Uncovering an undisclosed diagnosis: a glucose-6-phosphate dehydrogenase deficiency diagnosis in a critically ill adult.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency affects over 400 million people worldwide. The most common variant of G6PD deficiency in the United States is the A-variant, which is present amongst African-Americans. Most people with this variant, however, do not experience severe hemolysis unless under extreme circumstances. Here, we present the case of a 44-year-old African-American male who under circumstances of multiple admissions for critical illness eventually presented with a masked diagnosis of G6PD deficiency.

An Immunologic Storm: A Case of Encephalitis and Thrombotic Thrombocytopenic Purpura With Underlying Likely Sjogren's Syndrome Induced by a COVID-19 Immune Response.

Introduction: There are rare cases of Sjogren's syndrome presenting with manifestations of encephalitis. There are also rare patients with Sjogren's presenting with acute thrombotic thrombocytopenic purpura (TTP). There are no cases of both occurring together as the only symptoms of the syndrome. During the COVID-19 pandemic, more cases of autoimmunity are being described given its robust immune response. It is important to keep a wide differential about these varying clinical presentations. Case Presentation: Our patient is a 19-year-old female with a history of menorrhagia, recent COVID-19 infection, and remote suicidal ideation. She presented with headaches, vomiting, and psychosis. Her labs found platelets of 12,000 and she was soon discovered to have TTP. She was found to have contrast enhancing lesions scattered in her left hemisphere on magnetic resonance imaging as well as seizures. Her workup was negative for infection, but labs revealed a positive antinuclear antibody, elevated anti-Ro antibody (anti-SSA) and anti-La antibody (anti-SSB), and elevated COVID-19 antibodies. She was treated with antiepileptics, pulse dose steroids for 5 days, plasmapheresis, and weekly rituximab for 4 weeks. She had significant clinical improvement. Conclusion: Sjogren's syndrome can have varying presentations including TTP with or without encephalitis as a presenting feature. Autoimmunity can also be triggered from COVID-19 infection.

Evaluation of the Safety of Rapid Administration of Undiluted High-Dose Intravenous Levetiracetam.

Background: Intravenous (IV) levetiracetam (LEV) is an antiseizure medication traditionally given as an intermittent infusion to mitigate potential adverse effects given its acidic formulation. The process of compounding may lead to delays in treating status epilepticus, which is why administration of undiluted doses is of interest. Prior studies have shown safety of IV doses from 1000 mg to 4500 mg; however, assessments of adverse side effects outside IV site reactions have not been studied. Methods: A retrospective analysis was completed with patients who received 1500 mg doses of undiluted IV LEV. We included patients ≥ 18 years old that received at least 1 dose of IV LEV 1500 mg from January 2018 to February 2021. Study end points included assessment of hemodynamic disturbance (bradycardia [HR less than 50 beats per minute] or hypotension [SBP less than 90 mmHg] within 1 hour or documented infusion reaction within 12 hours of LEV. Descriptive statistics were utilized. Results: A total 213 doses of 1500 mg of IV LEV were administered to 107 patients. Peripheral lines were used for 85.9% of doses. Approximately half of doses (57) were administered to patients on the general wards, with the remainder in the intensive care unit or emergency department. Two patients (1.9%) experienced bradycardia; however, 1 patient had pre-existing bradycardia. Three patients (3.8%) experienced hypotension; however, those patients were receiving vasopressors prior to the dose. There were no cases of infusion reaction. Conclusion: Undiluted, rapid administration of IV LEV 1500 mg was well tolerated and safe.

Recognizing Aphemia and How to Differentiate From Aphasia in the Era of Telemedicine.

