eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Cortical Basal Ganglionic Degeneration: Differential Diagnoses & Workup

Author: Anna M Barrett, MD, Director, Stroke Rehabilitation Research Program, Kessler Medical Rehabilitation Research and Education Corporation; Associate Professor of Physical Medicine and Rehabilitation and Neurology and Neurosciences, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jul 9, 2007

Differential Diagnoses

Alzheimer Disease
Neuroacanthocytosis Syndromes
Anterior Circulation Stroke
Olivopontocerebellar Atrophy
Apraxia and Related Syndromes
Parkinson-Plus Syndromes
Cardioembolic Stroke
Progressive Supranuclear Palsy
Epilepsia Partialis Continua
Striatonigral Degeneration
Frontal and Temporal Lobe Dementia
Subdural Hematoma
Frontal Lobe Syndromes
Thyroid Disease
Glioblastoma Multiforme
Vitamin B-12 Associated Neurological Diseases
Huntington Disease
Whipple Disease
Hydrocephalus
Wilson Disease
Marchiafava-Bignami Disease
Neuroacanthocytosis

Other Problems to Be Considered

Dentatorubropallidoluysian atrophy
Drug-induced parkinsonism
Progressive pallidal atrophy
Vascular dementia

Workup

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
    • 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

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 CBGD.
    • Cortical atrophy usually occurs, and this can be more localized to the central sulci/supplementary motor area (SMA) 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.

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.

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.

Histologic Findings

Cortical findings include frontoparietal atrophy and astrogliosis, presence of swollen achromatic neurons (ballooned neurons or pale bodies), neuropil threads, and occasionally neurofibrillary tangles. Argyrophilic tau-immunoreactive inclusion bodies can be found subcortically in the substantia nigra, 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.

More on Cortical Basal Ganglionic Degeneration

Overview: Cortical Basal Ganglionic Degeneration
Differential Diagnoses & Workup: Cortical Basal Ganglionic Degeneration
Treatment & Medication: Cortical Basal Ganglionic Degeneration
Follow-up: Cortical Basal Ganglionic Degeneration
References
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References

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Further Reading

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Keywords

corticobasal degeneration, corticodentatonigral degeneration with neuronal achromasia, corticonigral degeneration with neuronal achromasia, extrapyramidal apractic syndrome, Pick complex disorders, Rebeiz disease, apraxia

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Contributor Information and Disclosures

Author

Anna M Barrett, MD, Director, Stroke Rehabilitation Research Program, Kessler Medical Rehabilitation Research and Education Corporation; Associate 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 Neurological Association, American Society of Neurorehabilitation, International Neuropsychological Society, and Society for Neuroscience
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Nestor Galvez-Jimenez, MD, Program Director of Movement Disorders, Department of Neurology, Division of Medicine, Director of Neurology Residency Training Program, Cleveland Clinic Florida
Nestor Galvez-Jimenez, MD is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor of Neurology, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida College of Medicine, Tampa General Hospital
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: Nothing to disclose.

Chief Editor

Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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