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Introduction/Aims: In traumatic nerve lesions (TNLs), motor unit potentials (MUPs) may be difficult to detect in early injury. Ultrasound‐guided electromyography (US‐EMG) can aid in identifying areas of muscle activation, but its sensitivity can be improved. In this study we compare the sensitivity of US‐EMG alone with US‐EMG after peripheral nerve stimulation (NC‐US‐EMG) to better identify active muscle regions. Methods: In this prospective study, 32 patients with severe TNLs were evaluated with standard EMG (ST‐EMG), US‐EMG, and NC‐US‐EMG at baseline (T0), after 2 to 3 months (T1), and after 5 to 6 months (T2). Results: NC‐US‐EMG was more sensitive in detecting MUPs compared with US‐EMG and ST‐EMG at T0 (19 patients vs 14 and 5 patients, respectively). In addition, both US‐guided techniques were more sensitive than ST‐EMG in detecting MUPs (ST‐EMG vs US‐EMG: P =.014; ST‐EMG vs NC‐US‐EMG: P =.003). At T1, ST‐EMG remained less sensitive NC‐US‐EMG (P =.019). No significant differences were observed among the three techniques at T2. Discussion: In the evaluation of severe TNLs, the combination of peripheral nerve stimulation and US increases the sensitivity of EMG for MUP detection at baseline and 2 to 3 months postinjury. See Editorial on pages 189‐190 in this issue [ABSTRACT FROM AUTHOR]

Carpal tunnel syndrome is the most common entrapment neuropathy, affecting quality of life for many people. Although it is a well recognised condition, new insights into epidemiology, diagnosis, and treatment have emerged in the past 6 years. The availability of disease-modifying treatments for rare systemic disorders associated with carpal tunnel syndrome (eg, amyloidosis) should alert clinicians to these diagnostic possibilities. Besides clinical evaluation and electrophysiology, the role of ultrasonography as a diagnostic tool has been confirmed and new ultrasound techniques have been applied, the clinical use and feasibility of which require further investigation. Surgical and non-surgical interventions are beneficial for the treatment of carpal tunnel syndrome and several treatment options are now available, giving clinicians the possibility to choose the best approach for every patient. New diagnostic and therapeutic techniques require further validation. [ABSTRACT FROM AUTHOR]

The present study aims to investigate the benefits induced by physical activity/practiced sport in Charcot-Marie-Tooth 1A (CMT1A). Patients were divided into sport and no-sport groups according to their sports performance habit. Thirty-one patients were enrolled, of which 14 practiced sports and 17 did not. Clinical assessments were administered to evaluate disability, self-esteem, depression, quality of life, and pain. Statistical analysis revealed significant differences in terms of gender in the no-sport group compared to the sport group (p = 0.04). Regarding the quality of life, physical function (p = 0.001), general health (p = 0.03), social function (p = 0.04), and mental health (p = 0.006) showed better patterns in the sport group than no-sport group. Moreover, neuropathic pain was reduced in the sport group according to the Neuropathic Pain Symptom Inventory (p = 0.001) and ID-PAIN (p = 0.03). The other administered questionnaires showed no significant differences. Our study confirms that CMT1A patients, who practice sports, with a similar severity of disability, may have a better physical quality of life while suffering less neuropathic pain than their peers who do not practice sports. Results recommend the prescription of sport in CMT1A patients. [ABSTRACT FROM AUTHOR]

The SARS-CoV-2 pandemic made the use of facemasks mandatory to prevent contact with the virus. Recent studies have revealed that intensive use of facemasks significantly exacerbated pre-existing headaches and triggered de novo headaches. In our experience, some subjects also complain of symptoms of neuropathic pain in the head/facial regions. Until now, the relationship between neuropathic pain and facemasks has not been documented. The aim of the study is to investigate the occurrence of neuropathic pain related to facemask use. It is a cross-sectional survey using a questionnaire, developed following a commonly accepted outcome research methodology. Participants, both health care and non-health care workers, responded to items included in the questionnaire about the type of facemasks, time and manner of wearing them, side effects such as skin lesions, symptoms of neuropathic pain, etc. [ABSTRACT FROM AUTHOR]

In their article, Frank and colleagues review the effectiveness of motor imagery in learning motor skills, proposing a perceptual-cognitive theory that may facilitate learning. Imagery effectiveness could be enhanced by different techniques, influencing neurophysiological processes. Identifying individuals who could benefit from MI is crucial, and incorporating MI into strong motor representations may lead to better outcomes. Combining MI with other treatments like virtual reality and brain stimulation can further enhance its effectiveness. The purpose of this commentary is to analyze these interventions in light of their potential to influence perceptual-cognitive states in order to strengthen imagery practice and achieve the desired outcomes. [ABSTRACT FROM AUTHOR]

Purpose: The coronavirus disease 2019 (COVID-19) pandemic forced hospitals to adopt tighter restrictions, the most impacting is no access to visitors. Disorder of consciousness (DOC) due to severe acquired brain injury is a condition needing neurorehabilitation and the role of relatives is essential, hence besides physical "disconnection" digital "re-connection" is crucial. We aimed to assess whether digital communication benefits in patients with DOC, considering the sensorial and emotional deprivation due to the COVID-19 emergency lock-down. Methods: For eleven consecutive patients with DOC admitted to our Intensive Neurorehabilitation Care (mean age: 45; females: 9), two observers registered neurobehavioral changes during a video-calls with their relatives. Heart-rate variability was measured before and during the calls. The video-call was performed by using two displays of different sizes: tablet (T-video-call) and large screen (LS-Video-call). Results: The video-calls impacted on the patients' vigilance and in the relationship with relatives. Moreover, positively impacted on their relatives. The current results showed significant greater impact on patients during the LS-video-call than when they are exposed to T-video-call. Conclusions: During the COVID-19 pandemic, besides the physical disconnection to stop the contagion spread, a "digital re-connection" is needed for all and especially for fragile population groups as patients with DOC. [ABSTRACT FROM AUTHOR]

