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| CASE REPORT |
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| Year : 2017 | Volume
: 7
| Issue : 1 | Page : 45-48 |
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Prosthodontic management of a completely edentulous patient with Huntington's disease: A rare case report
Poojya Ramdev, Sapna Bhat, GS Amarnath, CS Shruthi
Department of Prosthodontics, M R Ambedkar Dental College and Hospital, Bengaluru, Karnataka, India
| Date of Web Publication | 30-Jun-2017 |
Correspondence Address:
Poojya Ramdev
Department of Prosthodontics, M R Ambedkar Dental College and Hospital, Bengaluru - 560 005, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijmd.ijmd_29_16
Dentists have a large role in geriatric health care for the ever increasing elderly population with associated physical and neurological disorders. According to the World Health Organization, between 2015 and 2050, the proportion of the world's population over 60 years will nearly double from 12% to 22%. Huntington's disease is a rare neurodegenerative, adult-onset familial disorder, which can affect both men and women. Poor oral hygiene and increased susceptibility to dental caries and periodontal diseases predispose them to early edentulism. Providing an effective dental prosthesis will alleviate functional, esthetic, and psychological disabilities of the patient. This case report explains the steps involved in the complete denture rehabilitation of a patient with Huntington's disease, with some modification of techniques and materials to suit the special situation. Keywords: Edentulous cases; Huntington's disease; neurodegenerative disorder
How to cite this article:
Ramdev P, Bhat S, Amarnath G S, Shruthi C S. Prosthodontic management of a completely edentulous patient with Huntington's disease: A rare case report. Indian J Multidiscip Dent 2017;7:45-8 |
How to cite this URL:
Ramdev P, Bhat S, Amarnath G S, Shruthi C S. Prosthodontic management of a completely edentulous patient with Huntington's disease: A rare case report. Indian J Multidiscip Dent [serial online] 2017 [cited 2023 Mar 20];7:45-8. Available from: https://www.ijmdent.com/text.asp?2017/7/1/45/209271 |
| Introduction | |  |
Huntington's disease is a rare neurodegenerative disorder of the central nervous system, characterized by unwanted choreatic movements, behavioral, psychiatric disturbances, and dementia. Mean age of onset of symptoms is 30–50 years, which is related to the size of the mutation which is an unstable expansion of cytosine–adenine–guanine sequence in a gene.[1] The classic triad of clinical features includes movement disorder, cognitive impairment, and personality and psychiatric disorder.[2] Patients with extrapyramidal diseases often cannot maintain independent, efficient oral hygiene due to restricted motor ability of the upper extremities and lack of coordination. The hermetic closure of the mouth and lips and the associated ability to keep liquids and toothpaste in the mouth can become so weak that effective oral hygiene cannot be maintained. Problems caused by Huntington's disease are accentuated by a dentist's lack of neurological knowledge and a neurologist's lack of awareness of many of the pathological changes in the oral cavity.[3]
| Case Report | | |
A 62-year-old female patient with Huntington's disease was referred to the Department of Prosthodontics of M R Ambedkar Dental College and Hospital, Bengaluru, for complete denture fabrication [Figure 1]. She was diagnosed with Huntington's disease at the age of 60, and since then, she was under medication for the same.
The patient's chief complaint was difficulty in mastication and speech. She presented a history of fall from the staircase 2 years ago, which resulted in head injury over her parietal region. She presented with a choreiform gait characterized by irregular, jerky, involuntary movements of the extremities. She required assistance to walk and get in/out of the dental chair indicating a lower level of muscle coordination. The mandibular movements showed slight trembling and exaggerated lip movements during conversation. The speech of the patient was soft and slurred. An orthopantomogram radiograph was made to rule out the presence of any pathology. The intraoral examination revealed low well-rounded, resorbed maxillary and mandibular residual ridges with the American College of Prosthodontics Classification Class 4 type, and hence, implants were not considered in the treatment.
