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Abstract Details

Early Electrophysiological Prediction of Long-Term Disability in Chronic Inflammatory Demyelinating Polyneuropathy – A 14 Year Follow-Up Study
Neuromuscular and Clinical Neurophysiology (EMG)
P2 - Poster Session 2 (8:00 AM-9:00 AM)
1-010
To evaluate electrophysiological measures of axonal loss as correlates of long-term outcome in patients treated for chronic inflammatory demyelinating polyneuropathy (CIDP).
It is a general assumption that axonal loss is the pathophysiological mechanism that underlies the permanent disability in CIDP. However, comparisons of early electrophysiological changes with current clinical and electrophysiological status in the same patients are sparse.

A hospital-based long-term electrophysiological and clinical follow-up study was conducted in 2018 in treated CIDP patients who were studied electrophysiologically for the first time between 1985 and 2006. Motor and sensory nerve conduction studies were carried out at both occasions and the clinical status was evaluated at follow-up using the Inflammatory Rasch-built Overall Disability Scale (I-RODS).

Fourteen CIDP patients were included, five of whom were in remission. The median time since onset of CIDP was 17.5 years (range 12.0 – 30.0) and the median time elapsed between the initial and the follow-up electrophysiological studies was 14.2 years (range 11.4 – 24.2). Twelve patients walked independently, one needed ambulatory support and one had no walking function. The I-RODS was 74.5 (range 28.0 – 100.0) at follow-up. The median combined electrophysiological z-score of motor and sensory action potential amplitudes was -4.7 (range -11.5 – -1.6) at time of diagnosis and -3.4 (range -12.6 – -0.3) at follow-up (p=0.08, ns). Univariate regression analysis revealed a highly significant association between initial axonal loss and current I-RODS, the p-value and coefficient of determination (R2) being <0.0001 and 0.73 respectively. Also, there was a significant association between initial and long-term axonal loss following univariate regression analysis, the p-value and R2 being 0.01 and 0.42, respectively.

Axonal loss at the initial diagnostic electrophysiological examination was predictive for the long-term disability and for the axonal loss at follow-up. In addition, no worsening of the axonal loss was observed at follow-up.
Authors/Disclosures
Ali Al-Zuhairy, MD, PhD
PRESENTER
Dr. Al-Zuhairy has nothing to disclose.
Johannes Jakobsen, MD, DMSci The institution of Dr. Jakobsen has received research support from TAKEDA. Dr. Jakobsen has received publishing royalties from a publication relating to health care. Dr. Jakobsen has received personal compensation in the range of $5,000-$9,999 for serving as a Advisor with Danish Medicine Counsil.
Christian Krarup, MD, DMSc, FRcP, FAAN (Rigshospitalet) Dr. Krarup has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Shire/Takeda. Dr. Krarup has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Shire/Takeda. Dr. Krarup has received publishing royalties from a publication relating to health care.