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Atypical Parkinsonism

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Neuro-Geriatrics

Abstract

Atypical Parkinsonism encompasses four separate diseases (Dementia with Lewy Bodies (DLB), Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD), and Multiple System Atrophy (MSA)) with overlapping clinical features but unique underlying pathologies. Unlike Parkinson’s disease, these disorders are less responsive to levodopa therapy and have a more rapid and severe disease progression.

Certain clinical features can be used to differentiate these disorders, and are important in making an early and accurate diagnosis. Although largely unresponsive to levodopa therapy, there are motor and non-motor (i.e. autonomic, psychiatric, gastrointestinal) features in each disorder which are amenable to treatment.

This chapter provides an overview of the diagnosis and management of PSP, CBD, and MSA. Specific diagnostic criteria are provided for each disorder, along with a diagnostic algorithm to aid in differentiating these disorders in the clinical setting. An atypical parkinsonism care path outlines the various modalities that can be used to improve motor and non-motor symptoms.

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References

  1. Williams DR, Litvan I. Parkinsonian syndromes. Continuum. 2013;19(5 Movement Disorders):1189–212. doi:10.1212/01.CON.0000436152.24038.e0.

    PubMed  PubMed Central  Google Scholar 

  2. Fahn S, Jankovic J, Hallett M. Principles and practice of movement disorders. 2nd ed. Edinburgh: Elsevier/Saunders; 2011.

    Google Scholar 

  3. Lippa CF, Duda JE, Grossman M, Hurtig HI, Aarsland D, Boeve BF, et al. DLB and PDD boundary issues: diagnosis, treatment, molecular pathology, and biomarkers. Neurology. 2007;68(11):812–9. doi:10.1212/01.wnl.0000256715.13907.d3.

    Article  CAS  PubMed  Google Scholar 

  4. Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain. 2005;128(Pt 6):1247–58. doi:10.1093/brain/awh488.

    Article  PubMed  Google Scholar 

  5. Hoglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, et al. Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Movement Disorders 2017;32(6):853–64.

    Google Scholar 

  6. Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1986;36(7):1005–8.

    Article  CAS  PubMed  Google Scholar 

  7. Kristensen MO. Progressive supranuclear palsy—20 years later. Acta Neurol Scand. 1985;71(3):177–89.

    Article  CAS  PubMed  Google Scholar 

  8. Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, 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.

    Article  CAS  PubMed  Google Scholar 

  9. Williams DR, Watt HC, Lees AJ. Predictors of falls and fractures in bradykinetic rigid syndromes: a retrospective study. J Neurol Neurosurg Psychiatry. 2006;77(4):468–73. doi:10.1136/jnnp.2005.074070.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Daniel SE, de Bruin VM, Lees AJ. The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. Brain. 1995;118(Pt 3):759–70.

    Article  PubMed  Google Scholar 

  11. Litvan I, Mangone CA, McKee A, Verny M, Parsa A, Jellinger K, et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. J Neurol Neurosurg Psychiatry. 1996;60(6):615–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Krack P, Marion MH. “Apraxia of lid opening,” a focal eyelid dystonia: clinical study of 32 patients. Mov Disord. 1994;9(6):610–5. doi:10.1002/mds.870090605.

    Article  CAS  PubMed  Google Scholar 

  13. Litvan I, Mega MS, Cummings JL, Fairbanks L. Neuropsychiatric aspects of progressive supranuclear palsy. Neurology. 1996;47(5):1184–9.

    Article  CAS  PubMed  Google Scholar 

  14. Brown RG, Lacomblez L, Landwehrmeyer BG, Bak T, Uttner I, Dubois B, et al. Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain. 2010;133(Pt 8):2382–93. doi:10.1093/brain/awq158.

    Article  PubMed  Google Scholar 

  15. Nath U, Thomson R, Wood R, Ben-Shlomo Y, Lees A, Rooney C, et al. Population based mortality and quality of death certification in progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). J Neurol Neurosurg Psychiatry. 2005;76(4):498–502. doi:10.1136/jnnp.2004.039370.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Chen AL, Riley DE, King SA, Joshi AC, Serra A, Liao K, et al. The disturbance of gaze in progressive supranuclear palsy: implications for pathogenesis. Front Neurol. 2010;1:147. doi:10.3389/fneur.2010.00147.