BACKGROUND: Aphemia, or pure motor mutism, is a phenomenon that has been reported previously in the literature and typically is associated with small infarcts in the inferior dominant precentral gyrus, pars opercularis, or inferior perirolandic gyrus. Clinically, it is important to distinguish aphemia from aphasia syndromes. Telemedicine is becoming more prevalent and involving neurologists across the country. This is an important consideration when addressing aphemic patients as many mistakes can be made during a virtual exam clouding a patient's clinical picture. CASE PRESENTATION: Our patient is a 61-year-old female with a past medical history of hypertension, diabetes, and an old right frontoparietal stroke without any residual deficits. She presented after her family stated that she "quit speaking" for about seven hours. Initial neurological evaluation was done via telemedicine due to the COVID-19 pandemic and was pertinent for decreased consciousness, inability to answer either orientation question, a right facial droop, and aphasia. Later it was found that the patient exhibited a pure motor mutism rather than aphasia and had an MRI lesion in the left inferior precentral gyrus. CONCLUSION: Differentiating aphemia from aphasia is an important clinical skill for a neurologist to foster especially in the era of telemedicine. An intimate knowledge of the parts of a speech exam are vital in directing emergency staff during stroke evaluation. Additionally, distinguishing these clinical syndromes has implications with respect to prognosis and long-term rehabilitation.

Admission Neutrophil to Lymphocyte Ratio for Predicting Outcome in Subarachnoid Hemorrhage.

PURPOSE: We sought to evaluate the relationship between admission neutrophil-to-lymphocyte ratio (NLR) and functional outcome in aneurysmal subarachnoid hemorrhage (aSAH) patients. MATERIAL AND METHODS: Consecutive patients with aSAH were treated at two tertiary stroke centers during a five-year period. Functional outcome was defined as discharge modified Rankin score dichotomized at scores 0-2 (good) vs. 3-6 (poor). RESULTS: 474 aSAH patients were evaluated with a mean NLR 8.6 (SD 8.3). In multivariable logistic regression analysis, poor functional outcome was independently associated with higher NLR, older age, poorer clinical status on admission, prehospital statin use, and vasospasm. Increasing NLR analyzed as a continuous variable was independently associated with higher odds of poor functional outcome (OR 1.03, 95%CI 1.00-1.07, p=0.05) after adjustment for potential confounders. When dichotomized using ROC curve analysis, a threshold NLR value of greater than 6.48 was independently associated with higher odds of poor functional outcome (OR 1.71, 95%CI 1.07-2.74, p=0.03) after adjustment for potential confounders. CONCLUSIONS: Higher admission NLR is an independent predictor for poor functional outcome at discharge in aSAH patients. The evaluation of anti-inflammatory targets in the future may allow for improved functional outcome after aSAH.

Review of the Mobile Stroke Unit Experience Worldwide.

BACKGROUND: The treatment of stroke is dependent on a narrow therapeutic time window that requires interventions to be emergently pursued. Despite recent "FAST" initiatives that have underscored "time is brain," many patients still fail to present within the narrow time window to receive maximum treatment benefit from advanced stroke therapies, including recombinant tissue plasminogen activator (tPA) and mechanical thrombectomy. The convergence of emergency medical services, telemedicine, and mobile technology, including transportable computed tomography scanners, has presented a unique opportunity to advance patient stroke care in the prehospital field by shortening time to hyperacute stroke treatment with a mobile stroke unit (MSU). SUMMARY: In this review, we provide a look at the evolution of the MSU into its current status as well as future directions. Our summary statement includes historical and implementation information, economic cost, and published clinical outcome and time metrics, including the utilization rate of thrombolysis. KEY MESSAGES: Initially hypothesized in 2003, the first MSUs were launched in Germany and adopted worldwide in acute, prehospital stroke management. These specialized ambulances have made the diagnosis and treatment of many neurological emergencies, in addition to ischemic and hemorrhagic stroke, possible at the emergency site. Providing treatment as early as possible, including within the prehospital phase of stroke management, improves patient outcomes. As MSUs continue to collect data and improve their methods, shortened time metrics are expected, resulting in more patients who will benefit from faster treatment of their acute neurological emergencies in the prehospital field.