Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Acta Neuropathologica
Published in: Acta Neuropathologica 3/2003

01-09-2003 | Regular Paper

4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy

Authors: Omi Katsuse, Eizo Iseki, Tetsuaki Arai, Haruhiko Akiyama, Takashi Togo, Hirotake Uchikado, Masanori Kato, Rohan de Silva, Andrew Lees, Kenji Kosaka

Published in: Acta Neuropathologica | Issue 3/2003

Login to get access

Abstract

We report a 67-year-old man with 4-repeat (4R) tauopathy sharing both features of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Although CBD and PSP have a common pathological feature that 4R tau accumulates in neurons and glia, recent pathological studies have confirmed differences between the two disorders. Clinical features of the present case were asymmetrical apraxia, parkinsonism, memory disturbance, disorientation and left limb myoclonus with a 5-year history. Pathological features were the widespread occurrence of 4R tau-positive structures including pre-tangles, neurofibrillary tangles, astrocytic plaques, tufted astrocytes, coiled bodies and argyrophilic threads. Biochemically, immunoblotting of insoluble tau demonstrated the low molecular fragments of 37 kDa and 33 kDa observed in typical CBD and PSP, respectively, in addition to the presence of 4R tau isoforms. The present case shared tau-related pathological and biochemical features of CBD and PSP. These findings support that CBD and PSP are closely associated disorders having a pathogenesis common to 4R tauopathy.
Literature
1.
Arai T, Ikeda K, Akiyama H, Tsuchiya K, Yagishita S, Takamatsu J (2001) Intracellular processing of aggregated tau differs between corticobasal degeneration and progressive supranuclear palsy. NeuroReport 12:935–938PubMed
2.
Arai T, Ikeda K, Akiyama H, Shikamoto Y, Tsuchiya K, Yagishita S, Beach T, Rogers J, Schwab C, McGeer PL (2001) Distinct isoforms of tau aggregated in neurons and glial cells in brains of patients with Pick's disease, corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 101:167–173PubMed
3.
Arima K, Nakamura M, Sunohara N, Ogawa M, Anno M, Izumiyama Y, Hirai S, Ikeda K (1997) Ultrastructural characterization of the tau-immunoreactive tubules in the oligodendroglial perikarya and their inner loop processes in progressive supranuclear palsy. Acta Neuropathol 93:558–566CrossRefPubMed
4.
Bancher C, Brunner C, Lassmann H, Budka H, Jellinger K, Wiche G, Seitelberger F, Grundke-Iqbal I, Iqbal K, Wisniewski HM (1989) Accumulation of abnormally phosphorylated tau precedes the formation of neurofibrillary tangles in Alzheimer's disease. Brain Res 477:90–99PubMed
5.
Buee L, Delacourte A (1999) Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease. Brain Pathol 9:681–693PubMed
6.
Collins SJ, Ahlskog JE, Parisi JE, Maraganore DM (1995) Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria. J Neurol Neurosurg Psychiatry 58:167–173PubMed
7.
De Silva R, Lashley T, Gibb G, Hanger D, Hope A, Reid A, Bandopadhyay R, Utton M, Strand C, Jowett T, Khan N, Anderton B, Wood N, Holton J, Revesz T, Lees A (2003) Pathological inclusion bodies in tauopathies contain distinct complements of tau with three or four microtubule-binding repeat domains as demonstrated by new specific monoclonal antibodies. Neuropathol Appl Neurobiol (in press)
8.
Delacourte A, Sergeant N, Wattez A, Gauvreau D, Robitaille Y (1998) Vulnerable neuronal subsets in Alzheimer's and Pick's disease are distinguished by their tau isoform distribution and phosphorylation. Ann Neurol 43:193–204PubMed
9.