• CMT slowly progress over time, appropriate outcome measures are needed to detect it. • StepWatch™ Activity Monitor reflect real-life function in CMT patients. • StepWatch™ Activity Monitor showed to be able to detect progression over time. Charcot–Marie–Tooth (CMT) is the most common inherited neuropathy, yet has no available pharmacological therapy. Past pharmacotherapy trials failed to provide positive results, possibly due to a poor choice of outcome measures. We previously performed a study in which we validated the 6-minute walk test and StepWatch™ Activity Monitor in CMT. The aim of the current study was to determine if these outcome measures are sensitive to change over a 12-month period. In this longitudinal multicenter study, 149 out of 169 initially enrolled patients were re-evaluated after 12 months using the 6-minute walk test, StepWatch™ Activity Monitor and other outcome measures commonly adopted in CMT disease. Statistical analysis showed a worsening of the CMT-Neuropathy Score (p < 0.05), strength of distal muscles measured by myometry (p < 0.05) and StepWatch™ Activity Monitor outputs (p < 0.05). The 10 meter walking test (p > 0.05), muscular strength as detected by clinical evaluation (p > 0.05), 6-minute walk test (p > 0.05), pain (p > 0.05) and quality of life (p > 0.05) showed no change. In the current study, patients showed clinical worsening over 12 months, confirmed by a reduction of activity as detected by StepWatch™ Activity Monitor. The 6-minute walk test failed to detect change. [ABSTRACT FROM AUTHOR]

The population of industrialized nations is progressively aging, with Italy having one of the most elderly populations in the world. Natural aging may be associated with physical and cognitive impairments, often straining public resources. The present study aims to investigate the influence of gender on wellness of the nonagenarians. We evaluated quality of life among nonagenarians living in the Mugello area, an Italian location with a large population of individuals > 90 years, using the Health Survey Scoring SF-12. The 15-item Geriatric Depression Scale and Basic and Instrumental Activity of Daily Living scales were also assessed. The Mini-Mental State Examination was used to evaluate the cognitive status. In the current survey, women outnumbered men 2.7:1 confirming their higher longevity. However, on the basis of SF-12 scores, nonagenarian women felt worse than men, both physically (mean: women = 41.8 vs men = 44.4, p = 0.004) and mentally (mean: women = 46.7 vs men =48.5, p = 0.034), and their depression rates were higher: considering a General Depression Scale score ≥ 5 as a possible depression status; 37.5% of men reported depression vs. 48.5% of women (p = 0.021). Significant differences were observed also in daily activities, both basic (median: woman = 3 vs men = 5, p < 0.001) and instrumental (median woman = 1 vs me = 3, p < 0.001). Despite prior reports showing that women perform better than men in aging, our study confirms data reported in most national and European surveys: women live longer than men, but with poorer quality of life. The current study confirms the phenomenon known as the "male-female health-survival paradox." [ABSTRACT FROM AUTHOR]

Introduction: Nerve ultrasound in Charcot-Marie-Tooth (CMT) disease has focused mostly on the upper limbs. We performed an evaluation of a large cohort of CMT patients in which we sonographically characterized nerve abnormalities in different disease types, ages, and nerves.Methods: Seventy patients affected by different CMT types and hereditary neuropathy with liability to pressure palsies (HNPP) were evaluated, assessing median, ulnar, fibular, tibial, and sural nerves bilaterally. Data were correlated with age.Results: Nerve dimensions were correlated with CMT type, age, and nerve site. Nerves were larger in demyelinating than in axonal neuropathies. Nerve involvement was symmetric.Discussion: CMT1 patients had larger nerves than did patients with other CMT types. Patients with HNPP showed enlargement at entrapment sites. Our study confirms the general symmetry of ultrasound nerve patterns in CMT. When compared with ultrasound studies of nerves of the upper limbs, evaluation of the lower limbs did not provide additional information. Muscle Nerve 57: E18-E23, 2018. [ABSTRACT FROM AUTHOR]

Objective. Prevalence and clinical characteristics of neuropathic pain due to traumatic brachial plexus injury. Design. Observational epidemiological study. Setting. Hospital-based multicenter study. Subjects. One hundred seven prospectively enrolled patients with brachial plexus injury. Methods. All the patients underwent clinical examination and neurophysiological testing for a definitive diagnosis of the brachial plexus lesion. The DN4 questionnaire was used to identify neuropathic pain, and the Neuropathic Pain Symptom Inventory (NPSI) to evaluate the different symptoms of neuropathic pain. The SF36 questionnaire and the Beck Depression Inventory (BDI) were used to assess quality of life and mood disturbances in patients with neuropathic pain. Results. Of the 107 enrolled patients, 74 had pain (69%); neuropathic pain, as assessed by means of the DN4, was identified in 60 (56%) of these patients. According to the NPSI, the most frequent and severe pain type was the spontaneous burning pain. Clinical and neurophysiological findings showed that pain is unrelated to age but is associated with the severity of peripheral nerve damage. The SF36 questionnaire and BDI showed that neuropathic pain impairs quality of life and causes depression. Conclusions. Our study provides information on the prevalence, characteristics, and variables associated with neuropathic pain due to traumatic brachial plexus injuries that might provide a basis for improving the clinical management of this condition. [ABSTRACT FROM AUTHOR]

Technical advances are rapidly changing the clinical and instrumental approach to peripheral nerve diseases. Magnetic resonance neurography, diffusion tensor imaging and nerve ultrasonography are increasingly entering the diagnostic workup of peripheral neuropathies as tools that complement neurophysiology and enable investigation of proximal structures, such as plexuses and roots. Progress in the design of magnetic resonance scanners and sequences, and the development of high-frequency ultrasound probes mean that high-resolution peripheral nerve imaging is possible, enabling detailed examination of nerve size, morphology and internal fascicular structure that can integrate nerve conduction studies into clinical practice. In the growing field of small-fibre neuropathy, in which traditional nerve conduction studies are of little or no use, skin biopsy has become a reliable tool for diagnosis. Corneal confocal microscopy, nociceptive evoked potentials and microneurography are emerging techniques that are mainly used in clinical research settings, but have increasing relevance to clinical practice. We review these new and emerging techniques and their effects on diagnosis, treatment strategies and prognosis in a variety of peripheral neuropathies, including entrapments, brachial plexopathies, immune and inherited neuropathies, and small-fibre neuropathies. We discuss the most promising research findings and their potential for future application in clinical practice. [ABSTRACT FROM AUTHOR]

