K. Nagaraja Rao, Shamshad Begum, Sameeran Chate
Abstract: Repetitive mild form of self-injurious behavior has been described in mental retardation in association with various syndromes and psychoses. In absence of these syndromes self-injurious behavior among people with mental retardation has been usually reported in chronic care facilities. In contrast, we are reporting a case of genital self-mutilation, a severe and isolated form of self injurious behavior, in a mentally retarded individual in absence of any associated syndromes.
Introduction: Repetitive moderately severe self-injurious behavior has been described in mental retardation in association with psychoses (Holzer, Jacobson, & Folstein, 1996) and syndromes like Lesch-Nyhan syndrome, Cornelia de Lange syndrome, and Smith Magenis syndrome (King, Hodapp, & Dykens, 2000). Mild form has been reported in Prader-Willi syndrome, Tuberous Sclerosis and Phenylketonuria (King et al., 2000). In absence of these syndromes self-injurious behavior among people with mental retardation has been reported in chronic care facilities with incidence ranging from 3.5% to 40% (Winchel & Stanley, 1991). The nature of self injurious behavior in these syndromes are biting (Lesch-Nyhan syndrome), head banging, pulling out finger and toe nails, cutting with sharp objects, and pin pricks (Smith Magenis syndrome, Cornelia de Lange syndrome), skin picking (Prader-Willi syndrome) cutting with sharp objects and other injurious behavior (Tuberous Sclerosis, Phenylketonuria). Severity of self injury in people with mental retardation has also been linked to severity of cognitive disability (King et al., 2000). In contrast, we are reporting genital self-mutilation, a severe and an isolated form of self injurious behavior in a case of mild mental retardation in absence of any associated syndromes.
Case Report: In April 2002 an 18 years old Hindu boy was referred from a surgical ward with history of self amputation of testes and penis. Detailed history revealed that he was working in his father's shop. He reported sadness & suicidal ideas for the past one year apparently due to ncreased work load and inferior type of work compared to his brother. However he did not communicate his feelings to any one. A day prior to admission he cut both his penis and testes using a blade and threw them over the roof of the house, under the belief that genitals are very vital and by chopping them off the person would die instantly. There was no history of decreased sleep, decreased appetite, weakness or ideas of worthlessness. There was no history suggestive of delusions or hallucinations.
In the past at the age of one year he had a bout of fever followed by a few episodes of convulsions. He was treated with antiepileptics for three years. During and after the treatment he was symptom free. There was no past history of psychiatric illness.
The patient is second of five siblings born to nonconsanguineous parents. There was no family history of present or past psychiatric illness or mental retardation. He was born full term and had a normal delivery. His milestones were relatively delayed in comparison to other siblings. He had bed wetting until 14 years of age. He went to school up to 4th standard and discontinued as his performance in the school was very poor and he was disinterested in studies. He worked in a general store as a helper for few years prior to joining his father's shop. He had no knowledge of masturbation and did not have any sexual aberrations.There was no history of alcohol consumption, smoking, tobacco chewing or any other substance abuse. Premorbidly he was reserved and had few friends. He was sincere in his work. He was irritable and stubborn. He was religious, intellectually dull, and could not handle responsible jobs and money.
On examination he was moderately built and nourished.There was some gap between two incisors and gingiva was mildly hypertrophied.There were no other physical abnormalities. Systemic examination was normal. Special attention was paid to features of syndromes of importance and those were not found. His hemoglobin, blood cell counts, blood smear, liver function tests, serum urea, creatinine, uric acid, ultrasonography of abdomen, ophthalmic examination, and brain CT scan were normal.
He was dressed appropriately to his socio economic status. He had superficial rapport. He reported subjective sadness but objectively was emotionless. He answered questions in a matter of fact style and dispassionately. He did not have any remorse for his action and had no realization of consequences of his action. His orientation and memory were normal. Intellectually he had poor general fund of knowledge and mathematical ability. His IQ was 52 on intelligence tests.
He was diagnosed to have mild mental retardation with Depression NOS. He was put on Fluoxetine 20 mg daily and was discharged from the hospital after eight days. He came for follow up for four months. He reported no sadness and had resumed his previous work. He had no problems with micturition.
Genital self- mutilation is a rare severe form of self-injurious behavior. It is usually described in psychotic disorders (Greilsheimer & Groves 1979), mostly in schizophrenia as a result of delusions & hallucinations (Becker & Hartmann 1997; Martin & Gattaz,1991). On rare occasions it has been described in non- psychotic cases too (Haberman & Michael, 1979; Cawte, Kjagamara, & Barrett, 1966; Thompson & Abraham, 1983; Greilsheimer & Groves,1979). It has also been described in substance abuse and social isolation (Tobias, Turns, Lippmann, Pary, & Oropilla,1988). affective psychosis, alcohol intoxication (Krasucki, Kemp, & David,1995) and personality disorders. It has been ascribed to sexual conflicts and offences (Martin & Gattaz, 1991), erotic purposes (Wan, Soderdahl, & Blight, 1985), body image preoccupation and distortion (Walter & Streimer,1990; Krasucki et al., 1995), expression of internalized aggression and suicidal intent (Becker & Hartmann 1997; Yager 1989; Rao, Bharathi, & Chate,2002) . Repetitive moderately severe self-injurious behavior has been described in mental retardation in association with psychoses and some syndromes. Severity of self injury in mentally retardation has also been linked to severity of cognitive disability (King et al., 2000). In contrast, the present case is an example of severe and an isolated form of self injurious behavior in a case of mild mental retardation in absence of psychosis or any associated syndromes.
In the present case there was no evidence of hallucinations, delusions, social isolation, body image disturbance, or sexual conflicts. There was indirect evidence of frustration leading to internalized aggression resulting in an impulsive suicidal attempt. In addition, low intellectual capacity might have contributed to ignorance of long term consequence of the act and over reaction to apparently low magnitude of frustration precluding other ways of problem solving. Apparently he resorted to this mode of suicidal attempt under the notion that life lies in such vital organ. Cataleano et al. (1996) have reported a case of genital mutilation in a 22-year old man with borderline intellectual functioning. They have proposed that the patient was probably influenced by popular media, i.e. trials of John and Lorena Bobbitt.
Though sadness and suicidal ideas were reported lasting over a period of one year, it was not reflected in behavior. Hence it was possible that depression in this case was clinically subthreshold. His reported unwillingness and dissatisfaction with work without protest is a pointer towards chronic frustration and aggression turned inwards. However these were of low magnitude in that there was no behavioral concomitant over such a long period. Therefore the present act could be considered as an impulsive one.This suggests frustration, aggression, and impulsivity lying on a continuum, with breakthrough points at various levels and in various forms in particular individuals based on specific personality traits and psychopathology.
Biologically serotonergic depletion preceding genital self mutilation has been implicated in depression and personality disorder, which is in turn linked to aggression (Dolan, Anderson, & Deakin, 2001). Dopamine receptor mediated self mutilation behavior has been implicated in mental retardation with compulsive self mutilation (Criswell, Muller, & Breese, 1989). Both dopaminergic and serotonergic systems have been implicated in some patients with MR with psychosis (Holzer et al 1996). In the present case absence of obsessive traits and psychotic features and improvement with SSRIs suggests predominance of serotonergic depletion, which may have an important role in cases of sporadic impulsive act in a background of frustration and aggression turned inwards.
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For further information:
Dr. K. Nagaraja Rao, Department Of Psychiatry, JJM Medical College, Davangere. Karnataka. INDIA. Pin- 577004. Phone: 00-91- 819- 221068
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