The treatment objective was the complete denture rehabilitation of the patient to improve her nutrition status. The objective also included speech improvement and enhancing her psychological status. The patients consulting neurophysician's opinion were sought, and a fitness report was obtained for the dental treatment. The treatment options were discussed with the patient and her spouse in detail, and informed consent was obtained.
The patient was advised to consume the prescribed medication, 0.25 mg clonazepam and 25 mg revocon, 1 h before the treatment. The appointments were fixed in the morning for short duration of 45 min. Special emphasis was made to treat the patient in a compassionate, caring environment to alleviate anxiety. The dental chair was inclined at 45° position during impression procedures to avoid excessive saliva pooling and the risk of choking.
Primary impression was made with irreversible hydrocolloid using edentulous stock trays. A full-spacer design employing a double spacer over the rugae region in the maxillary and over the crest of the ridge in the mandibular cast was done, to provide relief, and special trays were fabricated from autopolymerization acrylic resin. Border molding was done in increments using green stick, and secondary impression was made with zinc oxide-eugenol paste [Figure 2]. The posterior palatal seal area was accurately marked in the patient's mouth and transferred to the cast. Since the residual ridges were resorbed, neutral zone technique was used to improve the stability of the dentures. Here, acrylic stops with grooves were provided over the temporary denture base, to stabilize the green stick impression compound. The green stick impression compound was then softened and placed inside the patient's mouth, and she was requested to perform physiological muscle function such as sucking, swallowing, and phonetics. A split putty index was made to guide teeth arrangement.
As the neuromuscular orientation caused interrupted jerky movements of the mandible, slight reduction in vertical dimension was done. Dawson's bilateral manipulation technique was utilized to guide the mandible to centric relation. Monoplane artificial acrylic teeth were selected to improve the masticatory efficiency and to avoid premature tooth contacts. An esthetic try-in was carried out in the subsequent appointment [Figure 3]. The denture was reinforced with a gold-plated mesh type of a framework which resulted in added benefit of sufficient strength, light weight, and biocompatibility [Figure 4]. Dentures were fabricated with high-impact denture resins. All laboratory procedures were carried out using semi-adjustable articulator. Rugae duplication was carried out using tin foil, to further enhance the speech and comfort of the patient. To prevent accidental loss of dentures, details of the patient such as name, age, and sex were engraved on a metal foil and embedded in the denture using a denture marker technique.
The denture was delivered to the patient and frequent water sipping with artificial saliva substitutes was indicated in these patients to manage xerostomia. Water-based denture cleansing aids were prescribed to improve oral hygiene [Figure 5]. The patient was discouraged the usage of mouthwashes to prevent the risk of accidental aspiration. The patient was asked to see the dentist at least every 4 months once, after educating the patient and the spouse or caregiver regarding the postinsertion care of the prosthesis for the long-term success of the treatment. Follow-up recall visits were planned for continuous evaluation, and corrections were performed if necessary. The prognosis of the treatment outcome was good, and the nutritional status of the patient significantly improved following complete denture rehabilitation.
