Use of wheelchair recliner in a patient with Hallervorden-Spatz Syndrome: A case report

Abstract

Background:  Hallervorden Spatz syndrome (HSS) is a rare neurodegenerative disease characterized by progressive extrapyramidal dysfunction and iron accumulation in the basal ganglia¹. It is autosomal recessive and affects approximately 1-3 people per million². It is characterized by movement disorders, particularly dystonia, dysarthria, gait disorders, and sudden onset of psychiatric symptoms^3,4. Orally, protrusive lingual dystonia and involuntary spasms of the masticatory, lingual, and pharyngeal muscles are observed². Our objective is to present the dental management of a patient with HSS in a wheelchair recliner. 

Clinical Presentation: Male patient, 29 years old, diagnosed at 19 years of age with HSS and swallowing disorder. Physical examination revealed dystonia, dysarthria, and gait disorders; Occasional wheelchair user. The intraoral exam showed a considerable accumulation of bacterial plaque, gingivitis, multiple caries, and teeth in residual root state.  Comprehensive dental treatment included manual scaling, multiple restorations, extractions of the residual root of teeth 1.5, 1.8, and 2.8 with deep caries and pulpitis. Dental care was performed in a wheelchair recliner, allowing better positioning and reducing the risks of dystonic movements that could interfere with dental treatment⁵. He was completely discharged, and a follow-up appointment was booked three months further in due to his high cariogenic risk.

Clinical Relevance: In the limited literature available regarding HSS, the need for the use of sedation to perform dental treatment is described on account of the challenge of cooperating due to anxiety and/or movement disorders, in addition to the limited mouth opening considering spasticity and stiffness muscular^6,7.

It is described that the mismanagement of secretions and psychomotor retardation makes it impossible to attend this kind of patient in the dental office in a conventional manner⁷, resulting in extraction as the procedure of choice¹. By installing the patient in a conventional dental chair, a precarious and distressing situation is created, as it is not possible to maintain a comfortable and stable position due to the lack of muscular control⁸. However, by having a recliner there is greater cooperation from the patient, allowing the exploration of less aggressive, lower-cost treatments with interdisciplinary management⁵.

Conclusion: The use of a wheelchair-recliner for the dental care of patients with movement disorders is a tool that considerably facilitates dental treatment⁵. The information obtained during the development of this case provides information that allows dentists to approach the management of patients with HSS.