Abstract
Background: Formal laboratory testing of autonomic function is reported to distinguish between patients with Parkinson's disease and those with multiple system atrophy (MSA), but such studies segregate patients according to clinical criteria that select those with autonomic dysfunction for the MSA category.
Objective: To characterise the profiles of autonomic disturbances in patients in whom the diagnosis of Parkinson's disease or MSA used criteria other than autonomic dysfunction.
Methods: 47 patients with parkinsonism and autonomic symptoms who had undergone autonomic laboratory testing were identified and their case records reviewed for non-autonomic features. They were classified clinically into three diagnostic groups: Parkinson's disease (19), MSA (14), and uncertain (14). The performance of the patients with Parkinson's disease was compared with that of the MSA patients on five autonomic tests: RR variation on deep breathing, heart rate changes with the Valsalva manoeuvre, tilt table testing, the sudomotor axon reflex test, and thermoregulatory sweat testing.
Results: None of the tests distinguished one group from the other with any statistical significance, alone or in combination. Parkinson's disease and MSA patients showed similar patterns of autonomic dysfunction on formal testing of cardiac sympathetic and parasympathetic, vasomotor, and central and peripheral sudomotor functions.
Conclusions: This study supports the clinical observation that Parkinson's disease is often indistinguishable from MSA when it involves the autonomic nervous system. The clinical combination of parkinsonism and dysautonomia is as likely to be caused by Parkinson's disease as by MSA. Current clinical criteria for Parkinson's disease and MSA that direct patients with dysautonomia into the MSA group may be inappropriate.
Full Text
The Full Text of this article is available as a PDF (203.6 KB).
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Bannister R., Oppenheimer D. R. Degenerative diseases of the nervous system associated with autonomic failure. Brain. 1972;95(3):457–474. doi: 10.1093/brain/95.3.457. [DOI] [PubMed] [Google Scholar]
- Benarroch E. E., Schmeichel A. M., Parisi J. E. Involvement of the ventrolateral medulla in parkinsonism with autonomic failure. Neurology. 2000 Feb 22;54(4):963–968. doi: 10.1212/wnl.54.4.963. [DOI] [PubMed] [Google Scholar]
- Bonnet A. M., Pichon J., Vidailhet M., Gouider-Khouja N., Robain G., Perrigot M., Agid Y. Urinary disturbances in striatonigral degeneration and Parkinson's disease: clinical and urodynamic aspects. Mov Disord. 1997 Jul;12(4):509–513. doi: 10.1002/mds.870120406. [DOI] [PubMed] [Google Scholar]
- Bordet R., Benhadjali J., Destee A., Hurtevent J. F., Bourriez J. L., Guieu J. D. Sympathetic skin response and R-R interval variability in multiple system atrophy and idiopathic Parkinson's disease. Mov Disord. 1996 May;11(3):268–272. doi: 10.1002/mds.870110309. [DOI] [PubMed] [Google Scholar]
- Burkhardt C. R., Filley C. M., Kleinschmidt-DeMasters B. K., de la Monte S., Norenberg M. D., Schneck S. A. Diffuse Lewy body disease and progressive dementia. Neurology. 1988 Oct;38(10):1520–1528. doi: 10.1212/wnl.38.10.1520. [DOI] [PubMed] [Google Scholar]
- Byrne E. J., Lennox G., Lowe J., Godwin-Austen R. B. Diffuse Lewy body disease: clinical features in 15 cases. J Neurol Neurosurg Psychiatry. 1989 Jun;52(6):709–717. doi: 10.1136/jnnp.52.6.709. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Cohen J., Low P., Fealey R., Sheps S., Jiang N. S. Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. Ann Neurol. 1987 Dec;22(6):692–699. doi: 10.1002/ana.410220604. [DOI] [PubMed] [Google Scholar]
- Colosimo C., Albanese A., Hughes A. J., de Bruin V. M., Lees A. J. Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson's disease. Arch Neurol. 1995 Mar;52(3):294–298. doi: 10.1001/archneur.1995.00540270090024. [DOI] [PubMed] [Google Scholar]
