What are the clinical subtypes of progressive supranuclear palsy (PSP)?

1. Savica R, Grossardt BR, Bower JH, Ahlskog JE, Rocca WA. Incidence and pathology of synucleinopathies and tauopathies related to parkinsonism. JAMA Neurol. 2013 Jul. 70 (7):859-66. [Medline].

2. Litvan I, Bhatia KP, Burn DJ, Goetz CG, Lang AE, McKeith I, et al. Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Disord. 2003 May. 18(5):467-86. [Medline].

3. 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 Aug 26. 71(9):670-6. [Medline]. [Full Text].

4. Lipp A, Sandroni P, Ahlskog JE, Fealey RD, Kimpinski K, Iodice V, et al. Prospective differentiation of multiple system atrophy from Parkinson disease, with and without autonomic failure. Arch Neurol. 2009 Jun. 66(6):742-50. [Medline]. [Full Text].

5. Wenning GK, Tison F, Ben Shlomo Y, Daniel SE, Quinn NP. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord. 1997 Mar. 12 (2):133-47. [Medline].

6. Tada M, Onodera O, Tada M, Ozawa T, Piao YS, Kakita A, et al. Early development of autonomic dysfunction may predict poor prognosis in patients with multiple system atrophy. Arch Neurol. 2007 Feb. 64 (2):256-60. [Medline].

7. Figueroa JJ, Singer W, Parsaik A, Benarroch EE, Ahlskog JE, Fealey RD, et al. Multiple system atrophy: prognostic indicators of survival. Mov Disord. 2014 Aug. 29 (9):1151-7. [Medline].

8. Pellecchia MT, Barone P, Mollica C, Salvatore E, Ianniciello M, Longo K, et al. Diffusion-weighted imaging in multiple system atrophy: a comparison between clinical subtypes. Mov Disord. 2009 Apr 15. 24(5):689-96. [Medline].

9. Massano J, Costa F, Nadais G. Teaching neuroImage: MRI in multiple system atrophy: "hot cross bun" sign and hyperintense rim bordering the putamina. Neurology. 2008 Oct 7. 71(15):e38. [Medline].

10. Tsukamoto K, Matsusue E, Kanasaki Y, Kakite S, Fujii S, Kaminou T, et al. Significance of apparent diffusion coefficient measurement for the differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and Parkinson's disease: evaluation by 3.0-T MR imaging. Neuroradiology. 2012 Jan 25. [Medline].

11. Deguchi K, Ikeda K, Kume K, Takata T, Kokudo Y, Kamada M, et al. Significance of the hot-cross bun sign on T2*-weighted MRI for the diagnosis of multiple system atrophy. J Neurol. 2015 Jun. 262 (6):1433-9. [Medline].

12. Lewis SJ, Pavese N, Rivero-Bosch M, Eggert K, Oertel W, Mathias CJ, et al. Brain monoamine systems in multiple system atrophy: A positron emission tomography study. Neurobiol Dis. 2012 Jan 12. [Medline].

13. Jellinger KA. Neuropathology of multiple system atrophy: new thoughts about pathogenesis. Mov Disord. 2014 Dec. 29 (14):1720-41. [Medline].

14. Iodice V, Lipp A, Ahlskog JE, Sandroni P, Fealey RD, Parisi JE, et al. Autopsy confirmed multiple system atrophy cases: Mayo experience and role of autonomic function tests. J Neurol Neurosurg Psychiatry. 2012 Jan 6. [Medline].

15. Benarroch EE, Schmeichel AM, Dugger BN, Sandroni P, Parisi JE, Low PA. Dopamine cell loss in the periaqueductal gray in multiple system atrophy and Lewy body dementia. Neurology. 2009 Jul 14. 73(2):106-12. [Medline]. [Full Text].

16. Kaufmann H, Freeman R, Biaggioni I, Low P, Pedder S, Hewitt LA, et al. Droxidopa for neurogenic orthostatic hypotension: a randomized, placebo-controlled, phase 3 trial. Neurology. 2014 Jul 22. 83 (4):328-35. [Medline].

17. Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. 2009 Mar. 8(3):270-9. [Medline].

18. Donker Kaat L, Boon AJ, Azmani A, Kamphorst W, Breteler MM, Anar B, et al. Familial aggregation of parkinsonism in progressive supranuclear palsy. Neurology. 2009 Jul 14. 73(2):98-105. [Medline].

19. Boelmans K, Holst B, Hackius M, Finsterbusch J, Gerloff C, Fiehler J, et al. Brain iron deposition fingerprints in Parkinson's disease and progressive supranuclear palsy. Mov Disord. 2012 Jan 30. [Medline].

