This article has Open Peer Review reports available.
Oral and dental health in Huntington‘s disease - an observational study
© Saft et al.; licensee BioMed Central Ltd. 2013
Received: 11 March 2013
Accepted: 29 August 2013
Published: 3 September 2013
Only a few case reports and case series dealing with oral and dental health care are available in literature until now. The aim of the present pilot study was to determine the status of dental health in comparison to matched controls and to heighten the neurologists’ and dentists’ awareness of the oral aspects of the disease.
42 Huntington’s disease (HD) participants were scored according to the Unified Huntington’s Disease Rating Scale. The dental status was assessed by using the well established score for decayed, missing, and filled teeth (DMFT) and the dental plaque score (Silness-Loe plaque index).
Compared to controls HD participants showed significantly more decayed teeth and more plaques in both plaque indices. A higher motor impairment and a lower functional status of the patients lead to a worsening in dental status.
Possible reasons for our findings are discussed. Apart from local oral complications general complications may also occur. Thus, as a consequence, we would encourage patients, caregivers, neurologists, and the dentists to ensure regular preventive dental examinations and dental treatments of individuals with Huntington’s disease even in the premanifest stage of this disease.
Problems caused by HD are accentuated by the dentist’s lack of neurological knowledge (i.e. pharmacological therapy, clinical fluctuations of drug therapy, and behavioural aspects) and the neurologist’s lack of awareness of many of the pathological changes in the oral cavity (i.e. oral and dental side effects and interactions of drugs used to treat HD, etiology of gingivitis, parodontitis, and caries).
Jackowski and colleagues stated in their case report that patients with extrapyramidal diseases, such as HD, are often not capable of maintaining independent and 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 liquid and toothpaste in the mouth might be impaired and, thus, an effective oral hygiene cannot be maintained. Moreover dyskinesia and hyperkinesia of the tongue and of the perioral musculature, combined with xerostomia and pooling of saliva, may have an influence. Finally, the different factors lead to a poor oral health status and loss of teeth. In the reported case an implant-supported complete denture led to a clear improvement in the patient's chewing function .
Deniz and colleagues mentioned cognitive decline and behavioural manifestations of the disease as well as medication side-effects as possible additional reasons contributing to a poor dental care status. In this case report, the patient was rehabilitated with a mandibular overdenture supported by two endosteal implants .
Based on our observations and the above mentioned case reports, the aim of this study was to evaluate the status of dental health in comparison to matched controls in HD.
Clinical characteristics of HD participants and controls
Age of onset a)
Age of onset
psychiatric symptoms b)
Duration of motor symptoms a)
Duration of psychiatric
The two established World Health Organization-approved dental indices for decayed, missing, and filled teeth (DMFT; 1938) and the dental plaque score (PI) developed by Silness and Loe (1964) were selected to quantify dental disease [13–15].
DMFT was used to assess the number of teeth with caries, including the number of present teeth, missing teeth, sound teeth, treated teeth, untreated teeth, and the total number of carious teeth (DMF teeth: decayed, missing, and filled teeth). For the PI score teeth surfaces were given a score from 0 (no plaque) to 3 (abundance of soft matter within the gingival pocket and/or on the tooth and gingival margin). The index was obtained by calculating the mean for all investigated teeth and surfaces. PI-I was used for the mean of all teeth of the patient, PI-II for the mean of defined index-teeth. The teeth 17, 16, 11, 24, 26, 27, 37, 36, 31, 44, 46 and 47 were used for analysis according to the method described before . If one of the index teeth was missing, the next adjacent tooth wase used for evaluations. Plaque indices were only possible to assess for 41 HD participants, because one patient refused further investigation.
Statistical analysis was performed using the commercial software program SPSS statistics. All measured parameters and clinical data were first analysed descriptively and are presented as mean ± SD. Normality of distribution of the data was tested using the Shapiro-Wilk-test. Independent t-test, respectively Mann–Whitney-U-test, was used to test for differences between the two groups. Bonferroni correction was used for multiple testing. Spearman-Rho correlation analysis was used for exploratory statistical correlation analysis. All participants gave written informed consent.
The local ethics committee of the Ruhr University Bochum approved this study.
Dental status of HD participants and controls assessed by the DMFT score (1938) for decayed, missing and filled teeth and Plaque indices score after Silness and Loe (1964)
DMFT filled teeth
DMFT sum score
Plaque Indices I a)
Plaque Indices II a)
No differences between male and female HD participants were found. Explorative correlation analysis of DMFT and plaque indices (PI) results given in Table 2 with clinical symptoms showed no significant correlation with any of the clinical characteristics from Table 1, except for the UHDRS motor score and missing teeth (p 0.025, r .345), PI-I (p 0.009, r .404) and PI-II (p 0.006, r .426). The age of onset of motor symptoms showed a significant correlation with missing teeth (p 0.006, r .432), as did the psychiatric onset (p 0.020, r .416) and the TFC score correlated negative with the plaque scores (PI-I: p 0.001, r - .501; PI-II: p 0.001, r - .503).
