T O U R E T T E S Y N D R O M E A N D D E N TA L T R E AT M E N T
ARTICLE ABSTRACT Purpose: Dental treatment of patients with Tourette syndrome (TS) may present special challenges to the dentist. The aim was to systematically review the literature regarding perioperative procedures including sedation and general anaesthesia (GA) of patients with TS. Materials and methods: Literature searches were performed in PubMed and Embase to identify papers concerning TS in combination with dental treatment, sedation, and/or GA in order to study outcomes regarding co-morbidity, perioperative complications, and drug interactions. Results: The literature search identified six publications (case reports or series) which addressed the topic. No unexpected adverse effects or drug interactions in relation to sedation or GA in TS patients and no perioperative complications were reported. Conclusions: The literature on TS is scarce and the evidence level is low. Therefore, guidelines regarding the dental treatment of patients with TS cannot be formulated at the present time.
KEY WORDS: sedation, general anaesthesia, dentistry, Tourette syndrome
Tourette syndrome and procedures related to dental treatment: a systematic review Jonas Kjeldbjerg Hansen, MD;1* Pernille Endrup Jacobsen, DDS, PhD;2,3 Janne Lytoft Simonsen, MSc, PhD;4 Ole Hovgaard, DDS, PhD;5 Dorte Haubek, DDS, Dr. Odont6 1Consultant,
The Paediatric Department, Viborg Regional Hospital, Central Jutland, Denmark; Professor, Section for Paediatric Dentistry, Department of Dentistry, Aarhus University, Denmark; 3Department of Specialised Oral Health Care, Viborg Regional Hospital, Central Jutland, Denmark; 4Research Librarian, Aarhus University Library Health Sciences, Aarhus University, Denmark; 5 Chief dentist, Department of Specialised Oral Health Care, Viborg Regional Hospital, Central Jutland, Denmark; 6Professor, Section for Paediatric Dentistry, Department of Dentistry, Aarhus University, Denmark. *Corresponding author email: [email protected] 2Assistant
Spec Care Dentist 35(3): 99-104, 2015
Introd uct ion
Tourette syndrome (TS) is a neurodevelopmental disorder with a prevalence of up to 1%.1-5 It is defined by multiple motor tics and one or more phonic tics, not necessarily occurring concomitantly, but lasting for more than 1 year and with a debut before 18 years of age. Tics manifest as sudden, rapid, recurrent, non-rhythmic, stereotyped movements, or sounds.1-5 Tics usually have a waxing and waning course. Stress and anxiety will exacerbate tics.3,4
Although tics are the defining feature of TS, co-morbid psychiatric conditions occur in up to 90% of TS patients and often cause more morbidity than the tics themselves.1,2,4,5 Attention deficit hyperactivity disorder is the most common co-morbid diagnosis, followed by obsessive-compulsive disorder. Other co-morbid conditions include anxiety disorder, depression, explosive outbursts, and learning disorders.1-5 Self-injurious behavior2-5 and autism spectrum disorders5 have also been associated with TS. Tics and the co-morbid conditions frequently require medical treatment. Treatment of tics most commonly involves alfa-adrenergic agonists or antidopaminergic drugs. Other drugs, including anticonvulsant (topiramate), benzodiazepines, and botulinum toxin A © 2014 Special Care Dentistry Association and Wiley Periodicals, Inc. DOI: 10.1111/scd.12098
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may also be used. Attention deficit hyperactivity disorder is most often treated with stimulants (methylphenidate and dextroamphetamine), alternatively norepinephrine re-uptake inhibitor (atomoxetine). Obsessive-compulsive disorder may be treated with a selective serotonin re-uptake inhibitor, rarely an atypical neuroleptic.1,3,4 Patients with TS pose several challenges to the dentist. Tics themselves can be a hazard to the patient if they occur during a dental procedure, and both tics and some of the co-morbid conditions could be expected to be aggravated by the stress and anxiety associated with undergoing dental treatment. These are all factors which can complicate dental procedures and may entail a need for the use of sedation of patients with TS.
