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The natural history of temporal variant frontotemporal dementia

From the Departments of Neurology (Drs. Seeley, Miller, Gorno-Tempni, Kramer, Weiner, and Rosen) and Radiology (Dr. Weiner), USCF Memory and Aging Center (Drs. Seeley, Miller, Gorno-Tempni, Kramer, and Rosen), University of California at San Francisco, San Francisco, CA; Department of Psychiatry (Dr. Bauer), McLean Hospital, San Francisco, CA; Magnetic Resonance Imaging Unit (Dr. Weiner), San Francisco Veterans Affairs Hospital, San Francisco, CA.

Address correspondence and reprint requests to Dr. William W. Seeley, Memory and Aging Center, UCSF Department of Neurology, 350 Parnassus Avenue, Suite 706, Box 1207, San Francisco, CA 94143-1207; e-mail: wseeley{at}memory.ucsf.edu

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.

Supported by the Janssen Foundation (W.W.S.), the John Douglas French Foundation (M.L.G.T.), and a K08 (H.J.R.) and grants from the National Institute of Aging (1P01 AG19724-01A1, P50 AG1657303-75271) and the State of California Department of Health Services.

Received August 6, 2004. Accepted in final form December 27, 2004.

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