Neurology: Serum β-synuclein concentration is significantly elevated in patients with Creutzfeldt-Jakob disease (CJD), which is expected to be a blood biomarker for early diagnosis and monitoring of synaptic integrity in CJD

Prion disease is a rapidly progressive and devastating neurodegenerative disease, including sporadic, hereditary and acquired forms
.

Identifying individuals at risk for inherited forms through predictive genetic testing can provide a unique opportunity for presymptomatic pharmacology to delay or prevent disease interventions before disease onset

Prion disease is a rapidly progressive and devastating neurodegenerative disease, including sporadic, hereditary and acquired forms

Synaptic degeneration is highly presynaptic terminal, β-synuclein enriched in several brain structures (eg thalamus, cerebellum, neocortex, hippocampus and striatum) as one of the reliable synaptic biomarker options .
Higher β-synuclein levels were further found in the cerebrospinal fluid of Creutzfeldt-Jakob disease (CJD) and Alzheimer's disease (AD) .
β-synuclein

A study published today in Neurology investigated whether beta-synuclein is a suitable blood marker for early diagnosis
of prion diseases and synaptic integrity .

The study used a newly established high-sensitivity digital enzyme-linked immunosorbent assay to detect blood beta-synuclein (nfl) from carriers of symptomatic prion disease and prosymptomatic prion protein gene (prnp) mutations.
) were cross-analyzed

A study published today in Neurology investigated whether beta-synuclein is a suitable blood marker for early diagnosis

The newly established digital enzyme-linked immunosorbent assay (digital ELISA) and single-molecule chip technology were used to analyze blood samples from human, prion, and amyotrophic lateral sclerosis animal models
.

In addition, β-synuclein was detected in the brain tissue of Creutzfeldt-Jakob disease (CJD) patients and controls

• A total of 308 patients were investigated, including 129 with prion disease, 8 with presymptomatic np mutation carriers, 60 with ALS, 68 with others, and 43 in the control group
  .
  
• In symptomatic cases of Creutzfeldt-Jakob disease, β-synuclein and nfl were significantly increased (p < 0.
  
  001) compared with all other diagnostic groups
  
  Blood β-synuclein was markedly elevated in CJD patients, reflecting persistent synaptic damage, and presented a well-differentiated profile
  
  

  Source: Halbgebauer S, Abu-Rumeileh S, Oeckl P, et al.

  
  Halbgebauer S, Abu-Rumeileh S, Oeckl P, et al.
  Blood β-Synuclein and Neurofilament Light Chain During the Course of Prion Disease [published online ahead of print, 2022 Feb 2].
  Neurology.
  2022;10.
  1212/WNL.
  0000000000200002.
  doi:10.
  1212/WNL.
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