SHORT COMMUNICATION The Cotard syndrome. Report of two patients: with a review of the extended spectrum of Ôde ´lire des ne ´ gationsÕ C. Gardner-Thorpe a and J. Pearn b a Department of Neurology, Royal Devon and Exeter Hospital, Exeter, UK; and b School of Medicine, University of Queensland, c/o Department of Paediatrics and Child Health, Royal Children’s Hospital, Brisbane, Queensland, Australia Keywords: arteriovenous malforma- tion, Cotard, delusions, history of neurology, Marfan syndrome, mul- tiple sclerosis, Paris Received 26 February 2003 Accepted 7 January 2004 The Cotard syndrome is characterized by the delusion where an individual insists that he has died or part of his body has decayed. Although described classically in schi- zophrenia and bipolar disorder, physical disorders including migraine, tumour and trauma have also been associated with the syndrome. Two new cases are described here, the one associated with arteriovenous malformations and the other with prob- able multiple sclerosis. The delusion has been embarrassing to each patient. Study of such cases may have wider implications for the understanding of the psychotic interpretation of body image, for example that occurring in anorexia nervosa. Introduction The Cotard delusion encompasses a psychotic delusion in which the subject believes he is dead or does not exist (Cotard, 1880, 1882). The syndrome is rare (Berrios and Luque, 1995). Because of the counterintuitive content of the delusion (from the perspective of the listener) Cotard delusion is dramatic and intriguing to the medical history-taker. The delusion was first reported in 1788 (Forstl and Beats, 1992) but not described in detail until Jules Cotard (1840–1889) (Ritti, 1894) delivered a lecture in June 1880 at the Socie´te´ Medico-Psychologique in Paris (Cotard, 1880). Cotard (Pearn and Gardner-Thorpe, 2002, 2003; Gardner-Thorpe and Pearn, 2003) des- cribed the syndrome of psychotic denial of self-exist- ence as a delusion of severe melancholia, especially when associated with extreme hypochondriasis. The delusion occurs in children (Allen et al., 2000), in ado- lescents (Cohen et al., 1997) and in the aged (Chiu, 1995). It is usually a feature of schizophrenia or bipolar disorder although it may follow cerebral trauma (Young et al., 1992) and be associated with other tem- poroparietal lesions of the non-dominant cerebral hemisphere. In his original paper of 1880 Cotard noted secondary reports from other French doctors who had consulted him about patients and who believed they had lost all their blood, and others who believed they lacked brain, head, stomach or heart (Cotard, 1880). Thus a spec- trum of these psychotic delusions was established, the most extreme of which was, in Cotard’s own words, Ôsometimes [a patient who] does not even have a bodyÕ (Cotard, 1880). In one sense the Cotard delusion is unique in that, in Cartesian logic or philosophy, it is the only self-certifiable delusion – but a genuine delusion for the patient nonetheless. All other professed beliefs require second party or multi-party confirmation before a delusional attribute can be assigned after initial history taking. We report two new cases of Cotard syndrome in order to emphasize that psychotic delusions may be a sign of specific organic pathology rather than due to schizophrenia or bipolar disorder, and also to highlight the broad spectrum of this delusion which may range from the belief that a body part is lost, or dead, to complete denial of its existence. Two patients with the Cotard delusion Case 1 A 46-year-old man with Marfan’s syndrome developed double vision, sudden loss of memory, and bumped into objects. He was admitted to a psychiatric ward with a diagnosis of transient ischaemic attack or stress-related amnesic episodes. The double vision resolved over 3 days. Nominal aphasia was noted but his memory improved. A computerized tomographic (CT) scan showed a left posterior parietal haemorrhagic infarct. Mitral leaflet prolapse was demonstrated on left cardiac ventriculography and the aortic root showed aneuris- mal dilatation. Aortic valve replacement was carried out and long-term anti-coagulant therapy commenced. Two years later he felt hot and shaky and was con- fused for half an hour. He remained light-headed and ÔmuzzyÕ, and complained of an ill-defined disturbance of vision. Correspondence: Dr Christopher Gardner-Thorpe, Consultant Neurologist, The Coach House, 1A College Road, Exeter, EX1 1TE, UK (e-mail: cgardnerthorpe@doctors.org.uk). Ó 2004 EFNS 563 European Journal of Neurology 2004, 11: 563–566