Search

Background and purpose Rehabilitation plays a central role in stroke recovery. Besides conventional therapy, technological treatments have become available. The effectiveness and appropriateness of technological rehabilitation are not yet well defined; hence, research focused on different variables impacting recovery is needed. Results from the literature identified cognitive reserve (CR) as a variable impacting on the cognitive outcome. In this paper, the aim was to evaluate whether CR influences the motor outcome in patients after stroke treated with conventional or robotic therapy and whether it may influence one treatment rather than another. Methods Seventy-five stroke patients were enrolled in five Italian neurological rehabilitation centres. Patients were assigned either to a robotic group, rehabilitation by means of robotic devices, or to a conventional group, where a traditional approach was used. Patients were evaluated at baseline and after rehabilitation treatment of 6 weeks through the Action Research Arm Test (ARAT), the Motricity Index (MI) and the Barthel Index (BI). CR was assessed at baseline using the Cognitive Reserve Index (CRI) questionnaire. Results Considering all patients, a weak correlation was found between the CRI related to leisure time and MI evolution (r = 0.276; P = 0.02). Amongst the patients who performed a robotic rehabilitation, a moderate correlation emerged between the CRI related to working activities and MI evolution (r = 0.422; P = 0.02). Conclusions Our results suggest that CR may influence the motor outcome. For each patient, CR and its subcategories should be considered in the choice between conventional and robotic treatment.

Background and purpose Charcot−Marie−Tooth (CMT) disease is the most common inherited neuropathy, but therapeutic options have been limited to symptom management. Past pharmacological trials have failed, possibly due to insensitive outcome measures (OMs). The aim of the current study was to evaluate the validity and reliability of the 6-min walk test (6MWT) and StepWatch™ Activity Monitoring (SAM) with other previously validated OMs in CMT disease. Methods A prospective multicenter study was performed, consecutively enrolling 168 CMT patients (104 with CMT1A, 27 with CMT1B, 37 with X-linked CMT) from Italian centers specializing in CMT care. Results Statistical analysis showed that the 6MWT was highly related with all previously used OMs. Some, but not all, SAM parameters were related to commonly used OMs but may provide more information about quality of life. Conclusions The current study demonstrated the validity and reliability of the 6MWT and SAM as OMs for CMT. Moreover, SAM provides data that correlate better with quality of life measures, making it useful in future rehabilitation trials.

Charcot Marie Tooth (CMT) disease is the most common inherited polyneuropathy. At the moment there is no pharmacological therapy for this pathology and the conservative treatment is mostly based on rehabilitation program. Moreover there is no medical consensus on it and the perception of its efficacy is mostly clinician-oriented.To evaluate, through ad hoc self-administered questionnaires, the patient and family/caregiver perspective on rehabilitation access and perceived benefit from it.Observational survey study.Clinical and genetic records of in and out-patients of third level hospitals and a patients association (ACMT-Rete) and familiar/caregiver.Patients affected by CMT and familiar/caregiver.Questionnaires showed that patients perceive physical and mental benefit from rehabilitation, but also perceived that do not perform the best rehabilitation program for their pathology. Familiar and caregiver, are not sure that rehabilitation is effective for their kin, probably because the benefits are too small to be seen by someone other than the patient.The study shows as the lack of a consensus on rehabilitation tailored on CMT patients need is perceived by patients and familiar/caregiver.The knowledge of patients perception is very important in order to obtain the best rehabilitation program for CMT disease.

Highlights ► Charcot-Marie-Tooth 1A cannot rely on effective pharmacological treatment. ► Several human trials failed to provide evidence of a therapeutic role of vitamin C. ► Human vitamin C's pharmacokinetics are tightly regulated. ► Ascorbic acid should not be given in excess of its transport system capacity., Charcot–Marie–Tooth 1A disease (CMT1A) is a disease for which no drug treatments are available. In 2004, it was reported that ascorbic acid reduced the severity of neuropathy in transgenic mice overexpressing PMP22, an animal model of human CMT1A, compared with untreated mice. Based on those results, clinical trials were undertaken at different centers worldwide and four of them have been completed, but none of them resulted in significant improvements. Based on the pharmacokinetics of ascorbic acid, we propose that the randomized clinical trial carried out thus far confirmed the tight control of ascorbic acid's absorption and proved its tolerability at one and two years. The pharmacokinetic considerations discussed in this article might largely explain the disappointing results of the recent CMT1A trials.

Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia whose main neurological feature is a demyelinating polyneuropathy. The aim of our study was to assess the pattern of ultrasound (US) nerve abnormalities in POEMS syndrome patients.Eight POEMS syndrome patients underwent neurological examination and US evaluation of the median, ulnar, fibular, tibial, and sural nerves. Nerve cross-sectional area and echogenicity abnormalities were analyzed.US abnormalities were mostly localized at entrapment sites. Enlargements outside the entrapment sites were uncommon. No correlation was found between muscle weakness and focal US findings.No specific pattern of US abnormalities was identified in this cohort of patients with POEMS syndrome. The lack of correlation between US and clinical findings may be secondary to the chronic nerve damage that is common in POEMS syndrome, where the diagnosis is often delayed.

