A report from scientists in the Netherlands indicates that patients with amyotrophic lateral sclerosis (ALS) who develop frontotemporal syndrome (FS), do not survive much longer when given a ventilation mask. In fact, 30 – 50% of patients with ALS and behavior/cognitive deficits due to FC do not respond to the breathing mask, called non-invasive ventilation (NIV)
The study “The Frontotemporal Syndrome Of ALS Is Associated With Poor Survival” was published in the journal Neurology.
Frontotemporal disorders are forms of dementia caused by damage to nerve cells in the frontal and temporal lobes of the brain. Many symptoms can result including trouble with body movement, communication, emotion, and decision making.
“The first aim of this study was to investigate whether a frontotemporal syndrome is an independent risk factor for poor survival in ALS,” the authors wrote in the report. “Second, we aimed to gain insight into the effect of the frontotemporal syndrome on NIV initiation and duration in ALS patients.”
To do so, researchers followed 110 ALS patients, 47 who had FS. They analyzed the effect of FS on survival, and the start and duration of NIV compared to patients without FS. They determined behavioral changes existed if the patient scored more than 22 points on the ALS-Frontotemporal-Dementia Questionnaire, or if they scored three or more points on two or more items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on two or more tests evaluating executive function, memory, or language. Survival was analyzed from the beginning of the symptoms and time from NIV start until death.
The analysis indicated that ALS patients with FS had a significantly shorter survival independent of prognostic factors such as older age at onset, bulbar onset, short time to diagnosis, and the presence of the C9orf72 repeat expansion which causes neurodegeneration — even after initiation of NIV. This observation indicates that FS is associated with shorter survival regardless of whether the patient starts NIV or not.
The team also noticed that ALS patients with behavioral changes, but not cognitive changes, less often initiated NIV but it was not possible in this study to investigate exactly what behavioral changes, such as apathy or impulsivity, could bring about the difference.
Future studies with larger groups of ALS patients are necessary to confirm the association between FS and poor outcome in ALS.