Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Introduction: Patients suffering from bilateral vestibular hypofunction (BVH) often experience ataxia as well as visual instability. Even though progress has been made in vestibular testing, insights regarding vestibular deficit in BVH remain incomplete since no method allows evaluation of frequency ranges of vestibular sensors in a continuous way. The aim of our study was to give a detailed description of the level of vestibular deficit in different ranges of vestibular stimulation and an exhaustive evaluation of the functional impact including dynamic visual acuity (DVA) in a cohort of BVH patients in different etiologies. Methods: We prospectively included 20 patients with chronic BVH. All patients underwent clinical evaluation and functional assessment including evaluation of their symptoms related to BVH, quality of life questionnaire and DVA in the horizontal and vertical plane. Patients underwent vestibulo-ocular reflex (VOR) testing using rotatory chair, caloric stimulation and video head impulse (vHIT) in the plane of the 6 canals, and cervical and ocular Vestibular evoked myogenic potentials. Results: Mean rotatory VOR gain was 0.07 (SD = 0.07). Mean rotatory VOR gain during vHIT for the lateral, anterior and posterior canals was respectively < 0.28, < 0.34, and < 0.20. Mean loss of DVA in the 4 directions was >0.30 LogMAR. In our population fall frequency was significantly higher in patients with lower UniPedal Stance Test (UPST), higher Dizziness Handicap Inventory and Ataxia Numeric Scale (ANS) scores, as well as greater loss of upwards DVA. Patients with ototoxic BVH had a significantly higher residual VOR gain during vHIT in the anterior canal plane and lower DHI than other patients. In the general population anterior canal function was significantly higher than lateral or posterior canal function. Conclusions: This study gives extensive descriptive results of residual vestibular function, DVA and quality of life in a population of patients suffering from severe BVH. UPST and ANS are good indicators for fall risk in case of BVH. Gentamicin induced BVH seems to have a lesser impact on quality of life than other etiologies.Anterior semi-circular canal function seems less deteriorated than lateral and posterior function.
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PMID:Bilateral Vestibulopathy: Vestibular Function, Dynamic Visual Acuity and Functional Impact. 3004 25

There are no currently available disease-modifying pharmacological treatments for most of the chronic hereditary ataxias; thus, effective rehabilitative strategies are crucial to help improve symptoms and therefore the quality of life. We propose to gather all available evidence on the use of video games, exergames, and apps for tablet and smartphone for the rehabilitation, diagnosis, and assessment of people with ataxias. Relevant literature published up to June 8, 2020, was retrieved searching the databases PubMed, ISI Web of Science, and the Cochrane Database. Data were extracted using a standardized form, and their methodological quality was assessed using RoB and QUADAS-2. Six studies of 434 retrieved articles met the predefined inclusion/exclusion criteria. Two of them were diagnostic, while 4 were experimental studies. Studies included participants ranging from 9 to 28 in trials and 70 to 248 in diagnostic studies. Although we found a small number of trials and of low methodological quality, all of them reported an improvement of motor outcomes and quality of life as measured by specific scales, including the SARA, BBS, DHI, and SF-36 scores. The main reason for such low quality in trials was that most of them were small and uncontrolled, thus non-randomized and unblinded. As video games, exergames, serious games, and apps were proven to be safe, feasible, and at least as effective as traditional rehabilitation, further and more high-quality studies should be carried out on the use of these promising technologies in people with different types of ataxia.
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PMID:The Use of New Mobile and Gaming Technologies for the Assessment and Rehabilitation of People with Ataxia: a Systematic Review and Meta-analysis. 3319 Jan 89