Profound olfactory dysfunction in myasthenia gravis

• Published in: PloS one Vol. 7; no. 10; p. e45544
• Main Authors: Leon-Sarmiento, Fidias E, Bayona, Edgardo A, Bayona-Prieto, Jaime, Osman, Allen, Doty, Richard L
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 17-10-2012; Public Library of Science (PLoS)
• Subjects: Acetylcholine receptors; Acetylcholine receptors (muscarinic); Age; Alzheimer's disease; Antibodies; Biology; Blood-brain barrier; Brain research; Case-Control Studies; Central nervous system; Cognitive ability; Disease control; Female; Health aspects; Humans; Immunoglobulins; Inhalation; Male; Medicine; Middle Aged; Myasthenia; Myasthenia gravis; Myasthenia Gravis - physiopathology; Nervous system; Neurodegenerative diseases; Neurological diseases; Neuromuscular diseases; Neuromuscular junctions; Olfaction; Olfaction disorders; Otolaryngology; Parkinson's disease; Patients; Respiration; Risk factors; Rodents; Sleep; Smell; Smell - physiology; Surgery; Synaptic transmission; Thymectomy; United States > US; Pennsylvania
• DOI: 10.1371/journal.pone.0045544
• EISSN: 1932-6203

In this study we demonstrate that myasthenia gravis, an autoimmune disease strongly identified with deficient acetylcholine receptor transmission at the post-synaptic neuromuscular junction, is accompanied by a profound loss of olfactory function. Twenty-seven MG patients, 27 matched healthy controls, and 11 patients with polymiositis, a disease with peripheral neuromuscular symptoms analogous to myasthenia gravis with no known central nervous system involvement, were tested. All were administered the University of Pennsylvania Smell Identification Test (UPSIT) and the Picture Identification Test (PIT), a test analogous in content and form to the UPSIT designed to control for non-olfactory cognitive confounds. The UPSIT scores of the myasthenia gravis patients were markedly lower than those of the age- and sex-matched normal controls [respective means (SDs) =20.15 (6.40) & 35.67 (4.95); p<0.0001], as well as those of the polymiositis patients who scored slightly below the normal range [33.30 (1.42); p<0.0001]. The latter finding, along with direct monitoring of the inhalation of the patients during testing, implies that the MG-related olfactory deficit is unlikely due to difficulties sniffing, per se. All PIT scores were within or near the normal range, although subtle deficits were apparent in both the MG and PM patients, conceivably reflecting influences of mild cognitive impairment. No relationships between performance on the UPSIT and thymectomy, time since diagnosis, type of treatment regimen, or the presence or absence of serum anti-nicotinic or muscarinic antibodies were apparent. Our findings suggest that MG influences olfactory function to the same degree as observed in a number of neurodegenerative diseases in which central nervous system cholinergic dysfunction has been documented.