FOREIGN ACCENT SYNDROME: WHAT CAN THE SLT EXPECT TO SEE IN CLINICAL PRACTICE?

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: WHAT CAN THE SLT EXPECT TO SEE IN CLINICAL PRACTICE? Dr. Stefanie Keulen Prof. Dr. Philippe Paquier Prof. Dr. Jo Verhoeven Prof. Dr. Peter Mariën stefanie.keulen@vub.

1 : WHAT CAN THE SLT EXPECT TO SEE IN CLINICAL PRACTICE? Dr. Stefanie Keulen Prof. Dr. Philippe Paquier Prof. Dr. Jo Verhoeven Prof. Dr. Peter Mariën Université Libre de Bruxelles (BE) Vrije Universiteit Brussel (BE) University of Groningen (NL) Université Libre de Bruxelles (BE) Vrije Universiteit Brussel (BE) Universiteit Antwerpen (BE) Universiteit Antwerpen (BE) City University London (UK) Vrije Universiteit Brussel (BE) ZNA General Hospital, Antwerp (BE)

DEFINITION FAS can be described as a motor speech disorder that causes patients to speak their mother tongue with an accent that is perceived as

report: Georg Monrad-Krohn (1947) - Whitaker (1982): operational criteria for the diagnosis of FAS > anecdotal > +ling. characteris.

2 DEFINITION FAS can be described as a motor speech disorder that causes patients to speak their mother tongue with an accent that is perceived as non-native (by their immediate environment, by medical staff) - First report: Pierre Marie (1907) - Second report: Arnold Pick (1919) - Third report: Georg Monrad-Krohn (1947) - Whitaker (1982): operational criteria for the diagnosis of FAS > anecdotal > +ling. characteris. > + neuroanatomy ; + psychological impact Monrad Krohn s patient Astrid L.: FAS after severe hemorrhagic stroke affecting the fronto-temporoparietal region (1947)

3 WHITAKER S OPERATIONAL CRITERIA 1) The accent is considered by the patient, by acquaintances and by the investigator, to sound foreign 2) It is unlike the patient s native dialect before the cerebral insult 3) It is clearly related to central nervous system damage (as opposed to an hysteric reaction, if such exist) 4) And there is no evidence in the patient s background of being a speaker of a foreign language (this is not like cases of polyglot aphasia). (Whitaker 1982, pp )

4 PROBLEMS FACING WHITAKER S CRITERIA Problematic retrospective interpretation of Whitaker s criteria (1982) Problematic prospective interpretation of FAS: 1. Subjectivity of the defining criteria 2. Not just damage to CNS 3. Bilingual and polyglot speakers

5 TYPOLOGY Verhoeven and Mariën (2010): introduction of taxonomic distinction between: Neurogenic FAS (acquired & developmental) Psychogenic FAS Mixed FAS Neurogenic is most frequently reported Psychogenic (or functional?) is increasingly reported in the past decades

6 TYPOLOGY Systematic review 112 cases published between (only authentic!): Neurogenic (n=87) Psychogenic/functional FAS (n=18) Overview of neurogenic / psychogenic cases published in the past decades Neurogenic FAS Psychogenic/functional FAS

7 Stroke (53.7%) FOREIGN ACCENT SYNDROME NEUROGENIC FAS 77.68% acquired, neurogenic FAS Different etiologies reported, though most common = stroke (almost 54% of all cases) Stroke-based FAS or vascular FAS (n=60) allows for lesion localization Trauma (13.39%) Tumor (2.68%) Multiple sclerosis (2.68%) Primary Progressive Aphasia (1.79%) Paediatric Autoimmune Neuropsychiatric Disorder associated with Streptococcus (0.89%) Vasculitis (0.89%) Garcin's syndrome (0.89%) Vascular dementia (0.89%)

