Dexmedetomidine improves success of paediatric MRI sedation

OBJECTIVE: To improve success rates of children requiring sedation for MRI. METHODS: Audits of sedation success for children attending planned MRI using three different approaches: (1) National Institute for Health and Care Excellence (NICE) guidance (chloral hydrate if <15 kg and oral midazolam if ≥15 kg), (2) Chloral hydrate for all patients, (3) Chloral hydrate±intranasal dexmedetomidine if <15 kg and intranasal dexmedetomidine alone if ≥15 kg. RESULTS: 74 patients had 85 MRI scan attempts. Overall success rates were significantly higher when using intranasal dexmedetomidine compared with following NICE guidance (81% vs 52% p=0.017). Dexmedetomidine performed better than oral midazolam for the same indication (76% vs 33% p=0.026). The side effect profile for dexmedetomidine was as reported in larger studies. CONCLUSIONS: Intranasal dexmedetomidine is an effective alternative to oral midazolam for sedation for MRI and as a rescue medication where chloral hydrate has been ineffective

Evaluation of speed-accuracy trade-off in a computer task in individuals with cerebral palsy: a cross-sectional study

Background. Individuals with Cerebral Palsy (CP) present with sensorimotor dysfunction which make the control and execution of movements difficult. This study aimed to verify the speed-accuracy trade-off in individuals with CP. Methods. Forty eight individuals with CP and 48 with typical development (TD) were evaluated (32 females and 64 males with a mean age of 15.02 ± 6.37 years: minimum 7 and maximum 30 years). Participants performed the “Fitts’ Reciprocal Aiming Task v.1.0 (Horizontal)” on a computer with different sizes and distance targets, composed by progressive indices of difficulty (IDs): ID2, ID4a and ID4b. Results. There were no statistical differences between the groups in relation to the slope of the curve (b1) and dispersion of the movement time (r2). However, the intercept (b0) values presented significant differences (F(1.95) = 11.3; p = .001]), with greater movement time in the CP group compared to the TD group. It means that for individuals with CP, regardless of index difficulty, found the task more difficult than for TD participants. Considering CP and TD groups, speed-accuracy trade-off was found when using different indices of difficulty (ID2 and ID4). However, when the same index of difficulty was used with a larger target and longer distance (ID4a) or with a narrow target and shorter distance (ID4b), only individuals with CP had more difficulty performing the tasks involving smaller targets. Marginally significant inverse correlations were identified between the values of b1 and age (r = −0.119, p = .052) and between r2 and Gross Motor Function Classification System (r = −0.280, p = .054), which did not occur with the Manual Ability Classification System. Conclusion. We conclude that the individuals with CP presented greater difficulty when the target was smaller and demanded more accuracy, and less difficulty when the task demanded speed. It is suggested that treatments should target tasks with accuracy demands, that could help in daily life tasks, since it is an element that is generally not considered by professionals during therapy

A systematic review of the reporting of tinnitus prevalence and severity

Introduction There is no standard diagnostic criterion for tinnitus, although some clinical assessment instruments do exist for identifying patient complaints. Within epidemiological studies the presence of tinnitus is determined primarily by self-report, typically in response to a single question. Using these methods prevalence figures vary widely. Given the variety of published estimates worldwide, we assessed and collated published prevalence estimates of tinnitus and tinnitus severity, creating a narrative synthesis of the data. The variability between prevalence estimates was investigated in order to determine any barriers to data synthesis and to identify reasons for heterogeneity. Methods and analysis: A systematic review included all adult population studies reporting the prevalence of tinnitus from January 1980 to July 2015. We searched five databases (Embase, Medline, PsychInfo, CINAHL and Web Of Science), using a combination of medical subject headings (MeSH) and relevant text words. Observational studies including cross-sectional studies were included, but studies estimating the incidence of tinnitus (e.g. cohort studies) were outside the scope of this systematic review. Results The databases identified 875 papers and a further 16 were identified through manual searching. After duplicates were removed, 515 remained. On the basis of the title, abstract and full-text screening, 400, 48 and 27 papers respectively were removed. This left 40 papers, reporting 39 different studies, for data extraction. Sixteen countries were represented, with the majority of the studies from the European region (38.5%). Publications since 2010 represented half of all included studies (48.7%). Overall prevalence figures for each study ranged from 5.1% to 42.7%. For the 12 studies that used the same definition of tinnitus, prevalence ranged from 11.9% to 30.3%. Twenty-six studies (66.7%) reported tinnitus prevalence by different age groups, and generally showed an increase in prevalence as age increases. Half the studies reported tinnitus prevalence by gender. The pattern generally showed higher tinnitus prevalence among males than females. There were 8 different types of definitions of tinnitus, the most common being “tinnitus lasting for more than five minutes at a time” (34.3%). Only seven studies gave any justification for the question that was used, or acknowledged the lack of standard questions for tinnitus. There is widespread inconsistency in defining and reporting tinnitus, leading to variability in prevalence estimates among studies. Nearly half of the included studies had a high risk of bias and this limits the generalisability of prevalence estimates. In addition, the available prevalence data is heterogeneous thereby preventing the ability to pool the data and perform meta-analyses. Sources of heterogeneity include different diagnostic criteria, different age groups, different study focus and differences in reporting and analysis of the results. Heterogeneity thus made comparison across studies impracticable. Conclusion Deriving global estimates of the prevalence of tinnitus involves combining results from studies which are consistent in their definition and measurement of tinnitus, survey methodology and in the reporting and analysis of the results. Ultimately comparison among studies is unachievable without such consistency. The strength of this systematic review is in providing a record of all the available, recent epidemiological data in each global region and in making recommendations for promoting standardisation

