Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy
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Abstract
Objective: Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome.
Methods: A national cross-sectional descriptive study was performed. Ninety-seven patients were identified; 88 participated. Logistic regression analysis was used to study determinants of outcome.
Results: Age at onset was younger in men than in women (38 vs 45 years, p = 0.05). Onset of weakness was in distal arm (61%) or distal leg (34%), and occasionally in the upper arm (5%). Initial diagnosis was motor neuron disease in one-third of patients. Brisk, but not pathologic, reflexes in weakened muscles were found in 8%. Conduction blocks were most frequently detected in the ulnar (80%) and median (77%) nerves, but occasionally only between Erb and axilla (6%), or in the musculocutaneous nerve (1%). Ninety-four percent responded to IVIg therapy: nonresponders had longer disease duration before the first treatment (p = 0.03). Seventy-six percent received IVIg maintenance treatment at the time of this study (median duration 6 years; range 0–17): the median dose increased over the years from 12 to 17 g per week (p < 0.01). Independent determinants of more severe weakness and disability were axon loss (p < 0.001; p < 0.0001) and longer disease duration without IVIg (p = 0.03; p = 0.07).
Conclusion: The results of this study may help aid recognition the clinical picture of MMN. Early IVIg treatment may help to postpone axonal degeneration and permanent deficits.
Classification of evidence: This study provides Class IV evidence that IVIg improves muscle strength of patients with MMN and disability (defined as an increase of ≥1 Medical Research Council grade in at least 2 muscle groups without decrease in other muscle groups) in 94% (95% confidence interval, 86.8%–97.4%) of patients.
Footnotes
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Study funding: Sponsored by an unconditional grant from Baxter International Inc.
Disclosure: Author disclosures are provided at the end of the article.
Received January 5, 2010. Accepted in final form May 17, 2010.
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Letters: Rapid online correspondence
- Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy
- Chafic Karam, Albert Einstein College of Medicine, Beth Israel Medical Center, New York, NY, Beth Israel Medical Center, New York, NY[email protected]
Submitted November 20, 2010 - Reply from the authors
- Elisabeth A. Cats, University Medical Center Utrecht, Heidelberglaan 100 3584 CX Utrecht, the Netherlands[email protected]
- W-Ludo van der Pol (Univ Med Cr Utrecht; [email protected]), Sanne Piepers (Univ Med Ctr Utrecht; [email protected]), Hessel Franssen (University Medical Center Utrecht; [email protected]), Leonard H. van den Berg
Submitted November 20, 2010
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