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.

Death by self-mutilation after oral cannabis consumption.

Major self-mutilation (amputation, castration, self-inflicted eye injuries) is frequently associated with psychiatric disorders and/or substance abuse. A 35-year-old man presented with behavioral disturbances of sudden onset after oral cannabis consumption and major self-mutilation (attempted amputation of the right arm, self-enucleation of both eyes and impalement) which resulted in death. During the enquiry, four fragments of a substance resembling cannabis resin were seized at the victim's home. Autopsy confirmed that death was related to hemorrhage following the mutilations. Toxicological findings showed cannabinoids in femoral blood (tetrahydrocannabinol (THC) 13.5 ng/mL, 11-hydroxy-tetrahydrocannabinol (11-OH-THC) 4.1 ng/mL, 11-nor-9-carboxy-THC (THC-COOH) 14.7 ng/mL, cannabidiol (CBD) 1.3 ng/mL, cannabinol (CBN) 0.7 ng/mL). Cannabinoid concentrations in hair (1.5 cm brown hair strand/1 segment) were consistent with concentrations measured in chronic users (THC 137 pg/mg, 11-OH-THC 1 pg/mg, CBD 9 pg/mg, CBN 94 pg/mg). Analysis of the fragments seized confirmed that this was cannabis resin with high levels of THC (31-35%). We discuss the implications of oral consumption of cannabis with a very high THC content.

A missense mutation in the CRBN gene that segregates with intellectual disability and self-mutilating behaviour in a consanguineous Saudi family.

BACKGROUND: Autosomal-recessive non-syndromic intellectual disability (ARNS-ID) is an aetiologically heterogeneous disorder. Although little is known about the function of human cereblon (CRBN), its relationship to mild cognitive deficits suggests that it is involved in the basic processes of human memory and learning. OBJECTIVES: We aim to identify the genetic cause of intellectual disability and self-mutilation in a consanguineous Saudi family with five affected members. METHODS: Clinical whole-exome sequencing was performed on the proband patient, and Sanger sequencing was done to validate and confirm segregation in other family members. RESULTS: A missense variant (c. 1171T>C) in the CRBN gene was identified in five individuals with severe intellectual disability (ID) in a consanguineous Saudi family. The homozygous variant was co-segregating in the family with the phenotype of severe ID, seizures and self-mutilating behaviour. The missense mutation (p.C391R) reported here results in the replacement of a conserved cysteine residue by an arginine in the CULT (cereblon domain of unknown activity, binding cellular ligands and thalidomide) domain of CRBN, which contains a zinc-binding site. CONCLUSIONS: These findings thus contribute to a growing list of ID disorders caused by CRBN mutations, broaden the spectrum of phenotypes attributable to ARNS-ID and provide new insight into genotype-phenotype correlations between CRBN mutations and the aetiology of ARNS-ID.

Regeneration patterns influence hindlimb automutilation after sciatic nerve repair using stem cells in rats.

INTRODUCTION: Hindlimb autophagy is common after rat sciatic total axotomy and is considered as a sign of neuropathic pain. We applied adult stem cells in a fibrin conduit in a total sciatic axotomy model to improve nerve regeneration, investigating whether a correlation could be detected between stem cells effects on regeneration and limb autophagy. MATERIAL AND METHODS: After sciatic nerve section, a 1-cm sciatic gap was crossed using fibrin conduits. Experimental groups included empty fibrin conduits, fibrin conduits seeded with primary Schwann cells, and fibrin conduits seeded with Schwann cell-like differentiated mesenchymal or adipose-derived stem cells (dMSCs and dASCs). Controls were represented by autografts and by sham rats (tot n=34). At 16 weeks post-implantation, regeneration pattern was analysed on histological sections and related to eventual autophagy. Hindlimbs were evaluated and scored according to autophagy Wall's scale and X-Rays radiological evaluation. RESULTS: All regenerative cell lines significantly improved myelination at the mid conduit level, compared to the empty tubes. However, dMSC could not significantly improve myelination at the distal stump, showing a more chaotic regeneration compared to both other cells groups and controls. Autophagy was correlated to this regeneration patterns, with higher autophagy scores in the empty and dMSC group. CONCLUSION: Hindlimb autophagy can be used as index of neuropathic pain due to nerve lesion or on-going immature regeneration. dMSC group was characterized by a less targeted regeneration comparing to dASC and primary Schwann cells, which confirmed their effectiveness in regeneration and potential in future clinical applications.

Foreign bodies in the sigmoid colon of a psychiatric patient following self-mutilation: a case report.

