COVID-19: a modern trigger for Guillain-Barre syndrome, myasthenia gravis, and small fiber neuropathy.

COVID-19 infection has had a profound impact on society. During the initial phase of the pandemic, there were several suggestions that COVID-19 may lead to acute and protracted neurologic sequelae. For example, peripheral neuropathies exhibited distinctive features as compared to those observed in critical care illness. The peripheral nervous system, lacking the protection afforded by the blood-brain barrier, has been a particular site of sequelae and complications subsequent to COVID-19 infection, including Guillain-Barre syndrome, myasthenia gravis, and small fiber neuropathy. We will discuss these disorders in terms of their clinical manifestations, diagnosis, and treatment as well as the pathophysiology in relation to COVID-19.

How Do Standing Neutral, Supine Lateral, Standing Flexion, and Standing Extension Radiographs Compare in Detecting the Presence and Magnitude of Stable and Dynamic Spondylolisthesis?

BACKGROUND: Clinical guidelines recommend standing radiographs as the most appropriate imaging for detecting degenerative spondylolisthesis, although reliable evidence about the standing position is absent. To our knowledge, no studies have compared different radiographic views and pairings to detect the presence and magnitude of stable and dynamic spondylolisthesis. QUESTIONS/PURPOSES: (1) What is the percentage of new patients presenting with back or leg pain with stable (3 mm or greater listhesis on standing radiographs) and dynamic (3 mm or greater listhesis difference on standing-supine radiographs) spondylolisthesis? (2) What is the difference in the magnitude of spondylolisthesis between standing and supine radiographs? (3) What is the difference in the magnitude of dynamic translation among flexion-extension, standing-supine, and flexion-supine radiographic pairs? METHODS: This cross-sectional, diagnostic study was performed at an urban, academic institution between September 2010 and July 2016; 579 patients 40 years or older received a standard radiographic three-view series (standing AP, standing lateral, and supine lateral radiographs) at a new patient visit. Of those individuals, 89% (518 of 579) did not have any of the following: history of spinal surgery, evidence of vertebral fracture, scoliosis greater than 30°, or poor image quality. In the absence of a reliable diagnosis of dynamic spondylolisthesis using this three-view series, patients may have had flexion and extension radiographs, and approximately 6% (31 of 518) had flexion and extension radiographs. A total of 53% (272 of 518) of patients were female, and the patients had a mean age of 60 ± 11 years. Listhesis distance (in mm) was measured by two raters as displacement of the posterior surface of the superior vertebral body in relation to the posterior surface of the inferior vertebral body from L1 to S1; interrater and intrarater reliability, assessed with intraclass correlation coefficients, was 0.91 and 0.86 to 0.95, respectively. The percentage of patients with and the magnitude of stable spondylolisthesis was estimated on and compared between standing neutral and supine lateral radiographs. The ability of common pairs of radiographs (flexion-extension, standing-supine, and flexion-supine) to detect dynamic spondylolisthesis was assessed. No single radiographic view or pair was considered the gold standard because stable or dynamic listhesis on any radiographic view is often considered positive in clinical practice. RESULTS: Among 518 patients, the percentage of patients with spondylolisthesis was 40% (95% CI 36% to 44%) on standing radiographs alone, and the percentage of patients with dynamic spondylolisthesis was 11% (95% CI 8% to 13%) on the standing-supine pair. Standing radiographs detected greater listhesis than supine radiographs did (6.5 ± 3.9 mm versus 4.9 ± 3.8 mm, difference 1.7 mm [95% CI 1.2 to 2.1 mm]; p < 0.001). Among 31 patients, no single radiographic pairing identified all patients with dynamic spondylolisthesis. The listhesis difference detected between flexion-extension was no different from the listhesis difference detected between standing-supine (1.8 ± 1.7 mm versus 2.0 ± 2.2 mm, difference 0.2 mm [95% CI -0.5 to 1.0 mm]; p = 0.53) and flexion-supine (1.8 ± 1.7 mm versus 2.5 ± 2.2 mm, difference 0.7 mm [95% CI 0.0 to 1.5]; p = 0.06). CONCLUSION: This study supports current clinical guidelines that lateral radiographs should be obtained with patients in the standing position, because all cases of stable spondylolisthesis of 3 mm or greater were detected on standing radiographs alone. Each radiographic pair did not detect different magnitudes of listhesis, and no single pair detected all cases of dynamic spondylolisthesis. Clinical concern for dynamic spondylolisthesis may justify standing neutral, supine lateral, standing flexion, and standing extension views. Future studies could identify and evaluate a set of radiographic views that provides the greatest capacity to diagnose stable and dynamic spondylolisthesis. LEVEL OF EVIDENCE: Level III, diagnostic study.

