Neurology and general medicine

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.

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

BACKGROUND Hypercoagulability with thrombosis and associated inflammation has been well-documented in COVID-19, and catastrophic cerebral venous sinus thromboses (CVSTs) have been described. Another COVID-19–related complication is bacterial superinfection, including sinusitis. Here, the authors reported three cases of COVID-19–associated sinusitis, meningitis, and CVST and summarized the literature about septic intracranial thrombotic events as a cause of headache and fever in COVID-19. OBSERVATIONS The authors described three adolescent patients with no pertinent past medical history and no prior COVID-19 vaccinations who presented with subacute headaches, photosensitivity, nausea, and vomiting after testing positive for COVID-19. Imaging showed subdural collections, CVST, cerebral edema, and severe sinus disease. Two patients had decline in mental status and progression of neurological symptoms. In all three, emergency cranial and sinonasal washouts uncovered pus that grew polymicrobial cultures. After receiving broad-spectrum antimicrobials and various additional treatments, including two of three patients receiving anticoagulation, all patients eventually became neurologically intact with varying ongoing sequelae. LESSONS These cases demonstrated similar original presentations among previously healthy adolescents with COVID-19 infections, concurrent sinusitis precipitating CVST, and subdural empyemas. Better recognition and understanding of the multisystem results of severe acute respiratory syndrome coronavirus 2 and the complicated sequelae allows for proper treatment. 

Intracranial complications of hypercoagulability and superinfection in the setting of COVID-19: illustrative cases

Background: Fabry disease (FD) is the second most common lysosomal storage disorder. This disorder affects multiple systems that include the cardiac, renal, and nervous system. The pulvinar sign (PS) is a relatively common sign seen in patients with FD. The PS is a bilateral, symmetrical pulvinar high signal relative to the signal intensity seen on unenhanced T1-weighted brain MR imaging. Methods: We conducted a systematic review with metanalysis to analyze the pool prevalence of the disorder. We used the Moose Guidelines and PRISMA Protocol for this systematic review and Robins 1 to access the BIAS of the study. To analyze the pool prevalence, we used “Open Meta-Analysis” software for analyzing the study. We used “Review Manager 5.4” to analyze the odds ratio between patients with and without the PS and patients with and without stroke among patients with FD. Results: We gather 12 studies from 2003 to 2021 for the analysis of this study. The pool prevalence of the study was 0.146 (0.076–0.217) (62/385 cases) with a 95% CI (0.0945–0.415) (p < 0.01). The prevalence was much higher in men (59 cases) than in women (3 cases). There was no relationship between the pulvinar sign and patients with stroke among patients with Fabry disease. Odds ratio 1.97 95% CI (0.35–11.21), p = 0.44; Tau2 = 0.77. There seems to be a correlation with renal failure (RF), but there were very few studies to conduct a metanalysis with RF. Conclusions: The prevalence of the PS among all studies was 23.9%; the prevalence of this sign is higher among males. We found that FD patients who had strokes did not have higher odds of presenting with the Pulvinar Sign than the FD patients who did not suffer a stroke. Patients with renal failure and FD seem to have a higher tendency to have the PS, but there were not enough studies to analyze that theory. Overall, we think the pulvinar sign has a poor prognostic value in patients with Fabry’s disease.

https://www.mdpi.com/2035-8377/14/2/41/pdf?version=1654073487

Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis

Cerebral venous sinus thrombosis (CVST) is the complete or partial occlusion of the main venous sinuses or cortical veins. The most known risk factors are oral contraceptives, pregnancy, thrombophilias, malignancy and infections. The SARS-CoV-2 infection has been associated with a hypercoagulable state and there are some reported cases of CVST in SARS-CoV-2 patients. Although infection is one of the possible causes of CVST, it is important to rule out malignancy. We report a case of a 27-year-old male, with a recent SARS-CoV-2 infection, who went to the emergency department for a severe left occipital headache and was diagnosis with CVST. An etiological study revealed a retroperitoneal mass, compatible with a paraganglioma.

Paraganglioma: An Unexpected Diagnosis in a Patient With Cerebral Venous Sinus Thrombosis and SARS-CoV-2 Infection

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.

Isolated Pulvinar/Hockey Stick Sign in Nonalcoholic Wernicke’s Encephalopathy

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.

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.

Neha Sharma

Papers

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

Isolated Pulvinar/Hockey Stick Sign in Nonalcoholic Wernicke’s Encephalopathy

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.