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 Volume 73, Number 18, November 3, 2009
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NEUROLOGY 2009;73:1443-1450
© 2009 American Academy of Neurology

Two distinct subtypes of right temporal variant frontotemporal dementia

K. A. Josephs, MST, MD, MS, J. L. Whitwell, PhD, D. S. Knopman, MD, B. F. Boeve, MD, P. Vemuri, PhD, M. L. Senjem, MS, J. E. Parisi, MD, R. J. Ivnik, PhD, D. W. Dickson, MD, R. C. Petersen, MD, PhD and C. R. Jack, Jr, MD

From the Departments of Neurology (Division of Behavioral Neurology) (K.A.J., D.S.K., B.F.B., R.C.P.), Radiology (J.L.W., P.V., C.R.J.), Information Technology (M.L.S.), Neuropsychology (R.J.I.), and Laboratory Medicine and Pathology (J.E.P.), Mayo Clinic, Rochester, MN; and Department of Neuroscience (Neuropathology) (D.W.D.), Mayo Clinic, Jacksonville, FL.

Address correspondence and reprint requests to Dr. Keith A. Josephs, Department of Neurology, Mayo Clinic, Rochester, MN 55905 josephs.keith{at}mayo.edu

Background: Right temporal frontotemporal dementia (FTD) is an anatomic variant of FTD associated with relatively distinct behavioral and cognitive symptoms. We aimed to determine whether right temporal FTD is a homogeneous clinical, imaging, and pathologic/genetic entity.

Methods: In this case-control study, 101 subjects with FTD were identified. Atlas-based parcellation generated temporal, frontal, and parietal grey matter volumes which were used to identify subjects with a right temporal dominant atrophy pattern. Clinical, neuropsychological, genetic, and neuropathologic features were reviewed. The subjects with right temporal FTD were grouped by initial clinical diagnosis and voxel-based morphometry was used to assess grey matter loss in the different groups, compared to controls, and each other.

Results: We identified 20 subjects with right temporal FTD. Twelve had been initially diagnosed with behavioral variant FTD (bvFTD), and the other 8 with semantic dementia (SMD). Personality change and inappropriate behaviors were more frequent in the bvFTD group, while prosopagnosia, word-finding difficulties, comprehension problems, and topographagnosia were more frequent in the SMD group. The bvFTD group showed greater loss in frontal lobes than the SMD group. The SMD group showed greater fusiform loss than the bvFTD group. All 8 bvFTD subjects with pathologic/genetic diagnosis showed abnormalities in tau protein (7 with tau mutations), while all three SMD subjects with pathology showed abnormalities in TDP-43 (p = 0.006).

Conclusions: We have identified 2 subtypes of right temporal variant frontotemporal dementia (FTD) allowing further differentiation of FTD subjects with underlying tau pathology from those with TDP-43 pathology.

Abbreviations: ADPR = Alzheimer Disease Patient Registry; ADRC = Alzheimer Disease Research Center; bvFTD = behavioral variant frontotemporal dementia; CDR-SB = Clinical Dementia Rating Scale sum of boxes; FDR = False Discovery Rate; FTD = frontotemporal dementia; MMSE = Mini-Mental State Examination; NPI = Neuropsychiatric Inventory; SMD = semantic dementia; TPM = tissue probability map; VBM = voxel-based morphometry.

Supplemental data at www.neurology.org

Supported by the NIH Roadmap Multidisciplinary Clinical Research Career Development Award Grant (K12/NICHD)-HD49078, by grants P50 AG16574, U01 AG06786, and R01 AG11378 from the National Institute on Aging, Bethesda, MD, and the generous support of the Robert H. and Clarice Smith and Abigail Van Buren Alzheimer's Disease Research Program of the Mayo Foundation, USA.

Disclosure: Author disclosures are provided at the end of the article.

Received April 20, 2009. Accepted in final form July 17, 2009.






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