News Express

Science News: Epidemiology and Natural History of Inclusion Body Myositis A 40-Year Population-Based Study

October 19, 2021

This study determines the prevalence and natural history of sporadic inclusion body myositis (sIBM) and tests the hypothesis that patients with sIBM have higher cancer or mortality rates than the general population.

Science News: Acute Flaccid Myelitis: Long-Term Outcomes Recorded in the CAPTURE Study Compared With Paediatric Transverse Myelitis

October 05, 2021

This is a prospective, multicenter, non-randomized, observational cohort study of children with myelitis seen at academic centers in North America. The study lasted 5 years and the length of follow-up was 1 year. Pediatric patients diagnosed with myelitis were eligible for enrollment in the study within 6 months of onset of symptoms. Patients were classified as transverse myelitis (TM) or the acute flaccid myelitis (AFM) variant based on clinical and radiographic findings.

Science News: Cardiovascular Health and Chronic Axonal Polyneuropathy: A Population-based Study

September 21, 2021

The authors in this study investigated participants of the Rotterdam Study between June 2013 and January 2017, and screened them for polyneuropathy categorized as having no, possible, probable or definite polyneuropathy.

Science News: Tau Protein as a Diagnostic and Prognostic Biomarker in Amyotrophic Lateral Sclerosis

August 29, 2021

This study highlights role of Tau protein as a potential diagnostic and prognostic biomarker in ALS patients.

Science News: Focal Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Onset, Course, and Distinct Features

August 29, 2021

Focal CIDP, described as involvement of brachial or lumbosacral plexus, or one or more peripheral nerves in one upper or lower limb, can pose diagnostic challenge.

Science News: The Value of Sensory Nerve Conduction Studies in the Diagnosis of Guillain-Barré Syndrome

August 18, 2021

Detecting Guillain-Barré syndrome (GBS) early is important for effective treatment. Present electrodiagnostic criteria are exclusively based on motor studies, although sensory testing abnormalities occur early in most patients. This article reviews the utility of sensory testing for GBS.

Science News: Rate of Change in Acetylcholine Receptor Antibody Levels Predicts Myasthenia Gravis Outcome

August 18, 2021

Up to 80% of myasthenia gravis (MG) patients are seropositive for anti-acetylcholine receptor antibodies (AChR-Ab) and these antibodies are routinely checked as diagnostic markers in clinical practice. However, their use in prognosis is thought to be limited.

Science News: Guillain-Barré Syndrome in Association With COVID-19 Infection: A Tale of Two Studies

August 03, 2021

As the COVID-19 pandemic has spread, there have been increasing reports of neurological manifestations. In one study 36.4% patients reported dysgeusia or anosmia, headaches, dizziness, stroke and encephalopathy. In another, up to 84% of patients with severe COVID-19 had neurological complaints. There have been reports of concomitant COVID-19 and GBS which led the authors to the above meta-analysis and multi-center case-control study. 

Science News: Comorbidities, Anthropometric, Demographic, and Lifestyle Risk Factors for Ulnar Neuropathy at the Elbow: A Case Control Study

July 25, 2021

Ulnar neuropathy at the elbow (UNE) is second only to carpal tunnel syndrome as the most frequently encountered focal neuropathy. The clinical presentation of UNE can vary from intermittent fourth and fifth digit paresthesias to anesthesia as well as weakness and atrophy of the ulnar muscles.

Science News: Myotonic Muscular Dystrophy Type 2 in CT, USA: A Single-Center Experience With 50 Patients

July 25, 2021

Myotonic muscular dystrophy type 2 (DM2) is a an autosomal dominant genetic disease stemming from expansion of the first intron of the Cellular Nucleic acid Binding Protein (CNBP) gene which encodes for a zinc finger protein. This disease tends to course with wasting muscle weakness, characteristic myotonia, and cataracts.

Science News: Comparison of Corticosteroid Tapering Regimens in Myasthenia Gravis: A Randomized Clinical Trial

July 13, 2021

This multicenter, parallel, single-blind randomized trial was conducted to compare the efficacy of the standard slow-tapering regimen of prednisone therapy with a rapid-tapering regimen in patients with generalized myasthenia gravis (MG) treated with Azathioprine (n=117).

Science News: Natural History of "Pure" Primary Lateral Sclerosis

July 13, 2021

This retrospective review of pure primary lateral sclerosis (PLS) patients seen at Mayo Clinic-Rochester over a 26 year period (1990-2016), describes clinical characteristics and laboratory findings of 43 patients.

Science News: Value of Systematic Genetic Screening of Patients with Amyotrophic Lateral Sclerosis

June 23, 2021

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease comprising injury and cell death of upper and lower motor neurons in the brainstem, spinal cord and cortex. The authors sought to determine the value added by screening ALS patients for genetic factors.

Science News: Relation of Exercise and Pain in Patients With Idiopathic Distal Axonal Polyneuropathies

June 23, 2021

This cross-sectional study looked at relation between self-reported exercise and severity of neuropathy in IPN.

Science News: Sensory Neuronopathies: A Case Series and Literature Review

June 14, 2021

In the first article, Drs. Amato and Ropper provide an elegant review of sensory neuronopathies. The article is limited to the acquired sensory ganglionopathies and not the rare hereditary ganglionopathies.

Science News: Amyloid Typing by Mass Spectrometry in Clinical Practice: a Comprehensive Review of 16,175 Samples

June 14, 2021

This is a very extensive summary of the experience of the Mayo Clinic over ten years – from 2008 to 2018 with analyzing the congo red positive biopsy samples from throughout the country from a variety of tissues (heart, fat pad, GI, kidney, liver, muscle, nerve, tendons, breast, lymph nodes, bone marrow, etc.) in a way that is able to report even rare amyloid types usually not detected by the standard amyloid immunohistochemistry panels.

Science News: Long-Term Safety and Efficacy of Patisiran for Hereditary Transthyretin-Mediated Amyloidosis With Polyneuropathy: 12-Month Results of an Open-Label Extension Study

May 31, 2021

This article summarizes the world wide experience of treating patients with HTTR with Patisiran in terms of safety and efficacy.

Science News: An Appraisal of Electrodiagnostic Studies in Stiff Person Syndrome

May 16, 2021

This is a review of electrodiagnostic (EDX) findings for diagnosis of stiff person syndrome (SPS). The authors performed a literature review of EDX findings in SPS as well as an online survey on the use of EDX testing for the evaluation of SPS among EDX laboratory test results accredited by the EDX Laboratory Accreditation Committee of the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM).

Science News: Peripheral Nerve Compression Syndromes in Children

April 21, 2021

Non-traumatic neuropathies of the upper limb are rare in children. Diagnostic and treatment parameters are lacking high quality evidence and guidelines.

Science News: Monitoring Clinical Course and Treatment Response in Chronic Inflammatory Demyelinating Polyneuropathy During Routine Care

April 12, 2021

Chronic inflammatory demyelinating polyneuropathy (CIDP) is without a disease activity biomarker and has broad clinical heterogeneity. This review article summarizes clinical and diagnostic tools which can be used in clinical practice to help the clinician to just the outcome of the patient and determine if the treatment is effective or not.