Psychiatric Diagnoses and Medications in Wolfram Syndrome

Publication: sciendo.com | Publication Date: December 31, 2022

Authors: Angela M. Reiersen, Jacob S. Noel, Tasha Doty, Richa A. Sinkre, Anagha Narayanan and Tamara Hershey

Background

Wolfram Syndrome is a rare genetic disorder usually resulting from pathogenic variation in the WFS1 gene, which leads to an exaggerated endoplasmic reticulum (ER) stress response. The disorder is typically characterized by diabetes insipidus, diabetes mellitus, optic nerve atrophy, hearing loss, and neurodegenerative features. Existing literature suggests it may also have psychiatric manifestations.

Objective To examine lifetime psychiatric diagnoses and medication history in Wolfram Syndrome.

Method Child, adolescent, and young adult Wolfram Syndrome participants (n=39) were assessed by a child & adolescent psychiatrist to determine best estimate DSM-5 lifetime psychiatric diagnoses as well as psychoactive medication history. In addition, the Child & Adolescent Symptom Inventory-5 (CASI-5) Parent Checklist was used to determine likely psychiatric diagnoses based on symptom counts in Wolfram Syndrome patients (n=33), type 1 diabetes (n=15), and healthy comparison (n=18) groups.

Reiersen,A.,Noel,J.,Doty,T.,Sinkre,R.,Narayanan,A. & Hershey,T.(2022).Psychiatric Diagnoses and Medications in Wolfram Syndrome. Scandinavian Journal of Child and Adolescent Psychiatry and Psychology,10(1) 163-174. Retrieved February 4, 2024, from https://doi.org/10.2478/sjcapp-2022-0017.