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Progressive Supranuclear Palsy: A Review of Co-existing Neurodegeneration

Published online by Cambridge University Press:  02 December 2014

J Keith-Rokosh  and
L C Ang
J Keith-Rokosh
Affiliation:
Department of Pathology, London Health Sciences Centre and University of Western Ontario, London, Ontario, Canada
L C Ang*
Affiliation:
Department of Pathology, London Health Sciences Centre and University of Western Ontario, London, Ontario, Canada
*
Department of Pathology, London Health Sciences Centre, University Hospital, 339 Windermere Rd., P.O. Box 5339, London, Ontario, N6A 5A5, Canada.
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Abstract

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Objectives:

The neuropathological findings of 32 progressive supranuclear palsy (PSP) cases over a period of 17 years were reviewed.

Results:

Of the 26 cases with adequate clinical data, 20 patients either presented with cognitive dysfunction or developed a cognitive impairment subsequently during the course of the disease. Co-existing changes of argyrophilic grains and corticobasal degeneration (CBD) were found in 28% and 32% of the cases respectively. Alzheimer-related pathology was found in 69% of cases but only 18.75% of cases fulfilled the consortium to establish a registry for Alzheimer's disease (CERAD) criteria for either definite or probable Alzheimer's disease. Lewy bodies were noted in four cases (12.5%), all in the subcortical regions. Only seven cases of PSP showed no pathological evidence of other co-existing neurodegenerative diseases. The severity of the cerebrovascular pathology in this cohort was insufficient to explain any clinical symptomatology.

Conclusions:

As in previous studies, this study has demonstrated the frequent co-existence of pathological changes usually noted in other neurodegenerative diseases in PSP. Whether these coexisting pathological changes contribute to the cognitive impairment in PSP remains uncertain.

Résumé:

RÉSUMÉ: Objectifs:

Les observations neuropathologiques provenant de 32 patients atteints de paralysie supranucléaire progressive (PSP) au cours d'une période de 17 ans ont été révisées.

Résultats:

Vingt patients parmi les 26 pour qui les données cliniques étaient adéquates ont consulté pour une dysfonction cognitive ou ont présenté une atteinte cognitive au cours de l'évolution de la maladie. Des changements au niveau des granules argentophiles et une dégénérescence corticobasale ont été observés chez 28% et 32% des patients respectivement. Une pathologie de type Alzheimer a été observée chez 69% des patients bien que seulement 18,75% des patients rencontraient les critères du CERAD pour une maladie d'Alzheimer certaine ou probable. Des corps de Lewy ont été observés chez quatre patients (12,5%), exclusivement dans les régions sous corticales. Seulement sept cas de PSP n'avaient aucune manifestation anatomopathologique d'une autre maladie neurodégénérative coexistante. La sévérité de la pathologie cérébrovasculaire dans cette cohorte était insuffisante pour expliquer la symptomatologie clinique.

Conclusions:

Cette étude, comme les études antérieures, démontre la coexistence fréquente dans la PSP de changements anatomopathologiques présents dans d'autres maladies neurodégénératives. La contribution de ces changements anatomopathologiques au déficit cognitif dans la PSP demeure obscure.

Information

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 35 , Issue 5 , November 2008 , pp. 602 - 608
Copyright
Copyright © The Canadian Journal of Neurological 2008

