Ep-PAINE-nym



Cotard’s Syndrome

 

Other Known Aliases – Cotard delusion, Walking Corpse Syndrome

 

DefinitionRare mental illness in which a person feels they are dead, do not exist, parts of them are decaying or rotting, or they have lost internal organs, blood, or extremities.

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Clinical SignificanceThe pathophysiology is not well understood and the two thoughts are that it is due to lesions or atrophy in the parietal and/or frontal lobes, or due to neural misfiring in the fusiform gyrus that is responsible for facial recognition.

The core concept of Cotard’s syndrome is a delusion of negation and classically progresses through three stages:

  • Germination Stage – symptoms of psychotic depression and hypochondria
  • Blooming Stage – full development of the syndrome and the appearance of the delusions of negation
  • Chronic Stage – severe delusions with chronic depressive symptoms

It is most common in patients with underlying schizophrenia and psychosis and patients often withdraw from society and the outside world.  Partly because of the delusions and partly due to personal neglect of appearance and hygiene.  There is no DSM-V diagnosis for Cotard’s syndrome, so it falls under the category of somatic delusions.

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History – Named after Jules Cotard (1840-1889), a Parisian neurologist, psychiatrist, and surgeon who received his medical doctorate in 1868 from the University of Paris and worked at the Hospice de la Salpétriére  under Jean Martin Charcot.  In June 1880, he read a report on “Du délire hypochondriaque dans une forme grave de la mélancolie anxieuse” where he described a case of a 43yo woman who believed she had no brain, nerves, or entrails and that she did not need food, for she was eternal and would live forever.  Emil Régis was the first to coin the eponym in 1893.  In 1889, his daughter contracted diptheria and for 15 days he refused to leave her bedside until she recovered.  Unfortunately, he contracted the same illness and succumbed to disease later that year.

 

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References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Berrios GE, Luque R. Cotard’s delusion or syndrome?: a conceptual history. Comprehensive psychiatry. ; 36(3):218-23. [pubmed]
  7. Pearn J, Gardner-Thorpe C. Jules Cotard (1840-1889): his life and the unique syndrome which bears his name. Neurology. 2002; 58(9):1400-3. [pubmed]
  8. Cotard J. Du délire hypocondriaque dans une forme grave de la mélancolie anxieuse. Ann Med Psychol (Paris). 1880;4:168-174.
  9. Régis E. Note historique et clinique sur le délire des négations. Gaz Med Paris. 1893;2:61-64.

#20 – Schizophrenia



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Epidemiology

Schizophrenia is ranked by the World Health Organization as one of the top mental illnesses in regards to global burden of illness and years lived with disability (YLDs) and disability-adjusted life-years (DALYs).

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The estimated prevalence of schizophrenia in the world approaches 1% (that’s 74 million people) and the incidence is around 1.5 per 10,000 population.  It is slightly more common in men than women (1.4:1) and they have an overall worse prognosis, but women tend to be diagnosed later in life.

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Sham PC


Other Disease Risk

The approach to behavioral medicine conditions is similar to sexually transmitted diseases….in that if you suspect ONE you should screen for ALL.  These diseases run in packs and schizophrenia is no exception.  There is an increased risk of co-diseases such as depressive disorders, anxiety disorders, substance abuse, and neurologic/endocrinologic disease. There have been many identified risk factors for the development of schizophrenia include environmental, infection, prenatal, obstetric factors, and family history.

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Pathogenesis

Unknown at this point, but seems to be a confluence of genetic and environmental factors.  Using genome-wide association studies (GWAS), researchers have have found that it is not one or two big genes that cause schizophrenia, but more of a polygenomic effect of multiple small genes with increasingly additive effects.  Currently, they have found 500,000 single nucleotide polymorphisms (SNPs) that have been associated with schizophrenia development with 108 of these showing significant association.


Clinical Manifestations

Schizophrenia is a syndrome with impairment in several domains.