Insurance and Employment Status Are Correlated With the Presence of Waddell Signs.

OBJECTIVE: The aim of the study was to determine whether employment or insurance status is associated with the presence and number of Waddell signs. DESIGN: In this cross-sectional study, adult low back pain patients were seen at a tertiary academic center for thoracic or lumbar back pain, due to a degenerative condition. Frequency data were compared with contingency table analysis, including χ2 and logistic regression. RESULTS: Of 462 patients, 26% had any Waddell signs and 10% had clinically significant Waddell signs. Nonemployed patients had a higher prevalence of Waddell signs than employed and retired patients (P = 0.0004 and P = 0.001, respectively). Subgroups of Medicaid participants as well as patients with secondary gain issues, including worker's compensation and motor vehicle accident, had a higher prevalence of 1+ Waddell signs than patients of other insurances (P < 0.0001 and P = 0.01, respectively). Medicaid participants had a higher prevalence of 3+ Waddell signs than other insurances (P = 0.002). CONCLUSIONS: The presence of Waddell signs is associated with employment and insurance status. This suggests that social factors may affect patients' perceptions of their thoracic or low back pain. Clinicians aware of these factors can provide individualized care to their patients prone to poor outcomes.

Physical Activity and the Health of Wheelchair Users: A Systematic Review in Multiple Sclerosis, Cerebral Palsy, and Spinal Cord Injury.

OBJECTIVE: To understand the benefits and harms of physical activity in people who may require a wheelchair with a focus on people with multiple sclerosis (MS), cerebral palsy (CP), and spinal cord injury (SCI). DATA SOURCES: Searches were conducted in MEDLINE, Cumulative Index to Nursing and Allied Health, PsycINFO, Cochrane CENTRAL, and Embase (January 2008 through November 2020). STUDY SELECTION: Randomized controlled trials, nonrandomized trials, and cohort studies of observed physical activity (at least 10 sessions on 10 days) in participants with MS, CP, and SCI. DATA EXTRACTION: We conducted dual data abstraction, quality assessment, and strength of evidence. Measures of physical functioning are reported individually where sufficient data exist and grouped as "function" where data are scant. DATA SYNTHESIS: No studies provided evidence for prevention of cardiovascular conditions, development of diabetes, or obesity. Among 168 included studies, 44% enrolled participants with MS (38% CP, 18% SCI). Studies in MS found walking ability may be improved with treadmill training and multimodal exercises; function may be improved with treadmill, balance exercises, and motion gaming; balance is likely improved with balance exercises and may be improved with aquatic exercises, robot-assisted gait training (RAGT), motion gaming, and multimodal exercises; activities of daily living (ADL), female sexual function, and spasticity may be improved with aquatic therapy; sleep may be improved with aerobic exercises and aerobic fitness with multimodal exercises. In CP, balance may be improved with hippotherapy and motion gaming; function may be improved with cycling, treadmill, and hippotherapy. In SCI, ADL may be improved with RAGT. CONCLUSIONS: Depending on population and type of exercise, physical activity was associated with improvements in walking, function, balance, depression, sleep, ADL, spasticity, female sexual function, and aerobic capacity. Few harms of physical activity were reported in studies. Future studies are needed to address evidence gaps and to confirm findings.