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Table 1. Search terms for the systematic literature search. PubMed
Embase
Tourette Syndrome (TS)
“Tourette Syndrome” [Mesh]
“gilles de la tourette syndrome”/exp
Dental treatment
Dental OR Dentist OR Dentists OR Tooth OR Teeth [Title/Abstract]
dental:ab OR dental:ti OR dentistry:ab OR dentistry:ti OR dentist:ab OR dentist:ti OR dentists:ab OR dentists:ti OR tooth:ab OR tooth:ti OR teeth:ab OR teeth:ti
Sedation or General anaesthesia (GA)
“Anesthesia” [Mesh] OR “Anesthesia Recovery Period” [Mesh] OR “Conscious Sedation” [Mesh] OR “Deep Sedation” [Mesh] OR “Anesthetics” [Mesh] OR “Postoperative Complications” [Mesh] OR “Perioperative Care” [Mesh] OR “Perioperative Period” [Mesh] NOT “Deep Brain Stimulation” [Mesh]
“anesthesia”/exp OR “anesthetic recovery”/exp OR “conscious sedation”/exp OR “deep sedation”/exp OR “anesthetic agent”/exp OR “postoperative complication”/exp OR “perioperative period”/exp NOT “brain depth stimulation”/exp
Search 1: Tourette Syndrome search AND Dental treatment search. Search 2: Tourette Syndrome search AND Sedation or general anaesthesia search.
Furthermore, possible interactions between the patient’s daily medication and drugs used in relation to the dental procedure must be taken into consideration.6 The purpose of this study was to systematically review this literature regarding the dental treatment of patients with TS, including sedation and GA and if possible provide clinical guidelines.
Mater ial and me tho ds
The study was performed as a systematic review of original publications reporting perioperative procedures, including the use of sedation, and/or GA in patients diagnosed with TS.
Search strategy The term “Tourette Syndrome” was searched for as a controlled search term. The search strategy was developed for PubMed and was revised appropriately for Embase to comply with differences in MeSH and Emtree terms. The MeSH and EMTREE terms were “exploded.” We conducted two separate searches. Search 1 focused on TS and dental treatment by combining “Tourette syndrome” with words like “dental” OR “teeth”, among others. Search 2 focused on TS in combination with sedation and GA by
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combining the search term “Tourette Syndrome” with MeSH terms like anaesthesia OR premedication (for detailed search strings see Table 1). Both databases were searched from 1980 to April 2014. Additionally, reference lists were hand-searched to identify the additional eligible studies. The eligibility criteria were clinical studies (follow-up, case-control, or crosssectional) or case reports involving humans of all ages that focused on the association between TS and complications in relation to dental treatment, sedation, and/or GA. Only papers written in English were included.
Selection of studies and data extraction The search produced 82 references, which were reduced to 70 references after screening for duplicates. Reports concerning perioperative complications in relation to sedation or GA were included whether they were combined with dental treatment or not. Subsequent screening of the title and the abstract identified 13 articles in which the term “Tourette Syndrome” was the diagnostic term applied by the authors of the paper, thereby making the publication relevant for this review. In case of disagreement, the abstract was discussed in plenum by
all authors until consensus as to the inclusion or exclusion was obtained. Reviews were excluded. Thirteen full-length articles were studied, and after excluding reviews, brief communications, etc., five publications, all case reports, were identified and formed the basis for the subsequent data extraction. One additional publication was identified by hand-searching the reference list of the included papers. The inclusion of publications is illustrated in Figure 1. The data extracted from the published reports reviewed are shown in Table 2 (demographics, various information, and the outcomes). It was impossible to pre-specify all the variables presented in Table 2 because of the heterogeneity of the information in the included studies. The data extraction form was revised by all authors to facilitate accuracy and clarity in the collection of data. The final data extraction form was used to evaluate all the included articles independently by two reviewers (JKH and PEJ).
