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A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome: a case report.

Lesch-Nyhan syndrome is a rare X-linked recessive disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. Persons affected with this incurable disease are developmentally and physically delayed, and suffer from self-injurious behavior. The most typical feature results in partial or total destruction of perioral tissues. The purpose of this paper was to discuss a case of Lesch-Nyhan syndrome with self-mutilative behavior and lip injuries, including some suggestions of a preventive approach avoiding the extraction of teeth. A soft mouthguard fabricated to prevent the destruction of perioral soft tissues and combined psychiatric pharmacologic therapy proved to have satisfactory results.

Child, Preschool↗

[Lesch-Nyhan syndrome without self-mutilation: biochemical and morphological studies on blood cells (author's transl)].

Absent enzyme activity of hypoxanthine-guanine-phosphoribosyl transferase in erythrocytes was demonstrated in the blood from a 21-year-old man with spastic tetraplegia, choreoathetosis, oligophrenia, and hyperruricaemia which developed after birth. But there was no tendency towards self-mutilation, considered to be characteristic of the full-blown picture of the disease. This case thus runs counter to the view that the enzyme defect is the cause of the inevitable, stereotyped change in behaviour. Unusual morphological changes of all blood-cell systems confirm the dependence of nucleic acid synthesis on the "salvage pathway" of the purine nucleotides, which is abnormal in this inherited disease.

Adult↗

Self-injurious behavior: a review of the behavior and biology of self-mutilation.

OBJECTIVE: The authors describe the clinical characteristics of self-injurious behavior, giving special emphasis to self-injurious behavior occurring among individuals with character disorders. DATA COLLECTION: They review data suggesting the involvement of serotonergic, dopaminergic, and opiate neurotransmitter systems in the expression of self-injurious behavior. FINDINGS: Self-injurious behavior occurs among mentally retarded individuals, psychotic patients, prison populations, and individuals with severe character disorders. Although theoretical psychological models of self-injurious behavior are helpful in understanding the patient's experience of self-injury, no generally useful therapeutic approach has yet evolved from these models. Data derived from animal models and treatment studies suggest the involvement of opiatergic and dopaminergic mechanisms in self-injury among the mentally retarded. Serotonergic influences on self-injurious behavior may be present in varying forms of this behavior. The scientific literature on the benefits of pharmacological agents for mentally retarded individuals is beset with a number of problems. Support is emerging, however, for the use of lithium and carbamazepine with self-injuring mentally retarded patients, and some behavioral interventions appear to be successful for mentally retarded individuals. Self-injuring patients with borderline personality disorder may benefit from milieu treatment. CONCLUSIONS: Although no form of treatment has yet been demonstrated to be of general benefit, the literature suggests that therapeutic trials with dopamine antagonists, serotonin reuptake inhibitors, and opiate antagonists may be of value.

Adolescent↗

Self-mutilation in a new mother: a strategy for separating from her infant.

A primiparous mother, aged 25 years, first presented for treatment when her infant was three months of age. A comprehensive case history disclosed that the mother had experienced two prior full-fledged psychotic episodes requiring hospitalization. During treatment, the mother underwent an abortion and burned her face with kitchen chemicals. This case history reveals a pattern of self-mutilating behavior whenever the mother is confronted with events threatening imminent separation or individuation.

Abortion, Induced↗

Cognitive-behavioral approaches to treating borderline and self-mutilating patients.

The cases of three self-injurious patients suffering with borderline personality disorder are briefly presented. From this clinical base, the consultant describes a cognitive-behavioral psychotherapeutic model and how it might be applied to the treatment of these and similar patients. A dialectical behavioral approach is used to describe three major dichotomies and their application. The consultant also illustrates how cognitive-behavioral therapy may be integrated with more dynamic-analytic approaches, and proposes a strategy and methodology that establish and reinforce the treatment alliance in developing a treatment plan.

Adult↗

Effects of lesions in the anterolateral columns and dorsolateral funiculi on self-mutilation behavior in rats.

