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[A cause of multiple failures in autoplasties: cutaneous pathomimesis].

The authors report a case of cutaneous pathomimesis of the hand with severe self-mutilation, contrasting with the minimal nature of the psychiatric illness. They recall several pathogenic and therapeutic elements for this disease which, although difficult, should be managed by a multidisciplinary approach.

Adult↗

[Effects of muscimol on aggressive behaviors induced by clonidine in mice].

A behavioral study was carried out to clarify a relationship between the GABAergic and purinergic central system in aggressive behaviors induced by clonidine in mice. Mice administered a high dose of clonidine (20 mg/kg, i.p.) exhibited aggressive behaviors such as biting and attacking. These behaviors are inhibited by L-PIA (N6-L-phenylisopropyl adenosine) and stimulated by caffeine, which suggest that a blockade of adenosine receptors is involved in these behaviors. Muscimol (0.5-2 mg/kg, i.p.), a GABA-a receptor agonist, not only markedly potentiated clonidine (20 mg/kg i.p.)-induced aggressive behaviors but also elicited characteristic behaviors such as gnawing, reinforced irritability, and self-mutilation. Bicuculline (1, 2 mg/kg, i.p.), a GABA-a receptor antagonist, or picrotoxin (1 mg/kg, i.p.), a chloride channel blocker, did not significantly affect clonidine (20 mg/kg, i.p.)-induced aggressive behaviors. The potentiating effects of muscimol (0.5, 1 mg/kg, i.p.) on clonidine-induced aggressive behaviors were antagonized by bicuculline (1, 2 mg/kg, i.p.), but not affected significantly by picrotoxin (1 mg/kg, i.p.). L-PIA (0.2 mg/kg, i.p.) reduced clonidine-induced aggressive behaviors and also reversed the potentiating effects of muscimol. Stereotyped gnawing behaviors induced by combined treatment of muscimol (0.5, 1 mg/kg, i.p.) and clonidine (20 mg/kg, i.p.) were not affected by bicuculline (2 mg/kg, i.p.). The results suggest that the potentiating effects of muscimol on clonidine-induced aggressive behaviors may be induced via the stimulation of GABA-a receptors, although not necessarily associated with chloride channel functions, and may involve certain interactions between the stimulation of GABA-a receptors and the inhibition of adenosine receptors.(ABSTRACT TRUNCATED AT 250 WORDS)

Aggression↗

Alcohol intoxication in teenagers using inhalant stupefacients.

The study was carried out 11 boys, 12-17 years old, treated in the Therapeutic-Educational Guidance Center for the Young because of using inhalant stupefacients and incliniation to alcohol abuse. The inhalant stupefacients were taken for a period of 6 months to three years, of alcohol--from 6 months to 2 years. The most common inhalant stupefacients were "Butapren" glue, trichlorethylene and "Roxy" fluid; wine and vodka were the alcohols used. No one patient displayed the full dependence syndrome, most of them (83%) had organic CNS damage, in 63% pathological EEG changes were recorded. The alcohol inebriation was accompanied by marked psychomotor excitation with cognitive disturbances, aggressiveness, anxiety, self-mutilation. One patient developed visual illusions and hallucinations. The findings suggest that the consequences of abuse of inhalant stupefacients significantly influence the syndrome of alcohol intoxication, potentiating the psychopathological signs.

Adolescent↗

[A neurologic model of early infantile autism].

Based on the abnormalities in sleep-wakefulness cycle of early infantile autism, the author discussed its pathophysiology focusing on its main lesion in the raphe nuclei. These neurons, located in the midline portion of the brainstem send their axons to various neurons of the upper and lower nervous systems, including the locus coeruleus and the dopamine neurons of the tegmentum, the former having a broad innervation and the latter a restricted area in the central nervous system. These monoaminergic neurons modulate the functions of the involved neurons and regulate their functional and structural maturation in the early developmental course. The early lesion of the raphe nuclei causes poor adaptation to environment which develops as abnormal circadian oscillation and pervasive lack of responsiveness. Combined hypofunction of the locus ceruleus, particularly of its dorsal bundle, results in the failure of extinction of acquired memory in mice which relates clinically to the excellent memory and resistance to change peculiar interests and attachments in humans. From early childhood, the disturbance of dopaminergic neurons becomes apparent clinically, and causes hyperkinesia and stereotyped activities. With the other two monoaminergic neurons, dopaminergic neurons cause occasional aggressiveness or self-mutilation. The latter behaviors are like those of pampered children and are simulated to "muricide" and "friendliness" observed in rats with these monoaminergic lesions. The particular language disturbance with echolalia is due to the right hemispheric dominance, which might have been caused by a delayed functional lateralization of the hemisphere resulting also from the delayed development of the circadian oscillation in infancy. The motor disturbances consisting of hypotonia and impaired locomotion might be due to decreased tonic innervations of the locus ceruleus and the raphe nuclei to the spinal locomotion center. CT examination of symptomatic autism showed the amygdala as one of the causative nuclei for the autistic behavior.

