open access

Vol 53, No 1 (2019)
Research paper
Published online: 2019-01-08
Submitted: 2018-12-06
Accepted: 2018-12-06
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Electrophysiological and clinical assessment of dysautonomia in multiple system atrophy (MSA) and progressive supranuclear palsy (PSP): a comparative study

Monika Nojszewska, Anna Potulska-Chromik, Zygmunt Jamrozik, Piotr Janik, Beata Zakrzewska-Pniewska
DOI: 10.5603/PJNNS.a2019.0005
·
Pubmed: 30620042
·
Neurol Neurochir Pol 2019;53(1):26-33.

open access

Vol 53, No 1 (2019)
Research paper
Published online: 2019-01-08
Submitted: 2018-12-06
Accepted: 2018-12-06

Abstract

Clinical rationale for the study. Autonomic nervous system (ANS) involvement in different parkinsonian syndromes has been frequently discussed. It is well established in multiple system atrophy (MSA), whereas it is less evident in progressive supranuclear palsy (PSP).

Aims of the study. The aims were to assess the presence and pattern of ANS involvement in MSA and PSP using noninvasive tests i.e. the sympathetic skin response (SSR) test and the R-R interval variation (RRIV) test; to analyse the relationship between clinical and electrophysiological abnormalities in both disorders; and to assess whether an autonomic profile might help to differentiate them.

Materials and methods. Clinical and electrophysiological assessments of dysautonomia were performed in 59 patients with MSA (24 cases of MSA-C and 35 cases of MSA-P), these 59 cases including 31 females, mean disease duration 4.2 ± 2.7 years, mean age 60.3 ± 8.4 years, and in 37 patients with PSP (12 females, mean disease duration 4.6 ± 3.6 years, mean age 67.5 ± 6.1 years) and the results were compared to the results obtained from 23 healthy controls matched for age and sex.

Results. Clinical dysautonomia assessed by an Autonomic Symptoms Questionnaire was observed in 97% of the MSA patients and in 84% of the PSP patients. SSR was abnormal in 64% and RRIV was abnormal in 73% of MSA cases. In PSP cases, these figures were 78% and 81% respectively. Dysautonomia was clinically more pronounced in MSA compared to PSP (p < 0.05), whereas electrophysiological testing revealed frequently subclinical ANS damage in PSP patients.

Conclusions and clinical implications. Our results point to the complementary role of electrophysiological tests in the diagnostic work-up of dysautonomia in parkinsonian syndromes.

Abstract

Clinical rationale for the study. Autonomic nervous system (ANS) involvement in different parkinsonian syndromes has been frequently discussed. It is well established in multiple system atrophy (MSA), whereas it is less evident in progressive supranuclear palsy (PSP).

Aims of the study. The aims were to assess the presence and pattern of ANS involvement in MSA and PSP using noninvasive tests i.e. the sympathetic skin response (SSR) test and the R-R interval variation (RRIV) test; to analyse the relationship between clinical and electrophysiological abnormalities in both disorders; and to assess whether an autonomic profile might help to differentiate them.

Materials and methods. Clinical and electrophysiological assessments of dysautonomia were performed in 59 patients with MSA (24 cases of MSA-C and 35 cases of MSA-P), these 59 cases including 31 females, mean disease duration 4.2 ± 2.7 years, mean age 60.3 ± 8.4 years, and in 37 patients with PSP (12 females, mean disease duration 4.6 ± 3.6 years, mean age 67.5 ± 6.1 years) and the results were compared to the results obtained from 23 healthy controls matched for age and sex.

Results. Clinical dysautonomia assessed by an Autonomic Symptoms Questionnaire was observed in 97% of the MSA patients and in 84% of the PSP patients. SSR was abnormal in 64% and RRIV was abnormal in 73% of MSA cases. In PSP cases, these figures were 78% and 81% respectively. Dysautonomia was clinically more pronounced in MSA compared to PSP (p < 0.05), whereas electrophysiological testing revealed frequently subclinical ANS damage in PSP patients.

Conclusions and clinical implications. Our results point to the complementary role of electrophysiological tests in the diagnostic work-up of dysautonomia in parkinsonian syndromes.

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Keywords

MSA, PSP, autonomic nervous system, dysautonomia, SSR, RRIV

About this article
Title

Electrophysiological and clinical assessment of dysautonomia in multiple system atrophy (MSA) and progressive supranuclear palsy (PSP): a comparative study

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 53, No 1 (2019)

Pages

26-33

Published online

2019-01-08

DOI

10.5603/PJNNS.a2019.0005

Pubmed

30620042

Bibliographic record

Neurol Neurochir Pol 2019;53(1):26-33.

