Cotard’s Syndrome: “Doctor, I’m here to tell you that I’m dead”

Cotard’s syndrome was described in 1880 by French neurologist Jules Cotard, after a middle-aged woman approached him for help after experiencing some very peculiar symptoms. The woman, whom he named Mademoiselle X, believed that she had “no brain, nerves, chest, or entrails, and was just skin and bone”, and believed that she did not need food, for she was not really alive. It turns out that Mademoiselle X was suffering from a rare mental disorder in which people believe that they are either dead, rotting, or have lost some vital internal organs (Berrios & Luque, 1995). For some of you, this might be ringing some bells… Zombies, anyone? Not surprisingly, this disease is also known as “walking corpse syndrome”, and while it might make you think of “The Night of the Living Dead”, this is definitely not just some scary movie.

Although researchers have not discovered an exact cause, Cotard’s syndrome has been found to manifest itself after some type of psychotic depression episode, but has also been linked to typhoid fever, Parkinson’s disease, and a drug commonly used to treat cold sores called acyclovir. While it is not know id Mademoiselle X had any prior medical history which may have been related to the development of this syndrome, a much more recent case has become popularized by the media, which can give us a little bit more insight into the life of someone with this syndrome. A man named Graham, sat down for an interview with New Scientist, to recount his battle with the condition. Graham had attempted to kill himself by electrocuting himself in the bathtub, after struggling with a severe bout of depression. Miraculously, he survived, but when he awoke from his attempted suicide, he felt as if his brain were dead. Graham told the magazine “I just felt as if my brain didn’t exist anymore… I lost my sense of smell and taste. I didn’t need to eat, or speak, or do anything”. Graham suffered from the disorder for so long that his teeth turned black, because he did not feel the need to brush them, since he was “dead”. He even went as far as spending the majority of his time in a graveyard, because he felt as if that was the closest he could get to actual death. When doctors tried to treat him with various medications, he would turn them away, saying that they wouldn’t do them him any good, because he no longer had a brain. He felt as if he were confined to some kind of half-life, with a dead brain and a living body.

So, how is this possible? One would think that, no matter what, since Graham was walking, talking, and moving about that would be enough proof for him to believe that he was alive. Remarkably, when doctors assessed the cerebral metabolism of Graham’s brain with a position emission tomography (PET) scan, which is an imaging technique that produces 3D models of functional processes in the body, they discovered that when Graham was saying he was “brain dead”, he was not far off form the truth. What they found was that Graham’s cerebral glucose metabolism was 22% below normal in areas of the brain such as the cingulate cortex and the dorsal prefrontal cortex (Fig. 1) (Charland-Verville et al., 2013). This is an important finding, because glucose metabolism is the major energy source for the brain, and there has been found to be an excellent correlation between the amount of local cerebral glucose utilization and blood flow to those local regions (Byrne, 1997). This means, that if Graham had 22% lower than normal cerebral glucose metabolism in these areas of the brain, he likely had decreased blood flow to these areas as well. Areas of the brain deprived of blood and glucose are going to suffer from decreased brain activity, resulting in abnormal brain function (Byrne, 1997). Doctors even noted that Graham had the brain function that resembled someone under anesthesia, or who was asleep. Furthermore, this observed pattern of metabolic depression was found to be much more widespread and severe than classically reported in major depressive disorder, so Graham’s observed brain activity couldn’t simply be attributed to his depression (Charland-Verville et al., 2013). At this point, one question you may be pondering is, “What are these areas of the brain responsible for, and how might decreased blood flow be affecting them?” Well, it turns out that the affected regions of Graham’s brain are actually responsible for conscious awareness, or our “core consciousness”. Keeping this in mind, it’s not really all that surprising that patients suffering from Cotard’s syndrome believe that they are dead, or no longer exist, seeing as the regions of the brain responsible for their core consciousness are not properly functioning.