Case Reports1. A Late Presentation of Loeys-Dietz Syndrome: Beware of TGFβ Receptor Mutations in Benign Joint Hypermobility

Background: Thoracic aortic aneurysms (TAA) and dissections are not uncommon causes of sudden death in young adults. Loeys-Dietz syndrome (LDS) is a rare, recently described, autosomal dominant, connective tissue disease characterized by aggressive arterial aneurysms, resulting from mutations in the transforming growth factor beta (TGFβ) receptor genes TGFBR1 and TGFBR2. Mean age at death is 26.1 years, most often due to aortic dissection. We report an unusually late presentation of LDS, diagnosed following elective surgery in a female with a long history of joint hypermobility. Methods: A 51-year-old Caucasian lady complained of chest pain and headache following a dural leak from spinal anaesthesia for an elective ankle arthroscopy. CT scan and echocardiography demonstrated a dilated aortic root and significant aortic regurgitation. MRA demonstrated aortic tortuosity, an infrarenal aortic aneurysm and aneurysms in the left renal and right internal mammary arteries. She underwent aortic root repair and aortic valve replacement. She had a background of long-standing joint pains secondary to hypermobility, easy bruising, unusual fracture susceptibility and mild bronchiectasis. She had one healthy child age 32, after which she suffered a uterine prolapse. Examination revealed mild Marfanoid features. Uvula, skin and ophthalmological examination was normal. Results: Fibrillin-1 testing for Marfan syndrome (MFS) was negative. Detection of a c.1270G > C (p.Gly424Arg) TGFBR2 mutation confirmed the diagnosis of LDS. Losartan was started for vascular protection. Conclusions: LDS is a severe inherited vasculopathy that usually presents in childhood. It is characterized by aortic root dilatation and ascending aneurysms. There is a higher risk of aortic dissection compared with MFS. Clinical features overlap with MFS and Ehlers Danlos syndrome Type IV, but differentiating dysmorphogenic features include ocular hypertelorism, bifid uvula and cleft palate. Echocardiography and MRA or CT scanning from head to pelvis is recommended to establish the extent of vascular involvement. Management involves early surgical intervention, including early valve-sparing aortic root replacement, genetic counselling and close monitoring in pregnancy. Despite being caused by loss of function mutations in either TGFβ receptor, paradoxical activation of TGFβ signalling is seen, suggesting that TGFβ antagonism may confer disease modifying effects similar to those observed in MFS. TGFβ antagonism can be achieved with angiotensin antagonists, such as Losartan, which is able to delay aortic aneurysm development in preclinical models and in patients with MFS. Our case emphasizes the importance of timely recognition of vasculopathy syndromes in patients with hypermobility and the need for early surgical intervention. It also highlights their heterogeneity and the potential for late presentation. Disclosures: The authors have declared no conflicts of interes

Traumatic brain injury: integrated approaches to improve prevention, clinical care, and research

No abstract available

Increased frequency of alpha-synuclein in the substantia nigra in human immunodeficiency virus infection.

The frequency of neurodegenerative markers among long surviving human immunodeficiency virus (HIV)-infected individuals is unknown, therefore, the present study investigated the frequency of alpha-synuclein, beta-amyloid, and HIV-associated brain pathology in the brains of older HIV-infected individuals. We examined the substantia nigra of 73 clinically well-characterized HIV-infected individuals aged 50 to 76 years from the National NeuroAIDS Tissue Consortium. We also examined the frontal and temporal cortical regions of a subset of 36 individuals. Neuritic alpha-synuclein expression was found in 16% (12/73) of the substantia nigra of the HIV+cases and none of the older control cases (0/18). beta-Amyloid deposits were prevalent and found in nearly all of the HIV+cases (35/36). Despite these increases of degenerative pathology, HIV-associated brain pathology was present in only 10% of cases. Among older HIV+adults, HIV-associated brain pathology does not appear elevated; however, the frequency of both alpha-synuclein and beta-amyloid is higher than that found in older healthy persons. The increased prevalence of alpha-synuclein and beta-amyloid in the brains of older HIV-infected individuals may predict an increased risk of developing neurodegenerative disease