- De Marinis M., Stocchi F., Gregori B., Accornero N. Sympathetic skin response and cardiovascular autonomic function tests in Parkinson's disease and multiple system atrophy with autonomic failure. Mov Disord. 2000 Nov;15(6):1215–1220. doi: 10.1002/1531-8257(200011)15:6<1215::aid-mds1023>3.0.co;2-j. [DOI] [PubMed] [Google Scholar]
- Gelb D. J., Oliver E., Gilman S. Diagnostic criteria for Parkinson disease. Arch Neurol. 1999 Jan;56(1):33–39. doi: 10.1001/archneur.56.1.33. [DOI] [PubMed] [Google Scholar]
- Gibb W. R., Lees A. J. The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease. J Neurol Neurosurg Psychiatry. 1988 Jun;51(6):745–752. doi: 10.1136/jnnp.51.6.745. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Gilman S., Low P. A., Quinn N., Albanese A., Ben-Shlomo Y., Fowler C. J., Kaufmann H., Klockgether T., Lang A. E., Lantos P. L. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. 1999 Feb 1;163(1):94–98. doi: 10.1016/s0022-510x(98)00304-9. [DOI] [PubMed] [Google Scholar]
- Gilman S., Low P. A., Quinn N., Albanese A., Ben-Shlomo Y., Fowler C. J., Kaufmann H., Klockgether T., Lang A. E., Lantos P. L. Consensus statement on the diagnosis of multiple system atrophy. J Auton Nerv Syst. 1998 Dec 11;74(2-3):189–192. [PubMed] [Google Scholar]
- Hayashi M., Isozaki E., Oda M., Tanabe H., Kimura J. Loss of large myelinated nerve fibres of the recurrent laryngeal nerve in patients with multiple system atrophy and vocal cord palsy. J Neurol Neurosurg Psychiatry. 1997 Mar;62(3):234–238. doi: 10.1136/jnnp.62.3.234. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Hely M. A., Reid W. G., Halliday G. M., McRitchie D. A., Leicester J., Joffe R., Brooks W., Broe G. A., Morris J. G. Diffuse Lewy body disease: clinical features in nine cases without coexistent Alzheimer's disease. J Neurol Neurosurg Psychiatry. 1996 May;60(5):531–538. doi: 10.1136/jnnp.60.5.531. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Holmberg B., Kallio M., Johnels B., Elam M. Cardiovascular reflex testing contributes to clinical evaluation and differential diagnosis of Parkinsonian syndromes. Mov Disord. 2001 Mar;16(2):217–225. doi: 10.1002/mds.1062. [DOI] [PubMed] [Google Scholar]
- Hughes A. J., Ben-Shlomo Y., Daniel S. E., Lees A. J. What features improve the accuracy of clinical diagnosis in Parkinson's disease: a clinicopathologic study. Neurology. 1992 Jun;42(6):1142–1146. doi: 10.1212/wnl.42.6.1142. [DOI] [PubMed] [Google Scholar]
- Hughes A. J., Daniel S. E., Blankson S., Lees A. J. A clinicopathologic study of 100 cases of Parkinson's disease. Arch Neurol. 1993 Feb;50(2):140–148. doi: 10.1001/archneur.1993.00540020018011. [DOI] [PubMed] [Google Scholar]
- Hughes A. J., Daniel S. E., Kilford L., Lees A. J. Accuracy of clinical diagnosis of idiopathic Parkinson's disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry. 1992 Mar;55(3):181–184. doi: 10.1136/jnnp.55.3.181. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Klein C., Brown R., Wenning G., Quinn N. The "cold hands sign" in multiple system atrophy. Mov Disord. 1997 Jul;12(4):514–518. doi: 10.1002/mds.870120407. [DOI] [PubMed] [Google Scholar]
- Kosaka K. Diffuse Lewy body disease in Japan. J Neurol. 1990 Jun;237(3):197–204. doi: 10.1007/BF00314594. [DOI] [PubMed] [Google Scholar]
- Kuzuhara S., Yoshimura M. Clinical and neuropathological aspects of diffuse Lewy body disease in the elderly. Adv Neurol. 1993;60:464–469. [PubMed] [Google Scholar]
- Magalhães M., Wenning G. K., Daniel S. E., Quinn N. P. Autonomic dysfunction in pathologically confirmed multiple system atrophy and idiopathic Parkinson's disease--a retrospective comparison. Acta Neurol Scand. 1995 Feb;91(2):98–102. doi: 10.1111/j.1600-0404.1995.tb00414.x. [DOI] [PubMed] [Google Scholar]
- Mathias C. J., Mallipeddi R., Bleasdale-Barr K. Symptoms associated with orthostatic hypotension in pure autonomic failure and multiple system atrophy. J Neurol. 1999 Oct;246(10):893–898. doi: 10.1007/s004150050479. [DOI] [PubMed] [Google Scholar]
- Pakiam A. S., Bergeron C., Lang A. E. Diffuse Lewy body disease presenting as multiple system atrophy. Can J Neurol Sci. 1999 May;26(2):127–131. [PubMed] [Google Scholar]
