRI results pointed to temporal encephalitis caused by HSV.

The novel severe acute respiratory syndrome (SARS-CoV-2) virus has spread rapidly worldwide in the last several year. COVID-19 presentation ranges widely from asymptomatic to acute respiratory failure. Interestingly, although neurological manifestations of COVID-19 have often been described in the literature, only a few cases reports describe status epilepticus associated with COVID-19 patients. This is a case of a 52-year-old previously healthy woman who presented to the emergency department with fever, worsening cough, shortness of breath, and hypoxia. She was found to be COVID-19-positive. She developed a bilateral tonic-clonic seizure 16 days after her first symptoms appeared. Continuous video encephalogram (CEEG) showed a generalized periodic pattern with triphasic morphology. This finding is suggestive of non-convulsive status epilepticus which resolved after valproic acid loading as given. The patient is fully recovered at 6 months follow-up and seizure-free on levetiracetam 750 mg twice daily. This case demonstrates a new onset of bilateral tonic-clonic seizure followed by non-convulsive status epilepticus associated with COVID-19 infection. As the spectrum of COVID-19 neurological manifestation has yet to be established, healthcare providers should be cognizant of the possibility of non-convulsive status epilepticus in COVID-19 patients in order to provide timely workup and management.

UNASSIGNED: To identify the etiology and outcomes of new onset seizure in adult patients presenting to the Emergency Department (ED), to improve knowledge among healthcare providers regarding diagnosis and hence improve the outcomes.
UNASSIGNED: This was a descriptive retrospective study conducted at the Emergency Department of Aga Khan University Hospital, Karachi, Pakistan. Adult patients (18 years and above), presented to the ED from January 01, 2019 to June 30, 2020, with new onset seizure were included consecutively. Descriptive data on patient demographics, seizure types, investigations performed, final diagnosis and disposition were collected retrospectively from patient\'s file and electronic record. Etiologies of seizure were classified as structural (metabolic, trauma, infection), genetic or idiopathic. The immediate outcomes were reported as hospitalization or discharge from the ED.
UNASSIGNED: In total 198 patients with new onset seizure were included. Majority of the patients were found in middle age group i.e., 35 to 65 years (44.4%). Gender distribution showed slightly higher percentage of females (55.1%). Generalized tonic clonic seizures were found to be the most common type of seizures (74.2%), followed by generalized tonic (12.1%) and focal onset aware seizures (7.5%). Out of total patients no cause was identified in eight patients (4%), whereas in total confirmed causes of new onset seizures, structural lesions of brain were found to be the most common cause (89.6%%), including neurological (23.6%), infectious (4.2%), systemic (13%), metabolic (7%) and toxicological (4%) causes respectively.
UNASSIGNED: This study explores the various etiologies of new onset seizures in adult patients presenting to the ED. The study emphasizes the need for a local guideline for the investigation of new onset seizures in adult patients that would direct emergency physicians in respect of appropriate management, thus to ensure better quality of patient care and outcomes.

Fahr\'s syndrome is a rare, genetically dominant, inherited, neurological disorder characterized by abnormal deposits of calcium in the basal ganglia and the cerebral cortex. Symptoms include motor dysfunction, dementia, headache, spastic paralysis, abnormal ocular findings and seizures. Hypoparathyroidism is the most common endocrine disorder related to this syndrome, however, there are other metabolic, infectious and genetic causes. This is a case report of a Fahr\'s syndrome patient presenting a three-month history of self-limited partial epileptic seizures. His cranial CT had bilateral symmetrical calcifications of the basal ganglia, subcortical tissue and dentate nucleus whereas his laboratory findings were compatible with hypoparathyroidism.

UNASSIGNED: To study the aetiolgic spectrum of new-onset seizures in the peripartum period in south India.
UNASSIGNED: This is a retrospective analysis of case records of women with new-onset seizures in the peripartum period admitted between 2005 and 2018 (13 years).
UNASSIGNED: Of the 41 women (mean age, 26.20 years; range, 19-35 years) admitted, 20 patients (48.7%) had hypertensive disorders of pregnancy (HDP). Generalized tonic-clonic seizure (88%) was the common seizure type. The aetiologies of new-onset seizures were: 1) pregnancy-related aetiologies in 33 (80.5%) and 2) non-pregnancy-related aetiologies in eight (19.5%). Of the pregnancy-related aetiologies, posterior reversible encephalopathy syndrome (PRES) was the commonest cause in 24 patients (58.5%). Seizure cluster presentation was common in patients with PRES (p=0.0087). Of the eight women with non-pregnancy-related aetiologies, endemic central nervous system (CNS) infections accounted for three (7.3%; brain tuberculoma in one and neurocystocercosis in two) of the aetiology. All the women had Glasgow outcome scale-5 outcome.
UNASSIGNED: PRES was the common cause of new-onset seizures in peripartum period in this cohort. Endemic infections of CNS accounted for 7.3% of the total aetiological spectrum. This study suggests that the possibility of PRES should be considerd in woman with HDP and seizure cluster in peripartum period.

Septum pellucidum is a thin midline membrane that separates the anterior horns of the lateral ventricle. Agenesis of septum pellucidum (ASP) is considered a continuum of forebrain maldevelopment. Isolated ASP is a rare radiographic finding of unclear significance. We report a case of a 42-year-old male with ASP who presented with a new-onset seizure and eye closure sensitivity seen in the electroencephalogram. Magnetic resonance imaging of the brain confirmed the ASP. In the absence of data about the association between seizure and ASP, further studies are needed to determine its significance.