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Studying Pre-formed Fibril Induced α-Synuclein Accumulation in Primary Embryonic Mouse Midbrain Dopamine Neurons
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Progressive supranuclear palsy phenotype mimicking synucleinopathies.

Kateřina Menšíková1, Radoslav Matěj, Lucie Tučková

  • 1Department of Neurology, Palacky University Medical School, Olomouc, Czech Republic. katerina.mensikova@fnol.cz

Journal of the Neurological Sciences
|April 18, 2013
PubMed
Summary

Dual pathology, including tauopathy and Alzheimer's disease, can mimic atypical parkinsonian syndromes like multiple system atrophy and dementia with Lewy bodies. These unique cases highlight the complex presentations of neurodegenerative disorders.

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Clinical Neurology

Background:

  • Atypical parkinsonian syndromes are pathologically classified into synucleinopathies and tauopathies.
  • Emerging evidence suggests shared pathological features between these groups.
  • Understanding these overlaps is crucial for accurate diagnosis.

Observation:

  • Two pathologically confirmed cases of tauopathy presented with phenotypes typically associated with synucleinopathies: multiple system atrophy and dementia with Lewy bodies.
  • A 67-year-old woman exhibited a multiple system atrophy phenotype, and a 70-year-old man presented with a dementia with Lewy bodies phenotype.
  • Clinical diagnoses were established using standard criteria, followed by detailed post-mortem neuropathological examination.

Findings:

  • Both cases revealed a rare combination of dual neurodegenerative pathologies.
  • Specifically, a 4R tauopathy (consistent with progressive supranuclear palsy) and the neocortical stage of Alzheimer's disease were identified.
  • The neuropathological findings demonstrated diffuse tauopathy alongside Alzheimer's pathology.

Implications:

  • These unique cases demonstrate that tauopathies, such as corticobasal syndrome and progressive supranuclear palsy, can mimic a wide spectrum of atypical parkinsonian syndromes.
  • The presence of concomitant neurodegenerative diseases, like Alzheimer's pathology, can significantly alter the clinical presentation.
  • This underscores the importance of considering dual pathologies when diagnosing atypical parkinsonian syndromes.