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Response to treatment in patients with Lewis-Sumner syndrome
Shahram Attarian1, Annie Verschueren, Jérome Franques
1Department of Neurology and Neuromuscular Disorders, CHU La Timone, 264 rue St. Pierre, Marseille 13385, France. sattarian@ap-hm.fr
Introduction:
Our aim was to document the classification of Lewis-Sumner syndrome (L-SS) based on the response to treatment and the pattern of progression over time.
Methods:
We retrospectively identified 15 patients with L-SS treated for at least 12 months.
Results:
After 1 year, intravenous immunoglobulin was effective in 7 patients, 6 were stable, and 2 worsened. After 5 years, 5 patients had improved, and 3 were stable. These 8 patients needed repeated treatment, and 2 patients gradually worsened. Two patients worsened dramatically after steroid treatment. In 3 patients the neuropathy progressed to the other limbs and developed into an asymmetric polyneuropathy. In 12 other patients the pattern remained multifocal and asymmetric.
Conclusions:
The possibility of decline resulting from corticosteroids, and the fact that no neurological progression toward a diffuse pattern was observed in the majority of patients suggests that L-SS is a different entity from chronic inflammatory demyelinating polyradiculoneuropathy.