An unusual case of acquired pedophilic behavior following compression of orbitofrontal cortex and hypothalamus by aClivusChordoma

Giuseppe Sartori, Cristina Scarpazza, Sara Codognotto, Pietro Pietrini.

This supplementary material has been provided by the authors to give readers additional information abouttheir work.

This supplementary material describes the neuropsychological tests selected to evaluate the cognitive profile of the individualwith acquired pedophilia and the ClivusChordoma.

The neuropsychological battery was administered twice as a part of a collegial expert evaluation requested by the Judge. The first evaluation was performed a few days before the magnetic resonance imaging scan that revealed the presence of the ClivusChordoma. The second evaluation was performed 8monthsafter surgery. Psychiatric and neurological examinations and behavioral history acquired throughinterviews with family members and the family physician indicated an almost full recovery, with the exception of some residual asymmetry in tendon reflexes.

The following tables reportdata regarding the neuropsychological evaluation. In table S1, the normative value references used for the correction of raw scores are indicated. In table S2, the patient’s performance before and after surgical resection of the ClivusChordomais reported.

Neuropsychological test / Reference / Italian Normative Value / Test Description / Cognitive function investigated
Raven’s Matrices / Raven JC, Court JH, Raven J. (1978). Manual for Raven’s Progressive matrices and vocabulary scales: generaloverview. London: H.K. Lewis. / Score up to 60: Raven, J. C. (1938) Standard Progressive Matrices: Sets A, B, C, D and E, H.K. Lewis (London) (translate in: Raven, J. C.,ProgressiveMatrici 1938 - manuale, OrganizzazioniSpeciali, Firenze, 1938).
Score up to 48:SpinnlerTognoni 1987 / Participants were presented with visual geometric design with a missing piece. The test taker is given six to eight choices to pick from and fill in the missing piece. / Fluid intelligence
abstract reasoning
Verbal Judgement / - / Spinnler M., Tognoni G., (1987) Standardizzazione e taratura italiana di test neuropsicologici.The Italian Journal of Neurological Science, Suppl. 8, n.6. / 4 subtests: 1) identify the differences between two words; 2) explain the meaning of proverbs; 3) understand the absurdness of some sentences; 4) classify words into the right category / Verbal reasoning, abstraction.
COWA / Spreen O, Strauss E. (1991). A compendium of neuropsychological tests: administration, norms and commentary.Oxford University Press. New York. / Novelli G, Papagno C, Capitani E, Laiacona N, Vallar G, Cappa SF. (1986) Tre test clinici di ricerca e produzionelessicale. Taratura su soggetti normali. Archivio di psicologia, Neurologia e Psichiatria. 47(4): 477–506. / Verbal fluency test in which the participants are asked to produce as many words as possible in one minutes. Words has to belong to the same category (i.e. animals) or begin with the same designated letter (i.e. F). / Spontaneous production of words; lexical stock; ability to access to the lexicon; strategic research in the lexicon
Idiomatic/Methaphoric expression comprehension / - / Papagno C, Cappa SF, Capitani E, Forelli A et al. (1995). La comprensione non letterale del linguaggio: taratura di un test di comprensione di metafore e di espressioni idiomatiche. ArchPsicolog, NeurolPsichiatria. 56: 402-20. / Participants were presented with idiomatic and methaphoric expression and they are requested to explain their meaning. / Abstract/literal interpretation
