Spastic paraplegia 15: Linkage and clinical description of three Tunisian families

Hereditary spastic paraplegias (HSP) are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by slowly progressive spasticity of the lower limbs. The locus designated spastic paraplegia 15 (SPG15), located in a 16‐Mb interval on chromosome 14q, is associated with a rare autosomal recessive complicated form of HSP known as Kjellin's syndrome. In this study, we describe three additional families, of Tunisian origin, linked to the SPG15 locus, one of which had a significant multipoint LOD score of 3.46. In accordance with previous reports, the phenotype of our patients consisted of early onset spastic paraparesis associated with mental impairment and severe progression. Retinal degeneration was not observed, however, but we extended the phenotype of this form to include peripheral neuropathy and white matter abnormalities on MRI. Interestingly, like retinal degeneration, thin corpus callosum is not a constant feature in this entity. © 2007 Movement Disorder Society     

Effect of piroxicam on the blood-retina barrier in experimentally induced diabetes in rats

The effect of piroxicam on the blood-retina barrier was evaluated in rats with experimentally induced diabetes. Diabetes was induced in rats by intraperitoneal injection of streptozocin (STZ). Diabetic rats were divided into two equal groups: those treated with piroxicam, a long-acting platelet inhibitor, and an untreated control group. Vitreous fluorophotometry (VFP) was performed both before and two weeks after induction of diabetes and piroxicam intake. Streptozocin-induced diabetes caused an alteration in the blood-retinal barrier evidenced by an increase in vitreous fluorescein concentration in diabetic rats compared with normal rats. Piroxicam intake did not lead to significant change in vitreous fluorescein concentrations. However, the examination had to be terminated at two weeks because of cataract formation. The piroxicam treated group showed less incidence of lens opacity formation (59.1% compared to 81.8% in the untreated group, p = 0.0006). Piroxicam administration appears to protect the diabetic rat eye against lens opacification.This work was supported in part by U.S. Public Health Service Grants EY02377, EY07541 and EY08137 from the National Eye Institute, National Institutes of Health, Bethesda, MD and by the Juvenile Diabetes Foundation International and Pfizer, Inc.     

Evaluation of the 24-hour blood pressure effects of eprosartan in patients with systemic hypertension

BACKGROUND: Eprosartan is a new nonphenyl angiotensin II receptor blocker, which has been approved for the treatment of hypertension. Although the drug has a relatively short plasma half-life of 5 to 9 h, clinical studies have suggested that its antihypertensive effect persists for 24 h. METHODS: We assessed both the changes in 24-h and trough blood pressure (BP) (last 4 h of the ambulatory BP while the patient was awake) of eprosartan at doses of 600 and 1,200 mg once daily in a randomized, double-blind, placebo-controlled trial. Ambulatory BP was monitored at placebo baseline and after 8 weeks of double-blind therapy. RESULTS: Two hundred patients randomized in the study with 177 patients completing the trial. The 24-h change in BP from baseline was 0.2/0.1 +/- 1.4/1.0 mm Hg, -7.9/ -5.4 +/- 1.0 mm Hg (P < .0001), and -7.4/-5.0 +/- 0.9 mm Hg (P < .0001) in the placebo, 600-mg eprosartan, and 1,200-mg eprosartan groups, respectively. Changes in trough ambulatory BP showed significant reductions of -6.3/-4.1 +/- 1.6/1.1 mm Hg and -7.7/-5.5 +/- 1.5/1.0 mm Hg for 600 mg of eprosartan and 1,200 mg of eprosartan, respectively. CONCLUSIONS: These data demonstrate that eprosartan at doses of 600 or 1200 mg significantly reduced BP throughout an entire 24-h dosing period. There were no differences between the 600- and 1,200-mg dose; thus, 600 mg once daily should be the only dose used in the treatment of hypertension with eprosartan.     

Blepharitis due to Demodex: myth or reality?]

PURPOSE: Demodex folliculorum has been incriminated in the development of blepharitis although much controversy persists. Certain authors suggest that Demodex is a direct pathogen in chronic palpebral conditions while others consider the saprophyte to be innocuous to skin. METHODS: We conducted a prospective study of eyelashes in 100 persons, searching for Demodex folliculorum and chronic blepharitis. Microscopy in immersion oil after storage in a moist chamber was performed. RESULTS: The incidence of Demodex folliculorum was very high in patients with blepharitis compared with normal controls. Incidence increased with age. Harmless cuffs around the base of the eyelashes was found in 4% with Demodex irradior. CONCLUSION: Demodex should be considered as the cause of chronic blepharitis. Anti-Demodex treatment is indicated when the parasite is found.     

