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Sensory Guillain-Barré Syndrome: Does It Exist?
Sensory symptoms are sometimes the mode of presentation in Guillain-Barré syndrome (GBS), but motor weakness usually develops during the course of the disease. Cases of GBS-like illness with purely sensory symptoms are rare and difficult to diagnose. During a 13-year period, these authors observed 8 patients with acute sensory neuropathy that met most of the diagnostic criteria for sensory GBS.
All patients experienced a sudden onset of sensory symptoms (accompanied by hyporeflexia or areflexia on exam) that reached maximal deficit within 4 weeks. Antecedent viral illness was observed in 5 of 8 patients. Albuminocytologic dissociation was seen in 4 patients whose spinal fluid was examined during the first 4 weeks of disease. All patients had purely sensory findings and no evidence of motor weakness on clinical examination; however, in 7 patients, evidence of demyelination was observed in motor nerve conduction studies, most prominently in prolonged terminal latencies. Abnormal sensory conduction was detected in only 2 patients. Outcome was favorable in all patients. Three patients responded either to prednisone, IV immunoglobulin, or plasma exchange. The authors describe differences in electrophysiologic findings and outcome in sensory GBS versus acute sensory neuronopathy (also called dorsal root ganglionitis) and conclude that they are distinct entities.
Comment: Are sensory GBS and acute sensory neuronopathy different entities? Certainly, both tempo of the illness and outcome in these patients are characteristic of GBS. That the diagnosis of sensory GBS rests on the demonstration of prolonged terminal latencies in motor nerves is ironic, however, because (as the authors state) this may reflect the difficulty in using surface electrodes to demonstrate demyelination in sensory nerve conduction, especially in proximal segments.
Acute sensory neuronopathy is characterized by the absence of sensory nerve action potentials in the presence of motor responses. It remains to be seen whether sensory GBS and acute sensory neuronopathy are associated with different antiglycolipid antibodies, as demonstrated for other GBS variants such as Miller-Fisher syndrome. Overall, these authors provide a lucid description of their patients, a thorough review of the literature, and interesting arguments for the existence of sensory GBS.
B Soliven
Betty Soliven, MD, is Associate Professor of Neurology, University of Chicago, Chicago, IL.
Published in Journal Watch Neurology March 28, 2001
Citation(s):
Oh SJ et al. Sensory Guillain-Barré syndrome. Neurology 2001 Jan 9 56 82-86.
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