Objective: Cerebral encephaloceles are being recognized as an etiology of drug-resistant epilepsy (DRE). Neuroimaging suggests a link between idiopathic intracranial hypertension (IIH), dural sinus stenosis (DSS), and encephaloceles. One hypothesis is that pulsatile CSF forces, secondary to elevated intracranial pressure, contribute to forming prominent arachnoid villi, which create pockets of CSF and develop encephaloceles. The impact of DSS and IIH management with stent placement for seizure control in patients with DRE and encephaloceles remains poorly understood. In this study, the authors explored the clinical outcomes in this patient population. Methods: This single-center case series retrospectively analyzed seizure frequency in 12 patients with DRE due to encephaloceles associated with IIH who underwent cerebral venous sinus stent placement between 2018 and 2024 at the University of Kansas Medical Center. Seizure burden was assessed after stent placement. Results: Twelve patients with encephaloceles and DRE met the inclusion criteria. The mean age was 49 years, and 9 patients were female. The mean BMI was 36.7 kg/m2. The mean age of seizure onset was 42.1 years. All patients had signs of IIH on MRI of the brain: empty sella (n = 8), transverse sinus stenosis (n = 12), and enlarged arachnoid granulations (n = 5). Seven patients underwent lumbar puncture with a mean opening pressure of 27.25 cm H2O. All patients had unilateral temporal lobe epilepsy and encephaloceles involving a sphenoid wing. One patient (8.3%) had an additional lesion in the basal temporal lobe, and another patient (8.3%) had bilateral cerebellar encephaloceles. The mean pre-stent venous pressure gradient was 14.25 mm Hg, and the mean post-stent gradient was 1.25 mm Hg; 50% of patients had a post-stent pressure gradient of 0 mm Hg. Following venous sinus stent insertion and additional surgical interventions, the mean seizure frequency decreased to 0.9 seizures per month. Eleven patients (91.7%) experienced > 50% reduction in seizure frequency postprocedure. Conclusions: The impact of endovascular stent placement for DSS and IIH on seizure burden was investigated. Seizure frequency reduction was observed in all patients. This study demonstrates a prominent role of endovascular stent insertion in patients with encephaloceles and DRE that warrants further exploration.

Objective: To characterize clinical and electrographic features of patients with beta-lactam antibiotic-induced neurotoxicity in presence of and without renal impairment (RI). Background: EEG findings like periodic discharges, triphasic waves, or status epilepticus have been associated with beta-lactam neurotoxicity in critically ill patients with renal impairment. Design/Methods: Medical records of patients undergoing EEG due to encephalopathy while receiving cefepime, cefazoline and meropenem at the KUMC from 2009–2018 were reviewed. Demographic data, creatinine levels, clinical information, electrographic findings, and AEDs used were obtained. Exclusion criteria included unknown antibiotic start date, no clinical changes after antibiotic onset, worsening unrelated to antibiotics, or mental improvement on antibiotics. Patients were divided into with and without RI. Descriptive statistical methods and Chi-square/Fisher's exact tests were applied. Results: 196 patients were identified. After applying our exclusion criteria, 39 patients were included, of which 14 (36%) were males and 25 (64%) were females. Thirty-three patients (85%) were on cefepime. Further analysis was focused on cefepime only since the number of patients on other antibiotics was nonsignificant. Symptoms reported were altered mental status (64%) [43% had RI], myoclonus (15%) [100% had RI] and seizures (9%) [67% had RI]. A tendency to have myoclonus was seen in patients with RI (p=0.049). EEG findings were generalized slowing (91%), triphasic waves (56%), GPDs (42%), and LPDs (12%). Six patients were found with NCSE, of which 4 (67%) had RI (p=0.490). Twenty-four (73%) patients symptomatically improved after stopping cefepime. Improvements in EEG were seen in 14 out of 22 patients who had repeat EEGs or were followed with EEG monitoring. Conclusions: Seizures were infrequently seen in patients with beta-lactam neurotoxicity. Removal of offending agents can improve clinical outcomes and prognosis in cefepime induced neurotoxicity. Disclosure: Dr. Ortiz Guerrero has nothing to disclose. Dr. Shah has nothing to disclose. Dr. Uysal has nothing to disclose.

