2017NeurodegenerationRequires access

Corticobasal Degeneration

Keith A. Josephs

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Abstract

The clinical features associated with corticobasal degeneration (CBD) include: cogwheel and Gegenhalten rigidity, action and postural tremor, bradykinesia, myoclonus, limb apraxia, alien limb phenomena, limb and neck dystonia, gait and posture difficulties, dysarthria, aphasia, apraxia of speech, and dementia. The corticobasal syndrome is characterized by the presence of asymmetric parkinsonism and cortical dysfunction. While the three most common syndromes associated with CBD are the corticobasal syndrome, the progressive supranuclear palsy syndrome, and progressive apraxia of speech with or without aphasia, there are other less common syndromes associated with CBD. Voxel-based morphometry (VBM) has been applied to subjects with CBD compared to controls and to patients with other neurodegenerative diseases. The majority of research performed to date has focused on the corticobasal syndrome. However, given current knowledge that the corticobasal syndrome is not specific to CBD, future biomarker development must focus on tau and CBD instead.

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What this paper is about

The clinical features associated with corticobasal degeneration (CBD) include: cogwheel and Gegenhalten rigidity, action and postural tremor, bradykinesia, myoclonus, limb apraxia, alien limb phenomena, limb and neck dystonia, gait and posture difficulties, dysarthria, aphasia, apraxia of speech, and dementia. The corticobasal syndrome is characterized by the presence of asymmetric parkinsonism and cortical dysfunction. While the three most common syndromes associated with CBD are the corticobasal syndrome, the progressive supranuclear palsy syndrome, and progressive apraxia of speech with or without aphasia, there are other less common syndromes associated with CBD. Voxel-based morphometry (VBM) has been applied to subjects with CBD compared to controls and to patients with other neurodegenerative diseases. The majority of research performed to date has focused on the corticobasal syndrome. However, given current knowledge that the corticobasal syndrome is not specific to CBD, future biomarker development must focus on tau and CBD instead.

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Available abstract

The clinical features associated with corticobasal degeneration (CBD) include: cogwheel and Gegenhalten rigidity, action and postural tremor, bradykinesia, myoclonus, limb apraxia, alien limb phenomena, limb and neck dystonia, gait and posture difficulties, dysarthria, aphasia, apraxia of speech, and dementia. The corticobasal syndrome is characterized by the presence of asymmetric parkinsonism and cortical dysfunction. While the three most common syndromes associated with CBD are the corticobasal syndrome, the progressive supranuclear palsy syndrome, and progressive apraxia of speech with or without aphasia, there are other less common syndromes associated with CBD. Voxel-based morphometry (VBM) has been applied to subjects with CBD compared to controls and to patients with other neurodegenerative diseases. The majority of research performed to date has focused on the corticobasal syndrome. However, given current knowledge that the corticobasal syndrome is not specific to CBD, future biomarker development must focus on tau and CBD instead.

Key concepts: Corticobasal degeneration, Apraxia, Progressive supranuclear palsy, Frontotemporal dementia, Dysarthria, Psychology, Parkinsonism, Aphasia

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