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Self-destructive dermatoses.

It appears certain that the causes of self-destructive dermatoses are many and complex. The disorder spans diagnostic categories and varies from unconscious picking at the skin to severe self-destructive actions. Although not limited to any one diagnosis, skin disorders appear to be more prevalent in depression. This association may involve activation of the hypothalamic-pituitary-adrenal axis commonly found in depression. Two specific types of commonly occurring dermatoses-neurotic excoriations and dermatitis artefacta-are reviewed in this article. The major distinction of these disorders centers on whether the patient can admit to self-mutilation. Because of the difficulties in dermatitis artefacta with insight and body-image, it has been compared with anorexia nervosa. Often, dermatitis artefacta coexists with anorexia nervosa. In both disorders, neurotic excoriations and dermatitis artefacta, the personality style tends to be introverted with emotional immaturity. These patients have difficulty when they are under stress; the problem is compounded because of poor communication skills. Pharmacotherapy is of limited usefulness, and psychotherapy is often times hindered by strong resistance to exploring long-standing emotional issues. Once an alliance is established with the therapist, however, these issues may be examined. Prognosis is variable but does seem to directly correlate with the duration of the illness. Young individuals may experience alleviation of symptoms after one session of psychotherapy, whereas older patients may never have resolution. Dermatologic abuse involving psychosis has many presentations; one of the most common involves infestation. Organic causes must always be excluded as part of the differential diagnosis. In schizophrenia, this presentation has one of the highest incidences of suicide.(ABSTRACT TRUNCATED AT 250 WORDS)

Conflict, Psychological↗

Evidence that D-1 dopamine receptors contribute to the supersensitive behavioral responses induced by L-dihydroxyphenylalanine in rats treated neonatally with 6-hydroxydopamine.

The present investigation supports the hypothesis that functionally supersensitive D-1 dopamine receptors are involved in the self-mutilation behavior (SMB) induced by L-dihydroxyphenylalanine (L-dopa) in rats treated neonatally with 6-hydroxydopamine (6-OHDA). This conclusion is based upon 1) the antagonism of this behavior by SCH-23390, a D-1 antagonist; 2) induction of SMB in neonatal-6-OHDA-treated rats by the D-1 agonist, SKF-38393; 3) the high correlation of the supersensitive locomotor responses to the D-1 agonist with the occurrence of L-dopa-induced SMB; and 4) the inability of the D-2 agonist, LY-171555, to induce SMB in rats treated neonatally with 6-OHDA. The specificity of SCH-23390 and SKF-38393 for the D-1 dopamine receptor was supported by the absence of action of SCH-23390 against locomotor activities induced by LY-171555 and its blockade of SKF-38393-induced locomotion in 6-OHDA-treated rats. Behavioral responses to D-1 and D-2 agonists did not show the same profile in adult and neonatally 6-OHDA-treated rats, providing further support for the view that the age at which dopaminergic neurons are destroyed has differing effects on motor output. Many of the behaviors observed when the D-2 dopamine receptor was activated by LY-171555 were apparent after SKF-38393 in neonatally 6-OHDA-treated rats. Similar behavioral responses to the D-1 and D-2 agonists were also observed in adult 6-OHDA-treated rats.(ABSTRACT TRUNCATED AT 250 WORDS)

2,3,4,5-Tetrahydro-7,8-dihydroxy-1-phenyl-1H-3-ben↗

[Morphology findings in intentional hand amputation].

Presented is the case of a 33 years old farmer, who alleged to have lost his left hand accidentally when grasping into a chuff-cutter. Immediate investigation of the amputate proved, that the hand must have been severed initially by a single cut and was only secondarily bruised by multiple blunt contusions. The farmer confessed to have mutilated himself with some kind of a guillotine which he had constructed for that particular purpose. The amputate was then thrown into the chuff-cutter to make his allegation appear credible and to prevent replantation. The motivation of this self-mutilation was the expectation of considerable insurance payments.

Adult↗

Current concepts of hyperuricemia and gout.

Recent studies have confirmed that gout is an inborn error of metabolism. It has now become evident that the hyperuricemia associated with gout might occur either due to overproduction of uric acid, underexcretion of uric acid or a combination of these processes. Furthermore, patients with excessive purine synthesis may have a specific enzyme defect resulting in altered feedback inhibition of purine synthesis. A neurological disease manifest by mental retardation, choreo-athetosis, aggressive behavior, lip-biting and self-mutilation and associated with decidedly increased purine biosynthesis serves as a prototype of this kind of disorder. Other defects in regulation of purine biosynthesis have been postulated but their existence not yet confirmed. It has been demonstrated that urate crystals which are deposited from hyperuricemic body fluids set up an acute inflammatory reaction by means of a variety of chemical mediators. Thus, acute gouty arthritis is now recognized as an example of "crystal induced" synovitis. The treatment of gout consists of (1) the control of acute gouty attacks, and (2) the maintenance of normal serum uric acid concentrations. This latter may be achieved either with uricosuric drugs or with xanthine oxidase inhibition. With these principles in mind, it is now possible to avoid many of the severe crippling effects of gout and to restore the vast majority of gouty patients to useful and productive lives.

Chemical Phenomena↗

Behavioral differences between neonatal and adult 6-hydroxydopamine-treated rats to dopamine agonists: relevance to neurological symptoms in clinical syndromes with reduced brain dopamine.

Administration of L-dopa or apomorphine to neonatal and adult 6-hydroxydopamine (6-OHDA)-treated rats resulted in different behavioral responses depending on the age at which dopaminergic fibers were destroyed. When neonatal 6-OHDA-treated rats were tested as adults, they exhibited marked stereotypies, self-biting and self-mutilation behavior (SMB) when given these dopamine agonists. Self-biting as well as the incidence of SMB in neonatal 6-OHDA-treated rats showed dose-related changes between 10 and 100 mg/kg of L-dopa. This SMB and self-biting after L-dopa was observed as early as 22 to 24 days of age. Adult 6-OHDA-treated rats did not exhibit SMB or self-biting to L-dopa (100 mg/kg) or apomorphine (10 mg/kg), but did display paw treading and head nodding--behaviors not observed in neonatal 6-OHDA-treated rats. In addition, the locomotor response to apomorphine (1 mg/kg) was significantly greater in adult 6-OHDA-treated rats than in neonatal 6-OHDA-treated rats. Brain dopamine was reduced markedly in striatum, nucleus accumbens and olfactory tubercles in both 6-OHDA treatment groups with the reduction being slightly greater in rats treated with 6-OHDA neonatally. Serotonin content was elevated in striatum of rats treated neonatally with 6-OHDA, but not in adult 6-OHDA-treated rats. SMB and behaviors observed after L-dopa in rats treated neonatally with 6-OHDA were not apparent after L-dopa in rats with brain serotonin or norepinephrine reduced. Rats with brain dopaminergic fibers destroyed neonatally exhibited self-biting and SMB after L-dopa, suggesting that neonatal reduction of this amine is responsible for the SMB and self-biting in neonatal 6-OHDA-treated rats. 5-Hydroxytryptophan administration to neonatal 6-OHDA-treated rats did not induce SMB, indicating that release of serotonin by L-dopa is not responsible for this behavior. Because inhibition of dopamine-beta-hydroxylase did not alter the SMB response to L-dopa observed in neonatal 6-OHDA-treated rats, norepinephrine synthesized from L-dopa does not appear to contribute to the response. High doses of a decarboxylase inhibitor sufficient to inhibit conversion of dopa to dopamine in brain did not reduce the incidence of SMB. Administration of haloperidol (1 mg/kg) reduced the incidence of SMB, but did not antagonize the self-biting or the taffy pulling exhibited by L-dopa. In contrast, cisflupentixol completely blocked the SMB and self-biting induced by L-dopa.(ABSTRACT TRUNCATED AT 400 WORDS)

Age Factors↗

Congenital sensory neuropathy with anhydrosis-a case report and investigation of autonomic nervous system abnormalities.

