Corticobasal Syndrome and Corticobasal Degeneration Differential Diagnoses

1. 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].

2. 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].

3. Boeve BF. The multiple phenotypes of corticobasal syndrome and corticobasal degeneration: implications for further study. J Mol Neurosci. 2011 Nov. 45(3):350-3. [Medline].

4. Alexander SK, Rittman T, Xuereb JH, Bak TH, Hodges JR, Rowe JB. Validation of the new consensus criteria for the diagnosis of corticobasal degeneration. J Neurol Neurosurg Psychiatry. 2014 Aug. 85 (8):925-9. [Medline].

5. Schofield EC, Caine D, Kril JJ, Cordato NJ, Halliday GM. Staging disease severity in movement disorder tauopathies: brain atrophy separates progressive supranuclear palsy from corticobasal degeneration. Mov Disord. 2005 Jan. 20(1):34-9. [Medline].

6. Whitwell JL, Jack CR Jr., Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, et al. Imaging correlates of pathology in corticobasal syndrome. Neurology. 2010. 75:1879-1887.

7. Kovacs GG, Xie SX, Robinson JL, Lee EB, Smith DH, Schuck T, et al. Sequential stages and distribution patterns of aging-related tau astrogliopathy (ARTAG) in the human brain. Acta Neuropathol Commun. 2018 Jun 11. 6 (1):50. [Medline].

8. Liu F, Gong CX. Tau exon 10 alternative splicing and tauopathies. Mol Neurodegener. 2008 Jul 10. 3:8. [Medline].

9. Bhaskar K, Konerth M, Kokiko-Cochran ON, Cardona A, Ransohoff RM, Lamb BT. Regulation of tau pathology by the microglial fractalkine receptor. Neuron. 2010 Oct 6. 68 (1):19-31. [Medline].

10. Ishizawa K, Dickson DW. Microglial activation parallels system degeneration in progressive supranuclear palsy and corticobasal degeneration. J Neuropathol Exp Neurol. 2001 Jun. 60 (6):647-57. [Medline].

11. Nardone R, Brigo F, Versace V, Sebastianelli L, Christova M, Golaszewski S, et al. Pathophysiology of corticobasal degeneration: Insights from neurophysiological studies. J Clin Neurosci. 2019 Feb. 60:17-23. [Medline].

12. Ahmed S, Fairen MD, Sabir MS, Pastor P, Ding J, Ispierto L, et al. MAPT p.V363I mutation: A rare cause of corticobasal degeneration. Neurol Genet. 2019 Aug. 5 (4):e347. [Medline].

13. Houlden H, Baker M, Morris HR, et al. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology. 2001 Jun 26. 56 (12):1702-6. [Medline].

14. Kouri N, Ross OA, Dombroski B, et al. Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy. Nat Commun. 2015 Jun 16. 6:7247. [Medline].

15. Bergeron C, Pollanen MS, Weyer L. Unusual clinical presentations of cortical-basal ganglionic degeneration. Ann Neurol. 1996 Dec. 40(6):893-900. [Medline].

16. Winter Y, Bezdolnyy Y, Katunina E, et al. Incidence of Parkinson's disease and atypical parkinsonism: Russian population-based study. Mov Disord. 2010 Feb 15. 25(3):349-56. [Medline].

17. Kompoliti K, Goetz CG, Boeve BF, Maraganore DM, Ahlskog JE, Marsden CD, et al. Clinical presentation and pharmacological therapy in corticobasal degeneration. Arch Neurol. 1998 Jul. 55 (7):957-61. [Medline].

18. 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 Feb. 64 (2):184-9. [Medline].

19. 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 Apr 17. 68 (16):1274-83. [Medline].

20. DePold Hohler A, Ransom BR, Chun MR, Tröster AI, Samii A. The youngest reported case of corticobasal degeneration. Parkinsonism Relat Disord. 2003 Oct. 10(1):47-50. [Medline].

