Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations.

Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Research Abstract Details 

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Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Abstract Text:

Jennifer L Whitwell,Clifford R Jack,Matthew Baker,Rosa Rademakers,Jennifer Adamson,Bradley F Boeve,David S Knopman,Joseph F Parisi,Ronald C Petersen,Dennis W Dickson,Michael L Hutton,Keith A Josephs,

BACKGROUND: Mutations in the progranulin gene (PGRN) have recently been identified as a cause of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) in some families. OBJECTIVE: To determine whether there is a difference in the patterns of atrophy in FTLD-U cases with and without PGRN mutations. DESIGN: Case-control study. SETTING: Brain bank of a tertiary care medical center. Patients Eight subjects who had screened positive for PGRN mutations (PGRN-positive) and who underwent volumetric magnetic resonance imaging were identified. Subjects were then matched by clinical diagnosis to a group of 8 subjects with a pathological diagnosis of FTLD-U who had screened negative for PGRN mutations (PGRN-negative). All subjects were then age-matched and sex-matched to a control subject. MAIN OUTCOME MEASURES: Voxel-based morphometry was used to assess the patterns of gray matter atrophy in the PGRN-positive group compared with the PGRN-negative group and compared with controls. RESULTS: The PGRN-positive group showed a widespread and severe pattern of gray matter loss predominantly affecting the frontal, temporal, and parietal lobes. The PGRN-negative group showed a less severe pattern of gray matter loss restricted mainly to the temporal and frontal lobes. On direct comparison, the PGRN-positive group showed greater gray matter loss in the frontal and parietal lobes compared with the PGRN-negative group. CONCLUSION: Findings from this study suggest that PGRN mutations may be associated with a specific and severe pattern of cerebral atrophy in subjects with FTLD-U.

Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Publishing Authors By Initials

JL Whitwell,CR Jack,M Baker,R Rademakers,J Adamson,BF Boeve,DS Knopman,JF Parisi,RC Petersen,DW Dickson,ML Hutton,KA Josephs,

For similar proteins: ubiquitins: ubiquitin research abstracts see: proteins: ubiquitins: ubiquitin research

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Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Archives of neurology

VOLUME: 64

Page Numbers: 371-6

Journal Abbreviation: Arch. Neurol.

ISSN: 0003-9942

DAY: 3

MONTH: Mar

YEAR: 2007

Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Information

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LANGUAGE: eng

NlmUniqueID: 372436

Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Keywords Mesh Terms:

KEYWORDS: Ubiquitin

MESH TERMS: metabolism

Chemical & Substance for Abstract: Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Information

Substance Name: Ubiquitin

Registry Number: 0

Grant and Affiliation Information for Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations.

AFFILIATION: Division of Behavioral Neurology, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.

Country: United States

AGENCY: United States NIA

GRANT: U01 AG06786

ACRONYM: AG

MEDLINETA: Arch Neurol

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