To our best knowledge, this, present study is one of only a few reporting on dental health in HD. The results support our hypothesis that the dental health status is impaired in HD compared to controls. In particular, HD participants showed significant more decayed teeth and plaques as controls. A causal relationship between the dental status and HD is only partly supported by the correlation between UHDRS subscales and scores of the DMFT and PI scores. A lower functional status of the patients lead to a worsening in dental health (higher plaque scores). There was, however, no correlation between a higher plaque score or decayed teeth and the UHDRS motor score. Thus only an indirect interrelation between dental health and movement disorders could be found. We did not analyse the chorea subscore and parameters of dental health. The weak positive correlation with the age of onset might reflect an additional effect of age. To our surprise, there was no correlation between dental status and duration of the disease, which might be explained by an impaired oral health in the premanifest stage already.
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 dys- and hyperkinesia of the tongue and of the perioral musculature might contribute to the impaired dental health status in HD . We could not reveal any correlation between dental status and the mini-mental score and, therefore, we are not able to underline the thesis of cognitive decline as a reason for an impaired teeth status. This might also be due to the relative small number of patients investigated in this pilot-study. Because of the small number of participants we neither analyzed medication or smoking as possible influencing factors, nor did we analyze behavioural manifestations of the disease (e.g. using an apathy score) as reasons for the poor dental status, which is a clear limitation of our study. We also did not ask how frequently the patients cleaned their teeth compared to controls. All factors, however, might contribute, especially apathy may be a reason for a lack of dental hygiene even in premanifest stages of the disease [16, 17]. Moreover, an impaired awareness of a poor dental status might be an explanation, since an impaired awareness of motor, functional and cognitive deficits is known in HD [18, 19]. As an additional possible reason an altered sensory processing and lack of pain recognition might contribute [20, 21]. Salivation can be altered due to medication effects but also due to altered autonomic function and hormonal influences [22–25]. In more advanced stages of the disease also dysphagia, vomiting and regurgitation influences the dental status . Thus, influences from the disease itself as well as secondary mechanisms like malnutrition, medication and general disability may contribute.
We also found a lower score for filled teeth, that is for treated teeth by a dentist and a higher level of missing teeth in the HD group. Both findings, however, failed to reach statistical significance. The lower scores might be explained by a higher rate of tooth extraction or due to trauma and the consequence of falls, which is supported by the correlation of missing teeth with the UHDRS motor score. A poorer dental status may lead earlier to a tooth extraction as the only causal therapy in HD patients.
It is discussed whether the dental care status predicts circulatory mortality and other causes of death. As possible pathways, the effects of masticatory dysfunction on dietary behaviour, nutrition and systemic diseases and inflammatory effects on the circulatory system are discussed [27–29]. Oral disease was found to be associated with excess cardiovascular disease risk, with possibly common pathogenetic mechanisms between poor oral health and cardiovascular disease .
In some cases "a bad tooth" was discussed as the cause of premature death (e.g. in the case of Theodore Roosevelt (1858–1919) the twenty-sixth President of the United States who anecdotally died from oral sepsis) [31, 32]. Thus, beside local complications it cannot be excluded that the impaired dental status in HD leads to an earlier death directly or by triggering cardiovascular diseases [33, 34].
Preventive dentistry is the most important aspect of dental treatment for the person with Huntington’s disease, as with all patients. Although the goals of preventive dentistry include self-sufficiency, the spouse or caregiver must be educated to supervise and evaluate hygiene procedures daily at home. As HD progresses, patients become increasingly incapable of completing even the simplest forms of oral care. The primary goals of dental treatment planning should be maintenance of natural dentition, early intervention, regular preventive treatment and preservation of self-care. HD subjects in any stage of the disease should see a dentist at least every 4 months whether they have natural teeth, complete dentures, or a mixture of teeth and dentures. Pathological conditions may be diagnosed early and treated in simple stages. When clinical findings suggest Huntington’s disease, the patient should be referred for oral and dental evaluation. Oral health parameters, including salivary flow gingival health (bleeding, plaque, calculus), periodontal health (pocket depth, recession, loss of attachment), and dental condition (number of teeth, decay, restored), must be measured at onset (baseline) and course of the disease. Some authors also recommend to visit the same dentist over time, if possible .
