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 12,436 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic-isolated focal dystonia (IIFD) is a movement disorder characterised by involuntary, sustained muscle contractions, leading to abnormal postures. Psychopathology is frequent in patients with IIFD, and while traditionally this was thought to be a secondary phenomenon, there is emerging evidence for shared neurobiological mechanisms. We conducted a single-centre cross-sectional study of 103 consecutive patients with IIFD and two comparison groups: 78 consecutive patients with hemifacial spasm (HFS) and 93 healthy control subjects. Assessments with regard to psychiatric disturbances were performed using self-report questionnaires, including the self-report version of the Yale-Brown Obsessive Compulsive Scale (Y-BOCS-SR), the Beck Anxiety Inventory (BAI), and the Beck Depression Inventory (BDI). Compared to healthy control subjects and patients with HFS, the IIFD group had higher OCS, anxiety, and depression scores as measured by the Y-BOCS-SR, BAI, and BDI, respectively. The Y-BOCS-SR, BAI, and BDI were highly correlated across all the subjects. Logistic regression analysis showed that the main driver of high obsessive-compulsive symptom scores, irrespective of neurological diagnosis, was the BDI, whereas it was BAI (and not BDI), that drives the association between the psychiatric rating scale scores and the neurological diagnosis. Our findings suggest that while clinically significant obsessive-compulsive symptoms are over-represented in IIFD patients relative to controls, the BAI may have better discriminatory power to distinguish between the psychiatric symptoms in IIFD patients.
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PMID:Psychiatric disorders in idiopathic-isolated focal dystonia. 2444 65

In this review, we will work around two simple definitions of two different entities, which most often co-exist in patients with lesions to central motor pathways: Spasticity is "Enhanced excitability of velocity-dependent responses to phasic stretch at rest", which will not be the subject of this review, while Spastic dystonia is tonic, chronic, involuntary muscle contraction in the absence of any stretch or any voluntary command (Gracies, 2005). Spastic dystonia is a much less well understood entity that will be the subject this review. Denny-Brown (1966) observed involuntary sustained muscle activity in monkeys with lesions restricted to the motor cortices . He further observed that such involuntary muscle activity persisted following abolition of sensory input to the spinal cord and concluded that a central mechanism rather than exaggerated stretch reflex activity had to be involved. He coined the term spastic dystonia to describe this involuntary tonic activity in the context of otherwise exaggerated stretch reflexes. Sustained involuntary muscle activity in the absence of any stretch or any voluntary command contributes to burdensome and disabling body deformities in patients with spastic paresis. Yet, little has been done since Denny-Brown's studies to determine the pathophysiology of this non- stretch or effort related sustained involuntary muscle activity following motor lesions and there is a clear need for research studies in order to improve current therapy. The purpose of the present review is to discuss some of the possible mechanisms that may be involved in the hope that this may guide future research. We discuss the existence of persistent inward currents in spinal motoneurones and present the evidence that the channels involved may be upregulated following central motor lesions. We also discuss a possible contribution from alterations in synaptic inputs from surviving or abnormally branched sensory and descending fibres leading to over-activity and lack of motor coordination. We finally discuss evidence of alterations in motor cortical representational maps and basal ganglia lesions.
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PMID:On Denny-Brown's 'spastic dystonia' - What is it and what causes it? 2916 22

To establish outcomes following cochlear implantation (CI) in patients with postsynaptic auditory neuropathy (AN). Systematic review and narrative synthesis. Databases searched: MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 98 studies in total, of which 14 met the inclusion criteria reporting outcomes in 25 patients with at least 28 CIs. Of these, 4 studies focused on Charcot-Marie-Tooth disease (CMT), 3 on Brown-Vialetto-Van-Laere syndrome (BVVL), 2 on Friedreich Ataxia (FRDA), 2 on Syndromic dominant optic atrophy (DOA+), 2 on Cerebellar ataxia - areflexia - pes cavus - optic atrophy - sensorineural hearing loss (CAPOS) syndrome, and 1 on Deafness-dystonia-optic neuronopathy (DDON) syndrome. All studies were Oxford Centre for Evidence Based Medicine (OCEBM) grade IV. Overall trend was towards good post-CI outcomes with 22 of the total 25 patients displaying modest to significant benefit. Hearing outcomes following CI in postsynaptic ANs are variable but generally good with patients showing improvements in hearing thresholds and speech perception. In the future, development of a clearer stratification system into pre, post, and central AN would have clinical and academic benefits. Further research is required to understand AN pathophysiology and develop better diagnostic tools for more accurate identification of lesion sites. Multicenter longitudinal studies with standardized comprehensive outcome measures including health-related quality of life data will be key in establishing a better understanding of short and long-term post-CI outcomes.
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PMID:Cochlear Implantation Outcomes in Post Synaptic Auditory Neuropathies: A Systematic Review and Narrative Synthesis. 3313 25


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