BACKGROUND: The act of deliberate injury to one's own body without the help of others is a well-known phenomenon in psychiatric patients. Insertion of foreign bodies into one or more orifices is not uncommon but insertion into a body cavity or the gastrointestinal tract by self-inflicted injury is quite rare. CASE PRESENTATION: A 32-year-old Ethiopian psychiatric patient presented with left lower abdominal pain of three months' duration following the insertion of foreign bodies via a self-inflicted wound in the left lower quadrant of his abdomen. Radiological evaluation demonstrated the presence of foreign bodies. A laparotomy revealed two metallic and three wooden materials in his sigmoid colon and a hole in his sigmoid that was tightly sealed with omentum. The foreign bodies were successfully removed, the hole was closed primarily, and our patient was discharged uneventfully. CONCLUSIONS: This case illustrates that a foreign body can be inserted into the colon through a self-inflicted wound in psychiatric patients, and patients may present months later without having developed generalized peritonitis.

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.

Successful treatment of self-inflicted tongue trauma patient using a special oral appliance.

A 7-year-old male presented with a painful ulcerative lesion on the right lateral tongue and left lower buccal mucosa due to self-inflicted trauma. Antibiotic medication and use of a mouthwash agent were not effective. We made a special oral appliance to cover the maxillary arch and teeth to protect the tongue. The patient showed immediate improvement and did not suffer from any complications. Invasive procedures such as biopsy were not needed. We believe that accurate clinical diagnosis is important and treatment with an oral appliance is effective in self-inflicted oral trauma in children.

Non-nutritive sucking associated with self-mutilating behavior / Hábito de sucção não-nutritiva associada à automutilação

Although sucking activity has been considered an essential behavior of early childhood to satisfy nutritive and non-nutritive needs, digit andpacifier sucking are deleterious oral habits that may interfere with child development. Furthermore, this clinical picture may be compounded by other concerning habits, such as self-mutilating behavior. This article reports 4-year follow-up of a child in whom non-nutritive sucking was associated with an unusual self-mutilating behavior; namely, the child would pull out her own hair after wrapping it around her finger every time she sucked on the pacifier. This occurred specially at bedtime, while she was watching TV, or when she was somewhat anxious, and remitted and recurred throughout the follow-up period. In an attempt to address this behavior, pacifier use was discontinued and the child?s head was shaved. Ultimately, the case was only solved through combined efforts involving the child, her family, and health professionals. Based on the parents? reports and clinical examination and follow-up findings, we emphasize the importance of investigating the origin of the problem and considering emotional aspects and its association with other habits in such cases.

Apesar de a atividade de sucção ser considerada um comportamento essencial da primeira infância por satisfazer as necessidades nutritivase não-nutritivas, a sucção do dedo e chupeta são hábitos deletérios ao desenvolvimento da criança. Além disso, esse quadro clínico podeser ainda mais preocupante se houver associação com outros hábitos, como o da automutilação. Neste contexto, no presente artigo sãorelatados 4 anos de acompanhamento de uma criança que costumava arrancar seus cabelos depois de enrolá-los nos dedos enquanto estavacom a chupeta na boca, sem demonstrar sinal algum de sintomatologia dolorosa. Tal fato ocorria especialmente próximo a períodos de sono, ansiedade ou quando assistia à televisão, havendo períodos de remissão e exacerbação. Na tentativa de solucionar o caso foi proposta, além da interrupção do uso da chupeta, a raspagem de todo o cabelo da criança. Contudo, a solução definitiva só foi possível quando houve o envolvimento coletivo, abrangendo criança, família e profissionais da saúde. Baseando-se no relato dos pais, no exame clínico e no acompanhamento do caso, enfatiza-se a importância de se investigar a origem do problema, considerando os aspectos emocionais e sua associação com outros hábitos.

[Delusional disorder, autoaggression und suicide]. / Wahnerkrankung, Autoaggression und Suizid.

The authors report on the suicide of a 27-year-old woman with extreme self-inflicted injuries. The victim suffered from schizophrenic psychosis with several stays in mental institutions and one previous suicide attempt. Autopsy revealed multiple cut and stab injuries in various body regions (51 stabs to the chest, cutting off parts of ears and nose, stab to the eye and transection of the scalp). Death was caused by exsanguination.

Trigeminal trophic syndrome: report of 3 cases affecting the scalp.

Trigeminal trophic syndrome (TTS) is a rare condition that results from a prior injury to the sensory distribution of the trigeminal nerve. Patients typically respond to the altered sensation with self-mutilation, most often of the nasal ala. We describe 3 patients with TTS who presented with self-induced ulcerations primarily involving the scalp. Two patients developed delusions of parasitosis (DOP) based on the resulting symptoms of TTS, which is a unique association. Trigeminal trophic syndrome may occur at extranasal sites and in any branch of the trigeminal nerve. The condition should be considered when ulcers are encountered in this nerve distribution. Symptoms such as formication may mimic DOP. Trigeminal trophic syndrome may be differentiated from DOP by the restriction of symptoms and ulcerations to the distribution of the trigeminal nerve.

