“Je pense, donc je suis”

Download Report

Transcript “Je pense, donc je suis”

When Your Leg Just Isn’t
Your Leg!?
Body Integrity Identity Disorder
Alison Wighton
NSW PAR October 2008
Case Report Mr DO
 28
year old Caucasian male with history of
suicide attempts and requests for
amputation of his right leg
 Transferred to Concord Hospital on
10/03/2008 from Liverpool Hospital, where
he had presented with frostbite from
deliberate soaking of his right leg in a
bucket of ice for five hours.
History of Presenting Illness
 History
of abnormal feelings about the
right leg since age 4.
 Possibly related to a TV character with an
amputation to cause an attraction to
amputees?
 Age 7-13 thoughts of leg less urgent
 Denied any altered sensations, lack of
coordination, inattention injuries or motor
problems with the leg as a child
History of Presenting Illness






Age 13 - thoughts recurred and the urge to be
rid of the limb became intense
Did not feel his right lower leg was part of him
Accidentally tripped down a drain, injuring the
right leg in the exact place that he wanted
amputation
Attempted to infect leg by rubbing dirt into the
wound
Did not seek medical attention
Day-dreamed of leg falling off
History of Presenting Illness
 2006
- deliberately amputated the tip of his
right middle finger with a knife and
discarded the amputated piece
 This was to suppress his immense
devastating feelings with his ‘extra’ leg
 Managed at Liverpool Hospital with
antidepressant treatment
 Feelings suppressed for short time
History of Presenting Illness
 Couple
initiated research on the internet
 Self diagnosis of Body Integrity Identity
Disorder (BIID) late 2007
 Joined online support groups to learn how
to deal with the diagnosis
 Jan 2008 - Free trip to California arranged
by Granada Television for exclusive right
to an interview.
Investigations
 Jan
2008 - met Dr Ramachandran and Dr
McGeoch at UCSD
 Tested with MRI brain and magnetoencephalography
 MRI showed an unusually large right
superior temporal gyrus
 Volumetric analysis of his MRI confirmed
superior parietal lobule ratio right : left of
0.73
Investigations
 On
magneto-encephalography, touching
his right foot produced just primary and
secondary somatosensory activation but
no activity in the superior right parietal
lobe.
 Had caloric vestibular stimulation
 Partial relief if mirror was placed such that
it created illusion that leg was no longer
there.
History of Presenting Illness
 26/02/08
 On
returning to Sydney he saw
Psychiatrist at Westmead Hospital
 He agreed with classic natural history of
Body Integrity Identity Disorder
 Referral to RPA Hospital for second
opinion
 Preliminary discussion with Vascular
surgeon and Rehabilitation physician
History of Presenting Illness
 Unsatisfied
with progress trying to seek
amputation
 Took matter into own hands …….
History of Presenting Illness

10/03/08 Took some pain killers before
soaking his leg in a bucket of dry ice for 5 hours
 Presented to Liverpool hospital with (R) LL
frostbite injury and self diagnosis of Body
Integrity Identity Disorder

