Cognitive level, quality of life and oral health of a Prader-Willi Syndrome patient - case report with long-term follow-up / Nível cognitivo, qualidade de vida e saúde bucal de um paciente com síndrome de Prader-Willi - Relato de caso com acompanhamento de longo prazo

Abstract

Introduction: Prader-Willi syndrome (PWS) is a rare, neurobehavioral genetic disorder that alters the child's development. Concerning oral health, PWS patients present dental aspects that are not completely understood. Objectives: The aim of this case report was to describe long-term follow-up of a patient with PWS, regarding to cognitive level, overall self-perceived quality of life and oral health, sleep quality, anxiety trait, and oral condition. Methods: The information about his cognitive ability and socio-behavioral behavior was collected applying the questionnaires: Mini-Mental State Examination (MMSE), simplified World Health Organization Abbreviated Quality of Life survey (WHOQOL-bref), Sleep Assessment Questionnaire (SAQ), State Trait Anxiety Inventory (STAI-T) Oral Health Impact Profile (OHIP-14). The clinical examination was performed according to the WHO methods for oral health surveys. Index of Decayed, Missing or Filled teeth (DMFT) and other changes in mineralized tissues, such as dental fluorosis or erosion, incisor molar hypomineralization, enamel hypoplasia, bruxism, dental trauma and positioning of teeth in dental arches were also recorded. In the periodontal analysis, the visible plaque index, bleeding on probing and final diagnosis of gingivitis were performed. Data about dental occlusion and temporomandibular disorders were also noted. After data collection and clinical examination, a saliva sample was collected to assess salivary flow rate, pH and buffering capacity. Results: The analysis of the questionnaires revealed an individual satisfied with his quality of life, but very anxious. Clinical examination point to gingival inflammation located in the region of anterior teeth, maxillary and mandibular anterior tooth crowding and malocclusion. Salivary parameters were normal. Conclusion: This case report suggests that the PWS patient perceives that his quality of life is good despite his anxiety episodes. Regarding oral health, gingival inflammation and malocclusion are the main problems.