The case of a 35-yearold biological woman with Asperger syndrome (AS) and gender identity disorder (GID) fulfilling DSM-IV criteria is reported. Against the background of recently emerging theories of cognitive male pattern underlying autism we present additional psychological assessments in order to discuss any possible interaction or discrimination between AS and GID. Whilst we explain GID as a secondary feature of AS, we examine the assumption of the necessity of treating GID in AS as a primary GID in accordance with international standards. We consider the treatment of GID as compelling, particularly because curative therapy for AS is lacking and with GID treatment in this vein, the patient gains psychosocial improvemen

Delsignore, Aba

Gundelfinger, Ronnie

Hepp, Urs

Kraemer, Bernd

Schnyder, Ulrich

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Eur Child Adolesc Psychiatry (2005)14:292–296 DOI 10.1007/s00787-005-0469-4 CASE REPORTECAP 469Bernd KraemerAba DelsignoreRonnie GundelfingerUlrich SchnyderUrs HeppComorbidity of Asperger syndrome and gender identity disorderAccepted: 24 February 2005B. Kraemer, M.D. () · A. Delsignore, M.A., M.Sc. · U. Schnyder, M.D. · U. Hepp, M.D.Psychiatric DepartmentUniversity HospitalCulmannstrasse 88091 Zurich, SwitzerlandTel.: +41-1/255-5280Fax: +41-1/255-4408E-Mail: bernd.kraemer@usz.chR. Gundelfinger, M.D.Zentrum fuerKinder und JugendpsychiatrieNeumuensterallee 38032 Zurich, Switzerland■ Abstract The case of a 35-year-old biological woman with As-perger syndrome (AS) and genderidentity disorder (GID) fulfillingDSM-IV criteria is reported.Against the background of recentlyemerging theories of cognitivemale pattern underlying autism wepresent additional psychologicalassessments in order to discuss anypossible interaction or discrimina-tion between AS and GID. Whilstwe explain GID as a secondaryfeature of AS, we examine theassumption of the necessity oftreating GID in AS as a primaryGID in accordance with interna-tional standards. We consider thetreatment of GID as compelling,particularly because curativetherapy for AS is lacking and withGID treatment in this vein, the pa-tient gains psychosocial improve-ment.■ Key words Asperger syndrome– autistic disorder – genderidentity – transsexualism –comorbidityIntroductionAsperger syndrome (AS) is a rare pervasive neurodevel-opmental disorder. Data regarding prevalence are ex-tremely inconsistent, ranging from 3 to nearly 500 per100.000 [5]. AS is characterized by a qualitative impair-ment in social interactions. Furthermore, there are ab-normalities of behaviour, islets of interests and specialactivities. No clinically significant general delay in lan-guage, cognitive development or environmental abilitiesare found. Considering the cognitive pattern of autism,Hans Asperger wrote in 1944:“the autistic personality isan extreme variant of male intelligence . . . in the autis-tic individual, the male pattern is exaggerated to the ex-treme” (translation U. Frith) [2]. Two currently dis-cussed theories “the Empathising-Systemising” (E-S)theory of sex differences and the extreme male brain(EMB) theory of autism are in line with Hans Asperger’sassumptions [2, 3].Gender identity disorder is presumably less frequentthan AS with an estimated prevalence depending ongender of 0.25 to 2.5 per 100,000 [13]. GID is character-ized by a strong and persistent cross-gender identifica-tion, and a persistent discomfort with the assigned natalsex and its associated gender role, which causes clini-cally significant distress and the wish of a cross-genderlife. Impairment of social or occupational functioning isseen in both AS and GID [1].We found two case reports noting the coexistence ofautism and GID in infancy. One author regarded GID asa disorder distinct from autism [12]. The other authormentions gender identity problems in conjunction withAS and obsessive compulsive disorder in a girl [16]. Twoother cases of cross-gender behaviour (not meeting thecriteria for GID) in autism in infancy have been pub-lished [15, 21]. For all these cases, there is no informa-tion available as to whether the gender atypical behav-iour persisted. To the best of our knowledge, this is thefirst report of an adult biological woman with concur-rent AS and GID.292_296_Kraemer_ECAP_469  15.06.2005  07:30 Uhr  Seite 292Author et al. 293Comorbidity of Asperger syndrome and gender identity disorderCase report■ Family historyThe patient is the first born of healthy parents. However,her father is described as somewhat reclusive and aloof,and some additional inappropriate stereotypical behav-iour is reported. Neither autism, GID, nor any other psy-chiatric disorder had been diagnosed in the family.