AANEM Education News Express

AANEM News Express

Science News: Motor Performance Deterioration Accelerates After 50 Years of Age in Charcot-Marie-Tooth Type 1A Patients

    5/31/2018
Submitted by Nandita S. Keole, MD, News Science Editorial Board

The aim of this study was to describe, by a case control and cross-sectional design, the correlation between clinical impairment and age in Charcot-Marie-Tooth type 1A (CMT1A) patients.

Science News - Diagnosis and Management of SMA Part 1: Recommendations for Diagnosis, Rehabilitation, Orthopedic, and Nutritional Care

    5/25/2018
Submitted by Leigh Maria K. Ramos-Platt, MD, News Science Editorial Board   
Additional comments by David B. Rosenfield, MD, News Science Editorial Board


In December 2016, the spinal muscular atrophy (SMA) community recognized the need for the SMA Standards of Care (SOC) document to be updated. Part 1 of the updated set of guidelines was recently published and this summary highlights those updates. 

Science News: Nusinersen Versus Sham Control in Later-onset Spinal Muscular Atrophy

    5/15/2018
Submitted by David R. Mayans, MD, News Science Editorial Board  
Additional comments by David B. Rosenfield, MD, News Science Editorial Board


This is another study adding to the growing body of evidence that Nusinersen is effective at not only slowing the progression of SMA, but leading to improvements in strength and gain of function in SMA patients. 

Science News: Associated Conditions in Small Fiber Neuropathy – A Large Cohort Study and Review of the Literature

    5/10/2018
Submitted by Nandita S. Keole, MD, News Science Editorial Board 
Additional comments by David B. Rosenfield, MD, News Science Editorial Board


Small fiber neuropathy (SFN) is a common disorder leading to neuropathic pain and autonomic symptoms. The objective of this study was to investigate associated conditions in a large cohort of SFN patients and compare the prevalence to healthy individuals. 

Science News: Brain-derived Neurotrophic Factor Derived from Sensory Neurons Plays a Critical Role in Chronic Pain

    5/3/2018
Submitted by Francisco E. Gomez, MD, News Science Editorial Board 
Additional comments by David B. Rosenfield, MD, News Science Editorial Board


In this ambitious study, investigators sought to determine the role of sensory neuron – brain-derived neurotrophic factor (BDNF) – in the genesis of chronic pain.

May is ALS Awareness Month and GBS/CIDP Awareness Month!

    5/1/2018
AANEM is pleased to play a role in spreading the word on amyotrophic lateral sclerosis (ALS), Guillain-Barré syndrome (GBS), and chronic inflammatory demyelinating polyneuropathy (CIDP) during ALS Awareness Month and GBS/CIDP Awareness Month this May. Read this article for further information about each of these diseases and learn about available AANEM resources to help expand your knowledge.  

Science News: Immune or Genetic-mediated Disruption of CASPR2 Causes Pain Hypersensitivity Due to Enhanced Primary Afferent Excitability

    5/1/2018
Submitted by Shan (Sarah) Chen, MD, PhD, News Science Editorial Board
Additional comments by David B. Rosenfield, MD, News Science Editorial Board


In this article in Neuron, the authors discuss that contactin-associated protein-like 2 (CASPR2) antibodies cause loss of Kv1 channel membrane expression and hyperexcitability in Dorsal Root Ganglion (DRG) without preexisting nerve injury or inflammation. These authors demonstrate in mice that one can passively transfer an autoimmune peripheral neuropathic disorder in which CASPR2 has an important role in regulating DRG excitability and posit that these antibodies might even have a role in autism spectrum disorders.


Science News: Two Reports on Spinal Muscular Atrophy Treatments - Nusinersen and Gene Replacement Therapy

    3/26/2018
Recent articles in the New England Journal of Medicine provide insight into spinal muscular atrophy treatments involving Nusinersen and gene replacement therapy.

Science News - Natural History of Charcot-Marie-Tooth Disease During Childhood: Natural History of Pediatric CMT

    3/21/2018
Submitted by John C. Kincaid, MD, News Science Editorial Board
Edited by Nandita S. Keole, MD, News Science Editorial Board


This summary provides details on a study that evaluated a group of 200 pediatric patients (ages 3- 20 years) with genetically classified types of Charcot-Marie-Tooth (CMT) disease assessed serially over a 2-year period using the CMT Pediatric Scale (CMT PedS). It provides information about the rate of natural progression of deficits in the CMT subtypes.


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