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 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 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.3.323 on 1 March 1993. Downloaded from 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 and 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 girlfriend of Erik Estrada (an American text of cerebral infarction of the right hemi- bilateral crossed optic ataxia and a discon- 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 5, 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 324 Letters to the Editor

MATTERS J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.3.323 on 1 March 1993. Downloaded from ARISING

False facilitation to repetitive stimula- tion

We have read with interest a letter from Drs Pullicino and Beck on incremental response to repetitive nerve stimulation in Guillain- Barre syndrome (GBS).' They suggested that acetylcholine (ACh) release from motor nerve terminals might be impaired in acute GBS. In motor neuron disease, abnormal decrement such as myasthenia gravis has now been well documented. However, the facilitation they showed seems to be a false positive result. In neuromuscular block in Lambert- Eaton syndrome, compound muscle action potential (CMAP) to the first stimulus is of Figure Sagittal MRI ofthe brain, Ti weighted image (TR 600, TE 15, 1 0 Tesla) low At low shows an extensive lesion (hyposignal) involving the whole corpus callosum. The very amplitude. frequency stim- anterior commissure was spared (arrow). ulation, a waning pattern similar to that seen in myasthenia gravis is a common find- placed in his right hand. Similarly, left ataxia as: 1) They are different types of ing.2 At high frequency repetitive stimula- pseudo-hemianopia was present: the patient movements (imitative gestures vs visually tion, marked facilitation more than 200 to correctly named 2 objects out of 15 present- guided reaching movements); 2) The move- 300% is thought to be confirmatory. The ed tachistoscopically in his left visual field, ment disorders are not observed in the same true facilitation results from an increase in but 15 out of 15 when they were presented movement fields (the whole movement field the number of ACh quanta released, which in his right visual field. The dichotic listen- vs the contralateral movement field), and 3) give rise to a larger end-plate potential. ing test also revealed total extinction on the With the same arm (left arm vs both arms). Electromyographers, however, have paid left side. The patient obviously experienced The crucial role played by the posterior attention to the false facilitation erroneously difficulty in reaching objects located in the parietal cortex in this function has been seen without true neuromuscular block. hemispace contralateral to his hand. While established,1 2 and a number of cases of Figure 1 is an example of false facilita- the patient was looking at a central visual optic ataxia following posterior parietal tion, recorded from a healthy normal sub- fixation point, a lateral target (pencil) was lesions have been published. However, ject aged 30. At 2 Hertz stimulation little presented in his peripheral visual field, optic ataxia may be observed in the absence change was noted. At higher frequency either right or left. The patient was instruc- of a parietal lesion. The posterior parietal stimulation, successive increase in ampli- ted to reach out and take this lateral target cortex is connected to the motor areas of tude was remarkable. The rate of increment (right or left) with one hand (right or left), the frontal lobes, ipsilaterally through pari- was highest at 50 Hertz stimulation, up to without moving his eyes from the central eto-frontal association fibres and contralat- 180% of the initial amplitude. The critical fixation point. Twenty trials were made for erally through the corpus callosum.3 Thus a finding is that duration of the negative each of the four combinations. When using lesion affecting one of these fascicles could phase became concomitantly shorter when the hand ipsilateral to the lateral target, the theoretically result in optic ataxia. A lesion the amplitude increased. As a result, the patient easily and accurately reached this affecting the intrahemispheric association area of negative phase remained relatively target: 20/20 correct responses were fibres could result in ipsilateral optic ataxia, unchanged, which is a typical finding for obtained for the right hand in the right but as such a lesion probably also partly false positive CMAP changes, either incre- hemispace, and 19/20 for the left hand in involves the primary motor cortex region, ment or decrement. In true facilitation in the left hemispace. However, when he had the ensuing motor deficit interferes with the http://jnnp.bmj.com/ to reach a target located contralaterally to demonstration of optic ataxia. A corpus the hand used, he experienced marked diffi- callosum lesion could result in bilateral culty: the direction of the arm movement crossed optic ataxia. This syndrome was was grossly inaccurate and the target was reported in one case of a split brain.4 To our missed. Only 5/20 correct responses were knowledge, no other case of bilateral obtained with the right hand in the left crossed optic ataxia following a lesion 2 hemisphere, and 9/20 with the left hand in restricted to the corpus callosum has been the right hemispace. MRI showed a lesion reported. Our case confirms that bilateral on October 5, 2021 by guest. Protected copyright. involving the whole extent of the corpus cal- crossed optic ataxia should be included in 5 losum (figure). The anterior commissure the classic signs of the disconnection syn- and the cerebellar peduncles were spared, drome. B GAYMARD and there were no visible lesions in the S RIVAUD 10 Hz region of the floor of the third ventricle. M-H RIGOLET There were no abnormalities in the cerebral C PIERROT-DESEILLIGNY cortex, in particular in the parietal lobes or Clinique Neurologique and Unite INSERM 289, the corona radiata. Hopital de la Salpetriire, Paris, France Correspondence to: B Gaymard, INSERM 289, 20 Hz The presence of a- disconnection syn- H6pital de la Salpetriere, 47 Bd de l'H6pital, drome, associated with a large lesion affect- 75651 Paris, Cedex 13, France. ing the corpus callosum was, in the context 1 Hyvarinen J. The posterior parietal cortex of of severe alcoholism, compatible with monkey and man. In: Barlow HB, Bullock Marchiafava-Bignami disease. The most H, Florey E, Grisser OJ, Peters A, eds. 50 Hz Studies of brain function, vol 8. Berlin interesting finding was the impairment of Heidelberg: Springer-Verlag, 1982. visually-guided reaching movements, in the 2 Ratcliff G, Davies-Jones GAB. Defective absence of motor weakness and somato- visual localization in focal brain wounds. sensory or visual field defects. This bilateral Brain 1972;95:49-60. Figure 1 A train of responses recordedfrom 3 Brodal A. The cerebral cortex, In: Neurological the abductor digiti minimi with electrical crossed visuo-motor impairment was con- anatomy. 3rd ed. Oxford: Oxford University sistent with stimulation ofvariousfrequency in a healthy bilateral crossed optic ataxia, Press, 1981:797-805. Smooth increment in that is, a specific impairment of visuo-motor 4 Gazzaniga MS, Bogen JE, Sperry RW. subject. amplitude is noted Observations on after dis- at higherfrequency stimulation. As the coordination.' Left ideomotor apraxia is a connection of the cerebral hemispheres in duration became concomitantly shorter, the different entity from bilateral crossed optic man. Brain 1965;88:222-36. negative area remained relatively unchanged.