AANEM Education News Express

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

    6/15/2021
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.

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

    6/15/2021
Submitted by: Bryan X. DeSouza, MD & Niranjan N. Singh, MD
Edited by: Hristelina Ilieva, MD, PhD   

Sensory neuronopathies also called sensory ganglionopathies are important disorders to understand especially in the setting of COVID-19 infections, which are characterized by clinical features of acute and subacute loss of smell and taste sensation.

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

    6/1/2021
Submitted by Hristelina Ilieva, MD, PhD                                                           
Edited by Niranjan Singh, MD

This article summarizes the world wide experience of treating patients with HTTR with Patisiran in terms of safety and efficacy. This is a multicenter, open-label extension (OLE) trial that enrolled patients at 43 hospitals or clinical centers in 19 countries.

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

    5/17/2021

Submitted by Betul Gundogdu, MD
Edited by Niranjan Singh, MD

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

    4/22/2021
Non-traumatic neuropathies of the upper limb are rare in children. Diagnostic and treatment parameters are lacking high quality evidence and guidelines.
Carpal tunnel syndrome (CTS) was noted in children with lysosomal storage diseases, and ultrasound (US) was found to be more sensitive than nerve conduction studies (NCSs) for diagnosis; however, NCS was preferred for follow up post operatively.

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

    4/13/2021
Chronic inflammatory demyelinating polyneuropathy (CIDP) is without a disease activity biomarker and has broad clinical heterogeneity.  There are several different metrics used in research trials, but we do not have a standard parameter to use in clinical practice to monitor the response to treatment.

Science News: Comparison Between Rituximab Treatment for New-Onset Generalized Myasthenia Gravis and Refractory Generalized Myasthenia Gravis

    3/30/2021
This was a retrospective cohort study conducted in a university hospital in Sweden to compare efficacy between rituximab and conventional immunosuppressants in the refractory and newly diagnosed generalized myasthenia gravis.

Science News: Which Ultrasound Parameter is More Accurate in the Diagnosis of Carpal Tunnel Syndrome

    3/18/2021
Ultrasound (US) can be useful to diagnosing carpal tunnel syndrome (CTS). Most US CTS studies have looked at median nerve cross sectional area (CSA). Others use resistive index (RI) or strain ratio (SR) as the criteria. This study compared CSA with RI and SR in CTS.

Science News: Peripheral Nerve Compression Syndromes in Children

    3/1/2021
Non-traumatic neuropathies of the upper limb are rare in children. Therefore, high quality evidence-based diagnostic guidelines are lacking.



 

Science News: Serum Neurofilament Light Chain Predicts Long-Term Prognosis in Guillain-Barré Syndrome Patients

    2/17/2021
This is presented by the International GBS Outcome Study (IGOS), based in Spain. The neurofilament light (NfL) chain is an increasingly studied biomarker for axonal injury, previously studied in GBS. This study evaluates it as a prognostic maker in GBS.