A Rare Presentation of Narcolepsy With Cataplexy After Vaccines in a Genetically Susceptible Elderly Woman: A Case Report.

Observing cataplexy episodes during an office visit is extremely rare as they are usually triggered by laughter or emotional stress. Narcolepsy usually occurs in the younger population. We report a case of a 65-year-old Caucasian female with a past medical history of obesity who developed excessive daytime sleepiness, fatigue, and sleep attacks five weeks after getting influenza and pneumococcal vaccines. The presentation of cataplexy was atypical. Several episodes of cataplexy were observed during the office visit without any emotional trigger. Further workup, including polysomnography (PSG), was positive for obstructive sleep apnea, controlled with continuous positive airway pressure (CPAP) use. Later, she had PSG with CPAP use, which optimally controlled obstructive sleep apnea, followed by multiple sleep latency tests (MSLT) with CPAP use. It was positive for narcolepsy with a mean sleep latency of 1.6 minutes with sleep onset rapid eye movement (REM) in five out of five naps. Her cerebrospinal fluid (CSF) hypocretin level was extremely low at 50 pg/ml, usually seen in narcolepsy with cataplexy. She was also positive for human leukocyte antigen (HLA) DBQ1*06:02. The diagnosis of narcolepsy with cataplexy was made, which improved with medications for narcolepsy.

Definitions of Drug-Resistant Epilepsy for Administrative Claims Data Research.

BACKGROUND AND OBJECTIVE: To assess the accuracy of definitions of drug-resistant epilepsy applied to administrative claims data. METHODS: We randomly sampled 450 patients from a tertiary health system with ≥1 epilepsy/convulsion encounter, ≥2 distinct antiseizure medications (ASMs) from 2014 to 2020, and ≥2 years of electronic medical records (EMR) data. We established a drug-resistant epilepsy diagnosis at a specific visit by reviewing EMR data and using a rubric based on the 2010 International League Against Epilepsy definition. We performed logistic regressions to assess clinically relevant predictors of drug-resistant epilepsy and to inform claims-based definitions. RESULTS: Of 450 patients reviewed, 150 were excluded for insufficient EMR data. Of the 300 patients included, 98 (33%) met criteria for current drug-resistant epilepsy. The strongest predictors of current drug-resistant epilepsy were drug-resistant epilepsy diagnosis code (odds ratio [OR] 16.9, 95% confidence interval [CI] 8.8-32.2), ≥2 ASMs in the prior 2 years (OR 13.0, 95% CI 5.1-33.3), ≥3 nongabapentinoid ASMs (OR 10.3, 95% CI 5.4-19.6), neurosurgery visit (OR 45.2, 95% CI 5.9-344.3), and epilepsy surgery (OR 30.7, 95% CI 7.1-133.3). We created claims-based drug-resistant epilepsy definitions (1) to maximize overall predictiveness (drug-resistant epilepsy diagnosis; sensitivity 0.86, specificity 0.74, area under the receiver operating characteristics curve [AUROC] 0.80), (2) to maximize sensitivity (drug-resistant epilepsy diagnosis or ≥3 ASMs; sensitivity 0.98, specificity 0.47, AUROC 0.72), and (3) to maximize specificity (drug-resistant epilepsy diagnosis and ≥3 nongabapentinoid ASMs; sensitivity 0.42, specificity 0.98, AUROC 0.70). DISCUSSION: Our findings provide validation for several claims-based definitions of drug-resistant epilepsy that can be applied to a variety of research questions.

Outcomes of seizures, status epilepticus, and EEG findings in critically ill patient with COVID-19.

