ATYPICAL MRI FINDINGS IN CEREBRAL ADRENOLEUKODYSTROPHY: A CASE REPORT.

Adrenoleukodystrophy is a rare X-linked hereditary disease that results in accumulation of very-long-chain fatty acids in all body tissues, thus causing demyelination of the white matter. Magnetic resonance imaging (MRI) is a reliable radiological modality to demonstrate the extension of brain lesions and severity of the disease. In the classic form, the parieto-occipital white matter is affected. Besides, atypical MRI findings such as primary frontal lobe involvement are rarely described. We report a case of adrenoleukodystrophy presenting with rare MRI findings such as bilateral symmetric frontal lobe white matter changes suggesting anterior predominance.

Is multiple sclerosis a risk factor for atherosclerosis?

BACKGROUND: Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system. We aimed to discuss possible predisposing factors to atherosclerosis such as carotid intima-media thickness (CIMT) and high-sensitivity C-reactive protein (Hs-CRP) levels in MS. METHODS: Thirty-five ambulatory patients with relapsing-remitting MS (RRMS) (22 females and 13 males) and 34 healthy controls (21 females and 13 males) with similar demographic variables were included. Blood cell counts, cholesterol levels, vitamin D and B12, Hs-CRP levels, body mass index (BMI), history of smoking, and CIMT of both groups, Expanded Disability Status Scale (EDSS) scores, and disease duration of patients were recorded. Patients with a history of other vascular diseases such as hypertension, diabetes mellitus, peripheral artery disease, and acute relapses were excluded. RESULTS: Sixty-nine participants were included. The mean age of the study population was 35.8±7.1 years. Right CIMT was significantly greater in the patient population (P<0.001). Spearman's correlation coefficient between age and right CIMT was r=0.41, P=0.01. When we compared the Hs-CRP with a cut-off value of ≤3, the right, left, and mean CIMT levels were not statistically significant (P=0.17; P=0.22; P=0.15). The mean serum vitamin D levels were higher in the patient group and this was statistically significant (P<0.001). The statistically significant factors identified with univariate analysis with P<0.2 were further entered into multivariate modelling. CONCLUSION: CIMT seems to be affected in patients with MS by means of the disease itself and age. Thus, CIMT might reflect the predisposition to subclinical atherosclerosis more than Hs-CRP. Further investigation in a large MS population is still needed.

The association of the circle of Willis anomaly and risk of stroke in patients with carotid artery disease.

RESULTS: In this retrospective study, we analyzed 175 patients who presented at our outpatient stroke clinic between January, 2013 and June, 2015 with either unilateral symptomatic or asymptomatic carotid artery disease, and who had had CT angiography imaging performed. Demographic properties, carotid artery stenosis and the anomaly of the circle of Willis was recorded. CONCLUSION: There was no statistically significant difference in patients with symptomatic and asymptomatic carotid artery disease in terms of the anomaly of the circle of Willis.

The association of the circle of Willis anomaly and risk of stroke in patients with carotid artery disease / A associação de anomalia de Círculo de Willis e o risco de AVC em pacientes com doença carotídea

ABSTRACT The circle of Willis is an important collateral system that maintains perfusion to the stenotic area from the contralateral carotid and basilar artery to the region of reduced brain perfusion. The aim of the present study was to compare the circle of Willis anomaly in patients with unilateral symptomatic and asymptomatic carotid artery disease. Results In this retrospective study, we analyzed 175 patients who presented at our outpatient stroke clinic between January, 2013 and June, 2015 with either unilateral symptomatic or asymptomatic carotid artery disease, and who had had CT angiography imaging performed. Demographic properties, carotid artery stenosis and the anomaly of the circle of Willis was recorded. Conclusion There was no statistically significant difference in patients with symptomatic and asymptomatic carotid artery disease in terms of the anomaly of the circle of Willis.

