Control/Tracking Number: 2026-A-532-AAIC

Foreign Accent Syndrome As An Early Clinical Marker Of Frontotemporal Neurodegeneration: A Case Report:


Author Block: Urdiales Laura¹, Manzano Sagrario², Garcia Pilar³, Hualde Amaia⁴, Orrego Shenda¹.
¹Psychiatry, Infanta Leonor Hospital, Madrid, Spain, ²Neurology, Infanta Leonor Hospital, Madrid, Spain, ³Head of Nuclear Medicine, Getafe Hospital, Madrid, Spain, ⁴Nuclear Medicine, Gregorio Marañon Hospital, Madrid, Spain.

Abstract:
Background: Foreign Accent Syndrome (FAS) is a rare motor speech disorder in which patients develop speech patterns perceived as foreign despite no prior exposure to that accent. Although classically linked to stroke or traumatic brain injury, FAS has also been described in neurodegenerative diseases, including primary progressive aphasia (PPA). Its presence may complicate the diagnostic process, particularly in differentiating Alzheimer’s disease (AD) from frontotemporal dementia (FTD). Understanding FAS within neurodegenerative contexts may aid in recognizing atypical presentations and improving diagnostic accuracy.
Methods: A comprehensive clinical evaluation and longitudinal follow-up were conducted, including neurological examination, standardized cognitive testing, laboratory studies, and structural and functional neuroimaging (MRI, 18F FDG PET/TC scan and amyloid PET (florbetaben)). Collateral information was gathered from family members to assess behavioral changes and functional decline.
Results: A 64-year-old male with ischemic heart disease with preserved ejection fraction and iron-deficiency anemia presented with a progressive mixed speech and language disorder. Symptoms began with stuttering and semantic alterations, progressing to fragmented speech with frequent phonemic paraphasias and a clear Foreign Accent Syndrome. Relatives reported impaired language comprehension, impulsivity, aggressive behavior, reduced autonomy in daily activities, and a 20-kg weight loss. Neurological examination was normal. Cognitive testing showed preserved scores on the MMSE (29/30), clock drawing test (10/10), and phototest (30), with intact praxis and graphomotor abilities, and no abnormal movements or parkinsonian signs. Laboratory tests—including vitamin B12, folate, thyroid hormones, and syphilis/HIV serologies—were unremarkable. Brain MRI showed marked temporal atrophy and also frontal and left parietal. The 18F FDG PET/TC scan revealed a pattern consistent with frontotemporal neurodegeneration. Amyloid PET showed no beta-amyloid deposition, supporting a diagnosis of frontotemporal dementia. The clinical, neuropsychological, and imaging findings led to the diagnosis of primary progressive aphasia within the FTD spectrum.
Conclusion: This case illustrates FAS as a potential early manifestation of frontotemporal neurodegeneration and the differential diagnosis between Alzheimer´s (logopenic variant) and progressive aphasias.Awareness of FAS may improve early recognition of atypical language-predominant dementias.