title: Widespread spinal cord involvement in progressive supranuclear palsy.
abstract: We describe the histopathologic features of spinal cord lesions in 10 cases of progressive supranuclear palsy (PSP) and review the literature. Histologic examination revealed atrophy with myelin pallor in the anterior funiculus and anterolateral funiculus in the cervical and thoracic segments in eight of the 10 cases, whereas the posterior funiculus was well preserved. The degrees of atrophy of the anterior funiculus and the anterolateral funiculus correlated with that of the tegmentum of the medulla oblongata. Myelin pallor of the lateral corticospinal tract was observed in two of the 10 cases. Microscopic observation of the spinal white matter, particularly the cervical segment, revealed a few to several neuropil threads, particularly in the white matter surrounding the anterior horn after Gallyas-Braak (GB) staining or AT-8 tau immunostaining. However, the posterior funiculus was completely preserved from the presence of argyrophilic or tau-positive structures. In the spinal gray matter, widespread distribution of neurons with cytoplasmic inclusions and neuropil threads was observed, particularly in the medial division of the anterior horn and intermediate gray matter, especially in the cervical segment. Globose-type neurofibrillary tangles and pretangles were found. The distribution of GB- or AT-8 tau-positive small neurons and neuropil threads resembled that of the spinal interneurons. In conclusion, the spinal cord, especially the cervical segment, is constantly involved in the pathologic process of PSP. We speculate that spinal interneurons and their neuronal processes, particularly in the medial division of the anterior horn and intermediate gray matter of the cervical segment, are most severely damaged in the PSP spinal cord.
authors: Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. iwasaki@sc4.so-net.ne.jp
authors: Yasushi
authors: Y
authors: Iwasaki
authors: Mari
authors: M
authors: Yoshida
authors: Yoshio
authors: Y
authors: Hashizume
authors: Manabu
authors: M
authors: Hattori
authors: Ikuko
authors: I
authors: Aiba
authors: Gen
authors: G
authors: Sobue
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002574:Cervical Vertebrae; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016874:Neurofibrillary Tangles / Q000473:pathology; D019600:Neuropil Threads / Q000473:pathology; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology*; D013494:Supranuclear Palsy, Progressive / Q000378:metabolism / Q000473:pathology*; D016875:tau Proteins / Q000378:metabolism
chemical_list: D016875:tau Proteins