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Cortical Basal Ganglionic Degeneration Clinical Presentation

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

History

Boeve et al describe the following clinical symptoms as part of the phenotypic syndromes of cortical basal ganglionic degeneration (CBGD)[6] :

  • 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[7, 8] - Disorder of skilled, learned, purposeful movement; this is one of the few disorders in which limb apraxia can appear in the history (ie, patients can be aware of the apraxia)
    • Alien limb ("My hand/leg has a mind of its own.")
  • Cortical (parietal) sensory loss
  • Visual or tactile spatial neglect
  • Constructional apraxia
  • Focal or asymmetric myoclonus
  • Apraxia of speech or nonfluent aphasia
  • Extrapyramidal dysfunction, including at least 1 of the following:
    • Focal or asymmetric appendicular rigidity (unresponsive to levodopa)[9]
    • Focal or asymmetric appendicular dystonia
  • In some cases, associated depression and postural instability
  • Unusual presentations, for example, primary progressive aphasia and progressive buccofacial apraxia
  • The presence of prominent delusions or hallucinations (not related to levodopa) suggests the patient does not have CBGD; they are more characteristic of diffuse Lewy body disease
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Physical

See the list below:

  • 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.[8]
  • 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.
  • Proprioceptive loss is not part of the syndrome; rather, this suggest abnormal movement may be related to PNS pathology (eg, neuropathy)
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Causes

See the list below:

  • 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 the genetic basis of the 4-repeat tauopathies in order to identify susceptible individuals and potential molecular targets for treatment. At this point, however, no definite relationship between clinically available genetic markers and CBGD has been demonstrated, although the gene for microtubular-associated protein tau (MAPT) on 17q has been of great interest in this and the other neurodegenerative conditions associated with tauopathies: progressive supranuclear palsy, Pick disease/frontotemporal dementia).
  • 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

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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