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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Variably protease-sensitive prionopathy (VPSPr) is a rare prion disease.
  • VPSPr can present with diverse clinical symptoms.
  • Frontotemporal dementia (FTD) and motor neuron diseases are distinct neurological conditions.

Purpose of the Study:

  • To describe two cases of VPSPr presenting with features of primary lateral sclerosis and FTD.
  • To detail the neuropathological findings in these VPSPr cases.
  • To characterize the prion protein (PrPSc) profile in VPSPr.

Main Methods:

  • Clinical case description and neurological examination.
  • Neuropathological analysis including spongiform changes, PrPSc deposits, and neuronal aggregates.
  • Western blot analysis to determine the proteolytic profile of PrPSc.

Main Results:

  • Both patients exhibited progressive upper motor neuron symptoms, insomnia, and cognitive decline.
  • Neuropathology showed spongiform encephalopathy with frontotemporal and thalamic involvement, fine synaptic PrPSc deposits, and microplaques.
  • Western blot revealed a VPSPr-specific proteolytic profile lacking the diglycosylated band.

Conclusions:

  • VPSPr can manifest with prominent motor neuron syndrome and FTD-like features.
  • These cases expand the clinical spectrum of VPSPr.
  • VPSPr should be considered in the differential diagnosis of FTD and motor neuron diseases.