r 322 Joumnal ofNeurology, Neurosurgery, and Psychiatry 1993;56:322-324

severe than at onset, while his previous rhage involving the right temporo-parietal

prosodic difficulties and headache had dis- region. A month later, the AVM was surgi- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.3.322-a on 1 March 1993. Downloaded from LETTERS TO appeared. Neurological examination, tonal cally removed. 2e that time, the patient audiometry and standard neuropsychologi- has experienced Mormal health. THE EDITOR cal examination were normal. In particular, The correct characterisation of this he showed no language impairment; oral patient's disturbances poses considerable and writtenl comprehension of complex pas- difficulties. It is neither auditory agnosia nor sages was good. He was able to recognise word deafness, given his correct perfor- simple and complex figures and recognise mance on neuropsychological tests. The faces, both familiar and unknown, on com- complexity of his deficits, on the other A case ofmusic imperception parison tasks. On a meaningful sound hand, rule out .their being due to "paracou- recognition test, he recognised animal, sia", that is, simple distortion in the percep- Defective perception of music due to an human and environmental sounds and tion of sound. altered capacity to discriminate the elemen- voices, effortlessly. The patient was This impairment could instead be classi- tary components of musical stimuli ambidextrous; there was no family history fied as a type of receptive amusia, which can (rhythm, pitch, timbre, intensity and dura- of left handedness. manifest itself not only as the incapacity to tion) produces an alteration in the aesthetic Examination of musical abilities (recogni- discriminate between melodic patterns, tim- enjoyment of and the emotional involve- tion and production of physical features of bre and pitch, but also as qualitative alter- ment in music.' To our knowledge, there is musical sounds, plus identification and/or ations of the acoustic experience, including no previous evidence of the existence of a reproduction of rhythm, melody and har- an emotional involvement in the music.' condition in which the primary disruption is mony; vocal and instrumental performance, The patient may have lost the ability to con- to the capacity to process the musical stim- and listening to musical compositions) were vert musical perception into something ulus as a whole. We report a case of a young normal. However, he complained of diffi- emotionally or intellectually meaningful musician who, as a result of a right tem- culties from the very beginning for the lis- (the third fundamental ability of musical poro-parietal lesion, presented with loss of tening task. On hearing the pieces played on function2). Disturbances of this type are, the gestalt capacity to process music, with the piano, he complained, "my perception unfortunately, difficult to view objectively the consequent loss of aesthetic pleasure. is changed... it's flat, it's no longer 3- because of their highly subjective nature. Our patient was a 24 year old male, who dimensional; it's only on two planes... These deficits are perhaps undervalued and was an amateur musician and a skilful gui- there's no emotion. .". His difficulties overlooked, and as a result, there are only tar player. He reported an early interest in increased as the presented compositions two cases in which the loss of aesthetic music: during primary school he played became more complex: ". . . this is even enjoyment in listening to music' or prob- keyboard instruments and during secondary worse: I can distinguish the different instru- lems in "conceiving of a whole piece" and school, the flute. He later started playing ments, but I can't perceive the whole... in "conjuring up the appropriate atmosphere the guitar, attending private lessons for two jazz pieces, the relationships between the for composition"4 are mentioned in the lit- years. accompaniment and the soloist escape me." erature. On 31 December 1987, after a muscular An EEG showed slight right temporal Even though much of our case is based strain (jumping over a gate), he noticed he abnormalities. A CT scan was scheduled, on the patient's subjective reports, it seems had difficulty in understanding "the but the patient did not keep the appoint- convincing for the selectivity of the distur- nuances of words and inflection of sen- ment. bance (altered perception of the whole and tences". The same day he realised that he On 31 April 1988 he had a generalised of the emotional component of music, with- had difficulties in understanding music: he tonic-clonic seizure: a CT scan (figure, left) out compromise to analytical perception) could not perceive the structure of musical and MRI (figure, right) showed the pres- and for the strict focal nature of the lesion pieces clearly; the relationship between the ence of a right temporo-parietal malacic (AVM involving the right plica curva and accompaniment and the soloist was indis- area, involving the plica curva and supra- the supramarginal gyrus). This supports the cemable, and aesthetic pleasure for the marginal gyrus. This area was post-haemor- hypothesis that the right hemisphere, or at musical world had completely vanished. At rhagic, with peripheral haemosiderin least some of its supramodal areas, is better the same time he complained of generalised deposits, and was surrounded by serpigi- than the left hemisphere' at performing the headache, described as pressure, mostly on nous hypointense images. A DSA showed process of appreciation of the entire sound. the right side. an fed MONICA MAZZONI arterio-venous malformation (AVM), POLICARPO MORETTI On 5 January 1988, he came under our by right sylvian branches. LUCIA PARDOSSI observation. He still complained of a recep- On 16 June 1990, he developed left MARCO VISTA tive musical impairment, although less hemianopia and headache due to a haemor- ALBERTO MURATORIO http://jnnp.bmj.com/ Neuropsychology Laboratory, Institute ofClinical Neurology, University ofPisa MICHELE PUGLIOLI Department ofNeuroradiology, Pisa, Italy Correspondence to: Dr Mazzoni, Istituto di Clinica Neurologica, Via Roma 67, 56126 Pisa, Italy. on October 2, 2021 by guest. Protected copyright. 1 Benton AL. Le amusie. In: Critchley M, Henson RA, eds. La Musica e il Cervello. Padova: Piccin, 1987:397-417. 2 Wertheim N. Esiste una localizzazione anatomica per le facolta' musicali? In Critchley M, Henson RA, eds. La musica e il cervello. Padova: Piccin, 1987:294-310. 3 Mazzucchi A, Marchini C, Budai R, Parma M. A case of receptive amusia with promi- nent timbre perception defect. Journal Neurol, Neurosurg Psychianty 1982;45:644-7. 4 Judd T, et al. (unpublished research) quoted by Kaplan JA, Gardner H. Artistry after uni- lateral brain disease. In: Boller F, Grafman J, eds. Handbook of neuropsychology. Amsterdam: Elsevier, 1989:147. 5 Gates A, Bradshaw JL. Music perception and cerebral asymmetries. Cortex 1977;13: 390-401.