Keywords

MSA
PSP
autonomic nervous system
dysautonomia
SSR
RRIV

Authors

Monika Nojszewska
Anna Potulska-Chromik
Zygmunt Jamrozik
Piotr Janik
Beata Zakrzewska-Pniewska

References (50)
  1. Weimer LH. Autonomic testing: common techniques and clinical applications. Neurologist. 2010; 16(4): 215–222.
  2. Fagius J, Wallin BG. Sympathetic reflex latencies and conduction velocities in normal man. J Neurol Sci. 1980; 47(3): 433–448.
  3. Fagius J, Wallin BG. Sympathetic reflex latencies and conduction velocities in patients with polyneuropathy. J Neurol Sci. 1980; 47(3): 449–461.
  4. Shahani BT, Halperin JJ, Boulu P, et al. Sympathetic skin response--a method of assessing unmyelinated axon dysfunction in peripheral neuropathies. J Neurol Neurosurg Psychiatry. 1984; 47(5): 536–542.
  5. Elie B, Guiheneuc P. Sympathetic skin response: normal results in different experimental conditions. Electroencephalogr Clin Neurophysiol. 1990; 76(3): 258–267.
  6. Shahani BT, Day TJ, Cros D, et al. RR interval variation and the sympathetic skin response in the assessment of autonomic function in peripheral neuropathy. Arch Neurol. 1990; 47(6): 659–664.
  7. Bordet R, Benhadjali J, Destee A, et al. Sympathetic skin response and R-R interval variability in multiple system atrophy and idiopathic Parkinson's disease. Mov Disord. 1996; 11(3): 268–272.
  8. Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008; 71(9): 670–676.
  9. Davis PH, Golbe LI, Duvoisin RC, et al. Prevalence and natural history of progressive supranuclear palsy. Neurology. 1988; 38(7): 1031–1034.
  10. Litvan I, Agid Y, Calne D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996; 47(1): 1–9.
  11. Santacruz P, Uttl B, Litvan I, et al. Progressive supranuclear palsy: a survey of the disease course. Neurology. 1998; 50(6): 1637–1647.
  12. Golbe LI. Progressive supranuclear palsy. Semin Neurol. 2014; 34(2): 151–159.
  13. Boxer AL, Yu JT, Golbe LI, et al. Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches. Lancet Neurol. 2017; 16(7): 552–563.
  14. Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. 1999; 163(1): 94–98.
  15. Payan CAM, Viallet F, Landwehrmeyer BG, et al. NNIPPS Study Group. Disease severity and progression in progressive supranuclear palsy and multiple system atrophy: validation of the NNIPPS--Parkinson Plus Scale. PLoS One. 2011; 6(8): e22293.
  16. O'Sullivan SS, Massey LA, Williams DR, et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain. 2008; 131(Pt 5): 1362–1372.
  17. Ben-Shlomo Y, Wenning GK, Tison F, et al. Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology. 1997; 48(2): 384–393.
  18. Wenning GK, Geser F, Krismer F, et al. European Multiple System Atrophy Study Group. The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol. 2013; 12(3): 264–274.
  19. Fanciulli A, Wenning GK. Multiple-system atrophy. N Engl J Med. 2015; 372(3): 249–263.
  20. Glasmacher SA, Leigh PN, Saha RA. Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2017; 88(5): 402–411.
  21. dell'Aquila C, Zoccolella S, Cardinali V, et al. Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients. Parkinsonism Relat Disord. 2013; 19(11): 980–985.
  22. Arena JE, Weigand SD, Whitwell JL, et al. Progressive supranuclear palsy: progression and survival. J Neurol. 2016; 263(2): 380–389.
  23. Gutrecht JA. Autonomic cardiovascular reflexes in progressive supranuclear palsy. J Auton Nerv Syst. 1992; 39(1): 29–35.
  24. van Dijk JG, Haan J, Koenderink M, et al. Autonomic nervous function in progressive supranuclear palsy. Arch Neurol. 1991; 48(10): 1083–1084.
  25. Kimber J, Mathias CJ, Lees AJ, et al. Physiological, pharmacological and neurohormonal assessment of autonomic function in progressive supranuclear palsy. Brain. 2000; 123 ( Pt 7): 1422–1430.
  26. Brefel-Courbon C, Thalamas C, Rascol O, et al. Lack of autonomic nervous dysfunction in progressive supranuclear palsy, a study of blood pressure variability. Clin Auton Res. 2000; 10(5): 309–312.
  27. Zakrzewska-Pniewska B, Gawel M, Szmidt-Salkowska E, et al. Clinical and functional assessment of dysautonomia and its correlation in Alzheimer's disease. Am J Alzheimers Dis Other Demen. 2012; 27(8): 592–599.
