Cortical Basal Ganglionic Degeneration Treatment & Management
- Author: A M Barrett, MD; Chief Editor: Selim R Benbadis, MD more...
See the list below:
- On first evaluation, discontinue anticholinergics or other medications that impair attention and memory. Discontinue any medications that may cause parkinsonism. Consider antioxidants or vitamin E if the patient has memory loss. Consider empiric treatment of depression and initiate a trial of levodopa/carbidopa (Sinemet) if rigidity and movement disorder are disabling. Institute a plan for titration of this medication to an appropriate level before declaring the patient to be a "levodopa/carbidopa failure." Consider botulinum toxin injections if the patient has painful limb dystonia. Obtain an EEG if the patient has polymyoclonus or rapid decline. Refer to occupational, physical, and speech therapists, as needed, for gait and safety evaluation, assistive devices, and an exercise program to maintain endurance and strength.
- On second evaluation, treat any systemic conditions identified on serologic testing. Discontinue Sinemet if ineffective, and begin empiric trial of second- or third-line dopaminergic agent or consider treatment with clonazepam for myoclonus. Consider a spinal tap if any symptoms suggestive of CNS Whipple disease are present; discuss this possibility with the patient and family. Refer the patient to a geriatric nurse practitioner, case manager, or other dementia resource persons when available. Share reading material on CBGD and dementia with the patient and family. Coordinate consultation with a behavioral neurologist or movement disorder specialist if the family desires.
- On third evaluation, treat any systemic conditions further identified, perform spinal tap, and consider brain biopsy if the diagnosis is still in doubt or if the family or patient may benefit. Consider further adjustment of dopaminergic therapies depending upon clinical response.
See the list below:
- Physical and occupational therapist: Sometimes physical and occupational therapy can be helpful to maintain endurance in patients with impaired gait or to teach patients with visual agnosia different strategies for performing activities of daily living.
- Speech therapist: These professionals can train patients with primary progressive aphasia or buccofacial apraxia to use an assistive communication device, similar to those used by patients with amyotrophic lateral sclerosis and other neuromuscular diseases. This training must be instituted early when patients are still capable of learning procedural/motor skills. If the diagnosis is in doubt, speech therapists with graduate training also can assess apraxia with quantitative standardized tests.
- A geriatric case manager, nurse clinician, or social worker can be very helpful in counseling patients and their families on issues relating to increasing disability and, ultimately, end-of-life care.
- A neuropsychological or experienced cognitive care provider (eg, behavioral neurologist, geriatric nurse) may be helpful in adapting the usual procedure for determining competence, communicating wishes and concerns, presenting hypothetical scenarios, and other tasks, since the cognitive problems of CBGD can specifically interfere with a person’s ability to speculate about the future and make complex contingent decisions.
- Many patients may wish to visit, if only once, a specialist in the area of behavioral neurology or movement disorders to confirm the diagnosis. Some patients find taking part in research studies, especially brain banking or genetic research, extremely gratifying, as they may derive meaning and a sense of purpose from contributing to the understanding and future treatment of others.
See the list below:
- Dysphagia may occur in some patients with prominent buccofacial apraxia.
- Speech therapy consultation for swallowing evaluation is recommended.
- Thickened liquids or soft foods (depending upon degree of impairment) may be necessary.
- Constipation is treated with conservative measures such increased fluid intake, high-fiber diets, encouragement of physical activity, stool softeners, and laxatives if necessary.
Activity is not restricted, but motor assistance is required as the disease progresses.
Boxer AL, Geschwind MD, Belfor N, et al. Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Arch Neurol. 2006 Jan. 63(1):81-6. [Medline].
Whitwell JL, Jack CR Jr., Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, et al. Imaging correlates of pathology in corticobasal syndrome. Neurology. 2010. 75:1879-1887.
Bergeron C, Pollanen MS, Weyer L. Unusual clinical presentations of cortical-basal ganglionic degeneration. Ann Neurol. 1996 Dec. 40(6):893-900. [Medline].
Winter Y, Bezdolnyy Y, Katunina E, et al. Incidence of Parkinson's disease and atypical parkinsonism: Russian population-based study. Mov Disord. 2010 Feb 15. 25(3):349-56. [Medline].
