Imaging review of neurofibromatosis: helpful aspects for early detection

Neurofibromatosis [NF] is divided into two types, NF type 1 and NF type 2. Optic nerve gliomas have a high degree of association with NF type 1. NF 2, less commonly seen, is a complex of cutaneous and deep neural tumors. It is an autosomal dominant familial disorder in which CNS is affected in about 15% of the cases. Bilateral acoustic neuromas are pathognomonic of NF type 2 which may be associated with meningiomas or ependymomas. Typical clinical manifestations of neurofibromatosis are cafe-au-lait spots and multiple cutaneous tumors. There is bone involvement as scoliosis, pseudoarthrosis of long bones, scalloping of vertebral bodies, abnormal rib tubulation and defective ossification of the skull. Extraskeletal manifestations of neurofibromatosis include optic nerve gliomas, pheochromocytoma, aneurysms of cerebral and renal arteries, acoustic neurilemmoma and superficial skin nodular neurofibromas. Here, we intend to present images of several cases of neurofibromatosis with different patterns of body involvement

Endotheilal function in adolescents with a history of premature coronary artery disease in one parent

In young adults, a family history of premature coronary artery disease [CAD], as well as genetic and environmental factors are independent risk factors for coronary artery disease. Endothelial function was studied in 30 children [21 boys and 9 girls with mean age of 14.9 +/- 2.3 years old]of patients with documented CAD [men .45 and women .50 years old]. Chidren did not have any history of diabetes mellitus, dyslipidemia, hypertension, and smoking [active/passive]. Using vascular ultrasound, we measured resting Basal Brachial artery Diameter [BBD] and Endothelium-Dependent Dilatation [EDD] in response to increased flow and sublingual glyceryltrinitrate [GTN], an Endothelium-Independent Dilation [EID]. These parameters were also measured in 30 control subjects with normal parents [18 boys and 12 girls with mean age of 14.2 +/- 2/5years old] and results were compared with each other. Adolescents in CAD group had abnormal Endothelial Dependent Dilatation or EDD/BBD [8.5 +/- 3.4% vs 11.8 +/- 4.5% in control subjects; P= 0.003].Endothelial Independent Dilatation [EID/BBD] in the positive fimily history group was significantly more than control subjects [18.5 +/- 6.7% vs 11.9 +/- 5.2%; P <0.001]. EDD/EID or the index of endothelial function was significantly lower in the positive family history group [0.92 +/- 0.05 vs 1 +/- 0.03; P<0.001]. There was no difference in EDD/EID index between those with history of premature CAD in mother [7 cases] and those with history of premature CAD in father [23 cases] [0.92 +/- 0.04 vs 0.91 +/- 0.05]. Normal adolescents without any cardiovascular risk factors but a history of premature coronary artery disease in one parent may have endothelial dysfunction, and there is no difference whether the CAD is in mother or father

Endothelial function in male body builders taking androgenic steroids

Adverse cardiovascular events have been reported in body builders taking anabolic steroids. Adverse effects of AAS on endothelial function can initiate atherosclerosis. This study evaluates endothelial function in body builders using AAS, compared with non-steroids using athletes as controls. We recruited 30 nonsmoking male body builders taking AAS, 14 in build up phase, 8 in work out phase, and 8 in post steroid phase, and 30 nonsmoking male athletes who denied ever using steroids. Serum lipids and fasting plasma glucose were measured to exclude dyslipidemia and diabetes. Brachial artery diameter was measured by ultrasound at rest, after cuff inflation, and after sublingual glyceriltrinitrate [GTN] to determine flow mediated dilation [FMD], nitro mediated dilation [NMD] and ratio of FMD to NMD [index of endothelial function]. Use of AAS was associated with higher body mass index [BMI] and low density lipoprotein-cholesterol [LDL-C]. Mean ratio of flow mediated dilatation after cuff deflation to post GTN dilatation of brachial artery [index of endothelial function] in body builders taking AAS was significantly lower than control group [0.96[0.05] versus 1[0.08]; p=0.03]. After adjusting BMI, age and weight, no significant difference was seen in index of endothelial function between two groups [p=0.21]. Our study indicates that taking AAS in body builders doesn't have direct effect on endothelial function. Future study with bigger sample size and measurement of AAS metabolites is recommended

Magnetic resonance angiography of intracranial aneurysms: comparison with intraarterial digital subtraction angiography

Background: the aim of this study is to determine sensitivity and specificity of 3D- Time-Of-Flight and Phase Contrast Magnetic resonance angiography [MRA] in comparison with intra-arterial digital subtraction angiography [IA- DSA] in detection of intracranial aneurysms

Methods: 54 patients with 22 aneurysms underwent MRA and then IA-DSA prospectively from October 2002 till December 2003

Results: MRA detected 20 aneurysm where as IA- DSA scored 22 [90.9%]. false positive in MRA was 18.1% [4 cases] and no false positive was in IA- DSA. So sensitivity of MRA in detection of intracranial aneurysms is 90.9%, and specificity is 88.8%. Positive predictive value of 83.3% and negative predictive value of 94.1% is also calculated

Conclusion: MRA is a valuable non- invasive technique in the detection of intracranial aneurysms that can be a proper screening test for this purpose