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Related Experiment Video

Updated: Feb 3, 2026

Use of the Protease Fluorescent Detection Kit to Determine Protease Activity
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Variably protease-sensitive prionopathy presenting within ALS/FTD spectrum.

Mikel Vicente-Pascual1,2,3, Marcello Rossi4, Josep Gámez5

  • 1Department of Neurology Hospital Clínic de Barcelona Barcelona Spain.

Annals of Clinical and Translational Neurology
|October 24, 2018
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Summary
This summary is machine-generated.

This study details a rare prion disease presenting as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Variably protease-sensitive prionopathy can mimic ALS/FTD with prolonged symptoms.

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Prion Diseases

Background:

  • Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are neurodegenerative diseases.
  • TDP-43 pathology is commonly associated with ALS and FTD.
  • Prion diseases are typically characterized by distinct neuropathological findings.

Purpose of the Study:

  • To describe the clinico-pathological features of two patients with a rare prion disease.
  • To investigate the association of this prion disease with the ALS/FTD spectrum.
  • To differentiate this condition from typical ALS/FTD.

Main Methods:

  • Clinical case reporting and neuropathological examination.
  • Analysis of brain tissue for spongiform change and prion protein deposits.
  • Western blot analysis for prion protein profiling.
  • Genetic analysis for prion protein gene mutations.

Main Results:

  • Two patients presented with clinical and neuropathological features overlapping ALS and FTD.
  • Case 2 showed frontotemporal atrophy without TDP-43 pathology.
  • Spongiform changes and pathological prion protein deposits were identified.
  • Western blot revealed a profile consistent with variably protease-sensitive prionopathy.
  • No prion protein gene mutations were detected.

Conclusions:

  • Variably protease-sensitive prionopathy can present with prolonged disease duration.
  • This prion disease can exhibit clinico-pathological features within the ALS/FTD spectrum.
  • It highlights the importance of considering prion diseases in atypical ALS/FTD presentations.