- Pierangeli G., Provini F., Maltoni P., Barletta G., Contin M., Lugaresi E., Montagna P., Cortelli P. Nocturnal body core temperature falls in Parkinson's disease but not in Multiple-System Atrophy. Mov Disord. 2001 Mar;16(2):226–232. doi: 10.1002/mds.1039. [DOI] [PubMed] [Google Scholar]
- Quinn N. Disproportionate antecollis in multiple system atrophy. Lancet. 1989 Apr 15;1(8642):844–844. doi: 10.1016/s0140-6736(89)92300-3. [DOI] [PubMed] [Google Scholar]
- Quinn N. Multiple system atrophy--the nature of the beast. J Neurol Neurosurg Psychiatry. 1989 Jun;Suppl:78–89. doi: 10.1136/jnnp.52.suppl.78. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Rajput A. H., Rozdilsky B., Ang L. Occurrence of resting tremor in Parkinson's disease. Neurology. 1991 Aug;41(8):1298–1299. doi: 10.1212/wnl.41.8.1298. [DOI] [PubMed] [Google Scholar]
- Rajput A. H., Rozdilsky B. Dysautonomia in Parkinsonism: a clinicopathological study. J Neurol Neurosurg Psychiatry. 1976 Nov;39(11):1092–1100. doi: 10.1136/jnnp.39.11.1092. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Rajput A. H., Rozdilsky B., Rajput A. Accuracy of clinical diagnosis in parkinsonism--a prospective study. Can J Neurol Sci. 1991 Aug;18(3):275–278. doi: 10.1017/s0317167100031814. [DOI] [PubMed] [Google Scholar]
- Rajput A. H., Rozdilsky B., Rajput A., Ang L. Levodopa efficacy and pathological basis of Parkinson syndrome. Clin Neuropharmacol. 1990 Dec;13(6):553–558. doi: 10.1097/00002826-199012000-00007. [DOI] [PubMed] [Google Scholar]
- Sakakibara R., Hattori T., Uchiyama T., Yamanishi T. Videourodynamic and sphincter motor unit potential analyses in Parkinson's disease and multiple system atrophy. J Neurol Neurosurg Psychiatry. 2001 Nov;71(5):600–606. doi: 10.1136/jnnp.71.5.600. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Stocchi F., Badiali D., Vacca L., D'Alba L., Bracci F., Ruggieri S., Torti M., Berardelli A., Corazziari E. Anorectal function in multiple system atrophy and Parkinson's disease. Mov Disord. 2000 Jan;15(1):71–76. doi: 10.1002/1531-8257(200001)15:1<71::aid-mds1012>3.0.co;2-w. [DOI] [PubMed] [Google Scholar]
- Stocchi F., Carbone A., Inghilleri M., Monge A., Ruggieri S., Berardelli A., Manfredi M. Urodynamic and neurophysiological evaluation in Parkinson's disease and multiple system atrophy. J Neurol Neurosurg Psychiatry. 1997 May;62(5):507–511. doi: 10.1136/jnnp.62.5.507. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wenning G. K., Ben-Shlomo Y., Hughes A., Daniel S. E., Lees A., Quinn N. P. What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson's disease? J Neurol Neurosurg Psychiatry. 2000 Apr;68(4):434–440. doi: 10.1136/jnnp.68.4.434. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wenning G. K., Ben-Shlomo Y., Magalhães M., Daniel S. E., Quinn N. P. Clinicopathological study of 35 cases of multiple system atrophy. J Neurol Neurosurg Psychiatry. 1995 Feb;58(2):160–166. doi: 10.1136/jnnp.58.2.160. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wenning G. K., Quinn N. P., Daniel S. E., Garratt H., Marsden C. D. Facial dystonia in pathologically proven multiple system atrophy: a video report. Mov Disord. 1996 Jan;11(1):107–109. doi: 10.1002/mds.870110125. [DOI] [PubMed] [Google Scholar]
- Wenning G. K., Scherfler C., Granata R., Bösch S., Verny M., Chaudhuri K. R., Jellinger K., Poewe W., Litvan I. Time course of symptomatic orthostatic hypotension and urinary incontinence in patients with postmortem confirmed parkinsonian syndromes: a clinicopathological study. J Neurol Neurosurg Psychiatry. 1999 Nov;67(5):620–623. doi: 10.1136/jnnp.67.5.620. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wenning G. K., Tison F., Ben Shlomo Y., Daniel S. E., Quinn N. P. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord. 1997 Mar;12(2):133–147. doi: 10.1002/mds.870120203. [DOI] [PubMed] [Google Scholar]
- de Vos R. A., Jansen E. N., Stam F. C., Ravid R., Swaab D. F. 'Lewy body disease': clinico-pathological correlations in 18 consecutive cases of Parkinson's disease with and without dementia. Clin Neurol Neurosurg. 1995 Feb;97(1):13–22. doi: 10.1016/0303-8467(94)00060-j. [DOI] [PubMed] [Google Scholar]