The evolution of primary progressive apraxia of speech

作者：Keith A. Josephs, Joseph R. Duffy, Edythe A. Strand, Mary M. Machulda, Matthew L. Senjem, Jeffrey L. Gunter, Christopher G. Schwarz, Robert I. Reid, Anthony J. Spychalla, Val J. Lowe, Clifford R. Jack, Jennifer L. Whitwell · 发表于：Brain · 年份：2014 · DOI：10.1093/brain/awu223 · 被引用次数：175 · 研究领域：Neurobiology of Language and Bilingualism、Advanced Neuroimaging Techniques and Applications、Language Development and Disorders

Primary progressive apraxia of speech is a recently described neurodegenerative disorder in which patients present with an isolated apraxia of speech and show focal degeneration of superior premotor cortex. Little is known about how these individuals progress over time, making it difficult to provide prognostic estimates. Thirteen subjects with primary progressive apraxia of speech underwent two serial comprehensive clinical and neuroimaging evaluations 2.4 years apart [median age of onset = 67 years (range: 49-76), seven females]. All underwent detailed speech and language, neurological and neuropsychological assessments, and magnetic resonance imaging, diffusion tensor imaging and (18)F-fluorodeoxyglucose positron emission tomography at both baseline and follow-up. Rates of change of whole brain, ventricle, and midbrain volumes were calculated using the boundary-shift integral and atlas-based parcellation, and rates of regional grey matter atrophy were assessed using tensor-based morphometry. White matter tract degeneration was assessed on diffusion-tensor imaging at each time-point. Patterns of hypometabolism were assessed at the single subject-level. Neuroimaging findings were compared with a cohort of 20 age, gender, and scan-interval matched healthy controls. All subjects developed extrapyramidal signs. In eight subjects the apraxia of speech remained the predominant feature. In the other five there was a striking progression of symptoms that had evolved into a progress...