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Lewis-Sumner syndrome and multifocal motor neuropathy
Annie Verschueren1, Jean Philippe Azulay, Shahram Attarian
1Department of Neurology and Neuromuscular Diseases, Timone Hospital, Boulevard Jean Moulin, 13005 Marseille, France. annie.verschueren@ap-hm.fr <annie.verschueren@ap-hm.fr>
Muscle & Nerve
|November 13, 2004
Summary
Lewis-Sumner syndrome (LSS) and multifocal motor neuropathy (MMN) share features but differ significantly. LSS exhibits sensory involvement and responds to steroids, unlike MMN, establishing it as a distinct neurological disorder.
Area of Science:
- Neurology
- Clinical Electrophysiology
- Immunology
Background:
- Lewis-Sumner syndrome (LSS) and multifocal motor neuropathy (MMN) are rare demyelinating neuropathies.
- Distinguishing between LSS and MMN is crucial for appropriate diagnosis and management.
Purpose of the Study:
- To compare the clinical, electrophysiological, laboratory, and pathological features of LSS and MMN.
- To identify key differentiating characteristics between LSS and MMN.
Main Methods:
- Comparative analysis of 13 LSS patients and 20 MMN patients.
- Evaluation of clinical presentation, electrophysiological findings (conduction blocks), laboratory results (CSF protein, antibodies), and response to therapy.
Main Results:
- Both LSS and MMN show common features like age at onset, weakness, mild wasting, cramps, fasciculations, partial areflexia, and stepwise disease course.
- Conduction blocks are hallmarks in both, with similar distribution and number.
- Key distinctions include sensory involvement in LSS (clinical and electrophysiological), frequent lower-limb onset in LSS, cranial nerve involvement in LSS relapses, absence of anti-GM1 antibodies in LSS, and steroid responsiveness in LSS versus MMN.
Conclusions:
- Lewis-Sumner syndrome is a distinct entity from multifocal motor neuropathy.
- LSS presents unique clinical and electrophysiological characteristics, including sensory deficits.
- LSS may represent an intermediate condition between chronic inflammatory demyelinating polyneuropathy and MMN.