The role of motor nerve cells: Early evidence of degeneration

The ALS Therapy Alliance has funded studies by leading scientists worldwide. This research has detected numerous findings, including early evidence of degeneration. In their research, these scientists have studied ALS mice, finding electrical abnormalities in their motor neurons long before the disease begins. The motor nerve cells are electrically overactive in a pattern that is likely to be injurious after many months.

Early evidence of degeneration was discovered by scientists at the Massachusetts Institute of Technology and Australian researcher Brigitte van Zundert. When studying motor neurons in brain slices of one week old ALS mice, there was evidence of abnormal behavior – excessive firing and abnormal circuitry and sprouting of motor neurons.

ALS is difficult to diagnose because the symptoms are similar to those of other neuromuscular disorders. The neurological exam usually shows evidence of muscle weakness and atrophy. In ALS, non-motor functions such as feeling, memory and cognition remain normal.