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

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Abstract

Objectives

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.

Methods

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.

Results

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.

Conclusions

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.

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References

  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):1891–1902

    Article  PubMed  Google Scholar 

  2. Davis M, Gendelman DS, Tischler MD, Gendelman PM (1982) A primary acoustic startle circuit: lesion and stimulation studies. J Neurosci 2:791–805

    PubMed  CAS  Google Scholar 

  3. Factor SA, Higgins DS, Qian J (2006) Primary progressive freezing gait: a syndrome with many causes. Neurology 66:411–414

    Article  PubMed  Google Scholar 

  4. Gibb WR, Lees AJ (1988) The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease. J Neurol Neurosurg Psychiatry 51:745–752

    Article  PubMed  CAS  Google Scholar 

  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 18:1273–1279

    Article  PubMed  Google Scholar 

  6. Hori A, Yasuhara A, Naito H, Yasuhara M (1986) Blink reflex elicited by auditory stimulation in the rabbit. J Neurol Sci 76:49–59

    Article  PubMed  CAS  Google Scholar 

  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 42:1142–1146

    PubMed  CAS  Google Scholar 

  8. Imai H, Narabayashi H, Sakata E (1987) "Pure akinesia" and the later added supranuclear ophthalmoplegia. Adv Neurol 45:207–212

    PubMed  CAS  Google Scholar 

  9. Juncos JL, Hirsch EC, Malessa S, Duyckaerts C, Hersh LB, Agid Y (1991) Mesencephalic cholinergic nuclei in progressive supranuclear palsy. Neurology 41:25–30

    PubMed  CAS  Google Scholar 

  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 18:1123–1131

    Article  PubMed  Google Scholar 

  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 16:62–71

    Article  PubMed  CAS  Google Scholar 

  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 47:1–9

    Article  PubMed  CAS  Google Scholar 

  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 41:1593–1597

    PubMed  CAS  Google Scholar 

  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 47:1113–1124

    PubMed  CAS  Google Scholar 

  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 19:181–189

    Article  PubMed  Google Scholar 

  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 51:1315–1320

    PubMed  CAS  Google Scholar 

  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):1181–1192

    Article  PubMed  Google Scholar 

  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 128:1247–1258

    Article  PubMed  Google Scholar 

  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 130:1566–1576

    Article  PubMed  Google Scholar 

  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 22:2235–2241

    Article  PubMed  Google Scholar 

  21. Williams DR, Lees AJ (2005) Visual hallucinations in the diagnosis of idiopathic Parkinson's disease: a retrospective autopsy study. Lancet Neurol 4:605–610

    Article  PubMed  Google Scholar 

  22. Williams DR, Watt HC, Lees AJ (2006) Predictors of falls and fractures in bradykinetic rigid syndromes: a retrospective study. J Neurol Neurosurg Psychiatry 77:468–473

    Article  PubMed  CAS  Google Scholar 

  23. Zweig RM, Whitehouse PJ, Casanova MF, Walker LC, Jankel WR, Price DL (1987) Loss of pedunculopontine neurons in progressive supranuclear palsy. Ann Neurol 22:18–25

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus), AUS

    David R. Williams*

  2. Rita Lila Weston Institute of Neurological Studies, University College London, UK

    David R. Williams* & Andrew J. Lees

  3. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, 8–11 Queen Square, London, WC1N 3BG, UK

    Louise M. F. Doyle* &  P. Brown

Authors
  1. David R. Williams*
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  2. Louise M. F. Doyle*
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  3. Andrew J. Lees
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  4. P. Brown
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Corresponding author

Correspondence to P. Brown.

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* Both authors contributed equally to this work

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Williams*, D.R., Doyle*, L.M.F., Lees, A.J. et al. The auditory startle response in parkinsonism may reveal the extent but not type of pathology. J Neurol 255, 628–632 (2008). https://doi.org/10.1007/s00415-008-0758-1

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  • DOI: https://doi.org/10.1007/s00415-008-0758-1

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