The main objective of this study is to assess the course of peroneal mononeuropathy (PM). The study design includes Clinical and Prospective study. The setting involves neurophysiological Service. From November 2002 to January 2004, we enroled 69 consecutive patients and prospectively followed up 49 patients with multiple measurements. Comparison was made between follow-up and baseline values, and baseline factors were used to predict the PM evolution in multiple regression analysis. At follow-up, we observed a significant improvement of all clinical, neurophysiological and disability measurements, and physical aspects of quality of life (QoL). Greater muscle strength of tibialis anterior and higher conduction velocity of peroneal nerve at baseline were seen to be positive prognostic factors. A better evolution of mental aspects of QoL was observed in the subacute group and in younger patients, while a better physical evolution in QoL was observed in women. Rehabilitation is ambiguously associated with a better Deambulation Index but lower ratings in mental aspects of QoL. In conclusion, PM shows a positive spontaneous course and rehabilitation seems to help the recovery of deambulation. Further studies on the effects of conventional rehabilitation are needed.

To study the Health Related Quality of Life (HRQoL) and metabolic assessment in 33 children affected with type 1 diabetes (18 males, 15 females; mean age 10.3 years).We used the Child Health Questionnaire-Parental Form 50 items (CHQ-PF50), measurements of metabolic control and we related them to patient management and family status. Quality of life (QoL) in diabetic children was worse than in the healthy sample. Interestingly, mean and last glycosylated hemoglobin (mean HbAlc r: -.4410 p.01 and last HbAlc r: -.4012 p.01), age of patients (r: -.4428; p.009) and number of glycaemia controls (r: -.37, p.03) were the most important parameters related to HRQoL parameters.This multidimensional study stressed that HRQoL is influenced by the metabolic assessment. Moreover, the report examined the parental perception of QoL in children with chronic diseases. Higher number of glycaemia controls/day, better metabolic control, lower age of children and earlier onset of diabetes produced better physical and psychological aspects of QoL. In comparison with adolescent patients, in children with diabetes, factors as number of insulin injections and daily snacks, and the level of education of the mother were not so important to influence QoL. Unexpectedly, in this sample, life habits, family features, and anthropometric parameters did not correlate with specific domains of QoL.

417

To our knowledge there is no evidence of multientrapment neuropathy related to growth hormone (GH) administration. We observed the case of a 45-year-old bodybuilder complaining of paresthesias and dysesthesias of the hands and feet after self-administration of high-dose of recombinant GH therapy. The patient complained of transitory symptoms after each cycle of GH therapy. The electrodiagnosis showed: 1) moderate right carpal tunnel syndrome (CTS) and minimal left CTS; 2) mild right ulnar entrapment at elbow; 3) a reduction of motor nerve conduction velocity of the left ulnar nerve across elbow; 4) a reduction of the amplitude of the sensory nerve action potential of the left ulnar nerve in digit-wrist segment; 5) the absence of the ulnar F wave bilaterally registering from abductor digiti minimus. Our data suggest a multiple entrapment condition of the upper limbs; furthermore, based on the history (paresthesia at feet), we can hypothesize a mild involvement of distal sensory fibres in the legs (neurophysiologically negative). The anecdotal history and neurophysiological findings of this case suggest an increased awareness to the adverse effects of GH on peripheral nerve.

Pain may affect all aspects of social life and reduce the quality of life. Neuropathic pain (NP) is common in patients affected by plexopathy, radiculopathy, mononeuropathy, peripheral neuropathy. Phantom limb pain (PLP) is a painful sensation that is common after amputation, and its pathophysiological mechanisms involve changes in the peripheral and central nervous system. Given the lack of conclusive evidence and specific guidelines on these topics, the aim of the Italian Consensus Conference on Pain on Neurorehabilitation (ICCPN) was to collect evidence and offer recommendations to answer currently open questions on the assessment and treatment of NP associated with the above conditions and PLP. When no evidence was available, recommendations were based on consensus between expert opinions. Current guidelines on the assessment and pharmacological treatment of NP can be applied to plexopathy, radiculopathy, mononeuropathy, peripheral neuropathy, while evidence for invasive treatments and physical therapy is generally poor because of the low quality of studies. Treatment of PLP is still unsatisfactory. Data on the functional outcome and impact of pain on neurorehabilitation outcome in these conditions are lacking. In most cases, a multidisciplinary approach is recommended to offer a better outcome and reduce side effects. High quality studies are requested to address the unmet needs in this field.

Objectives The incidence of chronic postoperative pain after total knee replacement (TKR) is approx. 20%, and hence preoperative risk factors are important to identify. Recent studies have indicated that preoperative inflammatory markers might hold prognostic information for the development of chronic postoperative pain. Long non-coding RNA (lncRNA) regulates the expression of genes related to e.g. inflammatory processes. The current study aimed to investigate the preoperative lncRNA signature as possible preoperative predictive markers for chronic postoperative pain following TKR. Methods Serum samples, collected preoperatively from 20 knee osteoarthritis (KOA) patients, were analyzed for 84 validated circulatory lncRNA. Pain intensity was assessed using a visual analog scale (VAS) before and one-year after TKR. Differences for the lncRNA expression were analyzed between patients with chronic postoperative pain (VAS≥3) and those with a normal postoperative recovery (VAS Results LncRNA Myeloid Zinc Finger 1 Antisense RNA 1 (MZF1-AS1) (fold change −3.99; p-value: 0.038) (shown to be involved neuropathic pain) Metastasis associated lung adenocarcinoma transcript 1 (MALAT1) (fold change −3.39; p-value: 0.044) (shown to be involved neuropathic pain); Patched 1 pseudogene (LOC100287846) (fold change −6.99; p-value: 0.029) (unknown in pain) were down-regulated preoperatively in the group with chronic postoperative pain compared to the group normal postoperative pain recovery. Conclusions These findings suggest, that TKR patients with chronic postoperative pain present preoperative downregulations of three specific lncRNA detectable at the systemic level. The presented study might give new insights into the complexity of chronic postoperative pain development and show how non-coding RNA plays a role in the underlying molecular mechanisms of pain.