Cortical Basal Ganglionic Degeneration
- Author: Anna M Barrett, MD; Chief Editor: Selim R Benbadis, MD more...
Background
Cortical basal ganglionic degeneration (CBGD), a sporadic neurodegenerative tauopathy, 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[26] or primary progressive apraxia in patients who had no prominent movement disorders earlier in their lives.
Pathophysiology
Both cortical and subcortical abnormalities are seen. 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.[9] 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; cortical loss may distinguish this disorder from progressive supranuclear palsy.[32]
Epidemiology
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 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.
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, within 10 years of onset.
Race
No racial predilection is known.
Sex
CBDG may be more common in women.
Age
Typically, CBGD presents between the ages of 60 and 80. No pathologically confirmed case of CBGD has ever been published with onset before 45 years, but the author of this chapter personally reviewed medical records for a man who died with pathologically confirmed CBGD whose first symptoms occurred at age 41.
Benito-León J, Alvarez-Linera J, Louis ED. Neurosyphilis masquerading as corticobasal degeneration. Mov Disord. Nov 2004;19(11):1367-70. [Medline].
Bergeron C, Pollanen MS, Weyer L. Unusual clinical presentations of cortical-basal ganglionic degeneration. Ann Neurol. Dec 1996;40(6):893-900. [Medline].
Boeve BF, Maraganore DM, Parisi JE et al. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology. Sep 11 1999;53(4):795-800. [Medline].
Fahn S, Przedborski S. Parkinsonism. In: Rowland LP, ed. Merritt's Textbook of Neurology. 11th ed. Baltimore: Williams & Wilkins; 2005:720.
Feany MB, Mattiace LA, Dickson DW. Neuropathologic overlap of progressive supranuclear palsy, Pick's disease and corticobasal degeneration. J Neuropathol Exp Neurol. Jan 1996;55(1):53-67. [Medline].
Fukui T, Sugita K, Kawamura M. Primary progressive apraxia in Pick's disease: a clinicopathologic study. Neurology. Aug 1996;47(2):467-73. [Medline].
Gibb WR, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain. Oct 1989;112 ( Pt 5):1171-92. [Medline].
Heilman KM. The apraxia of CBGD. Mov Disord. 1996;11:348.
Heilman KM, Rothi LJG. Apraxia. In: Heilman KM, Valenstein E, eds. Clinical Neuropsychology. 2nd ed. New York: Oxford University Press; 1985:131-50.
Kertesz A. Frontotemporal dementia, Pick disease, and corticobasal degeneration. One entity or 3? 1. Arch Neurol. Nov 1997;54(11):1427-9. [Medline].
Kertesz A, Hudson L, Mackenzie IR. The pathology and nosology of primary progressive aphasia. Neurology. Nov 1994;44(11):2065-72. [Medline].
Kertesz A, McMonagle P, Blair M, Davidson W, Munoz DG. The evolution and pathology of frontotemporal dementia. Brain. Sep 2005;128(Pt 9):1996-2005. [Medline].
Kertesz A, Munox DG. Pick's Disease and Pick complex. New York: Wiley-Liss; 1998.
Kompoliti K, Goetz CG, Boeve BF. Clinical presentation and pharmacological therapy in corticobasal degeneration. Arch Neurol. Jul 1998;55(7):957-61. [Medline].
Lang AE, Riley DE, Bergeron C. Cortical-basal ganglionic degeneration. In: Calne DB, ed. Neurodegenerative Diseases. Philadelphia: WB Saunders; 1994:877-94.
Leiguarda R, Lees AJ, Merello M. The nature of apraxia in corticobasal degeneration. J Neurol Neurosurg Psychiatry. Apr 1994;57(4):455-9. [Medline].
McMonagle P, Blair M, Kertesz A. Corticobasal degeneration and progressive aphasia. Neurology. Oct 24 2006;67(8):1444-51. [Medline].
Paulus W, Selim M. Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol (Berl). 1990;81(1):89-94. [Medline].
Rebeiz JJ, Kolodny EH, Richardson EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol. Jan 1968;18(1):20-33. [Medline].
Riley DE, Lang AE, Lewis A. Cortical-basal ganglionic degeneration. Neurology. Aug 1990;40(8):1203-12. [Medline].
Sakurai Y, Hashida H, Uesugi H. A clinical profile of corticobasal degeneration presenting as primary progressive aphasia. Eur Neurol. 1996;36(3):134-7. [Medline].
Sano M, Ernesto C, Thomas RG. A controlled trial of selegiline, alpha-tocopherol, or both as treatment for Alzheimer's disease. The Alzheimer's Disease Cooperative Study. N Engl J Med. Apr 24 1997;336(17):1216-22. [Medline].
Schofield EC, Caine D, Kril JJ, Cordato NJ, Halliday GM. Staging disease severity in movement disorder tauopathies: brain atrophy separates progressive supranuclear palsy from corticobasal degeneration. Mov Disord. Jan 2005;20(1):34-9. [Medline].
Watts RL, Williams RS, Growden JD. Corticobasal ganglionic degeneration. Neurology (Cleveland). 1985;35 (Suppl 1):178.
Borroni B, Garibotto V, Agosti C, Brambati SM, Bellelli G, Gasparotti R. White matter changes in corticobasal degeneration syndrome and correlation with limb apraxia. Arch Neurol. Jun 2008;65(6):796-801. [Medline].
Boxer AL, Geschwind MD, Belfor N, Gorno-Tempini ML, Schauer GF, Miller BL. Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Arch Neurol. Jan 2006;63(1):81-6. [Medline].
Duda GK, Slowinski J, Opala G, Gorzkowska A, Myga BJ, Wszolek ZK, et al. Corticobasal degeneration-clinicopathological considerations. Folia Neuropathol. 2006;44(4):257-264.
Reich SG, Grill SE. Corticobasal degeneration. Curr Treat Options Neurol. May 2009;11(3):179-85. [Medline].
Takao M, Tsuchiya K, Mimura M, Momoshima S, Kondo H, Akiyama H. Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case. Neuropathology. Dec 2006;26(6):569-78. [Medline].
Togasaki DM, Tanner CM. Epidemiologic aspects. Adv Neurol. 2000;82:53-9. [Medline].
Wenning GK, Litvan I, Jankovic J, Granata R, Mangone CA, McKee A, et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry. Feb 1998;64(2):184-9. [Medline].