8 NEUROGENIC FAS Stroke-based FAS (n=60): Over half of the lesion locations comprised (structural): Basal ganglia Frontal lobe (L) (esp. BA 4 & 6) Parietal lobe (L) But diaschisis (SPECT) Reported lesion locations Basal ganglia (53.3%) Frontal lobe (L) (46.7%) Parietal lobe (L) (33.3%) Insular region (L) (21.67%) Temporal region (L) (16.67%) Pons (11.67%) Frontal operculum (8.33%) Temporal lobe (R) (8.33%) Cerebellum (5%) Frontal lobe (R) (5%) Parietal lobe (R) (5%) Corpus Callosum (3.3%) Insular region (R) (3.3%)

9 40 FOREIGN ACCENT SYNDROME NEUROGENIC FAS Insights into stroke-based FAS (n=60) stroke Demographics & comorbid speech and language disorders: Significantly more women than men (>< stroke incidence?) First report: relatively young age: average age of 51 years (median = 53, SD= 12.8 y) (cfr. functional cases?) 41.7% initially mute (implication SMA, Broca, insula?) FAS-onset: usually in the acute phase (63.3%) Acute phase (up until 3 weeks poststroke) Lesion phase (3 weeks - 3 months post-stroke) Chronic phase (> 3 months poststroke) FAS AoS dysarthria aphasia N=60 Onset period not mentioned

10 NEUROGENIC FAS Insights into stroke-based FAS (n=60) stroke «Crossed FAS» incidence is quite high: 6.67% Cognition: Cognitive impairments were identified in 15% of all stroke cases (n=9/60) (neurocognitive exams were only reported in 45% of the stroke patients). 9 patients with cognitive deficits (6/9: aphasic!) Acalculia and working memory Patients with frontoparietal, cerebellar and basal ganglia lesions

11 NEUROGENIC FAS Insights into stroke-based FAS (n=60) stroke Remission: Almost 20% of all stroke patients remitted (1d - 3y) Most of these patients (66.6%) also had comorbid speech or language disorders 30% of these patients had persisting FAS (lesion phase) Aphasia: severity, than remission chances Wade et al. (1986): 18% remission, if aphasia is persistent in lesion phase Most of these patients had focal damage located in the frontal lobe, in or near (pre)central gyrus, inferior frontal gyrus or pons (9/12) Only three had basal ganglia involvement

12 WHAT DOES THE TYPICAL NEUROGENIC FAS PATIENT LOOK LIKE? Monolingual, right-handed female, early fifties Stroke, affecting basal ganglia, SMA, (pre)motor cortex Mute initially, with FAS emerging within three weeks after stroke Often accompanied by other speech disorders ( which may initially cover up the accent) Patient is usually aware of the accent (reports of anosognosia are rare) Total remission: 20% of the patients

13 PSYCHOGENIC SPEECH DISORDERS Diagnostic process: psychogenic speech disorders Some pitfalls and critiques: Speech disorder + psychological disturbance always psychogenic speech disorder functional? Exclusion criteria Requires elaborate knowledge on what are accepted neurological manifestations (clinical experience) What characterizes the psychogenic FAS patient?

14 PSYCHOGENIC (?) Etiologies Demographics: More women than men (3.5:1) (preponderance women psychiatric disturbances? - WHO) Mean age is quite young: 45y (Median=45y 6m; SD=11y) & falls into the age range in which psychiatric disturbances are usually withheld Conversion Disorder (33.33%) Schizophrenia (16.67%) Obsessive Compulsive Disorder (OCD) (11.11%) Neurosis (5.56%) Bipolar Disorder (5.56%) Post-traumatic Stress Disorder (5.56%) Mania (5.56%) Suspected Conversion Disorder (5.56%) Depression and suicidal ideation (5.56%)

