Cortical Basal Ganglionic Degeneration
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Cortical basal ganglionic degeneration (CBGD), a sporadic neurodegenerative tauopathy, may be considered both a syndrome of characteristic movement and cognitive dysfunction (corticobasal syndrome) and a pathologically defined disease. The corticobasal syndrome is defined by progressive dementia, parkinsonism, and limb apraxia, but these may occur as a result of a number of pathologic entities. The most characteristic are Pick complex disorders (see Pick Disease), but Alzheimer disease and even rare disorders such as CNS Whipple disease and Niemann-Pick type C can be associated with corticobasal syndrome. Histopathologically identifiable CBGD can also present clinically as primary progressive aphasia [1] or primary progressive apraxia in patients who had no prominent movement disorders earlier in their lives.
Both cortical and subcortical abnormalities are seen. The disorder is currently classified as a 4-repeat tauopathy, and although tau-immunoreactive neuronal and glial inclusions may be seen in Pick disease, progressive supranuclear palsy (PSP), and even Alzheimer disease, these disorders may differ in the proportions of 4-repeat as compared with 3-repeat microtubule-associated tau protein isoforms, with CBGD and progressive supranuclear palsy being the 2 predominantly 4-repeat tauopathies. Both neurons and glial cells are involved, and both cortical (pyramidal and nonpyramidal) neurons, as well as subcortical regions. Ballooned swollen neurons with loss of cytoplasmic staining (ie, achromasia) are a supportive feature when present in the cortex and the basal ganglia. CBGD may be associated with both cortical and subcortical neuronal loss, neuronal and glial tau pathology (including astrocytic plaques); cortical loss, predominantly affecting motor and premotor regions, [2] may distinguish this disorderfrom progressive supranuclear palsy.
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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 about 5% of cases of parkinsonism seen in clinics that specialize in movement disorders, or 0.62-0.92 per 100,000 per year, with an estimated prevalence of 4.9-7.3 per 100,000. [3] A study in Eastern European and Asian subjects reported an incidence of 0.02 case per 100,000 people. [4]
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, within 10 years of onset.
No racial predilection is known.
In several studies, CBDG was reported to be more common in women. [3]
Typically, CBGD presents between the ages of 50 and 80 years. No pathologically confirmed case of CBGD has ever been published with onset before 45 years, but the author of this article personally reviewed medical records for a man who died with pathologically confirmed CBGD whose first symptoms occurred at age 41 years, and a patient with the onset of corticobasal syndrome at age 28 years has been reported. [5]
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A M Barrett, MD, FAAN, FANA, FASNR Director, Stroke Rehabilitation Research Program, Kessler Foundation; Chief, Neurorehabilitation Program Innovation, Kessler Institute for Rehabilitation; Research Professor of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School
A M Barrett, MD, FAAN, FANA, FASNR is a member of the following medical societies: American Academy of Neurology, American Society of Neurorehabilitation, International Neuropsychological Society
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Kessler Foundation<br/>Received research grant from: SPR Therapeutics; DART Neuroscience; Wallerstein Foundation for Geriatric Improvement; NJ Commission on Brain Injury Research; NIDILRR; NIH.
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.
Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani 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, American Medical Association
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Acorda, Livanova, Eisai, Greenwich, Lundbeck, Neuropace, Sunovion, Upsher-Smith.<br/>Serve(d) as a speaker or a member of a speakers bureau for: Livanova, Eisai, Greenwich, Lundbeck, Neuropace, Sunovion.<br/>Received research grant from: Acorda, Livanova, Greenwich, Lundbeck, Sepracor, Sunovion, UCB, Upsher-Smith.
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.
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.
Cortical Basal Ganglionic Degeneration
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