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Early brain vulnerability in Wolfram syndrome.

Tamara Hershey1, Heather M Lugar, Joshua S Shimony

  • 1Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, United States of America. tammy@npg.wustl.edu

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Wolfram syndrome (WFS) causes brain abnormalities, including smaller volumes in the brainstem and cerebellum, even in early stages. These findings suggest WFS impacts early brain development, not just later neurodegeneration.

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Area of Science:

  • Neuroscience
  • Genetics
  • Rare Diseases

Background:

  • Wolfram syndrome (WFS) is a rare autosomal recessive disorder.
  • WFS is caused by WFS1 gene mutations, leading to endoplasmic reticulum (ER) stress and cell death.
  • Previous studies noted brain atrophy in late-stage WFS, but early developmental impacts were unknown.

Purpose of the Study:

  • To investigate the onset and regional distribution of brain abnormalities in early-stage Wolfram syndrome.
  • To compare brain structure and white matter integrity in young WFS patients with control groups.

Main Methods:

  • Structural magnetic resonance imaging (MRI) was used to assess brain volume and tissue integrity.
  • Neurological and cognitive assessments were performed on WFS patients.
  • WFS patients were compared to healthy and type 1 diabetic control groups.

Main Results:

  • WFS patients exhibited smaller intracranial volume, with preferential gray matter and white matter abnormalities in the brainstem, cerebellum, and optic radiations.
  • Abnormalities were present even in the youngest patients with mild symptoms.
  • Cognition remained intact, but anxiety, depression, and gait disturbances were observed.

Conclusions:

  • Wolfram syndrome is associated with reduced intracranial volume and specific brainstem and cerebellar abnormalities from the earliest symptomatic stages.
  • These findings indicate a significant impact on early brain development, alongside potential later neurodegenerative effects.
  • Longitudinal studies are needed to further understand the role of ER stress in WFS brain development.