Which pathologic findings indicate parkinsonism – dementia – amyotrophic lateral sclerosis complex (PDALS)?

Updated: Sep 24, 2018
  • Author: Stephen M Bloomfield, MD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Answer

Pathologic evaluation of the substantia nigra has found depigmentation, basophilic inclusion bodies, cell loss, and neurofibrillary tangles without senile plaques. This last finding also has been observed in the anterior horn cells and pyramidal tracts. Motor neuron disease tends to occur in younger patients, and older patients tend to develop parkinsonism and severe dementia. In Guam, the presentation varies between the 2 cultural groups that have populated the island. Both the ALS form and the parkinsonism-dementia form of PDALS are seen in the Chamorros group, while the Filipino group tends to have the parkinsonism-dementia syndrome.


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