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Corticobasal Degeneration

An asymmetric neurodegenerative tauopathy producing the corticobasal syndrome of limb apraxia, alien limb phenomenon, dystonia, and asymmetric parkinsonism.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Corticobasal Degeneration?

Corticobasal degeneration (CBD) is a 4R tauopathy with asymmetric atrophy of the frontoparietal cortex, basal ganglia, and brainstem and is one of the pathologies that cause corticobasal syndrome (CBS).

The classic phenotype is asymmetric limb apraxia, alien limb, cortical sensory loss, dystonia, myoclonus, and parkinsonism that does not respond to levodopa, often accompanied by progressive cognitive impairment with a frontal-executive pattern.

Diagnosis is clinical, supported by brain MRI showing asymmetric frontoparietal atrophy and FDG-PET showing asymmetric hypometabolism; tau PET imaging is being developed for in vivo support.

Symptoms

Asymmetric limb rigidity and bradykinesia
Limb apraxia and alien limb phenomenon
Cortical sensory loss with astereognosis and agraphesthesia
Limb dystonia and stimulus-sensitive myoclonus
Frontal-executive cognitive impairment, behavioral changes, and progressive aphasia
Falls and gait disturbance in advanced disease

Risk Factors

Age over 60
MAPT H1 haplotype
Rare familial cases with MAPT mutations
Possible environmental factors (poorly defined)
Overlap with other tauopathies (PSP, FTD)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Asymmetric limb stiffness and clumsiness with poor levodopa response
  • Alien limb phenomenon or marked apraxia
  • Progressive aphasia or behavior changes with motor symptoms
  • Recurrent falls or gait disturbance in older adults

Treatment Methods

01
Levodopa trial — usually with poor response, but worth attempting
02
Symptomatic management of dystonia (botulinum toxin), myoclonus (clonazepam, levetiracetam), and depression
03
Speech and language therapy, occupational therapy, and physical therapy
04
Multidisciplinary care with cognitive support, caregiver education, and falls-prevention measures
05
Advance care planning, palliative care, and consideration of clinical trials in tauopathies

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.