Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND.
Pathologic diagnoses in frontotemporal lobar degeneration (FTLD) include tau-positive FTLD and tau-negative FTLD. Two variants of tau-negative FTLD are FTLD with motor neuron disease (FTLD-MND) and FTLD with motor neuron disease type inclusions but without motor neuron disease (FTLD-U). An analysis of patient outcomes in these cases reveals that FTLD-MND has significantly shorter survival than FTLD-U, suggesting that FTLD-MND is a more aggressive disease process.
Josephs
K A
KA
Department of Neurology, Mayo Clinic, 200 First St S.W., Rochester, MN 55905, USA. josephs.keith@mayo.edu
Knopman
D S
DS
Whitwell
J L
JL
Boeve
B F
BF
Parisi
J E
JE
Petersen
R C
RC
Dickson
D W
DW
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D015897:Comorbidity; D003704:Dementia / Q000145:classification / Q000401:mortality* / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000145:classification / Q000401:mortality* / Q000473:pathology*; D009046:Motor Neurons / Q000378:metabolism / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000378:metabolism; D012189:Retrospective Studies; D012737:Sex Factors; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology; D015996:Survival Rate / Q000639:trends; D016875:tau Proteins / Q000378:metabolism*
D015415:Biomarkers; D009419:Nerve Tissue Proteins; D016875:tau Proteins