Cortical Basal Ganglionic Degeneration Clinical Presentation

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

History

Boeve et al proposed the following clinical diagnostic criteria for cortical basal ganglionic degeneration (CBGD):[3]

  • Insidious onset and progressive course
  • No identifiable alternative cause (stroke, tumor, etc)
  • Cortical dysfunction including at least 1 of the following:
    • Focal or asymmetric ideomotor apraxia[37] : Disorder of skilled, learned, purposeful movement; this is one of the few disorders in which limb apraxia can appear in the history (ie, patients are often aware of the apraxia).
    • Alien limb ("My hand/leg has a mind of its own.")
  • Cortical (parietal) sensory loss
  • Visual or tactile neglect
  • Constructional apraxia
  • Focal or asymmetric myoclonus
  • Apraxia of speech or nonfluent aphasia[35]
  • Extrapyramidal dysfunction, including at least 1 of the following:
    • Focal or asymmetric appendicular rigidity (unresponsive to levodopa)[37]
    • Focal or asymmetric appendicular dystonia
  • In some cases, associated depression and postural instability
  • Unusual presentations, for example, primary progressive aphasia and progressive buccofacial apraxia
  • Prominent delusions or hallucinations (not related to levodopa): These suggest that the patient does not have CBGD; they are more characteristic of diffuse Lewy body disease.
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Physical

  • Limb apraxia: Patients must make errors beyond using a body part as a tool (eg, using fingers as scissor blades). Errors often suggest ideomotor or limb-kinetic apraxia.
  • Other mental abnormalities include the following:
    • Amnesia
    • Generally, no "cognitive" abnormalities (eg, right-left disorientation, naming difficulty, acalculia), but rather "frontal-executive" deficits (eg, distractibility, perseveration, loss of judgment, motor planning deficit even on the less motor-impaired side)
  • Eye movements: These can be impaired, with restricted horizontal movements as well as upgaze; restricted downgaze is suggestive of progressive supranuclear palsy.
  • Dystonia: This is not purely action induced.
  • Myoclonus: Myoclonus must spread beyond fingers if stimulus sensitive.
  • Rigidity: This must be elicited easily without reinforcement.
  • No resting tremor is present.
  • No autonomic disturbance is present.
  • Cortical sensory loss: Loss of graphesthesia (ability to identify a letter drawn in the hand or on the finger) can be a sensitive test.
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Causes

  • The cause of CBGD is unknown.
  • Case reports suggest that a familial predisposition may exist in some individuals with this disorder.
  • Because of the clinical and pathologic relationships between CBGD, progressive supranuclear palsy, and Pick disease, interest in this disease has focused on chromosome arm 17q markers. At this point, however, no definite relationship between genetic markers in this region and CBGD has been demonstrated.
  • Limb apraxia and eye movement abnormalities appear to be highly associated with left cortical structural change.
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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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