Carpal tunnel syndrome is the most common peripheral nerve entrapment syndrome worldwide. The clinical symptoms and physical examination findings in patients with this syndrome are recognised widely and various treatments exist, including non-surgical and surgical options. Despite these advantages, there is a paucity of evidence about the best approaches for assessment of carpal tunnel syndrome and to guide treatment decisions. More objective methods for assessment, including electrodiagnostic testing and nerve imaging, provide additional information about the extent of axonal involvement and structural change, but their exact benefit to patients is unknown. Although the best means of integrating clinical, functional, and anatomical information for selecting treatment choices has not yet been identified, patients can be diagnosed quickly and respond well to treatment. The high prevalence of carpal tunnel syndrome, its effects on quality of life, and the cost that disease burden generates to health systems make it important to identify the research priorities that will be resolved in clinical trials. [ABSTRACT FROM AUTHOR]

Objective Postures and work-hobby activities may play a role in the origin and progression of ulnar neuropathy at the elbow (UNE), whose occurrence appears to be increasing. The time spent on mobile-phone has increased in the last decades leading to an increased time spent with flexed elbow (prolonged-phone-posture, PPP). We aimed to assess the effect of PPP both in patients with symptoms of UNE and in symptom-free subjects. Methods Patients with pure sensory symptoms of UNE and negative neurophysiological tests (MIN-UNE) and symptom-free subjects were enrolled. We evaluated ulnar motor nerve conduction velocity across elbow at baseline and after 6, 9, 12, 15, and 18 min of PPP in both groups. Fifty-six symptom-free subjects and fifty-eight patients were enrolled. Globally 186 ulnar nerves from 114 subjects were studied. Results Conduction velocity of ulnar nerve across the elbow significantly changed over PPP time in patients with MIN-UNE, showing a different evolution between the two groups. Conclusions PPP causes a modification of ulnar nerve functionality in patients with MIN-UNE. Significance PPP may cause transient stress of ulnar nerve at elbow. [ABSTRACT FROM AUTHOR]

Objectives To investigate the effect of pure passive movement on both cortical and subcortical somatosensory evoked potentials (SEPs). Methods Median nerve SEPs were recorded in 8 patients suffering from Parkinson’s disease (PD) and two patients with essential tremor. PD patients underwent electrode implantation in the subthalamic (STN) nucleus (3 patients) and pedunculopontine (PPTg) nucleus (5 patients), while 2 patients with essential tremor were implanted in the ventral intermediate nucleus (VIM) of the thalamus. In anesthetized patients, SEPs were recorded at rest and during a passive movement of the thumb of the stimulated wrist from the intracranial electrode contacts and from the scalp. Also the high-frequency oscillations (HFOs) were analyzed. Results Amplitudes of both deep and scalp components were decreased during passive movement, but the reduction was higher at cortical than subcortical level. Also the HFOs were reduced by movement. Conclusion The different amount of the movement-related decrease suggests that the cortical SEP gating is not only the result of a subcortical somatosensory volley attenuation, but a further mechanism acting at cortical level should be considered. Significance Our results are important for understanding the physiological mechanism of the sensory–motor interaction during passive movement. [ABSTRACT FROM AUTHOR]

Objective To investigate the generators of the somatosensory evoked potential (SEP) components recorded from the Pedunculopontine Tegmental nucleus (PPTg). Methods Twenty-two patients, suffering from Parkinson’s disease (PD), underwent electrode implantation in the PPTg area for deep brain stimulation (DBS). SEPs were recorded from the DBS electrode contacts to median nerve stimulation. Results SEPs recorded from the PPTg electrode contacts could be classified in 3 types, according to their waveforms. (1) The biphasic potential showed a positive peak (P16) whose latency (16.05 ± 0.61 ms) shifted of 0.18 ± 0.07 ms from the lower to the upper contact of the electrode. (2) The triphasic potential showed an initial positive peak (P15) whose latency (15.4 ± 0.2 ms) did not change across the DBS electrode contacts. (3) In the last SEP configuration (mixed biphasic and triphasic waveform), the positive peak was bifid including both the P15 and P16 potentials. Conclusion While the P16 potential is probably generated by the somatosensory volley travelling along the medial lemniscus, the P15 response represents a far-field potential probably generated at the cuneate nucleus level. Significance Our results show the physiological meaning of the somatosensory responses recorded from the PPTg nucleus area. [ABSTRACT FROM AUTHOR]

Highlights: [•] Polyneuropathy associated with IgM monoclonal gammopathy is an immune-mediated polyneuropathy clinically characterized by symmetrical, distal polyneuropathy with predominant sensory ataxia. [•] Many cases of IgM-related neuropathy with a clinical phenotype not fitting the classic sensory-ataxic form have been described in literature. [•] Present findings suggest that demyelinating abnormalities in nerve conduction studies are more often associated with classical forms while axonal impairment occurs more often in atypical phenotypes. [ABSTRACT FROM AUTHOR]

The Coronavirus Disease 2019 (COVID-19) pandemic is a severe ongoing global emergency. Despite high rates of asymptomatic patients, in many cases, the infection causes a rapid decline in pulmonary function due to an acute respiratory distress-like syndrome, leading to multi-organ failure and death. To date, recommendations about rehabilitation on COVID-19 are based on clinical data derived from other similar lung diseases. Rehabilitation literature lacks a standard taxonomy, limiting a proper evaluation of the most effective treatments for patients after COVID-19 infection. In this study, we assessed the clinical and rehabilitative associations and the geographical area involved in interstitial lung diseases (ILD) and in COVID-19, by a mathematical analysis based on graph theory. We performed a quantitative analysis of the literature in terms of lexical analysis and on how words are connected to each other. Despite a large difference in timeframe (throughout the last 23 years for ILD and in the last 1.5 years for COVID-19), the numbers of papers included in this study were similar. Our results show a clear discrepancy between rehabilitation proposed for COVID-19 and ILD. In ILD, the term "rehabilitation" and other related words such as "exercise" and "program" resulted in lower values of centrality and higher values of eccentricity, meaning relatively less importance of the training during the process of care in rehabilitation of patients with ILD. Conversely, "rehabilitation" was one of the most cited terms in COVID-19 literature, strongly associated with terms such as "exercise", "physical", and "program", entailing a multidimensional approach of the rehabilitation for these patients. This could also be due to the widespread studies conducted on rehabilitation on COVID-19, with Chinese and Italian researchers more involved. The assessment of the terms used for the description of the rehabilitation may help to program shared rehabilitation knowledge and avoid literature misunderstandings. [ABSTRACT FROM AUTHOR]

Abstract: Myasthenia gravis (MG) with antibodies to the muscle-specific receptor tyrosine kinase (MuSK-MG) is a rare disease which covers 5–8% of all MG patients. Symptoms are nearly always generalized, though more focal than in MG with anti-acetylcholine receptor antibodies, with predominant involvement of cranial, bulbar and axial muscles; early respiratory crises are frequent. Focal atrophy, mostly of facial, masseter and tongue muscles, occurs in a proportion of patients. Diagnosis is often challenging on account of atypical presentation with little or no symptom fluctuations, lack of response to acetylcholinesterase inhibitors in a high proportion of patients and negative results of electrodiagnostic studies when performed on limb muscles. Immunosuppression is the mainstay of treatment, since the response to acetylcholinesterase inhibitors is generally unsatisfactory and thymectomy does not appear to improve the course of the disease. Although corticosteroids result in marked improvement, disease flares are frequent during prednisone dosage tapering and most patients remain dependent on treatment. Since treatment with rituximab, in uncontrolled studies, induced sustained benefit in patients with refractory disease, B cell depletion is an attractive option for MuSK-MG patients unresponsive to conventional immunosuppressants. [Copyright &y& Elsevier]

Parkinson's disease (PD) is one of the most common neurodegenerative disorders characterized by decreased levels of the neurotransmitter dopamine (DA) in the striatum of the brain, as a result of degeneration of DA neurons. Levodopa (L-Dopa) crosses the blood-brain barrier and its administration replenishes the loss of DA in dopaminergic neurons in PD patients. Despite the evident beneficial effects, L-Dopa use may cause side effects and its toxicity found in in vitro assays has been attributed to the generation of reactive oxygen species (ROS): L-Dopa is converted to DA and its metabolism and autoxidation gives rise to quinones, semiquinones, and hydrogen peroxide. Despite this evidence, L-Dopa in some in vivo and in vitro experiments showed no toxic effects, or even antioxidant effects. Two major peripheral L-Dopa metabolic pathways, driven by the enzymes Aromatic L-amino acid decarboxylase (AADC) and catechol-O-methyl transferase (COMT), significantly deplete the amount of L-Dopa reaching the brain. The low bioavailability of L-Dopa may cause a wide variation in clinical response between patients. Strategies addressing to improve the bioavailability of L-Dopa include coadministering L-Dopa with carbidopa, a decarboxylase inhibitor, as multiple daily doses. We utilized catecholaminergic human neuroblastoma cells to study DNA damage and ROS production after L-Dopa and carbidopa treatments. Our data lead us to confirm that L-Dopa may have a protective effect on dopaminergic cells especially at certain concentrations, in particular, toward the production of ROS and their toxic effects on DNA. Furthermore in the combined treatment, with induction of ROS following administration of H2O2, carbidopa is effective in reducing the damage caused by reactive oxygen intermediates both alone and in combination with L-Dopa. In Parkinson's disease, the neurotransmitter dopamine is produced at decreased levels in the brain region substantia nigra. In this study, two pathways used for peripheral metabolism of L-Dopa were examined and a beneficial influence of carbidopa on reactive oxygen species and their toxic impact on host DNA were found. [ABSTRACT FROM AUTHOR]

Objective. The aim of this study was to quantify and characterize pain in patients undergoing lower limb postsurgical orthopedic rehabilitation and to investigate the impact of pain in slowing or interrupting their rehabilitation. Design. The study was designed as a multicenter cross-sectional study. Setting. The study was set in rehabilitation departments of the Don Gnocchi Foundation. Subjects. The study subjects were the inpatients attending rehabilitation. Interventions. There were no interventions used in the study. Outcome Measures. Pain intensity was measured with a numeric rating scale (NRS); pain characteristics were assessed with the McGill Pain Questionnaire and the ID Pain (able to discriminate nociceptive from neuropathic pain). Quality of life (QoL) was measured with the Short Form 36 Health Status Survey. A semi-structured questionnaire on pain occurrence, impact, and management was administered by the physiotherapist in charge of the patients and by the physician. Results. We studied 139 patients, 82% of whom complained of at least moderate pain (NRS ≥ 3). According to ID pain, 45.6% patients complained of probable (33.8%) or highly probable (11.8%) neuropathic pain. A higher pain intensity was significantly related to the probability of having neuropathic pain ( P < 0.001). Patients with more severe pain reported lower physical and mental QoL scores. In 38.6% of cases, pain interfered with the rehabilitation process, and in 18.5% it was the cause of physical therapy discontinuation. Conclusions. In light of the high occurrence and intensity of pain in the sample, and of the significant impact on the rehabilitation program, clinicians should pay more attention to pain, especially neuropathic pain, in postsurgical patients. Tailored pain pharmacological therapy could possibly improve patient compliance during the rehabilitation process and enhance long-term outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Nerve involvement in immune-related neuropathies is non-homogeneous, and therefore characterization of ultrasound (US) abnormalities is difficult. We developed two measures to quantify US abnormalities in immune-related neuropathies. Methods: Intranerve cross-sectional area (CSA) variability for each nerve was calculated as: maximal CSA/minimal CSA. Internerve CSA variability for each patient was calculated as: maximal intranerve CSA variability/minimal intranerve CSA variability. Six patients underwent US evaluation of the median, ulnar, and fibular nerves, and the abnormalities were scored with our newly developed measures. Results: The new measures were applicable to all nerves and patients. The highest degree of intra- and internerve CSA variability was observed in multifocal motor neuropathy, consistent with the asymmetric characteristics of this neuropathy. Conclusions: The application of intra- and internerve CSA variability measures allows us to quantify the heterogeneity of nerves and nerve segments and identify different US patterns in diverse immune-related neuropathies. Muscle Nerve, 2012 [ABSTRACT FROM AUTHOR]

