Alzheimer's Disease and Frontotemporal Dementias

See: NINDS Progressive supranuclear palsy Information Page and the 'We Move' site ('the Internet's most comprehensive resource for movement disorder information ')
and the 'Institut National de la Sante et de la Recherche Medical' website for more detail on PSP.

Type: PSP is a sporadic tauopathy belonging to the frontotemporal dementia (FTD) group of neurodegenerative diseases.

Regions affected: Basal ganglia , subthalamus and brainstem .

Tau pathology: Neuronal: Round or globose tangles of straight filaments. Glial: Tangles found in (tufted) astrocytes and (as coiled bodies) in oligodendrocytes. Neuropil threads.

Some Related References

Albers, DS and Augood, SJ (2001) New insights into progressive supranuclear palsy. TRENDS IN NEUROSCIENCES 24: 347-352

Albers, DS, Beal, MF (2002) Mitochondrial dysfunction in progressive supranuclear palsy. NEUROCHEMISTRY INTERNATIONAL 40: 559-564

Albers, DS, Swerdlow, RH, Manfredi, G, Gajewski, C, Yang, LC, Parker, WD, Beal, MF (2001) Further evidence for mitochondrial dysfunction in progressive supranuclear palsy. EXPERIMENTAL NEUROLOGY 168: 196-198

Arai, T, Ikeda, K, Akiyama, H, Tsuchiya, K, Yagishita, S, Takamatsu, J (2001) Intracellular processing of aggregated tan differs between corticobasal degeneration and progressive supranuclear palsy. NEUROREPORT 12: 935-938

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 NEUROPATHOLOGICA 101: 167-173

Arai, T, Ikeda, K, Akiyama, H, Tsuchiya, K, Iritani, S, Ishiguro, K, Yagishita, S, Oda, T, Odawara, T, Iseki, E (2003) Different immunoreactivities of the microtubule-binding region of tau and its molecular basis in brains from patients with Alzheimer's disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration. ACTA NEUROPATHOLOGICA 105: 489-498

Arima_K, Nakamura_M, Sunohara_N, Nishio_T, Ogawa_M, Hirai_S, Kawai_M, Ikeda_K. (1999) Immunohistochemical and ultrastructural characterization of neuritic clusters around ghost tangles in the hippocampal formation in progressive supranuclear palsy brains. ACTA NEUROPATHOLOGICA, 1999, Vol.97, No.6, pp.565-576

Berry, RW, Sweet, AP, Clark, FA, Lagalwar, S, Lapin, BR, Wang, T, Topgi, S, Guillozet-Bongaarts, AL, Cochran, EJ, Bigio, EH, Binder, LI (2004) Tau epitope display in progressive supranuclear palsy and corticobasal degeneration. JOURNAL OF NEUROCYTOLOGY 33: 287-295

Bigio, EH, Vono, MB, Satumtira, S, Adamson, J, Sontag, E, Hynan, LS, White, CL, Baker, M and Hutton, M (2001) Cortical synapse loss in progressive supranuclear palsy. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY 60: 403-410

Birdi, S, Rajput, AH, Fenton, M, Donat, JR, Rozdilsky, B, Robinson, C, Macaulay, R, George, D (2002) Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases . MOVEMENT DISORDERS 17: 1255-1264

Boeve, B, Dickson, D, Duffy, J, Bartleson, J, Trenerry, M, Petersen, R (2003) Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology. EUROPEAN NEUROLOGY 49: 72-78

Borghi, R, Giliberto, L, Assini, A, Delacourte, A, Perry, G, Smith, MA, Strocchi, P, Zaccheo, D, Tabaton, M (200?) Increase of cdk5 is related to neurofibrillary pathology in progressive supranuclear palsy. NEUROLOGY 58: 589-592

Borghi, R, Piccini, A, Delacourte, A, Strocchi, P, Zaccheo, D, Tabaton, M (2004) Protein levels of glycogen synthase 3 kinase are normal in progressive supranuclear palsy. NEUROSCIENCE LETTERS 366: 67-70

