Primary progressive aphasia: six questions in search of an answer

被引：18

作者：

Belder, Christopher R. S. ^{[1
,2
,3
]}

Marshall, Charles R. ^{[4
]}

Jiang, Jessica ^{[1
]}

Mazzeo, Salvatore ^{[1
,5
]}

Chokesuwattanaskul, Anthipa ^{[1
,6
,7
]}

Rohrer, Jonathan D. ^{[1
]}

Volkmer, Anna ^{[1
]}

Hardy, Chris J. D. ^{[1
]}

Warren, Jason D. ^{[1
]}

机构：

[1] UCL, UCL Queen Sq Inst Neurol, Dementia Res Ctr, Dept Neurodegenerat Dis, 8-11 Queen Sq, London WC1N 3BG, England

[2] UCL, UK Dementia Res Inst UCL, UCL Queen Sq Inst Neurol, London, England

[3] Univ Adelaide, Adelaide Med Sch, Adelaide, SA, Australia

[4] Queen Mary Univ London, Wolfson Inst Populat Hlth, Prevent Neurol Unit, London, England

[5] Univ Florence, Azienda Osped Univ Careggi, Dept Neurosci Psychol Drug Res & Child Hlth, Florence, Italy

[6] King Chulalongkorn Mem Hosp, Dept Internal Med, Div Neurol, Thai Red Cross Soc, Bangkok, Thailand

[7] Chulalongkorn Univ, Fac Med, Cognit Clin & Computat Neurosci Res Unit, Bangkok, Thailand

来源：

JOURNAL OF NEUROLOGY | 2024年 / 271卷 / 02期

基金：

美国国家卫生研究院; 英国医学研究理事会; 英国惠康基金;

关键词：

Primary progressive aphasia; Semantic dementia; Logopenic aphasia; Frontotemporal dementia; Alzheimer's disease; FRONTOTEMPORAL LOBAR DEGENERATION; LOGOPENIC-VARIANT; SEMANTIC DEMENTIA; DISEASE PROGRESSION; ALZHEIMERS-DISEASE; COHERENT CONCEPTS; TEMPORAL-LOBE; LANGUAGE; SPEECH; SUBTYPES;

D O I：

10.1007/s00415-023-12030-4

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Here, we review recent progress in the diagnosis and management of primary progressive aphasia-the language-led dementias. We pose six key unanswered questions that challenge current assumptions and highlight the unresolved difficulties that surround these diseases. How many syndromes of primary progressive aphasia are there-and is syndromic diagnosis even useful? Are these truly 'language-led' dementias? How can we diagnose (and track) primary progressive aphasia better? Can brain pathology be predicted in these diseases? What is their core pathophysiology? In addition, how can primary progressive aphasia best be treated? We propose that pathophysiological mechanisms linking proteinopathies to phenotypes may help resolve the clinical complexity of primary progressive aphasia, and may suggest novel diagnostic tools and markers and guide the deployment of effective therapies.

引用

页码：1028 / 1046

页数：19

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