Jalili syndrome (JS) is an autosomal recessive disorder characterized by cone-rod dystrophy (CRD) and amelogenesis imperfecta (AI), and caused by variants in CNNM4 encoding a magnesium transporter. [1][2][3] We identified a non-consanguineous multigenerational family of the Lua ethnic group in Northern Thailand with three members affected with JS.Proband (IV:2), an 11-year-old girl, had hypoplastic-hypocalcified AI, crossbite, 4-year advanced dental age, bilateral nystagmus, and retinal hypo/hyperpigmentary change, indicating bilateral macular dystrophy with hyperopia (Figure 1A). Her learning ability had reportedly gradually declined from age 7. Her IQ at age 9 was 66 (extremely low).IV:3, a 9-year-old girl, had hypoplastic-hypocalcified AI, crossbite, taurodont molars, 2-3-year advanced dental age, bilateral hyperopia, no ocular structure abnormalities, macular hypo/hyperpigmentation, loss of foveal light reflex (Figure 1B). Her IQ at age 7 was 75 (borderline). IV:3 and IV:2 exhibited developmental delay. Recent spectral domain-optical
Aims: To describe the procedures for complete denture fabrication in dementia and behavioral impairment patient and how to manage the case holistically. Methods and Results: The patient was a 77-year-old woman diagnosed with moderate dementia, Alzheimer’s disease with Behavioral and Psychological Symptoms of Dementia. The patient lost her previous complete dentures. She had signs of agnosia, communication problems and behavioral control. These aspects increased the difficulty in her daily life activities and in providing dental treatment. Our goal was to restore her oral function and to improve her overall general health by fabricating new complete dentures. An online consultation was used to oversee the behavioral management, adaptation in posture, and techniques in prosthodontic procedures between the postgraduate dental student providing this treatment and the prosthodontic specialist in an academic institute while they were in different places. The dental treatment in this case was provided holistically and patient-centered, the dental treatment outcomes were achieved through the follow-up visits. Conclusions: This case study revealed that a dementia patient could receive appropriate dental treatment in cooperation with the care team to maintain her oral health function. Behavioral management could be performed using non-pharmacological methods. After delivering the denture, the patient’s nutritional status and the quality of life largely depends on the caregiver when the dementia progresses.
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