DR.SRIRAM.R

UNCOMMON PSYCHIATRIC DISORDERS

DELUSIONAL MISIDENTIFICATION SYNDROMES (DMS)

OTHER MONOTHEMATIC DELUSIONSALIEN HAND SYNDROMEALICE IN WONDERLAND SYNDROMEJERUSALEM SYNDROMEPARIS SYNDROMEFUGUE STATEFOREIGN ACCENT SYNDROMESTOCKHOLM SYNDROME

Following, but not limited to -

LIMA SYNDROMESTENDHAL SYNDROMEDIOGENES SYNDROMEMUNCHAUSEN SYNDROMEMUNCHAUSEN SYNDROME BY PROXYAPOTEMNOPHILIAACROTOMOPHILIA

1.CAPGRAS SYNDROMEPerson holds a delusion that a friend, spouse,

parent, or other close family member has been replaced by an identical-looking impostor

Capgras syndrome is named after Joseph Capgras (1873–1950), a French psychiatrist who described the disorder in 1923 and called it “l’illusion des sosies”

Occurs in – paranoid schizophrenia, post brain injury, neurodegenerative conditions and dementia, diabetes, hypothyroidism, migraine, post-ketamine

F : M = 3:2

DELUSIONAL MISIDENTIFICATION SYNDROMES (DMS)

Reason - ?A disconnection between the amygdala and inferotemporal cortex (VS Ramachandran)

Facial recognition involves a conscious (how people look, sound) vs. unconscious (beliefs, emotions, preferences, personalities) pathway

Prosopagnosia (emotional arousal intact) vs. Capgras syndrome (problem with emotional arousal)

Treatment – Individual psychotherapy and antipsychotics

2. SYNDROME OF SUBJECTIVE DOUBLESPerson experiences the delusion that he or she has a

double or Doppelgänger with the same appearance, but usually with different character traits, that is leading a life of its own

First defined in 1978 by Greek psychiatrist George N. Christodoulou

Defining features of the delusion:- The existence of the delusion, by definition, is not a widely

accepted cultural belief.- The patient insists that the double he/she sees is real even

when presented with contradictory evidence.- Paranoia and/or spatial visualization ability impairments are

present.

May be seen associated with paranoid schizophrenia, bipolar depression, substance dependence, epilepsy, traumatic brain injury

Interpersonal counseling and antipsychotics are the treatments of choice

Variations of this include “Clonal pluralization of the self” where the other person is both physically as well as psychologically the same

Not defined in both ICD-10 as well as DSM-IV

3.INTERMETAMORPHOSISFirst described in 1932 by P. Courbon and J. Tusques

(Illusions d'intermétamorphose et de la charme)The main symptoms consist of patients believing

that they can see others change into someone else in both external appearance and internal personality

 The disorder is usually comorbid with neurological disorders or mental disorders eg. Alzheimer’s

Misidentification is present even when the person makes a phone call to the person he believes as someone else

4.FREGOLI SYNDROMEPerson holds a delusional belief that different people

are in fact a single person who changes appearance or is in disguise, and is paranoid in nature

Named after the Italian actor Leopoldo Fregoli, who was renowned for his ability to make quick changes of appearance during his stage act

Causes – Antiparkinsonism treatment, TBI to the right frontal and left temporo-parietal areas, fusiform gyrus

Treatment – Antipsychotics (trifluperazine), anticonvulsants and antidepressants

ALL OF THE DMS are monothematic, as they are delusions that concern only one topic

Others included in this category are1.COTARD SYNDROMEa.k.a Walking Corpse Syndrome is a delusional

belief that they are dead (either figuratively or literally), do not exist, are putrefying, or have lost their blood or internal organs

In rare instances, it can include delusions of immortality

 It is not listed in DSM-IV, DSM-IV-TR, nor ICD-10

OTHER MONOTHEMATIC DELUSIONS

3 distinct stages : In the first stage – Germination – patients exhibit psychotic depression and hypochondriacal symptoms. The second stage – Blooming – is characterized by the full blown development of the syndrome and the delusions of negation. The third stage – Chronic – is characterized by severe delusions and chronic depression

Causes – TBI to the parietal lobe, fusiform area, schizophrenia, migraine, valacyclovir (metabolite CMMG)

Rx – Antidepressants, antipsychotics, mood stabilizers, also ECT plus pharmacotherapy, hemodialysis (renal failure secondary to antivirals)

COTARD’S (contd.)