  28. Low PA. Clinical Autonomic Disorders: Classification and Clinical Evaluation. In: ed. Clinical Autonomic Disorders. Evaluation and Management. 2nd ed. Lippincott–Raven, New York. 1997: 3–13.
  29. Holmberg B, Kallio M, Johnels B, et al. Cardiovascular reflex testing contributes to clinical evaluation and differential diagnosis of Parkinsonian syndromes. Mov Disord. 2001; 16(2): 217–225.
  30. Sakakibara R, Hattori T, Uchiyama T, et al. Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation? J Neurol Neurosurg Psychiatry. 2000; 68(1): 65–69.
  31. Laurens B, Vergnet S, Lopez MC, et al. Multiple System Atrophy - State of the Art. Curr Neurol Neurosci Rep. 2017; 17(5): 41.
  32. Iodice V, Lipp A, Ahlskog JE, et al. Autopsy confirmed multiple system atrophy cases: Mayo experience and role of autonomic function tests. J Neurol Neurosurg Psychiatry. 2012; 83(4): 453–459.
  33. Kimpinski K, Iodice V, Burton DD, et al. The role of autonomic testing in the differentiation of Parkinson's disease from multiple system atrophy. J Neurol Sci. 2012; 317(1-2): 92–96.
  34. Wenning GK, Scherfler C, Granata R, et al. Time course of symptomatic orthostatic hypotension and urinary incontinence in patients with postmortem confirmed parkinsonian syndromes: a clinicopathological study. J Neurol Neurosurg Psychiatry. 1999; 67(5): 620–623.
  35. Kikkawa Y, Asahina M, Suzuki A, et al. Cutaneous sympathetic function and cardiovascular function in patients with progressive supranuclear palsy and Parkinson's disease. Parkinsonism Relat Disord. 2003; 10(2): 101–106.
  36. Sakakibara R, Hattori T, Tojo M, et al. Micturitional disturbance in progressive supranuclear palsy. J Auton Nerv Syst. 1993; 45(2): 101–106.
  37. Valldeoriola F, Valls-Solé J, Tolosa ES, et al. Striated anal sphincter denervation in patients with progressive supranuclear palsy. Mov Disord. 1995; 10(5): 550–555.
  38. Asahina M, Vichayanrat E, Low DA, et al. Autonomic dysfunction in parkinsonian disorders: assessment and pathophysiology. J Neurol Neurosurg Psychiatry. 2013; 84(6): 674–680.
  39. Drory VE, Korczyn AD. Sympathetic skin response: age effect. Neurology. 1993; 43(9): 1818–1820.
  40. De Marinis M, Stocchi F, Gregori B, et al. Sympathetic skin response and cardiovascular autonomic function tests in Parkinson's disease and multiple system atrophy with autonomic failure. Mov Disord. 2000; 15(6): 1215–1220.
  41. Asahina M, Kikkawa Y, Suzuki A, et al. Cutaneous sympathetic function in patients with multiple system atrophy. Clin Auton Res. 2003; 13(2): 91–95.
  42. Asahina M, Akaogi Y, Yamanaka Y, et al. Differences in skin sympathetic involvements between two chronic autonomic disorders: multiple system atrophy and pure autonomic failure. Parkinsonism Relat Disord. 2009; 15(5): 347–350.
  43. Reimann M, Schmidt C, Herting B, et al. Comprehensive autonomic assessment does not differentiate between Parkinson's disease, multiple system atrophy and progressive supranuclear palsy. J Neural Transm (Vienna). 2010; 117(1): 69–76.
  44. Yokota T, Hayashi M, Tanabe H, et al. Sympathetic skin response in patients with cerebellar degeneration. Arch Neurol. 1993; 50(4): 422–427.
  45. Wang SJ, Fuh JL, Shan DE, et al. Sympathetic skin response and R-R interval variation in Parkinson's disease. Mov Disord. 1993; 8(2): 151–157.
  46. Sasaki I, Takeuchi H, Deguchi K, et al. [Autonomic nervous function in progressive supranuclear palsy--comparison with Parkinson's disease and healthy controls]. Rinsho Shinkeigaku. 1994; 34(10): 975–979.
  47. Hay JE, Taylor PK, Nukada H. Auditory and inspiratory gasp-evoked sympathetic skin response: age effects. J Neurol Sci. 1997; 148(1): 19–23.
  48. Homma S, Nakajima Y, Toma S, et al. Intracerebral source localization of mental process-related potentials elicited prior to mental sweating response in humans. Neurosci Lett. 1998; 247(1): 25–28.
  49. Gurevich TYu, Groozman GB, Giladi N, et al. R-R interval variation in Parkinson's disease and multiple system atrophy. Acta Neurol Scand. 2004; 109(4): 276–279.
  50. Brisinda D, Sorbo AR, Di Giacopo R, et al. Cardiovascular autonomic nervous system evaluation in Parkinson disease and multiple system atrophy. J Neurol Sci. 2014; 336(1-2): 197–202.

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