Di Maria E, Tabaton M, Vigo T, Abbruzzese G, Bellone E, Donati C, Frasson E, Marchese R, Montagna P, Munoz DG, Pramstaller PP, Zanusso G, Ajmar F, Mandich P (2000) Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Ann Neurol 47:374–377PubMed
10.
Dickson DW (1999) Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol 246:6–15CrossRefPubMed
11.
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 61:935–946PubMed
12.
Goedert M, Spillantini MG, Cairns NJ, Crowther RA (1992) Tau proteins of Alzheimer paired helical filaments: abnormal phosphorylation of all six brain isoforms. Neuron 8:159–168PubMed
13.
Hauw JJ, Verny M, Delaere P, Cervera P, He Y, Duyckaerts C (1990) Constant neurofibrillary changes in the neocortex in progressive supranuclear palsy. Basic differences with Alzheimer's disease and aging. Neurosci Lett 119:182–186PubMed
14.
Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, McKee A, Tabaton M, Litvan I (1994) Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 44:2015–2019PubMed
15.
Houlden H, Baker M, Morris HR, MacDonald N, Pickering-Brown S, Adamson J, Lees AJ, Rossor MN, Quinn NP, Kertesz A, Khan MN, Hardy J, Lantos PL, St George-Hyslop P, Munoz DG, Mann D, Lang AE, Bergeron C, Bigio EH, Litvan I, Bhatia KP, Dickson D, Wood NW, Hutton M (2001) Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 56:1702–1706PubMed
16.
Ikeda K, Akiyama H, Haga C, Kondo H, Arima K, Oda T (1994) Argyrophilic thread-like structure in corticobasal degeneration and supranuclear palsy. Neurosci Lett 174:157–159PubMed
17.
Ikeda K, Akiyama H, Arai T, Nishimura T (1998) Glial tau pathology in neurodegenerative diseases: their nature and comparison with neuronal tangles. Neurobiol Aging 19:S85–91CrossRefPubMed
18.
Iseki E, Li F, Kosaka K, Nishimura T, Akiyama H, Ikeda K, Kato M (1997) Tau-immunohistochemical and -immunoelectron microscopical investigation of astrocytic plaques in progressive supranuclear palsy. Biomed Res 18:145–148
19.
Iseki E, Togo T, Suzuki K, Katsuse O, Marui W, Silva R de, Lees A, Yamamoto T, Kosaka K (2003) Dementia with Lewy bodies from the perspective of tauopathy. Acta Neuropathol 105:265–270PubMed
20.
Komori T, Arai N, Oda M, Nakayama H, Mori H, Yagishita S, Takahashi T, Amano N, Murayama S, Murakami S, Shibata N, Kobayashi M, Sasaki S, Iwata M (1998) Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 96:401–408CrossRefPubMed
21.
Lang AE, Riley DE, Bergeron C (1994) Cortical-basal ganglionic degeneration. In: Calne DB (ed) Neurodegenerative disease. Saunders, Philadelphia, pp 877–894
22.
Li F, Iseki E, Kosaka K, Ikeda K (1998) Immunohistochemical and immunoelectron microscopical investigation of tau-positive neurons lacking tangle-formation in progressive supranuclear palsy. Biomed Res 19:145–149
23.
Li F, Iseki E, Odawara T, Kosaka K, Yagishita S, Amano N (1998) Regional quantitative analysis of tau-positive neurons in progressive supranuclear palsy: comparison with Alzheimer's disease. J Neurol Sci 159:73–81CrossRefPubMed
24.
Litvan I, Agid Y, Jankovic J, Goetz C, Brandel JP, Lai EC, Wenning G, D'Olhaberriague L, Verny M, Chaudhuri KR, McKee A, Jellinger K, Bartko JJ, Mangone CA, Pearce RK (1996) Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology 46:922–930PubMed
25.
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 48:119–125PubMed
26.
Litvan I, Grimes DA, Lang AE, Jankovic J, McKee A, Verny M, Jellinger K, Chaudhuri KR, Pearce RK (1999) Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration. J Neurol 246:1–5CrossRef
27.
Mizusawa H, Yen S-H, Hirano A, Llena JF (1989) Pathology of the dentate nucleus in progressive supranuclear palsy: a histological, immunohistochemical and ultrastructural study. Acta Neuropathol 78:419–428PubMed
28.