We are grateful to all patients for participation.
Carsten Saft was supported by a FoRUM grant, University of Bochum (AZ: K040-2009).
- Boyle CA, Frolander C, Manley G: Providing dental care for patients with Huntington's disease. Dent Update. 2008, 35 (5): 333-336.PubMedGoogle Scholar
- Deniz E, Kokat AM, Noyan A: Implant-supported overdenture in an elderly patient with Huntington's disease. Gerodontology. 2011, 28 (2): 157-160. 10.1111/j.1741-2358.2009.00343.x.View ArticlePubMedGoogle Scholar
- Jackowski J, Andrich J, Kappeler H, Zöllner A, Johren P, Muller T: Implant-supported denture in a patient with Huntington's disease: interdisciplinary aspects. Spec Care Dentist. 2001, 21 (1): 15-20. 10.1111/j.1754-4505.2001.tb00218.x.View ArticlePubMedGoogle Scholar
- Kieser J, Jones G, Borlase G, MacFadyen E: Dental treatment of patients with neurodegenerative disease. The New Zealand Dent J. 1999, 95 (422): 130-134.Google Scholar
- da Fonseca MA, Walker PO: Dental management of a child with Huntington's disease: case report. Spec Care Dentist. 1993, 13 (2): 71-73. 10.1111/j.1754-4505.1993.tb01458.x.View ArticlePubMedGoogle Scholar
- Rada RE: Comprehensive dental treatment of a patient with Huntington's disease: literature review and case report. Spec Care Dentist. 2008, 28 (4): 131-135. 10.1111/j.1754-4505.2008.00027.x.View ArticlePubMedGoogle Scholar
- Bradford H, Britto LR, Leal G, Katz J: Endodontic treatment of a patient with Huntington's disease. J Endod. 2004, 30 (5): 366-369. 10.1097/00004770-200405000-00014.View ArticlePubMedGoogle Scholar
- Cangemi CF, Miller RJ: Huntington's disease: review and anesthetic case management. Anesth Prog. 1998, 45 (4): 150-153.PubMedPubMed CentralGoogle Scholar
- Feeney AW: Dental treatment considerations for patients with Huntington's chorea: a literature review and case report. J Conn State Dent Assoc. 1985, 59 (4): 118-123.PubMedGoogle Scholar
- Moline DO, Iglehart DR: Huntington's chorea: review and case report. Gen Dent. 1985, 33 (2): 131-133.PubMedGoogle Scholar
- Huntington SG: Unified Huntington's disease rating scale: reliability and consistency. Huntington study group. Mov Disord. 1996, 11 (2): 136-142.View ArticleGoogle Scholar
- Folstein MF, Folstein SE, McHugh PR: Mini-mental state. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res. 1975, 12 (3): 189-198. 10.1016/0022-3956(75)90026-6.View ArticlePubMedGoogle Scholar
- WHO Collaborating Centre for Education, Training and Research at the Faculty of Odontology. http://www.whocollab.od.mah.se,
- Silness J, Loe H: Periodontal disease in pregnancy. Ii. Correlation between oral hygiene and periodontal condtion. Acta Odontol Scand. 1964, 22: 121-135. 10.3109/00016356408993968.View ArticlePubMedGoogle Scholar
- Sheiham A, Sabbah W: Using universal patterns of caries for planning and evaluating dental care. Caries Res. 2010, 44 (2): 141-150. 10.1159/000308091.View ArticlePubMedGoogle Scholar
- Craufurd D, Thompson JC, Snowden JS: Behavioral changes in Huntington disease. Neuropsychiatry Neuropsychol Behav Neurol. 2001, 14 (4): 219-226.PubMedGoogle Scholar
- Kingma EM, van Duijn E, Timman R, van der Mast RC, Roos RA: Behavioural problems in Huntington's disease using the problem behaviours assessment. Gen Hosp Psychiatry. 2008, 30 (2): 155-161. 10.1016/j.genhosppsych.2007.11.005.View ArticlePubMedGoogle Scholar
- Hoth KF, Paulsen JS, Moser DJ, Tranel D, Clark LA, Bechara A: Patients with Huntington's disease have impaired awareness of cognitive, emotional, and functional abilities. J Clin Exp Neuropsychol. 2007, 29 (4): 365-376. 10.1080/13803390600718958.View ArticlePubMedGoogle Scholar
- Snowden JS, Craufurd D, Griffiths HL, Neary D: Awareness of involuntary movements in Huntington disease. Arch Neurol. 1998, 55 (6): 801-805. 10.1001/archneur.55.6.801.View ArticlePubMedGoogle Scholar
