Cotard’s Syndrome

“Yes, I’m warm, but things give off heat as they rot. And I suppose it is a pulse… but it’s a very weak pulse.”
~a former patient with Cotard’s Syndrome

Attending on a high-security forensic psychiatric ward has some perks for the curious of mind. The civil libertarians might not approve, but there is a little-realized facet of pre-trial evaluations that provides clinicians an opportunity to observe patients for far longer than would be the case in just about any other venue. You see, if a person is charged with a serious felony but is found incapable mentally of proceeding to trial, they will remain in a secure inpatient setting until such time that 1. they are restored to capacity to proceed, or 2. the charges are dropped. When the charge is, say, murder, prosecutors are reluctant to appear ‘soft on crime,’ but if the person is so mentally unstable that they might never be restored to capacity, you have a situation in which a person not convicted is nevertheless confined for indefinite periods of time in medico-legal limbo.

With 3d party payors wanting patients out the door in a matter of days, this limbo affords a clinician opportunities to observe pathology over a far longer span than would otherwise be possible in an inpatient setting. Just try getting Blue Cross Blue Shield to approve a hospital stay of fifteen years or more.

I will now dispel another fantasy held by many not involved in forensic psychiatric work: that it’s all like FBI profiling or CSI on television. It’s not. Much of it is routine and repetitive. Patients are admitted with symptoms of depression and anxiety (common), garden-variety schizophrenia (common), malingering and substance abuse (very common), and a smattering of less frequent conditions.

And then once in a very long while, perhaps a single time in a career, you see something astoundingly rare. It happened to me.

First some background: Jules Cotard, a former French army surgeon in the Franco-Prussian war who practiced at Hospice de la Salpetriere outside Paris, saw a female patient in 1880, whom he called Mademoiselle X. She held the belief that she was dead. Not a suicidal desire to die, mind you, but already dead. Patients with this particular condition maintain that they have died and, sometimes as an added delusion, are missing vital internal organs, an intact body, or a soul. They espouse being walking rotting corpses – totally utterly dead. Mdmse X had just such a presentation. She was convinced that she had no blood, no heart, and was a moldering cadaver. No amount of reassurance or production of evidence as to her actual state convinced her otherwise. She stopped eating – why would a corpse need to eat? – and eventually is said to have expired of starvation. Cotard called the condition le délire de négation, or nihilistic delusional state, but 21st century lay readers may know it better from media sensationalism as Walking Corpse Syndrome.

Delusions in general are challenging to understand and explain (and treat), especially to those in training or whose psychiatric patients to date have been middle class suburban neurotics on low-dose Prozac. A delusion is a fixed and unshakable belief that runs counter to reality and all evidence to the contrary. Delusions are insidious in that they usually do not appear overnight; there is almost always a prodromal phase in which the patient-to-be begins to focus on unusual ideas that might trigger some alarm amongst friends and family, but haven’t by that point reached the level where emergent psychiatric intervention is sought. Despite the detachment from reality, delusions do not involve visual or auditory hallucinations. While some delusions are fantastical (e.g., Martians have landed), others are entirely plausible (e.g., being followed), making their diagnosis difficult until the weight of evidence leaves no other choice. There are paranoid delusions – that spies or law enforcement are monitoring one’s actions. There are grandiose delusions – that one is famous and wealthy. There are erotic delusions – that a newscaster or movie actress is one’s secret lover. A delusional state likely drove John Hinckley to attempt to assassinate President Reagan. Delusional thoughts were reportedly evident in the suicide note left by the recent Washington Navy Yard shooter. Anna Anderson, the woman who claimed for decades to be Grand Duchess Anastasia of the House of Romanov, almost certainly suffered from delusions.

The beliefs espoused by those with Cotard’s Syndrome, in extremis, basically are no different. While Cotard’s Syndrome is not a freestanding diagnosis within the Diagnostic and Statistical Manual at present – possibly a reflection of its rarity – its psychotic presentation is solidly within the realm of the delusional. And while delusions of death are its hallmark, other aspects of the syndrome, such as the cessation of eating and bathing, or tactile hallucinations of worms eating the corpus, are unquestionably related. There is even one documented variant of a Cotard’s patient espousing immortality, premised on the belief that if he were dead but still able to talk and think, he must be a god. Go figure.

Cotard believed that there are two stages in the disease’s progression. For those more fortunate (?), the early phase, which was called germination, involves a psychotic depression with a fixation on hypochondriacal topics. If the patient is lucky, it will arrest there, and at least at present, with antidepressant medication and electroshock therapy, prognosis is guarded but not hopeless. Those who progress on the continuum, however, like Mdmse X, develop unremitting delusions of present-tense death, often resulting in a self-fulfilling prophesy of sorts.

