Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

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 (rho = 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.

[1]  L. Golbe,et al.  Prevalence and natural history of progressive supranuclear palsy , 1988, Neurology.

[2]  Irene Litvan,et al.  Progressive supranuclear palsy : clinical and research approaches , 1992 .

[3]  M. Woodward,et al.  Asymptomatic swallowing disorders in elderly patients with Parkinson's disease: a description of findings on clinical examination and videofluoroscopy in sixteen patients. , 1994, Age and ageing.

[4]  Americal Psychiatric Press Diagnostic and Statistical Manual of Mental Disorders, Third Edition , 1980 .

[5]  R. Loewenson,et al.  Parkinson's disease , 1973, Neurology.

[6]  H. Kirshner,et al.  Swallowing and speech production in Parkinson's disease , 1986, Annals of neurology.

[7]  S. Fahn Unified Parkinson's Disease Rating Scale , 1987 .

[8]  S. Fahn,et al.  Comparison of extrapyramidal features in 31 pathologically confirmed cases of diffuse lewy body disease and 34 pathologically confirmed cases of parkinson's disease , 1997, Neurology.

[9]  P. Ashby,et al.  Cortical‐basal ganglionic degeneration , 1990, Neurology.

[10]  M. Norenberg,et al.  Diffuse Lewy body disease and progressive dementia , 1988, Neurology.

[11]  I Litvan,et al.  Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination , 1998, Journal of neurology, neurosurgery, and psychiatry.

[12]  P. Lantos,et al.  Cortical Lewy body dementia: clinical features and classification. , 1989, Journal of neurology, neurosurgery, and psychiatry.

[13]  N. Quinn,et al.  Multiple system atrophy--the nature of the beast. , 1989, Journal of neurology, neurosurgery, and psychiatry.

[14]  K. Jellinger,et al.  Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. , 1996, Neurology.

[15]  R. Hichwa,et al.  Cerebellar and brainstem hypometabolism in olivopontocerebellar atrophy detected with positron emission tomography , 1988, Annals of neurology.

[16]  K. Marder,et al.  Cortical basal ganglionic degeneration. , 2001, Science of aging knowledge environment : SAGE KE.

[17]  Y. Agid,et al.  “Pure” striatonigral degeneration and Parkinson's disease: A comparative clinical study , 1995, Movement disorders : official journal of the Movement Disorder Society.

[18]  N. Quinn,et al.  Multiple system atrophy: A review of 203 pathologically proven cases , 1997, Movement disorders : official journal of the Movement Disorder Society.

[19]  J. Olszewski,et al.  Progressive Supranuclear Palsy: A Heterogeneous Degeneration Involving the Brain Stem, Basal Ganglia and Cerebellum With Vertical Gaze and Pseudobulbar Palsy, Nuchal Dystonia and Dementia , 1964 .

[20]  N P Quinn,et al.  Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. , 1994, Brain : a journal of neurology.

[21]  A. Rajput,et al.  Progressive Supranuclear Palsy , 2001, Drugs & aging.

[22]  C D Marsden,et al.  Corticobasal degeneration: A clinical study of 36 cases , 1994 .

[23]  E. Tolosa,et al.  Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). , 1994, Journal of neural transmission. Supplementum.

[24]  G. Halliday,et al.  Diffuse Lewy body disease: clinical features in nine cases without coexistent Alzheimer's disease. , 1996, Journal of neurology, neurosurgery, and psychiatry.

[25]  S. Fahn,et al.  Parkinsonian features of eight pathologically diagnosed cases of diffuse lewy body disease , 1995, Movement disorders : official journal of the Movement Disorder Society.

[26]  M. Hoehn,et al.  Parkinsonism , 1967, Neurology.

[27]  M. Hallett,et al.  Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) , 1996, Neurology.

[28]  I Litvan,et al.  Validity and Reliability of the Preliminary NINDS Neuropathologic Criteria for Progressive Supranuclear Palsy and Related Disorders , 1996, Journal of neuropathology and experimental neurology.

[29]  S. Fahn Members of the UPDRS Development Committee. Unified Parkinson's Disease Rating Scale , 1987 .

[30]  I. Litvan,et al.  Characterizing swallowing abnormalities in progressive supranuclear palsy , 1997, Neurology.

[31]  A. Colcher,et al.  Comparison of swallowing function in Parkinson's disease and progressive supranuclear palsy , 1997, Movement disorders : official journal of the Movement Disorder Society.

[32]  B. Sonies Swallowing and speech disturbances , 1992 .

[33]  L Junck,et al.  Characteristics of the dysarthria of multiple system atrophy. , 1996, Archives of neurology.

[34]  C. Marsden,et al.  Clinical presentation and pharmacological therapy in corticobasal degeneration. , 1998, Archives of neurology.

[35]  F Lhermitte,et al.  Does long‐term aggravation of Parkinson's disease result from nondopaminergic lesions? , 1987, Neurology.

[36]  E. Quigley,et al.  Gastrointestinal symptoms in Parkinson's disease , 1991, Movement disorders : official journal of the Movement Disorder Society.

[37]  R. Godwin-Austen,et al.  Diffuse Lewy body disease: clinical features in 15 cases. , 1989, Journal of neurology, neurosurgery, and psychiatry.

[38]  J. Perlmutter,et al.  Swallowing abnormalities and their response to treatment in Parkinson's disease , 1989, Neurology.

[39]  A. Bonnet,et al.  [The Unified Parkinson's Disease Rating Scale]. , 2000, Revue neurologique.