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Piccininni M, Falsini C, Pizzi A. Quality of life in hereditary neuromuscular diseases. Acta Neurol Scand 2003 DOI: 10.1046/j.1600-0404.2003.00185.x © Blackwell Munksgaard 2003. The aim of the study is to evaluate different aspects in the quality of life (QoL) in a group of patients affected by hereditary neuromuscular disease. Forty-five consecutive outpatients (mean age 49.46 ± 17.07 years, range 19–80 years) with hereditary neuromuscular disease underwent the Brooke scale and functional independent measure to assess the functional status, the Sickness Impact Profile (SIP) and Psychological General Well-Being Index (PGWBI), as a measure of the QoL. All patients had a poor QoL assessment with SIP. The higher disability level was not related to a worse QoL perception with the exception of the physical area. Females and patients above 49 years showed the worst QoL profile. The discomfort linked to the emotional and affective sphere, assessed with PGWBI, appeared low in the study sample. Therefore psychosocial aspects and economical and environmental factors may influence the QoL. [ABSTRACT FROM AUTHOR]

The Fugl–Meyer Assessment is the most used and highly recommended clinical assessment of sensorimotor function after stroke. A standardized use of the scale in different countries requires translation and cultural validation to the target language. The objective of the study was to develop an official Italian version of the scale by transcultural translation and validation. A standardized multistep translation protocol was adopted to achieve optimal conceptual and semantic equivalence. The developed Italian version was validated in 10 post-stroke hemiparetic patients. Items with low intra- and interrater agreement, quantified as percentage of agreement <70% and/or statistically significant disagreement in relative position or concentration, between different raters were identified and revised. All motor items received a high level of agreement with values well above 70%. Disagreements were observed in 6 items in the sensory, joint range and pain domains and 1 in one reflex item. Items showing disagreements were discussed and revised to establish the final Italian version. The culturally validated Italian Fugl–Meyer Assessment can reliably be used in research and in clinical practice. A standardized use will improve the quality of sensorimotor assessment in stroke across Italy and allow reliable comparisons of stroke populations internationally. The Fugl–Meyer Assessment is the gold standard for evaluation of sensorimotor impairment after stroke. Having access to a transculturally validated official Italian version of Fugl–Meyer Assessment will improve the quality of sensorimotor assessment after stroke among Italian health professionals and researchers. A wider standardized use of the Fugl–Meyer Assessment in Italy will allow reliable international comparison of stroke rehabilitation outcomes. [ABSTRACT FROM AUTHOR]

Background and aims: Poststroke shoulder pain is a common complication. We aimed to investigate the prevalence of poststroke shoulder pain, with attention to the neuropathic component, and the relationship between poststroke shoulder pain and upper limb improvement in motor function, strength, disability, and quality of life after upper limb rehabilitation. Methods: This is a secondary analysis of a multicenter randomized controlled trial to compare upper limb conventional or robotic rehabilitation on 224 patients enrolled in eight rehabilitation centers. We assessed poststroke shoulder pain (using the Numerical Rating Scale and the Douleur Neuropathique 4), and upper limb motor function, strength, disability, and quality of life at baseline (T0), after 30 rehabilitation sessions (T1), and three months after the end of rehabilitation (T2). Results: A moderate/severe poststroke shoulder pain was reported by 28.9% of patients, while 19.6% of them showed a neuropathic component. At T0, the intensity of pain was higher in women and in patients with neglect syndrome, positively correlated with the time since stroke and disability and negatively correlated with motor function, strength, and the physical aspects of the quality of life. Moderate/severe pain and neuropathic component significantly reduced after both treatments and this reduction was maintained at T2. Finally, the intensity of pain at baseline was negatively correlated with the improvement of upper limb motor function. Conclusions: Poststroke shoulder pain negatively impact on motor performance, strength, disability, and physical aspects of the quality of life as well as on upper limb motor recovery; however, it can be reduced after a robotic or a conventional rehabilitation. Therefore, we suggest considering poststroke shoulder pain when planning the rehabilitation intervention. [ABSTRACT FROM AUTHOR]

