Lewis-Sumner syndrome and multifocal motor neuropathy.
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.
Verschueren
Annie
A
Department of Neurology and Neuromuscular Diseases, Timone Hospital, Boulevard Jean Moulin, 13005 Marseille, France. annie.verschueren@ap-hm.fr <annie.verschueren@ap-hm.fr>
Azulay
Jean Philippe
JP
Attarian
Shahram
S
Boucraut
José
J
Pellissier
Jean François
JF
Pouget
Jean
J
D000200:Action Potentials; D000328:Adult; D001706:Biopsy; D002556:Cerebrospinal Fluid Proteins / Q000378:metabolism; D003711:Demyelinating Diseases / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology*; D009046:Motor Neurons / Q000473:pathology; D009475:Neurons, Afferent / Q000473:pathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology*; D012189:Retrospective Studies; D013256:Steroids / Q000008:administration & dosage
D002556:Cerebrospinal Fluid Proteins; D007166:Immunosuppressive Agents; D013256:Steroids