Nishimura M, Namba Y, Ikeda K, Oda M (1992) Glial fibrillary tangles with straight tubules in the brains of patients with progressive supranuclear palsy. Neurosci Lett 143:35–38CrossRefPubMed
29.
Nishimura M, Tomimoto H, Suenaga T, Namba Y, Ikeda K, Akiguchi I, Kimura J (1995) Immunocytochemical characterization of glial fibrillary tangles in Alzheimer's disease brain. Am J Pathol 146:1052–1058PubMed
30.
Otvos L Jr, Feiner L, Lang E, Szendrei GI, Goedert M, Lee VM (1994) Monoclonal antibody PHF-1 recognizes tau protein phosphorylated at serine residues 396 and 404. J Neurosci Res 39:669–673PubMed
31.
Rebeiz JJ, Kolodny EH, Richardson EP Jr (1967) Corticodentatonigral degeneration with neuronal achromasia: a progressive disorder of late adult life. Trans Am Neurol Assoc 92:23–26PubMed
32.
Sergeant N, David JP, Goedert M, Jakes R, Vermersch P, Buee L, Lefranc D, Wattez A, Delacourte A (1997) Two-dimensional characterization of paired helical filament-tau from Alzheimer's disease: demonstration of an additional 74-kDa component and age-related biochemical modifications. J Neurochem 69:834–844PubMed
33.
Sergeant N, Wattez A, Delacourte A (1999) Neurofibrillary degeneration in progressive supranuclear palsy and corticobasal degeneration: tau pathologies with exclusively "exon 10" isoforms. J Neurochem 72:1243–1249CrossRefPubMed
34.
Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. Arch Neurol10:333–359
35.
Tellez-Nagel I, Wisniewski HM (1973) Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch Neurol 29:324–327PubMed
36.
Togo T, Dickson DW (2002) Ballooned neurons in progressive supranuclear palsy are usually due to concurrent argyrophilic grain disease. Acta Neuropathol 104:53–56CrossRefPubMed
37.
Togo T, Sahara N, Yen SH, Cookson N, Ishizawa T, Hutton M, Silva R de, Lees A, Dickson DW (2002) Argyrophilic grain disease is a sporadic 4-repeat tauopathy. J Neuropathol Exp Neurol 61:547–556PubMed
38.
Verny M, Duyckaerts C, Agid Y, Hauw JJ (1996) The significance of cortical pathology in progressive supranuclear palsy. Clinico-pathological data in 10 cases. Brain 119:1123–1136PubMed
39.
Yamada T, McGeer PL, McGeer EG (1992) Appearance of paired nucleated, Tau-positive glia in patients with progressive supranuclear palsy brain tissue. Neurosci Lett 135:99–102PubMed
Metadata
Title
4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy
Authors
Omi Katsuse
Eizo Iseki
Tetsuaki Arai
Haruhiko Akiyama
Takashi Togo
Hirotake Uchikado
Masanori Kato
Rohan de Silva
Andrew Lees
Kenji Kosaka
Publication date
01-09-2003
Publisher
Springer-Verlag
Published in
Acta Neuropathologica / Issue 3/2003
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI
https://doi.org/10.1007/s00401-003-0728-8

Other articles of this Issue 3/2003

Acta Neuropathologica 3/2003 Go to the issue

Regular Paper

α-Synuclein pathology in Parkinson’s and Alzheimer’s disease brain: incidence and topographic distribution—a pilot study

Regular Paper

Characterization of newly established tumor lines from a spontaneous malignant schwannoma in F344 rats: nerve growth factor production, growth inhibition by transforming growth factor-β1, and macrophage-like phenotype expression

Regular Paper

Encephalopathy in megacystis-microcolon-intestinal hypoperistalsis syndrome patients on long-term total parenteral nutrition possibly due to selenium deficiency

Announcement

Announcements September 2003

Regular Paper

The effect of combined neurolytic blocking agent 5% phenol-glycerol in rat sciatic nerve

Regular Paper

Decreased estrogen receptor-α expression in hippocampal neurons in relation to hyperphosphorylated tau in Alzheimer patients