20. Boeve BF. Progressive supranuclear palsy. Parkinsonism Relat Disord. 2012 Jan. 18 Suppl 1:S192-4. [Medline].

21. Borroni B, Malinverno M, Gardoni F, Alberici A, Parnetti L, Premi E, et al. Tau forms in CSF as a reliable biomarker for progressive supranuclear palsy. Neurology. 2008 Nov 25. 71(22):1796-803. [Medline].

22. Goedert M, Jakes R. Mutations causing neurodegenerative tauopathies. Biochim Biophys Acta. 2005 Jan 3. 1739(2-3):240-50. [Medline].

23. dell'Aquila C, Zoccolella S, Cardinali V, de Mari M, Iliceto G, Tartaglione B, et al. Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients. Parkinsonism Relat Disord. 2013 Nov. 19 (11):980-5. [Medline].

24. Dubois B, Slachevsky A, Pillon B, Beato R, Villalponda JM, Litvan I. "Applause sign" helps to discriminate PSP from FTD and PD. Neurology. 2005 Jun 28. 64(12):2132-3. [Medline].

25. 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 Jun. 128:1247-58. [Medline].

26. Duff K, Gerstenecker A, Litvan I, investigators and coordinators of the ENGENE-PSP Study Group. Functional impairment in progressive supranuclear palsy. Neurology. 2013 Jan 22. 80 (4):380-4. [Medline].

27. Massey LA, Jäger 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 May 14. 80 (20):1856-61. [Medline].

28. Respondek G, Stamelou M, Kurz C, Ferguson LW, Rajput A, Chiu WZ, et al. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. Mov Disord. 2014 Dec. 29 (14):1758-66. [Medline].

29. Lang AE. Treatment of progressive supranuclear palsy and corticobasal degeneration. Mov Disord. 2005 Aug. 20 Suppl 12:S83-91. [Medline].

30. Zampieri C, Di Fabio RP. Improvement of gaze control after balance and eye movement training in patients with progressive supranuclear palsy: a quasi-randomized controlled trial. Arch Phys Med Rehabil. 2009 Feb. 90(2):263-70. [Medline].

31. Litvan I, Phipps M, Pharr VL, Hallett M, Grafman J, Salazar A. Randomized placebo-controlled trial of donepezil in patients with progressive supranuclear palsy. Neurology. 2001 Aug 14. 57(3):467-73. [Medline].

32. Mayr BJ, Bonelli RM, Niederwieser G, Költringer P, Reisecker F. Zolpidem in progressive supranuclear palsy. Eur J Neurol. 2002 Mar. 9(2):184-5. [Medline].

33. Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, DeArmond SJ, et al. Clinicopathological correlations in corticobasal degeneration. Ann Neurol. 2011 Aug. 70(2):327-40. [Medline]. [Full Text].

34. Tokumaru AM, Saito Y, Murayama S, Kazutomi K, Sakiyama Y, Toyoda M, et al. Imaging-pathologic correlation in corticobasal degeneration. AJNR Am J Neuroradiol. 2009 Nov. 30(10):1884-92. [Medline].

35. Armstrong MJ. Diagnosis and treatment of corticobasal degeneration. Curr Treat Options Neurol. 2014 Mar. 16 (3):282. [Medline].

36. Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29. 80 (5):496-503. [Medline].

37. Kouri N, Whitwell JL, Josephs KA, Rademakers R, Dickson DW. Corticobasal degeneration: a pathologically distinct 4R tauopathy. Nat Rev Neurol. 2011 May. 7(5):263-72. [Medline].

38. Rinne JO, Lee MS, Thompson PD, Marsden CD. Corticobasal degeneration. A clinical study of 36 cases. Brain. 1994 Oct. 117 ( Pt 5):1183-96. [Medline].

39. McKeith IG, Mosimann UP. Dementia with Lewy bodies and Parkinson's disease. Parkinsonism Relat Disord. 2004 May. 10 Suppl 1:S15-8. [Medline].

40. Perez F, Helmer C, Dartigues JF, Auriacombe S, Tison F. A 15-year population-based cohort study of the incidence of Parkinson's disease and dementia with Lewy bodies in an elderly French cohort. J Neurol Neurosurg Psychiatry. 2010 Jul. 81(7):742-6. [Medline].

41. Serra L, Cercignani M, Basile B, Spanò B, Perri R, Fadda L, et al. White Matter Damage along the Uncinate Fasciculus Contributes to Cognitive Decline in AD and DLB. Curr Alzheimer Res. 2012 Jan 23. [Medline].

42. Neef D, Walling AD. Dementia with Lewy bodies: an emerging disease. Am Fam Physician. 2006 Apr 1. 73(7):1223-9. [Medline].

43. McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology. 1996 Nov. 47(5):1113-24. [Medline].

44. Verghese J, Crystal HA, Dickson DW, Lipton RB. Validity of clinical criteria for the diagnosis of dementia with Lewy bodies. Neurology. 1999 Dec 10. 53(9):1974-82. [Medline].

45. Brooks DJ. Imaging amyloid in Parkinson's disease dementia and dementia with Lewy bodies with positron emission tomography. Mov Disord. 2009. 24 Suppl 2:S742-7. [Medline].

46. Villemagne VL, Okamura N, Pejoska S, Drago J, Mulligan RS, Chételat G, et al. Differential Diagnosis in Alzheimer's Disease and Dementia with Lewy Bodies via VMAT2 and Amyloid Imaging. Neurodegener Dis. 2012 Jan 17. [Medline].

47. Cordery RJ, Tyrrell PJ, Lantos PL, Rossor MN. Dementia with Lewy bodies studied with positron emission tomography. Arch Neurol. 2001 Mar. 58(3):505-8. [Medline].

48. Cummings J, Isaacson S, Mills R, Williams H, Chi-Burris K, Corbett A, et al. Pimavanserin for patients with Parkinson's disease psychosis: a randomised, placebo-controlled phase 3 trial. Lancet. 2014 Feb 8. 383 (9916):533-40. [Medline].

49. Eckert T, Barnes A, Dhawan V, Frucht S, Gordon MF, Feigin AS, et al. FDG PET in the differential diagnosis of parkinsonian disorders. Neuroimage. 2005 Jul 1. 26(3):912-21. [Medline].

50. Walter U, Behnke S, Eyding J, Niehaus L, Postert T, Seidel G, et al. Transcranial brain parenchyma sonography in movement disorders: state of the art. Ultrasound Med Biol. 2007 Jan. 33(1):15-25. [Medline].

51. Paviour DC, Thornton JS, Lees AJ, Jäger HR. Diffusion-weighted magnetic resonance imaging differentiates Parkinsonian variant of multiple-system atrophy from progressive supranuclear palsy. Mov Disord. 2007 Jan. 22(1):68-74. [Medline].

52. Stefani A, Lozano AM, Peppe A, Stanzione P, Galati S, Tropepi D, et al. Bilateral deep brain stimulation of the pedunculopontine and subthalamic nuclei in severe Parkinson's disease. Brain. 2007 Jun. 130:1596-607. [Medline].

Stephen M Bloomfield, MD Associate Professor, Department of Neurosurgery, JFK Neuroscience Institute at JFK Medical Center, Hackensack Meridian School of Medicine at Seton Hall University

Stephen M Bloomfield, MD is a member of the following medical societies: American Academy of Pain Medicine, American Association for the Advancement of Science, American Association of Neurological Surgeons, American Epilepsy Society, American Medical Association, American Pain Society, Congress of Neurological Surgeons, International Association for the Study of Pain, Society for Neuroscience, West Virginia State Medical Association

Disclosure: Nothing to disclose.

Philip A Hanna, MD Associate Professor of Neuroscience, JFK Neuroscience Institute at JFK Medical Center, Hackensack Meridian School of Medicine at Seton Hall University

Philip A Hanna, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Emad R Noor, MBChB Assistant Professor of Neurology and Clinical Neurophysiology, Hackensack Meridian School of Medicine at Seton Hall University; Attending Neurologist/Clinical Neurophysiologist, NJ Neuroscience Institute at JFK Medical Center

Emad R Noor, MBChB is a member of the following medical societies: American Academy of Neurology, American Medical Association, Egyptian Society of Neurology, Psychiatry, and Neurosurgery

Disclosure: Nothing to disclose.

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity (DigiTrace), Brain Sentinel, Cavion, Ceribell, Eisai, Greenwich, LivaNova, Neuropace, SK biopharmaceuticals, Sunovion<br/>Serve(d) as a speaker or a member of a speakers bureau for: Bioserenity (DigiTrace), Brain Sentinel, Cavion, Ceribell, Eisai, Greenwich, LivaNova, Neuropace, SK biopharmaceuticals, Sunovion<br/>Received research grant from: LivaNova, Greenwich, SK biopharmaceuticals, Takeda,.

Arif I Dalvi, MD Director, Movement Disorders Center, NorthShore University Health System; Clinical Associate Professor of Neurology, University of Chicago Pritzker Medical School

Arif I Dalvi, MD is a member of the following medical societies: International Parkinson and Movement Disorder Society, European Neurological Society

Disclosure: Nothing to disclose.

Nestor Galvez-Jimenez, MD, MSc, MHA Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society

Disclosure: Nothing to disclose.

Christopher Luzzio, MD Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment