Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.

@article{Kosaka2012PrimaryLS,
  title={Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.},
  author={Takayuki Kosaka and Yong-Juan Fu and Atsushi Shiga and Haruka Ishidaira and C. Tan and Takashi Tani and Ryoko Koike and Osamu Onodera and Masatoyo Nishizawa and Akiyoshi Kakita and Hitoshi Takahashi},
  journal={Neuropathology : official journal of the Japanese Society of Neuropathology},
  year={2012},
  volume={32 4},
  pages={373-84}
}
Primary lateral sclerosis (PLS) is clinically defined as a disorder selectively affecting the upper motor neuron (UMN) system. However, recently it has also been considered that PLS is heterogeneous in its clinical presentation. To elucidate the association of PLS, or disorders mimicking PLS, with 43-kDa TAR DNA-binding protein (TDP-43) abnormality, we examined two adult patients with motor neuron disease, which clinically was limited almost entirely to the UMN system, and was followed by… CONTINUE READING
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