Developmental Foreign Accent Syndrome: Report of a New Case

Abstract

This paper presents the case of a 17-year-old right-handed Belgian boy with developmental FAS and comorbid developmental apraxia of speech (DAS). Extensive neuropsychological and neurolinguistic investigations demonstrated a normal IQ but impaired planning (visuo-constructional dyspraxia). A Tc-99m-ECD SPECT revealed a significant hypoperfusion in the prefrontal and medial frontal regions, as well as in the lateral temporal regions. Hypoperfusion in the right cerebellum almost reached significance. It is hypothesized that these clinical findings support the view that FAS and DAS are related phenomena following impairment of the cerebro-cerebellar network.

Mild Developmental Foreign Accent Syndrome and Psychiatric Comorbidity: Altered White Matter Integrity in Speech and Emotion Regulation Networks

“…In the vast majority of individuals with FAS the condition is acquired (AFAS) after brain damage or it emerges during the course of psychiatric illnesses (e.g., schizophrenia). Even less frequently, FAS occurs during speech-language development ( Mariën et al, 2009 ; Keulen et al, 2016a ). In these cases, toddlers develop the language (lexicon and grammar), but not the pronunciation (accent) that is peculiar to the community to which they belong ( Flege et al, 2006 ).…”

“…Since only three cases of DFAS have been reported up to now ( Mariën et al, 2009 ; Keulen et al, 2016a ), there is no information about the interaction between the neural systems underpinning speech and behavior in DFAS. Data from a well-studied developmental speech disorder (stuttering) indicates that concomitant disorders (e.g., neuroticism, anxiety, depression, and emotional/behavioral problems) depend on a complex interaction between biological (genetics), psychological vulnerabilities, temperament, cognitive styles, and familiar and peer influences (see Bleek et al, 2012 ; Alm, 2014 ; Gunn et al, 2014 ; Iverach and Rapee, 2014 ).…”

“…However, not many studies have analyzed the interaction between prosodic and segmental errors. Although the number of subjects with DFAS described until now is scant ( Mariën et al, 2009 ; Keulen et al, 2016a ), it remains to be explored whether or not the same linguistic heterogeneity described in AFAS can be observed in developmental cases. It is hypothesized that the results of the present study would provide further information on this issue.…”

“…Information on the emotional consequences of AFAS is scarce ( Miller et al, 2011 ; Moreno-Torres et al, 2013 ) and data reported on DFAS revealed normal behavior ( Mariën et al, 2009 ; Keulen et al, 2016a ). Therefore, since the two subjects included in this study complained of cognitive failure and psychiatric symptoms, a second aim our study was to identify the type and severity of these complaints and their potential relationship to FAS.…”

“…Brain imaging studies on DFAS have revealed normal gross anatomy but decreased activity of several components of the large-scale bilateral speech production network including the cerebellum, basal ganglia, and prefrontal-medial frontal regions ( Mariën et al, 2009 ; Keulen et al, 2016a ), a variety of regions which are also involved in cases of AFAS (see Carbary et al, 2000 ; Scott et al, 2006 ; Katz et al, 2012 ; Moreno-Torres et al, 2013 ; Tomasino et al, 2013 , for reviews). Other affected regions in DFAS (left thalamus and lateral temporal regions and occipital cortex bilaterally; Mariën et al, 2009 ; Keulen et al, 2016a ) have also been described in cases of AFAS, yet these areas are not integral components of the speech production network ( Wise et al, 1999 ; Riecker et al, 2005 ; Sörös et al, 2006 ; Ackermann and Riecker, 2010 ). This latter finding increases the likelihood that abnormal activity in such regions could be the consequence of disorders other than DFAS, but which may coexist with it.…”