Neuropathology of variants of progressive supranuclear palsy

被引:250
作者
Dickson, Dennis W. [1 ]
Ahmed, Zeshan [2 ]
Algom, Avi A. [3 ]
Tsuboi, Yoshio [4 ]
Josephs, Keith A. [5 ]
机构
[1] Mayo Clin, Dept Neurosci, Jacksonville, FL 32224 USA
[2] UCL, Dept Mol Neurosci, Inst Neurol, London WC1E 6BT, England
[3] Assaf Harofeh Med Ctr, Dept Neurol, IL-70300 Zerifin, Israel
[4] Fukuoka Univ, Dept Neurol, Fukuoka 81401, Japan
[5] Mayo Clin, Dept Neurol, Rochester, MN USA
关键词
4R tau; clinical variants; neuropathology; progressive supranuclear palsy; tauopathy; RICHARDSON-OLSZEWSKI-SYNDROME; AMYOTROPHIC-LATERAL-SCLEROSIS; CORTICOBASAL DEGENERATION; ALZHEIMERS-DISEASE; SYNAPSE LOSS; TAU-GENE; PATHOLOGICAL HETEROGENEITY; COGNITIVE IMPAIRMENT; NONFLUENT APHASIA; PURE AKINESIA;
D O I
10.1097/WCO.0b013e32833be924
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Purpose of review Neurodegenerative tauopathies, of which progressive supranuclear palsy (PSP) is one of the most common, are clinically heterogeneous, reflecting differences in distribution and biochemical composition of tau pathology. This review highlights the range of clinical and pathologic presentations of PSP and its variants. Recent findings Progressive supranuclear palsy is a 4R tauopathy with neuronal and glial tau-immunoreactive lesions in neuroanatomically specific nuclei in the basal ganglia, diencephalon, brainstem and cerebellum, with restricted involvement of the neocortex. Hierarchical cluster analyses of clinical and pathologic features of PSP indicate that there are distinct clinicopathologic variants of PSP. In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP. In variants of PSP presenting with levodopa-responsive Parkinsonism, as well as pure akinesia and gait failure, there is less cortical pathology and more severe degeneration in the cardinal nuclei - globus pallidus, subthalamic nucleus and substantia nigra - than in typical PSP. Summary Clinical variants in PSP reflect varying anatomical distribution of tau pathology, but they share histopathologic, biochemical and genetic features with typical PSP. The basis for anatomical selective vulnerability in PSP and its variants remains to be determined.
引用
收藏
页码:394 / 400
页数:7
相关论文
共 66 条
[1]   Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy [J].
Ahmed, Zeshan ;
Josephs, Keith A. ;
Gonzalez, John ;
DelleDonne, Anthony ;
Dickson, Dennis W. .
BRAIN, 2008, 131 :460-472
[2]   SUBCORTICAL DEMENTIA OF PROGRESSIVE SUPRANUCLEAR PALSY [J].
ALBERT, ML ;
FELDMAN, RG ;
WILLIS, AL .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1974, 37 (02) :121-130
[3]   Ultrastructural characterization of the tau-immunoreactive tubules in the oligodendroglial perikarya and their inner loop processes in progressive supranuclear palsy [J].
Arima, K ;
Nakamura, M ;
Sunohara, N ;
Ogawa, M ;
Anno, M ;
Izumiyama, Y ;
Hirai, S ;
Ikeda, K .
ACTA NEUROPATHOLOGICA, 1997, 93 (06) :558-566
[4]   Association of an extended haplotype in the tau gene with progressive supranuclear palsy [J].
Baker, M ;
Litvan, I ;
Houlden, H ;
Adamson, J ;
Dickson, D ;
Perez-Tur, J ;
Hardy, J ;
Lynch, T ;
Bigio, E ;
Hutton, M .
HUMAN MOLECULAR GENETICS, 1999, 8 (04) :711-715
[5]   Neurofibrillary tangles mediate the association of amyloid load with clinical Alzheimer disease and level of cognitive function [J].
Bennett, DA ;
Schneider, JA ;
Wilson, RS ;
Bienias, JL ;
Arnold, SE .
ARCHIVES OF NEUROLOGY, 2004, 61 (03) :378-384
[6]  
Bergeron C, 1997, J NEUROPATH EXP NEUR, V56, P726
[7]   Cortical synapse loss in progressive supranuclear palsy [J].
Bigio, EH ;
Vono, MB ;
Satumtira, S ;
Adamson, J ;
Sontag, E ;
Hynan, LS ;
White, CL ;
Baker, M ;
Hutton, M .
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 2001, 60 (05) :403-410
[8]   Progressive supranuclear palsy with dementia: Cortical pathology [J].
Bigio, EH ;
Brown, DF ;
White, CL .
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 1999, 58 (04) :359-364
[9]   Pathologic heterogeneity in clinically diagnosed corticobasal degeneration [J].
Boeve, BF ;
Maraganore, DM ;
Parisi, JE ;
Ahlskog, JE ;
Graff-Radford, N ;
Caselli, RJ ;
Dickson, DW ;
Kokmen, E ;
Petersen, RC .
NEUROLOGY, 1999, 53 (04) :795-800
[10]   Alzheimer's disease presenting as corticobasal syndrome [J].
Chand, Pratap ;
Grafman, Jordan ;
Dickson, Dennis ;
Ishizawa, Keisuke ;
Litvan, Irene .
MOVEMENT DISORDERS, 2006, 21 (11) :2018-2022