Clearly Defined “Staging” in ALS May Give New Insights into Disease Progression

Researchers at the Perelman School of Medicine at the University of Pennsylvania and the University of Ulm in Germany have been able to classify four distinct neuropathological “stages” of ALS, the findings of which were recently published in the Annals of Neurology. Led by co-investigators Dr. John Trojanowski of the University of Pennsylvania and Dr. Heiko Braak of the University of Ulm, the research team looked for TDP-43 aggregates in the brains and spinal cords of post-mortem ALS patients. By examining where pathological TDP-43 aggregates were located in ALS patients, they were able to classify four distinct stages of TDP-43 “spreading” in the patients. The researchers first identified TDP-43 in the motor cortex and brainstem, primarily in the neurons that controlled breathing and movement. TDP-43 aggregates then spread to other regions of the brain, such as those involved in balance, as ALS symptoms progressed. Dr. Trojanowski hopes that these newly identified neuropathological stages will help elucidate the role that TDP-43 may play in transmitting ALS either from cell to cell and/or region to region. Click here to read this provocative story.

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