■ Pregnancy and perinatal periodThe patient’s mother reported a normal pregnancy; shetook no medication during pregnancy. Moreover, thereis no history of prenatal medication with sex hormones.The patient was born in an uncomplicated 3-weekpreterm pelvic presentation birth. She was healthy, herbirth weight was 3020 g and the birth length was 50 cm.■ Course of Asperger syndrome from infancy to adulthoodThe mother reported a normal early childhood develop-ment of her daughter. No language delay was observedand the cognitive and motor abilities met expectations.In social relationships, some inconsistencies were no-ticeable and she appeared unapproachable. From child-hood on, she took a special interest in details and a per-sistent fascination in structured and geometric entities.All in all, the parents and teachers were not alarmed andno medical or psychological examination was re-quested. When she was 33 years old the patient herselfinstigated an examination of AS as she had been awareof her symptoms for a long time.■ Development of GID from infancy onAs far as the patient can remember, she always wanted tobe a boy. In her sparse peer-contacts, she chose maleplay-mates, was interested in football and developedtomboy behaviour. She insisted on being a boy and re-fused girl’s clothing. Two heterosexual platonic partner-ships are reported, both abandoned because of her lackof social and emotional reciprocity. At the age of 34, shehad the coming-out of her GID, and sought treatment atour unit for GID at the Psychiatric Department of theUniversity Hospital of Zurich, where the diagnosis ofGID was confirmed and counselling was organized forher.■ Current life-situationToday she conducts her life as an independent adult. Sheis a graduate student, having successfully completed herapprenticeship.Methods■ Psychiatric diagnosisPsychiatric diagnosis was confirmed according to DSM-IV criteria by well-trained psychiatrists.■ Intelligence and executive functionsIntelligence and executive functioning are of particularinterest in AS, so, in order to estimate verbal and non-verbal intelligence, we administered computerized ver-sions of the Vocabulary Test (Wortschatztest WST) [18]and the Performance Assessment System [Leistungs-prüfsytem (LPS)] [10]. Executive function was testedwith the electronic version of the Tower of Hanoï in-volving three pegs and four/five discs [7].■ Assessments focusing on gender traits and body imageGender identity disorder led us to assess masculine (in-strumental) and feminine (expressive) traits with thePersonal Attitudes Questionnaire (PAQ) [17]. In order tomeasure body image disturbances, common amongtranssexual patients, we applied the Questionnaire forAssessment of the Own Body. [Fragebogen zurBeurteilung des eigenen Körpers (FbeK)] [19]. TheFbeK consists of four scales: 1. attractivity/self-confi-dence; 2. emphasizing body image; 3. insecurity/appre-hension; 4. body/sexual uneasiness.■ Perception of emotional statesIn addition to cognitive and gender identity centeredtesting, we were interested in the self-awareness and so-cial cognition of our patient. We expected that the addi-tional results from the Reading the Mind in the Eyes Test[4] would prove the mentalizing capacity and that theToronto Alexithymia Scale (TAS) (a 26-item scale as-sessing deficits in the identification and expression ofown emotions) [11, 20], plus the rating of external ori-ented, as opposite to analytical, thinking, would put usin a position to discuss the dynamic interrelation of ASand GID in our patient.292_296_Kraemer_ECAP_469  15.06.2005  07:30 Uhr  Seite 293294 European Child & Adolescent Psychiatry (2005) Vol. 14, No. 5© Steinkopff Verlag 2005ResultsTest results are presented in Table 1.