Corticobasal degeneration

Abhishek Lenka MD PhD (

Dr. Lenka of MedStar Georgetown University Hospital has no relevant financial relationships to disclose.

)
Joseph Jankovic MD, editor. (

Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research and training funding from Allergan, F Hoffmann-La Roche, Medtronic Neuromodulation, Merz, Neurocrine  Biosciences, Nuvelution, Revance, and Teva and consulting/advisory board honorariums from Abide, Lundbeck, Retrophin, Parexel, Teva, and Allergan.

)
Originally released January 10, 1995; last updated October 25, 2019; expires October 25, 2022

This article includes discussion of corticobasal degeneration, CBD, CBGD, cortical-basal ganglionic degeneration, corticodentatonigral degeneration, cortico-dento-nigral degeneration, corticonigral degeneration, cortico-dentato-nigral degeneration with neuronal achromasia, and corticobasal ganglionic degeneration. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Corticobasal degeneration is one 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 (Piboolnurak and Waters 2003). 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 (Rebeiz et al 1967). "Cortical basal ganglionic degeneration," "cortico-dento-nigral degeneration," "cortico-dentato-nigral degeneration," "corticonigral degeneration," and “corticobasal degeneration” are synonymous terms (Stover and Watts 2001). Although specifically described for the first time in modern times, earlier possible cases from the 19th and early 20th century have been cited (Goetz et al 2001). The celebrated composer Maurice Ravel may have suffered with this condition (Amaducci et al 2002).

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