Multifocal acquired demyelinating sensory and motor neuropathy presenting as a peripheral nerve tumor.
A man with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), or Lewis-Sumner syndrome, presented with a progressive left lumbosacral plexus lesion resembling a neurofibroma. After 7 years he developed a left ulnar nerve lesion with conduction block in its upper segment. Treatment with intravenous immunoglobulin improved the symptoms and signs of both lesions. We conclude that inflammatory neuropathy must be considered in the differential diagnosis of peripheral nerve tumors, and that unifocal lesions may precede multifocal involvement in MADSAM by several years. In addition, we discuss the clinical features in 9 patients attending a specialist peripheral nerve clinic and review the literature.
Allen
David C
DC
Department of Clinical Neurophysiology, Kings College Hospital, Denmark Hill Campus, London SE5 9RS, UK. davidcallen@doctors.org.uk
Smallman
Clare A
CA
Mills
Kerry R
KR
D020516:Brachial Plexus Neuropathies / Q000473:pathology; D003711:Demyelinating Diseases / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004568:Electrodiagnosis; D006801:Humans; D008160:Lumbosacral Plexus / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000175:diagnosis; D009431:Neural Conduction; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed; D014459:Ulnar Nerve / Q000000981:diagnostic imaging