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Hereditary sensory autonomic neuropathy Type IV.

Hereditary sensory autonomic neuropathy Type IV is an autosomal recessive disorder due to lack of maturation of small myelinated and unmyelinated fibers of peripheral nerves, which convey sensation of pain and temperature, therefore, resulting in self mutilation. There is anhidrosis due to lack of innervation of normal sweat glands resulting in recurrent episodes of hyperpyrexia. The clinical presentation of two children with this rare disease is described.

Child↗

[Injuries of the external male genital organs. Apropos of 20 cases].

The authors report 20 cases of trauma of the male external genital organs observed over a 10-year period, due to various causes, particularly road accidents, domestic quarrels, and self-mutilation. Lesions of the testis and its appendages were frequent and treatment was usually surgical.

Accidents, Traffic↗

Lesch-Nyhan syndrome: surgical treatment in a case with lip chewing. A case report.

Lesch-Nyhan Syndrome (LNS) is a biochemical disorder characterized by a virtually complete deficiency of an enzyme of purine metabolism, hypoxanthineguanine phosphoribosyltransferase. This sex-linked recessive inborn error of metabolism is characterized clinically by: hyperuricaemia, excessive production of uric acid, certain characteristic neurological features including self-mutilation, choreoathetosis, spasticity and mental retardation. This paper will review the clinical, biochemical and genetic features of Lesch-Nyhan Syndrome and report a case in which repeated lip chewing resulted in destruction of the lower lip and the need for surgical intervention.

Child↗

The role of the neuroma in autotomy following sciatic nerve section in rats.

A delay in the formation of the terminal neuroma following sciatic nerve section in rats was obtained by means of free nerve grafts sutured to the proximal stump of the sectioned sciatic nerve branches. The automutilating behaviour in these animals was statistically compared with that which follows single sciatic section and sciatic section plus end-to-end suture. The results showed that in animals with grafted nerve stumps, autotomy begins significantly later than in those with single sciatic section. However, when the self-mutilation started, it followed the same increasing evolution in both groups. These results suggest that autotomy after a nerve section is behaviour related to the aparition and nature of the terminal neuroma.

Animals↗

Congenital insensitivity to pain with anyhydrosis: morphological studies of skin and peripheral nerves.

Two male siblings born to consanguineous parents, with the diagnosis of congenital insensitivity to pain with anhydrosis are evaluated. The patients presented with unexplained bouts of fever, self-mutilation, repeated trauma and inability to sweat. Physical examination revealed both siblings to be insensitive to pain and temperature. The electron microscopic study of the skin was unremarkable whereas sural nerve biopsies yielded an essential lack of unmyelinated fibers.

Child, Preschool↗

Clinical importance of nightmare disorder in patients with dissociative disorders.

In the present study the prevalence of nightmare disorder (ND) was examined in patients with dissociative disorders (DD), and comparison was made between those with ND and those without nightmares in terms of clinical characteristics. The 30 patients with DD (5 male and 25 female) were recruited over 12 months in the Yüzüncü Yil University Research Hospital Department of Psychiatry. The subjects were diagnosed according to the Diagnostic and Statistical Manual of Mental Disorders (4th edn) criteria for ND. The Dissociative Experiences Scale, Beck Depression Inventory, and a semistructured interview schedule for childhood traumatic events were administered to the subjects. A 57% prevalence of ND was found among patients with DD. Among patients with DD, those with ND had a higher rate of self-mutilative behavior, a history of suicide attempt in the last year, and comorbidity with borderline personality disorder than those without ND. Nightmares or dreams should be considered in the therapy of DD patients.

Adult↗

Self-destructive behavior in battered children.

Fifty-nine physically abused children demonstrated a significantly higher incidence of self-destructive behavior than two control groups of nonabused children, one neglected and one normal. The self-destructive behavior, including suicide attempts and self-mutilation, was potentiated by interrelated variables operating in the abused child and his environment. Often enhanced by the ego deficits and impaired impulse control of the abused children, this behavior seemed to represent a learned pattern originating in early traumatic experiences with hostile primary objects.

