Lewis- Sumner Syndrome – a Case Report

Česká verzia

Authors: I. Okáčová ¹; B. Mičánkováihash2 ^{1,2 1,2}
Authors place of work: Neurologická klinika LF MU a FN Brno ¹; CEITEC – Středoevropský technologický institut, MU, Brno ²; Ústav patologie a patologické anatomie LF MU a FN Brno ³; Radiologická klinika LF MU a FN Brno ⁴
Published in the journal: Cesk Slov Neurol N 2012; 75/108(4): 498-502
Category: Case Report

Summary

Lewis-Sumner syndrome, also known as Multifocal Acquired Demyelinating Sensory And Motor neuropathy (MADSAM), is considered to be a focal asymmetric variant of the chronic inflammatory demyelinating polyneuropathy. The aim of this case report is to discuss this rare disease, its differential diagnosis and available treatment modalities.

Case report:
50 years old man experienced subacute onset of impaired sensation and weakness in his left hand. EMG examination revealed focal chronic sensory and motor lesion of median, ulnar and radial nerves of the left hand (primary demyelination with partial motor block), with signs of chronic axonopathy, located between axilla and the Erb’s point. MRI of the left brachial plexus showed spinal root enlargement. Sural nerve biopsy showed signs of demyelination. CSF was normal, except for a marked elevation of protein. Serum anti-GM1 antibody titer was below cut-off. Differential diagnosis included MMN (Multifocal Motor Neuropathy), hereditary neuropathy with liability to pressure palsy and neurofibroma of brachial plexus. The patient did not respond to corticosteroid treatment. Treatment with intravenous immunoglobulins led to clinical and electrophysiological improvement.

Key words:
Lewis-Sumner syndrome – multifocal motor neuropathy – chronic inflammatory demyelinating neuropathy

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