Given morphine for analgesia and Cephazolin

Transferred to Concord Hospital for
assessment….
Past Medical History
 MVA 1985-86
? Skull fracture
 History of migraine headache on and off
Medications
 Citalopram
months
20mg daily-for last three
Drug and Alcohol
 Drinks
average of 10g of alcohol per day
 Up to 100g at a sitting
 2001-2006 used Cannabis
 No other illicit drugs and never smoked
tobacco
Psychosocial History
 Unemployed,
receives parenting pension
 Previously worked in series of low skilled
occupations
 Lives with his de facto wife and their four
children (12,10,6,4) in a Dept Housing
property
 Partner receives Austudy allowance
Childhood
 Parents
divorced when he was seven
 Father remarried a woman he did not like
 Unstable and complicated upbringing
 Diagnosed with Attention Deficit Disorder
at age 7
 Short term treatment with Amphetamine
 Left school in year 10
Stressors
 1999
- mother murdered by her boyfriend
by beating her unconscious and then
burning house down with her in it. (19yrs)
 2000
- brother got him to unknowingly hold
stolen goods leading to imprisonment
Suicide Attempts
 1999-attempted
cutting his wrist in
response to mother’s death.
Treatment Course
10/03/08
 Pain management
 Peripheral foot perfusion checks 4/24
 Probably unlikely to require surgery
 Psychiatry consult
Imaging
 CT
Brain-NAD
 CXR under-inflated lungs with bibasal
collapse
 MRI Brain-normal
 SPECT Brain-normal
If you’re not good with blood and all things a
bit yucky……
LOOK AWAY NOW
17/03/2008
17/03/2008
 Blood
cultures-gram negative rods in 4/4
bottles
 Wound-heel pad gangrenous
 Commenced on Gentamycin and
Ceftazidine
Opinions
Rehabilitation team (Dr Ross Hawthorne)
 Extensive necrosis of heel pad, no benefit
from trying to save the foot or Syme’s
amputation.
 Supported trans-tibial amputation at the
level desired by the patient.
 Burns team supported the medical
indication for below knee amputation.
Opinions
Vascular team
 Agree with need for amputation, wait until
necrotic area fully demarcates
 Further positive cultures → gram positive
cocci- staph and strep
 Commenced on Vancomycin
19/03/2008
 Heel
necrosis worse and malodorous
 Right foot swollen and cellulitic up to mid
shin
 Cultures growing Staph aureus,
Enterococcus and Pseudomonas
 Commenced on Tazocin
20/03/2008
 Calf
muscle perfusion scan - non viable
right gastrocnemius muscle
21/03/2008
 Right
trans tibial amputation
 No post operative complications
The Result
Rehabilitation Phase
 Developed
Phantom limb pain
 Treated with Doxepin by Pain team and
patient educated about stump massage
 Rigid removable dressing commenced for
stump management
 Progressed well and became independent
with his LL and UL exercises and mobility
with crutches.
Function at Discharge
 Independent
with self care
 Independent stump care
 Independent mobility with crutches
 Home visit was conducted with OT
 Little equipment required for safe
discharge to Aunt’s house on 17/04/2008
 Prescription for interim prosthesis made
prior to discharge.
Attitude Since Amputation
 Feels
a weight lifted of his chest
 Wants to return to normal life and activities
 Feels no longer belongs to the BIID group
 States expectations have been met
 Has found acceptance from family
members by explaining BIID as
neurological condition
Physiotherapy Progression
 Was
quick to progress to independent
mobility with prosthesis unaided.
 Was starting to learn to run, however
attendance at outpatient physio has been
unreliable.
 Now is happy with current abilities and
finds he can play with kids at the park etc.
Body Integrity Identity Disorder
(BIID)
 Apotemnophilia,
or body integrity identity
disorder (BIID), is characterized by a
feeling of mismatch between the internal
feeling of how one’s body should be and
the physical reality of how it actually is.
Body Integrity Identity Disorder
(BIID)

The desire for amputation of a healthy limb was
first reported in 1785
(cited in Johnston &
Elliott, 2002)

Money et al (1977) used the term
apotemnophilia (amputation love) to describe
intense and intrusive thoughts to amputate a
lower extremity. These thoughts were related to
sexual fantasies and sexual arousal.
Sex Res1977;13:115-25)

Description of this disorder was limited to a few
case reports from 1977-2003
Body Integrity Identity Disorder
(BIID)
 Long
standing desire to be an amputee
 Rare, mainly men
 Often arises around 4 – 5 yrs age
 Often accompanied by sexual arousal but
not necessarily primary motive
 Can
arise in women
 Extremes….
BIID
 Patients
with this condition have an often
overwhelming desire for an amputation of
a specific limb at a specific level.
 Such patients are not psychotic or
delusional
 Such patients show a left - sided
preponderance for their desired
amputation
Apotemnophilia and Munchausen’s
Syndrome.

Munchausen's patient is obsessed with self
inducing symptoms repetitively for the sake of
being a patient where as an apotemnophile is
supposedly satisfied with just one amputation

Apotemnophiles need only one medical
intervention that leaves them with obvious
stigma of disability which will permanently satisfy
their need for love and attention.
Factitious Disability Disorder
 Bruno
1997- divided this disorder into 3
subsets



Devotees
Pretenders
Wannabes
Devotees
 Devotees
are non disabled people who
are sexually attracted to people with
disabilities, typically those with mobility
impairments and amputees
Pretenders
 Pretenders
are non-disabled people who
live as if they have a disability.
 Pretender paraplegics can confine
themselves to their chairs full time and
never walk.
 The pretender amputee has more difficulty
trying to be an amputee and feels
frustrated and dissatisfied.
Wannabes
 Wannabes
are usually non-disabled
individuals that want to become someone
with a physical disability.
 See themselves in bodies that are not fully
functioning.
 They have difficulty finding identity.
BIID
 The
first person to use the term BIID was
US psychiatrist Associate Professor
Michael First from Columbia University,
who interviewed 52 ‘wannabes’ as part of
a recent study.
The Results
 90%
had education beyond high school
 65% were currently employed.
 27% had surgical or self inflicted
amputation
 17% had major limb amputation and two
thirds had used methods that put
themselves at high risk
The Results

