Neurologica DiabolicaDr Wallis

Middlemore Medical SocietyMillbrook 2010

Case 1Doctor presents for a 3rd neurological opinion

regarding recurrent blackouts preceded by a gustatory aura. Past history of unexplained haematemesis. Exam, EEG, and CT scan normal. No response to anticonvulsant Rx.

Admitted to hospital. EEG normal during “fit”. Observed attacks are not epileptic. Other records emerge and disclose repeated admission to other hospitals for fits and status epilepticus. Multiple tests and lumbar punctures normal. On one occasion ended up in ICU where he sustained a pneumothorax from interventions.

Blood from emesis “not human, possibly bovine”Presents rifle (unloaded) and threatens to kill me. Arrested.Suspended by Medical Council. Suicide years later.

Case 2• Doctor’s wife with recurrent blackouts and

multiple hospital visits. Low blood sugars.• A lumbar puncture is normal except CSF

glucose is 0. Insulinoma suspected.• Admitted to neurology service where nurses

find insulin vial in her locker.• Radioactive-labelled insulin added to vial and

traces found in patient later.• Confronted with facts. Refuses to see

psychiatrist and disappears from radar

Case 3

• 11 year old boy admitted to neurosurgery with multiple unexplained episodes of coma. Lumbar puncture and angiogram normal.

• Examination during comatose episode shows features of metabolic encephalopathy.

• Benzodiazepines in toxicology screen.• Mother works as cleaner in hospital. Nurses find

diazepam tablets in her housecoat. She agrees to see psychiatrists and social workers.

Case 4• Blue collar worker repeatedly admitted obtunded or

comatose to hospital. • Usually found stuporous or unconscious sitting in front of

TV. Scans and lumbar punctures normal. Neurological exam normal. Referred with Dx “Recurrent idiopathic coma”.

• Simple amiable man. Wife is a switched on accountant and calls soon after another episode. Finds “white powder” at bottom of his tea cup next to TV. Analysis: benzodiazepine, which is also found in his hospital toxicology screen

• Wife locates lorazepam tabs in daughter’s belongings. Girl has rheumatoid arthritis and attends doctors frequently.

• Family all agree to go to psychiatrist but outcome of exercise is “sensitive and confidential”

Neurologica Diabolica Is Part Of Which Syndrome?

Who first described it?

Munchausen SyndromeTerm coined by endocrinologist

Richard Asher.Asher was also known for what he

called the “7 Sins of Medicine”

• Obscurity • Cruelty • Bad Manners • Over-specialisation • Love of the Rare • Common Stupidity • Sloth

1912-1969

† By his own hand

Munchausen SyndromeAsher described the common features of his patients as:• Repeatedly seeking admission into medical facilities

under apparent physical or mental distress, offering plausible stories supporting the nature of their disorder.

• Once admitted, they may submit themselves to radical interventions.

• They leave against medical advice, often after exaggerated arguments with the medical staff.

• They later turn up in other facilities with the same or other phenomena

• Named syndrome after Baron von Munchausen. Asher R. Munchausen's syndrome. Lancet

1951;1:339-341)

Why did Asher call it Munchausen syndrome?

Why Munchausen Syndrome?

Asher selected "Munchausen's syndrome" because of the similarity between the wanderings and fabrications of these patients and the travels and fantastic anecdotes attributed to Baron von Munchausen (1720-1797). But here the similarity ends, for the Baron never submitted himself to medical operations and procedures as these patients do.

Who was Munchausen?Doppelgängers: one real and one mythical

The authentic Baron Freiherr Karl Friedrich Hieronymus von Münchhausen (note correct spelling) was a German veteran of the Russian wars against the Turks.

He was well-known in European social

circles for his entertaining accounts of his military exploits, and was said to be prone to exaggeration.

A series of such droll tales were published in

the German periodical from 1781 to 1783. Vade Mecum für lustige Leute Wunderbare Reisen zu Wasser und

Lande, Feldzuge und lustige Abentheuer . . . des Freyherrn von Munchhausen-aus dem Englischen ... ubersetzt.

much to the embarrassment of the Baron

Who was the mythical Munchausen?

The Adventures of Baron Munchausen, one of the most famous books of tall tales, is attributed to Rudolf Erich Raspe, who in 1787 produced

an affidavit supporting veracity of his tales, purportedly signed by Lemuel Gulliver, Sinbad the Sailor, Aladdin, and the Lord Mayor of

London,

Munchausen’s Adventures

Forms of Munchausen

• Classical Munchausen (cases 1 & 2)

• New Age Munchausen

• Munchausen by proxy (cases 3 & 4)

Classical Munchausen

• Laparotomaphilia migrans • Hemorrhagica histrionica

• Dermatitis autogenetica

• Neurologica Diabolica

• Pseudologia phantastica

Classical Forms of Munchausen

Laparotomaphilia migrans, distinguished by repeated abdominal symptoms and laparotomies; alleged to be the commonest type

Dermatitis autogenetica, characterized by self-inflicted skin lesions or rashes

Tools of the trade

Hemorrhagica histrionicarecurrent bleeding without explanation

Neurologica Diabolica

Fake seizures, paralysis, blindness, and self-poisoning

Pseudologia phantasticatype 1

The patient makes false claims about distinguished accomplishments, educational credentials, relations to famous persons, etc. False grieving over non-exixtent friends’ or relatives’ maladies.

