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Volume 72, Number 19, May 12, 2009
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NEUROLOGY 2009;72:1677-1681
© 2009 American Academy of Neurology

Hyposmia in pure autonomic failure

L. Silveira-Moriyama, MD, C. Mathias, MB, DPhil, DSc, FMedSci, L. Mason, BSc(Hons), C. Best, RMN, N. P. Quinn, MD and A. J. Lees, MD

From the Reta Lila Weston Institute of Neurological Studies and Queen Square Brain Bank for Neurological Diseases (L.S.-M., A.J.L.) and Sobell Department of Motor Neuroscience and Movement Disorders (N.P.Q.), UCL Institute of Neurology; Autonomic Unit (C.M., L.M., C.B.), National Hospital for Neurology and Neurosurgery, UCL Hospitals Trust and Institute of Neurology; and Neurovascular Medicine Unit (C.M.), Faculty of Medicine, Imperial College, St. Mary's Hospital, London, UK.

Address correspondence and reprint requests to Prof. Andrew Lees, Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, 1 Wakefield St., London, WC1N 1PJ, UK a.lees{at}ion.ucl.ac.uk.

Background: Parkinson disease (PD), multiple system atrophy (MSA), and pure autonomic failure (PAF) all present with varying degrees of dysautonomia and are pathologically characterized by accumulation of {alpha}-synuclein. Hyposmia and olfactory pathway pathology are found in PD and MSA. We tested odor identification in 16 patients with PAF and compared the results with those found in patients with PD, patients with MSA, and control subjects.

Methods: The University of Pennsylvania Smell Identification Test (UPSIT) was used to evaluate the sense of smell in 16 patients with PAF, 14 patients with MSA, 191 patients with PD, and 145 control subjects. Multiple linear regression analyses were used to evaluate the effect of the diseases on the mean UPSIT score when adjusted for age, sex, and smoking habit.

Results: The mean UPSIT score was higher in the controls than in patients with PAF (p < 0.001) or MSA (p < 0.001); it was lower in patients with PD than in patients with PAF (p = 0.005) or patients with MSA (p = 0.006); and no difference was found between patients with MSA and patients with PAF (p = 0.9) when adjusted for age, gender, and smoking habits.

Conclusions: Hyposmia may be a feature of pure autonomic failure (PAF), but to a lesser degree than that found in Parkinson disease. Further research into the olfactory pathways in patients with PAF is warranted.

Abbreviations: DLB = dementia with Lewy bodies; MSA = multiple system atrophy; PAF = pure autonomic failure; PD = Parkinson disease; UPSIT = University of Pennsylvania Smell Identification Test.


Supplemental data at www.neurology.org.

Supported by the Reta Lila Weston Trust for Medical Research. Dr. Silveira-Moriyama is a beneficiary of a Reta Lila Weston fellowship.

Disclosure: The authors report no disclosures.

Received December 11, 2008. Accepted in final form February 10, 2009.







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