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. 2005 Apr 26;64(8):1384-90.
doi: 10.1212/01.WNL.0000158425.46019.5C.

The natural history of temporal variant frontotemporal dementia

W W Seeley  1 , A M Bauer, B L Miller, M L Gorno-Tempini, J H Kramer, M Weiner, H J Rosen
Affiliations

The natural history of temporal variant frontotemporal dementia

W W Seeley et al. Neurology. .

Abstract

Background: The temporal variant of frontotemporal dementia (tvFTD) features asymmetric anterior temporal/amygdala degeneration as well as ventromedial frontal, insular, and inferoposterior temporal involvement. Left temporal atrophy has been linked to loss of semantic knowledge, whereas behavioral symptoms dominate the right temporal variant.

Objective: To investigate the first symptoms and the timing of subsequent symptoms in patients with left versus right tvFTD.

Methods: Twenty-six patients with tvFTD were identified. Six had right > left temporal atrophy (right temporal lobe variant [RTLV]) and were matched with six having comparable left > right temporal atrophy (left temporal lobe variant [LTLV]). Clinical records were reviewed to generate individualized symptom chronologies.

Results: In all patients, first symptoms involved semantics (4/6 LTLV, 1/6 RTLV), behavior (4/6 RTLV, 1/6 LTLV), or both (1 LTLV, 1 RTLV). Semantic loss began with anomia, word-finding difficulties, and repetitive speech, whereas the early behavioral syndrome was characterized by emotional distance, irritability, and disruption of physiologic drives (sleep, appetite, libido). After an average of 3 years, patients developed whichever of the two initial syndromes--semantic or behavioral--that they lacked at onset. A third stage, 5 to 7 years from onset, saw the emergence of disinhibition, compulsions, impaired face recognition, altered food preference, and weight gain. Compulsions in LTLV were directed toward visual, nonverbal stimuli, whereas patients with RTLV were drawn to games with words and symbols.

Conclusions: The temporal variant of frontotemporal dementia follows a characteristic cognitive and behavioral progression that suggests early spread from one anterior temporal lobe to the other. Later symptoms implicate ventromedial frontal, insular, and inferoposterior temporal regions, but their precise anatomic correlates await confirmation.

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Figures

Figure 1
Figure 1
Coronal T1 MP-RAGE images of a representative pairing of patients with left and right temporal lobe variants (LTLV and RTLV) using temporal volume Z scores. The left side of each image represents the right side of the head. LATC = left anterior temporal cortex; RATC = right anterior temporal cortex.
Figure 2
Figure 2
Mean volume Z scores for anatomic regions of interest. Reference to side is relative to side of worst temporal atrophy. Ipsi = ipsilateral; Contra = contralateral; ATC = anterior temporal cortex; amy = amygdala; VMFC = ventromedial frontal cortex; TFC = total frontal cortex. Significant differences vs mean contralateral temporal Z score (†) indicated by *p ≤ 0.006, #p < 0.001.
Figure 3
Figure 3
Intervals to first symptom clusters and downstream symptoms occurring in ≥75% of patients. Symptoms present at onset were assigned a value of zero. Dotted line indicates overall mean duration of symptoms at time of entry. *Mean interval significantly greater than mean interval to opposite first symptom cluster (†), p < 0.05. LTLV = left temporal lobe variant; RTLV = right temporal lobe variant.
See this image and copyright information in PMC

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References

    1. Rosen HJ, Gorno-Tempini ML, Goldman WP, et al. Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology. 2002;58:198–208. - PubMed
    1. Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol. 2004;55:335–346. - PMC - PubMed
    1. Studholme C, Cardenas V, Blumenfeld R, et al. Deformation tensor morphometry of semantic dementia with quantitative validation. Neuroimage. 2004;21:1387–1398. - PubMed
    1. Rosso SM, Roks G, Stevens M, et al. Complex compulsive behaviour in the temporal variant of frontotemporal dementia. J Neurol. 2001;248:965–970. - PubMed
    1. Hodges JR, Patterson K, Oxbury S, Funnell E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain. 1992;115:1783–1806. - PubMed

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