Corticobasal Syndrome and Corticobasal Degeneration Treatment & Management

Updated: Dec 04, 2019
  • Author: Alexander Pantelyat, MD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Approach Considerations

The approach is centered on maximizing the patient's quality of life through rehabilitative interventions (physical, occupational, and speech/swallow therapy) and judicious use of symptomatic therapies.

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Medical Care

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  • Discontinue anticholinergics or other medications that impair attention and memory.

  • Discontinue any medications that may cause or worsen parkinsonism (dopamine blocking medications: all antipsychotics as well as metoclopramide and prochlorperazine).

  • Consider cholinesterase inhibitors such as rivastigmine or donepezil if memory loss is present.

  • Consider empiric treatment of depression

  • Initiate a trial of carbidopa/levodopa (Sinemet IR) if rigidity and movement disorder are disabling. Institute a plan for titration of this medication to an appropriate level (at least 900–1000mg levodopa per day for at least 1 month) before declaring the patient to be levodopa-unresponsive.

  • Consider botulinum toxin injections if the patient has bothersome or painful limb dystonia (with EMG +/- ultrasound guidance for localization), blepharospasm/eyelid opening apraxia, and/or sialorrhea.

  • Consider treating bothersome myoclonus with levetiracetam and/or clonazepam.

  • Refer early and continuously to occupational therapy (for upper extremity functional evaluation and management), physical therapy (gait and safety evaluation and training), and speech-language pathology (speech evaluation/cognitive training, swallow evaluation using modified barium with video), consider assistive devices for ambulation, ADLs and comunication, and an exercise program to maintain endurance and strength. 

  • Refer to Social Work to discuss long term care planning and state-specific as well as federal resources for patients, most of whom are Medicare-eligible

  • CurePSP has developed a free Allied Health (PT/OT/SLP/Social Work) guide to signs, symptoms, and management strategies for CBD, PSP, and MSA:  https://www.psp.org/wp-content/uploads/2016/08/ALLIED-HEALTH-BROCHURE_web.pdf

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Consultations

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  • Physical and occupational therapists: Physical and occupational therapy can be helpful to maintain endurance in patients with impaired gait and to teach patients with visual agnosia different strategies for performing activities of daily living.

  • Speech-language pathologist: 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.

  • Nutrition: dietary modifications, protein supplementation, PEG management (along with Gastroenterology) if elected by patient

  • A social worker and/or geriatric case manager 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 CBD can specifically interfere with a person’s ability to speculate about the future and make complex contingent decisions.

  • Early referral to Movement Disorders and/or Behavioral Neurology for confirmation of the diagnosis and advanced management. A growing number of tertiary referral centers have established advanced multidisciplinary (with PT/OT/SLP/Social Work, for example) care for patients with CBS and other parkinsonian disorders (see CurePSP Centers of Care: https://www.psp.org/ineedsupport/centers-of-care/). Clinical trials for neurodegenerative tauopathies are underway. Additionally, some patients find taking part in research studies, especially brain banking or genetics research, extremely gratifying, as they may derive meaning and a sense of purpose from contributing to the understanding and future treatment of others.

  • Referral to Psychiatry in cases of psychiatric comorbidity, frontal-behavioral presentation, or notable reactive depression.

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Diet

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  • Dysphagia may occur in many patients as a late symptom.

  • Speech therapy consultation for video barium swallow evaluation is recommended.

  • Thickened liquids or soft foods (depending upon degree of impairment) may be necessary under the guidance of a speech-language pathologist.

  • Constipation is common and is treated with conservative measures such increased fluid intake, high-fiber diets, encouragement of physical activity, stool softeners, and laxatives as necessary.

  • Referral to a Nutritionist: Liquid protein supplementation may be necessary to mitigate weight loss; Appetite stimulants can be considered

  • Discussion of patient wishes regarding gastrostomy tube placement (most elect not to pursue this) should be carried out and documented as early as possible

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Activity

Activity is not restricted and is in fact encouraged using a positive risk-taking approach, but increasing motor assistance is required as the disease progresses. Many corticobasal syndrome (CBS) patients lose ambulation in advanced disease stages and may require wheelchair, electric stair glide, and/or patient lift devices.

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Surgical Care

Deep brain stimulation surgery is not recommended for corticobasal syndrome (CBS) or other phenotypes associated with corticobasal degeneration (CBD) pathology. There is a lack of studies evaluating this modality for patients with CBS.

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Complications

Key complications in patients suffering from corticobasal degeneration (CBD) include:

  • Aspiration pneumonia in the setting of progressve dysphagia

  • Weight loss in the setting of progressive dysphagia (and potentially depression)

  • Sepsis due to complicated urinary tract infections in the setting of progressive neurogenic bladder dysfunction, or from wound infection in the setting of progressive loss of mobility

  • Loss of the ability to communicate due to progressive nonfluent aphasia coupled with motor and voice impairments limiting the choice of augmented/alternative communication devices

  • Loss of reading ability due to oculomotor difficulties and cortical visual impairment

  • Loss of the ability to socialize due to behavioral/personality changes

  • Injury due to falls in the setting of progressive imbalance (gait/limb apraxia may contribute to this)

  • Deconditioning due to reduced mobility and limited ability to exercise

  • Reactive depression with potential suicidality

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Prevention

No effective preventive strategies exist for corticobasal degeneration (CBD). Since CBD is commonly associated with dementia, general strategies aiming to develop cognitive reserve and mitigate effects of progressive neurodegeneration are advisable: pursuing education beyond a 4-year college degree, learning a musical instrument/singing, learning a new language, regular aerobic exercise, adhering to a Mediterranean diet, and maintaining social engagement. 

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Long-Term Monitoring

Regular follow-up with a movement and/or cognitive neurologist is indicated to monitor for symptom changes and emergent complications. For patients receiving botulinum toxin injections, follow up at 2–3 month intervals is necessary to parallel the timing of the toxin wearing off. Patients require long-term physical, occupational, and speech/swallow therapy, even if only on a weekly basis, in order to maintain residual function and quality of life.Annual (or more frequent if there is decompensation) video barium swallow assessments should be considered. 

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