  • Positive Symptoms
    • These are symptoms distort the patient’s reality and/or lead to disorganized thoughts and behaviors
      • Called “positive” because they did not have them prior to developing their illness so they were “added” to their psyche
    • Hallucinations
      • Auditory is most common (40-60%)
        • Can included voices, music, machinery
      • Visual
        • Anything from unformed distortions to fully formed, identifiable objects or human visages
      • Somatic
        • Sensation of being touched
      • Olfactory/Gustatory
    • Delusions
      • Defined as fixed, false belief even with contradictory evidence
      • Up to 80% of patients with schizophrenia
      • Can be categorized as:
        • Bizarre
          • Implausible or impossible
        • Non-bizarre
          • Understandable, but still untrue
        • Content of the delusions can be categorized as delusions of:
          • Reference
            • Beliefs that seemingly random events are meant for or are in reference to the patient (radio, TV, songs)
          • Grandiosity
            • Belief the patient has significant importance or power
          • Paranoia
            • Beliefs that something/someone is trying to affect the patient
          • Nihilism
            • Beliefs that the patient or other people are dead or don’t exist
          • Erotomania
            • Beliefs that the patient has a special relationship with someone
          • Disorganization
    • Schizophrenia is a disorder with disorganized behavior or thoughts. Behaviors can be observed but thoughts can only be inferred from the patient’s speech.  Some of these clues are:
      • Tangential
        • Patient strays from the conversation topic
      • Circumstantial
        • Patient will answer a question but in a long, roundabout way
      • Derailment
        • Suddenly switches topics without cue/logic/segue
      • Neoligism
        • Using made-up words only known to the patient
      • Word Salad
        • Known words used together in no meaning or pattern
  • Negative Symptoms
    • Absence of normal processes
    • Primary (Deficit Symptoms)
      • 2 categories
        • Diminished Expression Cluster
        • Avolition-Apathy Cluster
    • Secondary
      • Any of the above symptoms due to another cause
        • Paranoia leading to social isolation
        • Unchanging facial expression from antipsychiotic use
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Up To Date. 2016.

  • Cognitive Impairment
    • Usually precedes positive symptoms and are first observed by family members
    • Often one to two standard deviations lower than healthy controls
    • Common symptoms include decreases in:
      • Processing speed
      • Attention
      • Working memory
      • Verbal/visual learning
      • Reasoning
      • Executive function
      • Comprehension
      • Social cognition
  • Mood and Anxiety Symptoms
    • Higher incidence in patients with schizophrenia than the general population

Physical Manifestations

There are a few physical exam findings that can be seen in patients with schizophrenia that can precipitate further screening.

  • Neurologic Disturbances
    • Subtle impairments of sensory integration, motor coordination, and sequencing and need to distinguished from normal pathology of the disease and the side effects of medications
    • Disease specific
      • Right/left confusion
      • Agraphesthesia
      • Astereognosis
      • Catatonia
  • Medication Induced
    • Extrapyramidal
      • Tremor, bradykinesia, dystonia, tardive dyskinesia
    • Metabolic Disturbances
      • Weight gain, diabetes development, cardiovascular disease

Disease Course

The presentation and disease is highly variable and differ in regards to onset (rapid vs slow), symptom presentation (continuous vs intermittent) and outcome (poor vs controlled).  Each patient with the suspicion of schizophrenia should be screened for these variables.


DSM-V Criteria for Diagnosis

  • 2 or more characteristic symptoms (one must be delusions, hallucinations, or disorganized speech) present for a significant portion of time during a one-month period:
    • Delusions
    • Hallucinations
    • Disorganized speech
    • Grossly disorganized or catatonic behavior
    • Negative symptoms
  • For a significant portion of the time since the onset of the disturbance, one or more major areas of functioning such as work, interpersonal relations, or self-care are markedly below the level achieved prior to the onset.
    • When the onset is in childhood or adolescence:
      • Failure to achieve expected level of interpersonal, academic, or occupational achievement.
  • Continuous signs of the disturbance persist for at least six months.
    • The six-month period must include at least one month of symptoms (or less if successfully treated and may include periods of prodromal or residual symptoms.
  • Schizoaffective disorder and mood disorder with psychotic features have been ruled out because either:
    • (1) no major depressive, manic, or mixed episodes have occurred concurrently with the active-phase symptoms; or
    • (2) if mood episodes have occurred during active-phase symptoms, their total duration has been brief relative to the duration of the active and residual periods.
  • The disturbance is not due to the direct physiological effects of a substance (eg, a drug of abuse or medication) or a general medical condition.
  • If the patient has a history of autistic disorder or another pervasive developmental disorder, the additional diagnosis of schizophrenia is made only if prominent delusions or hallucinations are also present for at least a month (or less if successfully treated)

Differential Diagnosis

There are several other conditions that can mimic schizophrenia and need to be assessed during screening or diagnosis.

