Local corticosteroid injection versus placebo for carpal tunnel syndrome.

BACKGROUND: Carpal tunnel syndrome (CTS) is a very common clinical syndrome manifested by signs and symptoms of irritation of the median nerve at the carpal tunnel in the wrist. Direct and indirect costs of CTS are substantial, with estimated costs of two billion US dollars for CTS surgery in the USA in 1995 alone. Local corticosteroid injection has been used as a non-surgical treatment for CTS many years, but its effectiveness is still debated. OBJECTIVES: To evaluate the benefits and harms of corticosteroids injected in or around the carpal tunnel for the treatment of carpal tunnel syndrome compared to no treatment or a placebo injection. SEARCH METHODS: We used standard, extensive Cochrane search Methods. The searches were 7 June 2020 and 26 May 2022. SELECTION CRITERIA: We included randomised controlled trials (RCTs) or quasi-randomised trials of adults with CTS that included at least one comparison group of local injection of corticosteroid (LCI) into the wrist and one group that received a placebo or no treatment. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcome was 1. improvement in symptoms at up to three months of follow-up. Our secondary outcomes were 2. functional improvement, 3. improvement in symptoms at greater than three months of follow-up, 4. improvement in neurophysiological parameters, 5. improvement in imaging parameters, 6. requirement for carpal tunnel surgery, 7. improvement in quality of life and 8. ADVERSE EVENTS: We used GRADE to assess the certainty of evidence for each outcome. MAIN RESULTS: We included 14 trials with 994 participants/hands with CTS. Only nine studies (639 participants/hands) had useable data quantitatively and in general, these studies were at low risk of bias except for one quite high-risk study. The trials were conducted in hospital-based clinics across North America, Europe, Asia and the Middle East. All trials used participant-reported outcome measures for symptoms, function and quality of life. There is probably an improvement in symptoms measured at up to three months of follow-up favouring LCI (standardised mean difference (SMD) -0.77, 95% confidence interval (CI) -0.94 to -0.59; 8 RCTs, 579 participants; moderate-certainty evidence). Up to six months this was still evident favouring LCI (SMD -0.58, 95% CI -0.89 to -0.28; 4 RCTs, 234 participants/hands; moderate-certainty evidence). There is probably an improvement in function measured at up to three months favouring LCI (SMD -0.62, 95% CI -0.87 to -0.38; 7 RCTs, 499 participants; moderate-certainty evidence). We are uncertain if there is a difference in median nerve DML at up to three months of follow-up (mean difference (MD) -0.37 ms, 95% CI -0.75 to 0.02; 6 RCTs, 359 participants/hands; very low-certainty evidence). The requirement for surgery probably reduces slightly in the LCI group at one year (risk ratio 0.84, 95% CI 0.72 to 0.98; 1 RCT, 111 participants, moderate-certainty evidence). Quality of life, measured at up to three months of follow-up using the Short-Form 6 Dimensions questionnaire (scale from 0.29 to 1.0; higher is better) probably improved slightly in the LCI group (MD 0.07, 95% CI 0.02 to 0.12; 1 RCT, 111 participants; moderate-certainty evidence). Adverse events were uncommon (low-certainty evidence). One study reported 2/364 injections resulted in severe pain which resolved over "several weeks" and 1/364 injections caused a "sympathetic reaction" with a cool, pale hand that completely resolved in 20 minutes. One study (111 participants) reported no serious adverse events, but 65% of LCI-injected and 16% of the placebo-injected participants experienced mild-to-moderate pain lasting less than two weeks. About 9% of participants experienced localised swelling lasting less than two weeks. Four studies (229 participants) reported that they experienced no adverse events in their studies. Three studies (220 participants) did not specifically report adverse events. AUTHORS' CONCLUSIONS: Local corticosteroid injection is effective for the treatment of mild and moderate CTS with benefits lasting up to six months and a reduced need for surgery up to 12 months. Where serious adverse events were reported, they were rare.

