Neuro-Behcet's Disease, a Diagnostic Challenge.

Neuro-Behcet's disease (NBD) is a rare neurological manifestation of the systemic small vessel vasculitis called Behcet's disease. It can present in various ways with predilection for the brain stem, thalamo- hypothalamic regions, cerebellum and basal ganglia. In this case, we describe a case of young stroke that was later attributed to NBD.

National Trends and Outcomes of Endomyocardial Biopsy for Patients With Myocarditis: From the National Inpatient Sample Database.

BACKGROUND: The utility of endomyocardial biopsy (EMB) in the management of myocarditis in the era of advanced cardiac imaging has been challenged. METHODS AND RESULTS: The Nationwide Inpatient Sample Database (years 1998-2013) was queried to identify hospitalization records with a primary diagnosis of myocarditis, and underwent EMB procedure. We identified 22,299 hospitalization records with a diagnosis of myocarditis during the study period. Of those, 798 (3.6%) underwent EMB procedures. There was an average decrease in the incidence of EMB for myocarditis by 0.15% (P < .01) over the study period. Younger patients, women, and those with chronic kidney disease were more likely to undergo EMB. On multivariate analysis, patients with myocarditis who underwent EMB had higher in-hospital mortality (hazard ratio [HR] 1.97, 95% confidence interval [CI] 1.41-2.74) and longer median hospital stay (9 days vs 3 days; P < .001). EMB was associated with a higher incidence of cardiac tamponade (odds ratio [OR] 5.21, 95% CI 2.76-9.82), cardiogenic shock (OR 4.66, 95% CI 3.75-5.78), need for intra-aortic balloon pump (OR 3.52, 95% CI 2.49-4.97), and need for extracorporeal membrane oxygenation (OR 4.26, 95% CI 2.78-6.53). CONCLUSIONS: The use of EMB in hospitalizations with myocarditis has decreased over time. The use of EMB was associated with a higher likelihood of in-hospital mortality and morbidity. Whether these findings represent a causative association from the procedure or a consequence of more severe disease in this group could not be confirmed in this study.

Electrocardiographic left atrial abnormalities predict cardiovascular mortality.

OBJECTIVE: Clinical utilization of electrocardiography for diagnosis of left atrial abnormalities is hampered by variable P-wave morphologies, multiple empiric criteria, and lack of an imaging "gold standard". Our aim was to determine the prevalence of P-wave patterns and demonstrate which components have associations with cardiovascular death (CVD). METHODS: This is a retrospective analysis of 20,827 veterans <56 years of age who underwent electrocardiograms at a Veteran's Affairs Medical Center from 1987 to 1999, followed for a median duration of 17.8 years for CVD. Receiver Operating Characteristic, Kaplan-Meier and Cox Hazard analyses were applied, the latter with adjustment for age, gender and electrocardiography abnormalities. RESULTS: The mean age was 43.3 ±â€¯8 years, and 888 CVD (4.3%) occurred. A single positive deflection of the P-wave (Pattern 1) was present in 29% for V1 and 81% for V2. A singular negative P-wave (Pattern 2) was present in 4.6% for V1 and 1.6% in V2. A P-wave with an upward component followed by downward component (Pattern 3) was present in 64.5% for V1 and 17.5% for V2. When the downward component in Patterns 2 and/or 3 is at least -100 µV, a significant association is observed with CVD (adjusted hazard ratios [HRs] 2.9-4.1, P < 0.001). Total P-wave duration ≥140 ms was also associated with CVD (adjusted HR 2.2, P < 0.001). CONCLUSIONS: A negative P-wave in V1 or V2 ≤-100 µV, and P-wave with a duration of ≥140 ms, all have independent and significant associations with CVD, with HRs comparable to other electrocardiography abnormalities.

Neuro-Behcet's Disease Presenting as a Young Stroke.