Adolescent↗

Cortical involvement in dorsal horn cell hyperactivity and abnormal behavior in rats with dorsal root section.

Experiments were performed on rats using neurophysiological and behavioral techniques, in an attempt to study the role played by the somatosensory cortex in the abnormal spinal neuron activity and abnormal behavior observed after brachial plexus lesions. The onsets of both phenomena occur at the same postoperative period. Cortical controls exerted on spinal dorsal horn (DH) cells were studied using a transient and reversible cortical blockade, cortical spreading depression (CSD), applied contralateral to the spinal cord recording. In 28 intact animals, 55 cells were studied during the propagation of at least two CSDs. Only 4 of these cells presented a sustained decrease in their spontaneous activity during CSD, which may correspond to transient arrest of a descending tonic cortical facilitation. In 29 animals with dorsal root sections, 161 DH cells displayed abnormal burst activity, and 52 were examined with the CSD test. Thirty-five cells presented a long-duration change in their spontaneous activity during CSD; of these, 28 showed decreased activity (suppression of descending tonic facilitation) and 7 presented increased activity (suppression of descending tonic inhibition). More DH cells were influenced by the cortex in deafferented rats (67%) than in intact rats (7%). The cortical influence was also stronger, as the hyperactive cells were frequently rendered silent during CSD. These observations suggest that the abnormal activity is partly due to a descending cortical influence. Results of a behavioral study performed on 22 rats (one control group and two experimental groups with cortical ablations) showed that the self-mutilating behavior, which develops at the same time as the abnormal DH cell activity, was reduced by unilateral cortical ablation, independent of the cortical region removed. The possible pathways involved in this cortical influence are examined in the discussion.

Afferent Pathways↗

Lesch-Nyhan disease: clinical experience with nineteen patients.

The clinical phenotype in Lesch-Nyhan disease has been analyzed in 19 patients studied in hospital. In each case the diagnosis was made on the basis of inactivity of the enzyme hypoxanthine guanine phosphoribosyltransferase in erythrocyte lysates. All had hyperuricemia, and the presence of 'orange sand' in the diaper was a prominent early complaint. All had self-mutilative behavior, of which the most characteristic form was biting the fingers or lips. All had the neurological syndrome of spasticity and choreoathetoid involuntary movements. All but one had less-than-normal intelligence.

Adolescent↗

Influence of testosterone on autotomy in castrated male rats.

Sex-related differences exist in nociception and gonadal steroids influence the analgesic response in animals and humans. As we have shown previously, estrogen could modify autotomy in female rats using the sciatic nerve transection model. To further characterize the relationship between gonadal steroid and nociception, the role of testosterone on autotomy in sciatic nerve sectioned rats was investigated. Twenty male rats were subjected to orchiectomy (ORX). Then ten rats received subcutaneous sesame oil and the other ten were treated with testosterone propionate in sesame oil (TP; 500 microg/day/rat). All the rats underwent sciatic nerve resection in left hind limb. Degree of self-mutilation was measured daily for 8 weeks. TP reinstatement resulted in significantly lower autotomy scores in orchiectomized rats. The results demonstrated that testosterone could modify the autotomy behavior, an indicator of neuropathic pain, in rats after nerve injury.

Animals↗

[Differentiation and therapy of delinquent behavior in borderline disorders. Psychodynamic approach].

In the literature about borderline patients, self-destructive dependency needs are described, as well as oral-aggressive root conflicts. Using case studies, the author points out that in persons with borderline personality organisation, self-destructive forms of delinquency are as likely to manifest themselves as aggressive forms are. The first form is connected with sexual abuse, the second with physical abuse. Therefore, delinquency should be differentiated according to the traumatic experiences. Finally, depending on the direction of delinquency-either directed against oneself or against others-various therapy requirements are outlined: delinquency in the sense of self-mutilation primarily requires the team to perform a holding function, while aggressive delinquency primarily demands a firm setting. The author concludes that the outcome of therapy for borderline personality disorders depends on the assessment of the direction of the delinquency.

