Extending the clinicopathological spectrum of neurofilament inclusion disease |
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Authors: | Keith A. Josephs Hirotake Uchikado Rodney D. McComb Rifaat Bashir Zbigniew Wszolek Jerry Swanson Joseph Matsumoto Gerry Shaw Dennis W. Dickson |
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Affiliation: | (1) Department of Neurology, Mayo Clinic Rochester, Rochester, Minnesota, USA;(2) Department of Neurology, Mayo Clinic Jacksonville, Jacksonville, Florida, USA;(3) Department of Pathology (Neuropathology), Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, USA;(4) Department of Pathology, University of Nebraska Medical Center, Omaha, Nebraska, USA;(5) Department of Neurology, University of Nebraska Medical Center, Omaha, Nebraska, USA;(6) Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, Florida, USA |
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Abstract: | We describe features of a patient that broadens the clinical and pathological spectrum of neurofilament inclusion disease (NFID). The patient was a 52-year-old man with a 5–6 year history of progressive, asymmetrical spastic weakness of the upper and lower extremities; l-DOPA-unresponsive parkinsonism; and SPECT evidence of asymmetrical frontoparietal and basal ganglia hypoperfusion. The brain had marked frontoparietal parasagittal cortical atrophy, including the motor cortex, with histopathological evidence of neurofilament- and -internexin-immunoreactive neuronal inclusions. The corticospinal tract had degeneration, but there was minimal lower motor neuron pathology. There was also severe neuronal loss and gliosis in the posterolateral putamen and the substantia nigra, mimicking multiple system atrophy; however, glial cytoplasmic inclusions were not detected with -synuclein immunohistochemistry. This case extends the clinical and pathological spectrum of NFID to include cases with predominant parkinsonian and pyramidal features.Gerry Shaw holds equity in EnCor Biotechnology Inc., a company commercializing the alpha-internexin antibody used in this study, and may benefit by receiving royalties or equity growth. |
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Keywords: | Neurofilament inclusion disease Multiple system atrophy Striatonigral degeneration /content/j4017060610jp71l/xxlarge945.gif" alt=" agr" align=" BASELINE" BORDER=" 0" >-Internexin Motor neuron disease |
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