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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.

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

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