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. 2015 Aug 6;2(5):e138.
doi: 10.1212/NXI.0000000000000138. eCollection 2015 Oct.

CSF cytokine profile distinguishes multifocal motor neuropathy from progressive muscular atrophy

Takahiro Furukawa  1 , Naoko Matsui  1 , Koji Fujita  1 , Hiroyuki Nodera  1 , Fumitaka Shimizu  1 , Katsuichi Miyamoto  1 , Yukitoshi Takahashi  1 , Takashi Kanda  1 , Susumu Kusunoki  1 , Yuishin Izumi  1 , Ryuji Kaji  1
Affiliations

CSF cytokine profile distinguishes multifocal motor neuropathy from progressive muscular atrophy

Takahiro Furukawa et al. Neurol Neuroimmunol Neuroinflamm. .

Abstract

Objective: We aimed to compare the cytokine and chemokine profiles of patients with multifocal motor neuropathy (MMN) with those of patients with progressive muscular atrophy (PMA) and amyotrophic lateral sclerosis (ALS) to investigate immunologic differences in the CNS.

Methods: CSF from 12 patients with MMN, 8 with PMA, 26 with sporadic ALS, and 10 with other noninflammatory neurologic disorders was analyzed for 27 cytokines and chemokines using the multiplex bead array assay. Cytokine titers of the 4 groups were compared, and correlations between the titers of relevant cytokines and clinical parameters were evaluated.

Results: There were no obvious intrathecal changes except for interleukin (IL)-1 receptor antagonist in patients with MMN. In contrast, IL-4, IL-7, IL-17, eotaxin/CCL11, fibroblast growth factor-2 (FGF-2), granulocyte colony-stimulating factor (G-CSF), and platelet-derived growth factor BB titers were significantly elevated in patients with PMA and ALS; of these, FGF-2 and G-CSF titers were elevated compared with those in patients with MMN. IL-4 and IL-10 titers were high in patients with ALS, particularly patients with possible ALS presenting with a slowly progressive course or mild symptoms.

Conclusions: The CSF cytokine profile of patients with MMN is distinct from that of patients with PMA and ALS. The similarity of the cytokine profiles between patients with PMA and ALS suggests that PMA shares common immunologic features with ALS in the CNS, even without clinical evidence of upper motor neuron involvement.

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Figures

Figure 1
Figure 1. Cytokine titers determined by the multiplex bead assay
Plotted are cytokine titers (pg/mL) in CSF of patients with other noninflammatory neurologic diseases (ONDs, n = 10), multifocal motor neuropathy (MMN, n = 12), progressive muscular atrophy (PMA, n = 8), and amyotrophic lateral sclerosis (ALS, n = 26).*p < 0.05, **p < 0.01. FGF-2 = fibroblast growth factor-2; G-CSF = granulocyte colony-stimulating factor; IL = interleukin; IL-1ra = IL-1 receptor antagonist; IP-10 = interferon-γ–induced protein 10; MCP-1 = monocyte chemotactic protein-1; PDGF-BB = platelet-derived growth factor BB; TNF-α = tumor necrosis factor α; VEGF = vascular endothelial growth factor.
Figure 2
Figure 2. Correlation analysis and representative cytokine titers
Left column shows correlations between cytokine levels in CSF and clinical parameters. Right column shows CSF cytokine titers (pg/mL) in multifocal motor neuropathy (MMN, n = 12), progressive muscular atrophy (PMA, n = 8), possible amyotrophic lateral sclerosis (ALS, n = 8), and ALS except possible ALS (n = 18). ALSFRS-R = revised Amyotrophic Lateral Sclerosis Functional Rating Scale; IL = interleukin.
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