And interestingly, Cotard was probably not the first person to recognize the condition. An English physician, Charles Bonnet, described the case of an elderly female patient in 1788. She had been hit by a sudden cold draught in the house, the story went, and instantly came to realize that she was dead. She insisted on being stretched out in a coffin and hosting a wake. Apparently she was fussing over the way her burial shawl was arranged during her own memorial. Once she fell asleep, her family moved her to bed, and with treatment that included the ingestion of ground precious stones and opium (!), she is said to have improved over time, but would still relapse every few months. Why this condition is not know as Bonnet’s Syndrome I am not certain, except that from the vantage point of two centuries, the elderly woman with the draught seems far more histrionic than do those with a profoundly neurovegetative state manifested in fulminant Cotard’s Syndrome.

I have searched the medical literature on this subject and found few scientific articles. Cotard’s Syndrome itself appears rare; however, somatic (bodily) delusions become more common if the diagnostic net is cast wider and other entities are included – these aren’t ‘pure’ Cotard’s Syndrome, but they share clinical features. For example, those with schizophrenia often have irrational thinking, although there is no evidence that their delusions are more likely to involve present-tense death. Bipolar disorder can produce psychosis at the extremes of mania and depression, but these symptoms clear when the patient returns to a euthymic, or ‘even keeled,’ state. Certain medications can effect profound mental status changes, but such suggests a transient toxic delirium and not a more intractable delusion (i.e., as far as we know, Cotard’s can’t be ‘fixed’ by dialysis and metabolic homeostasis alone). Strokes can manifest a state called asomatognosia, in which a patient loses awareness of parts of the anatomy on the afflicted side of the body. Traumatic head injuries can yield disorganized and delusional thoughts, as can subsequent or unrelated seizure activity, end-stage liver failure, tumors, dementia, and even severe migraines – but none of these are Cotard’s.

Almost nothing is known of the actual pathophysiology of Cotard’s, except that PET scans have revealed a markedly decreased level of glucose metabolism in the (admittedly few) patients with the condition who have been studied. The degree of decreased metabolism is similar to, but markedly more than, that seen in patients taking certain tranquilizers, or those under anesthesia or in vegetative states.

In many ways, Cotard’s Syndrome is related to Capgras Syndrome, since both are seen as disconnects in the parts of the brain that govern recognition and related emotional response. Capgras’ Syndrome is a delusional state in which a patient believes that familiar faces are actually those of imposters. If synapses in pertinent parts of a patient’s brain malfunction and this recognition system fails – what psychiatrists call derealization – one develops Capgras’ (if one is viewing others) or Cotard’s (if one is viewing self). Or at least that’s the theory.

In 26 years of clinical practice, I have only encountered once a patient with Cotard’s Syndrome. It was while I was attending on a long term forensic unit at a state psychiatric hospital. At the time I only had a vague recollection of what was Cotard’s; it’s not really a subject stressed in medical schools or residencies, being subsumed by the larger taxonomic umbrella of (generic) ‘delusional disorders.’ I remember my patient as an elderly black male of wiry build with short graying hair and, surprisingly, a ready smile when addressed. He was also in good physical health – he had no known history of head injuries, epilepsy, migraines, cardiac disease, cirrhosis, or malignancies. He also did not have schizophrenia, bipolar disorder, or dementia, and was not delirious while on my service. As a matter of fact, except for having little formal education, he seemed ‘normal’ on the surface if not always talkative.

Patients suffering dementing processes can often appear cognitively intact if an interview lacks depth; this is because these patients can initially cover their deficits with socially expected superficialities. The same can hold true for other psychiatric illnesses. In the case of my patient with Cotard’s, if one didn’t bring up his mortal condition, he could talk about ward activities and the weather and what he had seen on television without any problem. But if one brought up the state of his health, he’d say, in no uncertain terms, that he was dead. Psychological testing – repeated over time – did not show evidence of malingering or over-endorsement of symptoms, and this fact, along with his consistent clinical presentation over the years, suggested that he was the ‘real deal.’

And as with almost all other delusional disorders, if you engaged my patient in a discussion of his physical state, no matter how persuasive your logic, he had a ready explanation for why that just wasn’t so. Why did he eat and drink? Well, it was an activity he picked up while alive, one that he did now merely out of force of habit after more than 70 years. Why was his blood still liquid and red when drawn for labs? He hadn’t been dead long enough for it to have coagulated. Why did he sleep? He didn’t actually sleep – he just lay in bed with his eyes closed so that he could concentrate on the worms eating him. Why did he bathe? Corpses don’t need to bathe since they don’t sweat (the bathing part was accurate… he rarely attended to hygiene unless forced). And so on.

Treatment? Therein lies the problem. If delusions of death stem from a psychotic mood disorder, the treatment can include antidepressants, anticonvulsants, lithium, and shock therapy, with a fair prognosis. However, when the delusional symptoms are not caused by another underlying etiology, the response to treatment is often poor and incomplete. In my experience, antipsychotic medications, for example, render such a patient less likely to verbally express delusional thoughts, but the thoughts are still ‘in there’ – as was seemingly the case with my patient. Though I later moved to a new position in a different hospital, I have learned since that he’s still hospitalized. And still incapable of proceeding to trial. And still ‘dead.’

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