The article presents a study on whether single-fiber conduction velocity (SF-CV) of axons could enhance detection of mild motor nerve function abnormalities in the early stages of diabetic polyneuropathy (DP). 21 Caucasian patients were enrolled in the endocrinology unit of a hospital in Rome, Italy. Symptoms reported by the subjects were associated with abnormal SF-CV and not with conventional conduction (C-CV) examinations. The study found that SF-CV was able to detect mild myelin damage with higher sensitivity.

The reported incidence of pregnancy-related carpal tunnel syndrome (PRCTS) ranges from 0.8% to 70%, and little is known of its natural history. We systematically reviewed the reported incidence of PRCTS and evaluated its natural history. We identified 214 studies that fulfilled our selection criteria. Six publications fulfilled the inclusion criteria. Five fulfilled the incidence criteria, 3 fulfilled the natural history criteria, and 2 of the 6 publications satisfied both. The reported incidence of neurophysiologically confirmed PRCTS ranged from 7% to 43%, whereas the incidence of clinically diagnosed PRCTS ranged from 31% to 62%. Symptoms persisted in more than 50% of the patients after 1 year and in about 30% after 3 years. Our review suggests that variations in the reported incidence of PRCTS largely depend on the methods used to detect this syndrome. Our data also suggest that symptoms persist in a substantial number of patients 1 or more years after delivery. Muscle Nerve 42: 697-702, 2010 [ABSTRACT FROM AUTHOR]

Abstract: Objective: To investigate the effect of the voluntary movement on the amplitude of the somatosensory evoked potentials (SEPs) recorded by an epidural electrode at level of the dorsal column nuclei (DCN). Methods: Five patients, suffering from chronic pain resistant to pharmacological treatment, underwent an epidural electrode implant at high cervical spinal cord level (C2) for neuromodulation. After tibial nerve stimulation, SEPs were recorded from the epidural electrode contacts, from a Cz lead, and from two electrodes placed over the 12th dorsal vertebra and 4th lumbar vertebra, respectively. SEPs were recorded at rest and during a voluntary flexo-extension movement of the stimulated foot. Beyond the low-frequency SEPs, also the high-frequency oscillations (HFOs), obtained by filtering the recorded traces by means of a 1000–2000Hz bandpass offline, were analysed. Results: The epidural electrode contacts recorded a triphasic potential (P1–N1–P2), whose negative peak showed a latency similar to that of the P30 far-field response obtained from the scalp. The epidural potential amplitude was significantly decreased by the voluntary movement, as compared to the rest (p <0.01). A main HFO peak, identifiable at around 1200Hz, was significantly lower in amplitude during movement than at rest (p =0.008). Conclusions: Our findings suggest that the epidural C2 triphasic wave is a potential arising from DCN. The low-frequency epidural SEP component is subtended by a 1200Hz HFO, probably generated by post-synaptic events. Significance: The amplitude reduction of the DCN response during movement is possibly due to decreased excitability of the DCN neurons receiving the somatosensory ascending input. [Copyright &y& Elsevier]

Charcot–Marie-Tooth (CMT) disease is the most frequent inherited neuropathy, no therapies are available at the moment but clinical trials are ongoing. For that reason it is very important to know the natural history of the disease. We report the results of the natural history of clinical features and quality of life (QoL) in patients with CMT2. Twenty patients were enrolled. At recruitment and at follow-up (2 years), all patients underwent neurological evaluation, QoL and disability assessments. The study-end evaluation took place 20–28 months after the baseline evaluation. During the 2-year follow-up period, CMT2 patients showed a mild reduction of strength of distal muscles of upper limbs and proximal muscles of lower limbs, a worsening sensory function and a mild increase in walking disability. However, there was no relevant worsening of QoL, except for a mild deterioration of one mental health domain. [ABSTRACT FROM AUTHOR]

The aim of this study was to assess quality of life (QoL) and evaluate the occurrence and characteristics of pain in facioscapulohumeral muscular dystrophy (FSHD) patients. No study has yet assessed QoL in a large group of FSHD patients and, overall, few studies have assessed pain in neuromuscular diseases. We performed a prospective study using a multidimensional protocol including: clinical (according to the Clinical Severity Scale Rev1); genetic (p13E-11 EcoRI fragments Rev1); QoL (Short Form-36); pain (Visual Analog Scale and Portenoy-6 questions); and depression (Beck Depression Inventory) assessment. QoL measures of FSHD were compared with those of Italian norms. Moreover, we correlated QoL and pain measurements with clinical findings. Sixty-five patients were enrolled in the study. QoL was statistically significantly reduced with respect to the Italian normative sample, mainly in physical domains. Our study demonstrated that pain is frequent in FSHD patients. More than half of the patients complained of at least moderate pain. Women complained of slightly higher levels of deterioration in the emotional aspects of QoL than men. Clinical pattern (as assessed by Clinical Severity Scale) was closely related to physical QoL domains: the higher the clinical involvement, the more severe the QoL deterioration. This study provided information that may be crucial in clinical practice: pain may be a relevant aspect in FSHD patients, and prevention strategies or relevant therapies should be considered as appropriate. Moreover, we must pay more attention to gender differences: women can suffer far greater deterioration in the emotional aspects of QoL. Further multidimensional observations are needed. Muscle Nerve 40: 200-205, 2009 [ABSTRACT FROM AUTHOR]

