Lower limb nerve ultrasound: A four-way comparison of acquired and inherited axonopathy, inherited neuronopathy and healthy controls.

INTRODUCTION/AIMS: In a recent study, we showed that nerve ultrasound of the upper limbs could distinguish inherited sensory neuronopathy from inherited axonopathy; surprisingly, no differences were found in the lower limb nerves. In this study, we compared lower limb nerve ultrasound measurements in inherited neuronopathy, inherited axonopathy, and acquired axonopathy. METHODS: Tibial and sural nerve ultrasound cross-sectional areas (CSAs) of 34 healthy controls were retrospectively compared with those of three patient groups: 17 with cerebellar ataxia with neuronopathy and vestibular areflexia syndrome (CANVAS), 18 with Charcot-Marie-Tooth type 2 (CMT2), and 18 with acquired length-dependent sensorimotor axonal neuropathy, using ANOVA with post-hoc Tukey honestly significance difference (HSD) (significance level set at p < .05). RESULTS: The nerve CSAs of CANVAS and CMT2 patients were not significantly different. Both the tibial and the sural nerve CSAs were significantly smaller in CANVAS and CMT2 compared with the acquired axonal neuropathy group. Tibial nerve CSAs of CANVAS and CMT2 were significantly smaller than controls. Tibial and sural nerve CSAs of the acquired axonal neuropathy group were also significantly larger than the controls'. DISCUSSION: Ultrasound of the lower limb nerves distinguished inherited from acquired axonopathy with the nerve size respectively reduced and increased in these two groups. This has potential implication for the differential diagnosis of these diseases in clinical practice.

Nerve ultrasound in CANVAS-spectrum disease: Reduced nerve size distinguishes genetically confirmed CANVAS from other axonal polyneuropathies.

BACKGROUND AND AIMS: Ultrasound nerve cross-sectional area (CSA) of patients affected with axonal neuropathy usually shows normal value. Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) seems to represent an exception, showing smaller CSA, but previous reports did not test for biallelic RFC1 gene repeat expansions. METHODS: We compared nerve CSA from CANVAS patients (tested positive for biallelic RFC1 gene repeat expansions) with the CSA from a group of patients with chronic idiopathic axonal polyneuropathy (CIAP) who tested negative for RFC1 gene repeat expansions, hereditary axonal neuropathy (Charcot-Marie-Tooth type 2, CMT2), and Friedreich ataxia (FRDA). RESULTS: We enrolled 15 CANVAS patients (eight men, mean age 66.3 ± 11.5 years, mean disease duration 9.3 ± 4.1 years), affected with sensory axonal neuronopathy. Controls consisted of 13 CIAP (mean age 68.5 ± 12.8 years, seven men), seven CMT2 (mean age 47.9 ± 18.1 years, four men), 12 FRDA (mean age 33.7 ± 8.8, five men). Nerve ultrasound was performed at median, ulnar, sciatic, sural, and tibial nerves and brachial plexus, bilaterally. The nerve CSA from CANVAS patients was significantly smaller than the one from the other cohorts at several sites with significant and high accuracy at Receiver-operating characteristic (ROC) curve analyses. RFC1 AAGGG pentanucleotide expansion, disease duration, and disability did not correlate with CSA at any site, after Bonferroni correction. INTERPRETATION: Decreased sonographic nerve sizes, in arms and legs, in patients with sensory neuropathy and normal motor conduction studies could point to CANVAS-spectrum disease and help guide appropriate genetic testing.

Ultrasound of peripheral nerves distinguishes inherited sensory neuronopathy of cerebellar ataxia with neuropathy and vestibular areflexia syndrome from inherited axonopathy.

Introduction/Aims Recent studies have shown that ultrasound of peripheral nerves can distinguish inherited sensory neuronopathy from acquired axonopathy with a high degree of accuracy. In this study we aimed to determine whether ultrasound can also distinguish inherited sensory neuronopathy from inherited axonopathy. Methods We compared the ultrasound cross-sectional areas (CSAs) of the median, ulnar, sural, and tibial nerves of retrospectively recruited patients with cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS), in whom sensory neuronopathy is a cardinal feature, with Charcot-Marie-Tooth type 2 (CMT2) disease patients, who have an inherited axonopathy, using the Kruskal-Wallis test and receiver-operating characteristic curves. Results There were 17 patients with CANVAS and 18 with CMT2. The upper limb nerve CSAs were significantly smaller in CANVAS than in CMT2 (P < .001), with the CSAs of the median nerve at mid-forearm and ulnar nerve at mid-arm being a third or less the size of those of the CMT2 patients. Nerve ultrasound reliably distinguished CANVAS from CMT2 with ROC areas under the curve between 0.97 and 0.99. The lower limb CSAs of the two patient groups were not significantly different. Discussion Ultrasound of the upper limb nerves distinguishes CANVAS sensory neuronopathy from inherited axonopathy with high accuracy and can therefore be proposed as a reliable additional tool in the investigation of these diseases.