CET / - / Della Sala S, MacPherson SE, Phillips LH, Sacco L, Spinnler H. (2003). How many camels are there in Italy? Cognitive estimates standardized on the Italian population. Neurol Sci. 24(1):10-5. / To answer to questions that are not immediately answerable but the response to which can be effectively guessed using general knowledge e.g. “What is the height of a traffic light?” / ability to produce reasonable cognitive estimates; abstract reasoning
WCST / Berg EA (1948). A simple objective technique for measuring flexibility in thinking. J Gen Psychol. 39:15–22. / Laiacona M, Inzaghi MG, De Tanti A, Capitani E (2000). Wisconsin card sorting test: a new global score, with Italian norms, and its relationship with the Weigl sorting test. Neurol Sci. 21(5):279-91. / Participants has to classify cards according to 4 different criteria. The only feedback is whether they are doing it correctly or not. The classification rule changes every 10 cards, and participants has to flexibly change the criteria used for classifying cards. / abstract reasoning,
cognitive flexibility, planning, conceptual shift, learning new rules, ability to change cognitive strategies when the environmental conditions change.
Hayling / Burgess PW, and Shallice T (1996) Response suppression, initiation and strategy use following lobe lesions,Neuropsychologia, 34, 263-273. / Burgess, P. & Shallice, T. (1997)The Hayling and Brixton Tests. Test manual.Bury St Edmunds, UK: Thames Valley Test Company. / Participants are asked to complete as quickly as possible incomplete sentences with a word which is not related to that sentence. i.e. “I eat the soup with…”
Right answer: e.g. umbrella / Ability to inhibit the predominant response
Overlapping figures / Rey A. (1966) Le troubles de la memoire et leurexamenpsychometrique. Dessart. Bruxelles / Mondini S, Mapelli D, Vestri A, Bisiacchi PS (2003).Esame Neuropsicologico Breve. Raffaello Cortina Editore. / Participants are presented with an image on which different overlapping figure’s contours are represented. They have to explore the image and to identify as much as figures they can discriminate. / Visuo-spatial exploration, perceptive recognition, denomination, response inhibition, ability to discriminate figures from the background
TMT / Reitan, R. M. (1958). Validity of the trail making test as an indicator of organic brain damage. Percept. Motor Skill 8, 271–276. / Mondini S, Mapelli D, Vestri A, Bisiacchi PS (2003).Esame Neuropsicologico Breve. Raffaello Cortina Editore. / Participants are asked to connect a sequence of 25 consecutive targets as quickly as possible. In the TMT-A the targets are numbers that should be connected in sequential order; in the TMT-B the targets are numbers and letter that should be connected in sequential order and alternating a number and a letter. / Visual attention, task switching, visual search speed, scanning, speed of processing, mental flexibility
Emotional attribution /
Blair TJ, Sellars C, Strickland I et al. (1995). Emotion attributions in the psychopath. PersonalityIndividualDifferences. 19, 431-437.
/ Prior, M., Marchi S., and Sartori, G. (2003). Cognizione Sociale e Comportamento, Vol. I. padovaUpselDomeneghini Editore. / 58 brief description of emotional scenes were presented (i.e. Jon lost the competition). For each scenes, participants are required to identify the emotion felt by the protagonist. / Ability to attribute the correct emotion to other’s
Social situation task / Dewey M (1991). Living with Asperger’s syndrome. In: Frith U (Eds.). Autism and Asperger’s syndrome. Cambridge University Press: Cambridge / Prior, M., Marchi S., and Sartori, G. (2003). Cognizione Sociale e Comportamento, Vol. I. padovaUpselDomeneghini Editore. / 25 description of social situations are presented, in which a protagonist interact with other characters. For each situation, participants are asked to classify the behavior the protagonist as normal or not normal. / Ability to discriminate the socially adequate behavior.

