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Neurol Neuroimmunol Neuroinflamm. 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.

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Department of Clinical Neuroscience (T.F., N.M., K.F., H.N., Y.I., R.K.), Institute of Health Biosciences, Tokushima University, Tokushima, Japan; Department of Neurology and Clinical Neuroscience (F.S., T.K.), Yamaguchi University Graduate School of Medicine, Ube, Japan; Department of Neurology (K.M., S.K.), Kinki University School of Medicine, Osaka, Japan; and National Epilepsy Center (Y.T.), Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan.



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.


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.


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.


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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