Journal of Neurology

, Volume 255, Issue 5, pp 628–632 | Cite as

The auditory startle response in parkinsonism may reveal the extent but not type of pathology

  • David R. Williams*
  • Louise M. F. Doyle*
  • Andrew J. Lees
  • P. BrownEmail author



We measured the acoustic startle response (ASR) and blink reflex (ABR) in patients with clinically diagnosed Parkinson's disease (PD) and progressive supranuclear palsy (PSP) and determined the specificity of an abnormal result for the diagnosis of PSP.


Thirty patients (11 PD, 19 PSP) and 12 age matched controls were studied. The PSP group was separated into clinical subgroups. Fourteen patients with early falls, cognitive dysfunction and eye movement abnormalities were classified as Richardson's disease (RD), 3 with parkinsonism in the absence of early falls, cognitive dysfunction or eye movement abnormalities were classified PSP-parkinsonism (PSP-P) and 2 who presented with gait unsteadiness and freezing without cognitive dysfunction or eye movement abnormalities were classified as pure akinesia with gait freezing (PAGF). Following an acoustic startle stimulus EMG activity was recorded in the orbicularis oculi and sternomastoid muscles. The likelihood ratio of an absent response being clinically diagnosed PSP was determined.


The ABR was present in 11/19 (58%) of patients with PSP, and 11/11 with PD and 12/12 controls (H2 p < 0.05). The ASR measured at the sternomastoid was present in only 2/19 (11%) of patients with PSP, but in 5/11 (45%) with PD and 6/12 (50 %) controls (H2 p < 0.05). The ABR was present in only 6/14 (43%) of the RD group, but in 3/3 PSP-P and 2/2 PAGF. Loss of the ABR was 100% specific for PSP, but only 42 % sensitive. Loss of the sternomastoid ASR had a likelihood ratio of 1.9 for the clinical diagnosis of PSP.


The ASR and ABR appear unlikely to be useful in differentiating PSP from PD.Abnormalities in these responses are worse in RD where the pathology is known to be more widespread than in PSP-P and PAGF.

