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Original Contributions |

Beneficial Plasma Exchange Response in Central Nervous System Inflammatory Demyelination

Setty M. Magaña, BS; B. Mark Keegan, MD; Brian G. Weinshenker, MD; Bradley J. Erickson, MD, PhD; Sean J. Pittock, MD; Vanda A. Lennon, MD, PhD; Moses Rodriguez, MD; Kristine Thomsen, BA; Stephen Weigand, MS; Jay Mandrekar, PhD; Linda Linbo, RN; Claudia F. Lucchinetti, MD
Arch Neurol. 2011;68(7):870-878. doi:10.1001/archneurol.2011.34.
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Background Plasma exchange (PLEX) is a beneficial rescue therapy for acute, steroid-refractory central nervous system inflammatory demyelinating disease (CNS-IDD). Despite the approximately 45% PLEX response rate reported among patients with CNS-IDD, determinants of interindividual differences in PLEX response are not well characterized.

Objective To perform an exploratory analysis of clinical, radiographic, and serological features associated with beneficial PLEX response.

Design Historical cohort study.

Setting Neurology practice, Mayo Clinic College of Medicine, Rochester, Minnesota.

Patients All Mayo Clinic patients treated with PLEX between January 5, 1999, and November 12, 2007, for a steroid-refractory CNS-IDD attack.

Main Outcome Measure The PLEX response in attack-related, targeted neurological deficit(s) assessed within the 6-month period following PLEX.

Results We identified 153 patients treated with PLEX for a steroid-refractory CNS-IDD, of whom 90 (59%) exhibited moderate to marked functional neurological improvement within 6 months following treatment. Pre-PLEX clinical features associated with a beneficial PLEX response were shorter disease duration (P = .02) and preserved deep tendon reflexes (P = .001); post-PLEX variables included a diagnosis of relapsing-remitting multiple sclerosis (P = .008) and a lower Expanded Disability Status Scale score (P < .001) at last follow-up. Plasma exchange was less effective for patients with multiple sclerosis who subsequently developed a progressive disease course (P = .046). Radiographic features associated with a beneficial PLEX response were presence of ring-enhancing lesions (odds ratio = 4.00; P = .03) and/or mass effect (odds ratio = 3.00; P = .02). No association was found between neuromyelitis optica–IgG serostatus and PLEX response.

Conclusions We have identified clinical and radiographic features that may aid in identifying patients with fulminant, steroid-refractory CNS-IDD attacks who are more likely to respond to PLEX.

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Figure 1. Ascertainment of plasma exchange (PLEX) cohort and sample size for each analysis. †Exclusions included receiving fewer than 2 exchanges (n = 2) and receiving PLEX for an attack related to a coexisting condition and not for a central nervous system inflammatory demyelinating disease (CNS-IDD) attack (diabetic neuropathy, n = 1; polycythemia vera, n = 1). MRI indicates magnetic resonance imaging.

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Figure 2. Frequency of presenting symptoms at the index attack (A) and plasma exchange response rate by presenting symptom among all patients with that symptom (B). Most patients had polysymptomatic attacks and thus could have more than 1 presenting symptom.

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Figure 3. Representative gadolinium enhancement patterns, including homogeneous (A), heterogeneous (B), diffuse or patchy (C), fluffy or cotton ball (D), punctate (<2 mm) (E), nodular (>2 mm) (F), open ring to cortex (G), and arc with colocalization of the ring-enhancing lesion (arrow) (H), and T2-weighted hypointense rim (arrow) colocalizing with ring enhancement (I). A-H, T1-weighted gadolinium-enhanced sequences.

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Figure 4. Plasma exchange response rate with 95% confidence interval (A) and odds ratio (OR) with 95% confidence interval (B) by brain magnetic resonance imaging feature on the largest lesion. A, Bars indicate the plasma exchange response rate; lines, 95% confidence interval. P values are based on unadjusted logistic regression models. B, The adjusted model was adjusted for days to plasma exchange on the log-transformed scale, Expanded Disability Status Scale score at the time of plasma exchange, and deep tendon reflexes. RELs indicates ring-enhancing lesions.

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