21. Coyle-Gilchrist IT, Dick KM, Patterson K, Vázquez Rodríquez P, Wehmann E, Wilcox A, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology. 2016 May 3. 86 (18):1736-43. [Medline].

22. Kansal K, Mareddy M, Sloane KL, Minc AA, Rabins PV, McGready JB, et al. Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis. Dement Geriatr Cogn Disord. 2016. 41 (1-2):109-22. [Medline].

23. Lansdall CJ, Coyle-Gilchrist ITS, Vázquez Rodríguez P, Wilcox A, Wehmann E, Robbins TW, et al. Prognostic importance of apathy in syndromes associated with frontotemporal lobar degeneration. Neurology. 2019 Apr 2. 92 (14):e1547-e1557. [Medline].

24. Crutch SJ, Schott JM, Rabinovici GD, et al. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017 Aug. 13 (8):870-884. [Medline].

25. Pantelyat A, Dreyfuss M, Moore P, Gross R, Schuck T, Irwin D, et al. Acalculia in autopsy-proven corticobasal degeneration. Neurology. 2011 Feb 15. 76 (7 Suppl 2):S61-3. [Medline].

26. Armstrong RA. Visual signs and symptoms of corticobasal degeneration. Clin Exp Optom. 2016 Nov. 99 (6):498-506. [Medline].

27. Parmera JB, Rodriguez RD, Studart Neto A, Nitrini R, Brucki SMD. Corticobasal syndrome: A diagnostic conundrum. Dement Neuropsychol. 2016 Oct-Dec. 10 (4):267-275. [Medline].

28. Pardini M, Huey ED, Spina S, Kreisl WC, Morbelli S, Wassermann EM, et al. FDG-PET patterns associated with underlying pathology in corticobasal syndrome. Neurology. 2019 Mar 5. 92 (10):e1121-e1135. [Medline].

29. Benito-León J, Alvarez-Linera J, Louis ED. Neurosyphilis masquerading as corticobasal degeneration. Mov Disord. 2004 Nov. 19(11):1367-70. [Medline].

30. Ge F, Ding J, Liu Y, Lin H, Chang T. Cerebrospinal fluid NFL in the differential diagnosis of parkinsonian disorders: A meta-analysis. Neurosci Lett. 2018 Oct 15. 685:35-41. [Medline].

31. Miyata M, Kakeda S, Toyoshima Y, Ide S, Okada K, Adachi H, et al. Potential usefulness of signal intensity of cerebral gyri on quantitative susceptibility mapping for discriminating corticobasal degeneration from progressive supranuclear palsy and Parkinson's disease. Neuroradiology. 2019 Nov. 61 (11):1251-1259. [Medline].

32. Hammesfahr S, Antke C, Mamlins E, Beu M, Wojtecki L, Ferrea S, et al. FP-CIT- and IBZM-SPECT in Corticobasal Syndrome: Results from a Clinical Follow-Up Study. Neurodegener Dis. 2016. 16 (5-6):342-7. [Medline].

33. McMillan CT, Irwin DJ, Nasrallah I, Phillips JS, Spindler M, Rascovsky K, et al. Multimodal evaluation demonstrates in vivo ¹⁸F-AV-1451 uptake in autopsy-confirmed corticobasal degeneration. Acta Neuropathol. 2016 Dec. 132 (6):935-937. [Medline].

34. Leuzy A, Chiotis K, Lemoine L, Gillberg PG, Almkvist O, Rodriguez-Vieitez E, et al. Tau PET imaging in neurodegenerative tauopathies-still a challenge. Mol Psychiatry. 2019 Aug. 24 (8):1112-1134. [Medline].

35. McMonagle P, Blair M, Kertesz A. Corticobasal degeneration and progressive aphasia. Neurology. 2006 Oct 24. 67(8):1444-51. [Medline].