Ultrasonographic (US) enlargement of the median nerve at the wrist is known to be consistent with carpal tunnel syndrome (CTS), although the effects of different measurement techniques, equipment, and patient populations remain unknown. The purpose of this study was to examine the similarities and differences of US findings in CTS between two electromyography (EMG) laboratories. In 2006 and 2007, US measurements of the median nerve were recorded independently and statistically analyzed in patients with CTS at two EMG laboratories (Duke University, Durham, NC, USA, and Università Cattolica del Sacro Cuore, Rome, Italy). Patient age, median nerve area in the forearm, and neurophysiologic score did not differ significantly between the two laboratories. The North Carolina group had a larger median nerve area at the wrist and wrist-to-forearm ratio than the Italian group, although both were elevated in reference to established values for the diagnosis of CTS. Median nerve US is less susceptible to differences between laboratories than previously thought, permitting greater generalization of findings. Muscle Nerve 40: 94-97, 2009 [ABSTRACT FROM AUTHOR]

The aim of this study was to validate the Italian version of the Neuropathic Pain Symptom Inventory (NPSI) in patients with neuropathic pain due to peripheral nerve diseases, and also to evaluate the validity of a new NPSI score: a frequency weighted NPSI score (NPSI-FW). First, the original version of the NPSI was translated into Italian. Then the validity and reliability of the Italian NPSI (I-NPSI) were tested in 392 Italian patients consecutively referred to 16 Italian outpatient services for peripheral nerve diseases, by correlating the I-NPSI scores with other pain scales. The repeatability and responsiveness were assessed. A significant correlation between the I-NPSI scores and all the other pain measures was seen. Reproducibility and responsiveness were good. Our study shows the validity of the I-NPSI and demonstrates its reliability for assessing neuropathic pain in patients with peripheral nerve diseases. The I-NPSI scores represent reliable measurements to assess neuropathic symptoms and effectiveness of treatment on them. [ABSTRACT FROM AUTHOR]

Quality of life (QoL), as defined by the Short Form 36, has previously been shown to be abnormal in patients with Charcot-Marie-Tooth disease (CMT), both for Physical Composite Scores (PCS) and Mental Composite Scores (MCS). We have now extended these observations in a multicenter evaluation of 89 patients with Charcot-Marie-Tooth disease type 1A, the most common form of CMT. Both the PCS and MCS were abnormal also in this cohort, compared with the Italian population at large. In particular, the ability to ambulate independently as well as toe and heel walk correlated well with QoL measures in our patients. [ABSTRACT FROM AUTHOR]

Abstract: Many antineoplastic drugs have been found to have carcinogenic, mutagenic and teratogenic activity and so hospital personnel handling these substances are potentially exposed to health risk. Understanding this risk derived from protracted occupational exposure has great relevance even if the workers normally adopt individual and environmental protective measures. To address this question we have studied the presence of DNA and chromosome damage in a population of nurses employed in Italian oncology units and in matched controls. We used the comet assay to evidence the presence of DNA strand breaks, due to both acute and chronic exposure, and the micronucleus (MN) test, which is a measure of clastogenic and aneugenic events. Furthermore, since the individual response to the exogenous insults may be genetically determined, we studied the possible influence of single nucleotide polymorphism in XRCC1 and XRCC3 DNA repair genes on induced genetic damage. We also considered the effects of confounding factors like smoking, age and gender. The results indicated that the exposed subjects had significantly high levels of genetic damage. Age and gender were associated with increased values in MN, both in control and in exposed groups; the smoking habit affects MN frequency in controls, but not in workers. Furthermore we found that exposed subjects bearing at least one XRCC1 variant allele (399Gln) show higher values of MN. The present data provide the evidence to show that occupational exposure to antineoplastic drugs, even if in safety controlled conditions, represents a serious health risk. Furthermore we have shown that the presence of XRCC1 genetic polymorphism could contribute to increase the genetic damage in susceptible individuals who are occupationally exposed to dangerous substances. [Copyright &y& Elsevier]

Abstract: Objective: To investigate the effect of the voluntary movement on the amplitude of the somatosensory evoked potentials (SEPs) recorded by an epidural electrode at level of the cervical spinal cord (CSC). Methods: Fourteen patients underwent an epidural electrode implant at CSC level for pain relief. After the median nerve stimulation, SEPs were recorded from the epidural electrode and from 4 surface electrodes (in frontal and parietal regions contralateral to the stimulated side, over the 6th cervical vertebra, and on the Erb’s point). SEPs were recorded at rest and during a voluntary flexo-extension movement of the stimulated wrist. Beyond the low-frequency SEPs, also the high-frequency oscillations (HFOs) were analysed. Results: The epidural electrode contacts recorded a triphasic potential (P1–N1–P2), whose negative peak showed the same latency as the cervical N13 response. The epidural potential amplitude was significantly decreased during the voluntary movement, as compared to the rest. Two main HFOs were identifiable: (1) the 1200Hz HFO which was significantly lower in amplitude during movement than at rest, and (2) the 500Hz HFO which was not modified by the voluntary movement. Conclusions: The low-frequency cervical SEP component is subtended by HFOs probably generated by: (1) postsynaptic potentials in the dorsal horn neurones (1200Hz), and (2) presynaptic ascending somatosensory inputs (500Hz). Significance: Our findings show that the voluntary movement may affect the somatosensory input processing also at CSC level. [Copyright &y& Elsevier]