Tendon Transfers for the Treatment of Finger Flexion Weakness in a Patient With Inclusion Body Myositis: A Case Report.

CASE: We describe a 71-year-old patient with inclusion body myositis (IBM), characterized by progressive atrophy and weakness in his left upper extremity. This patient underwent extensor carpi radialis longus to flexor pollicis longus and brachioradialis to flexor digitorum profundus tendon transfers in the left upper extremity to reduce IBM-related functional deficits. He had noticeable improvements in finger flexion after the transfers, which have been sustained for 2 years after the procedure. CONCLUSION: This case reinforces that this novel tendon transfer may be an effective treatment option to improve hand function and activities of daily living in patients with IBM.

CIDP mimics: a case series.

BACKGROUND: To report our experience with a group of patients referred for refractory CIDP who fulfilled "definite" electrodiagnostic EFNS criteria for CIDP but were found to have an alternate diagnosis. METHODS: Patients who were seen between 2017 and 2019 for refractory CIDP that fulfilled "definite" electrodiagnostic ENFS criteria for CIDP, but had an alternate diagnosis, were included. Patients who correctly had CIDP, anti MAG neuropathy, or MMN with conduction block, were excluded from the study. Demographics, clinical and electrophysiological characteristics, pertinent workup, final alternate diagnoses, and outcomes were collected. RESULTS: Seven patients were included: POEMS (n = 5), CANOMAD (n = 1), and neurolymphomatosis (n = 1). Most patients reported neuropathic pain and leg swelling (n = 6) or significant weight loss (n = 4). All patients had a monoclonal protein, and most patients who were tested had an elevated VEGF and CSF cyto-albuminologic dissociation. Electrophysiology showed pronounced intermediate more than distal demyelination, and axonal loss in the lower extremities. Response to steroids or IVIG varied, but some patients did respond to these treatments, especially early in the disease. CONCLUSION: Pain, systemic symptoms, suggestive electrophysiological findings, and/or a serum monoclonal protein should raise suspicion for CIDP mimics. Initial response to steroids or IVIG, over reliance on CSF, and electrophysiology findings can all be misleading.

Superior gluteal nerve injury following landmark-guided corticosteroid injection for greater trochanteric pain: A case report.

This report describes an isolated superior gluteal nerve injection injury following a corticosteroid injection for greater trochanteric pain syndrome. Ultrasound-guided injections may be beneficial to target multiple pain-producing regions of the hip while avoiding nerves and tendons.

Bilateral adhesive capsulitis following influenza vaccination: A case report.

Bilateral shoulder adhesive capsulitis may develop in association with the administration of an influenza vaccine. Vaccine administration should utilize proper technique to avoid injection into the shoulder capsule.

Refractory CIDP: Clinical characteristics, antibodies and response to alternative treatment.

OBJECTIVE: To review the clinical characteristics, antibodies, and response to alternative treatments in a cohort of patients with refractory CIDP. METHODS: We reviewed the charts of all CIDP patients seen at the Oregon Health & Science University neuromuscular clinic between 2017 and 2019. We collected demographics, clinical characteristics, antibodies, and response to treatments. RESULTS: Among 45 CIDP patients studied, 34 (76%) showed improvement with first-line therapy (steroids, IVIG and/or plasmapheresis) and 11 (24%) were considered refractory to first line therapy. Of the latter, 7 of 11 patients (64%) responded to alternative treatment (cyclophosphamide or rituximab). Three were refractory to all treatment. Most patients were ambulatory without aid and a few were in remission. One patient died from complications of alcoholic liver cirrhosis. Thrombosis was seen in three patients receiving IVIG. Six patients (13%) tested positive for Neurofascin (NF) antibodies. Four tested positive for NF155 IgM antibodies only and of those, one responded to IVIG, two partially responded to IVIG and one was refractory. One patient tested positive for NF155 IgG4. Another tested positive for NF155 IgG4 and NF155 IgM. Both patients with IgG4 antibodies were refractory to IVIG, one responded to rituximab and one was refractory to all treatment. CONCLUSION: Less than a quarter of our CIDP patients did not respond to steroids, IVIG, and/or plasmapheresis. Most of the refractory patients responded to rituximab or cyclophosphamide. Patients with IgG4 NF antibodies were resistant to IVIG. The majority of refractory CIDP patients were seronegative and disease management relied on clinical judgement.

