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Cortical Basal Ganglionic Degeneration Workup

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

Laboratory Studies

See the list below:

  • 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[10]
    • Thyroid function tests, and consider thyroid autoantibody screening
    • 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

See the list below:

  • MRI of brain
    • This study is particularly helpful in evaluating the size and appearance 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).[2]
    • 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

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  • 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 may thus be difficult to distinguish pathologically from progressive supranuclear palsy.

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Staging

At this time, specific relationships between histopathological findings and clinical progression are not established. The disease course is, on average, about 7 years.

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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.

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