Background
In 1880, Jules Cotard documented a characteristic syndrome after monitoring the unusual behavior of a 43-year-old woman.1 Known by the name Cotard’s Syndrome (CS), this extremely rare syndrome has still not been officially included in the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM) or recognized by the World Health Organization’s International Classification of Diseases (ICD). Although researchers have attempted to reinterpret CS and expand its horizons, its precise characterization has not been possible so far.1
Reported signs and symptoms
CS has been historically characterized by melancholic anxiety, suicidal tendencies, attempted self-infliction, delusions of damnation or possession, hypochondriac thoughts of non-existence or ruin of several organs, of the whole body, of the soul, of divinity, analgesia, and the idea of immortality.1 Other known signs and symptoms include denying the existence of the brain, mind, intellect, or the world in general. Denial of pregnancy and delusional paralysis have also been reported.2
Because the symptoms of CS develop over a period of time, researchers have proposed a three-stage model for the development of CS. The proposed CS model comprises a germination stage, a blooming stage, and a chronic stage. The germination stage is characterized by hypochondriasis and cenesthopathy. Symptoms of nihilistic delusions develop fully during the blooming stage. Chronic changes in mood and systematization of delusions are common during the last (chronic) stage.2
Etiology and pathogenesis
Most neuro-imaging data do not reveal any gross structural changes in the brain of patients with CS. Nevertheless, right frontal damage has commonly been reported in cases of delusional misidentification (including CS). Left lesions have also been reported. However, not all studies show right-sided hypofunctionality. In patients with neurological disorders, there is presumably a correlation between prefrontal dysfunction and nihilistic beliefs.3
Personality characteristics seem to play a key role in the development of CS. More importantly, the neuropsychological origin of CS is supposedly related to a malfunctioning information processing subsystem where face and body recognition are associated with a changed affective component. However, in the absence of this affective component, patients with CS may experience a feeling of derealization and depersonalization.3
Diagnosis and treatment
CS is also known to manifest itself with various diseases and physiological dysfunctions including, but not limited to, Parkinson’s disease, syphilis, epilepsy, cerebral trauma, brain tumors, migraine, postictal depression, and non‑herpetic encephalitis.2
An extensive literature survey reveals that depression is the most common diagnosis in patients presenting with CS. Accordingly, CS has been categorized into: (1) Psychotic depression (patients with melancholia and nihilistic delusions), (2) Cotard type 1, including the pure forms of nihilistic delusions without affective symptoms, and (3) Cotard type 2, with a mixed group of symptoms (depression, anxiety, and auditory hallucinations).2
Studies suggest that electroconvulsive therapy is effective in treating CS. However, case reports also highlight the benefits of antidepressants alone, antipsychotics alone, or a combination of both.2 Most patients benefit from a combination of medication and a form of talk therapy (e.g., psychotherapy or cognitive behavioral therapy).4
Conclusion
Further research is required to delineate the precise etiology of CS. A better understanding of this extremely rare disease will result into improved patient outcomes and more effective clinical interventions.
References
- Tomasetti C, Valchera A, Fornaro M, Vellante F, Orsolini L, Carano A, Ventriglio A, Di Giannantonio M, De Berardis D. The ‘dead man walking’ disorder: an update on Cotard’s syndrome. Int Rev Psychiatry. 2020 Aug-Sep;32(5–6):500–509. doi: 10.1080/09540261.2020.1769881. Epub 2020 Jun 5. PMID: 32500801.
- Grover S, Aneja J, Mahajan S, Varma S. Cotard’s syndrome: Two case reports and a brief review of literature. J Neurosci Rural Pract. 2014 Nov;5(Suppl 1):S59-62. doi: 10.4103/0976-3147.145206. PMID: 25540544; PMCID: PMC4271387.
- Debruyne H, Audenaert K. Towards understanding Cotard’s syndrome: an overview. Neuropsychiatry. 2012; 2(6), 481–486. doi: 10.2217/NPY.12.67
- Rath L. Cotard’s syndrome: What is it? [Internet]. WebMD. WebMD; [Accessed 2022 Sep 15]. Available from: https://www.webmd.com/schizophrenia/cotards-syndrome
