Oral self-inflicted accidental trauma in patients with neurological disorders: a case report of dental management in infants with cerebellar hypoplasia.

Self-inflicted oral injuries, accidental or otherwise, can cause major consequences. Measures need to be taken to protect individuals from chronic self-injurious behaviour; however, there are no official guidelines on the subject. The purpose of this article is to show the case of a 1-year-old patient with neurological disorders who, following the eruption of deciduous teeth, had self-inflicted a traumatic ulcer on his tongue and lower lip. Following a multidisciplinary approach involving several operating units of our hospital to make a diagnosis, an oral device was designed to completely cover the dental elements to prevent recurrence of the trauma and to prevent further worsening of the injuries already caused. The purpose of this work is to demonstrate that although the surgical approach, such as extraction of the dental elements, may be the quickest solution in situations similar to the one presented, the high biological cost and irreversibility of the result lead to seeking alternatives and more conservative solutions such as the one described.

Macrophage Activation in the Dorsal Root Ganglion in Rats Developing Autotomy after Peripheral Nerve Injury.

Autotomy, self-mutilation of a denervated limb, is common in animals after peripheral nerve injury (PNI) and is a reliable proxy for neuropathic pain in humans. Understanding the occurrence and treatment of autotomy remains challenging. The objective of this study was to investigate the occurrence of autotomy in nude and Wistar rats and evaluate the differences in macrophage activation and fiber sensitization contributing to the understanding of autotomy behavior. Autotomy in nude and Wistar rats was observed and evaluated 6 and 12 weeks after sciatic nerve repair surgery. The numbers of macrophages and the types of neurons in the dorsal root ganglion (DRG) between the two groups were compared by immunofluorescence studies. Immunostaining of T cells in the DRG was also assessed. Nude rats engaged in autotomy with less frequency than Wistar rats. Autotomy symptoms were also relatively less severe in nude rats. Immunofluorescence studies revealed increased macrophage accumulation and activation in the DRG of Wistar rats. The percentage of NF200+ neurons was higher at 6 and 12 weeks in Wistar rats compared to nude rats, but the percentage of CGRP+ neurons did not differ between two groups. Additionally, macrophages were concentrated around NF200-labeled A fibers. At 6 and 12 weeks following PNI, CD4+ T cells were not found in the DRG of the two groups. The accumulation and activation of macrophages in the DRG may account for the increased frequency and severity of autotomy in Wistar rats. Our results also suggest that A fiber neurons in the DRG play an important role in autotomy.

Self-insertion of foreign bodies into the orbit and periocular tissue.

We describe six patients with 12 separate episodes of self-inflicted periocular foreign body injuries, which presented to our institution recently. All patients were male, relatively young (mean 28.5 years old), incarcerated, and had significant underlying psychiatric conditions. The subjects had inserted staples (6), paperclips (2), or other small metallic wire segments (4) into the periocular region. Most cases (9/12) involved concurrent self-inflicted injury to other body parts. Ten cases involved foreign bodies inserted through the palpebral conjunctiva into the upper eyelid, while two cases involved insertion into the orbit. Identification and surgical retrieval of foreign bodies was successful in most cases (9/11) but was not attempted in one case. Self-inflicted periocular injuries, while rare, are challenging cases for which the ophthalmologist should be prepared. A multidisciplinary approach, including psychiatric assessment and treatment, is important for optimal care.

Midface toddler excoriation syndrome (MiTES) can be caused by autosomal recessive biallelic mutations in a gene for congenital insensitivity to pain, PRDM12.