A review of the clinical profile of congenital sensory neuropathy with anhydrosis is presented. It is stressed that major diagnostic criteria of this recessively inherited condition should be limited to insensitivity to pain with normal tactile perception, anhydrosis, recurrent unexplained fever, self-mutilation, mental retardation, hypotonia, histologically normal sweat glands and variable autonomic abnormality. A case conforming to this description is reported and compared with 13 published cases. Special investigations of the autonomic nervous system through measurement of urinary catecholamine metabolites and psychophysiologic variables were conducted on this patient. Based on the analysis of 5 X 24-hour urine, values of metabolites of dopamine and epinephrine were normal. Metabolites of norepinephrine, such as 3-methoxy-4-hydroxy phenylglycol and normetanephrine, however, were significantly low when compared with those of four controls, suggesting decreased peripheral and central norepinephrine activity. Polygraph recording and evaluation of some orienting response components revealed no obvious signs of autonomic perturbation and, specifically, no phasic electrodermal activity. These two findings (biochemical and electrodermal) strongly suggest an autonomic imbalance, specifically component, both central and peripheral. It is suggested that autonomic disorder is an integral part of the syndrome and may be demonstrated by special investigations.

Autonomic Nervous System Diseases↗

Congenital insensitivity to pain with anhidrosis in a 2-month-old boy.

In a 2-month-old boy with congenital insensitivity to pain and anhidrosis, the initial symptom was recurrent fever of unknown origin. After eruption of the first teeth, self-mutilation of the tongue was limited by extraction of the teeth. Unmyelinated fibers were essentially lacking, and the number of small myelinated fibers was decreased in sural nerve. This case is the youngest to be diagnosed and the first one in which nerve biopsy clearly showed the characteristic findings.

Humans↗

Local anesthetics in dental practice.

Local anesthetics are the most widely used drugs in dentistry today. Knowledge of the pharmacology and toxicology of these agents will result in their intelligent and judicious use. The choice of local anesthetic should be individualized for each patient. The duration of the dental procedure should be weighed against the duration of action of the local anesthetic; a decision should be made as to whether a vasoconstrictor is needed to prolong its action. However, if the duration of numbness is too long, the possibility of self-mutilation must be considered in certain patients (for example, children and the mentally retarded). In other patients with whom postoperative pain is expected, it may be beneficial to administer a long-acting local anesthetic such as bupivacaine for control of postoperative pain. The total dose of local anesthetic and vasoconstrictor must be determined for each patient based upon body weight; the maximal dosages for each agent should be known. Small children or frail individuals will require below average dosages. The use of a vasoconstrictor may constitute the limiting factor to the total number of local anesthetic cartridges that can be administered safely over a given period of time. Certain medical problems, such as cardiovascular system impairments or hyperthyroidism, may influence the choice of anesthetic and the quantity of vasoconstrictor. An understanding of the physicochemical properties of local anesthetics is also important to a rational process of selection. There are several causes for failure to achieve profound regional anesthesia. These include inflammation and/or infection, anatomic variation, intravascular injection, accessory innervation, and deflection of the needle. Inflammation and infection reduce the efficacy of a local anesthetic by reducing its bioavailability. Local anesthetics with low pKa values (for instance, mepivacaine) are the most effective in this clinical situation. Other causes of inadequate regional anesthesia are primarily related to technique of administration and can be circumvented by the use of the periodontal ligament injection. A comprehensive review of this technique and its application has been presented by the Council on Dental Materials and Equipment of the American Dental Association. Occasionally, a clinician may be unsuccessful at achieving regional anesthesia despite these additional measures. Highly anxious dental patients or patients with a genuine tolerance to local anesthetics normally pose the most problems. Transitional block or threshold block phenomena should also be suspected in these situations.(ABSTRACT TRUNCATED AT 400 WORDS)

Anesthesia, Dental↗

Clinical vampirism. A presentation of 3 cases and a re-evaluation of Haigh, the 'acid-bath murderer'.

Clinical vampirism is named after the mythical vampire, and is a recognizable, although rare, clinical entity characterized by periodic compulsive blood-drinking, affinity with the dead and uncertain identity. It is hypothetically the expression of an inherited archaic myth, the act of taking blood being a ritual that gives temporary relief. From ancient times vampirists have given substance to belief in the existence of supernatural vampires. Four vampirists, including Haigh, the 'acid-bath murderer', are described. From childhood they cut themselves, drank their own, exogenous human or animal blood to relieve a craving, dreamed of blood-shed, associated with the dead, and had a changing identity. They were intelligent, with no family mental or social pathology. Some self-cutters are auto-vampirists; females are not likely to assault others for blood, but males are potentially dangerous. Vampirism may be a cause of unpredictable repeated assault and murder, and should be looked for in violent criminals who are self-mutilators. No specific treatment is known.

