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Cortical Basal Ganglionic Degeneration Follow-up

  • Author: A M Barrett, MD; Chief Editor: Selim R Benbadis, MD  more...
 
Updated: Jun 03, 2014
 

Further Outpatient Care

Periodic follow-up is appropriate to adjust dopaminergic medications or treatment for depression or other conditions. It may also help the caregiver to make plans for future care when the patient becomes more disabled.

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Further Inpatient Care

Inpatient admission can facilitate a more rapid diagnostic workup; if deterioration has been rapid with a high degree of suspicion, a brain biopsy may be advisable. This can be completed over 1-2 days with initial contact made by a case manager.

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Complications

Patients with cerebral atrophy can develop subdural hematoma after a spinal tap. Some physicians admit for 23-hour observation when performing LP to observe for headache, sleepiness, or other changes in mental status.

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Prognosis

Unfortunately, this disorder is progressive, leading to increased cognitive and motor disability. Average course of the disease is about 7 years.

Aspiration pneumonia or other complications are usually the cause of death after the patient has lost the ability to care for him/herself and has lost mobility.

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Patient Education

See the list below:

  • A geriatric case manager can be very helpful in counseling the patient and family about the prognosis, community resources, need for supervision, etc.
  • Online resources may be useful for the families and caregivers of people with CBGD. The CBGD Caregivers Report is a very useful resource, sharing frankly about the experience of living with CBGD. Some may find this a helpful sources of information and support. The NINDS has an information page on CBGD with links to organizations such as the Foundation for PSP/CBD and Related Brain Diseases (CurePSP; www.psp.org).
  • For quality care, screen patients with movement disorders for fall risk by inquiring whether patients have had 2 or more falls over the past year or one or more falls with injury. Such patients would be eligible for physical therapy assessment and aggressive fall prevention via environmental management and caregiver/patient education.
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Contributor Information and Disclosures
Author

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

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

Disclosure: Received grant/research funds from Wallerstein Foundation for Geriatric Improvement for research; Received salary from Kessler Foundation for employment; Received grant/research funds from National Institutes of Health for research; Received grant/research funds from Healthcare Foundation of NJ for research; Received grant/research funds from National Institute on Disability, Independent Living & Rehab. Research for research.

Specialty Editor Board

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.

Chief Editor

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

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

Additional Contributors

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.

Acknowledgements

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.

References
  1. 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].

  2. 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.

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

  4. 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].

  5. 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].

  6. 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].

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

  8. 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].

  9. 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].

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

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

  12. 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].

  13. 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.

  14. 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].

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

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

  17. 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].

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

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

  20. 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].

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

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

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

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

  25. 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].

  26. 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].

  27. 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.

  28. 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].

  29. 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].

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

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

  32. Wenning GK, Litvan I, Jankovic J, 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]. [Full Text].

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