Burn, DJ, Lee, AJ (2002) Progressive supranuclear palsy: where are we now? LANCET NEUROLOGY 1: 359-369

Campdelacreu, J, Ezquerra, M, Munoz, E, Oliva, R, Tolosa, E (2003) Mutational study of the nuclear factor kappa B inducing kinase gene in patients with progressive supranuclear palsy. NEUROSCIENCE LETTERS 340: 158-160

Cantuti-Castelvetri, I, Keller-McGandy, CE, Albers, DS, Beal, MF, Vonsattel, JP, Standaert, DG, Augood, SJ (2002) Expression and activity of antioxidants in the brain in progressive supranuclear palsy. BRAIN RESEARCH 930: 170-181

Carrilho, PEM, Barbosa, ER (2002) Progressive supranuclear palsy in a sample of Brazilian population - Clinical features of 16 patients. ARQUIVOS DE NEURO-PSIQUIATRIA 60: 917-922

Chirichigno, JW, Manfredi, G, Beal, MF, Albers, DS (2002) Stress-induced mitochondrial depolarization and oxidative damage in PSP cybrids . BRAIN RESEARCH 951: 31-35

Colosimo, C, Osaki, Y, Vanacore, N, Lees, AJ (2003) Lack of association between progressive supranuclear palsy and arterial hypertension: A clinicopathological study. MOVEMENT DISORDERS 18: 694-697

Cordato, NJ, Pantelis, C, Halliday, GM, Velakoulis, D, Wood, SJ, Stuart, GW, Currie, J, Soo, M, Olivieri, G, Broe, GA, Morris, JGL (2002) Frontal atrophy correlates with behavioural changes in progressive supranuclear palsy. BRAIN 125: 789-800

de Silva, R, Hardy, J, Crook, J, Khan, N, Graham, EA, Morris, CM, Wood, NW, Lees, AJ (2002) The tau locus is not significantly associated with pathologically confirmed sporadic Parkinson's disease . NEUROSCIENCE LETTERS 330: 201-203

de Silva, R, Weiler, M, Morris, HR, Martin, ER, Wood, NW, Lees, AJ (2001) Strong association of a novel Tau promoter haplotype in progressive supranuclear palsy. NEUROSCIENCE LETTERS 311: 145-148

Ferrer, I, Barrachina, M, Puig, B (2002) Glycogen synthase kinase-3 is associated with neuronal and glial hyperphosphorylated tau deposits in Alzheimer's disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration . ACTA NEUROPATHOLOGICA 104: 583-591

Ferrer, I, Blanco, R, Carmona, M, Puig, B (2001) Phosphorylated c-MYC expression in Alzheimer disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY 27: 343-351

Ferrer, I, Blanco, R, Carmona, M, Ribera, R, Goutan, E, Puig, B, Rey, MJ, Cardozo, A, Vinals, F and Ribalta, T (2001) Phosphorylated map kinase (ERK1, ERK2) expression is associated with early tau deposition in neurones and glial cells, but not with increased nuclear DNA vulnerability and cell death, in Alzheimer disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration. BRAIN PATHOLOGY 11: 144-158

Goetz, CG (2003) Progressive supranucleare palsy. REVUE NEUROLOGIQUE 159: S25-S29

Goetz, CG, Leurgans, S, Lang, AE, Litvan, I (2003) Progression of gait, speech and swallowing deficits in progressive supranuclear palsy. NEUROLOGY 60: 917-922

Hartzler, AW, Zhu, XW, Siedlak, SL, Castellani, RJ, Avila, J, Perry, G, Smith, MA (2002) The p38 pathway is activated in Pick disease and progressive supranuclear palsy: a mechanistic link between mitogenic pathways, oxidative stress, and tau . NEUROBIOLOGY OF AGING 23: 855-859

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 NEUROPATHOLOGICA 106: 143-149

Higuchi, M, Ishihara, T, Zhang, B, Hong, M, Andreadis, A, Trojanowski, JQ, Lee, VMY (2002) Transgenic mouse model of tauopathies with glial pathology and nervous system degeneration. NEURON 35; 433-446

