Frontotemporal dementia: report of a familial case.
The authors describe a 49-year-old woman (R.K.) who presented with one year of progressive frontal lobe dysfunction, including signs of expressive aphasia. Signs of parkinsonism were absent until late in the clinical course. Neuropsychologic testing and neuroimaging studies are described. The patient died at age 55, after 7 years of symptoms. Family history was remarkable for a mother who died at the age of 45, after experiencing 7 years of progressive aphasia. R.K.'s brain showed asymmetric frontotemporal atrophy, which was more severe on the left side. Histopathologic analysis was remarkable for numerous tau-positive neurons with some classic-appearing Pick bodies and many ballooned neurons. Tau-positive glial cells were also present. The authors suggest that the abnormal tau aggregates are related to the symptoms experienced by affected members of this family.
Shafiq
M
M
Department of Neurology, MCP-Hahnemann University, Philadelphia, PA, USA.
Nee
L
L
Grafman
J
J
Tresser
N
N
Lee
V M
VM
Trojanowski
J Q
JQ
Lippa
C F
CF
D001037:Aphasia / Q000235:genetics; D001921:Brain / Q000473:pathology*; D003704:Dementia / Q000235:genetics / Q000473:pathology*; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008875:Middle Aged; D009457:Neuroglia / Q000473:pathology*; D009474:Neurons / Q000473:pathology*; D009483:Neuropsychological Tests; D010375:Pedigree; D016875:tau Proteins / Q000378:metabolism*
D016875:tau Proteins