Fr6goli and Figure Left: CT scan (2 May 1988) showing a ribbon-shaped malacic area in the right temporo-parietal region, the posterior part ofwhich has absorbed contrast agent The Fregoli delusion involves the belief that Right: MRI (5 May 1988) showing the malacic area involving the plica curva and the a familiar person disguises himself or herself supramarginal gyrus. as others. It was named after an Italian Letters to the Editor 323 actor, Leopoldo Fregoli, who was famous absence of a previous scar on his face. She some years previously the patient had a long for his ability to impersonate people. was convinced that Erik Estrada was in love love affair with this cousin (lasting over 20 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.3.322-a on 1 March 1993. Downloaded from In their original description of this delu- with her and planned to marry her one day. years, and leading to the birth of her only sion, Courbon and Fail' noted that their Past medical history revealed childhood child). patient experienced other whose until the age of 9 years, phenobar- Data for this previous case are also pre- content was mainly erotic. The association bitone being stopped at the age of 11 years. sented in the table, for comparison with the of Fregoli delusion and erotomania has only There was no family history of mental dis- patient we describe here. Both patients were been occasionally reported since, though in order. Routine haematological, biochemical impaired at recognising familiar faces, some Fregoli cases erotomania forms an and serological examinations were normal. matching disguised faces, and showed obvious background possibility.2' A review Physical examination revealed no abnormal- much poorer recognition memory for faces of misidentification syndromes and sexuali- ity. An EEG showed a moderate excess of than words. Although an EEG suggested ty by Barton and Barton2 noted that eroto- mixed irregular and rhythmic slow activity bilateral abnormalities for our present mania has been found to be a feature in at 2-6 HZ and 10-30uV in the central and patient, these face processing impairments various forms of delusional misidentifica- post-central regions. She refused neuro- point toward involvement of the right cere- tion, and included an additional description imaging. bral hemisphere, which has been noted as a of a case of a Fregoli-like delusion and ero- Neuropsychological tests of face process- feature in other cases of erotomania and tomania, in which a female patient claimed ing were also carried out. Details of the delusional misidentification.2'5 that another patient was an ex-boyfriend, tests used are given in Young, et al,5 which We gratefully acknowledge the support provided going under an assumed name, and that includes data from 10 male controls aged by ESRC grant R000231922. they were still deeply in love. 25-35 years. Results are summarised in the S WRIGHT We describe a further case for whom the table. The patient was able to recognise Department ofPsychiatry, Fregoli delusion arose in the context of photographs of emotional expressions Northern General Hospital, the form of erotomania known as de (happy, angry, sad, etc) without significant Herries Road, Sheffield S5 7AU, UK CGerambault's syndrome, in which patients difficulty. She was impaired at recognising A W YOUNG suddenly arrive at the delusional belief that photographs of familiar faces, but showed D J HELLAWELL Department ofPsychology, someone (usually of higher social standing) no tendency to misidentify unfamiliar faces University ofDurham, Science Laboratones, is in love with them.4 Although this patient as familiar (20/20 correct rejections of unfa- Durham DHI 3LE, UK had other delusions, the Fregoli and de miliar faces). In this face recognition test, Clerambault delusions dominated the clini- she did not claim that any of the pho- 1 Courbon P, Fail G. Syndrome d'illusion de cal picture, and were strongly held and tographs showed Erik Estrada, in disguise Fregoli et schizophrenie. Bulletin de la Societe persistent. or otherwise. She performed at the border- Clinique de Midicine Mentale 1927;15:121-5. The patient was a 35 year old, divorced, line of the impaired range on the Benton 2 Barton JL, Barton ES. Misidentification syndromes and sexuality. Bibliotheca unemployed woman who lived on her own. Test (which requires matching of unfamiliar Psychiatrica 1986;164: 105-20. She had a psychiatric history from the age faces) and was very poor at matching unfa- 3 De Pauw KW, Szulecka TK, Poltock TL. of 16, and was diagnosed as suffering miliar faces when they were masked by Fregoli syndrome after cerebral infarction. Y