Holmberg, B, Johnels, B, Blennow, K, Rosengren, L (2003) Cerebrospinal fluid a beta 42 is reduced in multiple system atrophy but normal in Parkinson's disease and progressive supranuclear palsy. MOVEMENT DISORDERS 18: 186-190

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, George-Hyslop, PS, 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: 1702-1706

Ishizawa, K, Dickson, DW (2001) Microglial activation parallels system degeneration in progressive supranuclear palsy and corticobasal degeneration. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY 60: 647-657

Josephs, KA, Ishizawa, T, Tsuboi, Y, Cookson, N, Dickson, DW (2002) A clinicopathological study of vascular progressive supranuclear palsy - A multi-infarct disorder presenting as progressive supranuclear palsy . ARCHIVES OF NEUROLOGY 59: 1597-1601

Judkins, AR, Forman, MS, Uryu, K, Hinkle, DA, Asbury, AK, Lee, VMY, Trojanowski, JQ (2002) Co-occurrence of Parkinson's disease with progressive supranuclear palsy. ACTA NEUROPATHOLOGICA 103: 526-530

Kasashima, S, Oda, Y (2003) Cholinergic neuronal loss in the basal forebrain and mesopontine tegmentum of progressive supranuclear palsy and corticobasal degeneration. ACTA NEUROPATHOLOGICA 105: 117-124

Kato, N, Arai, K, Hattori, T (2003) Study of the rostral midbrain atrophy in progressive supranuclear palsy. JOURNAL OF THE NEUROLOGICAL SCIENCES 210: 57-60

Katsuse, O, Iseki, E, Arai, T, Akiyama, H, Togo, T, Uchikado, H, Kato, M, de Silva, R, Lees, A, Kosaka, K (2003) 4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy. ACTA NEUROPATHOLOGICA 106: 251-260

Kertesz, A (2003) Pick complex: An integrative approach to frontotemporal dementia - Primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy. NEUROLOGIST 9: 311-317

Kim, JM, Lee, KH, Choi, YL, Choe, GY, Jeon, BS (2002) Levodopa-induced dyskinesia in an autopsy-proven case of progressive supranuclear palsy . MOVEMENT DISORDERS 17: 1089-1090

Kobayashi, T, Mori, H, Okuma, Y, Dickson, DW, Cookson, N, Tsuboi, Y, Motoi, Y, Tanaka, R, Miyashita, N, Anno, M, Narabayashi, H, Mizuno, Y (2002) Contrasting genotypes of the tau gene in two phenotypically distinct patients with P301L mutation of frontotemporal dementia and parkinsonism linked to chromosome 17. JOURNAL OF NEUROLOGY 249: 669-675

Litvan, I, Baker, M, Hutton, M (2001) Tau genotype: No effect on onset, symptom severity, or survival in progressive supranuclear palsy. NEUROLOGY 57: 138-140

Liu, WK, Le, TV, Adamson, J, Baker, M, Cookson, N, Hardy, J, Hutton, M, Yen, SH, Dickson, DW (2001) Relationship of the extended tau haplotype to tau biochemistry and neuropathology in progressive supranuclear palsy. ANNALS OF NEUROLOGY 50: 494-502

Macia, F, Ballan, G, Yekhlef, F, Delmer, O, Vital, C, Lagueny, A, Tison, F (2003) Progressive supranuclear palsy: a clinical, natural history and disability study. REVUE NEUROLOGIQUE 159: 31-42

Mochizuki, A, Ueda, Y, Komatsuzaki, Y, Tsuchiya, K, Arai, T, Shoji, S (2003) Progressive supranuclear palsy presenting with primary progressive aphasia - Clinicopathological report of an autopsy case. ACTA NEUROPATHOLOGICA 105: 610-614

Mori, H, Oda, M, Komori, T, Arai, N, Takanashi, M, Mizutani, T, Hirai, S, Mizuno, Y (2002) Lewy bodies in progressive supranuclear palsy. ACTA NEUROPATHOLOGICA 104: 273-278

