Abstract Details

Title Pre-treatment Axonal Loss as Biomarker for Long-term Disability in CIDP

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P8 - Poster Session 8 (11:45 AM-12:45 PM)

Poster/Presentation
Number 11-008

Objective To test the hypothesis that initial axonal loss is decisive for long-term disability and function in a consecutive group of CIDP patients.

Background In a recent pilot study of 14 selected CIDP patients it was observed that the amount of pre-treatment axonal loss might predict long-term disability and long-term axonal loss, whereas early demyelination was unrelated to the disability.

Design/Methods EMG and motor and sensory nerve conduction studies were carried out in 30 CIDP patients (22 males) at pre-treatment and at follow-up 5-28 years later. Changes in amplitudes were expressed as axonal Z-scores and changes in conduction as demyelination Z-scores and related to findings at the Inflammatory Rasch-built Overall Disability Scale (I-RODS), the Neuropathy Impairment Score (NIS), and isokinetic dynamometry (IKS%).

Results

At follow-up, the median I-RODS score was 73, the NIS was 23, and the IKS was 56%. The axonal Z-score was unchanged during the follow-up period. Conversely, the corresponding demyelinating Z-scores improved (p=0.02). The initial axonal loss was associated with disability (I-RODS, R²=0.29, p=0.002), neurological impairment (NIS, R²=0.34, p=0.0006) and isokinetic strength (IKS, R²=0.27, p=0.004). Also, axonal loss at follow-up was associated with I-RODS score, NIS and IKS, the R² being 0.22 (p=0.009), 0.47 (p<0.0001) and 0.41 (p=0.0002) respectively. Only the follow-up demyelination Z-score was associated with the I-RODS score, the NIS and the IKS (R²=0.14, 0.34 and 0.15, p=0.04, 0.0007 and 0.03, respectively).

Conclusions

The study indicates that pre-treatment axonal loss in CIDP is predictive of long-term disability, neurological impairment and strength.

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.

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