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Mind & Brain, the Journal of Psychiatry


delusion(s)’’ using PubMed and Web of Science. Relevant articles in English, French, Dutch, and German were gathered. Since data on this topic is scarce, we looked at every article for relevant cross references. A general Internet search on Google was also used. From this last result, we only included articles written in peer-reviewed journals. Using this method, we tried to obtain all available peer-reviewed data on this topic. This was necessary because there are no controlled studies available. All literature is dominated by case studies and some case series.


PRESENTATION AND COURSE As described earlier, nihilistic delusions concerning the


individual’s body are the central features of Cotard’s syn- drome. In an analysis of 100 cases, the most prominent symptoms in Cotard’s syndrome are: depressive mood (89%), nihilistic delusions concerning one’s own existence (69%), anxiety (65%), delusions of guilt (63%), delusions of immortality (55%), and hypochondriac delusions (58%).8


Some case reports concerning delusional denial of preg- nancy despite clear morphologic signs10 and delusional paralysis in a patient with psychomotor agitation11 are reported as a special form of Cotard’s syndrome. However, the classification of these delusions as nihilistic delusions concerning one’s body can be debated.


A classical description of the course of Cotard’s syndrome is


given by Enoch and Trethowan. In its early stages, Cotard’s syndrome is characterized by a vague feeling of anxiety, with a varying time span from weeks to years. This anxious state gradually augments and can result in nihilistic delusions where denial of life or denial of body parts are the prominent features. The patient looses sense of reality.12 Despite the delusion of being dead, these patients show an increased tendency to automutilation or suicidal behavior. Additional symptoms may include analgesia and mutism. The core symptoms always reflect a preoccupation with guilt, despair, and death.12 Delusions can be accompanied by a de ´normite


´lire


This has been described as the manic Cotard’s syndrome.12 As the syndrome often occurs in association with other psychotic states, symptoms of these specific disorders are likely to be present. As an example, nihilistic delusions grafted on a depressive illness are often associated by other characteristics of a depressive episode such as weight loss and sleeping difficulties and the syndrome associated with organic disease is often associated with other symptoms such as disorienta- tion or neurological signs.12 The duration of the syndrome can vary from weeks to years depending on the underlying disorder.13


d’e In 1999, Yamada et al14 made a proposal for staging


Cotard’s syndrome. Based on a case report, he defined three stages: germination stage, blooming stage, and chronic stage. The germination stage is characterized by important hypochondria, cenesthopathy, and depressive mood. A diagnosis of Cotard’s syndrome cannot be made in this stage yet. In the blooming stage, the characteristic features of


M&B 2011; 2:(1). July 2011 68 ´, a delusion of massive increase of body measures.


Cotard’s syndrome (nihilistic delusions, delusions of im- mortality together with anxiety and negativism) are seen. The last stage, the chronic stage is differentiated in two forms: one with persistent emotional disturbances (depressive type) and a second where depressive symptoms are less prominent (paranoid type).14 This hypothesis was supported in a second case report.15


EPIDEMIOLOGY


The prevalence and incidence of this rare syndrome is not known. Only one study reported on prevalence in a selected psychogeriatric population in Hong Kong. In 2 out of 349 patients, Cotard’s syndrome was diagnosed suggesting a prevalence of .57% in this population. A prevalence of 3.2% was reached when severely depressed elderly were included.16 A recent study found .62% (n3) of patients in a Mexican sample (screened over a 2-year period) of primary psychiatric patients having Cotard’s syndrome.17 Using the same methodology they found also .11% (n1) having Cotard’s syndrome in a sample of neurological patients with mental disturbances.17 The likelihood of developing Cotard’s delu- sion appears to increase with age.18 Berrios and Luque8 found a mean age of 56 years in their analysis of 100 cases. A more recent study of 138 cases reported a mean age of 47.7 years.19 Women appear to be more vulnerable.12 The syndrome is found in different ethnic groups.18 Cotard’s syndrome is also occasionally described in children and adolescents.1930 Diagnosis of Cotard’s syndrome in people under 25 years old was described to be associated with bipolar disorder.19


Since Cotard’s syndrome is conceptualized as part of an underlying disorder, several psychiatric and somatic diseases have been associated with the syndrome. Unipolar10,12,16,3144 or bipolar12,14,15,19,25,29,4549 depression are the most common associated psychiatric disorders but also comorbid psychotic disorder12,13,37,45,5053 has been reported. Some studies repor- ted on the occurrence of Cotard’s syndrome with more uncommon psychiatric syndromes (hydrophobia,41 lycantro- phy,48 folie a deux,54 Capgras delusion,12,21,51,5456 Capgras and Fregoli delusion,30 depersonalization disorder17). Also Par- kinsonian symptoms, disappearing with successful treat- ment, are described.17 Several organic conditions were also associated: dementia,13,57 major depressive episode in mild cognitive impairment,46 depression in frontotemporal atro- phy,58 severe mental retardation,59 typhoid fever,60 cerebral infarction,61 superior sagittal sinus thrombosis,62 brain tumors,63 temporal lobe epilepsy,61,64 limbic epileptic in- sults,64 postictal depression,28 cerebral arteriovenous mal- formation,65 cerebral arteriovenous malformation and epilepsy,61 migraine,66 Laurence-Moon-Bardet-Biedl syn- drome,67 multiple sclerosis,65 Parkinson’s disease,68,69 brain injury,7072 a noninfectious complication of heart transplan- tation73 as a consequence of an adverse drug reaction to aciclovir and its prodrug valaciclovir74 and in herpetic77 and nonherpetic17 encephalitis.


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