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Diffuse Lewy body disease presenting as multiple system atrophy.

A S Pakiam1, C Bergeron, A E Lang

  • 1Division of Neurology, Toronto Hospital, Toronto, Canada.

The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
|June 3, 1999
PubMed
Summary
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Diffuse Lewy body disease can rarely present with parkinsonism and severe autonomic dysfunction without initial cognitive or psychiatric symptoms. This case highlights an atypical presentation mimicking multiple system atrophy.

Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Diffuse Lewy body disease (DLBD) typically presents with cognitive or psychiatric symptoms, or parkinsonism alone.
  • Autonomic dysfunction in DLBD usually follows cognitive decline.

Observation:

  • A patient presented with parkinsonism (tremor, rigidity, bradykinesia) and significant early autonomic dysfunction (orthostasis, urinary incontinence).
  • Cognitive impairment was absent for seven years, developing only in the late stages of the illness.
  • The patient showed poor tolerance to dopaminergic therapy due to orthostasis.

Findings:

  • Neuropathological examination confirmed diffuse Lewy body disease with Lewy bodies in cortical and subcortical structures.
  • Neuronal loss was observed in the substantia nigra and brainstem/spinal cord autonomic nuclei.

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  • The presentation fulfilled criteria for the striatonigral form of multiple system atrophy (MSA).

    • Implications:

    • DLBD can present atypically with parkinsonism and marked autonomic dysfunction, mimicking MSA.
    • This underscores the importance of considering DLBD in the differential diagnosis of parkinsonian syndromes with prominent autonomic failure.
    • Recognizing this rare presentation is crucial for accurate diagnosis and management.