[Tear your eyes out ! A case of bilateral auto-enucleation]. / «Arrache-toi les yeux !¼ À propos d'un cas d'auto-énucléation bilatérale.

Bilateral autoenucleation is an extremely rare form of ocular self-mutilation. This gesture usually occurs in psychotic patients. In a moment of madness, a 28-year-old man brutally tore out both of his eyes. He was in acute relapse of schizophrenia after having interrupted all neuroleptic treatment for 6 months. Four days after admission, surgical enucleation was the only possible outcome. Facing the complexity of this clinical case, the ophthalmologist has a central role in the organization of long-term surgical, neurological and psychiatric care.

L'auto-énucléation bilatérale est une forme d'automutilation oculaire rarissime. Ce geste est observé presque toujours chez des patients psychotiques. Dans un moment de folie, un homme de 28 ans, s'est brutalement arraché les deux yeux. Il se trouvait en rechute aiguë de schizophrénie après avoir interrompu tout traitement neuroleptique depuis 6 mois. Quatre jours après son admission, l'énucléation chirurgicale fut la seule issue possible. Face à la complexité de ce cas clinique, l'ophtalmologue aura un rôle central dans l'organisation des soins chirurgicaux, neurologiques et psychiatriques au long cours.

Safety and Efficacy of Botulinum Toxin in the Treatment of Self-Biting Behavior in Lesch-Nyhan Disease.

BACKGROUND: Lesch-Nyhan disease (LND) is a disease of purine metabolism linked to chromosome X due to the absence or near-absence of enzyme hypoxanthine-guanine phosphoribosyltransferase. Patients with LND have a compulsive autoaggressive behavior that consists of self-mutilation by biting. METHODS: The objective of this study was to explore the safety and efficacy of botulinum toxin (BoNT) injected into the masticatory muscles and biceps brachii to reduce self-mutilation in patients with LND. We retrospectively analyzed six patients with LND who were treated with BoNT to prevent automutilatory behavior. RESULTS: The patient ages when started on treatment with BoNT were 4, 4.5, 6.6, 7.9, 13.9, and 32.3 years. Patients received a mean number of injections of 20, ranging from 3 to 29, over a period that ranged from 1.5 to 7.1 years. The maximum total dose of Botox was 21.3 units/kg mean and the maximum total dose of Dysport was 37.5 units/kg mean. A total of 119 injections were performed. Of these 113 (95%) were partially or completely effective. Only three of 119 injections (2.5%) produced adverse effects. CONCLUSIONS: Botulinum toxin is useful and safe for the treatment of self-biting behavior in patients with LND.

Reduction of self-mutilating behavior and improved oromotor function in a patient with Lesch-Nyhan syndrome following botulinum toxin injection: A case report.

Lesch-Nyhan syndrome is a genetic metabolic disorder often involving dystonia and self-mutilating behavior. This case report describes a 13-year-old boy with Lesch-Nyhan syndrome and self-mutilating behavior who received botulinum toxin injections to his bilateral masseter muscles after failing multiple other treatments. Following injections, the patient had reduction in self-biting, along with improvements in speech, mastication and feeding observed in speech therapy. Botulinum toxin injections to the masseters may help to improve oromotor function and reduce self-mutilating behaviors in children with Lesch-Nyhan syndrome who have failed more conservative treatments, providing opportunity for improved functional status and patient safety. Further investigation is indicated to establish optimal dosing. Additionally, the mechanism for the reduction of self-mutilating behavior is unclear and justifies additional investigation.

[Treatment of borderline personality disorder with opioid antagonists: buprenorphine, nalmefene, naloxone and naltrexone in the treatment of dissociative symptoms, self-mutilation and suicidal behavior]. / Tratamiento del Trastorno límite de la personalidad con antagonistas opioides: buprenorfina, nalmefeno, naloxona y naltrexona en el tratamiento de síntomas disociativos, automutilaciones y conducta suicida.

OBJECTIVE: Recent theory has proposed that a dysfunction of the opioid system modulates mood, reward and pain; seems to be unstable in people with Borderline Personality Disorder. Our purpose is to analyze the evidence on the efficacy of the use of buprenorphine, nalmefene, naloxone and naltrexone, in the treatment of dissociative symptoms, self-mutilation and suicidal behavior of these patients. METHOD: We conducted a systematic search of MEDLINE and LILACS databases, to retrieve relevant articles. Included studies were experimental and observational designs of borderline personality samples in which dissociative symptoms, self mutilation or suicidal behavior was reported as an outcome and evaluated with some impact measures. RESULTS: A total of eight studies were reviewed. These provided interesting expectations about posible treatment lines in Borderline Personality Disorder using opioid antagonists. The subgroup most benefited was the one who has analgesia and highest number of diagnostic criteria. CONCLUSIONS: Studies of higher methodological quality are needed, in larger population samples and using control of confounding variables that allow us to estimate a value power calculation, and thus be able to support firm conclusions.

