Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome.
Lewis-Sumner syndrome (LSS) is a dysimmune peripheral nerve disorder, characterized by a predominantly distal, asymmetric weakness mostly affecting the upper limbs with sensory impairment, and by the presence of multifocal persistent conduction blocks. The nosological position of this neuropathy in relation to multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is still debated. We report the clinical, biological and electrophysiological features, the course and the response to treatment in 23 LSS patients. The initial symptoms started in the distal part of an upper limb in 70% of patients. They were sensorimotor in 65% and purely sensory in 35% of patients. A cranial nerve involvement was observed in 26% of patients and a distal limb amyotrophy in 52%. The CSF protein level was normal in 67% of patients and mildly elevated in the remainder. None had serum anti-GM1 antibodies. There were multiple motor conduction blocks (average of 2.87/patient), predominantly located in the forearm, whereas demyelinating features outside the blocked nerves were rare. Abnormal distal sensory potentials were found in 87% of patients. The electrophysiological pattern suggests a very focal motor fibre demyelination sparing the nerve endings, whereas sensory fibre involvement was widespread. The course was chronic progressive in 71% of patients and relapsing-remitting in the others. During the follow-up study (median duration of 4 years), half of the patients progressed with a multifocal pattern and the distribution of the motor deficit remained similar to the initial presentation. The other patients showed a progression to the other limbs, suggesting a more diffuse process. Fifty-four percent of the patients treated with intravenous immunoglobulin showed an improvement, compared with 33% of the patients treated with oral steroids. Overall, 73% of patients had a positive response to immune-mediated therapy. LSS may be distinguished from MMN by the presence of sensory involvement, the absence of serum anti-GM1 antibodies and, in some cases, a positive response to steroids. In some of the patients in our study, LSS evolved into a more diffuse neuropathy sharing similarities with CIDP. Others had a clinical course characterized by a striking multifocal neuropathy, which suggests underlying mechanisms different from CIDP. Overall, whatever the clinical course, LSS responded to immune-mediated treatment in a manner similar to CIDP.
Viala
K
K
Fédération de Neurophysiologie Clinique, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75651 Paris cedex 13, France. karine.viala@psl.ap-hop-paris.fr
Renié
L
L
Maisonobe
T
T
Béhin
A
A
Neil
J
J
Léger
J M
JM
Bouche
P
P
D000200:Action Potentials / Q000502:physiology; D000328:Adult; D000368:Aged; D001132:Arm / Q000294:innervation; D003711:Demyelinating Diseases / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D018908:Muscle Weakness / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D009412:Nerve Fibers / Q000502:physiology; D009431:Neural Conduction / Q000502:physiology; D009475:Neurons, Afferent / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy; D011596:Psychomotor Disorders / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D012678:Sensation Disorders / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D013577:Syndrome; D016896:Treatment Outcome
D007136:Immunoglobulins