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Case Rep Neurol. 2011 Sep;3(3):242-7. doi: 10.1159/000331859. Epub 2011 Oct 21.

Amnesia in frontotemporal dementia with amyotrophic lateral sclerosis, masquerading Alzheimer's disease.

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Laboratory of Structural Neuropathology, Tokyo Metropolitan Institute of Medical Science, Tokyo Medical and Dental University, Tokyo, Japan.


A 68-year-old man with a clinical diagnosis of Alzheimer's disease (AD) later developed amyotrophic lateral sclerosis (ALS), which was confirmed at autopsy at age 72 years. Because neuronal loss and AD-type pathologies (Braak stage II for neurofibrillary tangles) were scant, TDP-43-positive intracytoplasmic inclusions in hippocampal dentate granular cells and in neurons in the subiculum and amygdala, even though small in amount, may represent the earliest lesions of ALS-related dementia and could be the cause of dementia in this patient. Although the persistent elevation of creatine kinase from the onset could be a pointer to the presence of motor involvement, more accurate characterization of dementia, which may differentiate ALS-related dementia and AD, is necessary.


Alzheimer's disease; Amyotrophic lateral sclerosis; Creatine kinase; Dementia

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