Key words

PSP Richardson's disease PSP-P startle response blink reflex 


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  1. 1.
    Brown P, Rothwell JC, Thompson PD, Britton TC, Day BL, Marsden CD (1991) New observations on the normal auditory startle reflex in man. Brain 114 (Pt 4):1891–1902PubMedCrossRefGoogle Scholar
  2. 2.
    Davis M, Gendelman DS, Tischler MD, Gendelman PM (1982) A primary acoustic startle circuit: lesion and stimulation studies. J Neurosci 2:791–805PubMedGoogle Scholar
  3. 3.
    Factor SA, Higgins DS, Qian J (2006) Primary progressive freezing gait: a syndrome with many causes. Neurology 66:411–414PubMedCrossRefGoogle Scholar
  4. 4.
    Gibb WR, Lees AJ (1988) The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease. J Neurol Neurosurg Psychiatry 51:745–752PubMedCrossRefGoogle Scholar
  5. 5.
    Gironell A, Kulisevsky J, Roig C, Pascual- Sedano B, Rodriguez-Fornells A, Otermin P (2003) Diagnostic potential of acoustic startle reflex, acoustic blink reflex, and electro-oculography in progressive supranuclear palsy: a prospective study. Mov Disord 18:1273–1279PubMedCrossRefGoogle Scholar
  6. 6.
    Hori A, Yasuhara A, Naito H, Yasuhara M (1986) Blink reflex elicited by auditory stimulation in the rabbit. J Neurol Sci 76:49–59PubMedCrossRefGoogle Scholar
  7. 7.
    Hughes AJ, Ben-Shlomo Y, Daniel SE, Lees AJ (1992) What features improve the accuracy of clinical diagnosis in Parkinson's disease: a clinicopathologic study. Neurology 42:1142–1146PubMedGoogle Scholar
  8. 8.
    Imai H, Narabayashi H, Sakata E (1987) "Pure akinesia" and the later added supranuclear ophthalmoplegia. Adv Neurol 45:207–212PubMedGoogle Scholar
  9. 9.
    Juncos JL, Hirsch EC, Malessa S, Duyckaerts C, Hersh LB, Agid Y (1991) Mesencephalic cholinergic nuclei in progressive supranuclear palsy. Neurology 41:25–30PubMedGoogle Scholar
  10. 10.
    Katzenschlager R, Cardozo A, Vila Cobo MR, Tolosa E, Lees AJ (2003) Unclassifiable parkinsonism in two European tertiary referral centres for movement disorders. Mov Disord 18:1123–1131PubMedCrossRefGoogle Scholar
  11. 11.
    Kofler M, Muller J, Wenning GK, Reggiani L, Hollosi P, Bosch S, Ransmayr G, Valls-Sole J, Poewe W (2001) The auditory startle reaction in parkinsonian disorders. Mov Disord 16:62–71PubMedCrossRefGoogle Scholar
  12. 12.
    Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, Goetz CG, Golbe LI, Grafman J, Growdon JH, Hallett M, Jankovic J, Quinn NP, Tolosa E, Zee DS (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson- Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47:1–9PubMedCrossRefGoogle Scholar
  13. 13.
    Malessa S, Hirsch EC, Cervera P, Javoy- Agid F, Duyckaerts C, Hauw JJ, Agid Y (1991) Progressive supranuclear palsy: loss of choline-acetyltransferase-like immunoreactive neurons in the pontine reticular formation. Neurology 41:1593–1597PubMedGoogle Scholar
  14. 14.
    McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G, Quinn NP, Edwardson JA, Ince PG, Bergeron C, Burns A, Miller BL, Lovestone S, Collerton D, Jansen EN, Ballard C, de Vos RA, Wilcock GK, Jellinger KA, Perry RH (1996) Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology 47:1113–1124PubMedGoogle Scholar
  15. 15.
    Osaki Y, Ben-Shlomo Y, Lees AJ, Daniel SE, Colosimo C, Wenning G, Quinn N (2004) Accuracy of clinical diagnosis of progressive supranuclear palsy. Mov Disord 19:181–189PubMedCrossRefGoogle Scholar
  16. 16.
    Valldeoriola F, Valls-Sole J, Tolosa E, Ventura PJ, Nobbe FA, Marti MJ (1998) Effects of a startling acoustic stimulus on reaction time in different parkinsonian syndromes. Neurology 51:1315–1320PubMedGoogle Scholar
  17. 17.
    Vidailhet M, Rothwell JC, Thompson PD, Lees AJ, Marsden CD (1992) The auditory startle response in the Steele- Richardson-Olszewski syndrome and Parkinson's disease. Brain 115 (Pt 4):1181–1192PubMedCrossRefGoogle Scholar
  18. 18.
    Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. Brain 128:1247–1258PubMedCrossRefGoogle Scholar
  19. 19.
    Williams DR, Holton J, Strand C, Pittman A, de Silva R, Lees AJ, Revesz T (2007) Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 130:1566–1576PubMedCrossRefGoogle Scholar
  20. 20.
    Williams DR, Holton J, Strand C, Revesz T, Lees A (2007) Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Movement Disorders 22:2235–2241PubMedCrossRefGoogle Scholar
  21. 21.
    Williams DR, Lees AJ (2005) Visual hallucinations in the diagnosis of idiopathic Parkinson's disease: a retrospective autopsy study. Lancet Neurol 4:605–610PubMedCrossRefGoogle Scholar
  22. 22.
    Williams DR, Watt HC, Lees AJ (2006) Predictors of falls and fractures in bradykinetic rigid syndromes: a retrospective study. J Neurol Neurosurg Psychiatry 77:468–473PubMedCrossRefGoogle Scholar
  23. 23.
    Zweig RM, Whitehouse PJ, Casanova MF, Walker LC, Jankel WR, Price DL (1987) Loss of pedunculopontine neurons in progressive supranuclear palsy. Ann Neurol 22:18–25PubMedCrossRefGoogle Scholar

Copyright information

© Steinkopff-Verlag 2008

Authors and Affiliations

  • David R. Williams*
    • 1
    • 2
  • Louise M. F. Doyle*
    • 3
  • Andrew J. Lees
    • 2
  • P. Brown
    • 3
    Email author
  1. 1.Faculty of Medicine (Neurosciences)Monash University (Alfred Hospital Campus)AUS
  2. 2.Rita Lila Weston Institute of Neurological StudiesUniversity College LondonUK
  3. 3.Sobell Department of Motor Neuroscience and Movement DisordersInstitute of NeurologyLondonUK

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