Abstract Details

Title Impairment and Disability in 20 CIDP Patients According to Disease Activity Status

Topic Peripheral Nerve

Presentation(s) (-)

Poster/Presentation
Number 006

Objective

Background CIDP is a rare dysimmune neuropathy with a large clinical heterogeneity leading to various degrees of disability. In an ongoing study dedicated to gene expression analysis from skin biopsies, 20 CIDP patients have been rated prospectively according to different scores and scales assessing impairment and disability.

Design/Methods Patients were diagnosed according to EFNS/PNS criteria and to disease activity status (CDAS). A Principal Component (PC) analysis and the correlation between the following scores were performed: Neurological Symptom Score (NSS); MRC sum score (MRC); Neurological Disability Score (NDS); Hammersmith Functional Motor Scale (HFMS); Inflammatory Neuropathy Cause and Treatment Sensory Sumscore (ISS); INCAT Overall Disability Sum Score (ODSS); INCAT Disability score (INCAT DS); Rasch-built Overall Disability Scale (R-ODS).

Results According to CDAS, most patients had stable disease (50%) or were in remission (35%); only 2 had unstable active disease. The first PC that best explained the variability within the cohort consisted of: CDAS, general disability scores (R-ODS, INCAT DS, ODSS and NDS), and motor scores (MRC, HFMS and motor component of NDS); these parameters also strongly correlated between them. A second PC explained to a lesser extent the variability of the group and consisted of sensory scores (ISS, sensory component of NDS), but these parameters did not correlate with the scores from the first PC, nor with CDAS.

Conclusions The evaluated scores outline 2 major clinically useful groups: 1) sensory scores (ISS, sensory component of NDS) and 2) disability and motor scores. The most useful scores appear the ones evaluating the disability, R-ODS topping the list, correlating both with motor disability and the disease activity. In contrast, the sensory score does not seem to fully evaluate the disease severity in the studied cohort.

Authors/Disclosures
Thierry Kuntzer, MD (Lausanne University Hospital CHUV) PRESENTER	No disclosure on file
	No disclosure on file
Susanne Renaud, MD	No disclosure on file
Andreas J. Steck, MD, FAAN	No disclosure on file
Neill R. Graff-Radford, MD, FAAN (Mayo Clinic Jacksonville)	The institution of Dr. Graff-Radford has received research support from Biogen. The institution of Dr. Graff-Radford has received research support from Lilly. The institution of Dr. Graff-Radford has received research support from Eisai. The institution of Dr. Graff-Radford has received research support from Biogen. Dr. Graff-Radford has received publishing royalties from a publication relating to health care.

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