    PubMed  PubMed Central  Google Scholar 

  17. Romano S, Colosimo C. Procerus sign in progressive supranuclear palsy. Neurology. 2001;57(10):1928.

    Article  CAS  PubMed  Google Scholar 

  18. Burrell JR, Hodges JR, Rowe JB. Cognition in corticobasal syndrome and progressive supranuclear palsy: a review. Mov Disord. 2014;29(5):684–93. doi:10.1002/mds.25872.

    Article  PubMed  Google Scholar 

  19. Kato N, Arai K, Hattori T. Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci. 2003;210(1–2):57–60.

    Article  PubMed  Google Scholar 

  20. Massey LA, Jager HR, Paviour DC, O’Sullivan SS, Ling H, Williams DR, et al. The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy. Neurology. 2013;80(20):1856–61. doi:10.1212/WNL.0b013e318292a2d2.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Bajaj N, Hauser RA, Grachev ID. Clinical utility of dopamine transporter single photon emission CT (DaT-SPECT) with (123I) ioflupane in diagnosis of parkinsonian syndromes. J Neurol Neurosurg Psychiatry. 2013;84(11):1288–95. doi:10.1136/jnnp-2012-304436.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Hoglinger GU, Melhem NM, Dickson DW, Sleiman PM, Wang LS, Klei L, et al. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nat Genet. 2011;43(7):699–705. doi:10.1038/ng.859.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1994;44(11):2015–9.

    Article  CAS  PubMed  Google Scholar 

  24. Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496–503. doi:10.1212/WNL.0b013e31827f0fd1.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Hornykiewicz O, et al. Cortical-basal ganglionic degeneration. Neurology. 1990;40(8):1203–12.

    Article  CAS  PubMed  Google Scholar 

  26. Graff-Radford J, Rubin MN, Jones DT, Aksamit AJ, Ahlskog JE, Knopman DS, et al. The alien limb phenomenon. J Neurol. 2013;260(7):1880–8. doi:10.1007/s00415-013-6898-y.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Wenning GK, Litvan I, Jankovic J, Granata R, Mangone CA, McKee A, et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry. 1998;64(2):184–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Murray R, Neumann M, Forman MS, Farmer J, Massimo L, Rice A, et al. Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology. 2007;68(16):1274–83. doi:10.1212/01.wnl.0000259519.78480.c3.

    Article  CAS  PubMed  Google Scholar 

  29. Litvan I, Grimes DA, Lang AE. Phenotypes and prognosis: clinicopathologic studies of corticobasal degeneration. Adv Neurol. 2000;82:183–96.

    CAS  PubMed  Google Scholar 

  30. Stover NP, Watts RL. Corticobasal degeneration. Semin Neurol. 2001;21(1):49–58.

    Article  CAS  PubMed  Google Scholar 

  31. Barbieri C, De Renzi E. The executive and ideational components of apraxia. Cortex. 1988;24(4):535–43.

    Article  CAS  PubMed  Google Scholar 

  32. Drago V, Foster PS, Edward D, Wargovich B, Heilman KM. Graphesthesia: a test of graphemic movement representations or tactile imagery? J Int Neuropsychol Soc. 2010;16(1):190–3. doi:10.1017/S1355617709990762.

    Article  CAS  PubMed  Google Scholar 

  33. Gerstmann J. Pure tactile agnosia. Cogn Neuropsychol. 2001;18(3):267–74. doi:10.1080/02643290042000116.

    Article  CAS  PubMed  Google Scholar 

  34. Vaishnavi S, Calhoun J, Southwood MH, Chatterjee A. Sensory and response interference by ipsilesional stimuli in tactile extinction. Cortex. 2000;36(1):81–92.

    Article  CAS  PubMed  Google Scholar 

  35. Savoiardo M, Grisoli M, Girotti F. Magnetic resonance imaging in CBD, related atypical parkinsonian disorders, and dementias. Adv Neurol. 2000;82:197–208.

    CAS  PubMed  Google Scholar 

  36. Ishii K. PET approaches for diagnosis of dementia. AJNR Am J Neuroradiol. 2014;35(11):2030–8. doi:10.3174/ajnr.A3695.

    Article  CAS  PubMed  Google Scholar 

  37. Dickson DW, Bergeron C, Chin SS, Duyckaerts C, Horoupian D, Ikeda K, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol. 2002;61(11):935–46.

    Article  CAS  PubMed  Google Scholar 

  38. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71(9):670–6. doi:10.1212/01.wnl.0000324625.00404.15.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Litvan I, Goetz CG, Jankovic J, Wenning GK, Booth V, Bartko JJ, et al. What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study. Arch Neurol. 1997;54(8):937–44.

