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Oral Self-Mutilation in Lesch-Nyhan Patients: A Cross-Sectional Study.
J Clin Med
October 2022
Orthodontics Department, School of Dentistry, University of Genova, 16100 Largo Rosanna Benzi 10, 16132 Genoa, Italy.
Lesch-Nyhan syndrome (LNS) is a rare genetic condition resulting from an inherited disorder of purine metabolism. It is characterized by the lack of one enzyme, hypoxanthine-guanine phos-phoribosyltransferase (HGPRT), which is responsible for purine salvage. The main manifestations of this syndrome are hyperuricaemia, reduction in cognitive abilities, self-aggressive behavior, choreoathetosis, spasticity, and retarded development.
View Article and Find Full Text PDFOral Self-Mutilation in Lesch-Nyhan Syndrome: A Case Report.
Cureus
August 2022
Pediatric Stomatology Department, Hospital Dona Estefânia - Centro Hospitalar Universitário de Lisboa Central, Lisbon, PRT.
Lesch-Nyhan syndrome (LNS) is an inherited recessive X-related disorder caused by a deficiency of the purine salvage enzyme hypoxanthine-guanine phosphoribosyltransferase. It is characterized by dystonia and compulsive self-mutilation, in particular, biting behavior on the oral mucosa, tongue, lips, fingers, and shoulders, typically before one year of age. The majority of these patients require several procedures, including dental extractions, to prevent significant secondary lesions.
View Article and Find Full Text PDFA case of autophagia with thalamic hemorrhage.
Ann Med Surg (Lond)
July 2022
Department of Neurology, Somali Mogadişu Türkiye Recep Tayyip Erdoğan Research and Training Hospital, Somalia.
Autophagia occurs when one is compelled to inflict pain upon oneself by biting and/or devouring portions of one's body. It is sometimes associated with psychiatric disorders or with acquired nervous system lesions and could be life-threatening (The Journal of Nervous and Mental Disease, February 2012) [2]. It is the first time that this behavior was seen in post-stroke patients, not reported in the medical literature before.
View Article and Find Full Text PDFAcquired Constriction Band Syndrome Affecting Multiple Digits - A Case Report.
J Hand Surg Asian Pac Vol
June 2022
Department of Plastic & Reconstructive and Aesthetic Surgery, Meram Faculty of Medicine, Necmettin Erbakan University, Konya, Turkey.
Rubber band syndrome is a condition that usually affects children because of wearing a rubber band on the wrist or the ankle. Depending on the degree of pressure caused by the band, patients may present acutely with ischemia and necrosis of the tissues distal to the bands or chronically with change in shape, oedema, loss of function, sensation and rarely amputation. This condition is very rare in adults and most reports in literature are in patients with cognitive impairment or psychiatric illness.
View Article and Find Full Text PDFSelf-biting behavior in patients with neonatal brachial plexus palsy.
Childs Nerv Syst
September 2022
Peripheral Nerve Surgery Unit, Department of Neurosurgery, University of São Paulo Medical School, São Paulo, SP, Brazil.
Article Synopsis
- - The study examined the occurrence of self-biting behavior in patients with neonatal brachial plexus palsy (NBPP), finding it in 2.5% of a group of 612 patients.
- - Most cases of self-biting were linked to severe motor impairments, with the behavior generally appearing between 8 and 46 months of age, and lasting only a few months.
- - Effective treatment involved physical restriction, and no finger amputations occurred among the patients, indicating that prompt intervention can mitigate serious complications.
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