Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome.
We report 11 patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, defined clinically by a multifocal pattern of motor and sensory loss, with nerve conduction studies showing conduction block and other features of demyelination. The clinical, laboratory, and histological features of these patients were contrasted with those of 16 patients with multifocal motor neuropathy (MMN). Eighty-two percent of MADSAM neuropathy patients had elevated protein concentrations in the cerebrospinal fluid, compared with 9% of the MMN patients (P < 0.001). No MADSAM neuropathy patient had elevated anti-GM1 antibody titers, compared with 56% of MMN patients (P < 0.01). In contrast to the subtle abnormalities described for MMN, MADSAM neuropathy patients had prominent demyelination on sensory nerve biopsies. Response to intravenous immunoglobulin treatment was similar in both groups (P = 1.0). Multifocal motor neuropathy patients typically do not respond to prednisone, but 3 of 6 MADSAM neuropathy patients improved with prednisone. MADSAM neuropathy more closely resembles chronic inflammatory demyelinating polyneuropathy and probably represents an asymmetrical variant. Given their different clinical patterns and responses to treatment, it is important to distinguish between MADSAM neuropathy and MMN.
Saperstein
D S
DS
Department of Neurology, University of Texas Southwestern Medical Center, Dallas, USA.
Amato
A A
AA
Wolfe
G I
GI
Katz
J S
JS
Nations
S P
SP
Jackson
C E
CE
Bryan
W W
WW
Burns
D K
DK
Barohn
R J
RJ
D000328:Adult; D000368:Aged; D001706:Biopsy; D003711:Demyelinating Diseases / Q000145:classification* / Q000175:diagnosis* / Q000628:therapy; D003937:Diagnosis, Differential; D004568:Electrodiagnosis; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous; D008297:Male; D008475:Median Nerve / Q000473:pathology / Q000502:physiology; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000145:classification* / Q000175:diagnosis* / Q000628:therapy; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000502:physiology; D010543:Peroneal Nerve / Q000473:pathology / Q000502:physiology; D011826:Radial Nerve / Q000473:pathology / Q000502:physiology; D013497:Sural Nerve / Q000473:pathology / Q000502:physiology; D013979:Tibial Nerve / Q000473:pathology / Q000502:physiology; D014459:Ulnar Nerve / Q000473:pathology / Q000502:physiology
D016756:Immunoglobulins, Intravenous