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Sporadic amyotrophic lateral sclerosis with pallido‐nigro‐luysian degeneration: A TDP‐43 immunohistochemical study
Author(s) -
Miki Yasuo,
Mori Fumiaki,
Nunomura Jinichi,
Ookawa Keizou,
Yajima Nobuhisa,
Yagihashi Soroku,
Wakabayashi Koichi
Publication year - 2010
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.2009.01046.x
Subject(s) - amyotrophic lateral sclerosis , substantia nigra , globus pallidus , pathology , medicine , subthalamic nucleus , frontotemporal dementia , hippocampus , basal ganglia , dementia , neuroscience , biology , parkinson's disease , central nervous system , disease , deep brain stimulation
Recently, Nishihira et al. demonstrated the presence of two types of TDP‐43 pathology in sporadic amyotrophic lateral sclerosis (ALS) (Acta Neuropathol 2008; 116: 169–182). Type 1 represents the TDP‐43 distribution pattern observed in classic ALS, whereas type 2 shows the presence of TDP‐43 inclusions in the frontotemporal cortex, hippocampal formation, neostriatum and substantia nigra and is significantly associated with dementia. However, ALS with pallido‐nigro‐luysian degeneration (PNLD) is very rare. We recently encountered a case of ALS with PNLD of 9 years duration, in which the patient received artificial respiratory support for 6 years. In our case, neuronal loss and TDP‐43‐positive neuronal cytoplasmic inclusions were found in the globus pallidus, substantia nigra and subthalamic nucleus. Neither neuronal loss nor TDP‐43‐immunoreactive inclusions were found in the frontotemporal cortex and hippocampus. These findings suggest that the pallido‐nigro‐luysian system is also involved in the disease process of ALS and that ALS with PNLD is different from ALS with dementia based on the distribution pattern of neuronal loss and TDP‐43 accumulation.