Foreign accent syndrome as the initial sign of primary progressive aphasia

Abstract

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.

A Lilt of Finland in Worcester, Massachusetts: A Case of Foreign Accent Syndrome

“…4 However, other reports have disclosed dissenting opinions amongst listeners as to "where" the accent would have originated. 6,8 In our patient, one listener thought the accent sounded "Russian"; several others, including the patient, believed it to be "Norwegian." In any case, his speech rate was thought to be slowed, intonation flatter (than American or Massachusetts-dialect English), with unexpected sentence accentations.…”

“…Usually occurring in consequence to some brain dysfunction, often stroke, two distinct types of FAS are described: [1] acquisition of an accent the patient was previously unfamiliar with or unexposed to, the unlearned foreign accent, and [2] the reversion to a previously learned accent. In addition to ischemic and hemorrhagic stroke, [2][3][4][5] traumatic brain injury, 3,6 multiple sclerosis, 7 and the herald of primary progressive aphasia 8 have all been reported as causes of the foreign accent syndrome.…”

Change of Accent as an Atypical Onset of non Fluent Primary Progressive Aphasia

“…Disrupted prosody is often found in advanced PPA-NFV as the consequence of articulation disturbances. Only one case of an Italian patient showing dysprosody as early onset of PPA-NFV has been reported [26]. Three years before the onset of PPA-NFV, this patient presented a speech disorder characterized by the foreign accent syndrome (FAS), that is a linguistic prosody disorder in which a new accent is perceived by listeners as foreign.…”

“…Regarding the PPA-NFV, a recent study by Luzzi et al [26] reported an atypical onset of the foreign accent syndrome (FAS). These authors reported a case of an Italian woman presenting a progressive change in her accent, so that listeners perceived her as a foreigner.…”

Multidisciplinary Assessment and Diagnosis of Conversion Disorder in a Patient with Foreign Accent Syndrome

“…The condition is often preceded or accompanied by other communication disorders such as aphasia, dysarthria, and apraxia of speech [18]. More recently, FAS has been associated with a variety of neurological illnesses, including multiple sclerosis [19,20], primary progressive aphasia [21], cerebellar hypoperfusion [22], and metastatic breast cancer [23]. Several case reports of patients with FAS and psychiatric illness without a known brain lesion have also appeared, including patients with bipolar disease [24], psychosis [25,26], psychogenic illness [27], and conversion disorder [28,29].…”