Cortical Basal Ganglionic Degeneration Treatment & Management

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

Medical Care

  • On first evaluation, discontinue anticholinergics or other medications that impair attention and memory. Discontinue any medications that may cause parkinsonism. Start vitamin E if the patient has memory loss. Consider empiric treatment of depression and initiate a trial of levodopa/carbidopa (Sinemet) if rigidity and movement disorder are disabling. Institute a plan for titration of this medication to an appropriate level before declaring the patient to be a "levodopa/carbidopa failure." Consider botulinum toxin injections if the patient has painful limb dystonia. Obtain an EEG if the patient has polymyoclonus or rapid decline. Refer to occupational, physical, and speech therapists, as needed, for gait and safety evaluation, assistive devices, and an exercise program to maintain endurance and strength.
  • On second evaluation, treat any systemic conditions identified on serologic testing. Discontinue Sinemet if ineffective, and begin empiric trial of second- or third-line dopaminergic agent or consider treatment with clonazepam for myoclonus. Consider a spinal tap if any symptoms suggestive of CNS Whipple disease are present; discuss this possibility with the patient and family. Refer the patient to a geriatric nurse practitioner, case manager, or other dementia resource persons when available. Share reading material on CBGD and dementia with the patient and family. Coordinate consultation with a behavioral neurologist or movement disorder specialist if the family desires.
  • On third evaluation, treat any systemic conditions further identified, perform spinal tap, and consider brain biopsy if the diagnosis is still in doubt or if the family or patient may benefit. Consider further adjustment of dopaminergic therapies depending upon clinical response.
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Consultations

  • Physical and occupational therapist: Sometimes physical and occupational therapy can be helpful to maintain endurance in patients with impaired gait or to teach patients with visual agnosia different strategies for performing activities of daily living.
  • Speech therapist: These professionals can train patients with primary progressive aphasia or buccofacial apraxia to use an assistive communication device, similar to those used by patients with amyotrophic lateral sclerosis and other neuromuscular diseases. This training must be instituted early when patients are still capable of learning procedural/motor skills. If the diagnosis is in doubt, speech therapists with graduate training also can assess apraxia with quantitative standardized tests.
  • A geriatric case manager, nurse clinician, or social worker can be very helpful in counseling patients and their families on issues relating to increasing disability and, ultimately, end-of-life care.
  • Many patients may wish to visit, if only once, a specialist in the area of behavioral neurology or movement disorders to confirm the diagnosis. Some patients find taking part in research studies extremely gratifying as this disorder remains untreatable.
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Diet

  • Dysphagia may occur in some patients with prominent buccofacial apraxia.
  • Speech therapy consultation for swallowing evaluation is recommended.
  • Thickened liquids or soft foods (depending upon degree of impairment) may be necessary.
  • Constipation is treated with conservative measures such increased fluid intake, high-fiber diets, encouragement of physical activity, stool softeners, and laxatives if necessary.
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Activity

Activity is not restricted, but motor assistance is required as the disease progresses.

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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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