What does the ALS Functional Rating Scale (ALSFRS-R) mean?

The severity of the disease is determined using the ALS Functional Rating Scale (ALSFRS-R). The ALS Functional Rating Scale is an interview with twelve questions on important symptoms and motor functions, for which five possible answers are given.

The first three questions relate to bulbar functions – a possible speech disorder (dysarthria), a swallowing disorder (dysphagia) and increased salivation due to difficulties in swallowing saliva. Two further questions cover functions of the upper extremities – handling a pen and using cutlery. Two further questions touch on the ability to care for oneself independently, including dressing, turning in bed and changing body position. The function of the lower extremities is assessed in two further questions, asking about the ability to walk and climb stairs. Finally, respiratory function is assessed in three questions. These ask about breathing effort (dyspnoea) during physical exertion and at rest, as well as the possible increase in breathlessness at rest or when lying down (orthopnoea). In a final question, the use of non-invasive ventilation (NIV) or a tracheostomy with invasive ventilation is determined.

Those affected select the answer that comes closest to the actual situation. Each answer is assigned a “scale point”. Depending on the answer, four to zero points are awarded per question. After completing the interview of the twelve questions, a total score is obtained, which can be between 48 points (no symptoms or deficits) and zero points (severe deficits).

The ALS Functional Rating Scale was originally established in the USA and translated into German (and numerous other languages) many years ago. The ALSFRS-R is therefore an internationally recognized and established scale for determining and comparing the severity of ALS.