Abstract
We examined spinal cord lesions in eight patients with a pathological diagnosis of corticobasal degeneration (CBD). Using Gallyas-Braak (G-B) staining or AT-8 tau immunostaining, a few neuropil threads were identified in the white matter of the CBD spinal cords, mainly in the anterior funiculus, whereas the posterior funiculus was well preserved without threads. In the gray matter of the CBD spinal cords, particularly in the intermediate gray matter, there were widespread neuropil threads and neuronal inclusions. Large motor neurons in the anterior horn, neurons in the intermediolateral column, and Clarke’s column were relatively well preserved from neuronal loss and gliosis. Neuronal inclusions were of the globose type, suggestive of neurofibrillary tangles (NFTs), or showed diffuse granular accumulations of cytoplasmic tau, suggestive of pretangles. No typical NFTs, recognized by Bodian silver staining, were identified. The distribution of neuropil threads and G-B- or AT-8 tau-positive small neurons resembled that of interneurons. No astrocytic plaques were present in any of the CBD spinal cords, and only a few coiled bodies were seen. Neuropil threads in the white and gray matter and neuronal inclusions in the gray matter were prominent in cervical segments, and their density decreased caudally. We suggest that the presence of neuropil threads, particularly in the cervical intermediate gray matter, and the presence of neuronal inclusions, particularly in cervical interneurons, is an essential pathological feature of CBD.
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Braak H, Braak E, Ohm T, Bohl J (1988) Silver impregnation of Alzheimer’s neurofibrillary changes counterstained for basophilic material and lipofuscin pigment. Stain Technol 63:197–200
Dickson DW, Bergeron C, Chin SS, Duyckaerts C, Horoupian D, Ikeda K, Jellinger K, Lantos PL, Lippa CF, Mirra SS, Tabaton M, Vonsattel JP, Wakabayashi K, Litvan I (2002) Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61:935–946
Fahn S (1995) Parkinsonism. In: Rowland LP (ed) Merritt’s textbook of neurology, 9th edn. Williams & Wilkins, Baltimore, pp 713–730
Gibb WR, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192
Hattori M, Hashizume Y, Yoshida M, Iwasaki Y, Hishikawa N, Ueda R, Ojika K (2003) Distribution of astrocytic plaques in the corticobasal degeneration brain and comparison with tuft-shaped astrocytes in the progressive supranuclear palsy brain. Acta Neuropathol 106:143–149
Iwasaki Y, Yoshida M, Hattori M, Goto A, Aiba I, Hashizume Y, Sobue G (2004) Distribution of tuft-shaped astrocytes in the cerebral cortex in progressive supranuclear palsy. Acta Neuropathol 108:399–405
Iwatsubo T, Hasegawa M, Ihara Y (1994) Neuronal and glial tau-positive inclusions in diverse neurologic diseases share common phosphorylation characteristic. Acta Neuropathol 88:129–136
Kikuchi H, Doh-ura K, Kira J, Iwaki T (1999) Preferential neurodegeneration in the cervical spinal cord of progressive supranuclear palsy. Acta Neuropathol 97:577–584
Kuypers HG (1982) A new look at the organization of the motor system. Prog Brain Res 57:381–403
Litvan I, Agid Y, Goetz C, Jankovic J, Wenning GK, Brandel JP, Lai EC,Verny M, Ray-Chaudhuri K, Mckee A, Jellinger K, Pearce RK, Bartko JJ (1997) Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 48:119–125
Mori H, Nishimura M, Namba Y, Oda M (1994) Corticobasal degeneration: a disease with widespread appearance of abnormal tau and neurofibrillary tangles, and its relation to progressive supranuclear palsy. Acta Neuropathol 88:113–121
Paulus W, Seli M (1990) Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol 81:89–94
Rebeiz JJ, Kolodny EH, Richardson EP Jr (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18:20–33
Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Hornykiewicz O, Black S (1990) Cortical-basal ganglionic degeneration. Neurology 40:1203–1212
Tsuchiya K, Ikeda K, Uchihara T, Oda T, Shimada H (1997) Distribution of cerebral cortical lesions in corticobasal degeneration: a clinicopathological study of five autopsy cases in Japan. Acta Neuropathol 94:416–424
Uchihara T, Nakamura A, Yamazaki M, Mori O (2001) Evolution from pretangle neurons to neurofibrillary tangles monitored by thiazin red combined with Gallyas method and double immunofluorescence. Acta Neuropathol 101:535–539
Uchihara T, Nakamura A, Yamazaki M, Mori O, Ikeda K, Tsuchiya K (2001) Different conformation of neuronal tau deposits distinguished by double immunofluorescence with AT8 and thiazine red combined with Gallyas method. Acta Neuropathol 102:462–466
Wakabayashi K, Oyanagi K, Makifuchi T, Ikuta F, Homma A, Homma Y, Horikawa Y, Tokiguchi S (1994) Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations. Acta Neuropathol 87:545–553
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Iwasaki, Y., Yoshida, M., Hattori, M. et al. Widespread spinal cord involvement in corticobasal degeneration. Acta Neuropathol 109, 632–638 (2005). https://doi.org/10.1007/s00401-005-1017-5
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DOI: https://doi.org/10.1007/s00401-005-1017-5