  • Schizophreniform
    • Meets all criteria for schizophrenia, but has had < 6 months of symptoms
  • Schizoaffective, bipolar, and major depressive disorders
    • More mood components than disorganized thoughts or behaviors
  • Substance abuse or withdrawal
  • Schizotypal disorders
    • Odd behaviors or beliefs but not to the level of delusions or hallucinations
  • Schizoid personality disorder
    • Long standing pattern of little interest in social relationships
schizoid-vs-schizotypal

MedComic


Schizophrenia Diagnostic Algorithm

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Up To Date. 2016.


Management

Two categories of treatments in schizophrenia:

  • Acute Phase
    • Psychotic relapse in a patient with a known diagnosis, or the first episode of psychosis in an undiagnosed patient.
    • Goal of acute management to decrease agitation and increase therapeutic medication
  • Maintenance Phase
    • Recovered from acute psychotic event
    • Goal of maintenance is minimize symptoms and functional impairments, avoid relapses, and promote recovery

Prescreening Considerations

  • BMI, waist circumference, heart rate, blood pressure
  • Signs of movement disorder
    • Extrapyramidal symptoms
      • Akathisia, parkinsonism, dystonias
    • Tardive dyskinesia
  • Laboratory Studies (when feasible)
    • CBC, BMP, lipid profile, LFT, TFT
    • EKG

Antipsychotic Medications

  • 1st generation (Typical)
    • All antipsychotics marketed before 1989 (when clozapine came out)
  • 2nd generation (Atypical)
    • Everything after clozapine
    • Cause fewer extrapyramidal side effects
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Up To Date. 2016.

Treatment Considerations

There are several medications available as IM, extended release injections that can be helpful in chronic maintenance in non-adherent patients.


Cottage Physician on Hysteria

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Cottage Physician, 1893.


References

  1. Vigo D, Thornicroft G, Atun R. Estimating the true global burden of mental illness. Lancet Psychiatry. 2016;3(2):171-8. [pubmed]
  2. McGrath J, Saha S, Chant D, Welham J. Schizophrenia: a concise overview of incidence, prevalence, and mortality. Epidemiologic Reviews. 2008;30:67-76. [pubmed]
  3. Abel KM, Drake R, Goldstein JM. Sex differences in schizophrenia. International Review of Psychiatry. 2010;22(5):417-28. [pubmed]
  4. Sham PC, MacLean CJ, Kendler KS. A typological model of schizophrenia based on age at onset, sex and familial morbidity. Acta Psychiatrica Scandinavica. 1994;89(2):135-41. [pubmed]
  5. Grossman LS, Harrow M, Rosen C, Faull R, Strauss GP. Sex differences in schizophrenia and other psychotic disorders: a 20-year longitudinal study of psychosis and recovery. Comprehensive Psychiatry. 2008;49(6):523-9. [pubmed]
  6. Usall J, Ochoa S, Araya S, Márquez M, . Gender differences and outcome in schizophrenia: a 2-year follow-up study in a large community sample. Journal of the Association of European Psychiatrists. 2003;18(6):282-4. [pubmed]
  7. Schizophrenia Working Group of the Psychiatric Genomics Consortium. Biological insights from 108 schizophrenia-associated genetic loci. Nature. 2014;511(7510):421-7. [pubmed]
  8. Owen MJ, Sawa A, Mortensen PB. Schizophrenia. Lancet. 2016;388(10039):86-97. [pubmed]
  9. Thomas P, Mathur P, Gottesman II, Nagpal R, Nimgaonkar VL, Deshpande SN. Correlates of hallucinations in schizophrenia: A cross-cultural evaluation. Schizophrenia Research. 2007;92(1-3):41-9. [pubmed]
  10. Andreasen NC, Olsen S. Negative v positive schizophrenia. Definition and validation. Archives of General Psychiatry. 1982;39(7):789-94. [pubmed]
  11. Nuechterlein KH, Barch DM, Gold JM, Goldberg TE, Green MF, Heaton RK. Identification of separable cognitive factors in schizophrenia. Schizophrenia Research. 2004;72(1):29-39. [pubmed]
  12. Gold JM, Hahn B, Strauss GP, Waltz JA. Turning it upside down: areas of preserved cognitive function in schizophrenia. Neuropsychology Review. 2009;19(3):294-311. [pubmed]
  13. Heinrichs DW, Buchanan RW. Significance and meaning of neurological signs in schizophrenia. The American Journal of Psychiatry. 1988;145(1):11-8. [pubmed]
  14. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), American Psychiatric Association, Arlington, VA 2013.
  15. Buchanan RW, Kreyenbuhl J, Kelly DL. The 2009 schizophrenia PORT psychopharmacological treatment recommendations and summary statements. Schizophrenia Bulletin. 2010;36(1):71-93. [pubmed]