■ Psychiatric diagnosisGID was diagnosed in 2003 by two of the authors (B. K.and U. H.) using established DSM-IV criteria [1]. In2001, the AS diagnosis was confirmed by M. AspergerFelder, M. D., Child and adolescent psychiatrist, thedaughter of Hans Asperger. (Currently re-examined andadditional assessment of the autism spectrum quotient(AQ) [14] and Asperger Syndrome Diagnostic Interview(ASDI) [9] administered by one of the authors, R. G.)■ Cognitive testingIntellectual assessments with the WST and the LPS re-vealed that the patient was functioning at the upper av-erage range of intelligence with no significant discrep-ancy between verbal and performance abilities [8]. Asexpected, the performance achieved at the Hanoï Towertask is well below the norm. The low score was antici-pated due to the well-known deficits in executive func-tions in AS [6, 8].■ Assessments focusing on gender traits and body imageThe PAQ showed lower femininity (emotionality) andhigher masculinity (instrumentality) scores comparedwith male controls. This fits the profile of patients withScale Patient score NormM (SD)WST (verbal intelligence)a 125 100 (15)LPS (performance-oriented intelligence)bDiscerning rules 60 50 (10)Logical reasoning 70 50 (10)Fluid intelligence 65 50 (10)Hanoï (first attempt)c4 discs: score (seconds) 3 (384s) 5 (1)score (number of moves) 0 (73m)5 discs: score (seconds) 4 (445s) 5 (1)score (number of moves) 0 (95m)Hanoï (second attempt)c4 discs: score (seconds) 7 (50s) 5 (1)score (number of moves) 7 (15m)5 discs: score (seconds) 7 (94s) 5 (1)score (number of moves) 8 (31m)Reading the Mind in the Eyes Test (RMET) 15 26 (3)TASbDifficulties in the identification of emotions 40 50 (10)Difficulties in the expression of emotions 79 50 (10)External oriented thinking 27 50 (10)Alexithymia score 46 50 (10)PAQ femalesd malesdFeminine traits (expressivity) 3.38 4.60 4.38Masculine traits (instrumentality) 4.50 3.50 3.75FBeKb femalee maleAttractivity/self-confidence 32 24 50 (10)Emphasizing body image 46 51 50 (10)Insecurity/apprehension 48 51 50 (10)Body/sexual uneasiness 51 57 50 (10)a IQ-score (estimation); b T-scores; c z-scores; d no SD available; e transformation of row-scores into T-scores is dif-ferent for males and femalesBold numbers indicate scores at least 2 SD below the normTable 1 Cognitive and “psychological” scores292_296_Kraemer_ECAP_469  15.06.2005  07:30 Uhr  Seite 294Author et al. 295Comorbidity of Asperger syndrome and gender identity disorderAsperger syndrome [2] and is consistent with female-to-male GID. As clinical experience has repeatedly shown,low attractivity/self-confidence (FbeK) fits the body im-age distortions described by patients with gender iden-tity syndrome.■ Perception of emotional statesThe number of correctly identified states of mind isclearly below the norm and corresponds to the perfor-mance achieved by patients with Asperger syndrome[4]. TAS scores indicate a correct identification of (own)emotions, but a significant deficit in their expression.The low score in external oriented thinking (TAS) con-firms a high abstract-logical functioning.■ Behaviour during the interviewDuring the Reading the Mind in the Eyes Test, the pa-tient was particularly distressed by the impossibility ofdefining consistent criteria allowing a clear identifica-tion of states of mind. Instead of trying to base her deci-sions on emotional intuition like normal controls, shepersevered with the same strategy based on logical cri-teria.She avoided any interaction with the examiner andtalked to herself as if alone in the room.DiscussionWe report the case of a 35-year-old biological female pa-tient diagnosed with AS as well as GID according toDSM-IV criteria.The coexistence of GID and AS is worthnoting as the prevalences of both disorders are low.Against the backdrop of currently discussed malecognitive characteristics in autism and well-known