The possible role of the anterolateral columns (ALCs) and dorsolateral funiculi (DLF) in pain mechanisms was examined from the effects of lesions in these tracts (alone or combined) on tests for chronic deafferentation pain (autotomy) in rats. Spinal lesions alone (i.e., without denervation) in either ALC or DLF or combined DLF-ALC did not lead to any form of self-mutilation behavior. Cervical surgery, without spinal lesion, followed by limb denervation (sham) resulted in similar autotomy characteristics to those observed following limb denervation alone (control). Both results were considered as one set of controls. ALC lesions simultaneous with, or 1-2 weeks prior to limb denervation (ipsilaterally or contralaterally) produced significant delay in onset of autotomy and decrease in percentage of rats showing this behavior. DLF lesions followed by limb denervation produced significant acceleration of onset of autotomy and increase in percentage of rats showing this behavior. Combined DLF-ALC lesions with limb denervation produced intermediate effects between those observed following either ALC or DLF lesions alone. These results give further support to the concept that autotomy is related to rostral transmission of nociceptive information and that a spino-bulbo-spinal inhibitory loop involving the DLF and ALC is triggered by chronic deafferentation pain.

Animals↗

Differential diagnosis of cerebral palsy: Lesch-Nyhan syndrome without self-mutilation.

Athetotic cerebral palsy was diagnosed in a 6-month-old boy with no history of perinatal trauma. Lesch-Nyhan syndrome (i.e., complete deficiency of hypoxanthine-guanine phosphoribosyltransferase [HGPRT] ) was diagnosed only when the boy began biting his lower lip at the age of 10 years. It is suggested, on the basis of this case and others like it in the literature, that the delayed onset or absence of self-mutilation in patients with Lesch-Nyhan syndrome may be more common than has been previously suspected. In all males said to have cerebral palsy, HGPRT deficiency must be ruled out, preferably by measuring the ratio of uric acid to creatinine in a random urine specimen.

Adenine Phosphoribosyltransferase↗

Genital self-mutilation.

A 53-year-old man was brought to the emergency department having removed both testicles and amputated his penis using a bread knife. Examination of the amputated penis showed it to be unsuitable for an attempted replant procedure. The patient was taken to theatre where the perineal wound was debrided and the remaining urethra brought down as a perineal urethrostomy, with a local cutaneous flap rotated to provide coverage for the urethra. Discussed herein are the incidence, predisposing factors, management and complications of genital self-mutilation in the adult male, and the existing literature is reviewed on the subject.

Amputation, Traumatic↗

Enhanced muscimol-induced behavioral responses after 6-OHDA lesions: relevance to susceptibility for self-mutilation behavior in neonatally lesioned rats.

Adult rats lesioned with 6-hydroxydopamine (6-OHDA), either as neonates or as adults, demonstrated increased turning, compared to unlesioned controls, when muscimol was unilaterally microinjected into the substantia nigra reticulata (SNR). At the higher doses of muscimol, the lesioned rats were so intensely lateralized that circling was impeded. These data suggest a functional supersensitivity of receptors associated with GABA function in the SNR of 6-OHDA-lesioned rats. When 30 ng muscimol was administered bilaterally into the SNR, self-mutilation behavior (SMB) was observed in 2/11 of the control unlesioned rats, in 0/8 adult 6-OHDA-lesioned rats, and in 11/11 of the neonatally-lesioned rats tested. The ability of muscimol to produce SMB in the rats lesioned as neonates was dose related. Behavioral observations indicated that behaviors associated with SMB (self-biting and taffy pulling) were present in neonatal, but not adult lesioned rats. Behavioral responses to dopamine agonist administration were also different between rats lesioned as neonates and those lesioned as adults with 6-OHDA. These data support the view that lesions of dopaminergic neurons cause an increased functional responsiveness of receptors acted upon by muscimol in the SNR, and that the increased susceptibility for SMB in neonatally lesioned rats is determined by neurons distal to the GABA receptor complex in the SNR.

Animals↗

[A contribution to the understanding of Infantile self-mutilation based on communication theory (author's transl)].