Table S1. Neuropsychological test references with abrief explanation. In the first column, the original reference is reported. In the second column, the reference of the Italian normative values used to correct the patient’s raw scoresareindicated. In the last two columns, a brief description of each test is provided, as well as the cognitive functions investigated. Abbreviations: COWA: Controlled Oral Word Association test with Phonemic (COWA/P) and semantic (COWA/S) cues. CET: Cognitive Estimation Task; WCST: Wisconsin Card Sorting Test; TMT: Trial Making Test.

Before tumor resection / After tumor resection
Raw score / Corrected score / Clinicalevaluation / Raw score / Corrected score / Clinicalevaluation / Pre-post surgerycomparison
General Cognitive Functions
Raven’sMatrices / 46/48
54/60 / Z=1.61
IQ=124 / NormalSuperior / 40/48
46/60 / z = 1.38
IQ = 120.7 / NormalSuperior / Stable
VerbalJudgement
Differences / 13/15 / 13/15 / Stable
Proverbs / 15/15 / 14/15 / Stable
Absurdness / 10/15 / 15/15 / Improved
Classification / NA / 15/15 / Improved
Total Score / NA / 57/60 / z = 0.87 / Improved
Language
COWA/P / 22 / CS=17, ES=1 / Impaired / 35 / CS = 30, ES = 3 / Normal / Improved
COWA/S / 44 / CS =42, ES =4 / Normal / 50 / CS = 48, ES = 4 / Normal / Stable
IdiomaticExpressionsComprehension / 28 / CS =23, ES =3 / Borderline / 31 / CS = 26, ES = 4 / Normal / Improved
MetaphoricExpressionsComprehension / 28 / CS =23, ES =4 / Normal / 31 / CS = 26, ES = 4 / Normal / Stable
FrontalFunctions
CET
Absolute errors / 16 / Z = 1.40 / Normal / 16 / Z = 1.40 / Normal / Stable
Bizzarre errors / 2 / Z= 0.44 / Normal / 2 / Z = 0.44 / Normal / Stable
WCST global score / 62 / CS=65.8,ES=2 / NormalInferior / 9 / CS =7.5, ES = 4 / Normal / Improved
Hayling Test
Part A / 15 sec / - / Normal / 13.84 sec / - / Normal / Stable
Part B / 63 sec / - / NormalInferior / 44.01 sec / - / Normal / Improved
Errors / 15 / - / Impaired / 4 / - / Normal / Improved
Total score / 17 / - / Impaired / 6 / - / Normal / Improved
OverlappingFigures / 23 / Z= -2.55 / Severelyimpaired / 31 / Z= - 0.98 / Normal / Improved
Attention
TMT- A / 35 sec / Z = -0.80 / NormalInferior / 38 sec / Z = 0.26 / Normal / Stable
TMT -B / 75 sec / Z= -0.90 / NormalInferior / 100 sec / Z = 0.23 / Normal / Stable
Beforetumorresection / Aftertumorresection
Raw score / Corrected score / Clinicalevaluation / Raw score / Corrected score / Clinicalevaluation / Pre-post surgerycomparison
Social Intelligence
EmotionalAttribution Test
Sadness / 7/10 / Z= -0.83 / Normal / 9/10 / z = - 0.83 / Normal / Stable
Fear / 8/10 / Z= -0.99 / Normal / 10/10 / z = 1.12 / Normal / Stable
Embarassment / 8/12 / Z= -1.09 / Normal / 9/12 / z = - 0.50 / Normal / Stable
Happiness / 8/10 / Impaired / 8/10 / Impaired / Stable
Disgust / 3/3 / Z= 0.46 / Normal / 3/3 / z = 0.44 / Normal / Stable
Anger / 5/10 / Z= -2.55 / Severelyimpaired / 4/10 / z = -3.35 / Severelyimpaired / Stable
Envy / 3/3 / Z = 0.99 / Normal / 3/3 / z = 0.99 / Normal / Stable
Social situation test
Normality / 14/15 / Z = -0.37 / Normal / 12/15 / z = -2.84 / Impaired / Worsened*
Violations / 22/25 / Z = -1.88 / Impaired / 23/25 / z = -0.96 / Normal / Improved
Severity of violations / 61/75 / Z= 1.09 / Normal / 57/75 / z = 0.53 / Normal / Stable

Table S2: Patient’s neuropsychological profile before and after tumor’s surgical resection.

CS= Correct Score; ES= Equivalent Score. ES=2,3,4= performance within the normal limit; ES=1 borderline performance; PE=0 impaired performance.Z= zeta point.COWA= Controlled oral word association test with phonemic (COWA/P) and semantic (COWA/S) cues. CET: Cognitive Estimation Task; WCST: Wisconsin Card Sorting Test; TMT: Trial Making Test; IQ= Intelligence Quotient; NA= Not Administrable.

*The patient manifested a worsened performance in identifying normal behavior, i.e., he categorized a normal behavior as not normal. It is likely that as a consequence of the abnormal social conduct that lead him to a trial, the patient tended to be more cautious in the interpretation and judgment of social scenes: it is socially more acceptable to classify a normal behavior (i.e. Jane was late so she skips breakfast) as not normal (his explanation: it is not healthy to skip breakfast) than to classify a abnormal behavior (i.e. to run naked in the street) as normal.