Practice Guidelines for Canadian Neurophysiology Laboratories During the COVID-19 Pandemic.

The COVID-19 pandemic has had a major impact on clinical practice. Safe standards of practice are essential to protect health care workers while still allowing them to provide good care. The Canadian Society of Clinical Neurophysiologists, the Canadian Association of Electroneurophysiology Technologists, the Association of Electromyography Technologists of Canada, the Board of Registration of Electromyography Technologists of Canada, and the Canadian Board of Registration of Electroencephalograph Technologists have combined to review current published literature about safe practices for neurophysiology laboratories. Herein, we present the results of our review and provide our expert opinion regarding the safe practice of neurophysiology during the COVID-19 pandemic in Canada.

A National Spinal Muscular Atrophy Registry for Real-World Evidence.

BACKGROUND: Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population. METHODS: The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials. RESULTS: The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner. CONCLUSION: Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.

Access to Allied Health Care Services in Canadian Interdisciplinary Complex Nerve Injury Programs.

Nerve transfer surgery for patients with nerve and spinal cord injuries can result in dramatic functional improvements. As a result, interdisciplinary complex nerve injury programs (CNIPs) have been established in many Canadian centers, providing electrodiagnostic and surgical consultations in a single encounter. We sought to determine which allied health care services are included in Canadian CNIPs, at the 3rd Annual Canadian Peripheral Nerve Symposium. Twenty CNIPs responded to a brief survey and reported access as follows: occupational therapy = 60%, physiotherapy = 40%, social work = 20%, and mental health = 10%. Access to allied health services is variable in CNIPs across Canada, possibly resulting in heterogeneity in patient care.

Amyotrophic lateral sclerosis presentation of a human T-lymphotropic virus type-1 myelopathy--insight into pathogenesis.

A 41-year-old human T-lymphotropic virus type 1-positive woman developed a syndrome with upper and lower motor neuron signs sometime after bilateral vertebral artery dissections. Over 2 years, she developed a progressive myelopathy affecting predominantly the motor system. She had the picture of a 'person in a barrel' and died from complications. At autopsy, spinal cord revealed inflammatory infiltrates and extensive gliosis involving mainly the anterior horns. The vertebral arterial dissections may have permitted the entry of infected lymphocytes and macrophages, secreting cytokines and metalloproteinases, into the medulla progressing to the spinal cord. Few cases with pathological correlation have been reported.

Psoriatic arthritis-associated polyneuropathy: a report of three cases.

Psoriatic arthritis (PA) occurs in about 30% of patients with psoriasis. Although polyneuropathy is described in association with many connective tissue diseases, it is rarely reported in the autoimmune dermatoses. We describe 3 patients with polyneuropathy associated with PA. The clinical and electrophysiologic features are consistent with a chronic distal symmetric sensorimotor axonal process. PA-associated neuropathy should be considered in the differential diagnosis of chronic length-dependent axonal polyneuropathies.

Prevalence of ulnar neuropathy in patients receiving hemodialysis.

BACKGROUND: Ulnar neuropathy can cause pain, weakness, and sensory changes in the hand and can result in functional impairment. Patients with end-stage renal disease receiving hemodialysis may be predisposed to ulnar neuropathy by factors such as arm positioning during hemodialysis, underlying polyneuropathy, and upper extremity vascular access. OBJECTIVE: To determine the prevalence of clinically evident ulnar neuropathy in a cohort of 102 patients with end-stage renal disease receiving hemodialysis. DESIGN: All eligible patients in a single dialysis unit were screened for symptoms and signs of ulnar neuropathy. Those with at least 1 symptom or sign underwent nerve conduction studies to confirm the presence of ulnar neuropathy. RESULTS: Clinically evident, electrophysiologically confirmed ulnar neuropathy was present in 37 (51%) of the 73 subjects with both screening and nerve conduction study data available. The true prevalence of ulnar neuropathy in this cohort was estimated between 41% and 60%. CONCLUSIONS: There is a high prevalence of ulnar neuropathy in patients with end-stage renal disease receiving hemodialysis, which has not been previously recognized. The high prevalence of ulnar neuropathy in this population suggests that preventative efforts are indicated to prevent this functionally limiting complication.

