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  • Updated 01.31.2021
  • Released 01.10.1995
  • Expires For CME 01.31.2024

Corticobasal degeneration

Introduction

Overview

Corticobasal degeneration is 1 of the atypical parkinsonian syndromes or Parkinsonism-plus syndromes that can mimic Parkinson disease, but it is distinct because of the added features of apraxia, dysphasia, cortical sensory signs, unusual dystonic postures, involuntary movements such as myoclonus, and “alien limb” sign. In this clinical article the author reviews the clinical features of the disease as well as the pathological findings. New potential genetic etiologies of the disease are presented. Although treatment remains symptomatic, accurate diagnosis can help prepare families and help clinicians better understand and treat this progressive, fatal disorder.

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.

• In addition to parkinsonism, patients with corticobasal degeneration may present with apraxia, dystonia, myoclonus, and alien-limb phenomenon.

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

Historical note and terminology

Several neurodegenerative disorders have prominent parkinsonian features within the context of more neurologic dysfunction than typically seen in Parkinson disease. At autopsy, patients with these disorders show more extensive and diffuse neuropathologic changes than the relatively isolated nigral degeneration of Parkinson disease. Because of the extensive clinical overlap among these various syndromes and Parkinson disease, the related syndromes have been historically termed "parkinsonism-plus" syndromes (161). Among these, clinicians and pathologists identify progressive supranuclear palsy, multiple system atrophy, olivopontocerebellar atrophy, striatonigral degeneration, and corticobasal degeneration, which was first described clearly in 1967 (167). "Cortical basal ganglionic degeneration," "cortico-dento-nigral degeneration," "cortico-dentato-nigral degeneration," "corticonigral degeneration," and “corticobasal degeneration” are synonymous terms (199). Although specifically described for the first time in modern times, earlier possible cases from the 19th and early 20th century have been cited (61). The celebrated composer Maurice Ravel may have suffered with this condition (04).

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