Corticobasal syndrome: Five new things
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Date
2014
Journal Title
Journal ISSN
Volume Title
Publisher
Lippincott Williams and Wilkins
Abstract
Corticobasal syndrome (CBS) is characterized by asymmetric involuntary movements including rigidity, tremor, dystonia, and myoclonus, and often associated with apraxia, cortical sensory deficits, and alien limb phenomena. Additionally, there are various nonmotor (cognitive and language) deficits. CBS is associated with several distinct histopathologies, including corticobasal degeneration, other forms of tau-related frontotemporal lobar degeneration such as progressive supranuclear palsy, and Alzheimer disease. Accurate antemortem diagnosis of underlying pathology in CBS is challenging, though certain clinical and imaging findings may be helpful. Five recent advances in the understanding of CBS are reviewed, including clinical and pathologic features, imaging and CSF biomarkers, the role of specific genes, and the concept of a spectrum of tauopathies. © 2014 American Academy of Neurology.
Description
Keywords
Leucine rich repeat kinase 2, Progranulin, Tau protein, Alien limb phenomena, Alzheimer disease, Apraxia, Apraxia of speech, Article, Athetosis, Babinski reflex, Blepharospasm, Brain disease, Carotid artery obstruction, Central pontine myelinolysis, Cerebellar ataxia, Cerebrospinal fluid, Cerebrotendinous xanthomatosis, Clinical feature, Corticobasal degeneration, Corticobasal syndrome, Creutzfeldt jakob disease, Diffuse lewy body disease, Disorders of higher cerebral function, Dysarthria, Dyskinesia, Fahr disease, Frontotemporal dementia, Gene mutation, Genetics, Human, Hyperreflexia, Limb apraxia, Motor neuron disease, Myoclonus, Neurosyphilis, Nuclear magnetic resonance imaging, Orolingual dyskinesia, Parkinsonism, Periodic limb movement disorder, Pick presenile dementia, Positron emission tomography, Priority journal, Progressive multifocal leukoencephalopathy, Progressive supranuclear palsy, Spinocerebellar ataxia, Tremor