Abstract Details

Title Pediatric Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Topic Autoimmune Neurology

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-010

Objective

Study clinical, functional and electrophysiological outcomes in pediatric CIDP.

Background Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare, acquired, immune-mediated neuropathy in children. Steroids, intravenous immunoglobulins (IVIG) and plasma exchange (PLEX) are commonly used. There is paucity of literature on clinical, functional and electrophysiological outcomes.

Design/Methods

Retrospective review of all children with confirmed CIDP diagnoses based on the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria followed in the Neuromuscular/EMG clinics at 6-12 monthly interval from 2012 to 2019. Demographic data, treatment, bilateral grip strength (kg), Rasch-built overall disability scores (RODS), baseline and follow-up motor nerve conduction studies (NCS) for distal latency, conduction velocity, proximal and distal amplitude with or without conduction block were recorded. Paired t-test and Pearson correlation coefficient were calculated.

Results Fifteen children (7 males, 8 females), average age 12.6 years (range 4 to 21 years), 87% (n=13) relapsing-remitting, 7% (n=1) refractory progressive and 7% (n=1) monophasic course were included. All subjects received 1 gm/kg IVIG, 27% (n=4) steroids and 13% (n=2) PLEX. IVIG frequency ranged from 2-3 weekly. Mean follow-up of the cohort was 2 years. Average grip strength (baseline R 17.7, L 15, follow-up R 17, L 17.5 Kg, p=0.64 R, p=0.28 L), RODS (baseline 33.1, follow-up 37.9, maximum 48, p=0.0234), right median motor NCS velocity (baseline 37, follow-up 37.3 m/s, p=0.63), distal abductor pollicis brevis muscle amplitude (baseline 6.9, follow-up 7.1 mv, p=0.73). Pearson correlation coefficients at baseline left and right grip strength, r=0.96 (p<0.0001, n=12), follow-up left and right grip strength r = 0.97 (p<0.0001).

Conclusions

Pediatric CIDP characterized by relapsing remitting course, chronic IVIG use with or without steroids. Pre- and post-treatment RODS questionnaire showed statistical significance. Grip strength, median motor distal amplitude and conduction velocity did not show statistically significant improvement, however, did not decline on therapy.

Authors/Disclosures
Sumit Verma, MD (Emory Children's Center) PRESENTER	Dr. Verma has nothing to disclose.
	No disclosure on file
Ghazal Ahmad, MD (Residence at Riverwatch)	Dr. Ahmad has nothing to disclose.

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Abstract Details