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Organic or Functional? A New Case of Foreign Accent Syndrome
ORGANIC OR FUNCTIONAL? A NEW CASE OF FOREIGN ACCENT SYNDROME J.M. Gurd1, J.S. Coleman2, A. Costello3 and J.C. Marshall1 (1Neuropsychology Unit, University Department of Clinical Neurology, Oxford OX2 6HE, UK; 2Phonetics Laboratory, University of Oxford OX1 2JF, UK; 3Neuropsychology Department, Regional Neuroscience Centre, Mapother House, De Crespigny Park, London SE5 9RS)
INTRODUCTION We describe a patient who presented with a French-sounding accent, although she only spoke English. In previous cases of foreign accent syndrome (FAS), neurological causes were clearly identifiable (Moen, 2000). Here the diagnostic picture is unclear. The case illustrates that voice disorders are: “a prototype of disorders which reflect the intricate interplay of emotional, cognitive and physiological functions” (Matas, 1991). CASE HISTORY This 47 year old right-handed lady had been admitted to hospital in November 1999 following the sudden onset of aphasia and headache. She had normal carotid Dopplers, a normal MRI scan and a CT scan showing a small high signal lesion in the cerebellar vermis. Within one month post-onset, she had developed a French-sounding accent, and shortly thereafter, a bizarre gait. On reexamination, tone, power, coordination and reflexes in her arms and legs were normal. Nonetheless, her gait disorder remained. A repeat MRI scan (May 2000) showed several small foci of T2 hyperintensity in the peripheral white matter of both frontal lobes, the left inferior frontal corona radiata (see Figure 1) and the left thalamus; several neuroradiologists judged these to be non-specific, given the patient’s age. EEG showed recurrent sharp and slow waves including sharp transients over the left temporal electrodes. There was no history of epilepsy. The cerebrospinal fluid showed oligoclonal bands. Neuropsychological examination showed verbal and performance IQs, shortand long-term memory, naming, reading and spelling skills which were within normal limits. The physicians doubted whether all of the patient’s symptoms could be accounted for organically. Cortex, (2001) 37, 715-718
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Fig. 1 – Axial FLAIR sequence MRI scan (parallel to the AC-PC line), showing areas of high intensity signal in the left inferior frontal corona radiata. This image is left-right reversed.
FOLLOW-UP EXAMINATIONS In December 1999, the patient contacted the Neuropsychology Unit (Oxford), having discovered our interest in FAS on the web (Gurd et al., 1988). She was not depressed, but her anxiety score on the Hospital Anxiety and Depression Scale was borderline. There was severe impairment of oral agility (from the B.D.A.E.): Nonverbal agility = 4/12; verbal agility = 7/12. The patient provided audio recordings made before the onset of her speech problems, to confirm her North Yorkshire accent. Phonetic analysis was performed on an “audio diary” recorded by the patient between December 1999 and March 2000 and on studio recordings we made in April 2000. Her French-like pronunciation was largely due to three specific disturbances in her speech, together with one aspect of her native English dialect:
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(1) Abnormal, “syllable-timed” rhythm: each syllable has approximately equal duration. This is characteristic of French, whereas normal English has more variation in syllable duration, according to the distribution of stress in the sentence, and the composition of the syllables in terms of their constituent vowels and consonants. This predates the appearance of other aspects of a French-sounding accent in the early recordings, and persisted at 5 months post-onset. (2) Exceptionally high pitch is sometimes placed on intonationally prominent syllables. (3) Her pronunciation of /h/ is very effortful: produced with abnormally loud laryngeal friction, or avoided altogether. The latter problem combined with impairment of pitch control suggests that poor control of the larynx is implicated, although the rhythm problem is not due to this impairment. Syllable-timed rhythm, /h/-dropping, and /h/-hyperarticulation are all characteristics of French pronunciation, while the pitch exaggerations contribute to the overall ‘foreignness’ of her speech. Also, the patient’s English diphthongs are actually quite monophthongal, as she originally had a North Yorkshire accent. This aspect of her undisturbed speech adds to the list of French-like features. As in some previous cases of FAS, there was no persisting aphasia or dysarthria (Takayama et al., 1993). There were, however, some