When Your Leg Just Isn’t Your Leg!?

1. When Your Leg Just Isn’t Your Leg!? Body Integrity Identity Disorder Alison Wighton NSW PAR October 2008

2. 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.

3. 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

4. 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

5. 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

6. 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.

7. 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

9. 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.

10. 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

11. History of Presenting Illness • Unsatisfied with progress trying to seek amputation • Took matter into own hands …….

12. 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….

13. Past Medical History • MVA 1985-86 ? Skull fracture • History of migraine headache on and off

14. Medications • Citalopram 20mg daily-for last three months

15. 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

16. 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

17. 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

18. 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

19. Suicide Attempts • 1999-attempted cutting his wrist in response to mother’s death.

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

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

22. If you’re not good with blood and all things a bit yucky…… LOOK AWAY NOW

23. 17/03/2008

24. 17/03/2008 • Blood cultures-gram negative rods in 4/4 bottles • Wound-heel pad gangrenous • Commenced on Gentamycin and Ceftazidine

25. 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.

26. 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

27. 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

28. 20/03/2008 • Calf muscle perfusion scan - non viable right gastrocnemius muscle

29. 21/03/2008 • Right trans tibial amputation • No post operative complications

30. The Result

31. 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.

32. 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.

33. 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

34. 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.

35. 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.

36. 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

37. 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….

39. 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

40. 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.

41. Factitious Disability Disorder • Bruno 1997- divided this disorder into 3 subsets • Devotees • Pretenders • Wannabes

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

43. 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.

44. 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.

45. 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.

46. 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

47. 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.

48. 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.

49. 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.

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