Animals↗

The role of the HPRT gene in human disease.

Human HPRT deficiency leads to two major forms of human disease. Partial enzyme deficiency results in gouty arthritis, while an almost complete deficiency leads to the Lesch-Nyhan disease. The latter is characterized by severe neurological dysfunction in addition to gouty arthritis, including retardation, choreoathetosis and aggressive and compulsive self-mutilation. The biochemical basis for the neurological symptoms is not understood. The human and mouse cDNA (RNA copy) genes have been isolated and sequenced. In addition, the amino acid sequence of the human protein has been directly determined. The human and mouse proteins differ at 7 amino acids out of the total, (including the N terminal methionine, which is processed off during maturation) of 218. There are 42 out of 654 nucleotide differences between the human and mouse genes in the amino acid coding region. The mouse genomic structure has been determined. It has 9 exons and 8 introns with a total size of approximately 36 kb. The human gene is very similar with identical intron-exon junction points and approximately the same total gene size. Both mouse and human presumed promotor region at the 5' end, lack a recognizable promotor in the form of a "TATAA" box and are very G-C rich, though not the same. This may be a feature of most "housekeeping" genes. HPRT gene point mutations in three gouty arthritis and one Lesch-Nyhan patient have been identified by peptide sequencing. Six gross gene rearrangements have been identified in Lesch-Nyhan HPRT genes. However it is likely that most mutations are point mutations or small deletions. So far all gene mutations identified are different from all others. The gene has been engineered into retrovirus vehicles which allows its efficient introduction into a wide variety of cells, including mouse marrow stem cells. This may allow treatment of Lesch-Nyhan patients as a model of gene therapy.

Amino Acid Sequence↗

[Who is left in the institutions? Some problems in connection with the process of deinstitutionalization].

The medical and psychiatric diagnoses of 168 clients aged 16 to 65 years in a Norwegian institution for the mentally retarded were registered together with medication, level of functioning, and types of problem behavior. Most of these clients (64.9%) were profoundly or deeply retarded, and only 6.5% were independent of continuous supervision or help. Medical diagnoses were found in 87.5%, psychiatric diagnoses in 89.1% (DSM-III, axis I). Daily use of medication was found in 81.0% of the clients, and 48.9% used psychotropic drugs on a daily schedule. 58.3% of the clients had exhibited violent behavior during the last year, defined as assaults on persons, self-mutilation or destructiveness. The article discusses the implications of these observations for primary health care, which is expected to supply the necessary service to these clients after 1991.

Adolescent↗

Tattoos, body experience, and body image boundary among violent male offenders.

The author compared a measure of body image boundary and medically significant bodily experiences between 21 tattooed and 24 nontattooed men incarcerated for violent crimes. Although the tattooed and nontattooed subjects had no significant differences in their body boundary concepts or most other bodily experiences, the tattooed men were found to have a different distribution of scars on their bodies. Upon more detailed examination, it seemed that these different distributions could be explained by the observation that the tattooed subjects were the only ones with self-inflicted cuts. This finding further supports the notion that tattoos, despite their ostensibly decorative quality, may be a form of self-mutilation.

Adult↗

Peripheral nerve injury associated with fracture or fracture-dislocation of the pelvis in dogs and cats: 34 cases (1978-1982).

Peripheral nerve injury was associated with fracture or fracture-dislocation of the pelvis in 23 dogs and 11 cats. In most cases, peripheral nerve injury resulted from ilial fracture with craniomedial displacement of bone fragments, or from sacroiliac fracture-dislocation with cranial displacement of the ilium. Sciatic nerve injury was associated with fracture of the acetabulum in 2 dogs. A combination of sensory, voluntary motor, and reflex abnormalities were observed in the ipsilateral extremity of affected animals. Signs of severe pain were noticed in 4 of the dogs and in 1 cat. Eight of the animals died or were euthanatized for reasons not directly related to the severity of peripheral nerve injury. Of the remaining 26 animals, 21 (81%) had good or excellent limb function 16 weeks after peripheral nerve injury was sustained. Limb function was lost or self-mutilation occurred in 4 (15%) of the animals.

Animals↗

[Cranial computerized tomography in incomplete Lesch-Nyhan syndrome].

The CT brain scan of a 17-year-old patient with primary hyperuricaemia and mental retardation is presented. The examination demonstrates subcortical and cortical atrophy of the brain. The HGPRTase level was below normal. Clinical evidence of self-mutilation or tophi was not found. This patient's condition was interpreted as an incomplete Lesch-Nyhan syndrome.

Adolescent↗

[Diagnosis and sequelae of sexual child abuse].

This paper reviews briefly epidemiological and clinical data on prevalence and incidence of child-sexual-abuse. Individual symptoms and family factors of intra-familial child sexual abuse are described and some possible psychopathological factors and vulnerability factors are listed. Two different forms of parasuicide and self-mutilating behaviour are distinguished and complicating factors of secondary traumatisation are described.

Adaptation, Psychological↗

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↗

[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↗