Manejo odontológico de paciente con Síndrome de Hallervorden-Spatz: relato de caso clínico: Síndrome de Hallervorden-Spatz :Relato de Caso

Gerana Araujo de Lucena Lira; Ivelyse  de S.Amaral Bernini; Isabela  Floriano; Suzana Cavalcante Monteiro de Oliveira; Tamara K.Tedesco; José Carlos  P.Imparato

doi:10.47990/alop.v12i1.299

Authors

Ms Gerana Lira Sao Leopoldo Mandic College
Ms Ivelyse Bernini Sao Leopoldo Mandic College https://orcid.org/0000-0002-6832-8497
Ms Isabela Floriano Sao Leopoldo Mandic College
Ms Suzana Oliveira Sao Leopoldo Mandic College
Ms Tamara Tedesco Sao Leopoldo Mandic College
Ms José Carlos Imparato Sao Leopoldo Mandic College

DOI:

https://doi.org/10.47990/alop.v12i1.299

Keywords:

Neurodegeneration Associated with Pantothenate Kinase, Endodontic treatment, Syndrome de Hallevorden -Spatz

Abstract

Hallervorden-Spatz syndrome is a rare, autosomal recessive neurodegenerative disorder characterized by large deposits of iron in the basal ganglia, which causes great motor and mental loss. It presents two forms of manifestations: the classic, which arises in childhood in the first decade of life; and the atypical, whose clinical manifestations appear later, between the second and third decades of life. The objective of this study was to describe a clinical case of endodontic outpatient treatment of a female patient, 28 years old, with classic manifestations of the syndrome, showing color change in element 11 with periapical lesion, justifying the indication for endodontics. The dental care of a rare neurodegenerative disease in an outpatient setting requires the dental surgeon's knowledge so that it is conducted efficiently and safely.

References

REFERENCIAS

Van Craenenbroeck A. et al. Hallervorden Spatz Disease: Historical Case Presentation in the Spotlight of Nosological Evolution. En: Movement Disorders. Bélgica, 2010; 25, n.15: 2486-2492.

Gregory AM, Hayflick SJ. Neurodegeneration with brain iron accumulation. En: Folia Neuropathologica. Estados Unidos. 2005; 43: 286-296.

Hanna AP, Gerhardstein LB. Hallervorden-Spatz Disease. En: Medscape Reference Drugs, Diseasers and Procedures. 2009.

Nord. Neurodegeneration with brain iron accumulation type 1. En: National

Organization for Rare Disorder. 2010. Disponible: www.raredisorders.org

Acceso en: 8 Mayo. 2011.

Sheehy ER et al. Self-inflicted Injury in a Case of Hallervorden-Spatz Disease, En: International Journal of Pediatric Dentistry. 1999; 9: 299-302.

López MEE et al. Reporte de Caso Clínico: Enfermedad de Hallervorden Spatz, intervención del equipo de salud oral. Acceso (2011 set 23). Disponible En:

Sharma MC et al. Hallervorden- Spatz disease:MR and pathological findings of a rare case. En: Neurology Indian. 2005; 53:1: 102-104.

Gregory AM, Hayflick SJ. Neurodegeneration with brain iron accumulation. En: Folia Neuropathologica. Estados Unidos. 2005: 43: 286-296.

Haddad AS, Mareti MBC. Anestesia geral no tratamento odontológico de pacientes com necessidades especiais. En: Haddad AS. Odontologia para pacientes com necessidades especiais. São Paulo: Livraria Santos; 2007; 501-14.

. Musselman RJ, Roy EK. Hospital management of the handicapped child. Dent Clin North Am. 1974; 18: 699-709.

Marega T et al. Alteraçãoes Psiquiátricas, Neurológicas e Comportamentais. Odontologia Especial. En:Odontologia Especial. São Paulo: Quintessence Editora, 2018: 214-227.