15 PSYCHOGENIC (?) Etiologies Demographics: Conversion disorder : problematic concept SLT will most frequently encounter psychogenic speech/language disorder in context of conversion disorder (Duffy 2016) Duffy (2016); markers: Sensory or voluntary motor system Atypical/bizarre quality to the complaint History of frequent, minor health problems/current stress Presence of model for symptoms Symbolic significance La belle indifférence Primary or secondary gain Reversible Conversion Disorder (33.33%) Schizophrenia (16.67%) Obsessive Compulsive Disorder (OCD) (11.11%) Neurosis (5.56%) Bipolar Disorder (5.56%) Post-traumatic Stress Disorder (5.56%) Mania (5.56%) Suspected Conversion Disorder (5.56%) Depression and suicidal ideation (5.56%)

16 PSYCHOGENIC (?) Comorbid speech and language disorders: - For 7/18 patients speech was qualified as telegraphic or agrammatic-like (in absence of aphasia) - Two patients were initially mute Psychopathology: In cases were psychopathology was not already established (n=5), the hypothesis of psychiatric disorder was reported to be formally assessed in 5 studies (MMPI, BDI, DIS-Q, ) three studies reported to have included malingering tests in their protocol (one: Cottingham & Boone, 2011: inconclusive )

17 PSYCHOGENIC (?) Cognition: Deficits concerned mnestic functioning, attention and executive functioning, processing speed, intelligence, fine motor skills, and visuospatial skills Remission: mostly reported in patients with conversion disorder ( cogniform disorder?, Delis & Wetter, 2007) Only 3 study included cognitive malingering tests (test of memory malingering: TOMM, Tombaugh, 1996) 38.89% of the psychogenic/functional patients remitted 4 patients: remission pharmacologically induced 3 patients: spontaneous remission

«TRUELY» FUNCTIONAL OR PSYCHOGENIC? Duffy (2016, p. 381): questions that can provide diagnostic assistance, as to diagnose patient with psychogenic disorder NEUROLOGICAL?

18 «TRUELY» FUNCTIONAL OR PSYCHOGENIC? Duffy (2016, p. 381): questions that can provide diagnostic assistance, as to diagnose patient with psychogenic disorder NEUROLOGICAL? All cases demonstrate neurologically inexplicable symptoms Unaccompanied by typical «comorbid» neurological speech and/or language disorders (dysarthria, AoS, aphasia) Accent-onset is either associated with an exarcerbation, or is situated months after incident associated with FAS (>< acute onset in stroke-based FAS) Symptom evolution: «fluctuating» (not only speech, also other neurological symptoms: e.g. immobility of legs chair test, give-way weakness, ) Dissociations in terms of linguistic modalities (writing vs. speaking - in absence of aphasia) «automatic» speech versus complex speech ORAL MECHANISM? Not consistently tested 5 patients were tested: two had difficulties with oral praxis upon testing (BDAE) DISTRACTIBLE? Reports indicate that speech is subject to distractibility Emotional breakthroughs induce accent changes ( and usually for the better) Possibility to consciously manipulate accent (voluntary accent change)

19 «TRUELY» FUNCTIONAL OR PSYCHOGENIC? FATIGUE? «Most MSD s do not fatigue dramatically over the course of examination» (Duffy 2013, p. 337) Accent is almost without exception reported to become more exaggerated when fatigue increases (cfr. muscle tone) REVERSIBLE? Better prognosis, reported fluctuations Schizophrenia, OCD, bipolar disorder: accent remitted after pharmacological aid. Fluctuations are reported in all patients (not necessarily reported with fatigue)

20 WHAT DOES THE TYPICAL PSYCHOGENIC/FUNCTIONAL FAS PATIENT LOOK LIKE? Monolingual, right-handed female, mid-forties Often there is a history of psychopathology Formal psychodiagnostic tests to confirm presence of psychopathology FAS onset is related to exacerbation in context of psychiatric disorders (e.g. schizophrenia), and in absence of a confirmed psychopathology: delayed onset vis-à-vis «traumatic event» Reported comorbid language symptoms are pseudo-agrammatism, jargon-speech, language mixing and switching, change of register Total remission: 39% of the patients (within follow-up period)

21 THANK YOU FOR YOUR ATTENTION! QUESTIONS? REMARKS?... S?:

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