Abstract
Objective: Multifocal motor neuropathy (MMN) is a rare inflammatory neuropathy, clinically characterized by exclusive motor involvement. We wished to evaluate the possible presence of sensory dysfunction, including the evaluation of small fibres, after a long-term disease course. Patients and methods: seven MMN patients, regularly followed in our Neurology Department, underwent clinical evaluation, neurophysiological examination by nerve conduction studies (NCSs), and Sudoscan. We compared neurophysiological data with a group of patients with other disorders of the peripheral nervous system. Results: NCSs showed a reduction of sensory nerve action potential amplitude in 2/7 MMN patients. Sudoscan showed borderline electrochemical skin conductance (ESC) values in 3/7 MMN patients (two of them with abnormal sensory NCSs). Conclusions: Our results confirm that sensory involvement may be found in some MMN after a long-term disease course, and it could also involve the small fibres.
Highlights
Multifocal motor neuropathy (MMN) is a rare disorder in which focal areas of multiple motor nerves are attacked by one’s own immune system [1,2,3]
In this study, we examined a cohort of MMN patients, regularly followed in our department, in order to investigate the possible presence of small fibre dysfunction after a long-term disease course by the use of Sudoscan
Summary
MMN (multifocal motor neuropathy) is a rare disorder in which focal areas of multiple motor nerves are attacked by one’s own immune system [1,2,3]. Antibodies to ganglioside GM1 are reported in 40–85% of cases [4,5,6,7]. MMN is a slowly progressive disorder, resulting in asymmetric limb weakness; patients frequently develop weakness in their hand(s), resulting in dropping of objects or sometimes inability to turn a key in a lock. The weakness associated with MMN can be recognized as fitting a specific nerve territory. Corticosteroids and plasma exchange are not effective, while treatment with intravenous immunoglobulin (IVIg) and/or cyclophosphamide generally delays or stops disease progression [8,9,10,11]
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