Autoaggressive behaviour in childhood often poses diagnostic and even more so, therapeutic problems to the physician. One of the causes on which these problems are based, is that hitherto there was no possibility to look into the "conditional structure" of autoaggressive behaviour in a large population of children using sound statistical methods. The results as reported in this study are based on an "automutilationquestionnaire" as developed by the authors. They support the hypothesis that the different kings of self-mutilation, although phenomenologically quite variable, represent a deficiency of communication. This communication deficiency is manifested in that the child, by being autoaggressive, seeks to satisfy for the insufficiently compensated asymmetric communication with his environment. This result emphasizes in particular the necessity to include the family in the therapeutic regimen of the treatment of the autoaggressive child.

Child↗

Bilateral stereotactic amygdalotomy for self-mutilation disorder. Case report and review of the literature.

Bilateral stereotactic amygdalotomy for the management of patients with severe aggressive behavior disturbances was first introduced by Hideki Narabayashi in 1961. Since then, more than 500 cases have been reported in scientific literature, with a variety of cited behavior improvement rates. The advances of psychopharmacology along with the existent skepticism of the medical community in regards to psychosurgery have resulted in a dramatic decrease in the number of amygdalotomies performed worldwide. In our current communication, we report a case of bilateral stereotactic amygdalotomy for a medically refractory self-mutilation disorder. We also review the pertinent literature with an emphasis on the selection of the most appropriate anatomical targets, the procedure-related complications and the outcomes of this occasionally helpful procedure.

Adult↗

Case report of an unusual use of lidocaine during episodes of self mutilation.

We are reporting on a case of polyintoxication by cocaine, lidocaine, methadone, and dextromoramide. This conclusion is supported by the analysis of a strand of hair. We note for the first time the detection of dextromoramide as well as lidocaine and desethyl-lidocaine in hair. Concentrations in hair were: cocaine = 2.4 ng/mg, benzoylecgonine = 0.3 ng/mg, methadone = 10.2 ng/mg, EDDP = 1.5 ng/mg, dextromoramide = 1.6 ng/mg, lidocaine = 115.9 ng/mg and desethyl-lidocaine = 1.6 ng/mg. The victim who was seeking an anesthesia effect without the loss of consciousness ingested cocktails during episodes of self mutilation. The wounds were of two different types and with different morphological locations: long and deep without ablation of tissue, clean lacerations found on the neck, the pectoral region, and the left upper extremity; either round or discoid with deep excavation found on the head (ears, forehead, chin, and lips) and also, on the neck and on the left upper extremity. Near the most recent wounds, needle marks were noticed indicating probable local infiltration of lidocaine.

Adult↗

Dopamine agonist induced self-mutilative biting behavior in monkeys with unilateral ventromedial tegmental lesions of the brainstem: possible pharmacological model for Lesch-Nyhan syndrome.

We have investigated the effects of various dopamine (DA) agonists on induction of abnormal involuntary movements (AIM) in a group of monkeys which had denervated nigro-striatal DA neurons for 10-14 years rendered by a unilateral surgical ventromedial tegmental (VMT) lesion of the brainstem. The surgical lesions were placed when the monkeys were 2-4 years old. The administration of mixed DA agonists, such as L-DOPA, apomorphine (Apo) and abeorphine 201-678, elicit a self-mutilative biting behavior (SMB) of the forelimb digits contralateral to the lesion, and spasticity of the contralateral hindlimb. These dysfunctions resemble, in some aspects, the neurological disturbances associated with Lesch-Nyhan syndrome. The SMB behavior was elicited by mixed DA agonists which predominantly stimulate D1, but not D2 DA receptors, and was prevented or abolished by the D1 DA antagonist SCH 23390 or by the D1 and D2 DA antagonist fluphenazine (Flu), but not by the D2 antagonist (+/-)sulpiride. These results suggest that DA agonist-induced SMB behavior is mediated by D1 and/or by both D1 and D2 DA receptor pathways. To study the relationships between HPRT, the defective enzyme in Lesch-Nyhan syndrome, and the DA neuronal systems, we have measured the effects of nigro-striatal DA degeneration and intrastriatal neuronal degeneration on HPRT activity. The unilateral 6-OHDA-induced nigro-striatal DA degeneration does not significantly alter the HPRT activity on the lesioned side of the striatum, while the quinolinic acid-induced intrastriatal neuronal degeneration significantly reduces the enzyme activity. These results suggest that HPRT is localized on intrastriatal neurons which are also known to contain DA receptors. It is postulated that HPRT deficiency in Lesch-Nyhan syndrome results in abnormal guanine nucleotide metabolism which may affect the regulation of DA receptors.

