When Your Leg Just Isn

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When Your Leg Just Isnt Your Leg!?

• Body Integrity Identity Disorder
• Alison Wighton
• NSW PAR October 2008

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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.

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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

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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

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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

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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.

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Investigations

• Jan 2008 - met Dr Ramachandran and Dr McGeoch at
  UCSD
• Tested with MRI brain and magneto-encephalography
• MRI showed an unusually large right superior
  temporal gyrus
• Volumetric analysis of his MRI confirmed
  superior parietal lobule ratio right left of
  0.73

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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.

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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

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History of Presenting Illness

• Unsatisfied with progress trying to seek
  amputation
• Took matter into own hands .

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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.

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Past Medical History

• MVA 1985-86 ? Skull fracture
• History of migraine headache on and off

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Medications

• Citalopram 20mg daily-for last three months

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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

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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

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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

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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

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Suicide Attempts

• 1999-attempted cutting his wrist in response to
  mothers death.

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Treatment Course

• 10/03/08
• Pain management
• Peripheral foot perfusion checks 4/24
• Probably unlikely to require surgery
• Psychiatry consult

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Imaging

• CT Brain-NAD
• CXR under-inflated lungs with bibasal collapse
• MRI Brain-normal
• SPECT Brain-normal

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• If youre not good with blood and all things a
  bit yucky
• LOOK AWAY NOW

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17/03/2008
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17/03/2008

• Blood cultures-gram negative rods in 4/4 bottles
• Wound-heel pad gangrenous
• Commenced on Gentamycin and Ceftazidine

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Opinions

• Rehabilitation team (Dr Ross Hawthorne)
• Extensive necrosis of heel pad, no benefit from
  trying to save the foot or Symes amputation.
• Supported trans-tibial amputation at the level
  desired by the patient.
• Burns team supported the medical indication for
  below knee amputation.

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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

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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

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20/03/2008

• Calf muscle perfusion scan - non viable right
  gastrocnemius muscle

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21/03/2008

• Right trans tibial amputation
• No post operative complications

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The Result
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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.

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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
  Aunts house on 17/04/2008
• Prescription for interim prosthesis made prior to
  discharge.

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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

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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.

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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
  ones body should be and the physical reality of
  how it actually is.

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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 Res197713115-25)
• Description of this disorder was limited to a few
  case reports from 1977-2003

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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.

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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

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Apotemnophilia and Munchausens 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.

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Factitious Disability Disorder

• Bruno 1997- divided this disorder into 3 subsets
• Devotees
• Pretenders
• Wannabes

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Devotees

• Devotees are non disabled people who are sexually
  attracted to people with disabilities, typically
  those with mobility impairments and amputees

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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.

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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.

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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.

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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

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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.

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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.

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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 Firsts subjects reported that their
  desire interfered with social functioning,
  occupational functioning, or leisure activities.

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Co-morbid Psychopathology

• Three quarters reported having had psychiatric
  condition sometime in their lives.
• Most commonly depression, anxiety and somatoform
  disorder.

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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

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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.

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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

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• 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.

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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

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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.

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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.

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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

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Ethics of Amputation

• Tim Bayne et al came up with three arguments for
  allowing self-demand amputation of healthy limbs
• Harm Minimization
• Autonomy
• Therapy

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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.

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Autonomy

• An individuals 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.

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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.

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What do you think???
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With thanks to Dr Veena Rayker for her assistance
in preparing this presentation.