Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Cortical Basal Ganglionic Degeneration Medication

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

Medication Summary

Unfortunately, no treatment trials of medication for CBGD have been completed to date; thus, no regimen is reported to be highly effective in slowing or reversing its motor or cognitive symptoms.[11] In primary progressive aphasia, cholinesterase inhibitors can be tried; these have been reported to benefit patients with other tauopathies.[7] Medications for Parkinson disease, including anticholinergics, levodopa, and dopamine agonists, may improve symptoms to some extent in many patients and usually are tried at some point during the course of the disease.

Next

Dopaminergic medications

Class Summary

These agents are dopamine receptor agonists.

Memantine (Axura, Namenda)

 

This agent, approved for the treatment of Alzheimer disease in the US, has both dopaminergic and neuroprotective properties. N-methyl-D-aspartate (NMDA) antagonist. Although no published evidence can be currently identified to support its use in CBGD, theoretically this agent might slow the progression of the disorder, or improve motor function.

Levodopa/carbidopa (Sinemet)

 

Unresponsiveness to this medication supports diagnosis of CBGD; thus, an empiric trial, titrated to high dose (many advocate minimum 4 g daily), is recommended in every patient.

Bromocriptine (Parlodel)

 

Semisynthetic ergot alkaloid derivative; strong dopamine D2-receptor agonist; partial dopamine D1-receptor agonist. Stimulates dopamine receptors in corpus striatum.

Approximately 28% absorbed from GI tract and metabolized in liver. Approximate elimination half-life is 50 h with 85% excreted in feces and 3-6% eliminated in urine.

Initiate at low dosage; slowly increase dosage to individualize therapy. Maintain levodopa dosage during introductory period.

Assess dosage titration every 2 wk. Gradually reduce dose in 2.5-mg decrements if severe adverse reactions occur.

Ropinirole (Requip)

 

Often not helpful but a trial probably worthwhile for patients with disabling rigidity.

Pramipexole (Mirapex)

 

Nonergot dopamine agonist with specificity to D2 dopamine receptor but has also been shown to bind to D3 and D4 receptors and may stimulate dopamine activity on nerves of striatum and substantia nigra. Often not very helpful, but trial worthwhile.

Amantadine (Symmetrel)

 

Unknown mechanism of action; may release dopamine from dopaminergic terminals.

Previous
Next

Neuroprotective agents

Class Summary

No studies demonstrate that therapy with neuroprotective drugs slows the course of CBGD. However, such therapy does affect the course of other neurodegenerative dementias; therefore, neuroprotective agents generally are offered empirically.

Vitamin E (Vitec, Aquasol E)

 

Might protect polyunsaturated fatty acids in membranes from attack by free radicals.

Previous
Next

Nonsteroidal anti-inflammatory agents (NSAIDS)

Class Summary

Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known, but they may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may exist as well, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.

Ibuprofen (Motrin, Advil)

 

Numerous studies suggest neuroprotective effect in preventing or slowing course of dementia of Alzheimer type.

Previous
Next

Benzodiazepines

Class Summary

Useful for the management of myoclonus. By binding to specific receptor sites, these agents appear to potentiate the effects of GABA and facilitate inhibitory GABA neurotransmission and other inhibitory transmitters.

Clonazepam (Klonopin)

 

Reduced disabling myoclonus in 23% patients in one trial. Suppresses muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters.

Previous
Next

Toxins

Class Summary

These may be useful for the management of dystonia, especially painful dystonia. Botulinum toxin can inhibit transmission of impulses in neuromuscular tissue.

OnabotulinumtoxinA (BOTOX®)

 

Useful in reducing excessive, abnormal muscular contractions. Binds to receptor sites on motor nerve terminals and after uptake inhibits release of acetylcholine, blocking transmission of impulses in neuromuscular tissue.

Re-examine patients 7-14 d after administering initial dose to assess for satisfactory response.

Increase doses twofold over previously administered dose for patients who experience incomplete paralysis of target muscle. Doses of 200-300 units usually administered; maximum safe dose believed to be 400 units.

Previous
Next

Cholinesterase inhibitors

Class Summary

Galantamine may reduce symptoms of primary progressive aphasia in cortical basal ganglionic degeneration. Other cholinesterase inhibitors may also be effective, although supportive data are preliminary and only available for galatamine at this time.

Galantamine (Razadyne, Razadyne ER, Reminyl)

 

Galantamine is a competitive and reversible inhibitor of acetylcholinesterase. While the mechanism of action us unknown, it may reversibly inhibit cholinesterase, which may, in turn, increase concentrations of acetylcholinesterase available for synaptic transmission in the CNS and enhance cholinergic function. There is no evidence that acetylcholinesterase inhibitors alter the course of underlying dementia.

Previous
 
 
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].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.