Parkinson's disease and virtual reality rehabilitation: cognitive reserve influences the walking and balance outcome.

INTRODUCTION: Parkinson's disease (PD) is a neurodegenerative pathology characterized by motor and non-motor symptoms that often lead to several impairments. Many studies show the efficacy of different rehabilitation protocols aimed to improve balance and gait functions in PD patients. However, multiple factors may influence rehabilitation outcome. Recently, it has been observed as the cognitive reserve (CR) may influence the rehabilitation outcome, helping to address the patient toward technological or conventional rehabilitation. Our study investigated how CR may affect motor rehabilitation outcomes in PD patients who undergo virtual reality (VR) rehabilitation, aimed at improving walking and balance. MATERIALS AND METHODS: Thirty patients affected by idiopathic PD were enrolled. Patients underwent 12 sessions VR training, over 6 weeks (45 min). Six-Minute Walk Test (6MWT) and Berg Balance Scale (BBS) were used to assess walking and balance, respectively. CR was assessed by Cognitive Reserve Index questionnaire (CRIq). RESULTS: Significant correlations between CR and change from baseline in walking and balance measures were found, with a significant positive correlation between CRIq and 6MWT (r=0.50, p=0.01) and between CRIq and BBS (r=0.41, p=0.04). DISCUSSION: Our results showed that PD patients with higher CR treated with VR improved significantly more in their balance and walking distance than those with lower CR. The current study suggests that VR when aimed to improve balance and walking in PD patients is more effective in patients with higher CR.

State of art in intra-articular hip injections of different medications for osteoarthritis: a systematic review.

BACKGROUND: Intra-articular hip injections for osteoarthritis represent a useful instrument to reduce pain and disability in the common clinical practice. Several medications can be injected locally with different level of evidence-based efficacy. OBJECTIVE: The objective of this systematic review is to investigate the effectiveness of intra-articular injections of different medications or substances for the pain treatment and the management of disability in subjects affected by hip osteoarthritis. METHODS: Two reviewers selected independently randomised controlled trials published in the last 10 years, using PubMed and Scopus databases. The risk of bias was evaluated with Cochrane library assessment tool. RESULTS: 12 randomised controlled trials have been selected. We found 8 papers comparing hyaluronic acid with platelet rich plasma, with corticosteroids and with saline solution; 1 paper compares two types of hyaluronic acid with different molecular weights; 3 papers study the effects of corticosteroids alone or compared to ketorolac or saline solution. CONCLUSIONS: The studies reviewed were heterogeneous regarding sample size, level of osteoarthritis, evaluated with Kellegren-Lawrence score, medications used and follow up timings. However, we have observed that intra-articular injections of platelet-rich plasma seem to decrease pain at short term and disability at long term, in patients affected by hip osteoarthritis better than hyaluronic acid. The association of hyaluronic acid and corticosteroids could give better results compared to hyaluronic acid alone, while the use of intra-articular ketorolac and saline solution requires more studies.

COVID-19 and hospital restrictions: physical disconnection and digital re-connection in disorders of consciousness.

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.

Back Pain in Adolescents: Characteristics, Quality of Life, and Drug Self-Management: A Cross-Sectional Study of 1301 Italian Students.

OBJECTIVES: Back pain is one of the major problems for the public health system in the western world. The purposes of this study were to assess back pain in a large cohort of adolescents; to evaluate the prevalence, intensity, and features; and obtain information about drug management of this symptom. METHODS: One thousand four hundred seventy-one healthy students aged 14 to 19 years were enrolled in the study. The subjects underwent a face-to-face interview using an ad hoc questionnaire, the Numeric Rating Scale, the Neuropathic Pain Symptom Inventory, and the Short-Form 36 questionnaire. RESULTS: Sixty-eight percent of adolescents reported moderate to severe pain, with a higher prevalence of moderate/severe pain in female. The intensity of pain was higher (P < 0.001) in females than in men. A correlation was found between pain and quality of life. Considering the adolescents with severe pain, 21.6% requested a doctor's opinion, and 18% used analgesics (63.2% of them without a doctor's prescription), with a frequency of about 2 times per month. CONCLUSIONS: We found that the prevalence of back pain in adolescents is very high, with the consequences on quality of life, and it becomes frequently a self-managed symptom. This should encourage research on the causes of pain in order to limit the risk factors underlying the pain development and obtain a good prevention strategy.

Effects of exercise training in heart failure with preserved ejection fraction: an updated systematic literature review.

Physical activity is associated with a lower risk of adverse cardiovascular outcomes, including heart failure (HF). Exercise training is a class IA level recommendation in patients with stable HF, but its impact is less clear in heart failure with preserved ejection fraction (HFpEF). The aim of this study was to analyze the effects of the exercise training on cardiovascular outcomes in patients with HFpEF. A systematic literature search was conducted on the main electronic databases, proceedings of major meetings, and reference lists of the identified studies, using specific terms for only English language studies published between 2000 and 2018. We followed the PRISMA to perform our review. Quality of studies was also assessed. The systematic review identified 9 studies on 348 patients, of moderate (n = 2) to good (n = 7) quality. The training consisted of a combination of supervised in-hospital and home-based outpatient programs, including aerobic exercise, endurance and resistance training, walking, and treadmill and bicycle ergometer. Most of the protocols ranged 12-16 weeks, with a frequency of 2-3 sessions weekly, lasting 20-60 min per session. There were significant improvements in peak oxygen uptake, 6-min walking test distance, and ventilatory threshold, whereas quality of life and echocardiographic parameters improved only in some studies. Endothelial function/arterial stiffness remained unchanged. No adverse events were reported. Appropriate exercise programs are able to get a favorable cardiovascular outcome in patients with HFpEF. This could also benefit in terms of quality of life, even if more controversial. Further researches are necessary.

Efficacy of electrical stimulation of denervated muscle: A multicenter, double-blind, randomized clinical trial.

BACKGROUND: This was a multicenter, double-blind, randomized clinical trial to investigate the efficacy of electrical stimulation of denervated muscle (ESDM) on recovery of patients with peripheral nerve injuries. METHODS: We enrolled 38 patients with traumatic peripheral nerve injuries with axonal damage and clinical impairment of two muscles, who were randomly treated with real or sham electrical stimulation (ES). Clinical and neurophysiological examinations were performed before treatment, at the end of treatment, and 3 mo posttreatment, by the same physician who was blinded to the ES allocation. RESULTS: All patients improved but there was no significant beneficial effect of ESDM compared with sham treatment. CONCLUSIONS: This study failed to demonstrate the efficacy of ESDM for peripheral nerve injuries. However, given the large number of variables related to ES and the heterogeneity in disease etiologies and clinical manifestations, future studies on homogeneous populations using different stimulation protocols may be useful.

Ultrasound evaluation of pupil: secrets of the "black hole" unveiled.

This Letter to the Editor is in response to Stevens and colleagues, who presented a study about pupillometry in patients with traumatic brain injury. They did not find any correlation between pupil diameter and intracranial pressure. We agree with the clinical importance of pupil assessment and we would like to suggest the application of transorbital ultrasound for this evaluation. This approach has been proposed in the past and, with our work, we show the possible quantification of symmetry of pupil diameter variation in response to a stimulus. This approach may represent a proficient and safe method for patients' supervision.

Different nerve ultrasound patterns in charcot-marie-tooth types and hereditary neuropathy with liability to pressure palsies.

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.

Ulnar neuropathy at wrist: entrapment at a very "congested" site.

Ulnar tunnel syndrome indicates ulnar neuropathy at different sites within the wrist. Several classifications of ulnar tunnel syndrome are present in literature, based upon typical nerve anatomy. However, anatomical variations are not uncommon and can complicate assessment. The etiology is also complex, due to the numerous potential causes of entrapment. Clinical examination, neurophysiological testing, and imaging are all used to support the diagnosis. At present, many therapeutic approaches are available, ranging from observation to surgical management. Although ulnar neuropathy at the wrist has undergone extensive prior study, unresolved questions on diagnosis and treatment remain. In the current paper, we review relevant literature and present the current knowledge on ulnar tunnel syndrome.

Gender effect on well-being of the oldest old: a survey of nonagenarians living in Tuscany: the Mugello study.

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."

Tarsal tunnel syndrome: still more opinions than evidence. Status of the art.

Tarsal tunnel syndrome is an entrapment neuropathy of the posterior tibial nerve or its terminal branches within its fibro-osseous tunnel beneath the flexor retinaculum on the medial side of the ankle. The condition is frequently underdiagnosed leading to controversies regarding its epidemiology and to an intense debate in the literature. With the advent of nerve imaging techniques, the diagnostic confirmation and the etiological identification have become more accurate. However, management of this entrapment neuropathy remains challenging because of many intervention strategies but limited robust evidence. Uncertainties still exist about the best conservative treatment, timing of surgical intervention, and best surgical approach. In the attempt to clarify these aspects and to provide the reader some understanding of the status of the art, we have reviewed the published literature on this controversial condition.

Thoracic outlet syndrome: wide literature for few cases. Status of the art.

Despite its low prevalence and incidence, considerable debate exists in the literature on thoracic outlet syndrome (TOS). From literature analysis on nerve entrapments, we realized that TOS is the second most commonly published entrapment syndrome in the literature (after carpal tunnel syndrome) and that it is even more reported than ulnar neuropathy at elbow, which, instead, is very frequent. Despite the large amount of articles, there is still controversy regarding its classification, clinical picture, diagnostic objective findings, diagnostic modalities, therapeutical strategies and outcomes. While some experts believe that TOS is underrated, overlooked and very frequent, others even doubt its existence as a nosological entity. In the attempt to shed more light on this condition, we performed a systematic review of the literature and report evidence and opinions around this controversial subject. Only articles focused on neurogenic TOS were considered. Understanding the status of the art and the underlying reasons of doubts and weaknesses could help clinical practice and set the stage for future research.