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Corticobasal syndrome with novel argyrophilic glial inclusions

Authors

  • Gregory A. Rippon MD,

    Corresponding author
    1. Department of Neurology, Columbia University College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Gertrude H. Sergievsky Center, Columbia University, New York, New York, USA
    3. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, New York, USA
    • Gertrude H. Sergievsky Center, Columbia University College of Physicians and Surgeons, 622 W. 168th Street, New York, NY 10032
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  • S.M. Staugaitis MD, PhD,

    1. Department of Neurosciences, Cleveland Clinic Foundation, Cleveland, Ohio, USA
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  • Steven S.M. Chin MD, PhD,

    1. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, New York, USA
    2. Department of Pathology, Columbia University College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • James E. Goldman MD, PhD,

    1. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, New York, USA
    2. Department of Pathology, Columbia University College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • K. Marder MD, MPH

    1. Department of Neurology, Columbia University College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Gertrude H. Sergievsky Center, Columbia University, New York, New York, USA
    3. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, New York, USA
    4. Department of Psychiatry, Columbia University College of Physicians and Surgeons, Columbia University, New York, New York, USA
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Abstract

A 42-year-old, left-handed woman first noted impaired dexterity of the dominant hand, soon followed by dysarthria and cognitive decline. Over a 4-year period, she developed severe left-sided apraxia with eventual neglect of the left arm and progressive extrapyramidal signs. Cognitive testing showed progressive executive, visuospatial, fluency, and naming impairment with relative preservation of memory. Single-photon emission computed tomography demonstrated asymmetric right posterior frontal and superior parietal hypoperfusion. The clinical impression was corticobasal degeneration. At autopsy, severe atrophy was seen in the perirolandic and frontal regions. There was marked neuronal loss and gliosis in the posterior frontal and precentral regions and less severe pathology in prefrontal, temporal, and parietal areas. Mild to moderate gliosis and neuronal loss were also seen in the putamen, globus pallidus, subthalamic, and dentate nuclei. Gallyas silver stain revealed numerous inclusions adjacent to oligodendrocyte nuclei in white and gray matter of affected cortical and subcortical regions. The gracile inclusions were wavy, slender, and stained positively with antibodies to ubiquitin and αB-crystallin but not to microtubule-associated proteins (tau, MAP1B, MAP2), tubulin, neurofilaments, glial fibrillary acidic protein, or α-synuclein. The argyrophilic inclusions identified in this case are distinct from those previously described in neurodegenerative diseases. © 2005 Movement Disorder Society

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