R es ul t s Basic results In total, seven TS cases were identified in six studies.7-12 All the included studies
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Outcomes Dental treatment Of the seven cases described, four had oral or dental procedures performed. One underwent dental treatment because of caries,12 and three underwent oral or dental procedures because of self-mutilation. One patient had performed self-extraction of teeth,7 one presented with self-mutilation of the tongue,8 and one presented with self-mutilation of the upper lip,11 all reported under oral-facial problems in Table 2. In two of these cases, the oral or dental self-mutilation was the presenting symptom that eventually led to a diagnosis of TS.7,11
Sedation Sedation was used in four of the seven cases. Three were sedated with a benzodiazepine (midazolam, diazepam),10-12 and one with morphine sulphate in combination with droperidol.8 Of these four cases, one case received no daily medication,10 and three received haloperidol either as mono therapy (one case) or in combination with other drugs (two cases).8,11,12 No interactions or unexpected adverse effects were described regardless of the type of sedation or the use of daily medication. Benzodiazepines used for the sedation were found efficient in controlling tics,10,12 and in one case, it was specified that no delayed arousal or exacerbation was seen.12 Figure 1. Flow diagram illustrating the inclusion of literature.
were case reports or case series and involved patients living on three continents (Asia, Europe, and North America; Table 2). The year of publication varied by 20 years, with two papers from 1986,8,9 one from 2002,12 one from 2003,11 one from 2005,7 and one from 2006.10
Clinical presentation In one case, a positive family history of disorders predisposing to TS was reported.7 In three cases, no family his-
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tory was found,8,10,11 and in three cases the family history was not mentioned.9,12 Information on whether the diagnosis of TS had been made according to generally applied criteria was not given in any of the described cases. Co-morbidity was described in five cases, and included obsessive-compulsive disorder/behavior, hyperactivity, selfmutilation, speech problems, emotional outbursts, and emotional lability.7–9,11 In two cases, no co-morbid conditions were reported.10,12
General anaesthesia Five patients underwent GA in relation to the procedure or surgery performed,8–10,12 but no complications in relation to GA was reported. The anaesthetics used varied greatly (Table 2). Three of the five patients were on daily medication.8,9,12 Regardless of which combinations of daily medication the patients were on and which anaesthetic medications were used, no drug interactions were reported. One paper did not mention which anaesthetics were used.8
Perioperative complications No perioperative complications were reported in the four patients undergoing
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Turkey
Woman, 21 yrs, pregnant
No
No
No
Not described
Midazolam
Succinylcholine, Not described Propofol 50% nitrous oxide in oxygene Desflurane 3% Cisatracurium Mepridine Oxycontin (for uterine contraction) Metoclopramide (antiemetic) Diclofenac potassium
No
No
No
Country/State
Gender, age
Family history
Daily medication against tics or co-morbid condition
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Specification of the diagnostic criteria of TS
Co-morbidity
Sedation
General anesthesia
Drug interactions
Peri-operative complications
Oro-facial problem
Self-extraction of teeth
Not described
Not described
Not described
Obsessive-compulsive behavior Hyperactivity Self-mutilation
No
No
Mother: Tics in childhood Father: Attention and concentration problems in school age
Girl, 4 yrs
Sweden
Case report (1)
Case report (1)
Design (no. of cases described in the report)
Leksell & Edvardson 20057
Sener et al. 200510
Reference
Stomatitis, decayed teeth
No
No
Atropine sulphate Thiamyl Vecuronium 30% nitrous oxide and 0.6-1% sevoflurane in oxygen
Midazolam
Not described
No
Haloperidol Fluvoxamine Milnacipran hydrochloride
Not described
Boy, 13 yrs
Japan
Case report (1)
Yoshikawa et al. 200212
Self-mutilation of the tongue
Not described
No
The patient had general anesthesia, but no information regarding which drugs was used
Morphine sulphate Droperidol
Hyperactivity Speech problem Self-mutilation
No
Haloperidol
No
Boy, 16 yrs
USA/California
Case report (1)
Lowe 19868
Table 2. Summary of findings in seven Tourette Syndrome cases in six studies.
Self-mutilation of the upper lip
No operation performed
No
Not relevant
Diazepam
Obsessive-compulsive disorder Self-mutilation
No
Haloperidol Bromazepam Fulnitrazepam
No
Boy, 13 yrs
Japan
Case report (1)
Shimoyama et al. 200311
No
Case 1: No Case 2: No
Case 1 + 2: No
Case 1: Morphine sulphate, fentanyl, hydroxyzine pamoate, thiopental, d-tubocurarine, succinylcholine, nitrous oxide in oxygen, enflurane Droperidol Case 2: Thiopental, halothane, nitrous oxide in oxygen
Not used
Case 1: Emotional outburst Case 2: Hyperactivity, emotional lability
No
Case 1: Haloperidol and clonidine Case 2: No daily medication
Case 1: Not described Case 2: Not described
Case 1: Male, 28 years Case 2: Boy, 12 years
USA/Colorado
Case report (2)
Morrison & Lockhart 19869
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The specific anesthetic chosen is less important than the general approach to the patient, taken by the operating room team. Intensification of sedation may temporarily be effective in order to prevent further selfmutilation. Dental care should be planned as soon as possible when signs of oral tics occur. Metoclopramide, ondansetron, midazolam and opioids may be used safely for anesthesia in TS. Conclusions
Cooperation between the dentist and the child neurologist is recommended in order to allow early diagnosis and treatment of TS.