2. MIRRORED SELF-MISIDENTIFICATIONDelusional belief that one's reflection in a mirror is

some other person (often believed to be someone who is following one around)

People may also treat their reflection as a companion or become indifferent to them

Many people mask their mirrors in order to evade who they believe to be a stranger

Along with masking their mirrors some throw objects at the mirrors

CAUSES – Impaired face perception and mirror agnosia

Seen in dementia, stroke, TBI and neurological illness

3.REDUPLICATIVE PARAMNESIADelusional belief that a place or location has been

duplicated, existing in two or more places simultaneously, or that it has been 'relocated' to another site

most commonly associated with acquired brain injury, particularly simultaneous damage to the right cerebral hemisphere and to both frontal lobes

first used in 1903 by neurologist Arnold Pick to describe a condition in a patient with suspected Alzheimer's disease

Also seen instroke,intracerebral hemorrhage, tumor, dementia, encep

halopathy and various psychiatric disorders

4.SOMATOPARAPHRENIAPerson denies ownership of a limb or an entire side of

one's bodyEven if provided with undeniable proof that the limb

belongs to and is attached to their own body, the patient produces elaborate confabulations about whose limb it really is, or how the limb ended up on their body

occur predominately in the left arm of one's body accompanied by left-sided paralysis and anosognosiaCAUSE – Damage to posterior cerebral temporo-parietal

junctionsTreatment – Mirror therapy, but condition persists after

the mirror is taken away

Appeared in a detailed case report published in German in 1908 by German neuro-psychiatrist, Kurt Goldstein

Goldstein described a right-handed woman who had suffered a stroke affecting her left side from which she had partially recovered by the time she was seen. However, her left arm seemed as though it belonged to another person and performed actions that appeared to occur independent of her will

Afflicted people lost the 'sense of agency' associated with the purposeful movement of the limb while retaining a sense of 'ownership' of the limb.

ALIEN HAND SYNDROME/ANARCHIC HAND

Affected hand is viewed as "wayward" or "disobedient," while the unaffected hand is under normal volitional control

Damage to the corpus callosum produces “intermanual conflict” or “ideomotor apraxia”

Caused by stroke or other brain damage, particularly in the areas of the corpus callosum, or frontal or parietal lobes, AD or CJD

There is no cure for the alien hand syndrome. However, the symptoms can be reduced and managed to some degree by keeping the alien hand occupied and involved in a task, for example by giving it an object to hold in its grasp, warm water application, biofeedback, soft foam orthosis etc

In reference to an influential description of the condition by John Todd (1914-1987) in 1955, a British psychiatrist who worked in Yorkshire

The optical system is entirely physically normal. The AIWS involves a change in perception as opposed to a malfunction of the eyes themselves

Patient's sense of body image, space, and/or time are distorted. Sufferers may experience micropsia or Lilliputian hallucinations, macropsia, or size distortion of other sensory modalities, which includes also an altered sense of velocity, produced by the distorted sense of size, perspective, and time

ALICE IN WONDERLAND SYNDROME/TODD SYNDROME

The sufferer will find that he is confused as to the size and shape of parts of (or all of) his body. These symptoms can be alarming, causing fear, even panic. Distortions can recur several times a day and may take some time to abate

Associated with migraines, brain tumors, or the use of psychoactive drugs (Muscimol) and can also present as the initial sign of the Epstein-Barr virus or during high fever

 Rest is the best treatment. If associated with migraines, treatment is the same as that for other migraine prophylaxis, including anticonvulsants, antidepressants, beta blockers, and calcium channel blockers, together with strict adherence to the migraine diet.

The Jerusalem syndrome is a group of mental phenomena involving the presence of either religiously themed obsessive ideas, delusions or other psychosis-like experiences that are triggered by a visit to the city of Jerusalem.

It is not endemic to one single religion or denomination but has affected Jews, Christians and Muslims of many different backgrounds.

First clinically described in the 1930s by Jerusalem psychiatrist Heinz Herman, one of the founders of modern psychiatric research in Israel

JERUSALEM SYNDROME

Bar-El et al. classified the syndrome into three major typesType I - Jerusalem syndrome imposed on a previous psychotic

illnessType II - Jerusalem syndrome superimposed on and complicated

by idiosyncratic ideasType III - Jerusalem syndrome as a discrete form,

uncompounded by previous mental illness. This describes the best-known type, whereby a previously mentally balanced person becomes psychotic after arriving in Jerusalem. The psychosis is characterised by an intense religious character and typically resolves to full recovery after a few weeks or after being removed from the locality. It shares some features with the diagnostic category of a "brief psychotic episode“

Rx – Antipsychotics , with careful withdrawal after cessation or psychosis/long term Rx in people with co-existing psychosis

Professor Hiroaki Ota, a Japanese psychiatrist working in France, is credited as the first person to diagnose the condition in 1986

Japanese visitors are observed to be especially susceptible. Around twenty Japanese tourists a year are affected by the syndrome out of the 6 million who visit.