ex-aggerated masculinity in female-to-male GID patients,we were interested in the question of whether, using ad-ditional investigations, an underpinning of differentia-tion or unification of these entities can be made.As expected for Asperger patients, verbal and non-verbal intelligence are situated in the upper norm, butexecutive functioning is disturbed. Reduced ability inexpressing one’s own emotions was found and the pa-tient also showed difficulties in identifying emotions inother people. These findings are in accordance with thewell-established cognitive profile of AS patients.The development of GID comprises the patient’saversion to her body from early childhood on,dysphoricreaction on secondary sex characteristics, her refusal towear girl’s clothes and others. Our findings of low femi-ninity and high masculinity PAQ scores are consistentwith female-to-male GID. Low attractivity/self-confi-dence as found in the FbeK are consistent with body im-age distortions, observed in clinical work with female-to-male GID. Our results meet the criteria of primaryGID but, in our opinion, are thrown into doubt, throughcoexistent AS. Nonetheless, in the following, we pointout the considerable overlap in cognitive domain anddare to hypothesize that AS is the evolutionary and dy-namic underlying disorder.As expected for AS, we noticed over-developed logi-cal thinking and accentuation of logical-abstract abili-ties, as well as an imbalance of low emotionality and ahigh level of instrumental, non-emotional attributes in-cluding activity, lack of emotionality and perseverance.These characteristics are generally associated with mas-culinity and may have led to a subjective consciousnessin our patient of being male. In this regard, primary cog-nition and perception in AS may be interpreted as mas-culine attributed and pave the way to the development offemale-to-male GID. The adaptation of the male genderidentity, from early life on, possibly enabled the patientto better integrate the lack of emotionality and the ac-centuated logical-abstract abilities. The extremely highlevel of masculinity can be interpreted as an additionalcompensatory effort to accentuate the biologically ab-sent male side.Taking this into account, we believe that, over theyears, our patient has developed GID as a consequenceof adopting male emotional and cognitive traits due toAS. Following this argument, GID in this patient couldbe regarded as a sequel to AS.Although we explained GID as the secondary feature,we realize there is no curative therapy for AS. Againstthis backdrop, we developed the assumption of the ne-cessity of treating GID in AS as a primary GID. The maleidentification has proven stable over time and the real-life test led to a remarkable psycho-social stabilization.The patient improved in psychological well-being andsatisfaction during cross-gender living. These issues ledto our decision to treat GID in our patient in accordancewith international standards.As we have demonstrated, AS and GID overlap con-siderably in their male cognitive pattern. Cognitive pro-files are expressions of how the brain mediates behav-iour. The extreme male brain theory of autism iswell-known and we conceptualized the hypothesis thatour patient, based on a male cognitive pattern, devel-oped collaterally GID. On the other hand, it would havebeen equally easy to argue the opposite, i. e. that a GIDpatient additionally develops AS. This would not alterour treatment recommendations, but it gives a perspec-tive on the field of brain research which is both neces-sary and exciting.292_296_Kraemer_ECAP_469  15.06.2005  07:30 Uhr  Seite 295296 European Child & Adolescent Psychiatry (2005) Vol. 14, No. 5© Steinkopff Verlag 20051. APA (1994) Diagnostic and statisticalmanual of mental disorders (DSM-IV).American Psychiatric Association,Washington2. Baron-Cohen S (2002) The extrememale brain theory of autism. Trends inCognitive Sciences 6(6):248–2543. 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Comorbidity of Asperger syndrome and gender identity disorder

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