Diseases and Disorders

Cotard’s Syndrome: The Real Walking Dead

Shruthi Ganesh


Abstract

Cotard’s syndrome is a rare condition under which patients may deny the existence of certain parts of their body, have delusions of immortality, and even believe that they are deceased. First observed by Dr. Jules Cotard, the syndrome has multiple symptoms, including nihilistic delusions, depression, and anxiety; in addition, it often accompanies other psychological disorders. The underlying pathophysiology is still being researched, but distinct abnormalities in the brain have been found to be associated with the syndrome. Treatment options, such as electroconvulsive therapy and pharmacotherapy, are also proven to work in several case studies.

 

Introduction

Delivering a lecture in Paris in the year 1880, French neurologist Dr. Jules Cotard presented a bewildering case: a 43-year-old woman who denies the existence of several parts of her body. The woman, “Mademoiselle X,”  claimed she “has no brain, no nerves, no chest” and “only skin and bones of a decomposing body.” He referred to the condition that would later be named after him as “Le délire des négations,” or “Nihilistic Delusions.” Since then, there have been a number of cases involving this mysterious mental illness [1].

Formerly known as Le délire des négations, Cotard’s syndrome (Walking Corpse Syndrome) is a rare condition in which a person denies the existence of certain parts of their body, or in extreme cases, their entire existence. Many patients also have delusions of immortality and other various nihilistic delusions, such as the belief of their organs rotting. The syndrome commonly accompanies other neuropsychiatric disorders, such as schizophrenia, psychosis, and extreme depression. Although Cotard’s syndrome is not listed as a separate disorder in both the Diagnostic and Statistical Manual of Mental Disorders, 5th edition (DSM-5) and the International Classification of Diseases, 10th revision, Clinical Modification (ICD-10-CM), it is still important to understand and research the neuropsychological mechanisms that underlie this unique condition in order to advance existing treatment for those who suffer from this syndrome.

 

Symptoms and Studies

Cotard initially characterized this syndrome as a type of depression with the following symptoms: ideas of damnation or rejection, pain insensitivity, anxious melancholia, delusions concerning the existence of one’s body, and delusions of immortality [6]. Although the disease is popularly recognized by the symptom in which a patient believes they are dead or non-existent, an analysis of 100 cases found that the most common symptom is actually a depressive mood (89%) [12]. Nihilistic delusions concerning one’s own existence followed behind (69%). Anxiety, delusions of guilt, delusions of immortality, and hypochondriac delusions were also present in 60% or less in the sample. Enoch and Trethowan asserted that the syndrome slowly progresses, starting as vague anxiety and developing into nihilistic delusions, suicidal behavior, and other behavioral manifestations of feelings of guilt, despair, and death [3].

In 1999, Yarnada suggested three stages of the illness: the germination stage, which featured significant hypochondria and depression; the blooming stage, which included nihilistic delusions and immortality delusions; and the chronic stage, which consisted of either a depressive type of the syndrome or a paranoid type of the syndrome [4]. This theory, however, is not substantially supported by many, as the syndrome’s symptoms are not as consistent and concrete as the theory suggests. 

Although research regarding Cotard’s syndrome is undeveloped, and studies mostly consist of relatively small sample populations, the results are still significant. For example, in a 2017 study  that analyzed medical records of patients with Cotard’s syndrome between 1996 and 2016, data showed a gender predilection for males and a median age of 52 for affected individuals, contradicting a previously held misconception that the syndrome mainly affected the elderly [5]. 

 

Pathophysiology

Like all psychological disorders, Cotard’s syndrome stems from neurological abnormalities that cause various nihilistic and depressive symptoms. Cotard’s syndrome falls under the category of “delusional misidentification syndromes,” or DSMs, and is thus pathophysiologically similar to other disorders, such as Capgras syndrome. These disorders have a key neurological commonality: disconnections in neural circuits that directly influence the perception of self, which induce feelings of depersonalization. In particular, a deep right frontal lesion could disrupt connections between the limbic region and frontal lobe, which affect familiarity of self, people, and places.

For Cotard’s syndrome specifically, studies point to a misfiring in the fusiform gyrus of the brain as a cause for the delusions. One of the earlier CT imaging studies associated the syndrome with multifocal brain atrophy and interhemispheric fissure enlargement [9]. Damages to the frontal lobe, as discussed earlier, were also shown to accompany changes in the non-dominant temporoparietal areas. It is possible for Cotard’s syndrome to result from a traumatic brain injury, as shown in a case study of a young man who started believing he was dead following an injury that affected the temporo-parietal areas of the right cerebral hemisphere [13].  In short, as Debruyne suggests, “these studies point to an important role for the fronto-temporo-parietal circuitry in the pathophysiology of the syndrome.”

 

Treatment

Several treatment options have been presented, although only in case report form. Electroconvulsive therapy (ECT) has been cited as the most common treatment, in which small electric currents are induced in the brain to create brief seizures under general anesthesia [2]. Partnered with pharmacotherapy, ECT has been shown to work exceptionally well. In a case study conducted in 2010, a 37-year-old female patient with Cotard’s syndrome was completely cured with the administration of ECT along with venlafaxine and olanzapine [14].  Another case report saw an improvement in Cotard’s symptoms after treatment with fluoxetine and risperidone. Chan also found a combination of venlafaxine and quetiapine to provide relief of nihilistic delusions in a 68-year-old patient [11]. Like many treatments, ECT does come with its own risks; memory loss, confusion, and heart problems are just a few of its  side effects. 

Since Cotard’s syndrome is linked with many other neuropsychological disorders, it has similar psychopathological pathways to other existing diseases with treatment such as Capgras syndrome,  which is why there are already existing treatments to help control this mental disorder.  However, more research is needed to formulate a treatment that specifically addresses Cotard’s syndrome. Hopefully, future studies will analyze pathophysiology in greater detail to gain a greater understanding of this rare disease and treat it more efficiently in patients.


References


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Shruthi Ganesh

Shruthi Ganesh


High School Junior at Poolesville High School, MD, USA. Planning on studying Neuroscience in undergrad to become a future physician-scientist with an MD/PhD