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.

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.

Self-Mutilation of the Fingers After a Supracondylar Humerus Fracture: The Deleterious Effects of a Median Nerve Palsy: A Case Report.

CASE: A toddler underwent percutaneous pin fixation for a type III supracondylar humerus fracture (SHF). At home, her cast fell off and her k-wires pulled out. Subsequently, she presented to the emergency department for biting the tip of her index finger off and chewing on her thumb and middle fingers. She was diagnosed with a median nerve (MN) palsy with associated paresthesia. CONCLUSION: In very young children, it may be difficult to cope with the sensation of paresthesias after a MN palsy after a SHF. Self-mutilation of the fingers may be an unfortunate complication from these paresthesias.

Post-surgical evisceration with or without autocannibalism in 333 dogs-a survey of veterinarians.

Background: Few reports of post-surgical evisceration, with or without autocannibalism, in dogs exist. Aims: To collect a large case series of dogs experiencing post-surgical evisceration, with or without autocannibalism. Methods: We surveyed practicing veterinarians who were members of the Veterinary Information Network about their experiences with post-surgical evisceration in dogs, variably accompanied by autocannibalism (ingestion of eviscerated organs or tissues). Responses were analyzed using descriptive statistics. Results: We identified 333 cases with sufficient information for analysis: 221 with evisceration and autocannibalism, and 112 with just evisceration. Most cases occurred following surgery for reproductive reasons (desexing, cesarean section, and pyometra). Most occurred in young adult or adult dogs. Most dogs received analgesia perioperatively had routine closure (simple interrupted or simple continuous muscle layer closure) and most did not wear an E-collar post-surgically. Most dogs eviscerated within 3 days of the initial surgery. Approximately 64% underwent surgical repair and survived long-term without complications, more frequently if the evisceration was not accompanied by autocannibalism. Conclusion: Our study suggests that post-surgical evisceration and autocannibalism can generally be successfully managed by practitioners and do not confer a uniformly poor outcome for the dog.

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.

Is hypogonadism the cause of penile self-amputation in a patient with prior testes self-mutilation?

INTRODUCTION: This paper presents a rare event of genital self-mutilation and few cases are reported in the literature. CASE DESCRIPTION: We report on a patient who performed partial self-penectomy 18 months after bilateral testicular amputation, as a form of self-mutilation. CONCLUSIONS: The main hypotheses to explain the recurrence of self-mutilation would be hypogonadism.

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.

Overcoming the oral aspects of -self-mutilation: description of a method.

BACKGROUND/AIM: Self-injurious behavior (SIB) is a serious and chronic condition frequently seen in special needs populations, affecting 10% to 17% of individuals diagnosed with intellectual and/or developmental disabilities. A 2.5-year-old infant with SIB, whose presenting symptoms were severe tongue and lip lacerations accompanied by much hemorrhage, is presented here to illustrate the problem and to show how this may be prevented. MATERIALS AND METHODS: An appliance is described which effectively limits the damage caused by SIB and permits rapid healing of existing injuries. RESULTS: The method provides for a stable, retentive, and comfortable device on the infant's undererupted and largely nonretentive crowns of the deciduous teeth as well as for all permanent teeth in children, adolescents, and adults. CONCLUSIONS: The appliance has been successfully employed for the past 10 years in patients with SIB who have attended for treatment in the Special Needs Clinic in our Department.

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.

Cornelia de Lange and Ehlers-Danlos: comorbidity of two rare syndromes.

We present a case of a young adult with both Cornelia de Lange syndrome and Ehlers-Danlos syndrome. The patient showed non-verbal autism, intellectual disability and severe/intractable self-harming behaviours that led to a life-threatening complication (ie, septicaemia). A significant reduction in the self-harming behaviours was attained in a multidisciplinary neurobehavioural inpatient unit after addressing all causes of somatic pains, managing pain using level II and III analgesics, stabilising the patient's mood, limiting the iatrogenic effects of multiple prescriptions and offering a specific psychoeducational approach.

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.

Cannabis use and genital self-mutilation: an update of case reports.

We reported and compared two case reports of genital self-mutilation with concurrent increasing psychotic symptoms resulting from substance abuse such as cannabis and alcohol.