Correspondence |

TREM2, Frontotemporal Dementia–Like Disease, Nasu-Hakola Disease, and Alzheimer Dementia: A Chicken and Egg Problem?

Marino Muxfeldt Bianchin, MD, PhD; Ana Lucia Abujamra, MD, PhD; Ivan Izquierdo, MD, PhD
JAMA Neurol. 2013;70(6):805-806. doi:10.1001/jamaneurol.2013.453.
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We read with great interest the article by Guerreiro et al1 on the loss of function of the triggering receptor expressed on myeloid cells 2 (TREM2) gene causing Nasu-Hakola disease (NHD) and some frontotemporal dementia (FTD)–like diseases.1,2 Recently, TREM2 allele variants were also found to be associated with Alzheimer disease (AD).3 However, NHD, FTD-like disease, and AD differ enormously regarding the mechanism of disease, clinical aspects, and neuropathological findings. How can these differences be conciliated?

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Figure. Mechanism suggested for TREM2 involvement in Nasu-Hakola disease (NHD)/frontotemporal dementia–like syndromes or in Alzheimer disease (AD). A, TREM2/TYR0BP loss of function leads to primary microglia dysfunction and an inflammatory process causing neural damage and NHD or FTD-like phenotypes. B, The neuronal damage seems to come first in AD, provoking a secondary TREM2-dependent inflammatory response that might, in its turn, affect AD progression.




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