BACKGROUND: Midface toddler excoriation syndrome (MiTES) is a condition recently reported in three unrelated children. Habitual scratching from the first year of life inflicted deep, chronic, scarring wounds around the nose and eyes. One child had a mild neurological deficit but there was no other evidence of insensitivity to pain. Bilateral distribution and localization to the midface distinguish MiTES from other causes of self-inflicted skin damage such as trigeminal trophic syndrome. An earlier study of five siblings from a consanguineous Irish family, with lesions corresponding to MiTES plus other sensory deficits, showed homozygous mutations in a gene for hereditary sensory and autonomic neuropathy type VIII (HSAN8), PRDM12. OBJECTIVES: To study further cases of MiTES, including analysis of PRDM12. METHODS: We describe five further children, from four families, with facial lesions typical of MiTES, in whom mutation analysis of PRDM12 was carried out. RESULTS: Homozygous or compound heterozygous pathogenic expansions of the PRDM12 polyalanine tract were found in four of five affected individuals, in three families. CONCLUSIONS: Our finding of autosomal recessive mutations in PRDM12 in four of five patients with MiTES extends the phenotypic spectrum of PRDM12 mutations, which usually cause HSAN8, characterized by mutilating self-inflicted wounds of the extremities, lips and tongue. By contrast, MiTES shows severe midfacial lesions with little if any evidence of generalized pain insensitivity. The condition is probably genetically heterogeneous, and other congenital insensitivity to pain and HSAN genes such as SCN11A may be implicated. This new understanding of the nature of MiTES, which can masquerade as factitious disease, will facilitate appropriate management.

Joubert Plus Syndrome with Self-Mutilation: A Case report.

BACKGROUND: Joubert syndrome is a very rare condition with dismal prognosis. It is characterized by several abnormalities including molar tooth sign on MRI. When coupled with mega cisterna magna- a feature of the Dandy Walker syndrome- it is categorized as Joubert plus syndrome. CASE REPORT: A 16 month old male child with Joubert syndrome was referred to the Pediatric Dentistry Department Clinic, Faculty of Dentistry Alexandria University, complaining of severe tongue and lower lip injury due to self-mutilation. He required multiple teeth extractions under general anesthesia to prevent further tongue and lip mutilation. CONCLUSION: Joubert plus syndrome is a very rare occurring condition. Because self-mutilation is sometimes fatal, a treatment plan tailored to each patient's need is mandatory. A multidisciplinary approach is recommended.

Potential relationship of self-injurious behavior to right temporo-parietal lesions.

Self-injurious behavior (SIB) is associated with several neurologic and psychiatric syndromes but rarely with focal lesions. Two patients with lesions of the right temporo-parietal junction presented to psychiatric inpatient services with SIB in the absence of notable neurologic deficits or suicidal ideation. Right temporo-parietal lesions may be associated with disturbances of agency and body ownership, both of which may facilitate SIB. Misoplegia, or hatred of a limb, may be associated with SIB and has been reported without hemiplegia with a right temporo-parietal lesion. Further study is warranted to improve our understanding of the mechanisms underlying SIB.

Hereditary Sensory Autonomic Neuropathy II, a rare disease in a large Pakistani family.

Hereditary Sensory Autonomic Neuropathy II (HSAN II) is a rare genetic disorder, characterized by severe loss of pain, temperature and touch sensation. Injuries in these patients can progress to necrosis and shedding of digits and limbs. Here we report two cases of HSAN II belonging to a Pakistani family. Individual 1, a forty five year old man, had complete loss of pain sensation since birth. Self-mutilation and complication of injuries resulted in the shedding of all the digits and right foot and surgical amputation of left leg. Individual 2, a five year old girl,had delay in healing of wounds and self-mutilation. Examination showed a complete lack of pain sensation throughout her body and hyporeflexia. As the genetic cause of HSAN II is unknown, identification of more patients will allow further research on this disease and possibly develop a cure.

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.

Self-mutilation of the tongue in a patient with schizophrenia.

Attempts at tongue replantation are rare, possibly because of the friable nature of the tongue vasculature. We describe the successful replantation of the tongue in a patient with schizophrenia who attempted self-mutilation. Anastomosis of the right deep lingual artery and vein was performed under microscopy. To ensure that the patient did not make another mutilation attempt, he was kept under sedation in the intensive care unit, and a Denhardt mouth gag was placed for 5 days. Currently, his pronunciation is close to normal, and he has recovered some degree of somatic and gustatory sensation. It may be argued that the amputated tongue should be discarded; however, successful replantation can be achieved with high satisfaction for the patient and family, especially in a psychiatric patient.

