Downloaded from http://jnnp.bmj.com/ on March 18, 2015 - Published by group.bmj.com Short report Simultaneous Doppelgänger and limb amputation impression in right frontal opercular stroke Joelle Nsimire Chabwine,1,2 Cristina Granziera,2 Maria Isabel Vargas,3 Tatiana Aboulaffia,2 Laurence Caratsch,2 Armin Schnider,1 Theodor Landis,2 Fabienne Perren2 1 Department of Clinical Neurosciences, Division of Neuro-Rehabilitation, University Hospital, Medical Faculty, and Neuroscience Centre of Geneva, Geneva, Switzerland 2 Department of Clinical Neurosciences, Division of Neurology, University Hospital, Medical Faculty, and Neuroscience Centre of Geneva, Geneva, Switzerland 3 Division of Neuro-Radiology, University Hospital, Medical Faculty, and Neuroscience Centre of Geneva, Geneva, Switzerland Correspondence to Dr F Perren, University Hospital and Medical Faculty of Geneva, Department of Clinical Neurosciences, Division of Neurology, 4, Rue Gabrielle Perret-Gentil, CH-1211 Geneva 14, Switzerland; fabienne.perren@hcuge.ch JNC and CG contributed equally to this paper. Received 25 June 2010 Revised 26 September 2010 Accepted 28 September 2010 Published Online First 3 November 2010 ABSTRACT A case is described of a patient who presented almost simultaneously the impression that his left arm was amputated and the feeling of the presence of his invisible Doppelgänger. While these body scheme disorders have both been described after (right) parietal lesions, a right frontal opercular ischaemic stroke was found in the neurological work up. Diffusion tensor imaging showed that the stroke involved the ventral bundle of the superior longitudinal fasciculus that connects the parietal to the frontal lobe. The unusual clinical presentation of this frontal lesion may have been due to a ‘diaschisis’-like phenomenon via the superior longitudinal fasciculus. INTRODUCTION Body scheme disorders constitute a wide group of neuropsychological disturbances affecting the feeling of self and body. The feeling of a presence (FOP) refers to the awareness of the physical presence of somebody nearby.1 2 When this presence is recognised as one’s own ‘alter ego’ it is called an ‘invisible Doppelgänger ’ and describes a form of reduplicative disorder relating to the body.2 3 The feeling of limb amputation is defined as the impression that a part of the body is missing.4 Equivalence between the terms ‘asomatognosia’ and the feeling of limb amputation is not clearly established in the literature.4e6 In this paper, we will consider both terms as synonyms, following the definition of Arzy et al.5 The FOP is reported in healthy subjects during long periods of social isolation3 6 and in a large variety of focal and diffuse cerebral pathologies: brain masses, head injury, brain infectious or inflammatory diseases, epilepsy, psychiatric disorders, migraine, degenerative diseases, etc.3 7e9 Contrary to asomatognosia which is often caused by cerebrovascular lesions,10 11 the FOP is rarely associated with cerebral strokes. Lesions implicated in both disorders seem to be preferentially located in the parietal lobe alone or in association with temporal or occipital lobes.3 5 While lateralisation of lesions in the FOP remains controversial,3 8 deficits causing asomatognosia are generally localised in the right hemisphere.3 In this paper, we report the case of a patient who had both the FOP and the feeling of left arm amputation due to a right frontal opercular ischaemic stroke, and investigated the underlying anatomy with diffusion tensor imaging (DTI) tractography. J Neurol Neurosurg Psychiatry 2011;82:1209e1211. doi:10.1136/jnnp.2010.221333 PATIENT AND METHODS This 48-year-old, right-handed manager, with no neurological or psychiatric history, experienced on awaking a strong feeling that his left arm was missing. He was puzzled, but a few minutes later, he could ‘see’ again his left arm but failed to control it for a while. While taking a shower, he suddenly felt a presence behind him on his left side. He knew there was a ‘person’ present, he felt the shower curtain moving and someone blowing at the nape of his neck. He was afraid but turned around. There was nobody there! When reassuming his former position the conviction that there was another person immediately came back and he suddenly realised that this ‘person’ was himself, his ‘Doppelgänger’. On the way to work, he was feeling unsteady and was told he was talking like a drunken person (he thought this was due to leftsided face weakness). Nevertheless, he reached his office, intending to work, unaware of how seriously he was affected. As unsteadiness and speech difficulties persisted, he finally came to the emergency room where initially a mild left hemiparesis was noted, which recovered rapidly. The whole episode lasted approximately 3 h. Comprehensive neuropsychological examination of the patient showed a mildly disinhibited behaviour with a few non-lateralised errors and omissions on computerised attentional and working memory tasks. The rest of the examination was normal with, in particular, no signs of neglect. Diffusion weighted brain MRI revealed a small right frontal opercular ischaemic lesion (figure 1AeC). EEG on admission was normal. The neurovascular workup did not reveal a specific stroke aetiology. Diffusion tensor imaging tractography (DT-MRI) was performed 10 days after the acute event (figure 1D) targeting the superior longitudinal fasciculus, a major association fibre tract connecting parieto-temporal areas to the frontal lobe.12 