Auto cannibalism in mental retardation.

Mental retardation (MR) deems an individual more vulnerable to psychopathologies. The individual may develop an array of behavioral disturbances manifesting themselves in the form of aggressive and destructive conduct, violent fits of anger, stereotyped, or self-injuring behavior. Self-injurious behavior is heterogeneous in nature ranging from mild to severe variant. We report a case of a 7-year-old boy with MR with self-inflicted severe oral injuries of cannibalistic nature presenting as cleft lip and palate. A more extensive research is needed on the problem behaviors in mentally retarded patients for early detection and effective and timely intervention leading to a better outcome.

Introduction

Mental retardation (MR) is a developmental disability in which there is global delay or deficiency in development of language, cognitive, motor, and social functions. Persons with MR are more vulnerable to psychopathology with comorbidity about 3-4 times more than the general population. The varied presentation of behavioral disturbances during their lifespan can include aggressive and destructive conduct, anger attacks, stereotyped, or self-injurious behavior (SIB).[12]

Self-injury is defined as repeated, self-inflicted, nonaccidental injury, producing temporary or permanent tissue damage. Various terms have been used to denote SIB like self-harm, deliberate self-harm, self-mutilation, parasuicide, self-wounding, autoaggression, and purposive accidents. The most frequently encountered SIB in MR are head banging, self-biting, and self-scratching.[3] SIB, when continues over a protracted period, is extremely persistent and difficult to treat.[4] Early intervention may very well prevent the SIB from developing into a serious problem.

We present here a case of a 7-year-old boy with MR with self-inflicted severe oral injuries of cannibalistic nature appearing as cleft lip and palate.

Case Report

A 7-year-old male child presented to department of paediatrics due to severely injured upper lip, palate, and nasal septum. After detailed evaluation, it was found that the child was suffering from severe delayed milestones and SIB. Therefore simultaneously, psychiatric opinion was also taken. The behaviors include hitting, scratching, and biting his face. These behaviors occurred intermittently in the past. During the past 6 months, they have increased in frequency and intensity and caused removal of his upper lip and palate. His parents state that the behaviors occur for “no reason at all.” He could be watching TV or playing with a toy and begin scratching and biting his mouth. His parents have managed the behavior by verbal scolding. If that was not effective, they would hold him to prevent him from scratching himself or move him onto his bed with hands tied. Due to severity of injuries he has caused to himself, his hands were mostly bandaged. These behaviors were very disruptive to the family as they were occurring most of the time. There was no evidence of hallucinations, delusions, social isolation, body image disturbance, or sexual conflicts. The patient was not on any medications. He has not had seizures. He slept 9 h each night without snoring. There had been no change in his appetite or sleep. His parents estimate that he has about five words that he uses communicatively. According to his mother, she was 28 years of age when he was born; it was a normal vaginal delivery at term and was uneventful. The patient was fully immunized. In comparison to his other two elder siblings, he had delayed milestones in terms of language, motor skills, communication, and self-care.

The detailed physical examination showed most of upper lip, nasal septum, and the adjoining maxillary area severely torn apart making upper palate and nasal septum a single cavity. All the front teeth were missing [Figure 1].

Figure 1

Facial dysmorphology depicting self inflicted severe oral injuries of cannabilistic nature appearing as cleft lip and palate

Formal assessment revealed his Intelligence Quotient (IQ) to be about 25. Apart from his low hemoglobin (7 mg%), all other laboratory parameters (liver function tests, sodium, potassium, magnesium, bilirubin, creatinine, urea, uric acid, and glucose) were within normal limits. Brain computed tomography scan was normal.

The diagnosis of self-inflicted lesions and SIB was established. He was started on tab. risperidone 1 mg/day and was started on behavioral therapy on principle of token economy. The parents were counselled and initiated on parent training therapy.