[Self mutilation in borderline personality disorder. Pain as a self-treatment? A case report]. / Automutilación en Trastorno Borderline de Personalidad. ¿El dolor como auto tratamiento? Un caso clínico.

The objective of this article is to highlight the complexity of the phenomenon of self-mutilation, or self-injury, among individuals with Borderline Personality Disorder. The practice of self-mutilation in this population is strikingly common, with some authors estimating the prevalence to be up to 75%. Particularly noteworthy is the high percentage of patients with Borderline Personality Disorder who state that the act of self-injury has an analgesic effect. This article lays out a few theories that support this finding. Frequently, patients who self-mutilate do not aim to commit suicide but rather, to relieve internal tension. However, clinical interventions are often largely centered on interpreting the self-injurious act as a suicide attempt, with complex therapeutic consequences. A clinical vignette is presented to highlight the aforementioned issues.

Self-mutilative behaviors in male substance-dependent inpatients and relationship with anger and aggression: mediator effect of childhood trauma.

The aim of this study was to investigate the relationship of self-mutilation (SM) with anger and aggression in male substance-dependent inpatients. Also, we wanted to evaluate the mediator effect of childhood trauma on these relationships while controlling variables such as age, substance of dependence (alcohol/drug), and negative effect. Participants were consecutively admitted 200 male substance-dependent inpatients. Patients were investigated with the Self-mutilative Behaviour Questionnaire, the Childhood Trauma Reports, the Buss-Perry's Aggression Questionnaire, the State-Trait Anger Expression Inventory, the Beck Depression Inventory, and the State-Trait Anxiety Inventory. Rate of being married, current age, and age onset of regular substance use were lower, whereas being unemployed and history of childhood trauma (HCT) were higher in group with SM (n = 124, or 62.0%). Higher mean scale scores were found in SM group. Predictors of SM were being younger, impaired anger control, and physical aggression in logistic regression model. Being younger and the outward expression of anger (anger-out) predicted SM in the subgroup of patients without HCT, whereas being younger, severity of anger, and the inward expression of anger (anger-in) predicted SM in the subgroup of patients with HCT. Thus, to reduce self-mutilative behavior among substance-dependent patients, clinicians must improve anger control, particularly in younger patients. Type of strategy for coping with anger, which must be worked on, may differ in different subgroup patients, that is, focusing anger toward self among those with HCT, whereas anger toward others among those without.

Bilateral corneal perforations and autoproptosis as self-induced manifestations of ocular Munchausen's syndrome.

PURPOSE: To report a patient with bilateral corneal perforations and autoproptosis in a case of ocular Munchausen's syndrome. DESIGN: Case report. PARTICIPANT: A 26-year-old white male referred to the oculoplastics service with one month history of decreased vision bilaterally and painful right eye. Multiple eyelid scars and right corneal opacity were noted. The patient was previously seen at another institution for rapid loss of vision in both eyes. INTERVENTIONS: An orbit decompression among many procedures failed to controlled extreme pain and proptosis. MAIN OUTCOME MEASURES: Resolution of proptosis, stabilization of vision, pain resolution. RESULTS: Three weeks after enucleation of the right eye was offered, patient presented with spontaneous left ruptured globe. After multiple episodes of self-mutilation and infections, both eyes were exenterated. CONCLUSIONS: Munchausen syndrome can be seen with ophthalmic manifestations and should be considered in the differential diagnosis when ocular abnormalities cannot be explained after a thorough evaluation. Recognition of this psychiatric disease is not only important for correct medical diagnosis and treatment, but also essential in protecting the patients from unnecessary invasive and aggressive medical procedures.

Self-mutilative behaviours in male alcohol-dependent inpatients and relationship with posttraumatic stress disorder.