- Boecker H, Ceballos-Baumann A, Bartenstein P, Weindl A, Siebner HR, Fassbender T, Munz F, Schwaiger M, Conrad B: Sensory processing in Parkinson's and Huntington's disease: investigations with 3D H(2)(15)O-PET. Brain. 1999, 122 (Pt 9): 1651-1665.View ArticlePubMedGoogle Scholar
- Saft C, Schuttke A, Beste C, Andrich J, Heindel W, Pfleiderer B: fMRI reveals altered auditory processing in manifest and premanifest Huntington's disease. Neuropsychologia. 2008, 46 (5): 1279-1289. 10.1016/j.neuropsychologia.2007.12.002.View ArticlePubMedGoogle Scholar
- Andrich J, Schmitz T, Saft C, Postert T, Kraus P, Epplen JT, Przuntek H, Agelink MW: Autonomic nervous system function in Huntington's disease. J Neurol Neurosurg Psychiatry. 2002, 72 (6): 726-731. 10.1136/jnnp.72.6.726.View ArticlePubMedPubMed CentralGoogle Scholar
- Kobal J, Melik Z, Cankar K, Bajrovic FF, Meglic B, Peterlin B, Zaletel M: Autonomic dysfunction in presymptomatic and early symptomatic Huntington's disease. Acta Neurol Scand. 2010, 121 (6): 392-399.View ArticlePubMedGoogle Scholar
- Petersen A, Bjorkqvist M: Hypothalamic-endocrine aspects in Huntington's disease. Eur J Neurosci. 2006, 24 (4): 961-967. 10.1111/j.1460-9568.2006.04985.x.View ArticlePubMedGoogle Scholar
- Bjorkqvist M, Petersen A, Bacos K, Isaacs J, Norlen P, Gil J, Popovic N, Sundler F, Bates GP, Tabrizi SJ, et al: Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease. Hum Mol Genet. 2006, 15 (10): 1713-1721. 10.1093/hmg/ddl094.View ArticlePubMedGoogle Scholar
- Andrich JE, Wobben M, Klotz P, Goetze O, Saft C: Upper gastrointestinal findings in Huntington's disease: patients suffer but do not complain. J Neural Transm. 2009, 116 (12): 1607-1611. 10.1007/s00702-009-0310-1.View ArticlePubMedGoogle Scholar
- Polzer I, Schwahn C, Volzke H, Mundt T, Biffar R: The association of tooth loss with all-cause and circulatory mortality. Is there a benefit of replaced teeth? A systematic review and meta-analysis. Clin Oral Investig. 2012, 16 (2): 333-51. 10.1007/s00784-011-0625-9.View ArticlePubMedGoogle Scholar
- Hoke M, Schillinger T, Mlekusch W, Wagner O, Minar E, Schillinger M: The impact of dental disease on mortality in patients with asymptomatic carotid atherosclerosis. Swiss Med Wkly. 2011, 141: w13236-PubMedGoogle Scholar
- Sadeghi M, Lynch CD, Arsalan A: Is there a correlation between dental caries and body mass index-for-age among adolescents in Iran?. Community Dent Health. 2011, 28 (2): 174-177.PubMedGoogle Scholar
- Mucci LA, Hsieh CC, Williams PL, Arora M, Adami HO, de Faire U, Douglass CW, Pedersen NL: Do genetic factors explain the association between poor oral health and cardiovascular disease? A prospective study among Swedish twins. Am J Epidemiol. 2009, 170 (5): 615-621. 10.1093/aje/kwp177.View ArticlePubMedPubMed CentralGoogle Scholar
- Christen AG, Christen JA: Theodore Roosevelt's "presidential smile" and questionable dental health. J Hist Dent. 2007, 55 (2): 85-90.PubMedGoogle Scholar
- Ocampo Flores P, Limon Mejia AL, Bustillos Lucas J, Silva Sanchez V: Death from generalized sepsis of dental origin. Contribution to clinical casuistry. Rev ADM. 1991, 48 (1): 45-51.PubMedGoogle Scholar
- Lanska DJ, Lanska MJ, Lavine L, Schoenberg BS: Conditions associated with Huntington's disease at death. A case–control study. Arch Neurol. 1988, 45 (8): 878-880. 10.1001/archneur.1988.00520320068017.View ArticlePubMedGoogle Scholar
- Lanska DJ, Lavine L, Lanska MJ, Schoenberg BS: Huntington's disease mortality in the United States. Neurology. 1988, 38 (5): 769-772. 10.1212/WNL.38.5.769.View ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/13/114/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.