DePold Hohler A, Ransom BR, Chun MR, Tröster AI, Samii A. The youngest reported case of corticobasal degeneration. Parkinsonism Relat Disord. 2003 Oct. 10(1):47-50. [Medline].
Boeve BF. The multiple phenotypes of corticobasal syndrome and corticobasal degeneration: implications for further study. J Mol Neurosci. 2011 Nov. 45(3):350-3. [Medline].
Heilman KM, Rothi LJG. Apraxia. Heilman KM, Valenstein E, eds. Clinical Neuropsychology. 2nd ed. New York: Oxford University Press; 1985. 131-50.
Kertesz A, Morlog D, Light M, et al. Galantamine in frontotemporal dementia and primary progressive aphasia. Dement Geriatr Cogn Disord. 2008. 25(2):178-85. [Medline].
Kouri N, Whitwell JL, Josephs KA, Rademakers R, Dickson DW. Corticobasal degeneration: a pathologically distinct 4R tauopathy. Nat Rev Neurol. 2011 May. 7(5):263-72. [Medline].
Benito-León J, Alvarez-Linera J, Louis ED. Neurosyphilis masquerading as corticobasal degeneration. Mov Disord. 2004 Nov. 19(11):1367-70. [Medline].
McMonagle P, Blair M, Kertesz A. Corticobasal degeneration and progressive aphasia. Neurology. 2006 Oct 24. 67(8):1444-51. [Medline].
Borroni B, Garibotto V, Agosti C, et al. White matter changes in corticobasal degeneration syndrome and correlation with limb apraxia. Arch Neurol. 2008 Jun. 65(6):796-801. [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.
Fukui T, Sugita K, Kawamura M, Shiota J, Nakano I. Primary progressive apraxia in Pick's disease: a clinicopathologic study. Neurology. 1996 Aug. 47(2):467-73. [Medline].
Heilman KM. The apraxia of CBGD. Mov Disord. 1996. 11:348.
Josephs KA. Frontotemporal dementia and related disorders: deciphering the enigma. Ann Neurol. 2008 Jul. 64(1):4-14. [Medline].
Kertesz A, Blair M, McMonagle P, Munoz DG. The diagnosis and course of frontotemporal dementia. Alzheimer Dis Assoc Disord. 2007 Apr-Jun. 21(2):155-63. [Medline].
Kompoliti K, Goetz CG, Boeve BF, et al. Clinical presentation and pharmacological therapy in corticobasal degeneration. Arch Neurol. 1998 Jul. 55(7):957-61. [Medline].
Lang AE, Riley DE, Bergeron C. Cortical-basal ganglionic degeneration. Calne DB, ed. Neurodegenerative Diseases. Philadelphia: WB Saunders; 1994. 877-94.
Paulus W, Selim M. Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol. 1990. 81(1):89-94. [Medline].
Rebeiz JJ, Kolodny EH, Richardson EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol. 1968 Jan. 18(1):20-33. [Medline].
Reich SG, Grill SE. Corticobasal degeneration. Curr Treat Options Neurol. 2009 May. 11(3):179-85. [Medline].
Riley DE, Lang AE, Lewis A, et al. Cortical-basal ganglionic degeneration. Neurology. 1990 Aug. 40(8):1203-12. [Medline].
Sakurai Y, Hashida H, Uesugi H, et al. 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, et al. 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. 1997 Apr 24. 336(17):1216-22. [Medline].
Schellenberg GD, Höglinger G, Sleiman P, Rademakers R, Lambertus K, deSilva R, et al. A genome-wide association study of progressive supranuclear pals (PSP) and corticobasal degeneration (CBD). Alzh Dis Assoc Dis. 2010. 6(4, Suppl):S84-85.
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. 2005 Jan. 20(1):34-9. [Medline].
Takao M, Tsuchiya K, Mimura M, et al. Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case. Neuropathology. 2006 Dec. 26(6):569-78. [Medline].
Togasaki DM, Tanner CM. Epidemiologic aspects. Adv Neurol. 2000. 82:53-9. [Medline].
Watts RL, Williams RS, Growden JD. Corticobasal ganglionic degeneration. Neurology (Cleveland). 1985. 35 (Suppl 1):178.
Wenning GK, Litvan I, Jankovic J, et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry. 1998 Feb. 64(2):184-9. [Medline]. [Full Text].