36. Heilman KM, Rothi LJG. Apraxia. Heilman KM, Valenstein E, eds. Clinical Neuropsychology. 2nd ed. New York: Oxford University Press; 1985. 131-50.

37. Moretti DV. Available and future treatments for atypical parkinsonism. A systematic review. CNS Neurosci Ther. 2019 Feb. 25 (2):159-174. [Medline].

38. Cho JW, Lee JH. Suppression of myoclonus in corticobasal degeneration by levetiracetam. J Mov Disord. 2014 Apr. 7 (1):28-30. [Medline].

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

40. Boxer AL, Geschwind MD, Belfor N, et al. Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Arch Neurol. 2006 Jan. 63(1):81-6. [Medline].

41. Kertesz A, Morlog D, Light M, et al. Galantamine in frontotemporal dementia and primary progressive aphasia. Dement Geriatr Cogn Disord. 2008. 25(2):178-85. [Medline].

42. Borroni B, Garibotto V, Agosti C, et al. White matter changes in corticobasal degeneration syndrome and correlation with limb apraxia. Arch Neurol. 2008 Jun. 65(6):796-801. [Medline].

43. Duda GK, Slowinski J, Opala G, Gorzkowska A, Myga BJ, Wszolek ZK, et al. Corticobasal degeneration-clinicopathological considerations. Folia Neuropathol. 2006. 44(4):257-264.

44. Fukui T, Sugita K, Kawamura M, Shiota J, Nakano I. Primary progressive apraxia in Pick's disease: a clinicopathologic study. Neurology. 1996 Aug. 47(2):467-73. [Medline].

45. Heilman KM. The apraxia of CBGD. Mov Disord. 1996. 11:348.

46. Josephs KA. Frontotemporal dementia and related disorders: deciphering the enigma. Ann Neurol. 2008 Jul. 64(1):4-14. [Medline].

47. Kertesz A, Blair M, McMonagle P, Munoz DG. The diagnosis and course of frontotemporal dementia. Alzheimer Dis Assoc Disord. 2007 Apr-Jun. 21(2):155-63. [Medline].

48. Lang AE, Riley DE, Bergeron C. Cortical-basal ganglionic degeneration. Calne DB, ed. Neurodegenerative Diseases. Philadelphia: WB Saunders; 1994. 877-94.

49. Leiguarda R, Lees AJ, Merello M, Starkstein S, Marsden CD. The nature of apraxia in corticobasal degeneration. J Neurol Neurosurg Psychiatry. 1994 Apr. 57(4):455-9. [Medline]. [Full Text].

50. Paulus W, Selim M. Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol. 1990. 81(1):89-94. [Medline].

51. Rebeiz JJ, Kolodny EH, Richardson EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol. 1968 Jan. 18(1):20-33. [Medline].

52. Reich SG, Grill SE. Corticobasal degeneration. Curr Treat Options Neurol. 2009 May. 11(3):179-85. [Medline].

53. Riley DE, Lang AE, Lewis A, et al. Cortical-basal ganglionic degeneration. Neurology. 1990 Aug. 40(8):1203-12. [Medline].

54. Sakurai Y, Hashida H, Uesugi H, et al. A clinical profile of corticobasal degeneration presenting as primary progressive aphasia. Eur Neurol. 1996. 36(3):134-7. [Medline].

55. Sano M, Ernesto C, Thomas RG, et al. A controlled trial of selegiline, alpha-tocopherol, or both as treatment for Alzheimer's disease. The Alzheimer's Disease Cooperative Study. N Engl J Med. 1997 Apr 24. 336(17):1216-22. [Medline].

56. Schellenberg GD, Höglinger G, Sleiman P, Rademakers R, Lambertus K, deSilva R, et al. A genome-wide association study of progressive supranuclear pals (PSP) and corticobasal degeneration (CBD). Alzh Dis Assoc Dis. 2010. 6(4, Suppl):S84-85.

57. Takao M, Tsuchiya K, Mimura M, et al. Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case. Neuropathology. 2006 Dec. 26(6):569-78. [Medline].