Adult↗

[Spectacles for dogs and cats (author's transl)].

Indications for using spectacles in dogs and cats are reviewed. These indications are classified into the following categories: (1) correction of visual acuity, (2) protection against external irritants such as ultraviolet rays and wind, (3) prevention of self-mutilation and (4) psychological reasons. The only justifiable indications for the use of spectacles or sun-glasses in dogs or cats consist in the treatment or prevention of some ophthalmic disorders.

Animals↗

Factitious vesicocutaneous fistula: an enigma in diagnosis and treatment.

A case report of a patient with a recurrent factitial vesicocutaneous and a tensor fasciae latae musculocutaneous flap were employed in reconstruction. Difficulty in diagnosis and treatment of this patient with factitial disease is emphasized. Excessive hospitalization and cost reflect these difficulties. A life-threatening disease was modified by planned reconstruction coordinated with antidepressant medication and psychotherapy. Treatment centered on avoiding insight and maintaining denial ASA defense mechanism. The transference of self-mutilation in the suprapubic region to the donor thigh was most important in this patient's survival.

Adult↗

Lesch-Nyhan syndrome in an Arab family. Detection and biochemical manifestation of heterozygosity.

A large Arab family affected with the rare X-linked Lesch-Nyhan syndrome is reported on. Two hemizygous boys, two and nine years of age, had the classical biochemical and clinical-neurological syndrome. The activity of erythrocyte hypoxanthine-guanine phosphoribosyltransferase (HGPRT) was below the detectable limit (greater than 0.1% of normal). They were mentally and physically retarded and exhibited spasticity and choreoathetosis; the older of the two also exhibited self-mutilation. The mother and three of her seven daughters, all clinically asymptomatic, were proven to be heterozygous for HGPRT deficiency, by demonstration of an increased rate of de novo purine synthesis in cultured skin fibroblasts. Erythrocyte HGPRT activity was normal in the three heterozygous daughters, but was significantly reduced in the mother. However, in all four heterozygotes, erythrocyte HGPRT/adenine phosphoribosyltransferase ratio was lower than in all other family members. All heterozygotes had blood uric acid levels within the normal range, although higher than in the normal women in the family. The ratio uric acid/creatinine concentration in the urine was significantly elevated in one of the heterozygotes, and in the upper normal limit in two others, indicating excessive purine production.

Adolescent↗

Youth in crisis: dimensions of self-destructive conduct among adolescent prisoners.

Self-mutilation and attempted suidcide among adolescent prisoners are explored in relation to concrete coping tests posed in prison and to self-esteem problems posed by failure of external (family) and internal (peer) support systems. Crisis sequences are traced using verbatim excerpts from interviews with self-destructive prisoners and conceptualized in terms of enduring adolescent needs and concerns. Some general observations regarding strategies of intervention with crisisprone prisoners are included.

Adjustment Disorders↗

Ataxia and disorders of purine metabolism: defects in hypoxanthine guanine phosphoribosyl transferase and clinical ataxia.

A relationship between disordered metabolism of purines and the central nervous system has been established by the Lesch-Nyhan syndrome. In this disorder a virtually complete defect in the activity of HGPRT is associated with a syndrome of severe mental retardation, choreoathetoid cerebral palsy, and bizarre, self-mutilative behavior. In patients with partial defects in HGPRT, two have had symptoms that have been labeled spinocerebellar. Neither were appreciably ataxic, and the relationship between the symptoms and the enzyme defect remains to be established. Analysis of HGPRT in members of a large kindred with spinocerebellar degeneration revealed normal levels of the enzyme. These observations suggest that a relationship between the activity of HGPRT and clinical ataxia is remote.

Ataxia↗

Trauma and the development of borderline personality disorder.

