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  • Updated 03.06.2024
  • Released 01.10.1995
  • Expires For CME 03.06.2027

Corticobasal degeneration



Corticobasal degeneration is one of the atypical parkinsonian syndromes or parkinsonism-plus syndromes most often characterized by parkinsonism (typically unilateral); apraxia; cortical sensory signs; dystonia; involuntary movements, such as myoclonus; and “alien limb” sign. In this article, the author reviews the clinical features of the disease as well as the pathological findings. Although there is no cure and treatment remains symptomatic, accurate diagnosis can help with patient and family counseling and guide the treating physicians.

Key points

• Corticobasal degeneration, which is considered as an atypical parkinsonian syndrome or parkinsonism-plus syndrome, is a tauopathy.

• The pathological hallmark is the deposition of abnormally hyper-phosphorylated microtubule associated protein tau in various parts of the brain. The location of abnormal tau deposition accounts for its heterogeneous presentation.

• The most common phenotype or presentation of corticobasal degeneration is corticobasal syndrome, which is characterized by parkinsonism with accompanying apraxia, dystonia, myoclonus, and alien-limb phenomenon.

• Corticobasal syndrome may be seen in other clinically pathologically overlapping tauopathies, such as progressive supranuclear palsy. Features at presentation may aid in distinguishing between the tauopathies.

• Currently, no specific treatment is available for corticobasal degeneration.

Historical note and terminology

Corticobasal degeneration was first described by Rebeiz and colleagues via a case series of three patients with progressive asymmetrical parkinsonism and myoclonus (79). In this original description, the term “corticodentatonigral degeneration” was used to describe the pathological changes observed. The disorder was not further described until 1989 by Gibb and colleagues, when the term “corticobasal degeneration” was introduced (28). In the subsequent years, the terms “corticobasal degeneration” and “corticobasal syndrome” have largely been used interchangeably, lending to confusion. To clarify, corticobasal degeneration is the term used to denote the atypical parkinsonian syndrome or parkinsonism-plus syndrome with corresponding pathology demonstrating a specific 4-repeat tauopathy, and corticobasal syndrome is the most common phenotype.

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