Luzzi S, Viticchi G, Piccirilli M, Fabi K, Pesallaccia M, Bartolini M, Provinciali L, Snowden J S
Department of Neuroscience, University of Ancona, Italy.
J Neurol Neurosurg Psychiatry. 2008 Jan;79(1):79-81. doi: 10.1136/jnnp.2006.113365. Epub 2007 Jul 17.
Foreign accent syndrome (FAS) is a rare speech disorder characterised by the emergence of a new accent, perceived by listeners as foreign. FAS has usually been described following focal brain insults, such as stroke. We describe the unusual case of a woman presenting with FAS as the earliest symptom of progressive degenerative brain disease. At presentation, she showed no language or other cognitive impairment, and functional and structural brain imaging were normal. Follow-up 1 year later revealed the emergence of mild expressive language problems. Repeat functional neuroimaging showed mild hypoperfusion of the perisylvian speech area of the left hemisphere, and structural imaging showed mild left perisylvian atrophy. We interpret the case as an unusual presentation of primary progressive non-fluent aphasia. The case provides further evidence of the variable and circumscribed nature of the clinical presentation of focal cerebral degeneration.
外国口音综合征（FAS）是一种罕见的言语障碍，其特征是出现一种新的口音，听众会认为这是外国口音。FAS通常在局灶性脑损伤（如中风）后出现。我们描述了一名女性的罕见病例，她以FAS作为进行性退行性脑疾病的最早症状。就诊时，她没有语言或其他认知障碍，脑功能和结构成像均正常。1年后的随访显示出现了轻度的表达性语言问题。重复的功能神经成像显示左半球外侧裂周围言语区轻度灌注不足，结构成像显示左侧外侧裂周围轻度萎缩。我们将该病例解释为原发性进行性非流利性失语的一种不寻常表现。该病例进一步证明了局灶性脑变性临床表现的多变性和局限性。