errors characteristic of apraxia of speech (Varley and Whiteside, 2001): absence of lip rounding, the production of both vowels and consonants of excessively long duration, and, on rare occasions, an impairment of stress placement, even on highly familiar words (e.g. DIvision, someONE). DISCUSSION The patient displays an acquired speech disorder, some aspects of which cause the listener to perceive her speech as somehow ‘foreign’. A number of these features are sufficiently ‘French-like’ for the listener to assimilate her accent to that characteristic of a speaker of that language: French accents are well-known to English listeners and thus form a category to which the patient’s speech can readily be assigned (Kurowski et al., 1996; Carbary et al., 2000; Dankovicova et al., 2001). The following questions arise: (a) Is the entire range of symptoms explicable organically by the small lesions and neurological findings (e.g. EEG & CSF)? (b) Is there a mixture of organic and functional symptoms? Is the speech disorder of organic origin, but the bizarre gait a functional overlay (Keane, 1989)? It is perhaps significant that the abnormality of gait recovered without medical intervention? (c) Are the organic symptoms functionally exaggerated? Minor neurological abnormalities may underlie both the speech and the gait disorder, but may not be sufficiently marked to account for the severity of these symptoms. A catastrophic cascade may have been provoked by a minor brain injury. (d) Is the entire range of symptoms functional? The case notes reported that none of the neurological
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J.M. Gurd and Others
signs (including CT, MRI, EEG) were clinically significant. Conversion disorders can mimic virtually all neurological conditions (Halligan et al., 2001). However, this seems unlikely in view of the findings of the neurological investigations even though they were somewhat equivocal. Clinical lore suggests that medical students frequently believe themselves to be suffering from whatever disease they are currently studying. Patients could always have done likewise, but would have needed access to a medical library. Now patients can put themselves in the same position as medical students at the click of a mouse. Were some aspects of her foreign accent in any way provoked by our patient’s knowledge of the clinical category of FAS? If so, what are the more general implications for the reliable (and valid) diagnosis of `neurological’ disease in the age of the internet (Khan et al., 2000)? Acknowledgements: JMG and JCM are supported by the Medical Research Council. We thank Drs. J. Barnaby, G.J. Saldanha, and Y. Zoukos for data. We are also grateful to Dr. P. Anslow, B. Mastrude, Dr. N. Brooks, and Dr. G.R. Fink for their comments on the case. REFERENCES CARBARY TJ, PATTERSON JP and SNYDER PJ. Foreign accent syndrome following a catastrophic second injury: MRI correlates, linguistic and voice pattern analyses. Brain and Cognition, 43: 78-85, 2000. DANKOVICOVA J, GURD JM, MARSHALL JC, COLEMAN JS, MACMAHON MKC, STUART-SMITH J and SLATER A. Aspects of non-native pronunciation in a case of altered accent following stroke (foreign accent syndrome). Clinical Linguistics and Phonetics (in press). GURD JM, BESSELL NJ, BLADON RAW and BAMFORD JM. A case of foreign accent syndrome, with follow-up clinical, neuropsychological and phonetic descriptions. Neuropsychologia, 26: 237-251, 1988. HALLIGAN PW, BASS C and MARSHALL JC (eds). Contemporary Approaches to the Study of Hysteria: Clinical and Theoretical Perspectives. Oxford: Oxford University Press, 2001. KEANE JR. Hysterical gait disorders: 60 cases. Neurology, 39: 586-589, 1989. KHAN I, FAYAZ I, RIDGLEY J and WENNBERG R. Factitious clock drawing and constructional apraxia. Journal of Neurology, Neurosurgery and Psychiatry, 68: 106-107, 2000. KUROWSKI KM, BLUMSTEIN SE and ALEXANDER M. The foreign accent syndrome: A reconsideration. Brain and Language, 54: 1-25, 1996. MATAS M. Psychogenic voice disorders: literature review and case report. Canadian Journal of Psychiatry, 36: 363-365, 1991. MOEN I. Foreign accent syndrome: A review of contemporary explanations. Aphasiology, 14: 5-15, 2000. TAKAYAMA Y, SUGISHITA M, KIDO T, OGAWA M and AKIGUCHI I. A case of foreign accent syndrome without aphasia caused by a lesion of the left precentral gyrus. Neurology, 43: 1361-1363, 1993. VARLEY RA and WHITESIDE SP. What is the underlying impairment in acquired apraxia of speech? Aphasiology, 15: 39-49, 2001. Dr. J.M. Gurd, Neuropsychology Unit, University Department of Clinical Neurology, The Radcliffe Infirmary, Woodstock Road, Oxford OX2 6HE, UK. E-mail: [email protected].
