High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis.

BACKGROUND AND PURPOSE: High-resolution MR imaging is an emerging tool for evaluating intracranial artery disease. It has an advantage of defining vessel wall characteristics of intracranial vascular diseases. We investigated high-resolution MR imaging arterial wall characteristics of CNS vasculitis and reversible cerebral vasoconstriction syndrome to determine wall pattern changes during a follow-up period. MATERIALS AND METHODS: We retrospectively reviewed 3T-high-resolution MR imaging vessel wall studies performed on 26 patients with a confirmed diagnosis of CNS vasculitis and reversible cerebral vasoconstriction syndrome during a follow-up period. Vessel wall imaging protocol included black-blood contrast-enhanced T1-weighted sequences with fat suppression and a saturation band, and time-of-flight MRA of the circle of Willis. Vessel wall characteristics including enhancement, wall thickening, and lumen narrowing were collected. RESULTS: Thirteen patients with CNS vasculitis and 13 patients with reversible cerebral vasoconstriction syndrome were included. In the CNS vasculitis group, 9 patients showed smooth, concentric wall enhancement and thickening; 3 patients had smooth, eccentric wall enhancement and thickening; and 1 patient was without wall enhancement and thickening. Six of 13 patients had follow-up imaging; 4 patients showed stable smooth, concentric enhancement and thickening; and 2 patients had resoluton of initial imaging findings. In the reversible cerebral vasoconstriction syndrome group, 10 patients showed diffuse, uniform wall thickening with negligible-to-mild enhancement. Nine patients had follow-up imaging, with 8 patients showing complete resolution of the initial findings. CONCLUSIONS: Postgadolinium 3T-high-resolution MR imaging appears to be a feasible tool in differentiating vessel wall patterns of CNS vasculitis and reversible cerebral vasoconstriction syndrome changes during a follow-up period.

Relation of the leukocyte count to recurrent cardiac events in stable patients after acute myocardial infarction.

Increasing evidence implicates inflammation as a risk factor for coronary artery disease. We determined whether an elevated leukocyte count is associated with an increased risk of death or reinfarction in stable patients with a past acute myocardial infarction (AMI). The current analysis is a substudy of the Multicenter Diltiazem Postinfarction Trial, which investigated the effect of diltiazem on mortality and reinfarction in 2,466 patients hospitalized for AMI. We included 1,294 patients in whom a leukocyte count was obtained 6 months after the index AMI. The composite end point of reinfarction or death was used as the primary end point of the study and reinfarction or cardiac death was used as a secondary end point. The study population was divided into 4 quartiles (Q1, Q2, Q3, and Q4) based on the leukocyte count. During a mean follow-up period of 25 months, 163 patients reached the primary end point: 8.7%, 10.9%, 14.0%, and 16.7%, in Q1, Q2, Q3, and, Q4 respectively (p = 0.01). After adjusting for potential covariates, Cox proportional-hazards analysis revealed that an increased leukocyte count was associated with an increased risk of both the primary end point (hazard ratio/1 quartile increase in leukocyte count, 1.26; p = 0.003; 95% confidence interval 1.08 to 1.47) and secondary end point (hazard ratio, 1.18/1-quartile increase; p = 0.05; 95% confidence interval 1.00 to 1.40). In conclusion, an increased leukocyte count measured in the stable post-AMI period is associated with an increased risk of cardiac events. These findings indicate that the leukocyte count may be another marker of an atherosclerotic inflammatory process that contributes to cardiac events in postinfarction patients.

Arthritis in familial Mediterranean fever.

We studied the rheumatic and various clinical manifestations of familial Mediterranean fever (FMF) in Lebanon. A retrospective review was performed of the medical records of 74 FMF patients seen at the American University of Beirut Medical Centre (AUB-MC) from 1979 to 1996. We also reviewed the medical literature from 1968 to 2000 using MEDLINE and the key words "familial Mediterranean fever" and "arthritis". Arthritis was the presenting symptom in 12 cases (16.2%). Twenty-three patients (31%) had definite arthritis during the course of the disease that was monoarticular in 16 (70%), oligoarticular in six (26%), and polyarticular (rheumatoid-like) in one (4%). Arthritis of the large joints of the knees and ankles was the most frequent articular involvement. The arthritis was transient, monoarticular, nonerosive, and nondeforming in the majority of cases. Four patients (5.4%) had chronic arthritis, with one requiring total hip replacement. As in previous reports on arthritis of FMF, the majority of FMF patients studied in Lebanon had a transient monoarticular nonerosive and nondeforming type of arthritis affecting predominantly the large joints of the lower extremities.