Rapid Onset of Femoral Head Osteonecrosis After a Single Intra-articular Hip Joint Injection of Corticosteroid.

We outline a case in which osteonecrosis of the femoral head developed in temporal association with a single intra-articular injection of corticosteroid (triamcinolone acetonide) in a 72-yr-old woman, resulting in a total hip arthroplasty. We conclude that the risk of developing osteonecrosis after a single intra-articular injection of corticosteroid needs to be considered in the informed consent process.

Clinical evaluation of a 31-year-old woman with crural monomelic amyotrophy.

This report describes the clinical presentation of a female patient diagnosed with crural MMA. Careful clinical correlation is necessary to distinguish crural MMA from other motor neuron diseases. When crural MMA is diagnosed, treatment options aim to alleviate symptoms.

Radiation-Induced Painful Neurogenic Hypertrophy Treated With Botulinum Toxin A.

Painful neurogenic hypertrophy is a rare complication of radiation therapy. We report a 27-year-old woman with a history of adenoid cystic carcinoma of the submandibular gland presented with painful twitching of her left shoulder. Electrodiagnostic studies were consistent with a diagnosis of radiation-induced spinal accessory nerve hyperactivity. The patient failed conventional medical therapy. She was treated with an injection of botulinum toxin A, and within 1 month experienced significant relief of symptoms. We thus conclude that Botulinum toxin is a therapeutic option for the pain associated with radiation-induced peripheral nerve hyperactivity.

Evidence-based guideline summary: diagnosis and treatment of limb-girdle and distal dystrophies: report of the guideline development subcommittee of the American Academy of Neurology and the practice issues review panel of the American Association of Neuromuscular & Electrodiagnostic Medicine.

OBJECTIVE: To review the current evidence and make practice recommendations regarding the diagnosis and treatment of limb-girdle muscular dystrophies (LGMDs). METHODS: Systematic review and practice recommendation development using the American Academy of Neurology guideline development process. RESULTS: Most LGMDs are rare, with estimated prevalences ranging from 0.07 per 100,000 to 0.43 per 100,000. The frequency of some muscular dystrophies varies based on the ethnic background of the population studied. Some LGMD subtypes have distinguishing features, including pattern of muscle involvement, cardiac abnormalities, extramuscular involvement, and muscle biopsy findings. The few published therapeutic trials were not designed to establish clinical efficacy of any treatment. PRINCIPAL RECOMMENDATIONS: For patients with suspected muscular dystrophy, clinicians should use a clinical approach to guide genetic diagnosis based on clinical phenotype, inheritance pattern, and associated manifestations (Level B). Clinicians should refer newly diagnosed patients with an LGMD subtype and high risk of cardiac complications for cardiology evaluation even if they are asymptomatic from a cardiac standpoint (Level B). In patients with LGMD with a known high risk of respiratory failure, clinicians should obtain periodic pulmonary function testing (Level B). Clinicians should refer patients with muscular dystrophy to a clinic that has access to multiple specialties designed specifically to care for patients with neuromuscular disorders (Level B). Clinicians should not offer patients with LGMD gene therapy, myoblast transplantation, neutralizing antibody to myostatin, or growth hormone outside of a research study designed to determine efficacy and safety of the treatment (Level R). Detailed results and recommendations are available on the Neurology® Web site at Neurology.org.