Very early semantic dementia with progressive temporal lobe atrophy: an 8-year longitudinal study.
BACKGROUND
Semantic dementia is a syndrome within the spectrum of frontotemporal lobar degenerations characterized by fluent progressive aphasia (particularly anomia) and loss of word meaning.


OBJECTIVE
To report a unique case of very early semantic dementia with a slowly progressive course, allowing insights into the early natural history of this disorder.


METHODS
Case report.


METHODS
A tertiary care center.


METHODS
A 62-year-old woman who presented with "memory loss" complaints.


METHODS
Clinical course, neuropsychological data, and magnetic resonance imaging results.


RESULTS
The patient was first evaluated when the results of standard neuropsychological measures were normal but subtle left anterior temporal lobe atrophy was present. During the follow-up period of 8 years, she developed profound anomia and loss of word meaning associated with progressive left anterior temporal lobe atrophy, consistent with semantic dementia. In more recent years, anterograde memory impairment and mild prosopagnosia evolved in association with left hippocampal atrophy and subtle atrophy in the homologous gyri of the right anterior temporal lobe. She remains functionally independent despite her current deficits.


CONCLUSIONS
Early identification of patients who will develop semantic dementia is difficult and might be missed with standard clinical, neuropsychological, and structural neuroimaging evaluations. Recognition of this relatively rare syndrome is important for early diagnosis and prognostication and particularly for therapeutic interventions in the future.
Czarnecki
Kathrin
K
Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Duffy
Joseph R
JR
Nehl
Carissa R
CR
Cross
Shelley A
SA
Molano
Jennifer R
JR
Jack
Clifford R
CR
Shiung
Maria M
MM
Josephs
Keith A
KA
Boeve
Bradley F
BF
D001284:Atrophy / Q000150:complications* / Q000473:pathology*; D003704:Dementia / Q000150:complications* / Q000473:pathology*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging / Q000379:methods; D008875:Middle Aged; D009483:Neuropsychological Tests; D013702:Temporal Lobe / Q000473:pathology*