An open-label pilot study of N-acetylcysteine for skin-picking in Prader-Willi syndrome.

Prader-Willi syndrome (PWS) is a complex neurodevelopmental disorder caused by an abnormality on the long arm of chromosome 15 (q11-q13) that results in a host of behavioral characteristics including excessive interest in food, skin picking, difficulty with a change in routine, and obsessive and compulsive behaviors. Skin-picking can result in serious and potentially life-threatening infections. Recent evidence suggests that the excitatory neurotransmitter glutamate is dysregulated in obsessive-compulsive behaviors, and modulation of the glutaminergic pathway may decrease compulsive behaviors, such as recurrent hair pulling or skin-picking behaviors. N-acetylcysteine (NAC), a derivative of the amino acid cysteine, is thought to act either via modulation of NMDA glutamate receptors or by increasing glutathione in pilot studies. Thirty-five individuals with confirmed PWS (ages 5-39 years, 23 females/12 males) and skin-picking behavior for more than 1 year were treated with N-acetylcysteine (Pharma-NAC®) at a dose of 450-1,200 mg/day. Skin-picking symptoms and open lesions were assessed after 12 weeks of treatment by counting and measuring lesions before and after the medication. All 35 individuals had improvement in skin-picking behaviors. Ten (29%) individuals (six males and four females) did not have complete resolution of skin-picking behavior, but had significant reduction in the number of active lesions. Longer-term, placebo-controlled trials are needed to further assess the potential benefit of this treatment.

Case presentation: long-term treatment.

The long-term (14 years) psychodynamic psychotherapy and pharmacotherapy of a depressed, suicidal, self-mutilating female patient is described. Her diagnoses included Chronic Posttraumatic Stress Disorder, Borderline Personality Disorder, and Recurrent Major Depression. Treatment was punctuated with repeated hospitalizations for self-mutilation (cutting) and suicidal ideation. A major determinant for her psychopathology was sexual abuse by her father from ages 6 to 14. This resulted in feelings of guilt and rage that she repressed and acted out through self-mutilating and suicidal behavior. A prolonged negative transference gradually became ambivalent, then positive. This was associated with her internalization of the healing qualities of the therapeutic relationship. She also gained insight into the reasons for her need to punish herself. Her initial self-representation as unworthy and bad was transformed into perceiving herself as a worthwhile, loving person. This case illustrates the role of long-term treatment for a complex, life-threatening, psychiatric disorder.

Serial stellate ganglion blocks for intractable postherpetic itching in a pediatric patient: a case report.

BACKGROUND: While intractable itching may be rarely associated with postherpetic neuralgia, it can have catastrophic complications if present. METHOD: We highlight a severe case of postherpetic itching in a 10-year-old male with Fanconi's and aplastic anemia, refractory to conventional treatments and requiring intravenous sedation. RESULTS: Our use of 3 sequential stellate ganglion blocks with 5.5 mL of 0.25% bupivacaine provided significant improvement of the symptoms for 4 months after the last procedure. CONCLUSION: Although further evaluation is needed, we feel that novel use of sympathetic blockade may provide treatment for intractable itching. Highlighted is the possible influence of the sympathetic system in the pathophysiology of postherpetic itch. IMPLICATION: The use of serial stellate ganglion blocks may be a treatment option for patients with intractable itching and postherpertic neuralgia of the neck and arm region. This technique may lead to more permanent solutions such as pulse radiofrequency lesion or chemical neurolysis of sympathetic ganglions for postherpetic itch.

Psychogenic excoriation responding to fluoxetine: a case report.