Abstract: Objective: To evaluate whether, for patients with suspected myelin impairment, the sensitivity of motor nerve conduction studies can be increased by using an SFEMG electrode which makes it possible to study conduction velocity in a small number of axons (SF-CV). Methods: We studied 22 consecutive patients with suspected neuropathy through conventional motor conduction study and through SF-CV. For each patient we selected a nerve that was normal at conventional neurography and studied it through SF-CV. Also, we performed SF-CV in 15 healthy subjects. We considered 36m/s as the low limit of normal SF-CV (the normal value commonly accepted in the literature for the slowest alpha motor axons). Results: In the healthy subjects we never observed abnormal SF-CV values. Of the 22 patients, in 18 the conventional tests showed abnormal findings suggestive of neuropathy. The remaining 4 patients were completely normal at the conventional tests. Through SF-CV we studied 22 nerves that were normal at the conventional tests. Fourteen of 22 (64%) nerves presented pathological SF-CV test. Half of the patients with normal findings at the conventional tests showed pathological SF-CV test. Conclusions: SF-CV evaluation may be useful in detecting early, mild, or partial myelin damage, because it makes it possible to detect nerve conduction slowing when conventional tests are normal. Significance: Increasing sensitivity of motor conduction evaluation. [Copyright &y& Elsevier]

Abstract: Objective: To correlate the patient-derived physical function score, as measured by the disease specific Myasthenia Gravis Questionnaire (MGQ), and the score obtained with commonly used generic 36-item questionnaire, the Short-Form health survey (SF-36), with the degree of abnormal neuromuscular transmission measured by Single-Fibre EMG (SFEMG) and repetitive nerve stimulation (RNS) in Myasthenia Gravis (MG) patients. Methods: SFEMG and RNS were performed in the deltoid muscle and SFEMG was also performed in the orbicularis oculi muscle in 45 MG patients. The patients were asked to fill out the MGQ, which has been translated and validated into Swedish, and the SF-36. The sum of MGQ items generates a global MGQ score. Items are also divided into 3 muscle specific domains: generalized, bulbar and ocular. Results: The global MGQ score and generalized domain score of MGQ were strongly correlated with the degree of abnormal neuromuscular transmission, as measured by SFEMG in the deltoid muscle. Scores from ocular and bulbar domains of MGQ were not related to neurophysiological findings. Physical composite scores of SF-36 correlated significantly with the abnormal SFEMG findings in the deltoid. Conclusions: The degree of disturbed neuromuscular transmission in a proximal limb muscle correlates with a patient''s subjective experience of generalized myasthenic dysfunction. Significance: The observed correlation adds a new dimension to neurophysiological examinations in patients with MG. [Copyright &y& Elsevier]

Neuropathy can severely affect patients' quality of life (QoL), causing disability and decreased motor efficiency while modifying their perception of the external environment. We performed a large, multiperspective, and multimeasurement assessment using validated clinical measurements of disability and QoL. With regard to general disability, more than 10% of patients needed assistance to walk. With regard to arm disability, about 10% of patients had severe disability defined as impairment in carrying out simple tasks. General disability was similar both in polyneuropathic and multineuropathic groups, but arm disability appeared more severe in multineuropathic patients. QoL profiles were highly deteriorated in our sample with respect to the Italian normative sample. All age brackets were highly impaired except for older patients for whom the QoL picture was only mildly impaired with respect to Italian norms. We believe that our study provides reliable data on QoL in patients with neuropathies and that it may represent a quantitative step forward in understanding the disability of patients with neuropathy. [ABSTRACT FROM AUTHOR]

A number of studies have focused cm quality of life in children with spina bifida, concentrating for the most part on younger children. We assessed health-related quality of life and disability in adolescents with spina bifida and correlated these with the clinical and neurophysiologic picture. Twelve consecutive spina bifida cases were evaluated through a multiperspective protocol by means of (1) clinical and neurophysiologic assessment, (2) a self-administered questionnaire for general health — Short Form-36 (SF-36) — and (3) standardized disability measurements (Functional Independence Measure, Barthel Index, Deambulation Index). Relationships between disability measurement, the adolescents' perspective, and conventional clinical and neurophysiologic assessment were evaluated. The Functional Independence Measure is strongly related to the clinical measurement of muscle strength (P<.03, r>.6). The Barthel Index and Deambulation Index are related to clinical measurement of muscle strength (P<.03, r>.6), as well as to electromyographic findings in the proximal muscles of inferior limbs (P<.03, r>.6). Although the function of lower limb muscles, especially the proximal muscles, is closely related to impairment of physical aspects of quality of life, it is only partially related to the mental aspects (P<.03, r>.6). [ABSTRACT FROM AUTHOR]

This study prospectively compared preoperative and postoperative (6 months after surgical release) clinical and electrophysiological evaluation and self-administered Boston questionnaire (BQ) findings of a series of elderly carpal tunnel syndrome (CTS) patients with those of young and adult patients. Three hundred twenty three consecutive hands (282 patients) underwent surgery between 1997 and 2000. Patients were divided into four age groups: group 1 (20–40 years), 49 hands; group 2 (41–54 years), 96 hands; group 3 (55–69 years), 106 hands; and group 4 (70–90 years), 72 hands. Sex ratio, education, duration and bilaterality of CTS, history of wrist and hand trauma, connective tissue and thyroid diseases, diabetes, polyneuropathy, renal failure, and other nerve entrapment syndromes were recorded. The elderly group (group 4) only had more cases of diabetes than groups 1 and 2 (young and adult patients). Before surgery, elderly patients showed more severe clinical objective and electrophysiological findings than young and adult patients. Preoperative subjective findings (BQ scores) were similar in all four groups. Although their absolute postoperative BQ scores and clinical objective and electrophysiological stages improved, elderly patients showed less improvement in all findings than the 20–54 age groups, presumably due to greater preoperative damage and less repair capacity of the compressed nerve. This, however, is not a contraindication for surgical release in elderly patients. [ABSTRACT FROM AUTHOR]

