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MedUni Vienna: Creutzfeldt-Jakob disease: Mood alterations identified as a possible early symptom

07.01.2025

Creutzfeldt-Jakob disease (CJD) is a very rare neurodegenerative disease that is fatal within a few months and still poses many questions to scientists. To date, no specific symptoms are known that precede the onset of the disease. A suspected diagnosis can usually be made on the basis of a particular combination of symptoms and examination findings, but can only be confirmed after the death of the affected person. In a study, a research team from MedUni Vienna has now identified mood changes as a possible prodromal symptom. The results, recently published in the renowned journal "JAMA Neurology", could improve the understanding and diagnosis of CJD and provide new impetus for the development of therapies.

In their search for new ways to detect early symptoms of Creutzfeldt-Jakob disease, the research team led by Raphael Wurm and Hakan Cetin (both from MedUni Vienna's Department of Neurology) found what they were looking for in a drug prescription database provided by the Austrian Social Insurance Association. "We wanted to investigate which drugs patients with CJD were prescribed in the years before the onset of the disease," says first author Raphael Wurm, outlining the initial situation. The researchers linked the entries in this register with the data of 129 patients in whom CJD was confirmed by autopsy at the Austrian Reference Centre for Prion Diseases (ÖRPE). All suspected cases of CJD in Austria are reported to this centre, which is situated at MedUni Vienna, on behalf of the Ministry of Health. After the legally required autopsy, the corresponding neuropathological examinations are also carried out there. Over 1,300 persons of similar age, sex and area of residence without CJD formed the comparison group in the study.

More prescriptions for antidepressants

The analysis showed that CJD patients were prescribed antidepressants of the selective serotonin reuptake inhibitor (SSRI) class significantly more often than people without CJD. Specifically, it was found that CJD patients were almost three times more likely to be prescribed SSRIs in the year before the onset of the disease than the control group. SSRIs are drugs that are often used to treat depression and other mood disorders. "It is remarkable that we see an increasing trend towards higher prescription SSRI rates in patients with CJD as early as three years before the onset of the disease," says Raphael Wurm. "This is the first time we have found objective evidence that people with CJD can experience alterations in mood or symptoms of anxiety before the frank clinical onset of the disease, which is often also noticed by relatives. These findings can help to better determine when the diseases breaks out, to better understand the early symptoms of this disease and thus possibly provide new directions for treatment," says study leader Hakan Cetin, summarising the relevance of the results.

15 to 20 cases per year in Austria

Creutzfeldt-Jakob disease is a rare, rapidly progressive neurodegenerative disease of the brain that leads to death within a few months. CJD manifests itself in disorders of balance, coordinated movement, memory and consciousness, personality changes and, rarely, epileptic seizures. CJD became known to the public in connection with the disease in cattle as BSE (bovine spongiform encephalopathy or "mad cow disease") in the 1990s, although the disease had already been described in humans in the 1920s. The study examined the most common form of CJD, known as "sporadic CJD" (85 per cent), which occurs without known genetic or external triggers and has no connection to BSE. In Austria, 15 to 20 cases of this form are recorded each year. While CJD is not curable, the diagnostic possibilities have improved significantly in recent years with the help of cerebrospinal fluid examinations and brain imaging, but a diagnosis can currently only be confirmed after death by examining the brain.

The identification of mood changes as a possible early symptom is a step towards a better diagnosis: "However, it is important for us to emphasise that CJD is a very rare disease, while mood alterations such as depressive episodes are very common. However, if neurological symptoms such as walking difficulties or cognitive changes are also present, a doctor should be consulted in any case," say Raphael Wurm and Hakan Cetin.

Publication: JAMA Neurology

Mood alterations in the prodromal phase of sporadic Creutzfeldt-Jakob disease
Raphael Wurm, Sigrid Klotz, Astrid Erber, Felix Gruber, Stefan Leitner, Berthold Reichardt, Elisabeth Stögmann, Eva Schernhammer, Ellen Gelpi, Hakan Cetin
doi:10.1001/jamaneurol.2024.4447
https://jamanetwork.com/journals/jamaneurology/article-abstract/2828525

Contact

Mag. Johannes Angerer
Medizinische Universität Wien
Leiter Kommunikation und Öffentlichkeitsarbeit
Telefon: 01/40160-11501
E-Mail: pr@meduniwien.ac.at
Website: https://www.meduniwien.ac.at/pr

Mag.a Karin Kirschbichler
Medizinische Universität Wien
Kommunikation und Öffentlichkeitsarbeit
Tel.: 01/ 40 160-11505
E-Mail: pr@meduniwien.ac.at
Website: https://www.meduniwien.ac.at/pr