[Autoerotic fatalities in Greater Dusseldorf]. / Autoerotische Todesfälle im Grossraum Düsseldorf.

Autoerotic fatalities in the Greater Dusseldorf area correspond to the relevant medicolegal literature. Our results included exclusively young to middle-aged, usually single men who were found dead in their city apartments. Clothing and devices used showed a great variety. Women's or fetish clothing and complex shackling or hanging devices were disproportionately frequent. In most cases, death occurred due to hanging or ligature strangulation. There was no increased incidence of underlying psychiatric disorders. In most of the deceased no or at least no remarkable alcohol intoxication was found. Occasionally, it may be difficult to reliably differentiate autoerotic accidents, accidents occurring in connection with practices of bondage & discipline, dominance & submission (BDSM) from natural death, suicide or homicide.

Self-mutilation of tongue and lip in a patient with simple schizophrenia.

Self-inflicted lesions that cause mutilation are observed in schizophrenic patients. This case report describes the diagnosis and treatment of simple schizophrenia in a 31-year-old male patient who bit his own tongue and lower lip. The dental treatment proposed included the construction of a splint to prevent new lesions and to allow healing of existing ones. The treatment afforded to avoid tooth extraction before the patient responded to psychiatric drug treatment. The importance of a transdisciplinary approach to self-mutilation due to psychiatric disorders is stressed. The interaction between the two teams, stomatology and psychiatry, was crucial for the improvement of the patient's condition.

Medicolegal aspects of 3 cases of bizarre self-mutilation.

Three cases of bizarre self-mutilation are reported because of the unusual methods used and the unexpected good sequel in one of them.

Modern induced skull deformity in adults.

The practice of induced skull deformity has long existed in numerous disparate cultures, but for the first time in history it can be applied to adults. While extremely limited in application, some ideas have persisted in the far fringes of modern Western culture with remarkable tenacity. Practitioners of extreme body modification undergo procedures, outside the sphere of traditional medical practice, to make striking, permanent, nontraditional esthetic tissue distortions with the goal of transgressing societal norms. The International Trepanation Advocacy Group represents another example of a fringe cultural movement, whose goal, rather than being purely aesthetic in nature, is to promote elective trepanation as a method for achieving a heightened level of consciousness. Both movements have relatively short and well-defined histories. Despite their tiny numbers of adherents, neurosurgeons may be called on to address relevant patient concerns preprocedurally, or complications postprocedurally, and would benefit from awareness of these peculiar subcultures.

Secondary Ekbom syndrome to organic disorder: report of three cases.

The Ekbom syndrome, also known as delusion of parasitosis or acarophobia is an obsessive phobic state in which the patient thinks, imagines or believes that his or her skin is infested by parasites. In the hallucinatory state, he/she removes parts of the skin, identifying them as parasites. It can be primary or secondary to other organic or psychiatric diseases. Generally speaking these patients take a long time to seek for medical support and the dermatologist is almost always the first physician to see them. Here we describe three patients with delusional parasitosis associated with organic disorders.

Síndrome de Ekbom secundária a transtorno orgânico: relato de três casos / Secondary Ekbom Syndrome to organic disorder: report of three cases

A Síndrome de Ekbom, também conhecida como delírio de parasitose ou acarofobia, é um estado fóbico obsessivo no qual o paciente pensa, imagina ou acredita que está infestado por parasitas na pele. Em estado alucinatório, retira fragmentos de pele, identificando-os como parasitas. Pode tratar-se de um quadro psiquiátrico primário ou secundário a outros transtornos orgânicos. Geralmente, esses pacientes demoram a procurar ajuda médica, e o dermatologista, quase sempre, é o primeiro profissional procurado. Descrevemos o caso de três pacientes dos quais apresentaram delírio de parasitose, associados a transtornos orgânicos.

The Ekbom syndrome, also known as delusion of parasitosis or acarophobia is an obsessive phobic state in which the patient thinks, imagines or believes that his or her skin is infested by parasites. In the hallucinatory state, he/she removes parts of the skin, identifying them as parasites. It can be primary or secondary to other organic or psychiatric diseases. Generally speaking these patients take a long time to seek for medical support and the dermatologist is almost always the first physician to see them. Here we describe three patients with delusional parasitosis associated with organic disorders.