Figure 1: Brain areas with impaired cerebral glucose metabolism in the patient. The graph illustrates decreased metabolic activity in the posterior cingulate in the patient as compared to age-matched healthy controls.

Is there any hope for people suffering from this disorder, or are they domed to live the rest of their days feeling like walking corpses? When Mademoiselle X was first diagnosed in 1880, there was unfortunately no treatment available, and she ended up starving to death, because she refused to eat. We’ve come a long way since then, and while we can’t exactly say there is a “cure” for Cotard’s syndrome, there are treatment options available. Various “drug cocktails” are currently being used to treat/manage this syndrome, including tricyclic antidepressants, such as imipramine, antipsychotics, such as sulpiride, and lithium, which is generally used to treat manic episodes associated with bipolar disorder (Fig. 2) (Charland-Verville et al., 2013; Yamada et al., 1999). While pharmacotherapies have been promising for some patients, such as Graham, they simply haven’t been working well for the treatment of others. This has caused doctors to turn to a more controversial treatment, electroconvulsive therapy (ECT), or perhaps more commonly known as “electric shock”. ECT is a psychiatric treatment in which seizures are electrically induced in patients to provide them with relief from psychiatric illnesses. It has been previously used as a last line of intervention for people suffering from major depressive disorder, schizophrenia, mania, or catatonia (Rudorfer et al., 2003). In a case study published in 1999, a woman who had been diagnosed with Cotard’s syndrome, and who was showing the same classic signs, similar to those experienced by Graham, was continuously re-admitted to the hospital, because drug treatments were not working for her. This is when doctors turned to ECT. Over the course of 1 month, the patient underwent 12 ECT sessions under general anesthesia. After her third session, she stated, “I can feel a depressive mood!” an improvement over the total lack of emotion she had prior to beginning treatment. After her ninth session, doctors confirmed that all the patient’s symptoms had disappeared. After the total of twelve sessions, the patient was taken off all medications and continued to show remission for 33 months post-treatment, proving that ECT is a viable treatment option (Yamada et al., 1999).

Figure 2: Cotard’s syndrome is often treated with “drug cocktails” including antidepressants and antipsychotics.

Obviously, patients with this syndrome aren’t exactly like the flesh-eating zombies from shows like “The Walking Dead” or movies like “World War Z” and they’re clearly not actually dead or rotting. However, since there is no actual cure,  people with Cotard’s syndrome might be “living” a very real nightmare that might not be that easy to wake up from.

 

 

References

Berrios GE, Luque R.(1995). Cotard’s delusion or syndrome? A conceptual history.Compr Psychiatry. 36:218-223.

Byrne, J. H.  (ed.), Neuroscience Online: An Electronic Textbook for the  Neurosciences
http://nba.uth.tmc.edu/neuroscience/ 
Department of Neurobiology and Anatomy, The University of Texas Medical School at Houston (UTHealth)
© 1997, all rights reserved.

Charland-Verville V, Bruno MA, Bahri MA, Demertzi A, Desseilles M, Chatelle C, Vanhaudenhuyse A, Hustinx R, Bernard C, Tshibanda L, Laureys S, Zeman A. (2013). Brain dead yet mind alive: a positron emission tomography case study   of brain metabolism in Cotard’s syndrome. Cortex. 49(7): 1997-9.

Rudorfer MV, Henry ME, Sackeim HA. (2003). “Electroconvulsive therapy”. In A Tasman, J Kay, JA Lieberman (eds) Psychiatry, Second Edition. Chichester: John Wiley & Sons Ltd, 1865–1901.

Swamy K, Sanju G, Jaimon M. (2007). An overview of the neurological correlates of Cotard syndrome. Eur J Psychiat. 21(2): 99-116.

Yamada K, Katsuragi S, Fujii I. (1999) A case study of Cotard’s syndrome: stages and diagnosis. Acta Psychiatr Scand. 100(5): 396-399.