An Autopsy Confirmed Case of Amyotrophic Lateral Sclerosis with TDP Pathology

The phosphorylated 43-kDa transactive response DNA-binding protein (TDP-43) was identified as a major disease in sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. We present case with progressive muscle weakness who diagnosed ALS. On postmortem examination, TDP-43 immunoreactive neuronal cytoplasmic inclusions were noted motor cortex, hippocampus anterior horns of spinal cord, which compatible ALS-TDP, stage 4. This is the first documented autopsy-confirmed ALS ALS-TDP pathology Korea.