ALS End-stage Brain Damage Isn’t Limited to Motor Neurons, Study Finds

Patients with amyotrophic lateral sclerosis (ALS) who have progressed to a stage in which they’ve lost all voluntary movements, including the ability to communicate, have damage in numerous brain regions and isn’t limited to motor neurons.

The study, “Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V),” published in the journal Acta Neuropathologica Communications, suggests that such brain damage can be used to identify patients likely to progress to a total paralytic stage.

Attention by physicians to non-movement symptoms could be important in predicting the progress to a total locked-in stage, particularly in patients with a rapid disease course.

Earlier studies of ALS patients who have progressed to a completely “locked-in” stage, what clinicians refer to as communication stage 5, have shown various forms of brain damage. These studies have all been case reports.

To get a more comprehensive picture, researchers at the Tokyo Metropolitan Neurological Hospital in Japan examined the medical records of all locked-in patients from the 320 ALS patients at two Japanese institutions.

They found 11 patients who had progressed to a total locked-in stage. None of them had shown signs of cognitive or behavioral problems before progressing to the late-stage disease. Six of the patients had aggregates of phosphorylated TDP inside nerve cells, two had FUS aggregates, and three had SOD1 aggregates.

excerpt © 2016 ALS News Today. All rights reserved.