RESUMO O Círculo de Willis é um importante sistema colateral que mantém a perfusão à área estenótica da carótida contralateral e da artéria basilar para a região de perfusão cerebral reduzida. O objetivo do presente estudo foi comparar a anomalia do Círculo de Willis em pacientes com doença carotídea assintomática e sintomática unilateral. Resultados Neste estudo retrospectivo, foram analisados 175 pacientes que foram à nossa clínica ambulatorial de AVC, entre janeiro de 2013 e junho de 2015, com doença carotídea assintomática ou sintomática unilateral, e que fizeram angiografia por tomografia computadorizada. Propriedades demográficas, estenose da artéria carótida e anomalia do Círculo de Willis foram registradas. Conclusão Não houve diferença estatisticamente significativa em pacientes com doença carotídea sintomática e assintomática em termos de anomalia do Círculo de Willis.

Calculation of an Optic Nerve Injury Risk Profile Before Sphenoid Sinus Surgery.

OBJECTIVES: Our objective was to analyze variations in the optic nerve (ON) course and surrounding structures in an effort to construct an optic nerve injury risk profile before endoscopic intranasal sphenoidal, or endoscopic endonasal transphenoidal, skull-base surgery, and eventually to construct and formulate a common classification by combining the known classes. The authors used computed tomography (CT) toward this end. METHODS: The authors retrospectively reviewed 200 consecutive CT scans (400 sides) of the paranasal sinuses. The pneumatization of the anterior clinoid process, the relationships of the ONs to the sphenoidal sinuses, and ON dehiscence were evaluated. The authors then created a formula by which risk profiles can be constructed for patients for whom sphenoid or parasellar surgery is planned. RESULTS: Pneumatization of the anterior clinoid process was evident in 28.25%. Dehiscence of the bony wall of the ON was evident in 9.5%. The ON course lay adjacent to the sphenoidal sinus, causing sinus wall indentation, in 23%. Cumulative optic nerve injury risk scoring showed that, radiologically, surgery on 8.5% and 1.5% of sphenoid sinuses described here carried severe or critical risk of ON injury, respectively. CONCLUSIONS: Head-and-neck surgeons and neurosurgeons should be aware of variations in ON course. The authors composed an optic nerve injury risk classification category based on the sum of individual weights of each of these classes. Reductions in ON injuries require careful evaluation of potential variant anatomies. Preoperative CT scans must be meticulously reviewed to avoid ON injury.

Association of Neutrophil/Lymphocyte Ratio with Plaque Morphology in Patients with Asymptomatic Intermediate Carotid Artery Stenosis.

BACKGROUND AND OBJECTIVES: Non-calcified carotid plaques are more unstable than calcified plaques, and they are associated with a higher risk of rupture, thromboembolism, and consequently, stroke. The purpose of the present study is to compare calcified and non-calcified plaques that cause intermediate carotid artery stenosis with respect to neutrophil/lymphocyte ratio (NLR). SUBJECTS AND METHODS: A total number of 139 asymptomatic patients with 50-70% stenosis of the carotid artery were included in this study. Carotid Doppler ultrasound imaging and computed tomography angiography were performed to divide the carotid artery plaques into two groups as calcified and non-calcified. Patients included in the calcified (n=73) and non-calcified (n=66) plaque groups were compared with respect to total neutrophil count, lymphocyte count and NLR. RESULTS: Total lymphocyte count was statistically significantly lower in the non-calcified plaque group compared to the calcified plaque group (total lymphocyte count in non-calcified/calcified plaque groups [103/mm3]: 2.1/2.3, respectively) (p=0.002). NLR was statistically significantly higher in the non-calcified plaque group compared to the calcified plaque group (NLR in non-calcified/calcified plaque groups: 2.6/2.1, respectively) (p<0.001). The cut-off value for NLR was found to be >2.54. Multivariate regression analysis showed that NLR was independently associated with non-calcified carotid artery plaques (odds ratio 5.686, 95% CI 2.498-12.944, p<0.001). CONCLUSIONS: NLR is increased in the presence of non-calcified carotid artery plaques that cause asymptomatic intermediate stenosis. Increased NLR can be used as a marker to assess the risk of rupture of non-calcified carotid artery plaques.

Predictors of Symptom Development in Intermediate Carotid Artery Stenosis: Mean Platelet Volume and Platelet Distribution Width.

