Cortical Basal Ganglionic Degeneration Workup

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

Laboratory Studies

  • Ceruloplasmin - Also performed in patients with atypical parkinsonism or Parkinsonlike syndrome
  • Workup for reversible systemic causes of cognitive deficits
    • B-12 level
    • Rapid plasma reagin (RPR) or Venereal Disease Research Laboratory (VDRL) test, which may be falsely negative in patients older than 65 years, to rule out neurosyphilis[1]
    • Thyroid function tests
    • Electrolytes
    • CBC with differential and platelets
    • If appropriate or other evidence of systemic disease - Rheumatologic workup, including antinuclear antibody (ANA), erythrocyte sedimentation rate (ESR), liver function tests, and ammonia level
    • Manual smear for acanthocytes or genetic testing for Huntington disease if patient has chorea
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Imaging Studies

  • MRI of brain
    • This study is particularly helpful in evaluating the size of the midbrain if any disturbance of eye movements is noted and progressive supranuclear palsy is being considered. Midbrain size should be relatively normal in cortical basal ganglionic degeneration (CBGD).
    • Cortical atrophy usually occurs, and this can be more localized to the central sulci/supplementary motor area (SMA) and superior frontal gyrus than to the temporal/parietal cortex (the latter pattern is seen in dementia of the Alzheimer type).
    • Abnormal signal in basal ganglia can occur with metal deposition in Wilson disease or Hallervorden-Spatz disease.
  • Functional brain imaging is not generally needed, but it can be helpful in some patients to document that cognitive changes are neurological and not psychological in origin. Position emission tomography (PET) and single-photon emission computed tomography (SPECT) reveal asymmetric activity in both cortical (frontal-parietal) and subcortical (basal ganglia) regions.
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Other Tests

  • Neuropsychological testing or evaluation of limb apraxia by a cognitive neurologist, speech pathologist, or occupational therapist with advanced training and experience with neurodegenerative disorders is recommended. This can be useful to differentiate the more common patients with concomitant parkinsonism and Alzheimer disease, who also can be apraxic but should not have as severe a motor coordination deficit or alien limb sign.
  • Electroencephalography (EEG) in cases of polymyoclonus or short history of rapid decline
  • Somatosensory evoked potentials: These are not generally a part of the clinical workup. If done as part of the workup of reflex myoclonus, they should not show giant potentials.
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Procedures

In patients with prominent segmental myoclonus (especially if involving the face); eye movement disorder; and history of celiac sprue, chronic diarrhea, or unexplained arthritis, consider further workup to rule out the diagnosis of CNS Whipple disease.

  • Lumbar puncture (LP) may be done to examine cerebrospinal fluid (CSF) for cells and elevated protein; the polymerase chain reaction (PCR) test for the organism Tropheryma whippleii should also be done.
  • Consider jejunal biopsy; it can show changes characteristic of Whipple disease in the gut.
  • If diagnosis is strongly desired (familial pattern) or features are atypical (rapid course), consider brain biopsy.
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Histologic Findings

Cortical findings include frontoparietal atrophy and astrogliosis, neurophil threads, and occasionally neurofibrillary tangles and presence of swollen achromatic neurons (ballooned neurons or pale bodies). Argyrophilic tau-immunoreactive inclusion bodies can be found subcortically in the substantia nigra, where neuronal loss can also occur, as well as the basal ganglia and dentato-rubro-thalamic tracts. Although this description sounds different from that of progressive supranuclear palsy, tau-positive inclusions of CBGD may be coiled and thus they can be confused with tau-positive neurofibrillary tangles. Some cases of CBGD are thus difficult to distinguish pathologically from progressive supranuclear palsy.

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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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