Pseudologia phantasticatype 2

Feigning psychiatric illness

Other Classical Types Munchausen• Cardiac type characterized by various symptoms

associated with cardiovascular distress and use cardiac stimulants (usually caffeine and enhanced by fake ECGs)

• Ophthalmic type caused by instilling caustic substances into conjunctiva

• Febrile type caused by the ingestion of drugs, self-inoculation of contaminated material, and changing or tampering with thermometers

• Hypoglycaemic type with insulin or sulfonylureas• Reflux or gastric type by ingestion corrosive agents

and animal blood• Metabolic type by ingestion of hypertonic salt sollutions

and sedatives

Aetiology Classical Munchausen

Motivation is unknown but several explanations offered:

• underlying organic problem• substance abuse• resentment of physicians,• childhood deprivation and rejection • subintentional death wishes• castration fantasies• relief of aggression and guilt through

operations, and• seeing the physician as a father figure.

Terms Describing Personality Characteristics in Munchausen

• Aggressiveness• Ambivalence• Antisocial nature• Anxiety• Attention-seeking behavior• Brillance• Childishness• Cleverness• Deficient sense of reality• Depression• Drama• Egocentricity• Emotional lability• Evasiveness• Ability to act• Histrionics• Ability to act • Ability to act

•Intellectualizations•Disturbance of identity•Impulsiveness•Inadequate social functioning•Intensity of affect•Loneliness•Manipulativeness•Masochism•Nomadic behavior•Paranoia•Pathologic lying•Restlessness•Self-destructive behavior•Suspiciousness•Unruliness•Lack of stability in interpersonal relations

Aetiology Classical Munchausen (cont.)

Currently believed that different motives operate indifferent patients and even in the same patients onseparate occasions. Ultimately, the disorder probablyrepresents a final common pathway for predisposedpersons.

(i.e. nobody has the faintest clue)

But…….

Munchausen's syndrome gained official recognition in the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders (DSM III) (1980)

New Age Munchausen(Not described or dreamed of by Asher )

• Munchausen by Google (Virtual Munchausen)

• HIV Munchausen

• Veterinary Munchausen

Munchausen by Google(Virtual Munchausen)

• Download X-Rays and scans from Google Science and present them as their films

• Join specialised internet groups and appropriate their ailments. With the exponential increase in the number of people with internet access, "virtual" support groups have multiplied. Many of them, conducted by e-mail or internet postings and sometimes requiring a subscription, have been established for persons with particular diseases. (ie “fibromyalgia, chronic fatigue syndrome, some cancers)

HIV Munchausen Feigned HIV, in which people mimic infection with or

disease due to HIV, accounted for 1.7% of admissions to a specialist HIV unit in Central London over a 5 year period. 12 patients feigned HIV/AIDS, 11 were HIV antibody-negative, and one refused testing.

Presenting histories were sometimes grandiose, unusually tragic, or unlikely in relation to the patients' healthy appearance, and often included admissions to other specialist HIV units. Substance abuse was suspected in over half of the patients described, a higher frequency than that observed in our HIV-infected patient population

Veterinary Munchausen

“Shouldn’t he be lying at the foot of the bed?”“Here he is again. Poor Fido.”

Differential diagnosis(1) Authentic physical disorder

(2) Malingering: conscious, volitional, situationally appropriate behaviour aimed toward clear secondary gain

(3) Somatoform: (aka hysteria) nonorganic illness in which the signs and symptoms are non-volitionally simulated and secondary to unconscious drives and for which there is no apparent secondary gain

Munchausen by Proxy(Cases 3 and 4)

Plagiarised from classical Munchausen and adapted to apply to someone else (usually children) who presented with a false illness or story invented caused by someone else (a proxy). First described by Meadow (whom we shall hear more about soon) in 1977.

• Illness in a child which is fabricated by a parent, or someone who is in loco parentis.

• The child is presented for medical assessment and care, usually persistently, often resulting in multiple medical procedures.

• The perpetrator denies the aetiology of the child's illness.

• Acute symptoms and signs of illness cease when the child is separated from the perpetrator (or victim dies)

Types of Munchausen by Proxy

• recurrent apnoea (smothering)• fictitious epilepsy (reported by proxy)• false history and fabrication of lab samples • false history and repetitive poisoning (hypoglycaemics,

anticoagulants, sedatives, salt solutions) Proxies are usually but not exclusively mothers (Mommy

Munchausen). May act when unobserved in hospital. Trapped by video surveillance or less convincingly by deductive reasoning. If the premises are true the conclusion must also be. ie 3 of Mrs Smith’s children have died unexpectedly. This cannot be by chance or known disease, so maybe they died of unnatural causes.

Munchausen by Proxy• Problematic term, as victim is innocent and

motivations of proxy may vary from mildy mischievous to murderous ie :

(1)Transfer of proxy’s emotional “needs” to the victim( “true” Munchausen by proxy)

(2) Abuse (3) Criminal: resulting in death or maiming. Of note Mommy Munchausens are regarded by

legal system as “crying for help” and until recently usually escaped jail. Daddy Munchausens usually jailed. Often more than one victim in the family. Coexisting vet Munchausen common.

Munchausen by proxy can backfire• Professor Sir Roy Meadow

endorsed the dictum that “one sudden infant death is a tragedy, two is suspicious and three is murder, until proved otherwise.

• testified that the odds against two cot deaths occurring in the same family was 73,000,000:1. Wrong. The Royal Statistical Society issued a press release stating that the figure had "no

statistical basis" Estimates are mostly in the region of 1:100.

• Meadow struck off but eventually managed to have decision reversed but not before he was discredited.