58. Togasaki DM, Tanner CM. Epidemiologic aspects. Adv Neurol. 2000. 82:53-9. [Medline].

59. Watts RL, Williams RS, Growden JD. Corticobasal ganglionic degeneration. Neurology (Cleveland). 1985. 35 (Suppl 1):178.

60. Giagkou N, Stamelou M. Therapeutic Management of the Overlapping Syndromes of Atypical Parkinsonism. CNS Drugs. 2018 Sep. 32 (9):827-837. [Medline].

61. Lamb R, Rohrer JD, Lees AJ, Morris HR. Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options. Curr Treat Options Neurol. 2016 Sep. 18 (9):42. [Medline].

62. Marsili L, Suppa A, Berardelli A, Colosimo C. Therapeutic interventions in parkinsonism: Corticobasal degeneration. Parkinsonism Relat Disord. 2016 Jan. 22 Suppl 1:S96-100. [Medline].

63. Hodges JR, Davies R, Xuereb J, Kril J, Halliday G. Survival in frontotemporal dementia. Neurology. 2003 Aug 12. 61 (3):349-54. [Medline].

64. Hassan A, Whitwell JL, Boeve BF, Jack CR Jr, Parisi JE, Dickson DW, et al. Symmetric corticobasal degeneration (S-CBD). Parkinsonism Relat Disord. 2010 Mar. 16 (3):208-14. [Medline].

65. Ali F, Josephs KA. Corticobasal degeneration: key emerging issues. J Neurol. 2018 Feb. 265 (2):439-445. [Medline].

Alexander Pantelyat, MD Assistant Professor, Department of Neurology, Johns Hopkins University School of Medicine; Director, Johns Hopkins Atypical Parkinsonism Center; Co-Director, Johns Hopkins Center for Music and Medicine; Co-Director, Johns Hopkins Movement Disorders Fellowship Program

Alexander Pantelyat, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Association for Music and Medicine, International Parkinson and Movement Disorder Society, Maryland State Medical Society

Disclosure: Nothing to disclose.

Maitreyi Murthy, MBBS, MPhil Clinical and Research Fellow, Department of Neurology, Movement Disorders Division, Johns Hopkins University School of Medicine

Maitreyi Murthy, MBBS, MPhil is a member of the following medical societies: American Academy of Neurology, Movement Disorder Society

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: Received salary from Medscape for employment. for: Medscape.

Nestor Galvez-Jimenez, MD, MSc, MHA The Pauline M Braathen Endowed Chair in Neurology, 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, International Parkinson and Movement Disorder Society

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: Alliance, Bioserenity, Ceribell, Eisai, Greenwich, LivaNova, Neurelis, Neuropace, Nexus, RSC, SK life science, Sunovion<br/>Serve(d) as a speaker or a member of a speakers bureau for: Alliance, Aquestive, Bioserenity, Eisai, Greenwich, LivaNova, Neurelis, SK life science, Sunovion<br/>Received research grant from: Cerevel, LivaNova, Greenwich, SK biopharmaceuticals, Takeda.

A M Barrett, MD, FAAN, FANA, FASNR Director, Stroke Rehabilitation Research Program, Kessler Foundation; Chief, Neurorehabilitation Program Innovation, Kessler Institute for Rehabilitation; Research Professor of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School

A M Barrett, MD, FAAN, FANA, FASNR is a member of the following medical societies: American Academy of Neurology, American Society of Neurorehabilitation, International Neuropsychological Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Kessler Foundation<br/>Received research grant from: SPR Therapeutics; DART Neuroscience; Wallerstein Foundation for Geriatric Improvement; NJ Commission on Brain Injury Research; NIDILRR; NIH.

Stephen T Gancher, MD Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University

Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Thanks are owed to the family of the man who provided medical records for the author's review, to confirm that pathologically confirmed corticobasal syndrome has occurred with onset before age 45 years.

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