Adult↗

Ritual burns--the Buddhist tradition.

The mystical significance of fire is common to many cultures and religions. For the Buddhist community acts of self-mutilation by burning reflect a tradition for which the direct precedent is set in the ancient scriptures of the Lotus Sutra. Burns are produced by contact with incense and treated with oil and vegetable dressings. Two cases are presented and discussed with reference to early Buddhist beliefs.

Adult↗

[Factitious purpura].

Three identical case histories consisting of circular delimited petechial purpura around the mouth and chin in children aged 13-15 years are presented. This form of purpura was undoubtedly due to self-mutilation by establishing of a vacuum over the skin produced by a tumbler from which the air had been partially aspirated. This is regarded as a symptom of hysterical conversion on account of stressing conditions at home or in school. Strategies for making the patient and the parents aware of the causal connection and the possibilities for intervention are mentioned.

Adolescent↗

Clinical manifestations of congenital insensitivity of the hand and classification of syndromes.

Lesions of the upper extremity, particularly of the hand, are common in congenital insensitivity syndromes. Five cases are described with findings including fractures, infections, stiffness of fingers, self-mutilation, and traumatic amputations. The best form of treatment is preventative, since reconstructive surgery has little to offer. If conservative efforts are of no avail, amputation of the part is, unfortunately, the best way to obtain satisfactory results.

Adolescent↗

Further observations on the use of 5-hydroxytryptophan in a child with Lesch-Nyhan syndrome.

The effects of 5-hydroxytryptophan (5-HTP), a serotonin precursor, and carbidopa, a peripheral aromatic amino acid decarboxylase inhibitor, were studied in a boy with the Lesch-Nyhan syndrome. No effects on the self-mutilating behaviors of this disorder were noted. However, sleep patterns were normalized, with a reduction in the proportionate amounts of wakefulness and an augmentation of active sleep. A sharp reduction in waking behavioral dyskinesias was also correlated to increased drug dosage.

5-Hydroxytryptophan↗

Relation of clinical variables to dissociative phenomena in eating disorders.

In this study 30 female patients with eating disorders were compared with 30 age-matched normal female subjects. The patients demonstrated significantly higher levels of dissociative psychopathology than the comparison subjects. Furthermore, the presence of severe dissociative experience appeared to be specifically related to a propensity for self-mutilation and suicidal behavior. These findings are discussed in light of recent data which suggest that neurochemical systems shown to be abnormal in patients with eating disorders may be key pathophysiologic substrates for dissociative experience.

Adolescent↗

Undetected abuse among intensive case management clients.

A study of the first 89 clients referred to an intensive case management program revealed substantial previously undetected physical and sexual abuse, yet none of the clients had ever been asked about their abuse experiences. Fifty percent of the clients were adult children of alcoholics, and 34 percent were childhood sexual abuse victims. The adult children of alcoholics experienced significantly more of every kind of abuse, including incest and childhood physical and sexual abuse, than clients whose parents were not alcoholic. Substance abuse problems were reported by more than half of the clients and by 75 percent of the adult children of alcoholics. Self-mutilation and heavy use of mental health services were also correlated with having an alcoholic parent. The authors suggest that mental health providers make routine inquiry about clients' family history of alcoholism and history of physical and sexual abuse.

Adult↗

[Disturbances of body experience in schizophrenic patients].

Disturbances of body experience in schizophrenia patients occur frequently. They vary phenomenologically and lack exact and distinct definitions. Their theoretical and clinical relevance remains widely unclear. This review summarises the literature on clinically relevant symptoms such as coenaesthesis and body hallucinations, disturbances of pain perception, out-of-body-experiences, dysmorphophobia and self-injuries or self-mutilation. Empirical studies on the concepts of body schema, body concept and body cathexis are reported. Many of these studies have serious methodological shortcomings. The correlation of disturbances of body experience with other psychopathology is considered. Standardised methods for assessing these disturbances are listed. Effects of body-oriented psychotherapy have been suggested, but not empirically tested. Finally, the possible relevance of further research in this field is discussed.

Body Image↗