Very early semantic dementia with progressive temporal lobe atrophy an 8-year longitudinal study

Kathrin Czarnecki, Joseph R. Duffy, Carissa R. Nehl, Shelley A. Cross, Jennifer R. Molano, Clifford R Jr. Jack, Maria M. Shiung, Keith Anthony Josephs, Bradley F Boeve

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Background: Semantic dementia is a syndrome within the spectrum of frontotemporal lobar degenerations characterized by fluent progressive aphasia (particularly ano-mia) 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. Design: Case report. Setting: A tertiary care center. Patient: A 62-year-old woman who presented with "memory loss" complaints. Main Outcome Measures: Clinical course, neuropsy-chological data, and magnetic resonance imaging results. Results: The patient was first evaluated when the results of standard neuropsychological measures were nor-mal 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 prosop-agnosia 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 struc tural neuroimaging evaluations. Recognition of this relatively rare syndrome is important for early diagnosis and prognostication and particularly for therapeutic interventions in the future.

Original languageEnglish (US)
Pages (from-to)1659-1663
Number of pages5
JournalArchives of Neurology
Volume65
Issue number12
DOIs
StatePublished - Dec 2008

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Frontotemporal Dementia
Temporal Lobe
Atrophy
Longitudinal Studies
Anomia
Wernicke Aphasia
Frontotemporal Lobar Degeneration
Agnosia
Aphasia
Memory Disorders
Natural History
Tertiary Care Centers
Neuroimaging
Early Diagnosis
Magnetic Resonance Imaging
Outcome Assessment (Health Care)
Semantic Dementia
Longitudinal Study
Syndrome
Word Meaning

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Very early semantic dementia with progressive temporal lobe atrophy an 8-year longitudinal study. / Czarnecki, Kathrin; Duffy, Joseph R.; Nehl, Carissa R.; Cross, Shelley A.; Molano, Jennifer R.; Jack, Clifford R Jr.; Shiung, Maria M.; Josephs, Keith Anthony; Boeve, Bradley F.

In: Archives of Neurology, Vol. 65, No. 12, 12.2008, p. 1659-1663.

Research output: Contribution to journalArticle

Czarnecki, Kathrin ; Duffy, Joseph R. ; Nehl, Carissa R. ; Cross, Shelley A. ; Molano, Jennifer R. ; Jack, Clifford R Jr. ; Shiung, Maria M. ; Josephs, Keith Anthony ; Boeve, Bradley F. / Very early semantic dementia with progressive temporal lobe atrophy an 8-year longitudinal study. In: Archives of Neurology. 2008 ; Vol. 65, No. 12. pp. 1659-1663.
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