| Discussion | | |
Geriatric health care is an important aspect of healthcare system around the world, and dentists play a significant role in restoring the quality of life in elderly patients. Huntington's disease is an autosomal dominant disorder caused by a faulty gene, which produces a protein called huntingtin, on chromosome 4, which means that there is a one in two chance of inheriting the disorder from an affected parent. The faulty gene leads to damage of the nerve cells in specific areas of the brain, including the basal ganglia and cerebral cortex, leading to a complex mixture of physical, cognitive, and emotional problems, and resulting in profound disability. It affects both males and females equally. Its onset is commonly between the ages of 30 and 50 although there are about 10% of juvenile cases where onset is before the age of 20.[4]
Psychiatric symptoms such as obsessions, aggression, and compulsions are frequently present in the early stages of the disease, before the onset of motor symptoms. The physical symptoms present challenges for daily routine including dental care. Most people eventually exhibit jerky, random, uncontrollable “choreiform” movements although some exhibit bradykinesia (slow movements) and dystonia (stiffness). These abnormal movements are initially exhibited as a general lack of coordination and unsteady gait, gradually increasing as the disease progresses.[4]
To follow the patient systematically, mainly for research purposes, several scales have been developed. The best known are the Shoulson and Fahn Capability Scale and the Unified Huntington's Disease Rating Scale (UHDRS). The UHDRS consists of a motor, behavior, cognitive, and functional part, preceded by a history and medication scheme. For the behavior signs, a new scale was developed by Craufurd: the problem behavior scale.[1] The current gold standard is DNA determination, showing a cytosine–adenine–guanine repeat of at least 36 on the huntingtin gene on chromosome.[1]
The dietician plays a significant role throughout all the stages of progression of Huntington's disease. These patients are known to have a lower body mass index, which becomes more marked as the disease progresses. The cause of weight loss in Huntington's disease is not fully understood and may be due to a combination of factors such as increased energy expenditure, metabolic changes, and reduced oral intake. Care plans including nutrition support measures are appropriate for use. This may include the use of food fortification and increasing nourishing snacks and drinks. The European Huntington's Disease Network Dietitians Standards of Care Group recommends 0.8–1.5 g protein/kg/day fat and carbohydrate as general population and a total of 25–35 kcal/kg/day.[5]
A small study of the use of oral nutritional supplements in patients with Huntington's disease found that provision of two supplement drinks per day providing a total of 473 kcal, over 90 days, was effective in promoting weight gain, increasing mid-upper arm circumference, and percentage body fat in over 68% of participants in the study.[5]
Although the pathogenesis has still not been resolved and a cure is not available, many therapeutic options are available for treating symptoms and signs with a view to improving quality of life. Treatment consists of drug prescription and nonmedication advice. Nonmedical interventions available are physiotherapy, occupational therapy, speech therapy, dietician, psychologist, social worker, and nurse.[1]
Preventive dentistry is the most important aspect of dental treatment and individuals in any stage of the disease should see a dentist every 4 months whether they have natural teeth, complete dentures, or a mixture of teeth and dentures. An inefficient oral hygiene due to restricted motor ability of the upper extremities, a lack of coordination, and/or an impairment of the hermetic closure of the mouth and lips due to more days and hyperkinesia of the tongue and of the perioral musculature might contribute to the impaired dental health status.[3]
Success of dental treatment has been attributed to the dental team approach and short duration appointments to limit patient stress.[4] Smiling, direct eye contact, and gentle touch are known to alleviate anxiety. The semi-reclined 45° position during impression procedure is advantageous for avoiding excessive saliva pooling and avoiding the risk of choking. Conscious sedation can be used to help control movements, to manage swallowing problems, and to reduce the risk of aspiration.[4] Many studies have stated that patients with Huntington's disease are often not capable of maintaining oral hygiene due to restricted motor ability of upper extremities and lack of coordination, hyperkinesia of the tongue and perioral musculature. Therefore, effective complete denture rehabilitation will help Huntington's disease patients in alleviating both psychological and physical debilities to a significant extent.
| Summary | | |
Oral health care for patients suffering from Huntington's disease requires conscious effort and concern depending on the progression of the disease. More dental professionals need to be involved to provide a functional, esthetic, and psychological rehabilitation of such patients. Thus, the success of the prosthesis depends on careful approach with diligent handling of Huntington's disease patients during the entire treatment procedure.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Roos RA. Huntington's disease: A clinical review. Orphanet J Rare Dis 2010;5:40.  |
| 2. | Manley G, Lane H, Carlsson A. European Huntington's Disease Networks Standards of Care Dental Care Group. Guideline for oral healthcare of adults with Huntington's disease. Neurodegener Dis Manag 2012;2:55-65. |
| 3. | Saft C, Andrich JE, Müller T, Becker J, Jackowski J. Oral and dental health in Huntington's disease-An observational study. BMC Neurol 2013;13:114. |
| 4. | Lewis D, Fiske J, Dougall A. Access to special care dentistry, part 7. Special care dentistry services: Seamless care for people in their middle years – Part 1. Br Dent J 2008;205:305-17. |
| 5. | Howle N. Nutrition and Huntington's disease. Complete Nutr 2015;15:42-4. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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