Characterized by a number of psychiatric symptoms such as acute delusional states, hallucinations, feelings of persecution, derealization, depersonalization, anxiety, and also psychosomatic manifestations such as dizziness, tachycardia, sweating, and others

PARIS SYNDROME

Hypotheses why Japanese people are affected include their apparent suggestibility regarding an idealized image of Paris, but the confrontation with very different cultural habits, a strong language barrier, and physical and mental exhaustion have also been suspected as triggers

Psychotherapeutic and supportive approaches should be used as treatment

Fugue state, previously also called dissociative fugue or psychogenic fugue, is a rare psychiatric disorder characterized by reversible amnesia for one's personal identity, which includes the memories, personality, belongings and other identifying characteristics of one's individuality and life

Usually, the fugue state lasts hours to days, but it has lasted for months.

Precipitated by a strong emotional or physical stressor or stressful episode. After recovery from the fugue, there may be amnesia for the precipitating stressor.

FUGUE STATE

Dissociative fugue usually involves unplanned travel or wandering around, sometimes accompanied by the establishment of a new identity

Previous memories usually return intact, but usually there remains complete amnesia for the fugue.

observed in the context of severe psychological or physical trauma, the ingestion of psychotropic substances, or a general medical condition. It has also been related to bipolar disorder, depression, delirium, and dementia

Psychotherapeutic and supportive approaches should be used as Rx

The condition was first described in 1907 by the French neurologist Pierre Marie

The foreign accent syndrome is a rare condition whereby someone speaks their native language as if they had a foreign accent

This syndrome usually follows a migraine, head injury, trauma, or stroke affecting the speech center of the brain

 Cerebellum may be involved in certain casesContrary to popular beliefs that individuals with FAS

exhibit their accent without any effort, these individuals feel as if they are suffering from a speech disorder

FOREIGN ACCENT SYNDROME

Named after a bank robbery in Stockholm, Sweden. The bank robbers held bank employees hostage from August 23 to August 28 in 1973 and the hostages became emotionally attached to their hostage-takers. They even defended their captors after they were freed, refusing to testify against them.

A psychological response that can be observed seen in a victim, in which the victim shows signs of sympathy, loyalty, or even voluntary compliance with the victimizer, regardless of the risk in which the victim has been placed.

The syndrome is most often discussed in the context of hostage abduction, but has also been described in relationship to rape, spousal and child abuse

STOCKHOLM SYNDROME/CAPTURE BONDING

A famous example of Stockholm syndrome is Patty Hearst. She was a millionaire's daughter who was kidnapped in 1974 and later took part in a robbery organized by her and her kidnapper

Severe form of reaction formation that takes place under enormous physiologic and emotional stress

As in all cases of severe trauma, psychotherapeutic and supportive approaches should be used, and comorbid conditions should be identified and managed as appropriate.

Exact inverse of Stockholm syndrome. In this case, hostage-takers or victimizers become sympathetic to the wishes and needs of the hostages or victims

Named after the Japanese embassy hostage crisis in Lima, Peru, that lasted from December 17, 1996 until April 22, 1997

Within a few days of the hostage crisis, the militants had released most of the captives, with seeming disregard for their importance, including the future president of Peru, and the mother of the current president

LIMA SYNDROME

Named after the famous 19th-century French author Stendhal (pseudonym of Henri-Marie Beyle), who described his experience with the phenomenon during his 1817 visit to Florence

Characterized by physical and emotional anxiety up to the level of a panic attack, dissociative experiences, confusion, and even hallucinations when an individual is exposed to ART

The syndrome is usually triggered by art that is perceived as particularly beautiful or when the individual is exposed to large quantities of art that are concentrated in a single place

Stendhal syndrome is self-limited and not followed by lasting or severe mental sequelae

STENDHALSYNDROME/HYPERKULTUREMIA/FLORENCE SYNDROME

Disorder characterized by extreme self-neglect, domestic squalor, social withdrawal, apathy, compulsive hoarding of garbage, and lack of shame and catatonia.

The origin of the syndrome is unknown, although the term “Diogenes” was coined by A. N. G. Clarke et al. in the mid‑1970s and has been commonly used since then.

