eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Cortical Basal Ganglionic Degeneration

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

Introduction

Background

Cortical basal ganglionic degeneration (CBGD) may be considered a syndrome rather than a disease. Its defining clinical characteristics (ie, progressive dementia, parkinsonism, limb apraxia) may occur as a result of heterogenous neuropathological conditions such as Pick complex disorders (see Pick Disease), Alzheimer disease, and even rare disorders such as CNS Whipple disease and Niemann-Pick type C. Histopathologically identifiable CBGD can also present clinically as primary progressive aphasia or primary progressive apraxia in patients who had no prominent movement disorders earlier in their lives.

Pathophysiology

Both cortical and subcortical abnormalities are seen. Ballooned swollen neurons with loss of cytoplasmic staining (ie, achromasia) are present in the cortex and also may be seen in the basal ganglia. As in Pick disease and progressive supranuclear palsy, tau-immunoreactive neuronal and glial inclusions may be seen in cortical (pyramidal and nonpyramidal) neurons as well as subcortical regions. However, inclusions in CBGD, unlike Pick bodies, are not immunoreactive to ubiquitin.

Frequency

United States

Data on incidence and prevalence of this disorder are still being collected. Clinical reports have multiplied geometrically in the last 20 years, suggesting either that clinical evaluation has become more sensitive or that the syndrome is appearing more frequently. It is estimated to account for less than 3% of cases of parkinsonism seen in clinics that specialize in movement disorders.

Mortality/Morbidity

This is a progressive neurodegenerative disorder with increasing levels of disability and loss of independence. Individuals with CBGD do not usually die of the disorder itself but of complications of the bedridden state, such as aspiration pneumonia and infections.

Race

No racial predilection is known.

Sex

No sexual predilection is known.

Age

Typically, CBGD presents in the sixth or seventh decade of life. No pathologically confirmed case of CBGD has ever had its onset at an age younger than 45 years.

Clinical

History

Lang proposed the clinical criteria for diagnosis of cortical basal ganglionic degeneration (CBGD) (enumerated here with some revisions); it usually is accompanied by the pathology of cortical basal ganglionic degeneration.

  • Chronic progressive course
  • Asymmetric onset of extrapyramidal dysfunction
  • Higher cortical dysfunction
    • Limb apraxia - 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.")
  • Movement disorder
    • Rigid/akinetic syndrome resistant to therapeutic doses of levodopa
    • Dystonic limb posturing (not purely action induced)
    • Spontaneous and reflex focal myoclonus
    • According to Fahn, occasional action tremor
  • 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.

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.

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.

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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