Asthma and the risk of cardiac events in the Long QT syndrome. Long QT Syndrome Investigative Group.

While acquiring data for the International Long QT Syndrome Registry, we noticed that a number of patients referred for long QT syndrome (LQTS) were affected by asthma. The effect of asthma comorbidity on clinical course of LQTS has not been studied. This study aimed to evaluate the prevalence of asthma in patients with LQTS, determine the influence of asthma comorbidity on outcome of LQTS patients, and to investigate the confounding effects of beta mimetics and beta blockers on the occurrence of cardiac events in asthmatic patients. The influence of asthma on risk of cardiac events (syncope, aborted cardiac arrest, or LQTS death) was evaluated after accounting for age, gender, QTc, and RR interval duration, beta-blocker and beta-mimetic use. Asthma was identified in 226 (5.2%) of 4,310 studied LQTS family members. Longer QTc duration was associated with higher incidence of asthma (p <0.001). Asthma was independently associated with significantly increased risk of cardiac events in affected LQTS patients (hazard ratio 1.32; p = 0.048) and in borderline-affected family members (hazard ratio 2.08; p = 0.004) after adjustment for QTc, RR interval, and gender. An increased risk of cardiac events in asthmatic patients observed before beta-blocker therapy was reduced after initiation of treatment with beta blockers. In conclusion, the occurrence of asthma in LQTS patients increases with QTc duration. Asthma comorbidity in LQTS patients is associated with an increased risk of cardiac events. The asthma-associated increase in the risk of LQTS-related cardiac events is diminished after initiation of beta-blocker therapy, suggesting a possible role of beta-receptor modulation underlying asthma-LQTS association.

Takayasu's arteritis presenting as fever of unknown origin: report of two cases and literature review.

OBJECTIVES: This study reports two patients with Takayasu's arteritis presenting with a fever of unknown origin (FUO) and reviews the literature on that association. METHODS: We describe the clinical presentation, course, and outcome of the two patients, and reviewed the medical literature from 1968 till 1997 using MEDLINE and the key words fever, diagnosis, and Takayasu's arteritis. RESULTS: Takayasu's arteritis is rarely reported as a cause of FUO. Noninvasive diagnostic techniques such as magnetic resonance imaging, computed tomography scanning, gallium-67 scintigraphy, and ultrasonography may help in the diagnosis of Takayasu's arteritis in the prepulseless stage. CONCLUSION: Takayasu's arteritis should be considered in the differential diagnosis of FUO, especially in young women.

Miliary tuberculosis presenting as tenosynovitis in a case of rheumatoid arthritis.

A 77-year-old woman with a seropositive nodular rheumatoid arthritis and vasculitis, who was treated with high doses of corticosteroids and intravenous cyclophosphamide, developed miliary tuberculosis that was heralded by a tenosynovitis in her right wrist. A 1-year course of anti-tuberculous therapy resulted in complete resolution of the tenosynovitis and disseminated infection.

Electrocardiographic findings in patients with diphenhydramine overdose.

QT interval prolongation and torsades de pointes ventricular tachycardia have been reported after therapeutic doses and overdosage of second generation antihistamines, such terfenadine and astemizol. Diphenhydramine (DPHM), a first generation H1 antagonist, is the most frequently used antihistaminic drug. Despite its widespread use, there are no data about cardiac action and electrocardiographic consequences of DPHM overdose. The 12-lead electrocardiograms of 126 patients (mean age 26 +/- 11 years) who had DPHM overdose were evaluated. The ingestion of large doses of DPHM (in majority of cases the dose was >500 mg) was primarily suicidal. Repolarization duration, dispersion, and morphology were evaluated in DPHM overdose patients and compared with those of healthy subjects. Mean heart rate of DPHM overdose patients was 103 +/- 25 beats/min. The QTc duration was significantly longer (453 +/- 43 vs 416 +/- 35 ms, respectively, p <0.001) and mean T-wave amplitude significantly lower (0.20 +/- 0.10 vs 0.33 +/- 0.15 mV, respectively, p <0.001) in DPHM-overdose patients than in control subjects. Dispersion of repolarization was significantly lower in DPHM-overdose patients than in control subjects (42 +/- 25 vs 52 +/- 21 ms, respectively; p = 0.003). None of the DPHM-overdose patients experienced torsades de pointes. In conclusion, DPHM overdose is associated with a significant increase in heart rate and a significant but moderate QTc prolongation. None of the studied patients, including those who had apparent QTc prolongation, experienced torsades de pointes ventricular tachycardia.