The aim of this study was to investigate the relationship between self-mutilation (SM) and posttraumatic stress disorder (PTSD) in male alcohol-dependent inpatients, and to examine whether there is something unique about self-mutilaters with the PTSD/alcohol-dependence co-morbidity, compared with self-mutilaters without PTSD in this population. Participants were 156 consecutively admitted male alcohol-dependent inpatients. Patients were investigated with the Self-mutilative Behaviour Questionnaire (SMBQ), the Traumatic Experiences Checklist (TEC), the Clinician Administered PTSD Scale (CAPS), the Symptom Checklist-Revised (SCL-90-R) and the Michigan Alcoholism Screening Test (MAST). Among alcohol-dependent inpatients, 34.0% (n=53) were considered as group with SM. Rate of being unemployed, history of any trauma, history of suicide attempt and lifetime PTSD diagnosis were higher, whereas being married, current age, age at onset of regular alcohol use and duration of education were lower in the group with SM. Mean scores of SCL-90 subscales, TEC and MAST were higher in the SM group. Although SM might be related with PTSD among male alcohol-dependent inpatients, predictors of SM were age at onset of regular alcohol use, history of suicide attempt, anxiety, depression and hostility. Age at onset of regular alcohol use, history of suicide attempt, anxiety, depression and somatisation predicted SM in the subgroup of patients without PTSD, whereas hostility predicted SM alone in the subgroup of patients with PTSD. Results support the anti-suicide and the affect-regulation models of SM in the non-PTSD group, whereas they support the hostility model of SM in the subgroup with PTSD in alcohol-dependent inpatients. Thus, to reduce self-mutilative behaviour (SMB)among alcohol-dependent patients, clinicians must address different subjects in different subgroup patients; that is, focussing hostility in those with PTSD co-morbidity.

Self-mutilation in patients after nerve injury may not be due to deafferentation pain: a case report.

OBJECTIVE: Animals with transected nerves may develop self-mutilating behavior (autotomy) directed at the denervated body part. Autotomy is often thought to be a response to deafferentation pain produced by pathological changes in the dorsal horn, and self-mutilation after dorsal rhizotomy has consequently been used as an outcome measure for the investigation of chronic pain in animal models. A less recognized hypothesis suggests that autotomy is simply an animal's efforts to remove the useless part. We report a case of self-mutilation of the thumb and fingers in a patient with loss of all sensory modalities in the arm after brachial plexus avulsion. CONCLUSION: Asking the patient about the reasons for his self-mutilation provides insights into the cause of autotomy which cannot be established from animal studies. We suggest that autotomy may not be a result of chronic pain, and discuss the human experience and alternative underlying pathological processes.

A case of autoenucleation associated with a contralateral field defect.

PURPOSE: To report a case of autoenucleation associated with contralateral field defect. DESIGN: Observational case report. METHODS: A 36-year-old man was referred to the emergency ward with his right eye attached to a fork. His history revealed drug abuse with ecstasy. RESULTS: Visual field examination revealed a temporal hemianopia on the left eye. There was no change in the visual field defect after intravenous steroid, two months after initial presentation. CONCLUSIONS: Contralateral visual field defect may be associated with autoenucleation. A visual field test is recommended in all cases with traumatic enucleation.

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 a self-inflicted intracranial nail gun injury.

A 30-year-old man walked into the emergency department after a suicide attempt by firing a nail from a pneumatic nail gun directed at his left temple. He was haemodynamically stable and neurologically intact, able to recall all events and moving all extremities with a Glascow Coma Scale of 15. CT of the brain showed a 6.3 cm nail in the right frontal region without major intracerebral vessel disruption. He was taken to the operating room for left temporal wound washout, debridement of gross contamination and closure with titanium cranial fixation plate. The foreign body was not accessible on initial surgical intervention and was left in place to define anatomy and plan for subsequent removal. Thin slice CT images were used to create 3D reconstructions to facilitate stereotactic navigation and foreign body removal via right craniotomy the following day. The patient tolerated the procedures well and recovered with full neurological function.