Discussion

In Diagnostic and Statistical Manual-IV-Text Revised, the term self-inflicted injury is being acknowledged in form of disturbances of stereotyped movements described as repeated motor-nerve behavior apparently intentional and a finalistic which for themselves can be specified as self-injury behavior if such behavior can cause physical damage which requires specific treatment or could cause physical injury if protective measures are not taken.[2] The 10th Revision of The Classification of Mental and Behavioural Disorders: Clinical Descriptions and Diagnostic Guidelines inserts self-inflected injury as disturbance caused by Stereotyped Movements”, that is, movements which are “voluntary, repetitive, stereotyped, nonfunctional,” which can be subdivided into “self-infliction” and “nonself-infliction.”[5]

Various taxonomical approaches have been used to classify SIB.[6] The classifications have been based on (a) Severity: minor and major, mild and severe, (b) Frequency (number per year): 1-3 times as mild, 4-11 times as moderate, and more than 11 times as severe. (c) Site: Self-cutting of skin with subtypes low-lethality delicate cutters and high-lethality coarse cutters, ocular self-mutilation and genital self-mutilation, (d) Psychiatric diagnoses: like MR, psychosis, or personality disorders.[6]

Self-mutilation is caused by a repetitive ritual. It may include various forms of injuries like banging the head, kicking the limbs, skin picking, carving words on the skin, sand papering the face, dripping acid on the hands, biting, burning, cutting, pulling out finger and toe nails, chewing fingers, or mutilation of body parts such as removal of eyes, ears, genitalia, tongue, teeth, digits and limbs.[6] At times, severe mutilations may be associated with autocannibalism and autosurgery.

Meta-analysis reports a significant association between severe intellectual disability and the prevalence of self-injury, stereotypy, and destruction of property.[7] Literature reports the incidence of SIB ranging from 3.5% to 40% in MR.[8] The prevalence and severity of self-injurious and aggressive behaviors increases with age and may persist for decades.[910] Early longitudinal developmental studies reported that stereotyped movements and SIB in MR began as early as 12 months and increased over the next year.[8] SIB mostly involves back and lower arms, back of hands, back of the legs, and head particularly face.[1]

SIB has been described in MR in association with psychoses and specific syndromes which may present with some specific behaviours like skin picking in Prader-Willi syndrome, biting in Lesch-Nyhan syndrome, head banging, pulling out nails, cutting, and pin pricks in Smith-Magenis syndrome, Cornelia de Lange syndrome, tuberous sclerosis, or phenylketonuria.[11] SIB has also been described in medical illnesses such as epilepsy, encephalitis, diabetes, hypothyroidism, and eating disorders and prison population.[6] SIB is also observed in other psychiatric disorders like stereotypic movement disorder, Tourette's disorder, borderline personality disorder, and psychotic disorder.[12] Indian studies regarding SIB in MR patients have mostly described reports of genital self-mutilation.[6]

Research has hypothesized psychological, behavioral, physiological, or neurobiological factors as etiological reasons of SIB in individuals with MR.[13] Various factors have been linked to development of SIB such as sensory impairment leading to excessive self-rubbing or scratching or head banging to reduce pain. Other factors implicated are poor expressive skills, attention seeking, avoidance, escape, boredom, and learned behavior.[14]

A greater degree of intellectual disability, the presence of social impairment or specific genetic syndromes, associations between social contact and the presence of repetitive behaviour are few markers which is suggested to make an individual with MR prone to SIB.[71516]

Behavioral assessments can be done directly by clinical observation, caregiver observation, self-monitoring, and automated recording, or indirectly based on interviews with patient and significant others, rating scales and questionnaires.[17] Targeting such group would aid in early intervention strategies which have been known to have better overall outcome.[18] Literature on the benefits of pharmacological agents for individuals with MR having SIB best supports antipsychotics, lithium, and carbamazepine with some behavioral interventions.[18]

Conclusion

Severe SIB may present not only as a diagnostic but a management challenge. A more comprehensive understanding of problem behaviors is needed to guide development of more effective treatment in order to decrease maladaptive behaviours as still limited knowledge is available on this issue. In order to address these behaviors, research is needed on early intervention and prevention techniques so that maladaptive behaviors can be decreased as quickly and as effectively as possible in turn leading to better outcome.