Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia

Christian Sandøe Musaeus1, Jette Stokholm Pedersen1, Troels Wesenberg Kjær2,3

  • 1Danish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.

Insights

CHMP2B gene mutations cause frontotemporal dementia (FTD). EEG microstate analysis revealed altered brain network dynamics in symptomatic and pre-symptomatic carriers, suggesting potential biomarkers for neurodegeneration.

Area of Science:

  • Neuroscience
  • Genetics
  • Neurology

Background:

  • Frontotemporal dementia (FTD) is a neurodegenerative disorder.
  • CHMP2B gene mutations cause a rare, autosomal dominant form of FTD (CHMP2B-FTD).
  • CHMP2B-FTD patients exhibit distinct executive dysfunction and offer insights into early electroencephalographic (EEG) changes.

Purpose of the Study:

  • To investigate EEG microstate dynamics in CHMP2B-FTD.
  • To compare symptomatic and pre-symptomatic CHMP2B mutation carriers with non-carriers.
  • To explore potential early electrophysiological markers of neurodegeneration.

Main Methods:

  • EEG microstate analysis was performed on symptomatic CHMP2B-FTD patients (n=5), pre-symptomatic carriers (n=5), and non-carriers (n=6).
  • Data was parsed into four archetypal microstates, and global power was calculated.
  • Microstate occurrence and duration were analyzed in relation to disease duration and symptom onset.

Main Results:

  • A trend towards lower occurrence of microstate D was observed in CHMP2B-FTD patients (p=0.177).
  • Patients with recent symptom onset (<1 year) showed increased microstate D duration.
  • Patients with longer disease duration (>2 years) exhibited decreased microstate D duration.

Conclusions:

  • EEG microstate analysis reveals dynamic changes in brain networks associated with CHMP2B-FTD.
  • The biphasic pattern of microstate D duration may reflect neurodegenerative processes.
  • These findings could inform the development of biomarkers for early detection and monitoring of neurodegenerative diseases.

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