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Projeto de investigação

REDEFINING RESTLESS LEGS SYNDROME

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A pilot study of botulinum toxin for jerky, position-specific, upper limb action tremor
Publication . Saifee, Tabish A.; Teodoro, Tiago; Erro, Roberto; Edwards, Mark J.; Cordivari, Carla
Background: We aimed to investigate the efficacy and safety of botulinum toxin (BT) injections for jerky action tremor of the upper limb. Methods: We performed an uncontrolled, prospective study of electromyography (EMG)-guided BT injections for jerky, position-specific, upper limb action tremor. The primary outcome was clinical global impression at 3-6 weeks after baseline. Results: Eight patients with jerky, position-specific action tremor involving the upper limb were consecutively recruited. After a median follow-up of 4.4 weeks (interquartile range [IQR] 3.6-6 weeks), four of them rated themselves as "improved" and two as "much improved." Five of these six subjects reported improvements in specific activities of daily living (bringing liquids to mouth, feeding, shaving, and dressing). Upper limb subscore of the Fahn-Tolosa-Marin Tremor Rating Scale (FTM) significantly decreased from 4.5 (4-6) to 3 (2-5) (p = 0.01). Discussion: This pilot, prospective cohort study suggests that EMG-guided BT injections may improve jerky, position-specific, upper limb action tremor. Placebo-controlled studies evaluating larger samples of patients are warranted to confirm these findings.
A peripheral pathway to restless legs syndrome? Clues from familial amyloid polyneuropathy
Publication . Teodoro, Tiago; Viana, Pedro; Abreu, Daisy; Conceição, isabel; Peralta, Ana; Ferreira, Joaquim J
Background: The relationship between restless legs syndrome (RLS) and peripheral neuropathy remains unclear. In order to clarify this relationship, we investigated if RLS is increased in familial amyloid polyneuropathy related to transthyretin (TTR-FAP) and investigated factors associated with RLS in this population. Methods: RLS frequency was compared between TTR-FAP patients and controls. Secondly, TTR-FAP patients with and without RLS were compared regarding demographic and clinical characteristics. Results: RLS frequency was significantly increased in TTR-FAP, with 18/98 (18.4%) cases contrasting with 5/104 (4.8%) controls (p-value 0.002). This difference remained significant after adjusting for confounders. In TTR-FAP patients, female sex (p-value 0.037), obesity (p-value 0.036) and weight excess (p-value 0.048) were associated with RLS, contrary to other classical RLS risk factors. Conclusions: RLS frequency is increased in TTR-FAP, thus supporting an association between RLS and neuropathy. This may represent a peripheral pathway in RLS pathogenesis. Furthermore, our results suggest that female sex and obesity/weight excess may be risk factors for RLS development among TTR-FAP patients.
A simplified version of the Psychogenic Movement Disorders Rating Scale: the Simplified Functional Movement Disorders Rating Scale (S-FMDRS)
Publication . Nielsen, Glenn; Ricciardi, Luciana; Meppelink, Anne Marthe; Holt, Kate; Teodoro, Tiago; Edwards, Mark
Background: The Psychogenic Movement Disorders Rating Scale (PMDRS) has potential as a useful objective assessment in clinical research, but the current scale has limitations. We developed a simplified version (S-FMDRS) and assessed inter-rater reliability, concurrent validity, and sensitivity. Methods: Fifty-two videos of subjects with functional (psychogenic) movement disorders (FMD) were rated according to the PMDRS and S-FMDRS by three neurologists. Inter-rater reliability was assessed using intraclass correlation coefficient (ICC). Agreement of symptomatic body regions and movement disorder classification was assessed using Light's kappa. Spearman's correlation coefficient was used to assess concurrent validity. A physiotherapist also rated videos on the S-FMDRS. The simplified scale was piloted in a feasibility study of physiotherapy for FMD to assess sensitivity. Results: ICC of total scores was 0.84 for the original scale and 0.85 for the simplified scale. Light's kappa for agreement of symptomatic body regions and movement disorder classification was moderate to low. Concurrent validity was demonstrated by Spearman's correlation between the two scales ranging from 0.84 to 0.95. The simplified scale was sensitive to change, with an effect size in the feasibility study of 0.79. Inter-rater reliability between physiotherapist and neurologist was high (ICC 0.85). Discussion: Both versions of the scale had good inter-rater reliability for the total score. Low agreement on movement disorder classification and identification of symptomatic body regions support our argument for a simplified scale. Conclusions: The S-FMDRS has high inter-rater reliability and good sensitivity to change. Further psychometric evaluation is warranted.

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Fundação para a Ciência e a Tecnologia

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Número da atribuição

SFRH/SINTD/95267/2013

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