Compulsive skin picking, 'acne excoriee', neurotic (psychogenic) excoriation, dermatotillomania, occurring in 2% dermatology patients mostly in women, is a result of excessive scratching, picking, gouging or squeezing of the skin using teeth, tweezers, nail files, pins and knives, etc. The lesions are usually found on face and also on upper limbs and upper back, areas patients can easily reach. They may occur in absence or in response to skin pathology or sensation of itching. A young female patient attended OPD with the complaints of multiple excoriated lesions over the face, arms and forearms. The diagnosis was psychogenic excoriation which is an uncommon psychodermatological condition. She was treated with fluoxetine and behaviour therapy. The patient recovered fully with above treatment at the end of 3 months. Psychogenic excoriation is an uncommon psychodermatological condition which responds well to selective serotonin reuptake inhibitors and behaviour therapy (habit reversal training).

Botulinum toxin: treatment of self-mutilation in patients with Lesch-Nyhan syndrome.

Lesch-Nyhan syndrome (LNS) is a rare X-linked recessive disorder involving purine metabolism caused by the congenital absence of hypoxanthine guanine phosphoribosyl transferase. A characteristic feature of LNS is the appearance of intractable self-injurious behavior, usually in the form of severe lip and finger biting. The mechanism behind this severe self-mutilating behavior is unknown, and is one of the main challenges in the management of this condition. We here report the case of a 30-year-old man with a confirmed diagnosis of LNS who was successfully treated for self-mutilation of his lips with repeated botulinum toxin A (BTX-A) injections in the facial perioral muscles. Our findings suggest that treatment with BTX-A helped reduce self-abusive behavior in this patient. Our case illustrates that BTX-A injections can be a useful therapeutic approach in patients with self-abusive behavior.

Antipsychotics in the treatment of autism.

Atypical antipsychotics have become indispensable in the treatment of a variety of symptoms in autism. They are frequently used to treat irritability and associated behaviors including aggression and self injury. They may also be efficacious for hyperactivity and stereotyped behavior. This review presents the rationale for the use of this drug class in autism and reviews the most important studies published on this topic to date. Significant adverse effects, including weight gain and the possibility of tardive dyskinesia, are reviewed. Future research directions are discussed.

Genetic, developmental and personality correlates of self-mutilation in depressed patients.

OBJECTIVE: To examine whether the T allele of G protein beta3 (GNbeta3) is associated with self-mutilation in depressed patients. METHOD: A history of self-mutilation was systematically inquired about when recruiting depressed patients for a long-term treatment trial. Risk factors such as borderline personality disorder and childhood abuse experiences were systematically assessed, and patients were genotyped for polymorphisms of GNbeta3. RESULTS: The T allele of GNbeta3, borderline personality disorder and childhood sexual abuse were all significantly associated with self-mutilation in depressed patients. These associations were significant in both univariate and multivariate analyses, and as predicted were stronger in young depressed patients than in depressed patients of all ages. CONCLUSIONS: If the association between the T allele of GNbeta3 and self-mutilation can be replicated, this may provide clues to understanding the neurobiology of self-mutilation.

Botulinum toxin as a novel treatment for self-mutilation in Lesch-Nyhan syndrome.

Lesch-Nyhan syndrome (LNS) is an X-linked recessive disorder resulting from a deficiency of the metabolic enzyme hypozanthine-guanine phosphoribosyltransferase (HPRT). This syndrome presents with abnormal metabolic and neurological manifestations including hyperuricemia, mental retardation*, spastic cerebral palsy (CP), dystonia, and self-mutilation. The mechanism behind the severe self-mutilating behavior exhibited by patients with LNS is unknown and remains one of the greatest obstacles in providing care to these patients. This report describes a 10-year-old male child with confirmed LNS who was treated for self-mutilation of his hands, tongue, and lips with repeated botulinum toxin A (BTX-A) injections into the bilateral masseters. Our findings suggest that treatment with BTX-A affects both the central and peripheral nervous systems, resulting in reduced self-abusive behavior in this patient.

Spinal neurons involved in the generation of at-level pain following spinal injury in the rat.

Using a conjugate of substance P and the ribosome-inactivating protein saporin, neurons expressing the neurokinin-1 receptor in lamina I of the spinal cord were targeted to determine their role in the expression of a spontaneous pain behavior following intraspinal injections of quisqualic acid in the rat. Treatment was carried out at the time of injury in order to prevent the onset of the behavior, and following onset in order to evaluate the potential clinical utility of this intervention. Treatment at the time of injury resulted in significant decreases in onset-time and severity of pain behavior, while treatment at the time of onset led to a significant reduction of the spontaneous self-directed behavior. The results suggest that the substrate for at-level pain following spinal cord injury includes a population of spinal neurons expressing the neurokinin-1 receptor in the superficial laminae of the spinal cord.