Abstract Details

Title Upper Extremity Peripheral Nerve Ultrasound Shows Proximal to Distal Tapering of Nerves in Demyelinating Charcot-Marie-Tooth Disease but Not in Acquired Inflammatory Demyelinating Neuropathies

Topic Peripheral Nerve

Presentation(s) (-)

Poster/Presentation
Number 004

Objective

Background Differentiating demyelinating CMT from acquired demyelinating neuropathies may be challenging. Prior reports of nerve ultrasound in patients with demyelinating CMT and inflammatory demyelinating neuropathies have shown nerve enlargement.

Design/Methods Consecutive patients with demyelinating CMT (n=8) or acquired demyelinating neuropathy (chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, acute inflammatory demyelinating polyradiculoneuropathy) of less than 20 years duration (n=15) were recruited. Controls (n=12) with normal electrodiagnostic studies were included for comparison. Both groups underwent blinded US imaging of the median and ulnar nerves. Nerve cross sectional area (CSA) was measured at the wrist, forearm, elbow and axilla. Axillary CSA/Wrist CSA ratio, mean CSA, and CSA-Var (maximal CSA/minimal CSA) were also calculated.

Results Mean CSA and CSA-Var of the median nerve were significantly increased in both inherited (24.2 vs. 8.69mm2, p=0.002 and 2.21 vs.1.4, p=0.04) and acquired demyelinating neuropathies (12.23 vs. 8.69mm2, p=0.003, and 2.17 vs.1.4, p= 0.0002). Mean CSA and CSA-Var of the ulnar nerve were significantly increased in acquired demyelinating neuropathies (8.7 vs. 7.08mm2, p=0.04 and 2.2 vs.1.6, p=0.04). Mean CSA, but not CSA-Var, of the ulnar nerve was significantly increased in inherited demyelinating neuropathies (14.45 vs. 7.08mm2, p=0.01and 1.93 vs. 1.6, p=0.25). The axillary/wrist CSA ratio was significantly increased in demyelinating CMT patients for both median (1.67 vs. 0.96, p=0.035) and ulnar (1.91 vs. 1.46, p=0.02) nerves, but not in patients with acquired demyelinating neuropathies.

Conclusions While increased nerve size and size variability is found in both hereditary and acquired demyelinating neuropathies, only those with hereditary demyelinating neuropathy demonstrated proximal to distal tapering of nerve size. This sonographic feature may help distinguish inherited from acquired demyelinating neuropathies.

Authors/Disclosures
Hani Kushlaf, MD, FAAN (University of Cincinnati) PRESENTER	Dr. Kushlaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion AstraZeneca Rare Disease. Dr. Kushlaf has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Kushlaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Catalyst Pharmaceuticals. Dr. Kushlaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Kushlaf has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta Therapeutics . Dr. Kushlaf has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amicus Therapeutics .
Vern C. Juel, MD, FAAN (Duke University Medical Center)	Dr. Juel has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunovant. Dr. Juel has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Juel has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Accordant Health Services. Dr. Juel has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen. The institution of Dr. Juel has received research support from argenx. The institution of Dr. Juel has received research support from Alexion. The institution of Dr. Juel has received research support from Janssen. The institution of Dr. Juel has received research support from NIH Rare Diseases Network.
Lisa Hobson-Webb, MD, FAAN (Duke University Medical Center)	An immediate family member of Dr. Hobson-Webb has received personal compensation for serving as an employee of G1 Therapeutics. Dr. Hobson-Webb has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley Publishing. The institution of Dr. Hobson-Webb has received research support from Sanofi Genzyme.
Fay B. Horak, PhD (OHSU)	No disclosure on file

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