Nerv Ment Dis 1987;175:433-8. from chronic paranoid . She various disguises. On the Warrington 4 Enoch MD, Trethowan WH. Uncommon psy- stopped medication 6 weeks before admis- Recognition Memory Test, she showed nor- chiatric syndromes, 3rd ed. Bristol: John sion. mal recognition memory for words but Wright, 1991. She was agitated and verbally hostile, and severely impaired recognition memory for 5 Young AW, Ellis HD, Szulecka TK, de Pauw KW. Face processing impairments and reported auditory of famous faces. delusional misidentification. Behavioural actors who she said were her friends. She The patient was started on a Neurology 1990;3: 153-68. claimed to be telepathic, saying her actor fluphenazine depot and her mental state friends put their thoughts into her head, improved considerably. Twelve months and that her thoughts were broadcast to later, however, she still believes she is to Bilateral crossed optic ataxia in a them. She showed grandiose delusions, marry "Erik" and that he continues to visit corpus callosum lesion believing she could arrange to stop all tele- her regularly, albeit in disguise. vision and radio communications by telling Her pattem of impairment on face Optic ataxia is a disorder of visually-guided her actor friends to go on strike using her processing tests was comparable to that hand movements, usually resulting from a "telepathic powers". found for another case we investigated, in lesion affecting the posterior parietal The patient believed that she was the which the Fregoli delusion arose in the con- cortex.' We recently observed a patient with bilateral crossed optic ataxia and a discon-

girlfriend of Erik Estrada (an American text of cerebral infarction of the right hemi- http://jnnp.bmj.com/ actor and pin-up), with whom she commu- sphere." That case did not show our nection syndrome. MRI showed a large nicated across the Atlantic via telepathy. patient's flagrant erotomania, but there was corpus callosum lesion without any other She also believed that Erik Estrada visited a definite possibility of an erotomanic ele- visible lesion. her home city regularly, disguised as ment in her delusion. She thought that she A 37 year old right handed man was acquaintances or her current boyfriend. She was being pursued by her cousin and a admitted to intensive care with an acute stated that she knew her actual boyfriend female accomplice, both of whom adopted respiratory distress syndrome caused by was Erik Estrada in disguise due to the different disguises. It was later found that severe lung disease. The patient had a long history of alcoholism. One month later, the patient's condition had improved and he on October 2, 2021 by guest. Protected copyright. Table Performance offace processing tasks, and means and standard deviations for control subjects was alert and cooperative. On neurological ofcomparable age. Data from another Fregoli patient,3 s are presentedfor comparison (this patient examination, there were no sensory loss or was aged 68, so herperformance has been compared to a different set ofcontrols). motor weakness, no cranial nerve abnor- malities, cerebellar syndrome or gait distur- Controls bance. Visual acuity was 10/10, bilaterally. The visual fields (Goldmann perimetry) Previous case Present case Mean (SD) and visual evoked responses were normal. FACIAL EXPRESSIONS Eye movements were recorded using elec- Labelling: 20/24 20/24 22-00 (1-24) tro-oculography. Horizontal smooth pursuit IDENTIFICATION OF FAMILIAR FACES gain was normal, and horizontal visually- Highfamiliarity faces normal and Occupation: 15/20*** 12/20*** 17-58 (1-08) guided saccades had accuracy Name: 8/20*** 12/20** 16 17 (1-53) latency. Higher cortical function testing Unfamiliarfaces showed a slight impairment of recent mem- Correct rejections: 20/20 20/20 ory, but normal verbal comprehension, UNFAMILIAR FACE MATCHING speech and reading. However, left ideo- Benton test: 42/54 39/54b "Disguise" task: 13/24*** 16/24** 21-60 (1-90) motor apraxia and left hand agraphia were RECOGNMON MEMORY present, suggesting the existence of a dis- Warrington RMT: Faces: 36/50d'* 33/50d2** 43 90 (3 65) connection syndrome. Warrington RMT: Words: 47/50d' 47/50d2 45-71 (4-76) There was also left astereognosis: the patient correctly named only 2 objects out [Asterisked scores are significantly impaired in comparison to the performance of controls: * z > 1-65, p < 0 05; **z > 2-33, p < 0-01; ***z > 3-10, p < 0-001. b=borderline of impaired range on of 12 when they were placed in his left test's norms. dl d2=significant discrepancy between faces and words scores]. hand, but made no errors when they were