Sedation or GA as described efficiently prevented tics during the procedures.
Morphine sulphate and droperidol were used without complications.
Morrison & Lockhart 19869 Sener et al. 200510 Reference
Table 2. Contined.
Leksell & Edvardson 20057
Yoshikawa et al. 200212
Lowe 19868
Shimoyama et al. 200311
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oral or dental procedures or the three patients subjected to other types of surgery (caesarean delivery,10 retinal surgery, herniotomy9).
Di scus s ion
The present review revealed only a few studies regarding procedures related to the treatment of patients with TS in a clinical dental setting. Furthermore, the total number of reported cases was low. These factors weakened the possibility of providing general recommendations or guidelines regarding dental treatment of patients with TS. The present review was methodologically carried out as a systematic review, however, the low evidence of the existing literature, leads to a more descriptive reporting of the listed outcomes (Table 2). On the other hand, the sparse number of papers facilitated the opportunity to include all interesting findings and thereby revealing the importance of the dental teams to be aware of the early symptoms of TS. The studies were published over a time span of 20 years and originated from three different continents. Thus, a great variety of drugs and combinations of drugs had been used. Accordingly, the conclusions of this study should be generally applicable. The method followed in the present review was in accordance with the PRISMA statement, and thus aimed to secure a systematic selection and evaluation of all relevant material on the topic. Due to the low level of evidence of the papers identified, it was not relevant to use quality score assessment instruments as NOS or GRADE. Although the search strategy was developed for a specific database, the number of references identified in the GA search in Embase was three times larger than in PubMed. Embase has a more detailed pharmacological profile, and the search result contained a large number of publications with a medical approach, and thus not relevant for the purpose of this review. The majority of these publications were later excluded in the data selection. In the seven cases reported on in the present literature, it was not specified
whether the diagnosis of TS fulfilled the diagnostic criteria according to DSM-IV or ICD-10.7-12 How the diagnosis was reached in each case was described in variable detail. However, based on a thorough review of the presented cases, it is reasonable to expect that the cases identified in the case reports correspond to the focus of this study.
Dental treatment Self-mutilation is a common co-morbid feature in patients with TS. The case reports by Lowe8 and Shimoyama et al.11 illustrate the risk of severe injury to the tongue or lip caused by biting during tics. In both cases, a splint was recommended to prevent further injury because the pain itself can have a self-reinforcing effect on the tics. The report by Leksel and Edvardson7 describes a 4-year old girl with unexplainable toothache. The dentition of the child was caries-free, but after a few consultations the primary canines suddenly showed extreme mobility. In this case, the dentist was the initial healthcare professional to observe clinical symptoms of TS, and the dentist’s referral to a neurologist resulted in a confirmed diagnosis of TS. The toothache was caused by extreme grinding during tics with tooth mobility and muscle tenderness as a result. As self-mutilation is a known co-morbid feature of TS2-5 and from the findings in this study, we would recommend that a diagnosis of TS should be considered and evaluated in patients presenting with dental or oral self-mutilation. In relation to TS patients, the dentist could be a relevant professional to include in the interdisciplinary diagnostic team. Tics occurring during dental or oral procedures can be a hazard to the patient. Tics themselves can be exacerbated by stress and anxiety,3,4,13 which could be elicited by the planned dental procedure(s). It has been recommended that time should be set aside for the anaesthesiologist to make a pre-anaesthetic consultation with the patient and his family prior to GA to reduce the patient’s anxiety, and thus reduce possible exacerbations of the patient’s motor symptoms.9 The study by Morrison and Lockhart9 specifically addressed pre-anaesthetic anxiety
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r eduction in patients with TS. This recommendation could also be applicable to the normal clinical dental setting where anxiety most commonly is reduced by thorough information and the acceptance of the procedures carried out. However, an individual approach is always necessary to identify the factors provoking the anxiety.14 On the basis of the reports in the literature and thus of the present review, it is not possible to provide general recommendations regarding stress reducing procedures in TS patients in a clinical dental setting with or without sedation or under GA.
