New patient-designed scale captures long-term effects of autoimmune encephalitis

A team of researchers including Encephalitis International’s CEO Dr Ava Easton MBE have  produced the first questionnaire designed specifically to measure how autoimmune encephalitis affects patients’ lives in their own words.

Background

Autoimmune encephalitis (AE) is a severe neurological condition in which the body’s own antibodies attack the brain. With early recognition and treatment many patients can achieve a favourable functional recovery.

Neverthelesss, many are left with lasting difficulties: problems with memory, attention, concentration, fatigue, anxiety, low mood, and returning to work or study; which can persist for years. The scales doctors usually rely on, such as the modified Rankin Scale (mRS), were designed for conditions like stroke and focus on physical disability. As a result, the more subtle cognitive, psychiatric, emotional, and social effects of encephalitis are often missed.

The research

Led by researchers at Erasmus University Medical Centre in the Netherlands, a national referral centre for autoimmune encephalitis, the team built a tool the researchers have named PROSE (the Patient-Reported Outcome Scale for Encephalitis) from the ground up, working directly with patients to ensure it reflected what matters most to them.

Starting from a large pool of existing questionnaire items and adding new ones drawn from patient focus groups, the researchers refined and tested the tool with 202 patients across three common forms of the disease (anti-NMDAR, anti-LGI1, and anti-CASPR2 encephalitis). Some of the key findings were:

• The scale contains 40 questions and takes just 10–20 minutes to complete.
• It covers six areas of life: cognition, behaviour, emotional wellbeing, physical wellbeing, daily activities, and participation.
• Around 1 in 5 of the final questions came directly from patient input, ensuring the scale reflects lived experience.
• It provided stable results when patients were re-tested two weeks apart.
• It could detect improvement over time, particularly in the first 18 months after diagnosis, and remained useful years later when other clinical scales fell short.
• Scores worked equally well across different ages, sexes, and educational backgrounds.

The lead author of the study was Juliette Brenner MD.

In addition to CEO of Encephalitis International Dr Ava Easton MBE, the research team also included Encephalitis International’s Scientific Advisory Panel member Professor Maarten Titulaer, Associate Professor of Neurology and co-director of the Academic Center for Neuro-inflammatory disorders at the Erasmus University Medical Center, Rotterdam.

The authors are listed as:

Juliette Brenner MD, Yvette S. Crijnen MD, Ilse Kulderij MSc, Sammy H.C. Olijslagers MD, Cinthia J. Ruhe MSc, Anke A.G. Tolido MSc, Laura P. Kersten MSc, Julia C.P. Verkoelen BSc, Chelsey N. Kret BSc, Brigit Thomassen MSc, Finn I. van Wijnen BSc, Juna M. de Vries MD, PhD, Tessa M. Bienfait MD, Tessa Brand, Jeroen Kerstens MD, Robin W. van Steenhoven MD, Anna E. M. Bastiaansen MD, PhD, Marienke A.A.M. de Bruijn MD, PhD, Agnes van Sonderen MD, PhD, Sharon Veenbergen PhD, Ava Easton PhD, Peter A. E. Sillevis Smitt, Esther van den Berg, Melissa Mandarakas Mandarakas PhD, and Maarten J. Titulaer.

Looking ahead

The authors envision PROSE being used alongside existing clinical and cognitive assessments, both in everyday care and in research. Because it measures outcomes on a consistent scale, it could make clinical trials more sensitive and help compare treatments.

There are some caveats, however. PROSE is not suitable for the earliest, most acute phase of illness, or for the most severely affected patients who cannot yet answer for themselves. It was also developed in a Dutch-speaking adult cohort, so further validation is needed in other languages, cultures, as well as in children.

Nevertheless, this research offers a meaningful step towards capturing the full picture of life after encephalitis, giving patients a stronger voice in their own recovery.

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To view the original paper, click here.

To view our factsheet on autoimmune encephalitis, click here.

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