A Dextral Primary Progressive Aphasia Patient with Right Dominant Hypometabolism and Tau Accumulation and Left Dominant Amyloid Accumulation

Young Kyoung Jang, Seongbeom Park, Hee Jin Kim, Hanna Cho, Chul Hyoung Lyoo, Sang Won Seo, Duk L. Na

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3 Citations (Scopus)

Abstract

Background: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). Patients and Methods: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA. Unlike other LPA patients, however, this patient showed a right hemisphere predominant glucose hypometabolism and tau accumulation and a left hemisphere predominant amyloid deposition. The right-handed patient presented with comprehension difficulty followed by problems naming familiar objects. This isolated language problem had deteriorated rapidly for 2 years, followed by memory difficulties and impairment of daily activities. Using a Korean version of the Western Aphasia Battery, aphasia was consistent with a severe form of Wernicke's aphasia. According to the brain magnetic resonance imaging and 18F-fludeoxyglucose positron emission tomography results, right hemisphere atrophy and hypometabolism, more predominant on the right hemisphere than the left, were apparent despite the fact that Edinburgh Handedness Questionnaire scores indicated strong right-handedness. On Pittsburgh compound B-PET, amyloid accumulation was asymmetrical with the left hemisphere being more predominant than the right, whereas 18F-T807-PET showed a right dominant tau accumulation. Conclusions: This is the first report of atypical PPA, in which the patient exhibited crossed aphasia and asymmetrical amyloid accumulation.

Original languageEnglish
Pages (from-to)78-86
Number of pages9
JournalCase Reports in Neurology
Volume8
Issue number1
DOIs
Publication statusPublished - 2016 Jan 1

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Primary Progressive Aphasia
Aphasia
Patient Rights
Amyloid
Functional Laterality
Primary Progressive Nonfluent Aphasia
Language
Wernicke Aphasia
Frontotemporal Dementia
Aptitude
Positron-Emission Tomography
Cognition
Atrophy
Magnetic Resonance Imaging
Glucose
Brain

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

Cite this

Jang, Young Kyoung ; Park, Seongbeom ; Kim, Hee Jin ; Cho, Hanna ; Lyoo, Chul Hyoung ; Seo, Sang Won ; Na, Duk L. / A Dextral Primary Progressive Aphasia Patient with Right Dominant Hypometabolism and Tau Accumulation and Left Dominant Amyloid Accumulation. In: Case Reports in Neurology. 2016 ; Vol. 8, No. 1. pp. 78-86.
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A Dextral Primary Progressive Aphasia Patient with Right Dominant Hypometabolism and Tau Accumulation and Left Dominant Amyloid Accumulation. / Jang, Young Kyoung; Park, Seongbeom; Kim, Hee Jin; Cho, Hanna; Lyoo, Chul Hyoung; Seo, Sang Won; Na, Duk L.

In: Case Reports in Neurology, Vol. 8, No. 1, 01.01.2016, p. 78-86.

Research output: Contribution to journalArticle

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T1 - A Dextral Primary Progressive Aphasia Patient with Right Dominant Hypometabolism and Tau Accumulation and Left Dominant Amyloid Accumulation

AU - Jang, Young Kyoung

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AU - Kim, Hee Jin

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AU - Lyoo, Chul Hyoung

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AU - Na, Duk L.

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N2 - Background: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). Patients and Methods: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA. Unlike other LPA patients, however, this patient showed a right hemisphere predominant glucose hypometabolism and tau accumulation and a left hemisphere predominant amyloid deposition. The right-handed patient presented with comprehension difficulty followed by problems naming familiar objects. This isolated language problem had deteriorated rapidly for 2 years, followed by memory difficulties and impairment of daily activities. Using a Korean version of the Western Aphasia Battery, aphasia was consistent with a severe form of Wernicke's aphasia. According to the brain magnetic resonance imaging and 18F-fludeoxyglucose positron emission tomography results, right hemisphere atrophy and hypometabolism, more predominant on the right hemisphere than the left, were apparent despite the fact that Edinburgh Handedness Questionnaire scores indicated strong right-handedness. On Pittsburgh compound B-PET, amyloid accumulation was asymmetrical with the left hemisphere being more predominant than the right, whereas 18F-T807-PET showed a right dominant tau accumulation. Conclusions: This is the first report of atypical PPA, in which the patient exhibited crossed aphasia and asymmetrical amyloid accumulation.

AB - Background: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). Patients and Methods: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA. Unlike other LPA patients, however, this patient showed a right hemisphere predominant glucose hypometabolism and tau accumulation and a left hemisphere predominant amyloid deposition. The right-handed patient presented with comprehension difficulty followed by problems naming familiar objects. This isolated language problem had deteriorated rapidly for 2 years, followed by memory difficulties and impairment of daily activities. Using a Korean version of the Western Aphasia Battery, aphasia was consistent with a severe form of Wernicke's aphasia. According to the brain magnetic resonance imaging and 18F-fludeoxyglucose positron emission tomography results, right hemisphere atrophy and hypometabolism, more predominant on the right hemisphere than the left, were apparent despite the fact that Edinburgh Handedness Questionnaire scores indicated strong right-handedness. On Pittsburgh compound B-PET, amyloid accumulation was asymmetrical with the left hemisphere being more predominant than the right, whereas 18F-T807-PET showed a right dominant tau accumulation. Conclusions: This is the first report of atypical PPA, in which the patient exhibited crossed aphasia and asymmetrical amyloid accumulation.

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