Sedation Sedation may be necessary in order to reduce tics during the procedure. Benzodiazepines were used in three cases and appeared to be both safe and efficient in reducing tics.10-12 Morphine sulphate in combination with droperidol was used in one case with sedative effect and without adverse effects.8 The review shows that benzodiazepines are the most up-to-date and widely used drugs,10-12 and may be used to obtain sedation for dental or oral procedures in patients with TS. However, the data are too sparse to provide exact knowledge of the efficacy and safety in this setting.
General anaesthesia In five cases, GA was used.8–10,12 In all cases, tics were efficiently inhibited. No adverse effects or drug interactions were reported. However, it was not possible to compare the cases, because of differences in the anaesthetics used, and because some patients were on daily medications and some were not, and in some cases the anaesthesia was combined with sedation. Therefore, it is not possible to provide recommendations regarding GA in patients with TS undergoing dental treatment. In contrast, we cannot confirm the concerns stated by Friedlander and Cummings regarding interactions between the patient’s daily medication and the drugs used for conscious sedation or GA.6 In addition to tics, up to 90% of patients with TS suffer from co-morbid disorders.1,4 Co-morbidity was described in five cases, but its possible impact on the management of the patient was not described in any of the cases.7–9,11
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Therefore, many questions regarding dental treatment of patients with TS cannot be answered based on the published reports at present. Studies specifically addressing co-morbid conditions related to TS may provide useful information regarding handling of TS patients in a clinical dental setting, but this was beyond the scope of this study.
Co nclus ion
In conclusion, the literature on TS and its impact on procedures in dental treatment are too sparse at present to provide general recommendations on the dental treatment of TS patients. Based on the findings of the present review and general knowledge on TS, it seems appropriate to suggest a reduction of the patient’s anxiety prior to and during the procedures carried out. The findings concerning sedation and GA are too limited to enable formulation of general guidelines concerning the use of sedatives or anaesthetics, including their safety and efficacy in patients with TS. Therefore, a thorough review of the patient’s daily medication and a consideration of possible interactions with medications planned for sedation and local or general anaesthesia should be done in each case. Original research reports are warranted to elucidate the unanswered questions regarding the treatment of patients with TS in a clinical dental setting. Previous studies have addressed general aspects of movement disorders in the dental setting,15,16 however, this was not the aim of the present systematic review. It is recommended that patients presenting to the dentist with oral or dental self-mutilation should be referred to a relevant specialist for further evaluation of possible TS.
Co nf lict s of Int er es t None to declare.
References 1. Bloch M, State M, Pittenger C. Recent advances in Tourette syndrome. Curr Opin Neurol 2011;24:119-25.