Repetitive nerve stimulation for the evaluation of peripheral nerve hyperexcitability.

OBJECTIVE: To examine the utility of repetitive nerve stimulation (RNS) in the evaluation of peripheral nerve hyperexcitability (PNH). BACKGROUND: PNH describes a group of disorders characterized by muscle cramps, twitching and stiffness. When severe, PNH may be characterized by the presence of continuous muscle fiber activity on routine needle electromyography (EMG). In milder forms of the disease, nerve hyperexcitability may be evidenced by the presence of after-discharges or cramp potentials following RNS. METHODS: Fifty-four patients were prospectively recruited and classified into one of three groups-PNH, other neuromuscular disease and controls. We recorded and quantified the after-discharges and cramp potentials following RNS at 1, 5, 10 and 30 Hz. RESULTS: The proportion of nerves with after-discharges and/or cramp potentials was significantly greater in the PNH group than the control group at both 5 Hz (p=0.03) and 10 Hz (p=0.01), as well as in the neuromuscular disease group compared to controls at 5 Hz (p=0.02). There was also a significant concordance between complaints of muscle cramps and fasciculations and the finding of after-discharges and/or cramp potentials at both 5 Hz (p=0.005) and 10 Hz (p=0.004). At a stimulation frequency of 10 Hz, the sensitivity of RNS for the diagnosis of PNH (primary or secondary) was 79% and the specificity was 88%. CONCLUSION: Our findings suggest that RNS at or below a stimulation frequency of 10 Hz (when positive) is a useful test for the diagnosis of PNH, whether it is primary or secondary.

Pseudomigraine with lymphocytic pleocytosis: a calcium channelopathy? Clinical description of 10 cases and genetic analysis of the familial hemiplegic migraine gene CACNA1A.

OBJECTIVE: To report the clinical findings of 10 patients diagnosed with pseudomigraine with lymphocytic pleocytosis and the results of mutational analysis of the CACNA1A gene in 8 of these patients. BACKGROUND: Pseudomigraine with lymphocytic pleocytosis, also referred to as headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL), is characterized by episodic transient neurologic dysfunction associated with moderate to severe headache and cerebrospinal fluid lymphocytic pleocytosis. Episodes are recurrent and the condition is self-limiting. The etiology of this sporadic condition remains unknown, but the episodic nature and its ability to be triggered by angiography is somewhat reminiscent of the phenotypic features of familial hemiplegic migraine, a condition caused by mutations in the CACNA1A gene. DESIGN/METHODS: Utilizing retrospective chart review, we describe the clinical features of pseudomigraine with lymphocytic pleocytosis in 10 patients. Whole blood was taken from 8 patients (2 were lost to follow-up) and used for DNA testing. The CACNA1A gene was screened for mutations using heteroduplex analysis and direct DNA sequencing. RESULTS: Clinical features of pseudomigraine with lymphocytic pleocytosis included transient episodes of weakness, sensory and visual symptoms, aphasia, and confusion lasting minutes up to 4 hours. Sensory symptoms, typically affecting the face and arm, were the most common presentation. Localization of symptoms did not conform to vascular territories. Headache was typically throbbing and most often bilateral. Genetic analysis did not identify any mutations in the CACNA1A gene. CONCLUSIONS: Similarities between familial hemiplegic migraine and pseudomigraine with lymphocytic pleocytosis include recurrent headache with reversible neurologic deficit, cerebrospinal fluid lymphocytic pleocytosis, and triggers such as angiography. Even so, heteroduplex analysis and DNA sequencing failed to identify any sporadic mutations or shared polymorphisms in the exons or the intron/exon boundaries of the CACNA1A gene. These results do not support a role of the CACNA1A gene in the etiology of pseudomigraine with lymphocytic pleocytosis.