He found that 15% of wannabes identified
sexual arousal as a reason for amputation, 63%
wanted to be restored to their "true identity" and
37% said the limb "felt different".
 Thirteen percent said the limb didn't feel like
their own and six people had tried to perform
their own amputation, including using a
chainsaw.
 87% reported being sexually attracted to other
amputees.
Desired Location for Amputation
 95%
wanted an amputation of major limb
 92% wanted above knee amputation
 55% wanted left sided amputation
 In 77% the site of desired amputation was
fixed since it started in childhood.
The Results

Most felt the somatosensory perception of the
limb did not differ from that of their other limbs.
 65% had onset prior to age 8; and 98% had
onset by age 16 years.
 Majority reported exposure to an amputee in
childhood.
 44% of First’s subjects reported that their desire
interfered with social functioning, occupational
functioning, or leisure activities.
Co-morbid Psychopathology
 Three
quarters reported having had
psychiatric condition sometime in their
lives.
 Most commonly depression, anxiety and
somatoform disorder.
Treatment Efficacy
 65%
had psychotherapy, for none of the
subjects it reduced the desire for
amputation
 40% were treated with psychotropic
medications - no appreciable effect from
the medication on the desire for
amputation
 12% patients had amputation at their
desired level
Causes of BIID
 There
is no one single causal factor for the
development of BIID.
 One theory states that a child, upon
seeing an amputee, may imprint his or her
psyche, and the child adopts this body
image as an "ideal".
 Another popular theory suggests that a
child who feels unloved may believe that
becoming an amputee will attract the
sympathy and love he or she needs.
Biological Theory
 BIID
is a neuro-psychological condition in
which there is an anomaly in the cerebral
cortex relating to the limbs. It could be
conceptualized as a congenital form of
somatoparaphrenia, a condition that often
follows a stroke affecting the parietal lobe
 Possibility of genetic basis

Research shows most of the BIID population
had experienced a significant childhood event.
 Can show up as early as 4 or 5 years old.
 Typically no change in the desire for amputation.

Participants who received amputation reported
after amputation, they feel better than ever and
lose the desire for further amputation.
Extreme Measures
 Because
most surgeons refuse to
amputate a healthy limb, some people with
BIID go to extreme measures to get rid the
limb.




Paying for surgery “under the table”
Homemade devices
Using ice, train tracks, electric saws, etc.
At home “accidents”
Treatment
 Medication
such as antidepressants help
little but can treat concurrent conditions
such as depression
 Most sufferers gain little help from
psychiatric and psychological therapy, it
helps to control the desire rather than to
abolish it.
Mirror Feedback Treatment

During the therapy the patients are instructed to
use the mirror in a way that the mirror image
produces an illusion of one absent limb.
 This technique is be used to convey the visual
illusion to the patient that his arm has been
amputated or is missing.
 This might provide a sort of ‘‘dress-rehearsal’’ for
the amputation and may de-sensitise and
eliminate the desire.
Vestibular Caloric Stimulation

Cold caloric irrigation, temporarily ameliorates
the symptoms of somatoparaphrenia.
 As per researchers cold-water caloric irrigation
to, at least temporarily, alleviate these patients’
intense desire for an amputation.
 Such a reduction of symptom intensity in BIID
sufferers post irrigation would be suggestive of a
similar aetiology.
 Perhaps with repeated irrigations BIID patients
might come to accept the rejected limb into their
body image;
Ethics of Amputation
 Tim
Bayne et al came up with three
arguments for allowing self-demand
amputation of healthy limbs:
 Harm Minimization
 Autonomy
 Therapy
Harm Minimisation
 Given
that many patients will go ahead
with amputations in any case, and risk
extensive injury or death in doing so, it
might be argued that surgeons should
accede to the requests, at least of those
patients who they judge are likely to take
matters into their own hands.
Autonomy
 An
individual’s conception of his or her
good should be respected in medical
decision-making contexts.
 Where a wannabe has a long-standing
and informed request for amputation, it
therefore seems permissible for a surgeon
to act on this request.
Therapy
The argument rests on four premises:
 (i) wannabes endure serious suffering as a result
of their condition;
 (ii) amputation will — or is likely to — secure
 relief from this suffering;
 (iii) this relief cannot be secured by less drastic
means;
 (iv) securing relief from this suffering is worth the
cost of amputation.
What do you think???
With thanks to Dr Veena Rayker for
her assistance in preparing this
presentation.