Frontotemporal dementia and motor neuron disease: report of 3 cases in Taiwan and literature review.
OBJECTIVE
Case reports and a review of literature of the coexistence of motor neuron disease (MND) and frontotemporal dementia (FTD).


METHODS
All three patients demonstrated generalized lower motor neuron signs and very few upper motor neuron signs. In the level of patterns of cognitive impairments, neuropsychological studies do not distinguish between patients with onset of weakness from bulbar palsy and patients with onset of weakness from limbs. All patients of FTD had their onset of MND or amyotrophic lateral sclerosis symptoms within a two-year interval which is similar to previous reports. FTD combined with MND may shorten the survival to less than three years shorter than cases with FTD only. Respiratory failure occurred one to two years after onset of the behavioral symptoms in all patients.


CONCLUSIONS
We reported three patients of FTD with MND to remind clinicians that these two disorders may occur together on the same patient and that these two disorders may belong to one broad spectrum neurodegenerative disease.


BACKGROUND
motor neuron disease, amyotrophic lateral sclerosis, frontotemporal dementia, frontotemporal lobar degeneration.
Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan.
Kai-Hsiang
KH
Chen
Ming-Jang
MJ
Chiu
Ting-Wen
TW
Cheng
Jen-Jen
JJ
Su
D000690:Amyotrophic Lateral Sclerosis / Q000150:complications; D057180:Frontotemporal Dementia / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications*