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Lewis–Sumner syndrome and multifocal motor neuropathy
Author(s) -
Verschueren Annie,
Azulay Jean Philippe,
Attarian Shahram,
Boucraut José,
Pellissier Jean François,
Pouget Jean
Publication year - 2005
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20236
Subject(s) - mismatch negativity , multifocal motor neuropathy , medicine , fasciculation , electrophysiology , electromyography , weakness , chronic inflammatory demyelinating polyneuropathy , polyradiculoneuropathy , polyneuropathy , audiology , pathology , surgery , anesthesia , electroencephalography , physical medicine and rehabilitation , pediatrics , guillain barre syndrome , immunology , antibody , psychiatry
Abstract We compared the clinical, electrophysiological, laboratory, and pathological features of 13 patients with Lewis–Sumner syndrome (LSS) with those of 20 patients with multifocal motor neuropathy (MMN). LSS and MMN patients have several common clinical features: age at onset, weakness in the distribution of individual peripheral nerves, mild wasting, cramps and fasciculations, partial areflexia, and frequent stepwise disease course. Cerebrospinal fluid protein level was normal or slightly elevated, but always less than 100 mg/dl. Conduction blocks are the electrophysiological hallmarks of these two neuropathies, and no differences in distribution and number of blocks were found. Contrary to MMN, lower‐limb involvement at onset was frequent in LSS but extension to the upper limbs was a frequent later feature of the disease. Cranial nerve involvement was noted in 4 LSS patients during relapses and absent in all MMN patients. The major distinguishing features were the clinical and electrophysiological sensory involvement in LSS, and the lack of anti‐GM1 antibodies in LSS, whereas IgM anti‐GM1 were found in 40% of MMN patients. Some LSS patients responded to steroid therapy, whereas this was ineffective in MMN. From these features, LSS can be considered an entity distinct from MMN, with its own clinical, laboratory, and electrophysiological characteristics, and as an intermediate link between chronic inflammatory demyelinating polyneuropathy and MMN. Muscle Nerve, 2005