OBJECTIVE:Stereoelectroencephalography (sEEG) is an intracranial encephalography method of expanding use. The need for increased epilepsy surgery access has led to the consideration of sEEG adoption by new or expanding surgical epilepsy programs. Data regarding safety and efficacy are uncommon outside of high-volume, well-established centers, which may be less applicable to newer or low-volume centers. The objective of this study was to add to the sEEG outcomes in the literature from the perspective of a rapidly expanding center.METHODS:A retrospective chart review of consecutive sEEG cases from January 2016 to December 2019 was performed. Data extraction included demographic data, surgical data, and outcome data, which pertinently examined surgical method, progression to therapeutic procedure, clinically significant adverse events, and Engel outcomes.RESULTS:One hundred and fifty-two sEEG procedures were performed on 131 patients. Procedures averaged 10.5 electrodes for a total of 1603 electrodes. The majority (84%) of patients progressed to a therapeutic procedure. Six clinically significant complications occurred: three retained electrodes, two hemorrhages, and one failure to complete investigation. Only one complication resulted in a permanent deficit. Engel 1 outcome was achieved in 63.3% of patients reaching one-year follow-up after a curative procedure.SIGNIFICANCE:New or expanding epilepsy surgery centers can appropriately consider the use of sEEG. The complication rate is low and the majority of patients progress to therapeutic surgery. Procedural safety, progression to therapeutic intervention, and Engel outcomes are comparable to cohorts from long-established epilepsy surgery programs.

Objective: To acquaint neurologists with underrecognized SESA syndrome described as an uncommon variant of non-convulsive status epilepticus in patient with chronic alcohol abuse. Background: Neurologists are aware of the diversity of neurological syndromes associated with alcoholism. Traditionally, alcohol withdrawal syndrome (AWS), delirium tremens, hepatic encephalopathy, alcoholic hallucinosis, and Wernicke encephalopathy or Korsakoff psychosis are best known. Seizures have best been known to occur in association with alcohol withdrawal syndrome. However often there are seizures and non-convulsive status epilepticus that can occur de novo in patients with chronic alcohol abuse that does not fit the pattern of a withdrawal syndrome. Design/Methods: A 49-year-old female with h/o alcohol abuse and with no previous h/o seizures presented to the hospital with altered mental status, aphasia and right sided extremity and facial weakness. Patient was noted to have a GTC seizure during the initial evaluation. MRI brain was negative for stroke but revealed signal changes in the left hippocampus and thalamus. CSF studies did not reveal any infections. EEG revealed continuous 1 – 1.5 Hz lateralized left posterior quadrant periodic discharges (LPDs) often with polyspike components raising suspicion for ictal interictal continuum. Patient did not have any convulsive seizures in the hospital and was managed with AEDs with slow improvement in mental status over the next 10 days. Results: Our patient had SESA syndrome with typical syndromic association of recent alcohol intake, de novo ictal – interictral continuum / NCSE and prolonged multifactorial confusional state with focal findings. Conclusions: The SESA syndrome appears as a distinct neurological disorder in which the encephalopathy occurs in the context of focal motor or GTCSs but often not proximate to alcohol cessation. Underrecognized SESA may present as NCSE with focal clinical, EEG and imaging abnormalities, warranting expedited diagnosis and antiseizure treatment to minimize morbidity. Disclosure: Dr. Diawara has nothing to disclose. Dr. Shah has nothing to disclose.

