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Progression of Dysarthria and Dysphagia in Postmortem-Confirmed Parkinsonian Disorders
Jörg Müller, MD;
Gregor K. Wenning, MD, PhD;
Marc Verny, MD;
Ann McKee, MD;
K. Ray Chaudhuri, MD;
Kurt Jellinger, MD;
Werner Poewe, MD;
Irene Litvan, MD
Arch Neurol. 2001;58:259-264.
Background Dysarthria and dysphagia are known to occur in parkinsonian syndromes
such as Parkinson disease (PD), dementia with Lewy bodies (DLB), corticobasal
degeneration (CBD), multiple system atrophy (MSA), and progressive supranuclear
palsy (PSP). Differences in the evolution of these symptoms have not been
studied systematically in postmortem-confirmed cases.
Objective To study differences in the evolution of dysarthria and dysphagia in
postmortem-confirmed parkinsonian disorders.
Patients and Methods Eighty-three pathologically confirmed cases (PD, n = 17; MSA, n = 15;
DLB, n = 14; PSP, n = 24; and CBD, n = 13) formed the basis for a multicenter
clinicopathological study organized by the National Institute of Neurological
Disorders and Stroke, Bethesda, Md. Cases with enough clinicopathological
documentation for the purpose of the study were selected from research and
neuropathological files of 7 medical centers in 4 countries (Austria, France,
England, and the United States).
Results Median dysarthria latencies were short in PSP and MSA (24 months each),
intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months).
Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months),
CBD (64 months), and MSA (67 months), and long in PD (130 months). Dysarthria
or dysphagia within 1 year of disease onset was a distinguishing feature for
atypical parkinsonian disorders (APDs) (specificity, 100%) but failed to further
distinguish among the APDs. Survival time after onset of a complaint of dysphagia
was similar in PD, MSA, and PSP (15 to 24 months, P
= .7) and latency to a complaint of dysphagia was highly correlated with total
survival time ( = 0.88; P<.001) in all disorders.
Conclusions Latency to onset of dysarthria and dysphagia clearly differentiated
PD from the APDs, but did not help distinguish different APDs. Survival after
onset of dysphagia was similarly poor among all parkinsonian disorders. Evaluation
and adequate treatment of patients with PD who complain of dysphagia might
prevent or delay complications such as aspiration pneumonia, which in turn
may improve quality of life and increase survival time.
From the Department of Neurology, University Hospital Innsbruck, Innsbruck,
Austria (Drs Müller, Wenning, and Poewe); Raymond Escourolle Neuropathology
Laboratory, Hôpital de la Salpétrière, Paris, France (Dr
Verny); the Veterans Administration Medical Center Geriatric Research Education
Clinic Center, Bedford, Mass, and the Departments of Neurology and Pathology,
Boston University Medical School, Boston, Mass (Dr McKee); the Department
of Neurology, Institute of Psychiatry, London, England (Dr Chaudhuri); Ludwig
Boltzmann Institute of Clinical Neurology, Vienna, Austria (Dr Jellinger);
and the Cognitive Neuropharmacology Unit, Defense and Veteran Head Injury
Program, Henry M. Jackson Foundation and Medical Neurology Branch, National
Institute of Neurological Disorders and Stroke, National Institutes of Health,
Bethesda, Md (Dr Litvan).
Corresponding author: Irene Litvan, MD, Cognitive Neuropharmacology
Unit, The Champlain Building, 6410 Rockledge Dr, Suite 600, Bethesda, MD 20817-1844.
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