INTRODUCTION We describe a patient who presented with a French-sounding accent, although she only spoke English. In previous cases of foreign accent syndrome (FAS), neurological causes were clearly identifiable (Moen, 2000). Here the diagnostic picture is unclear. The case illustrates that voice disorders are: “a prototype of disorders which reflect the intricate interplay of emotional, cognitive and physiological functions” (Matas, 1991). CASE HISTORY This 47 year old right-handed lady had been admitted to hospital in November 1999 following the sudden onset of aphasia and headache. She had normal carotid Dopplers, a normal MRI scan and a CT scan showing a small high signal lesion in the cerebellar vermis. Within one month post-onset, she had developed a French-sounding accent, and shortly thereafter, a bizarre gait. On reexamination, tone, power, coordination and reflexes in her arms and legs were normal. Nonetheless, her gait disorder remained. A repeat MRI scan (May 2000) showed several small foci of T2 hyperintensity in the peripheral white matter of both frontal lobes, the left inferior frontal corona radiata (see Figure 1) and the left thalamus; several neuroradiologists judged these to be non-specific, given the patient’s age. EEG showed recurrent sharp and slow waves including sharp transients over the left temporal electrodes. There was no history of epilepsy. The cerebrospinal fluid showed oligoclonal bands. Neuropsychological examination showed verbal and performance IQs, shortand long-term memory, naming, reading and spelling skills which were within normal limits. The physicians doubted whether all of the patient’s symptoms could be accounted for organically. Cortex, (2001) 37, 715-718
716
J.M. Gurd and Others
Fig. 1 – Axial FLAIR sequence MRI scan (parallel to the AC-PC line), showing areas of high intensity signal in the left inferior frontal corona radiata. This image is left-right reversed.
FOLLOW-UP EXAMINATIONS In December 1999, the patient contacted the Neuropsychology Unit (Oxford), having discovered our interest in FAS on the web (Gurd et al., 1988). She was not depressed, but her anxiety score on the Hospital Anxiety and Depression Scale was borderline. There was severe impairment of oral agility (from the B.D.A.E.): Nonverbal agility = 4/12; verbal agility = 7/12. The patient provided audio recordings made before the onset of her speech problems, to confirm her North Yorkshire accent. Phonetic analysis was performed on an “audio diary” recorded by the patient between December 1999 and March 2000 and on studio recordings we made in April 2000. Her French-like pronunciation was largely due to three specific disturbances in her speech, together with one aspect of her native English dialect:
Cortex Forum
717
(1) Abnormal, “syllable-timed” rhythm: each syllable has approximately equal duration. This is characteristic of French, whereas normal English has more variation in syllable duration, according to the distribution of stress in the sentence, and the composition of the syllables in terms of their constituent vowels and consonants. This predates the appearance of other aspects of a French-sounding accent in the early recordings, and persisted at 5 months post-onset. (2) Exceptionally high pitch is sometimes placed on intonationally prominent syllables. (3) Her pronunciation of /h/ is very effortful: produced with abnormally loud laryngeal friction, or avoided altogether. The latter problem combined with impairment of pitch control suggests that poor control of the larynx is implicated, although the rhythm problem is not due to this impairment. Syllable-timed rhythm, /h/-dropping, and /h/-hyperarticulation are all characteristics of French pronunciation, while the pitch exaggerations contribute to the overall ‘foreignness’ of her speech. Also, the patient’s English diphthongs are actually quite monophthongal, as she originally had a North Yorkshire accent. This aspect of her undisturbed speech adds to the list of French-like features. As in some previous cases of FAS, there was no persisting aphasia or dysarthria (Takayama et al., 1993). There were, however, some errors characteristic of apraxia of speech (Varley and Whiteside, 2001): absence of lip rounding, the production of both vowels and consonants of excessively long duration, and, on rare occasions, an impairment of stress placement, even on highly familiar