AANEM News Express Related to  NSEB

AANEM News Express

Science News: Utility of Shear Wave Elastography and High-Definition Color for Diagnosing Carpal Tunnel Syndrome

This prospective study investigated shear wave elastography and high definition color in carpal tunnel syndrome (CTS) patients with variable degrees of severity. The authors recruited patients with clinical evidence of CTS and healthy volunteers as a control group. The patients underwent EDX testing, and the severity of CTS was determined using Canterbury grading (6 grades). The patients were subsequently classified into nerve conduction study (NCS)-negative (normal NCS), mild-to-moderate CTS (grades 1–3), and severe CTS (grades 4–6).

Science News: Amyotrophic Lateral Sclerosis Mortality Rates in Latin America and the Caribbean: A Meta-Analysis

Heterogeneity of amyotrophic lateral sclerosis (ALS) epidemiology has been noted amongst geographic population. Previous studies have shown lower frequency of ALS amongst non Caucasian descents. Furthermore, interesting findings like lowest mortality from ALS in mixed ancestry has been noted. This study conducted a meta-analysis of population-based data to describe ALS mortality rates in Latin America.

Science News: A New Tool for Assessment of Motor Axon Excitability. A Multicenter Prospective Study

This study is a multicenter study that assessed a novel electrodiagnostic test, ‘iMAX’, to test the motor axon excitability. The study was performed in four centers in Belgium and France. The authors aimed to evaluate the test reliability and consistency among the four centers, provide normative values, and determine its ability to detect impaired motor axon excitability in patients with peripheral neuropathies. 

Science News: Dermatomyositis: Muscle Pathology According to Antibody Subtypes

Dermatoyositis (DM) is a rare disease that causes muscle weakness and skin rash. This study provides good evidence for a variety of DM subtypes and highlights the importance of looking at myxovirus resistant protein expression in muscle biopsies when evaluating inflammatory myopathies.

Science News: ALS in Finland: Major Genetic Variants and Clinical Characteristics of Patients With and Without the C9orf72 Hexanucleotide Repeat Expansion

In this cohort study, the authors analyzed the frequency of major genetic variants and clinical features of patient with amyotrophic lateral sclerosis (ALS) with or without C9orf72 repeat expansion. The article explains the correlation between ethnicity and genotype phenotype correlations. 

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

Submitted by: Niranjan N. Singh, MD
Edited by: Betul Gondogdu, MD

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 treated with Azathioprine (n=117). The slow-tapering arm included a gradual increase of the prednisone dose to 1.5 mg/kg every other day and a slow decrease once remission was achieved.

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

Submitted by: Hristelina Ilieva, MD, PhD
Edited by: Betul Gondogdu, MD

This retrospective review of pure PLS patients seen at Mayo Clinic-Rochester over a 26 year period (1990-2016), describes clinical characteristics and laboratory findings of 43 patients. Of these, 25 (58%) were female; median onset-age was 50 years (range: 38-78), and median follow-up duration was 9 years (range:4- 36).

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

Submitted by: Francisco Gomez, MD
Edited by: Hristelina Ilieva, MD, PhD

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.  Overt respiratory failure and death occur on average at 2–5 years of onset.

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

Submitted by: Pritikanta Paul, MD
Edited by: Hristelina Ilevia, MD, PhD

Benefits of exercise in peripheral neuropathy related to diabetes mellitus or metabolic syndrome were studied. Exercise improves glycemic control and control of other metabolic measures resulting into improved small fiber function and reducing risk of developing neuropathy.

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

Submitted by: Hristelina Ilieva, MD, PhD
Edited by: Niranjan Singh, MD

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 IHC (immunohistochemistry) panels.