Cortical Basal Ganglionic Degeneration Medication

  • Author: Anna M Barrett, MD; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Feb 19, 2010
 

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.[17] 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.

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Dopaminergic medications

Class Summary

These agents are dopamine receptor agonists.

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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.

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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.

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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.

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Ropinirole (Requip)

 

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

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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.

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Amantadine (Symmetrel)

 

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

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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.

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Vitamin E (Vitec, Aquasol E)

 

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

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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.

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Ibuprofen (Motrin, Advil)

 

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

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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.

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Clonazepam (Klonopin)

 

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

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Toxins

Class Summary

Botulinum toxin can inhibit transmission of impulses in neuromuscular tissue.

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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.

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Contributor Information and Disclosures
Author

Anna M Barrett, MD  Director, Stroke Rehabilitation Research Program, Kessler Foundation Research Center; Professor of Physical Medicine and Rehabilitation and Neurology and Neurosciences, University of Medicine and Dentistry of New Jersey, New Jersey Medical School

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

Disclosure: Pfizer/Eisai Grant/research funds Other; Wallerstein Foundation for Geriatric Improvement Grant/research funds Speaking and teaching; O'Brien Technologies Grant/research funds Independent contractor

Specialty Editor Board

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, and Movement Disorders 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: Medscape Salary Employment

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.

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 Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

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 Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Acknowledgments

The author would like to thank Natalie Staats Reiss, PhD for assisting with the update of this article.

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