Psychogenic Nonepileptic Seizures (PNES) are defined as typical seizure-like activities or behaviors without cortical epileptiform discharges. The diagnosis of PNES remains a challenge and continuous video electroencephalography (cEEG) remains the gold standard to differentiate between epileptic seizures and PNES. Despite advances made in our understanding of PNES, diagnosing and treating this well-known entity remains a challenge. 25% of patients with seizures will be misdiagnosed as having PNES or epileptic seizures even by certified Neurologists. Do patient characteristics and demographics increase our accuracy to diagnose PNES? We present a case of a mother and her son, both diagnosed with PNES by cEEG monitoring in our epilepsy monitoring unit. While familial prevalence of conversion disorders is reported in the literature, this will be the first case report describing the incidence of PNES in two members of the same family. Do psychogenic epilepsies run in families? Will the diagnosis of PNES in one family member increase the probability of PNES in their relatives with seizures? Larger patient pools need to be studied to draw a definite conclusion regarding our observation.

Background: Simulation-based learning has improved medical knowledge, proficiency and performance. The treatment window for acute stroke is limited, making preparedness exceedingly critical. We aimed to assess improvement in performance in first responders to ‘stroke codes’, namely junior Neurology and Emergency Medicine (EM) residents. EM residents were included because EM physicians often make tPA decisions at peripheral and non-academic hospitals. Methods: 14 Neurology and 10 EM residents participated at Upstate Medical University, Syracuse, New York. The simulation consisted of senior Neurology residents playing the roles of stroke patient, CT technician and radiologist, nurse, pharmacist and the stroke fellow on-call. The simulation included the time from patient arrival to acute stroke treatment. Each junior Neurology and EM resident completed this simulation, while the rest of the group observed. Cases included stroke mimics, strokes with large vessel occlusions, dissections, cases ineligible for acute intervention, intracerebral and subarachnoid hemorrhages, basilar thrombosis, wake-up strokes, seizure at onset and dissections. Participants reviewed non-contrast CT head, EKG and CT angiography of the head and neck to make decisions about tPA administration and endovascular interventions. This was followed by a debriefing session by the attending and chief residents. Participants filled a pre-and post-simulation graded questionnaire on medical knowledge and confidence (range 0-25, with higher scores reflecting higher performance). Results: The mean score was 18 (range 14-23) pre-simulation and 22 (range 18-25) post-simulation for Neurology residents and 14 (range 8-24) pre-simulation and 18 (range 10-23) post-simulation for EM residents. For all residents, pre-and post-median score was 17 and 19 (Wilcoxon signed-rank test p<0.0001). Conclusion: Because running an effective stroke code requires coordination and rapid analysis, stroke simulation is an effective way of improving knowledge and confidence among residents and should be integrated into the Neurology and EM residency curriculum.

Objective: Pourfour du Petit syndrome (PdP) is an extraordinarily unusual clinical condition produced by oculosympathetic hyperactivity. It is characterized by the unilateral appearance of mydriasis, lid retraction and exophthalmos. It is named after a French anatomist and ophthalmologist who was also the first one to identify clinical symptoms of what is now known as Horner’s Syndrome Background: NA Design/Methods: NA Results: Patient was a 70 year old female who presented to hospital after being found unresponsive. Neurologic exam was significant for obtunded patient with bilateral ptosis, unreactive pupils with intact brain stem reflexes and withdrawing to painful stimulus. MRI brain revealed acute infarcts in bilateral thalami with extension to the cerebral peduncles and central midbrain along with right cerebellum and right occipital lobe. During hospital stay, she was noticed developing symptoms of sympathetic storming. This was particularly vivid on physical stimulation-the patient had episodes of dilatation of bilateral pupils along with hyperhidrosis and agitation with upper extremity movements. These episodes were controlled with addition of clonidine with good outcome. Conclusions: PdP syndrome has previously described in injuries to cervical sympathetic chain and brachial plexus, post-traumatic syringomyelia, aortic malformation, thoracic, esophageal and lung carcinoma and maxillofacial surgery. We had interesting dilemma where she had bilateral complete third nerve palsy from a basilar artery stroke affecting the midbrain. She was noted to periods of sympathetic hyperactivity and this seemed to be extremely pronounced especially as the parasympathetic activity was lost. Our hypothesis is that the occurrence of periods of sympathetic over activity associated with bilateral mydriasis and hyperhidrosis is similar to picture observed with PdP syndrome albiet now being caused by a central lesion. Neurologists should be aware about PdP syndrome which is a reverse Horner’s Syndrome and its etiologies to help localize the lesion. Disclosure: Dr. Nurmukhamedov has nothing to disclose. Dr. Rakhesh has nothing to disclose. Dr. Shah has nothing to disclose. Dr. Culebras has nothing to disclose.