Animals↗

Hereditary compulsive self-mutillating behaviour in laboratory rabbits.

During the last few years an increasing number of cases of extensive automutilation has been observed in a rabbit breeding colony of Checkered crosses. Digits and pads of the front feet were traumatized. No other behavioural abnormalities or signs of disease were evident. Self-mutillation was seen both in stock, breeding and experimental animals, in rabbits kept singly in cages and in those housed in groups on the ground, in rabbits kept in different buildings and under the care of different staff members. This behavioural abnormality of Checkered crosses has also been observed in animals after being placed into other institutions or private homes. No evidence of an agent responsible for the occurrence of self-injury could be found with parasitological, mycological, histological, clinical or haematological examination. Twelve to 16 animals are affected yearly in a colony varying in size between 130 and 230 rabbits. Following complete healing, relapses occurred up to 3 times per year, on either the same or the opposite front foot. In the last 21 cases episodes of automutillation could be regularly interrupted with the dopamine antagonist, haloperidol. Similar signs of auto-mutillation were never seen in animals of another breeding line kept in the same building and under the same conditions nor in animals brought in from other breeding colonies. A relatively high coefficient of inbreeding can be presupposed in this 15-year-old breeding colony of Checkered crosses. A genetic predisposition for the behavioural anomaly described appears very likely.

Animals↗

Self-mutilative behavior in adolescents who attempt suicide by overdose.

OBJECTIVE: To examine the cognitive/affective and behavioral symptoms of adolescent suicide attempters with self-mutilative behavior (SMB). METHOD: Adolescents evaluated after a suicide attempt that occurred between 1996 and 2000 were divided into two groups: suicide attempters with a history of SMB (n = 52) and suicide attempters without SMB (n = 43). The groups were then compared on psychiatric diagnosis, suicide intent, depression, hopelessness, loneliness, anger, reckless behavior, substance use, and family functioning. RESULTS: Carving on the skin and picking at a wound were the most commonly reported SMBs, occurring in about one third of the sample. The SMB group was significantly more likely to be diagnosed with oppositional defiant disorder, major depression, and dysthymia and had higher scores on measures of hopelessness, loneliness, anger, risk taking, reckless behavior, and alcohol use than did the non-SMB group. Loneliness increased the odds of SMB almost 6-fold. CONCLUSIONS: Adolescent suicide attempts with SMB are associated with greater cognitive/affective and behavioral symptoms. Adolescent suicide attempters should be routinely screened for SMB.

Adolescent↗

Cervical cord tethering due to split cord malformation at the cervico-dorsal junction presenting with self-mutilation of the fingers.

An unusual case of cervical spinal cord tethering with diplomyelia is described. A 12-month old female presented with self-mutilation of the fingers due to sensory loss in the hands, absent reflexes, poor muscle tone, and reduced distal upper-limb movements. There was a deep skin dimple overlying the T1 spinous process. Imaging showed angulation of the lower cervical cord and an operation revealed a low cervical meningocele and a split cord malformation with tethering of one half of the cord; the cord was untethered. In this report the literature is reviewed.

Female↗

[Complications of dysmorphophobia. Description of a self-mutilation case].

Dysmorphophobia, also known as Body Dysmorphic Disorder (BDD), can become a serious illness that results in severe complications such as social isolation, self-mutilations, suicide attempts, and even suicide. Many authors currently include BDD among the spectrum of obsessive-compulsive disorders. There are two distinguishable variants of BDD: psychotic and non-psychotic. The current trend considers these variants as one same disorder characterized by an insight spectrum. However, the psychotic variant exhibits more severe symptoms. We present a case of dysmorphophobia with psychotic symptoms that required psychiatric hospitalization due to serious complications. We discuss the presence of tactile and propioceptive sensations in some BDD patients and their contribution to their distress. Finally, we discuss a great propensity of BDD patients to conceal their symptoms. Thus, it is important for the clinician to specifically inquire about these symptoms, especially in high-risk groups, to prevent occurrence of serious complications.

Adult↗