PAINE PANCE Pearl – Psychiatry

The DSM-V diagnostic criteria for autism spectrum disorders includes all of the following:

  1. Persistent deficits in social communication and interactions in multiple settings with each of the following:
    1. Social-emotional reciprocity
    2. Nonverbal communicative behaviors used for social interaction
    3. Developing, maintaining, and understanding relationships
  2. Restricted, repetitive patterns of behavior, interests, or activities as demonstrated by at least 2 of the following:
    1. Stereotyped or repetitive movements, use of objects, or speech
    2. Insistence on sameness, unwavering adherence to routines, or ritualized patterns of behavior
    3. Highly restricted, fixated interests that are abnormal in strength or focus
    4. Increased or decreased response to sensory input or unusual interest in sensory aspects of the environment
  3. The symptoms must impair function.
  4. The symptoms must be present in the early developmental period. However, they may become apparent only after social demands exceed limited capacity; in later life, symptoms may be masked by learned strategies.
  5. The symptoms are not better explained by intellectual disability or global developmental delay.

The severity of social communication/interaction and restricted/repetitive behavior can be graded using the following

  1. Level 1 – Requiring Support
  2. Level 2 – Requiring Substantial Support
  3. Level 3 – Requiring Very Substantial Support

Testing Tools Available

  • Autism Behavior Checklist (ABC)
    • 57 questions
    • 5 categories
      • Sensory, elating, body and object use, language, and social and self-help
  • Gilliam Autism Rating Score (GARS-3)
    • 56 questions
    • 6 categories
      • Restrictive/repetitive behaviors, social interaction, social communication, emotional responses, cognitive style, and maladaptive speech
  • Autism Diagnostic Interview-Revised (ADI-R)
    • 2-3 hour clinical interview
  • Childhood Autism Rating Scale – Second Edition (CARS-2)
    • 15 item focused interview
  • Autism Diagnostic Observation Schedule – Second Edition (ADOS-2)
    • Reference standard in research

References

  1. American Psychiatric Association. Autism spectrum disorder. In: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Association, Arlington, VA 2013. p.50.
  2. Krug DA, Arick J, Almond P. Behavior checklist for identifying severely handicapped individuals with high levels of autistic behavior. Journal of Child Psychology and Psychiatry, and Allied Disciplines. 1980;21(3):221-9. [pubmed]
  3. Gilliam JE. Gilliam Autism Rating Scale (GARS). Pro-Ed, Austin, TX 1995.
  4. Lord C, Rutter M, Le Couteur A. Autism Diagnostic Interview-Revised: a revised version of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders. Journal of Autism and Developmental Disorders. 1994;24(5):659-85. [pubmed]
  5. Filipek PA, Accardo PJ, Baranek GT. The screening and diagnosis of autistic spectrum disorders. Journal of Autism and Developmental Disorders. 1999;29(6):439-84.[pubmed]

PAINE PANCE Pearl – Psychiatry

You are seeing an 8yo with his mother for increasing behavioral problems and decreasing school performance.  She is concerned about ADHD because several of his friends have been diagnosed and are now on medications and doing well.  On physical exam, he is not making consistent eye contact in the room, shows poor impulse control, and is does not want to speak with you when you ask him questions.  You are now entertaining the possibility of an autism spectrum disorder.

 

What are the DSM-V diagnostic criteria for an autism spectrum disorder and what are some of the available testing tools?