We performed a multi-perspective follow up in 25 patients with autoimmune neurological disorders treated with intravenous immunoglobulin (IVIg) to evaluate the early effects on patients' health-related quality of life (HRQoL). Diagnoses were based on clinical picture, neurophysiological assessment, nerve biopsy, and therapeutic response. As patient-oriented assessment, we used the Short Form 36 questionnaire (SF-36), the most used generic health tool, and the disability of arm shoulder and hand questionnaire (DASH). Our data indicate that physical aspects of patients' HRQoL improve early and strongly after IVIg. This agrees with the muscle strength improvement previously reported. But, the mental aspects of the HRQoL are not influenced by the therapies. Traditional outcome assessment in neurological diseases has always been based on physician-derived and instrumental findings. Over the last two decades, clinical researchers have underlined the need for a standardized evaluation of concepts such as HRQoL. We believe that this study represents a further step toward an evidence-based therapeutic approach of some neurological diseases and that further fundamental steps should be taken. [ABSTRACT FROM AUTHOR]

Heart rate variability (HRV) is a biomarker of autonomic nervous system (ANS) reaction in persons with severe acquired brain injury (sABI) who undergo a rehabilitation treatment, such as focal muscle vibration (FMV). This study aims to evaluate if and how FMV can modulate HRV and to compare potential differences in FMV modulation in HRV between patients with sABI and healthy controls. Ten patients with sABI and seven healthy controls have been recruited. Each individual underwent the same stimulation protocol (four consecutive trains of vibration of 5 minutes each with a 1-minute pause). HRV was analyzed through the ratio of frequency domain heart-rate variability (LF/HF). In the control group, after performing FMV, a significant LF/HF difference was observed in the in the second vibration session compared to the POST phase. Patients with SABI treated on the affected side showed a statistically significant LF/HF difference in the PRE compared to the first vibration session. These preliminary results suggest that FMV may modify the cardiac ANS activity in patients with sABI. [ABSTRACT FROM AUTHOR]

Disorders of Consciousness (DoCs) after severe acquired brain injury involve substantial impairment of cognition and physical functioning, requiring comprehensive rehabilitation and support. Technological interventions, such as immersive Virtual Reality (VR), have shown promising results in promoting neural activity and enhancing cognitive and motor recovery. VR can induce physical sensations that may activate the Autonomic Nervous System (ANS) and induce ANS-regulated responses. This study aimed to investigate the effects of immersive VR on the ANS in patients with DoCs through the analysis of the electrodermal activity (EDA). EDA was measured with a wearable device during a single immersive VR session consisting of static and dynamic videos depicting naturalistic environments. A pilot case–control study was conducted with 12 healthy participants and 12 individuals with DoCs. Results showed higher EDA values in patients than in healthy participants (p = 0.035), suggesting stronger autonomic activation during immersive VR exposure, while healthy subjects, in turn, showed a decrease in EDA values. Our results revealed a significant interaction between conditions and groups (p = 0.003), with patients showing significantly increased EDA values from the baseline compared to dynamic video observation (p = 0.014) and final rest (p = 0.007). These results suggest that immersive VR can elicit sympathetic arousal in patients with DoCs. This study highlights the potential of immersive VR as a tool to strengthen autonomic responses in patients with impaired consciousness. [ABSTRACT FROM AUTHOR]

Background: Sleep abnormalities have been reported in Charcot–Marie–Tooth disease (CMT), but data are scanty. We investigated their presence and correlation in a large CMT patients' series. Methods: Epworth Sleepiness Scale (ESS) and Pittsburgh Sleep Quality Index (PSQI) were administered to CMT patients of the Italian registry and controls. ESS score > 10 indicated abnormal daytime somnolence, PSQI score > 5 bad sleep quality. We analyzed correlation with disease severity and characteristics, Hospital Anxiety and Depression Scale (HADS), Modified Fatigue Impact Scale (MFIS), Body Mass Index, drug use. Results: ESS and PSQI questionnaires were filled by 257 and 253 CMT patients, respectively, and 58 controls. Median PSQI score was higher in CMT patients than controls (6 vs 4, p = 0.006), with no difference for ESS score. Abnormal somnolence and poor sleep quality occurred in 23% and 56% of patients; such patients had more frequently anxiety/depression, abnormal fatigue, and positive sensory symptoms than those with normal ESS/PSQI. Moreover, patients with PSQI score > 5 had more severe disease (median CMT Examination Score, CMTES, 8 vs 6, p = 0.006) and more frequent use of anxiolytic/antidepressant drugs (29% vs 7%, p < 0.001). Conclusions: Bad sleep quality and daytime sleepiness are frequent in CMT and correlated with anxiety, depression and fatigue, confirming that different components affect sleep. Sleep disorders, such as sleep apnea and restless leg syndrome, not specifically investigated here, are other factors known to impact on sleep quality and somnolence. CMT patients' management must include sleep behavior assessment and evaluation of its correlated factors, including general distress and fatigue. [ABSTRACT FROM AUTHOR]

Background and purpose: Data are reported from the Italian CMT Registry. Methods: The Italian CMT Registry is a dual registry where the patient registers and chooses a reference center where the attending clinician collects a minimal dataset of information and administers the Charcot–Marie–Tooth (CMT) Examination/Neuropathy Score. Entered data are encrypted. Results: Overall, 1012 patients had registered (535 females) and 711 had received a genetic diagnosis. Demyelinating CMT (65.3%) was more common than axonal CMT2 (24.6%) and intermediate CMT (9.0%). The PMP22 duplication was the most frequent mutation (45.2%), followed by variants in GJB1 and MPZ (both ~10%) and MFN2 (3.3%) genes. A relatively high mutation rate in some "rare" genes (HSPB1 1.6%, NEFL 1.5%, SH3TC2 1.5%) and the presence of multiple mutation clusters across Italy was observed. CMT4A was the most disabling type, followed by CMT4C and CMT1E. Disease progression rate differed depending on the CMT subtype. Foot deformities and walking difficulties were the main features. Shoe inserts and orthotic aids were used by almost one‐half of all patients. Scoliosis was present in 20% of patients, especially in CMT4C. Recessive forms had more frequently walking delay, walking support need and wheelchair use. Hip dysplasia occurred in early‐onset CMT. Conclusions: The Italian CMT Registry has proven to be a powerful data source to collect information about epidemiology and genetic distribution, clinical features and disease progression of CMT in Italy and is a useful tool for recruiting patients in forthcoming clinical trials. [ABSTRACT FROM AUTHOR]