The name derives from Diogenes of Sinope, an ancient Greek philosopher, a Cynic and an ultimate minimalist, who allegedly lived in a large jar in Athens

DIOGENES SYNDROME/SENILE SQUALOR SYNDROME

These symptoms suggest damages on the prefrontal areas of the brain, due to its relation to decision making.

The frontal lobes are of particular interest, because they are known to be involved in higher order cognitive processes, such as reasoning, decision-making and conflict monitoring.

Diogenes Syndrome tends to occur among the elderly with dementias.

Results after hospitalization tend to be poor. There are other approaches to improve the patient’s

condition. Day care facilities have often been successful with maturing the patient’s physical and emotional state, as well as helping them with socialization.

Psychiatric factitious disorder wherein those affected feign disease, illness, or psychological trauma to draw attention, sympathy, or reassurance to themselves.

They also have a history of recurrent hospitalization, travelling, and dramatic, untrue, and extremely improbable tales of their past experiences.

This disorder is distinct from hypochondriasis and other somatoform disorders in that those with the latter do not intentionally produce their somatic symptoms.

MUNCHAUSEN SYNDROME/HOSPITAL ADDICTION SYNDROME/THICK CHART SYNDROME

Named after Baron von Munchausen (1720-1797), an 18th-century German officer who was known for embellishing the stories of his life and experiences.

Some will secretively injure themselves to cause signs like blood in the urine or cyanosis of a limb, ingest bacteria, etc

Patients may have multiple scars on abdomen due to repeated "emergency" operations

Risk factors for developing Münchausen syndrome include childhood traumas, growing up with parents/caretakers who were emotionally unavailable due to illness or emotional problems, a serious illness as a child, failed aspirations to work in the medical field, personality disorders, and a low self-esteem

Rx is CBT and/or pharmacotherapy and is based on cause.

Type of factitious disorder in which a person acts as if an individual he or she is caring for has a physical or mental illness when the person is not really sick.

Victim is usually a child <6yrs of age, and the parent may suffer from Munchausen syndrome, CHILD ABUSE present.

Common characteristics in a person with Munchausen syndrome by proxy include:

Often a parent, usually a mother, but can be the adult child of an elderly patient;

Might be a healthcare professional; Is very friendly and cooperative with the healthcare providers; Appears quite concerned (some might seem overly concerned)

about the child or designated patient; and Might also suffer from Munchausen syndrome

MUNCHAUSEN SYNDROME BY PROXY

Other possible warning signs of Munchausen syndrome by proxy in children or cared-for adults include:

• The child has a history of many hospitalizations, often with strange symptoms;

• The child's reported condition and symptoms do not agree with the results of diagnostic tests;

• Worsening of the child's symptoms generally is reported by the mother and is not witnessed by the hospital staff;

• There might be more than 1 unusual illness or death of children in the family;

• The child's condition improves in the hospital, but symptoms recur when the child returns home;

• Blood in lab samples might not match the blood of the child; and

• There might be signs of chemicals in the child's blood, stool, or urine.

People who perpetrate this type of abuse are often affected by concomitant psychiatric problems, like depression, spouse abuse, psychopathy, or psychosis.

Etiologic and treatment considerations are identical to those in Munchhausen syndrome. The major difference lies in the fact that the first concern is to ensure the safety and protection of any real or potential victims.

Erotic interest in being or looking like an amputee.First described in a 1977 article by psychologists Gregg

Furth and John Money.Associated with Body integrity identity disorder (BIID)

in which otherwise sane and rational individuals express a strong and specific desire for the amputation of a healthy limb or limbs.

Has features in common with somatoparaphrenia.Inadequate activation of the right superior parietal lobe

(SPL) leads to the unnatural situation in which the sufferers can feel the limb in question being touched without it actually incorporating into their body image, with a resulting desire for amputation

APOTEMNOPHILIA

After amputation most report to being happy with their decision and often state, paradoxically, that they are ‘complete’ at last.

An individual with true apotemnophilia may be chronically unsatisfied with their sexual relationships, or even completely sexually dysfunctional until their desire for amputation is realized and it is a paraphilia.

Apotemnophilia (sexual arousal present) vs. BIID (sexual arousal absent)

Apotemnophiles may have associated depression, isolation and confusion.

Rx is CBT, aversion therapy and/or pharmacotherapy.

Acrotomophilia is a form of sexual fetishism whereby the person without amputation or the wish to be amputated has a strong erotic interest in other people who are missing limbs.

In the body integrity identity disorder community, these people are referred to as “devotees”.

However, there might be some relationship between APOTEMNOPHILIA and this, with some individuals exhibiting both conditions.

Rx is essentially the same as that of apotemnophiles.

ACROTOMOPHILIA

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