The North American Opiate Medication Initiative (NAOMI): Profile of Participants in North America’s First Trial of Heroin-Assisted Treatment

The North American Opiate Medication Initiative (NAOMI) is a randomized controlled trial evaluating the feasibility and effectiveness of heroin-assisted treatment (HAT) in the Canadian context. Our objective is to analyze the profile of the NAOMI participant cohort in the context of illicit opioid use in Canada and to evaluate its comparability with patient profiles of European HAT studies. Recruitment began in February 2005 and ended in March 2007. Inclusion criteria included opioid dependence, 5 or more years of opioid use, regular opioid injection, and at least two previous opiate addiction treatment attempts. Standardized assessment instruments such as the European Addiction Severity Index and the Maudsley Addiction Profile were employed. A total of 251 individuals were randomized from Vancouver, BC (192, 76.5%), and Montreal, Quebec (59, 23.5%); 38.5% were female, the mean age was 39.7 years (SD:8.6), and participants had injected drugs for 16.5 years (SD:9.9), on average. In the prior month, heroin was used a mean of 26.5 days (SD:7.4) and cocaine 16 days (SD;12.6). Vancouver had significantly more patients residing in unstable housing (88.5 vs. 22%; p < 0.001) and higher use of smoked crack cocaine (16.9 days vs. 2.3 days in the prior month; p < 0.001), while a significantly higher proportion of Montreal participants reported needle sharing in the prior 6 months (25% vs. 3.7%; p < 0.001). In many respects, the patient cohort was similar to the European trials; however, NAOMI had a higher proportion of female participants and participants residing in unstable housing. This study suggests that the NAOMI study successfully recruited participants with a profile indicated for HAT. It also raises concern about the high levels of crack cocaine use and social marginalization. Oviedo-Joekes, Marsh, Anis, and Schechter are with the School of Population and Public Health, University of British Columbia, Vancouver, BC, Canada; Oviedo-Joekes, Nosyk, Chettiar, Marsh, Krausz, Anis, and Schechter are with the Centre for Health Evaluation and Outcome Sciences, Providence Health Care, Vancouver, BC, Canada; Brissette and Schneeberger are with the Centre de recherche du l’Université de Montréal, Montreal, QC, Canada; Marsh and Krausz are with the Department of Psychiatry, University of British Columbia, Vancouver, BC, Canada; Marsh is with the Vancouver Coastal Health, Vancouver, BC, Canada; Marsh is with the Centre for Addiction Research British Columbia, Vancouver, BC, Canada.     

Cisatracurium in a myasthénic patient undergoing thymectomy

PURPOSE: The report investigates cisatracurium neuromuscular block in a myasthenic patient undergoing thymectomy. CLINICAL FEATURES: A myasthenic patient (Osserman II B) was prepared preoperatively with 240 mg x day(-1) pyridostigmine. The neuromuscular block produced by 0.05 mg x kg(-1) cisatracurium was monitored by Datex electromyography. The electromyographic response was compared with that in a control group of five non-myasthenic patients. In the myasthenic patient, cisatracurium resulted in a rapid onset of complete (97-98%) neuromuscular block, while a slow onset of partial (80-90%) block was achieved in the control group. Also, administration of 0.05 mg x kg(-1) neostigmine at the end of surgery reversed the neuromuscular block of cisatracurium in the non-myasthenic patients, but did not change the rate of spontaneous recovery in the myasthenic patient. CONCLUSION: The myasthenic patient is sensitive to cisatracurium, as evidenced by a more rapid onset and more marked neuromuscular block compared with the control non-myasthenic patients. This may be attributed to the decreased number of functional endplate acetylcholine receptors in the myasthenic patient, with a consequent decrease of the safety margin of neuromuscular transmission. Also, in contrast with the control group, the rate of recovery from neuromuscular block in the myasthenic patient was not enhanced by neostigmine at the end of surgery. This may be attributed to the prior inhibition of acetylcholinesterase by the preoperative pyridostigmine, as well as by possible desensitization of the cholinergic receptors secondary to prolonged pyridostigmine therapy.