Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study

Alexis X. Curet Burleson; Nha Trang Thu Pham; Marina Buciuc; Hugo Botha; Joseph R. Duffy; Heather M. Clark; Rene L. Utianski; Mary M. Machulda; Matthew C. Baker; Rosa Rademakers; Val J. Lowe; Jennifer L. Whitwell; Keith A. Josephs

doi:10.1159/000513979

Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study

Alexis X. Curet Burleson, Nha Trang Thu Pham, Marina Buciuc, Hugo Botha, Joseph R. Duffy, Heather M. Clark, Rene L. Utianski, Mary M. Machulda, Matthew C. Baker, Rosa Rademakers, Val J. Lowe, Jennifer L. Whitwell, Keith A. Josephs

Research output: Contribution to journal › Article › peer-review

Abstract

Introduction: Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time. Methods: Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up. Results: The right-dominant group performed worse on the NPI-Q and had a greater frequency of abnormal behaviors and more severe disinhibition compared to the left-dominant group. Performance on the NPI-Q and severity of disinhibition correlated with right medial and lateral, but not left, temporal lobe hypometabolism. Severity of abnormal behaviors worsened over time in most left-dominant and left+ patients but appeared to improve in the 2 right-dominant patients with longitudinal follow-up. All groups showed progressive worsening of metabolism in both temporal lobes over time, with hypometabolism spreading from anteromedial to posterior temporal regions. However, the degree of temporal lobe asymmetry remained relatively constant over time. Conclusion: In SD, neurobehavioral features, especially disinhibition, are associated with right medial and lateral temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism.

Original language	English (US)
Pages (from-to)	17-28
Number of pages	12
Journal	Dementia and geriatric cognitive disorders
Volume	50
Issue number	1
DOIs	https://doi.org/10.1159/000513979
State	Published - Jun 1 2021

Keywords

C9ORF72
FDG-PET
Right temporal pole
Right temporal variant
Semantic dementia

ASJC Scopus subject areas

Geriatrics and Gerontology
Cognitive Neuroscience
Psychiatry and Mental health

Access to Document

10.1159/000513979

Cite this

Curet Burleson, A. X., Pham, N. T. T., Buciuc, M., Botha, H., Duffy, J. R., Clark, H. M., Utianski, R. L., Machulda, M. M., Baker, M. C., Rademakers, R., Lowe, V. J., Whitwell, J. L., & Josephs, K. A. (2021). Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study. Dementia and geriatric cognitive disorders, 50(1), 17-28. https://doi.org/10.1159/000513979

Curet Burleson, AX, Pham, NTT, Buciuc, M, Botha, H, Duffy, JR, Clark, HM , Utianski, RL , Machulda, MM, Baker, MC, Rademakers, R, Lowe, VJ , Whitwell, JL & Josephs, KA 2021, 'Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study', Dementia and geriatric cognitive disorders, vol. 50, no. 1, pp. 17-28. https://doi.org/10.1159/000513979

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title = "Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study",

abstract = "Introduction: Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time. Methods: Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up. Results: The right-dominant group performed worse on the NPI-Q and had a greater frequency of abnormal behaviors and more severe disinhibition compared to the left-dominant group. Performance on the NPI-Q and severity of disinhibition correlated with right medial and lateral, but not left, temporal lobe hypometabolism. Severity of abnormal behaviors worsened over time in most left-dominant and left+ patients but appeared to improve in the 2 right-dominant patients with longitudinal follow-up. All groups showed progressive worsening of metabolism in both temporal lobes over time, with hypometabolism spreading from anteromedial to posterior temporal regions. However, the degree of temporal lobe asymmetry remained relatively constant over time. Conclusion: In SD, neurobehavioral features, especially disinhibition, are associated with right medial and lateral temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism.",

keywords = "C9ORF72, FDG-PET, Right temporal pole, Right temporal variant, Semantic dementia",

author = "{Curet Burleson}, {Alexis X.} and Pham, {Nha Trang Thu} and Marina Buciuc and Hugo Botha and Duffy, {Joseph R.} and Clark, {Heather M.} and Utianski, {Rene L.} and Machulda, {Mary M.} and Baker, {Matthew C.} and Rosa Rademakers and Lowe, {Val J.} and Whitwell, {Jennifer L.} and Josephs, {Keith A.}",

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year = "2021",

month = jun,

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doi = "10.1159/000513979",

language = "English (US)",

volume = "50",

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T1 - Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia

T2 - A Longitudinal Study

AU - Curet Burleson, Alexis X.

AU - Pham, Nha Trang Thu

AU - Buciuc, Marina

AU - Botha, Hugo

AU - Duffy, Joseph R.

AU - Clark, Heather M.

AU - Utianski, Rene L.

AU - Machulda, Mary M.

AU - Baker, Matthew C.

AU - Rademakers, Rosa

AU - Lowe, Val J.

AU - Whitwell, Jennifer L.

AU - Josephs, Keith A.

PY - 2021/6/1

Y1 - 2021/6/1

N2 - Introduction: Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time. Methods: Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up. Results: The right-dominant group performed worse on the NPI-Q and had a greater frequency of abnormal behaviors and more severe disinhibition compared to the left-dominant group. Performance on the NPI-Q and severity of disinhibition correlated with right medial and lateral, but not left, temporal lobe hypometabolism. Severity of abnormal behaviors worsened over time in most left-dominant and left+ patients but appeared to improve in the 2 right-dominant patients with longitudinal follow-up. All groups showed progressive worsening of metabolism in both temporal lobes over time, with hypometabolism spreading from anteromedial to posterior temporal regions. However, the degree of temporal lobe asymmetry remained relatively constant over time. Conclusion: In SD, neurobehavioral features, especially disinhibition, are associated with right medial and lateral temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism.

AB - Introduction: Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time. Methods: Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up. Results: The right-dominant group performed worse on the NPI-Q and had a greater frequency of abnormal behaviors and more severe disinhibition compared to the left-dominant group. Performance on the NPI-Q and severity of disinhibition correlated with right medial and lateral, but not left, temporal lobe hypometabolism. Severity of abnormal behaviors worsened over time in most left-dominant and left+ patients but appeared to improve in the 2 right-dominant patients with longitudinal follow-up. All groups showed progressive worsening of metabolism in both temporal lobes over time, with hypometabolism spreading from anteromedial to posterior temporal regions. However, the degree of temporal lobe asymmetry remained relatively constant over time. Conclusion: In SD, neurobehavioral features, especially disinhibition, are associated with right medial and lateral temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism.

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Neurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study

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