Neuro-Behcet's disease (NBD) is a rare neurological manifestation of the systemic small vessel vasculitis called Behcet's disease. It can present in various ways with predilection for the brain stem, thalamo- hypothalamic regions, cerebellum and basal ganglia. In this case, we describe a case of young stroke that was later attributed to NBD.

An Elusive Diagnosis: Case Reports of Secondary Hemophagocytic Lymphohistiocytosis and Review of Current Literature.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and serious hematologic disorder characterized by severe immune system dysregulation with a cytokine storm and histologic evidence of hemophagocytosis. It can be inherited or develop secondary to other diseases. We present three cases of secondary HLH in patients with distinct backgrounds. Our objective is to characterize the unique features of the disease, its underlying associations, treatment, and potential prognostic variables. The first case was a 20-year-old male with a history of intravenous (IV) drug abuse who presented with multi-organ failure and septic shock. A diagnosis of HLH was suspected after finding a ferritin of >100,000 ng/mL and confirmed with bone marrow biopsy. Furthermore, the patient was found to have significant Epstein-Barr virus (EBV) viremia. He responded well to the HLH-94 protocol with the addition of rituximab and ganciclovir. The second case was a 50-year-old female with a history of human immunodeficiency virus (HIV) who presented with multi-organ failure and severe anemia. Ferritin was also significantly elevated and a bone marrow biopsy confirmed the diagnosis of HLH. She was started on HLH-94 protocol. Despite treatment, the patient expired due to worsening renal failure and shock. Her autopsy report also showed evidence of Hodgkin's lymphoma. The third case was a 57-year-old male with a history of Crohn's disease treated with infliximab and adalimumab, who presented with multi-organ failure and pancytopenia. A diagnosis of HLH was made based on clinical findings and later confirmed on bone marrow biopsy. He responded to HLH-94 protocol but experienced fatal gastrointestinal bleeding. Patients presenting with HLH are often critically ill and deteriorate rapidly. The diagnosis is often challenging to establish due to its variable presentation and association with other pathologies. A moderate index of suspicion should be present for patients who have febrile illness with pancytopenia, multi-organ failure, high ferritin, and low fibrinogen levels. We discuss associations with viral infections, hematologic malignancies and immunosuppressive therapy. Treatment is directed at suppressing the immune response and for secondary HLH, addressing the underlying conditions, such as use of rituximab for EBV viremia and treatment of lymphoma.

Meta-analysis of randomized trials on percutaneous patent foramen ovale closure for prevention of migraine.

BACKGROUND: The role of percutaneous patent foramen ovale (PFO) closure for prevention of migraine is controversial. METHODS: We performed a computerised search of MEDLINE, EMBASE and COCHRANE databases through December 2017 for randomised trials evaluating PFO closure versus control in patients with migraine headaches (with or without aura). The main study outcome was the reduction in monthly migraine attacks after PFO closure compared with the control group. RESULTS: The final analysis included three randomised trials with a total of 484 patients. Reduction in monthly migraine attacks was higher in PFO closure compared with the control group (standardised mean difference-SMD = 0.25; 95% CI: 0.06-0.43; p = .01). There was higher reduction of monthly migraine days in PFO closure group compared with control group (SMD = 0.30; 95% CI: 0.08-0.53; p = .01). There was no statistically significant difference in complete resolution of migraine attacks (OR: 3.67; 95% CI: 0.66-20.41; p = .14) and in responders' rate (OR: 1.92; 95% CI: 0.76-4.85; p = .17) between PFO closure and control groups. In patients whose majority of migraine attacks are with aura, there was an observed reduction in migraine attacks in PFO closure compared with control groups (SMD = 0.86; 95% CI: 0.07-1.65; p = .03). CONCLUSION: PFO closure might be beneficial in migraine patients by reducing migraine attacks and migraine days, especially in patients whose majority of migraine attacks are with aura. However, those benefits were not associated with an improvement in responders' rate or complete resolution of migraine; raising concerns on the magnitude of clinical benefit of PFO closure in migraine prevention.