2. Cavanna AE, Servo S, Monaco F, Robertson MM. The behavioral spectrum of Gilles de la Tourette syndrome. J Neuropsychiatry Clin Neurosci 2009;21:13-23. 3. Jankovic J, Kurlan R. Tourette syndrome: evolving concepts. Mov Disord 2011;26: 1149-56. 4. McNaught KS, Mink JW. Advances in understanding and treatment of Tourette syndrome. Nat Rev Neurol 2011;7:667-76. 5. Zinner SH, Coffey BJ. Developmental and behavioral disorders grown up: Tourette’s disorder. J Dev Behav Pediatr 2009;30:560-73. 6. Friedlander AH, Cummings JL. Dental treatment of patients with Gilles de la Tourette’s syndrome. Oral Surg, Oral Med, Oral Pathol. 1992;73:299-303. 7. Leksell E, Edvardson S. A case of Tourette syndrome presenting with oral self-injurious behaviour. Int J Paediatr Dent 2005;15:370-4. 8. Lowe O. Tourette’s syndrome: management of oral complications. ASDC J Dent Child 1986;53:456-60. 9. Morrison JE Jr, Lockhart CH. Tourette syndrome: anesthetic implications. Anesth Analg 1986;65:200-2. 10. Sener EB, Kocamanoglu S, Ustun E, Tur A. Anesthetic management for cesarean delivery in a woman with Gilles de la Tourette’s syndrome. Int J Obstet Anesth 2006;15:163-5. 11. Shimoyama T, Horie N, Kato T, Nasu D, Kaneko T. Tourette’s syndrome with rapid deterioration by self-mutilation of the upper lip. J Clin Pediatr Dent 2003;27:177-80. 12. Yoshikawa F, Takagi T, Fukayama H, Miwa Z, Umino M. Intravenous sedation and general anesthesia for a patient with Gilles de la Tourette’s syndrome undergoing dental treatment. Acta Anaesthesiol Scand 2002;46: 1279-80. 13. Lobbezoo F, Naeije M. Dental implications of some common movement disorders: a concise review. Arch. Oral Biol. 2007;52:395-8. 14. Porritt J, Marshman Z, Rodd HD. Understanding children’s dental anxiety and psychological approaches to its reduction. Int J Paediatr Dent 2012;22:397-405. 15. Browner NM, Frucht S. Movement disorders in dental practice. In: Lamster IB, Northridge ME, eds. Improving Oral Health for the Elderly: An Interdisciplinary Approach, New York: Springer; 2008:79-97. 16. Frucht SJ. Movement disorder emergencies in the perioperative period. Neurol. Clin. 2004;22:379-87.
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ARTICLE ABSTRACT Purpose: Dental treatment of patients with Tourette syndrome (TS) may present special challenges to the dentist. The aim was to systematically review the literature regarding perioperative procedures including sedation and general anaesthesia (GA) of patients with TS. Materials and methods: Literature searches were performed in PubMed and Embase to identify papers concerning TS in combination with dental treatment, sedation, and/or GA in order to study outcomes regarding co-morbidity, perioperative complications, and drug interactions. Results: The literature search identified six publications (case reports or series) which addressed the topic. No unexpected adverse effects or drug interactions in relation to sedation or GA in TS patients and no perioperative complications were reported. Conclusions: The literature on TS is scarce and the evidence level is low. Therefore, guidelines regarding the dental treatment of patients with TS cannot be formulated at the present time.
KEY WORDS: sedation, general anaesthesia, dentistry, Tourette syndrome
Tourette syndrome and procedures related to dental treatment: a systematic review Jonas Kjeldbjerg Hansen, MD;1* Pernille Endrup Jacobsen, DDS, PhD;2,3 Janne Lytoft Simonsen, MSc, PhD;4 Ole Hovgaard, DDS, PhD;5 Dorte Haubek, DDS, Dr. Odont6 1Consultant,
The Paediatric Department, Viborg Regional Hospital, Central Jutland, Denmark; Professor, Section for Paediatric Dentistry, Department of Dentistry, Aarhus University, Denmark; 3Department of Specialised Oral Health Care, Viborg Regional Hospital, Central Jutland, Denmark; 4Research Librarian, Aarhus University Library Health Sciences, Aarhus University, Denmark; 5 Chief dentist, Department of Specialised Oral Health Care, Viborg Regional Hospital, Central Jutland, Denmark; 6Professor, Section for Paediatric Dentistry, Department of Dentistry, Aarhus University, Denmark. *Corresponding author email: [email protected] 2Assistant
Spec Care Dentist 35(3): 99-104, 2015
Introd uct ion
Tourette syndrome (TS) is a neurodevelopmental disorder with a prevalence of up to 1%.1-5 It is defined by multiple motor tics and one or more phonic tics, not necessarily occurring concomitantly, but lasting for more than 1 year and with a debut before 18 years of age. Tics manifest as sudden, rapid, recurrent, non-rhythmic, stereotyped movements, or sounds.1-5 Tics usually have a waxing and waning course. Stress and anxiety will exacerbate tics.3,4
Although tics are the defining feature of TS, co-morbid psychiatric conditions occur in up to 90% of TS patients and often cause more morbidity than the tics themselves.1,2,4,5 Attention deficit hyperactivity disorder is the most common co-morbid diagnosis, followed by obsessive-compulsive disorder. