Dental appliance therapy in pantothenate kinase‐associated neurodegeneration: Case report

Abstract

Pantothenate kinase-associated neurodegeneration (PKAN) is a rare condition associated with severe protrusive lingual dystonia, a form of oromandibular dystonia. Dental appliance therapy has been described for oromandibular dystonia however there is a lack of literature regarding its application specifically to PKAN. In this report, the authors describe the use of an appliance in conjunction with botulinum toxin injections for the symptomatic treatment of this condition. A satisfactory outcome is achieved which suggests this technique may be of use to other clinicians.

What should a dentist be aware of concerning symptoms of sleep disorders in the oral cavity?

“…However, additional symptoms may be seen in dystonia that are not usually associated with SB. These include dysphagia, dysarthria, breathing difficulties and disturbances in jaw movements [6,11,12,32,39]. Dystonia episodes can be observed as involuntary, repetitive, sustained (tonic) or spasmodic (rapid or clonic) muscle contractions [20].…”

“…Pathological muscle spasms can cause jaw closing, jaw opening, jaw deviation, facial grimacing, tongue movements, lip pursing, mouth-closing disturbance, tooth wear/loss, or damage to the oral soft tissues. Furthermore, it may also lead to dysphagia, dysarthria, difficulties in breathing, weight loss secondary to mechanical problems with food intake, temporomandibular disorders, pain, and impaired sleep quality, or a combination of these symptoms [11,12,32,39]. Dystonia can also cause depression and anxiety [6].…”