Morris, HR, Gibb, G, Katzenschlager, R, Wood, NW, Hanger, DP, Strand, C, Lashley, T, Daniel, SE, Lees, AJ, Anderton, BH, Revesz, T (2002) Pathological, clinical and genetic heterogeneity in progressive supranuclear palsy. BRAIN 125: 969-975

Morris, HR, Katzenschlager, R, Janssen, JC, Brown, JM, Ozansoy, M, Quinn, N, Revesz, T, Rossor, MN, Daniel, SE, Wood, NW, Lees, AJ (2002) Sequence analysis of tau in familial and sporadic progressive supranuclear palsy. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY 72: 388-390

Morris, HR, Osaki, Y, Holton, J, Lees, AJ, Wood, NW, Revesz, T, Quinn, N (2003) Tau exon 10 +16 mutation FTDP-17 presenting clinically as sporadic young onset PSP. NEUROLOGY 61: 102-104

Morris, HR, Schrag, A, Nath, U, Burn, D, Quinn, NP, Daniel, S, Wood, NW, Lees, AJ (2001) Effect of ApoE and tau on age of onset of progressive supranuclear palsy and multiple system atrophy. NEUROSCIENCE LETTERS 312: 118-120

Nakashima, H, Terada, S, Ishizu, H, Tanabe, Y, Yokota, O, Ishihara, T, Takata, H, Ihara, Y, Hayabara, T, Kuroda, S (2003) An autopsied case of dementia with Lewy bodies with supranuclear gaze palsy. NEUROLOGICAL RESEARCH 25: 533-537

Nath, U, Ben-Shlomo, Y, Thomson, RG, Lees, AJ, Burn, DJ (2003) Clinical features and natural history of progressive supranuclear palsy - A clinical cohort study. NEUROLOGY 60: 910-916

Oide, T, Ohara, S, Yazawa, M, Inoue, K, Itoh, N, Tokuda, T, Ikeda, S (2002) Progressive supranuclear palsy with asymmetric tau pathology presenting with unilateral limb dystonia. ACTA NEUROPATHOLOGICA 104: 209-214

Oyanagi, K, Tsuchiya, K, Yamazaki, M, Ikeda, K (2001) Substantia nigra in progressive supranuclear palsy, corticobasal degeneration, and Parkinsonism-dementia complex of Guam: Specific pathological features. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY 60: 393-402

Park, LCH, Albers, DS, Xu, H, Lindsay, JG, Beal, MF, Gibson, GE (2001) Mitochondrial impairment in the cerebellum of the patients withprogressive supranuclear palsy. JOURNAL OF NEUROSCIENCE RESEARCH 66: 1028-1034

Pastor, P, Pastor, E, Carnero, C, Vela, R, Garcia, T, Amer, G, Tolosa, E, Oliva, R (2001) Familial atypical progressive supranuclear palsy associated with homozigosity for the delN296 mutation in the tau gene. ANNALS OF NEUROLOGY 49: 263-267

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Pharr, V, Uttl, B, Stark, M, Litvan, I, Fantie, B, Grafman, J (2001) Comparison of apraxia in corticobasal degeneration and progressive supranuclear palsy. NEUROLOGY 56: 957-963

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Poorkaj, P, Muma, NA, Zhukareva, V, Cochran, EJ, Shannon, KM, Hurtig, H, Koller, WC, Bird, TD, Trojanowski, JQ, Lee, VMY, Schellenberg, GD (2002) An (R)5(L) tau mutation in a subject with a progressive supranuclear palsy phenotype . ANNALS OF NEUROLOGY 52: 511-516

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Rub, U, Del Tredici, K, Schultz, C, de Vost, RAI, Steur, ENHJ, Arai, K, Braak, H (2002) Progressive supranuclear palsy: neuronal and glial cytoskeletal pathology in the higher order processing autonomic nuclei of the lower brainstem. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY 28: 12-22

Shimamura, M, Uyama, E, Hirano, T, Murakami, T, Mita, S, Kitamoto, T, Uchino, M (2003) A unique case of sporadic Creutzfeldt-Jacob disease presenting as progressive supranuclear palsy. INTERNAL MEDICINE 42: 195-198

Sobrido, MJ, Abu-Khalil, A, Weintraub, S, Johnson, N, Quinn, B, Cummings, JL, Mesulam, MM, Geschwind, DH (2003) Possible association of the tau H1/H1 genotype with primary progressive aphasia. NEUROLOGY 60: 862-864

Steele, JC, Caparros-Lefebvre, D, Lees, AJ, Sacks, OW (2002) Progressive supranuclear palsy and its relation to pacific foci of the parkinsonism-dementia complex and Guadeloupean parkinsonism . PARKINSONISM & RELATED DISORDERS 9: 39-54

Takahashi, M, Weidenheim, KM, Dickson, DW, Ksiezak-Reding, H (2002) Morphological and biochemical correlations of abnormal tau filaments in progressive supranuclear palsy. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY 61: 33-45

Takanashi, M, Mori, H, Arima, K, Mizuno, Y, Hattori, N (2002) Expression patterns of tau mRNA isoforms correlate with susceptible lesions in progressive supranuclear palsy and corticobasal degeneration . MOLECULAR BRAIN RESEARCH 104: 210-219

Tawana, K, Ramsden, DB (2001) Progressive supranuclear palsy. JOURNAL OF CLINICAL PATHOLOGY-MOLECULAR PATHOLOGY 54: 427-434

Togo, T, Dickson, DW (2002) Ballooned neurons in progressive supranuclear palsy are usually due to concurrent argyrophilic grain disease. ACTA NEUROPATHOLOGICA 104: 53-56

Togo, T, Dickson, DW (2002) Tau accumulation in astrocytes in progressive supranuclear palsy is a degenerative rather than a reactive process . ACTA NEUROPATHOLOGICA 104: 398-402

Togo, T, Dickson, DW (2002) Tau accumulation in astrocytes in progressive supranuclear palsy is a degenerative rather than a reactive process . ACTA NEUROPATHOLOGICA

Tsuboi, Y, Ahlskog, JE, Apaydin, H, Parisi, JE, Dickson, DW (2001) Lewy bodies are not increased in progressive supranuclear palsy compared with normal controls. NEUROLOGY 57: 1675-1678

Tsuboi, Y, Josephs, KA, Cookson, N, Dickson, DW (2003) APOE E4 is a determinant for Alzheimer type pathology in progressive supranuclear palsy. NEUROLOGY 60: 240-245

Tsuboi, Y, Slowinski, J, Josephs, A, Honer, WG, Wszolek, ZK, Dickson, DW (2003) Atrophy of superior cerebellar peduncle in progressive supranuclear palsy. NEUROLOGY 60: 1766-1769

Weeks, RA, Scaravilli, F, Lees, AJ, Carroll, C, Husain, M, Rudge, P (2003) Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome. MOVEMENT DISORDERS 18: 331-336

Wszolek, ZK, Tsuboi, Y, Uitti, RJ, Reed, L, Hutton, ML, Dickson, DW (2001) Progressive supranuclear palsy as a disease phenotype caused by the S305S tau gene mutation. BRAIN 124: 1666-1668

Zemaitaitis, MO, Kim, SY, Halverson, RA, Troncoso, JC, Lee, JM, Muma, NA (2003) Transglutaminase activity, protein, and mRNA expression are increased in progressive supranuclear palsy. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY 62: 173-184

Zhukareva, V, Sundarraj, S, Mann, D, Sjogren, M, Blenow, K, Clark, CM, McKeel, DW, Goate, A, Lippa, CF, Vonsattel, JP, Growdon, JH, Trojanowski, JQ, Lee, VMY (2003) Selective reduction of soluble Tau proteins in sporadic and familial frontotemporal dementias: an international follow-up study. ACTA NEUROPATHOLOGICA 105: 469-476