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 alpha-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 alpha-synuclein immunohistochemistry. This case extends the clinical and pathological spectrum of NFID to include cases with predominant parkinsonian and pyramidal features.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Josephs KA,Uchikado H,McComb RD,Bashir R,Wszolek Z,Swanson J,Matsumoto J,Shaw G,Dickson DWdoi
10.1007/s00401-004-0974-4subject
Has Abstractpub_date
2005-04-01 00:00:00pages
427-32issue
4eissn
0001-6322issn
1432-0533journal_volume
109pub_type
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journal_title:Acta neuropathologica
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