OBJECTIVE: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has a myriad of neurological manifestations and its effects on the nervous system are increasingly recognized. Seizures and status epilepticus (SE) are reported in the novel coronavirus disease (COVID-19), both new onset and worsening of existing epilepsy; however, the exact prevalence is still unknown. The primary aim of this study was to correlate the presence of seizures, status epilepticus, and specific critical care EEG patterns with patient functional outcomes in those with COVID-19. METHODS: This is a retrospective, multicenter cohort of COVID-19-positive patients in Southeast Michigan who underwent electroencephalography (EEG) from March 12th through May 15th, 2020. All patients had confirmed nasopharyngeal PCR for COVID-19. EEG patterns were characterized per 2012 ACNS critical care EEG terminology. Clinical and demographic variables were collected by medical chart review. Outcomes were divided into recovered, recovered with disability, or deceased. RESULTS: Out of the total of 4100 patients hospitalized with COVID-19, 110 patients (2.68%) had EEG during their hospitalization; 64% were male, 67% were African American with mean age of 63 years (range 20-87). The majority (70%) had severe COVID-19, were intubated, or had multi-organ failure. The median length of hospitalization was 26.5 days (IQR = 15 to 44 days). During hospitalization, of the patients who had EEG, 21.8% had new-onset seizure including 7% with status epilepticus, majority (87.5%) with no prior epilepsy. Forty-nine (45%) patients died in the hospital, 46 (42%) recovered but maintained a disability and 15 (14%) recovered without a disability. The EEG findings associated with outcomes were background slowing/attenuation (recovered 60% vs recovered/disabled 96% vs died 96%, p < 0.001) and normal (recovered 27% vs recovered/disabled 0% vs died 1%, p < 0.001). However, these findings were no longer significant after adjusting for severity of COVID-19. CONCLUSION: In this large multicenter study from Southeast Michigan, one of the early COVID-19 epicenters in the US, none of the EEG findings were significantly correlated with outcomes in critically ill COVID-19 patients. Although seizures and status epilepticus could be encountered in COVID-19, the occurrence did not correlate with the patients' functional outcome.

Cauda Equina Syndrome in Neurosarcoidosis.

Neurosarcoidosis (NS) is a mimicker of many infectious, neoplastic, and inflammatory diseases. It most commonly involves the cranial nerves followed by meninges, ventricles, hypothalamic-pituitary axis, spinal cord, and brainstem/cerebellum. While NS myelopathy has been increasingly recognized, pathophysiological/prognostic and management principles in NS-mediated cauda equina (CE) and conus medullaris (CM) syndromes, which constitute a small and rare minority of this subset, remain elusive. We present the case of a 49 -year-old Hispanic man who developed a peripheral facial palsy and primary hypogonadism within a span of 12 months and eventually got diagnosed with NS after he presented with CE syndrome. We also performed an extensive literature review, with a discussion on the underlying pathophysiology and current management recommendations for NS-mediated CE/CM syndrome. CE/CM syndromes in a middle-aged man should prompt the consideration of NS as a possible differential diagnosis. While steroid responsive, the majority of NS-CE/CM patients are left with residual neurodeficits with quick relapses when steroids are tapered, making the case for early institution of immunosuppressive therapies.

Early Magnetic Resonance Imaging Decreases Hospital Length of Stay in Patients with Ischemic Stroke.

BACKGROUND: Imaging modalities are important part of stroke evaluation. Noncontrast head computed tomography (CT) is the initial imaging modality in acute stroke and although important to rule out acute hemorrhage and making a decision on thrombolytic treatment, ischemic changes may not be visible on CT for up to 24 hours. Magnetic resonance imaging (MRI) brain is an invaluable tool to confirm an ischemic stroke and facilitates stroke evaluation. Objective of this study was to investigate the correlation between time to MRI and length of hospital stay. METHODS: A total of 432 patients admitted to Hartford Hospital (Comprehensive Stroke Center) with a focal neurological deficit in the year 2014 and got a CT head and MRI brain were enrolled in the study. Data collection was done via stroke database and retrospective chart review. Patients with any hemorrhage or age <18 years were excluded from the study. Patients were categorized as having had an early (within 12 hours) or a late (more than 12 hours) MRI. We used chi-square and Wilcoxon ranked sum test to compare time from arrival to MRI and length of stay in the hospital. RESULTS: There was a statistically significant difference in hospital length of stay between patients who obtained MRI within 12 hours, as compared with patients who had MRI greater than 12 hours after admission, early MRI group 3 days (1.8, 4.9) versus 4 days (2.6, 7.0), P < .001. CONCLUSIONS: Our study suggests that brain MRI performed within 12 hours of admission facilitates stroke evaluation and decreases hospital length of stay. It provides evidence for cost effectiveness of MRI in ischemic stroke.

New-onset headaches secondary to spontaneous intracranial hypotension.

We describe the case of a 54-year-old man who presented with new-onset positional headaches and seizures, which were determined to be secondary to spontaneous intracranial hypotension due to a cervicothoracic spinal cerebrospinal fluid leak, and its associated complications.