Study design:Between-groups design with repeated measures.Objective:To quantify spastic hypertonia in spinal cord-injured (SCI) individuals.Setting:Rehabilitative Center, Italy.Subjects:29 individuals with a motor complete SCI (American Spinal Injury Association impairment scale grade A or B) and 22 controls.Methods:According to the modified Ashworth scale (MAS), patients were subgrouped as SCI-1 (MAS=1, 1+) and SCI-2 (MAS=2, 3). Passive flexo-extensions of the knee were applied using an isokinetic device (LIDO Active) at 30°, 60°, 90° and 120° s−1. We measured the peak torque, mean torque (MT) and work. Simultaneous electromyography (EMG) was recorded from leg muscles.Results:At the speed of 120° s−1 all SCI-2 patients presented EMG reflex activities in the hamstring muscle. All biomechanical parameter values increased significantly according to speed, but analysis of variance revealed a significant interaction between the angular velocity and group (F(d.f. 6, 138)=8.89, P<0.0001); post hoc analysis showed significantly greater torque parameter values in the SCI-2 group compared with the SCI-1 group and the control group at 90° and 120° s−1. Receiver operating characteristic curves showed that using peak torque values the probability of correctly classifying a patient into SCI-1 and SCI-2 was 95%, compared with 70% for MT and 68% for work.Conclusions:The isokinetic device is useful for distinguishing individuals with a high level of spastic hypertonus. Examination of EMG activity may help ascertain whether increased muscle tone is caused by reflex hyper excitability and to determine whether muscle spasm is present. Peak torque and simultaneous EMG assessment should be considered for the evaluation of individuals with SCI in the rehabilitative context, that is, in measuring therapeutic interventions. [ABSTRACT FROM AUTHOR]

About 50% of post-stroke patients remain aphasic a condition that greatly impede their reintegration to society. Results of Repetitive Transcranial Magnetic Stimulation (rTMS) as supplementary treatment for sub-acute post-stroke aphasia are controversial. We assessed the efficacy of inhibitory rTMS with conventional speech therapy to help recovery language performance in sub-acute stroke aphasia.In a randomised case-control double-blind study 12 non-fluent aphasic were consecutively enrolled. All patients suffered a first-ever stroke in the sub-acute stage defined as time since lesion onset from 1 month post-stroke. Patients were randomized either to receive real (low frequency on right-hemisphere homologue of Broca’s area) or sham rTMS, to a 3-week (5 day in a week). Immediately after each rTMS all patients received speech therapy. The speech performance was evaluated with Italian version of Aachener Aphasia Test (AAT), before (T0) and after (T1) treatments.Seven patients were treated with real rTMS and 5 patients with sham rTMS. Total and sub totals AAT scores showed a similar improvement in real and in sham rTMS groups between T0 and T1.In early phase of rehabilitation (< 1 month) inhibitory rTMS over the right posterior inferior frontal gyrus, in combination with speech therapy, did not seem significantly to improve language recovery respect to conventional speech therapy only. [ABSTRACT FROM AUTHOR]

The purpose of this study was to assess the beneficial effect of BTX- A in the treatment of hemiplegic shoulder pain in chronic phase. 31 consecutive hemiplegic patients with different degree of hypertonus (groups 1 and 2) of the muscles of the painful shoulder, referred to 5 Rehabilitation Centers and underwent to neuromuscular blockade with pure BTX-A (150 Kd). The rating scales were: the FAT, the MAS, the pROM, the MRC and the VAS at rest and during nursing. Evaluations were performed at enrollment (T0), after 1month (T1) and after 3months post-treatment with BTX (T3). At T1 It has been observed a statistically significant reduction in VAS at rest and during nursing (p <0.0001);in the MAS (p =0.04) and passive ROM (p <0.0001) at the shoulder level, without further changes at T3. At T2 reappearance of hypertonus without pain. The degree of pain reduction was similar in both groups of patients with different degrees of spasticity. There were no significant changes obtained with regard to the MRC and FAT. Our data suggest a role of BTX in reducing shoulder pain through an antinociceptive mechanism independent from the level of spasticity. [Copyright &y& Elsevier]

Botulinum toxin type A (BoNT/A) provides a valuable tool in the multi-modal treatment of spasticity. Incobotulinumtoxin A (InBoNT/A) is a highly purified BoNT/A agent, free of any complexing clostridial proteins.Clinical experience suggests that InBoNT/A is identical from efficacy, duration of therapeutic effect and tolerability profile. Our aim was to compare the effect of the CBoNT/A vs InBoNT/A on spasticity of the upper limb, evaluated with Modificated Aswhort Scale (MAS), and on amplitude of compound motor action potentials (CMAP) of biceps muscle in patients after brain stroke. A randomized, double-blind, parallel-group pilot single-centre study. Clinical and neurophysiological evaluation were performed at enrollment (T0), to 24h (T1),one (T2) and three (T3) months from toxin injection. We recruited 9 subject. Both BoNTA reduced MAS scores but with no statistically significant difference. At T1 patients treated with IBoNT/A showed a greater reduction in CMAP compared with those treated with CBoNT/A. This difference is reduced at T2 but is again evident at T3. These data seem to indicate a tendency of IBoNT/A to present a more precocious and a longer duration effect respect to CBoNT/A. The low power of the statistical analysis, however, suggests to recruit a larger sample of patients. [Copyright &y& Elsevier]