A Missed Case of Area Postrema Syndrome Presenting with Neuromyelitis Optica Spectrum Disorder.

BACKGROUND Neuromyelitis optica spectrum disorder (NMOSD), which is also known as Devic disease, is a chronic disorder of the brain and spinal cord that includes inflammation of the optic nerve and spinal cord. Area postrema syndrome (APS) is due to involvement of the bulbar emetic reflex center, and has previously been described in NMOSD. Patients with APS may present with nausea, vomiting, or hiccups. This report is of a 33-year-old Asian American woman with history of APS who presented with NMOSD. CASE REPORT A 33-year-old Southeast Asian woman, 2 months postpartum, presented with fever, hypersomnolence, altered mental status, and difficulty ambulating. Neurological examination revealed a lethargic woman with poor attention span, broad-based gait ataxia, and positive Romberg's sign. Laboratory work-up showed sodium 123 milliequivalent/L (mEq/L). Brain magnetic resonance imaging (MRI) with contrast revealed bilateral, non-enhancing, patchy fluid-attenuated inversion recovery (FLAIR) hyperintensities in the anteroinferomedial thalamus extending to the mammillary bodies. Additional history revealed hospitalization for intractable nausea, vomiting, and hiccups 2 years ago. NMOSD was confirmed with positive AQP-4 antibody, prompting treatment with intravenous (i.v.) methylprednisolone, followed by plasmapheresis. Repeat brain MRI showed mild improvement of bilateral thalamic FLAIR hyperintensities and no clinical recurrence was reported with Rituximab treatment. CONCLUSIONS This case highlights the importance of the diagnostic diligence required for NMOSD diagnosis. Multiple etiologies can mimic the clinical presentation of acute diencephalic syndrome; thus, a broad differential needs to be considered. This report presents the diagnostic work-up and management of a patient with a complex neurological condition that was diagnosed as NMOSD.

Clinically Isolated Brainstem Progressive Multifocal Leukoencephalopathy: Diagnostic Challenges.

BACKGROUND Acute brainstem syndrome (ABS), as the initial manifestation of progressive multifocal leukoencephalopathy (PML), is rarely reported. Appropriate history and neurodiagnostic testing are essential to encompass the extended spectrum of clinical and radiological differentials of ABS. CASE REPORT A 47-year-old woman presented to the emergency department with slurred speech, dizziness, right-sided facial droop, and numbness. Brain magnetic resonance imaging (MRI) revealed non-enhancing hyperintensities in the right lateral pons and brachium pontis, eventually extending to the bilateral middle cerebellar peduncle, pons, left>right cerebellar hemisphere, right thalamocapsular region, and midbrain region. Lumbar puncture revealed 3 cerebrospinal fluid-specific oligoclonal bands. Initial diagnosis of multiple sclerosis led to high-dose intravenous steroid treatment. The patient continued to deteriorate, leading to multiple emergency department visits and hospital admissions. Additional history revealing previously diagnosed, treatment-naive HIV prompted a repeat lumbar puncture. Cerebrospinal fluid polymerase chain reaction (PCR) for JC polyomavirus (JCV) was positive, leading to the diagnosis of clinically isolated brainstem PML. Unfortunately, the patient developed pneumonia and hypoxic respiratory failure, which ultimately led to her death. CONCLUSIONS This case highlights the need for considering isolated brainstem PML, as a diagnostic possibility, in patients presenting with acute-subacute brainstem symptoms and compatible neuroimaging findings. Clinicians need to be aware of varying PML presentations with brainstem or diencephalic variants, as well as monofocal lesions. The prognosis for PML has improved somewhat, secondary to immune reconstitution by highly active antiretroviral therapy, risk stratification in drug-induced PML, and other emerging treatments, such as pembrolizumab.

Diagnostic and Therapeutic Challenges of Cerebral Venous Thrombosis in SARS-CoV-2 Infection: A Case Report and Review of Literature.

Headache, a common prodromal symptom of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, can also be a manifestation of cerebral venous thrombosis (CVT), secondary to COVID-19. CVT management continues to evolve, with direct oral anticoagulants (DOACs) emerging as an alternative to warfarin. A 44-year-old Asian female, with no past medical history, presented to the emergency room (ER) with complaints of nonproductive cough and left-sided headache. She denied a history of COVID-19 vaccination, and SARS-CoV-2 testing (with reverse transcriptase-polymerase chain reaction) was positive. Non-contrast computed tomography (CT) of the head revealed left transverse sinus hyperdensity, consistent with dense vein sign, and magnetic resonance venography (MRV) confirmed the presence of thrombus. The initial treatment included subcutaneous enoxaparin with headache resolution, and she was discharged on apixaban. Five weeks later, a non-contrast head CT showed resolution of the dense vein sign and recanalisation of left transverse sinus was seen on MRV. This report has highlighted the need for increased awareness of coagulopathy and thrombotic events, including cerebral venous thrombosis, in patients infected with SARS-CoV-2. Unremitting headache, in context of SARS-CoV-2 infection, should be evaluated with appropriate neurovascular imaging. Controlled studies are required to compare the safety and efficacy of DOACs with warfarin for management of cerebral venous thrombosis.

New Horizons for Diagnostic Pitfalls of Cerebral Venous Thrombosis: Clinical Utility of a Newly Developed Cerebral Venous Thrombosis Diagnostic Score: A Case Report and Literature Review.

BACKGROUND Diagnosing cerebral venous thrombosis (CVT) poses significant challenges owing to a nonspecific clinical presentation, poorly correlated laboratory biomarkers, and low sensitivity of non-contrast head computed tomography (CT). We describe a case of missed CVT diagnosis, due to low clinical suspicion and nonrecognition of anemia as a prothrombotic factor, especially during an ulcerative colitis (UC) flare. A recently proposed CVT clinical probability score can guide clinicians in pursuing further neurovascular imaging. CASE REPORT A 35-year-old man, with treatment-naive UC, presented to the Emergency Department (ED) with new-onset diffuse headache, 4 weeks of bloody diarrhea, and weight loss. Initial ED laboratory studies revealed severe anemia and unremarkable non-contrast head CT. Two days later, the patient returned to the ED for worsening headache. Non-contrast head CT revealed a left temporal hypodensity. This was later confirmed as acute ischemia on magnetic resonance imaging (MRI). MR venogram revealed thrombosis of the left transverse and sigmoid sinuses, leading to initiation of therapeutic subcutaneous anticoagulation. Repeat MRI, secondary to worsening headache, revealed the development of petechial hemorrhages within the core of venous ischemia in the left temporal lobe. Therapeutic anticoagulation, along with symptomatic management of UC, led to clinical stabilization. CONCLUSIONS CVT should be suspected in patients with UC, especially in the context of anemia, presenting with new-onset or worsening headaches. Recognizing anemia as a thrombogenic factor is crucial. Diagnosis of CVT is challenging due to non-focal symptoms and poorly correlating diagnostic tests. We endorse implementing the CVT clinical probability score into AHA/ASA CVT guidelines to enhance diagnostic accuracy.

Convexal Subarachnoid Hemorrhage Caused by Infective Endocarditis in a Patient with Advanced Human Immunodeficiency Virus (HIV): The Culprits and Bystanders.

BACKGROUND Convexal subarachnoid hemorrhage (cSAH), a rare form of non-aneurysmal subarachnoid hemorrhage, is confined to cerebral convexities without extension into basal cisterns or ventricles. Typical presentation includes thunderclap/progressive headache or transient focal neurological symptoms; rare manifestations include seizures, intractable vomiting, or altered mental status. Here, we report the first case of convexal subarachnoid hemorrhage and multifocal ischemic lesions caused by infective endocarditis (IE) in a treatment-naïve advanced HIV patient. CASE REPORT A 52-year-old HAART-naïve, HIV-positive, African American man presented with altered mental status, shortness of breath, nonproductive cough, and generalized weakness. His past medical history was significant for congestive heart failure, chronic obstructive pulmonary disease, and end-stage renal disease (noncompliant with hemodialysis). Head computed tomography (CT) showed an isolated sulcal hemorrhage in the mid-left frontal lobe. Fluid-attenuated inversion recovery/gradient recalled echo sequences confirmed a hemorrhage in the left-mid-frontal sulcus, and diffusion-weighted imaging revealed multifocal bilateral ischemic lesions. Transesophageal echocardiography exhibited mitral valve vegetations. Multifocal ischemic lesions and cSAH caused by infectious endocarditis were confirmed. Initiation of intravenous vancomycin and piperacillin-tazobactam allowed the patient to have resolution of his altered mental status. A head CT 5 days later revealed the resolution of cSAH. CONCLUSIONS Infective endocarditis should be considered as an underlying etiology of cSAH, especially when present with multifocal ischemic lesions. Risk factors contributing to the development of cSAH in the IE patient population should be explored in future studies. HIV has not been previously reported in this subgroup and its prevalence should be considered. The prognosis for cSAH in relation to IE is generally favorable.

Isolated Pulvinar/Hockey Stick Sign in Nonalcoholic Wernicke's Encephalopathy.

BACKGROUND Wernicke's encephalopathy (WE), a commonly misdiagnosed and underdiagnosed pathology, presents with altered mental status, ataxia, and ophthalmoplegia. WE is most commonly caused by excessive alcohol use, but also has diverse nonalcoholic etiologies. Here we describe 2 cases of nonalcoholic WE with different etiologies that were initially misdiagnosed due to lack of correlation of magnetic resonance imaging (MRI) findings with clinical information. CASE REPORT Patient A, a 50-year-old woman with recent gastric sleeve surgery, presented with horizontal gaze-evoked nystagmus, ataxia, and altered mental status. MRI fluid-attenuated inversion recovery (FLAIR) revealed isolated bilateral, symmetrical, thalamic hyperintensities, initially diagnosed as variant Creutzfeldt-Jakob disease. A review of imaging and clinical presentation provided an alternate diagnosis of nonalcoholic WE secondary to nutritional deficiency. Intravenous (IV) thiamine improved symptoms with resolution of MRI findings 6 months later. Patient B, a 64-year-old woman, presented with nausea, vomiting, dizziness, altered mental status, and weight loss. MRI FLAIR revealed isolated bilateral, symmetrical, thalamic hyperintensities, initially determined to be ischemia, prompting stroke management. A diagnosis of nonalcoholic WE was suggested, given the patient's low thiamine levels and history of malnutrition, and was confirmed by her excellent therapeutic response to IV thiamine. CONCLUSIONS Nonalcoholic WE remains a challenging diagnosis because of the variable clinical presentation, myriad of underlying etiologies, and lack of standardized diagnostic laboratory tests. A multidisciplinary approach with close collaboration between the radiologist and clinical care team is critical to narrow down the differential and initiate correct management. WE is a reversible disease with catastrophic consequences if it is not recognized and treated promptly.