"How are you?" - Mental Health Challenges in Children and Adolescents with Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) (2021-ongoing)

Project Team Members (OUS): Kristin Romvig Øvergaard and Beate Ørbeck (OUS-KPHA); Ingrid Helland (OUS-Department of Neurology for Children)

Collaborators: Irene Rothschild (NCL Association)

The main goal of the project is to investigate the occurrence of mental health challenges in children and adolescents with JNCL and identify the areas in which they may struggle. 

The project will provide insights into the occurrence of mental health challenges in JNCL at different ages. This information will be clinically useful to understand the questions that should be addressed during medical check-ups and to identify the need for treatment. The innovation potential of the project lies in patient benefit, clinical and health value, as well as the improvement of healthcare services.

JNCL, also known as Spielmeyer-Vogt or Batten disease, is the most common form of hereditary neurodegenerative diseases in Norway, with approximately 30 known patients. It is a well-known clinical experience that patients with JNCL may also develop mental health challenges. However, the research literature has been modest so far, characterized by case reports and studies with descriptions lacking the use of standardized instruments. We are now including families, collecting information through questionnaires and psychiatric diagnostic interviews.

Publications:

Øvergaard KR, Ørbeck B. Children with neurodegenerative disease and obsessive-compulsive behaviour. Tidsskr Nor Laegeforen. 2020 Apr 20;140(6). English, Norwegian. doi: 10.4045/tidsskr.19.0703. PMID: 32321233.