13 In particular, its ventral component, originating from the supramaginal gyrus, terminates in the premotor and prefrontal areas12 via the frontal operculum (figure 1E,F). We found that the ischaemic lesion was indeed located within the ventral component of the superior longitudinal fasciculus, albeit not large enough to interrupt in a statistically significant way diffusion within the tract (figure 1D). DISCUSSION AND CONCLUSION Our patient is unusual for three reasons: (i) he presented with two very different body scheme 1209 Downloaded from http://jnnp.bmj.com/ on March 18, 2015 - Published by group.bmj.com Short report Figure 1 Brain MRI images obtained on day 2 after stroke. Axial diffusion tensor imaging (DTI) (A, B) and coronal FLAIR (C) compatible with an acute ischaemic lesion (white arrows). Tractography realised on day 10 after stroke, showing the ischaemic lesion (D, yellow circle and E) in the frontal opercular region involving the ventral bundle of superior longitudinal fasciculus. Schemes from Makris et al12 ((F) obtained with the kind permission of Cerebral Cortex) further illustrate that the ventral bundle of the superior longitudinal fasciculus passes through the frontal operculum and corresponds to the bundle in (D) and (E). (The colour coding of the obtained fibres is based on standard RGB code applied to the vector at every segment of each fibre. Blue indicates the rostro-caudal direction; red the medio-lateral plane; and green the dorso-ventral orientation.) illusions almost at once, the missing of a limb (asomatognosia) and the ‘doubling’ of a person (FOP, Doppelgänger); (ii) these phenomena occurred subsequent to a small right fronto-opercular lesion rather than the usual lesion of the posterior parietal cortex or of the temporo-parietal junction (usually associated with this type of disturbance of body representation); and (iii) DT-MRI tractography may provide a clue as to why a frontal lesion may mimic ‘posterior ’ symptoms. The combination of asomatognosia and FOP, the ‘missing and the double’, is rare but not unique; in 1925, Pötzl found an association between a FOP and somatoparaphrenia (delusional elaboration of disownership) subsequent to a right inferior parietal lobule haemorrhage.14 This is the ‘classic’ lesion site for body scheme disorders.15 While the FOP, when associated with a circumscribed cerebral pathology is rarely due to acute vascular lesions, but rather to focal seizures, asomatognosia is strongly associated with mostly right parietal pathology, in particular strokes. It has been reported that in addition to parietal or parieto-temporal pathology, an extension of the lesion into frontal areas may influence the clinical presentation, orbitofrontal lesion extension being more associated with delusional elaboration of disownership and latero-frontal extension more with asomatognosia.10 However, to the best of our knowledge neither FOP nor asomatognosia alone, and much less in combination, have ever been reported subsequent to a small frontal lesion. The only description of an acute small right frontal lesion which led to asomatognosia that we know of is that by Arzy et al5 who described a patient who transiently lost her forearm (asomatognosia) subsequent to two very small lesions, one in the right premotor cortex and the other in the motor cortex. They interpreted this finding with the recent discovery from functional MRI which showed that illusory embodiment, as tested with the ‘rubber hand illusion’, activated both the posterior parietal cortex and the premotor cortex, but that only premotor activation correlated with the strength of the illusion.16 In our case, the small lesion was fronto-opercular and thus the symptoms were not explicable by the same functional neuroimaging 1210 J Neurol Neurosurg Psychiatry 2011;82:1209e1211. doi:10.1136/jnnp.2010.221333 Downloaded from http://jnnp.bmj.com/ on March 18, 2015 - Published by group.bmj.com Short report hypothesis. However, the DTI fibre tracking analysis might offer an alternative explanation. DTI showed that the ischaemic lesion involved the ventral bundle of the superior longitudinal fasciculus which connects the parietal to the frontal lobe (figure 1DeF).12 We propose that the transitory asomatognosia and FOP were due to a far reaching ‘diaschisis’-like effect of the fronto-opercular acute lesion on parietal functions via the ventral bundle of the superior longitudinal fasciculus. The small size of the lesion probably caused partial interruption of the this bundle, leading to brief functional, rather than persistent, disturbance, as witnessed by the fast recovery from neurologic deficits. Furthermore, DTI tractography was performed 10 days after the stroke when the patient was totally free of symptoms. 4. 5. 6. 7. 8. 9. 10. Competing interests None. 11. Patient consent Obtained. 12. Provenance and peer review Not commissioned; externally peer reviewed. 13. REFERENCES 1. 2. 3. Brugger P. Are “presences” preferentially felt along the left side of one’s body? 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Über Störungen der Selbstwahrnehmung bei linksseitiger Hemipligie. Zeit Ges Neurol Psychiatr 1925;93:117e68. Critchley M. The parietal lobes. London: Edward Arnold, 1953 (reprinted Hafner, New York, 1966). Ehrsson HH, Spence C, Passingham RE. That’s my hand! Activity in premotor cortex reflects feeling of ownership of a limb. Science 2004;305:875e7. Advancing Postgraduates. Enhancing Healthcare. 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