words (e.g. DIvision, someONE). DISCUSSION The patient displays an acquired speech disorder, some aspects of which cause the listener to perceive her speech as somehow ‘foreign’. A number of these features are sufficiently ‘French-like’ for the listener to assimilate her accent to that characteristic of a speaker of that language: French accents are well-known to English listeners and thus form a category to which the patient’s speech can readily be assigned (Kurowski et al., 1996; Carbary et al., 2000; Dankovicova et al., 2001). The following questions arise: (a) Is the entire range of symptoms explicable organically by the small lesions and neurological findings (e.g. EEG & CSF)? (b) Is there a mixture of organic and functional symptoms? Is the speech disorder of organic origin, but the bizarre gait a functional overlay (Keane, 1989)? It is perhaps significant that the abnormality of gait recovered without medical intervention? (c) Are the organic symptoms functionally exaggerated? Minor neurological abnormalities may underlie both the speech and the gait disorder, but may not be sufficiently marked to account for the severity of these symptoms. A catastrophic cascade may have been provoked by a minor brain injury. (d) Is the entire range of symptoms functional? The case notes reported that none of the neurological
718
J.M. Gurd and Others
signs (including CT, MRI, EEG) were clinically significant. Conversion disorders can mimic virtually all neurological conditions (Halligan et al., 2001). However, this seems unlikely in view of the findings of the neurological investigations even though they were somewhat equivocal. Clinical lore suggests that medical students frequently believe themselves to be suffering from whatever disease they are currently studying. Patients could always have done likewise, but would have needed access to a medical library. Now patients can put themselves in the same position as medical students at the click of a mouse. Were some aspects of her foreign accent in any way provoked by our patient’s knowledge of the clinical category of FAS? If so, what are the more general implications for the reliable (and valid) diagnosis of `neurological’ disease in the age of the internet (Khan et al., 2000)? Acknowledgements: JMG and JCM are supported by the Medical Research Council. We thank Drs. J. Barnaby, G.J. Saldanha, and Y. Zoukos for data. We are also grateful to Dr. P. Anslow, B. Mastrude, Dr. N. Brooks, and Dr. G.R. Fink for their comments on the case. REFERENCES CARBARY TJ, PATTERSON JP and SNYDER PJ. Foreign accent syndrome following a catastrophic second injury: MRI correlates, linguistic and voice pattern analyses. Brain and Cognition, 43: 78-85, 2000. DANKOVICOVA J, GURD JM, MARSHALL JC, COLEMAN JS, MACMAHON MKC, STUART-SMITH J and SLATER A. Aspects of non-native pronunciation in a case of altered accent following stroke (foreign accent syndrome). Clinical Linguistics and Phonetics (in press). GURD JM, BESSELL NJ, BLADON RAW and BAMFORD JM. A case of foreign accent syndrome, with follow-up clinical, neuropsychological and phonetic descriptions. Neuropsychologia, 26: 237-251, 1988. HALLIGAN PW, BASS C and MARSHALL JC (eds). Contemporary Approaches to the Study of Hysteria: Clinical and Theoretical Perspectives. Oxford: Oxford University Press, 2001. KEANE JR. Hysterical gait disorders: 60 cases. Neurology, 39: 586-589, 1989. KHAN I, FAYAZ I, RIDGLEY J and WENNBERG R. Factitious clock drawing and constructional apraxia. Journal of Neurology, Neurosurgery and Psychiatry, 68: 106-107, 2000. KUROWSKI KM, BLUMSTEIN SE and ALEXANDER M. The foreign accent syndrome: A reconsideration. Brain and Language, 54: 1-25, 1996. MATAS M. Psychogenic voice disorders: literature review and case report. Canadian Journal of Psychiatry, 36: 363-365, 1991. MOEN I. Foreign accent syndrome: A review of contemporary explanations. Aphasiology, 14: 5-15, 2000. TAKAYAMA Y, SUGISHITA M, KIDO T, OGAWA M and AKIGUCHI I. A case of foreign accent syndrome without aphasia caused by a lesion of the left precentral gyrus. Neurology, 43: 1361-1363, 1993. VARLEY RA and WHITESIDE SP. What is the underlying impairment in acquired apraxia of speech? Aphasiology, 15: 39-49, 2001. Dr. J.M. Gurd, Neuropsychology Unit, University Department of Clinical Neurology, The Radcliffe Infirmary, Woodstock Road, Oxford OX2 6HE, UK. E-mail: [email protected].