Background: The available information on postictal semiology and behavior in patients with psychogenic nonepileptic seizure (PNES) is limited. In this study, we explore the differences in postictal semiology and behavior between patients with epileptic seizure (ES) and PNES and focus on clinical features that may be helpful in differentiating these two conditions. Methods: In this retrospective study, video-electroencephalograph (video-EEG) of 144 seizures from 64 patients with PNES and 66 seizures from 42 patients with ES were reviewed Three novel postictal behaviors were compared between the two groups: a) abrupt, brief, and rapid blinking or shaking of the head as if regaining sensorium or "coming out" of the ictal event; b) looking around the room with a scanning and uncertain look; c) posing a question of "what happened?" or a similar question to the others present in the room. In addition, differences in several other postictal characteristics were studied. Results: At least one of the three specific postictal behaviors was seen in 20.1% of patients with PNES but none of the patients with ES resulting in 100% specificity and 36% sensitivity. While mean ictal duration was significantly longer in patients with PNES (175.3 +/- 168 s) compared to ES (105.4 +/- 102.9 s), the interval between the end of ictal episode and first correct verbal response (74.5 +/- 1262 s versus 139.4 +/- 185.4 s), or first followed command (84.1 +/- 133.8 s versus 141.1 +/- 192.1 s) were shorter. In the group with PNES, 59.7% of patients demonstrated a whispering or altered voice during the first correct verbal response versus 21.2% of patients in the group with ES. The first followed command was slow or erroneous in 49.3% of the patients with PNES and in 19.7% of the patients with ES. Conclusion: In this study, we found significant differences between postictal semiology and behavior of patients with PNES and ES that can be helpful tools in distinguishing between these two conditions. Specifically, three novel postictal behaviors were studied and were found to have a high specificity, but low sensitivity, in distinguishing PNES from ES. (C) 2018 Elsevier Inc. All rights reserved.

Objective: To demonstrate use of root cause analysis to improve patient care in the outpatient neurology setting. Background: Root cause analysis (RCA) is a problem-solving method that identifies multiple and intersecting factors underlying problems in a complicated system such as health care. The RCA goal is to ascertain where and how a problem occurred. Determining the cause(s) of error allows system modifications to prevent future errors. Extending RCA to outpatient neurology is a novel use of this tool. Design/Methods: Presented is the case of an 80-year-old woman with a history of left-sided breast cancer with mastectomy in 1998, and new complaints of left arm weakness and paresthesias, slowly progressive since November 2014. In August 2015 the patient presented for a 3 rd opinion to the Neurology Senior Ambulatory Resident (SAR) clinic, where senior residents see interesting outpatients and urgent referrals under supervision. Metastatic disease was suspected. Cervical-spine and brachial plexus MRIs and repeat EMG were ordered. However, multiple successive delays occurred. In February 2016, biopsy showed recurrent breast cancer involving the upper and middle trunks of the brachial plexus. Results: An RCA to identify the causes of this 6-month diagnostic delay occurred within the residency training Quality and Patient Safety course. The RCA revealed delays in follow-up appointment scheduling, duplicative MRIs and EMG, poor communication between Radiology and Neurology leading to ordering errors with delays in correction, and delay in obtaining the biopsy once it was requested. Based on these root causes, our department developed procedures and policies to prevent similar problems. Interventions to date include designating a nurse to monitor test completion in critical cases and generating a SAR patient list to track high-acuity patient flow. Conclusions: RCA is a powerful tool in quality improvement endeavors related to patient safety in the clinic. Disclosure: Dr. Shah has nothing to disclose. Dr. Cherukuri has nothing to disclose. Dr. Izadyar has nothing to disclose. Dr. Bradshaw has received research support from Cytokinetics. Dr. Sanders has nothing to disclose.