Platelets play an important role in the pathogenesis of atherothrombosis. Platelet activation is associated with increased mean platelet volume (MPV) and platelet distribution width (PDW). In this study, we investigated the relation of MPV and PDW with the risk of stroke in patients with intermediate (50%-70%) carotid artery stenosis. A total of 254 patients (115 symptomatic and 139 asymptomatic) with intermediate carotid artery stenosis were enrolled in this study. Symptomatic and asymptomatic patients were compared in regard to MPV and PDW. Mean platelet volume was significantly greater in the symptomatic group compared with the asymptomatic group (11.1 and 9.4 fL, respectively; P < .001). Platelet distribution width was significantly greater in the symptomatic group compared with the asymptomatic group (15.0% and 11.9%, respectively; P < .001). Multivariate regression analysis showed that an MPV ≥10.2 fL and a PDW ≥14.3% were independent predictors of developing symptomatic carotid artery stenosis. Mean platelet volume and PDW are increased in the presence of symptomatic intermediate carotid artery stenosis. Increased MPV and PDW may be independent predictors of developing symptomatic carotid artery plaque.

Magnetic resonance imaging findings of two sisters with Van der Knaap leukoencephalopathy.

Megalencephalic leukoencephalopathy with subcortical cysts, or Van der Knaap leukoencephalopathy, is a rare disease which is characterised by macrocephaly and neurological disorders with autosomal recessive inheritance. Magnetic resonance imaging is very helpful for determining distinctive findings and distinguishing other diseases. We present the radiological findings of two sisters (aged 6 and 10 years) diagnosed with Van der Knaap leukoencephalopathy.

Acute Carotid Artery Stent Thrombosis Due to Dual Antiplatelet Resistance.

Carotid artery stenting (CAS) is a revascularization modality that is an alternative to carotid endarterectomy. The efficacy of CAS in primary and secondary prevention from ischemic stroke has been demonstrated in various trials. Acute thrombosis of CAS is a rare complication that can lead to dramatic and catastrophic consequences. We discuss a case of acute CAS thrombosis in a patient who had previously undergone successful CAS. CAS was performed in a 73-year-old man who had had dysarthria lasting 2 weeks with 95% stenosis in his left internal carotid artery. An acute cerebrovascular event resulting in right-sided hemiplegia developed 24 h after the procedure. Computed tomographic carotid angiography revealed complete occlusion of the stent with thrombus. The cause of stent thrombosis was thought to be antiaggregant resistance to both acetylsalicylic acid and clopidogrel. The most important cause of acute CAS thrombosis is inadequate or ineffective antiaggregant therapy. Evaluating patients who are candidates for CAS for acetylsalicylic acid and clopidogrel resistance may preclude this complication.

Effect of angiotensin II type 1 receptor blocker on osteoporotic rat femurs.

BACKGROUND: Osteoblasts and osteoclasts are known to express Ang II type I (AT1) receptor in cell cultures, suggesting the existence of local renin-angiotensin system (RAS) in bone. This study was designed to investigate the effects of losartan as AT1 receptor blocker on ovariectomized rats' femur. METHODS: Losartan (5 mg/kg/day) was administered via oral gavage for 8 weeks. Bone mineral density (BMD) was measured using dual energy X-ray absorptiometry, while tensile and three-point bending tests were performed for evaluation of biomechanical properties of bone. The trabecular porosity was analyzed by scanning electron microscopy. RESULTS: There was a significant decrease in BMD values of ovariectomized rats' femurs which were reversed by losartan treatment. According to tensile test results, ultimate tensile strength and strain values of losartan treated ovariectomized rats' femurs increased and decreased, respectively, when compared to that of ovariectomized animals. Losartan treatment also caused a significant recovery in flexural strength and modulus parameters regarding respective control values, which mean losartan treated ovariectomized rats' femur had more force tolerance until break than ovariectomized rats' femur. Quantitative microscopic analysis showed larger trabecular porosity in ovariectomized rats than control rat femurs and it was significantly decreased after losartan treatment. CONCLUSION: Blockage of AT1 receptor increased strength, mass and trabecular connections of ovariectomized rat femurs. Therefore, it is tempting to speculate that drugs, including AT1 receptor blockers, may be used for the treatment of osteoporosis or reduction of its detrimental effects in the future.