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Diffuse Lewy Body Disease Presenting as Multiple System Atrophy

Published online by Cambridge University Press:  05 August 2019

Anthony S.-I. Pakiam ,
Catherine Bergeron  and
Anthony E. Lang
Anthony S.-I. Pakiam
Affiliation:
Division of Neurology, The Toronto Hospital, Toronto, Canada
Catherine Bergeron
Affiliation:
Department of Pathology (Neuropathology), The Toronto Hospital, Toronto, Canada
Anthony E. Lang*
Affiliation:
Division of Neurology, The Toronto Hospital, Toronto, Canada
*
Reprint requests to: Dr. Anthony Lang, Division of Neurology, The Toronto Hospital, 399 Bathurst Street, MP 11-306, Toronto, Ontario, Canada M5T 2S8
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Abstract:

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Objectives:

The majority of patients with diffuse Lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse Lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities.

Methods:

Case report based on a retrospective chart review and neuropathological examination.

Results:

We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous Lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse Lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord.

Conclusions:

In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse Lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.

Résumé:

Résumé:Buts:

La majorité des patients atteints de la maladie à corps de Lewy diffuse ont des manifestations cognitives ou psychiatriques qui font partie du tableau initial. Une forte minorité consulte pour des manifestations exclusivement parkinsoniennes. Une dysautonomie peut également faire partie du tableau, typiquement après l'apparition des changements cognitifs. Notre but est de démontrer que la maladie à corps de Lewy diffuse peut aussi donner un tableau de parkinsonisme accompagné d'une dysautonomie sévère en l'absence d'anomalies cognitives ou psychiatriques importantes.

Méthode:

Nous présentons une revue rétrospective du dossier et de l'examen neuropathologique d'un cas.

Résultats:

Nous rapportons le cas d'une patiente chez qui un diagnostic clinique d'atrophie multisystémique a été posé parce qu'elle présentait un parkinsonisme associé à une dysautonomie sévère et précoce. Le parkinsonisme était caractérisé par un tremblement postural, de la rigidité et de la bradykinésie, alors que les manifestations de la dysautonomie étaient des chutes répétées dues à l'orthostatisme et l'apparition éventuelle d'incontinence urinaire pendant l'évolution de sa maladie. Elle tolérait mal la thérapie dopaminergique à cause de l'exagération de l'orthostatisme. La lévodopa était peu efficace et le bénéfice devenait évident seulement parce qu'elle présentait une augmentation de la rigidité quand on tentait de la cesser. Il n'y avait pas d'histoire d'hallucinations spontanées ou induites par la médication, de délire ou de fluctuations cognitives et, contrairement à l'importance et à la progression de ses manifestations parkinsoniennes et dysautonomiques pendant les premières années d'évolution de sa maladie, la détérioration cognitive n'est apparue que dans la phase finale de sa maladie, sept ans après le début des symptômes. L'examen neuropathologique a montré de nombreux corps de Lewy dans le néocortex ainsi que dans les structures sous-corticales, ce qui est compatible avec un diagnostic de maladie à corps de Lewy diffuse. Il y avait une perte neuronale marquée dans la substance noire ainsi que dans les noyaux du système nerveux autonome du tronc cérébral et de la moelle épinière.

Conclusions:

Le tableau initial de la maladie à corps de Lewy diffuse peut être soit des manifestations cognitives, psychiatriques ou parkinsoniennes mais aussi un parkinsonisme accompagné d'une dysautonomie importante, ce qui remplit les critères qu'on a récemment proposés pour l'AMS-P probable.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 26 , Issue 2 , May 1999 , pp. 127 - 131
Copyright
Copyright © The Canadian Journal of Neurological 1999

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