MR imaging of the superior profile of the midbrain: differential diagnosis between progressive supranuclear palsy and Parkinson disease

BACKGROUND AND PURPOSE: Quantitative evaluation of midbrain atrophy may be useful in differentiating progressive supranulear palsy (PSP) from Parkinson disease (PD); however, this finding is not specific of PSP, and quantitative measurements are not always practical. We determined whether an abnormal superior midbrain profile (flat or concave aspect) is a more practical diagnostic parameter for PSP. METHODS: MR imaging studies of 25 patients with PSP and 27 with PD were reviewed by means of five parameters: midbrain superior profile on midsagittal T1-weighted images, midbrain atrophy, tegmental abnormal T2 hyperintensity, abnormal T2 putaminal hypointensity or hyperintensity on axial proton density-weighted images. We also measured the anteroposterior diameter of the midbrain on axial T2-weighted sections at the level of the superior colliculus. RESULTS: The finding of an abnormal superior profile of the midbrain had 68% sensitivity and 88.8% specificity. Midbrain atrophy had 68% sensitivity and 77.7% specificity. Tegmental T2 hyperintensity had 100% specificity but poor sensitivity (28%). Only 14.8% of patients with PD and 24% of those with PSP had abnormal putaminal T2 hypointensity; none had proton-density hyperintensity. With PSP, the average midbrain diameter was smaller than that with PD, but an important overlap was observed. Reader discordance was lower for the midbrain superior profile sign (eight of 52 cases); this was similar for tegmental hyperintensity (nine of 52 cases) and higher for midbrain atrophy (16 of 52 cases). CONCLUSION: An abnormal superior profile of the midbrain facilitates the distinction of PSP from PD and may support the clinical differential diagnosis of parkinsonism.     

Demonstration of acute ischemic lesions in the fetal brain by diffusion magnetic resonance imaging

The etiology of amyotrophic lateral sclerosis remains unknown in the majority of cases. Homozygous SMN1 (survival motor neuron) gene deletion causes spinal muscular atrophy, and SMN2 gene deletions are possible risk factors in lower motor neuron disease. We studied SMN1 and SMN2 genes copy numbers in 167 amyotrophic lateral sclerosis patients and in 167 matched controls. We noted that 16% of amyotrophic lateral sclerosis patients had an abnormal copy number of the SMN1 gene (1 or 3 copies), compared with 4% of controls. An abnormal SMN1 gene locus may be a susceptibility factor for amyotrophic lateral sclerosis.     

Hydrocarbon exposure and Parkinson's disease

BACKGROUND: Single cases of parkinsonism have been associated with hydrocarbon solvents. OBJECTIVE: To determine whether exposure to hydrocarbon solvents is related to PD. METHODS: Cohort study of 990 patients with PD according to Core Assessment Program for Intracerebral Transplantations (CAPIT) criteria, selected from 1455 consecutive subjects presenting at a referral center; case-control study assessing Unified PD Rating Scale scores (motor score as primary endpoint) in all subjects with positive history of hydrocarbon solvent exposure (n = 188), matched for duration of disease and gender to 188 subjects selected from the remaining 802 with a negative history. Two subgroups in the case-control study included the following: 1) response to apomorphine (n = 26); 2) brain MRI (n = 15). PET imaging (n = 9) was compared with that of historic controls. RESULTS: Exposed patients were younger (61.0 +/- 9.4 versus 64.7 +/- 9.4 years, p = 0.002), predominantly male (76.4% versus 45.2%, p = 0.0001), less educated (8.4 +/- 4.2 versus 10.1 +/- 4.4 years, p = 0.0001), and younger at onset of disease (55.2 +/- 9.8 versus 58.6 +/- 10 years, p = 0.014). Exposure to hydrocarbon solvents directly correlated to disease severity (r = 0. 311) and inversely correlated to latency period (r = -0.252). Nine blue-collar occupations accounted for 91.1% of exposures. CONCLUSIONS: Occupations involving the use of hydrocarbon solvents are a risk factor for earlier onset of symptoms of PD and more severe disease throughout its course. Hydrocarbon solvents may be involved in the etiopathogenesis of PD, which does not have a major genetic component.     

Single stage repair of symptomatic type IV thoracoabdominal aortic and iliac aneurysm in Behçet's disease. Case report

A 50-year-old man with Beh?et's disease (BD) diagnosed ten years previously, was submitted to emergency operation of two symptomatic type IV thoracoabdominal aortic and left common iliac aneurysms repair. Despite the rarity of vascular lesions in the course of BD, the uncommon clinical situation of double symptomatic aneurysms was successfully treated with surgical management that appears more difficult because of the inflammatory process associated with obliterative endoarteritis involving all periaortic tissues.     

Menorrhagia, hypermenorrhea and disorders of hemostasis

Menorrhagia is a common clinical problem. About 10% of women aged between 30 and 49 take medical advice for abnormal uterine bleeding. Underlying bleeding disorders are found in about 20% of women, once pelvic abnormalities are excluded. It is important to characterize a bleeding disorder since specific treatments can be provided. Moreover, these women are susceptible to present other haemorrhagic complications, particularly in case of invasive procedures. This review aims at drawing the attention a) on the importance of a careful medical history to assess menorrhagia and a possible bleeding disorder, b) on von Willebrand disease, the main coagulation disorder involved in case of menorrhagia and c) on several therapeutic possibilities.     

Mucoceles of the sphenoidal sinus: a report of four cases and review of the literature

Mucoceles of the sphenoidal sinus represent less than 2% of paranasal sinus mucoceles. The purpose of this paper is to report our experience in patients with mucoceles of the sphenoidal sinus. Four patients with this localization were observed in our department from 1994 to 2005. Symptoms included headache, visual loss, and rhinorrhea. All patients underwent computed tomography (CT) and magnetic resonance imaging (MRI). Surgery was performed using an endonasal endoscopic procedure, in one case a navigational system was used. In light of these observations and a review of literature, the etiopathogenic characteristics, clinical aspects, and therapeutic indications of this localization are discussed.