References

1. Golbe, LI. The epidemiology of PSP. J Neural Transm Suppl. 1994; 42:263–73.Google Scholar
2. Schrag, A, Ben-Shlomo, Y, Quinn, NP. Prevalence of PSP and Multisystem Atrophy: a cross-sectional study. Lancet. 1999; 354 (9192):1771–5.Google Scholar
3. Steele, JC, Richardson, JC, Olszewski, J. Progressive supranuclear palsy. Arch Neurol. 1964; 10:333–59.Google Scholar
4. Hauw, J, Agid, Y. Progressive Supranuclear Palsy (PSP) or Steele-Richardson-Olszewski Disease. In: Dickson, DW, editor. Neurodegeneration: the molecular pathology of dementia and movement disorders. Basel: ISN Neuropath Press; 2003. p. 103–14.Google Scholar
5. Litvan, I, Grafman, J, Gomez, C, Chase, TN. Memory impairment in patients with progressive supranuclear palsy. Arch Neurol. 1989; 46(7):765–7.Google Scholar
6. Josephs, KA, Dickson, D. Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. Mov Disord. 2003; 18:1018–26.Google Scholar
7. Braak, H, Jellinger, K, Braak, E, Bohl, J. Allocortical neurofibrillary changes in progressive supranuclear palsy. Acta Neuropathol. 1992; 84:478–83.Google Scholar
8. Bergeron, C, Pollanen, MS, Weyer, L, Lang, AE. Cortical degeneration in progressive supranuclear palsy. A comparison with cortical-basal ganglionic degeneration. J Neuropathol Exp Neurol. 1997; 56(6):726–34.Google Scholar
9. Hauw, JJ, Daniel, SE, Dickson, D, Horupian, DS, Jellinger, K, Lantos, PL, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1994; 44:2015–19.Google Scholar
10. Braak, H, Braak, E. Neuropathological staging of Alzheimer-related changes. Acta Neuropathol. 1991; 82:239–59.Google Scholar
11. Braak, H, Alafuzoff, I, Arzberger, T, Kretzschmar, H, Del Tredici, K. Staging of Alzheimer’s disease-associated neurofibrillary tangle pathology using paraffin sections and immunocytochemistry. Acta Neuropathol. 2006; 112:389–404.Google Scholar
12. Gearing, M, Mirra, SS, Hedreen, JC, Sumi, SM, Hansen, LA, Heyman, A. The consortium to establish a registry for Alzheimer’s disease (CERAD). Part X. Neuropathology confirmation of the clinical diagnosis of Alzheimer’s disease. Neurology. 1995;45:461–6.Google Scholar
13. Gearing, M, Olson, DA, Watts, R, Mirra, SC. Progressive supranuclear palsy: neuropathological and clinical heterogeneity. Neurology. 1994; 44(6):1015–24.Google Scholar
14. Newell, KL, Hyman, BT, Growdon, JH, Hedley-White, ET. Application of the National Institute on Aging (NIA)-Regean Institute Criteria for Neuropathological Diagnosis of Alzheimer’s disease. J Neuropathol Exp Neurol. 1999; 58: 1147–55.Google Scholar
15. Tsuboi, Y, Josephs, KA, Boeve, BF, Litvan, I, Caselli, RJ, Caviness, JN, et al. Increased tau burden in the corticies of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord. 2005; 20(8):982–8.Google Scholar
16. Uchikado, H, DelleDonne, A, Ahmed, Z, Dickson, DW. Lewy bodies in progressive supranuclear palsy represent an independent disease process. J Neuropathol Exp Neurol. 2006; 65(4):387–95.Google Scholar
17. Braak, H, Del Tredici, K, Bratzke, H, Hamm-Clement, J, Sandmannkeil, D, Rüb, U. Staging of the intracerebral inclusion body pathology associated with idiopathic Parkinson’s disease (preclinical and clinical stages). J Neurol. 2002; 249 Suppl 3: 1–5.Google Scholar
18. Wakabayashi, K, Takahashi, H. Pathological heterogeneity in progressive supranuclear palsy and corticobasal degeneration. Neuropathology. 2004; 24:79–86.Google Scholar
19. Mackenzie, IR, Hudson, LP. Achromatic neurons in the cortex of progressive supranuclear palsy. Acta Neuropathol. 1995; 90: 615–19.Google Scholar
20. Mori, H, Oda, M. Ballooned neurons in corticobasilar degeneration and progressive supranuclear palsy. Neuropathology. 1997; 17:248–52.Google Scholar
21. Togo, T, Dickson, DW. Ballooned neurons in progressive supranuclear palsy are usually due to concurrent argyrophilic grain disease. Acta Neuropathol. 2002; 104:53–6.Google Scholar
22. Martinez-Lage, P, Munoz, DG. Prevalence and disease associations of argyrophilic grains of Braak. J Neuropathol Exp Neurol. 1997; 56(2):157–64.Google Scholar