Prolonged and severe trauma, particularly trauma that occurs early in the life cycle, tends to result in a chronic inability to modulate emotions. When this occurs, people can mobilize a range of behaviors that are best understood as attempts at self-soothing. Some of these attempts include clinging and indiscriminate relationships with others in which old traumas are re-enacted over time, as well as more self-directed behaviors such as self-mutilation, eating disorders, and substance abuse. Usually, these behaviors will coexist. Patients with complicated trauma histories often repetitively attempt suicide or engage in chronic self-destructive behavior, and need to address issues of childhood trauma, neglect, and abandonment, both in the past and as re-experienced in current relationships. When treating these patients, therapists must anticipate that painful affects related to interpersonal safety, anger, and emotional needs may give rise to dissociative episodes, which may, in turn, be accompanied by increased self-destructive behavior. The therapy must clarify how current stresses are experienced as a return of past traumas and how small disruptions in present relationships are seen as a repetition of prior abandonment. As part of this, it is essential that the therapist provide validation and support, and avoid participating in a re-enactment of the trauma. Fear needs to be tamed in order for people to be able to think and be conscious of current needs. This bodily response of fear can be mitigated by safety of attachments, security of meaning schemes, and by a body whose reactions to environmental stress can be predicted and controlled. One of the great mysteries of the processing of traumatic experience is that as long as the trauma is experienced as speechless terror, the body continues to keep score and react to conditioned stimuli as a return of the trauma. When the mind is able to create symbolic representations of these past experiences, however, there often seems to be a taming of terror, a desomatization of experience. As Ducey and van der Kolk found in the Rorschachs of Vietnam veterans, patients were unresponsive to outside influences (good or bad) as long as they remained in a state of psychic numbing. Faced with intrusions of past trauma in their current emotional life, patients' initial sense of being overwhelmed was mastered only when a link between past trauma and current perceptions became understood.(ABSTRACT TRUNCATED AT 400 WORDS)

Borderline Personality Disorder↗

Lesch-Nyhan Syndrome: report on two brothers.

Lesch-Nyhan syndrome is a rare X-linked disease characterized by over-production of uric acid and a central nervous system (CNS) disorder consisting of mental retardation, spasticity, choreoathetosis, and a compulsive form of self-mutilation. A deficiency in hypoxanthine-guanine phosphoribosyl transferase (HPRT) provides the underlying metabolic basis for this disease. A 12 month-old male baby who had orange crystals over the diapers since he was 3 months old was brought to our hospital due to developmental delay. Mental retardation and athetosis were also noted. Chemical analysis revealed hyperuricemia (uric acid 8.6 mg/dl). Urine routine showed microscopic hematuria and uric acid crystals. The activity of HPRT in erythrocyte lysates of parents were both within normal limits, but that of the patient was very low (0.0547 nm/min/mg protein, < 0.05% of control). His younger brother was born 2 months after this disorder diagnosed in this patient. The younger brother was noted to have uric acid crystals over the diapers when he was 40 days old and hyperuricemia (10.6 mg/dl) showed up later. He was also a case of Lesch-Nyhan syndrome since the activity of HPRT in erythrocyte lysates was also low (0.0327 nmol/min/mg protein, < 0.05% of control). Further studies, including carrier detection and deoxyribonucleic acid (DNA) analysis, could be helpful for genetic counseling. This syndrome is rare among Chinese, and this may be due to underdiagnosis.

Family Health↗

Use of naltrexone for treatment of psychogenically induced dermatoses in five zoo animals.

Naltrexone was used successfully in controlling self-mutilation behavior in 3 zoo animals from 2 families (Felidae and Sciuridae). It had no effect on 2 other zoo animals from 2 families (Canidae and Ursidae). Long-term administration of glucocorticoids was not satisfactory in controlling the behaviors, and concerns were raised about potential long-term adverse effects, especially with regard to fertility. Detrimental effects were not noticed in any of the zoo animals treated with naltrexone. Two males successfully sired healthy offspring.

Animals↗

[Self-castration with suicide].

Suicide of a 31 year old man with multiple slashes and stab wounds including complete amputation of penis, scrotum and testicles is reported. Wound patterns in genital self-mutilations are discussed. Despite serious loss of blood the reported survival periods are remarkable; death from bleeding seems to be rare. A review of similar cases shows that self-castrations as well as other self-inflicted genital mutilations are usually associated with psychiatric disorders and transsexuality or hypersexuality.

Adult↗