    Article  CAS  PubMed  Google Scholar 

  40. Kirchhof K, Apostolidis AN, Mathias CJ, Fowler CJ. Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study. Int J Impot Res. 2003;15(4):293–8. doi:10.1038/sj.ijir.3901014.

    Article  CAS  PubMed  Google Scholar 

  41. Kollensperger M, Geser F, Seppi K, Stampfer-Kountchev M, Sawires M, Scherfler C, et al. Red flags for multiple system atrophy. Mov Disord. 2008;23(8):1093–9. doi:10.1002/mds.21992.

    Article  PubMed  Google Scholar 

  42. Kawai Y, Suenaga M, Takeda A, Ito M, Watanabe H, Tanaka F, et al. Cognitive impairments in multiple system atrophy: MSA-C vs MSA-P. Neurology. 2008;70(16 Pt 2):1390–6. doi:10.1212/01.wnl.0000310413.04462.6a.

    Article  CAS  PubMed  Google Scholar 

  43. Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain. 1994;117(Pt 4):835–45.

    Article  PubMed  Google Scholar 

  44. Colosimo C, Albanese A, Hughes AJ, de Bruin VM, Lees AJ. Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson’s disease. Arch Neurol. 1995;52(3):294–8.

    Article  CAS  PubMed  Google Scholar 

  45. Kluin KJ, Gilman S, Lohman M, Junck L. Characteristics of the dysarthria of multiple system atrophy. Arch Neurol. 1996;53(6):545–8.

    Article  CAS  PubMed  Google Scholar 

  46. da Rocha AJ, Maia AC Jr, da Silva CJ, Braga FT, Ferreira NP, Barsottini OG, et al. Pyramidal tract degeneration in multiple system atrophy: the relevance of magnetization transfer imaging. Mov Disord. 2007;22(2):238–44. doi:10.1002/mds.21229.

    Article  PubMed  Google Scholar 

  47. Bhattacharya K, Saadia D, Eisenkraft B, Yahr M, Olanow W, Drayer B, et al. Brain magnetic resonance imaging in multiple-system atrophy and Parkinson disease: a diagnostic algorithm. Arch Neurol. 2002;59(5):835–42.

    Article  PubMed  Google Scholar 

  48. Seppi K, Schocke MF, Wenning GK, Poewe W. How to diagnose MSA early: the role of magnetic resonance imaging. J Neural Transm. 2005;112(12):1625–34. doi:10.1007/s00702-005-0332-2.

    Article  CAS  PubMed  Google Scholar 

  49. Papp MI, Kahn JE, Lantos PL. Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome). J Neurol Sci. 1989;94(1–3):79–100.

    Article  CAS  PubMed  Google Scholar 

  50. Broustal O, Camuzat A, Guillot-Noel L, Guy N, Millecamps S, Deffond D, et al. FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis. J Alzheimers Dis. 2010;22(3):765–9.

    Google Scholar 

  51. Maetzler W, Nieuwhof F, Hasmann SE, Bloem BR. Emerging therapies for gait disability and balance impairment: promises and pitfalls. Mov Disord. 2013;28(11):1576–86. doi:10.1002/ mds.25682.

    Google Scholar 

  52. Zampieri C, Di Fabio RP. Balance and eye movement training to improve gait in people with progressive supranuclear palsy: quasi-randomized clinical trial. Phys Ther. 2008;88(12):1460– 73. doi:10.2522/ptj.20070302.

    Google Scholar 

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Authors and Affiliations

  1. Cleveland Clinic Lou Ruvo Center for Brain Health, 888 W. Bonneville Avenue, Las Vegas, NV, 89106, USA

    Brent Bluett D.O

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  1. Brent Bluett D.O
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Correspondence to Brent Bluett D.O .

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  1. Lou Ruvo Center for Brain Health, Cleveland Clinic, Neurological Institute, Cleveland, Ohio, USA

    Babak Tousi

  2. Lou Ruvo Center for Brain Health, Cleveland Clinic, Las Vegas, Nevada, USA

    Jeffrey Cummings

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Bluett, B. (2017). Atypical Parkinsonism. In: Tousi, B., Cummings, J. (eds) Neuro-Geriatrics. Springer, Cham. https://doi.org/10.1007/978-3-319-56484-5_11

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  • DOI: https://doi.org/10.1007/978-3-319-56484-5_11

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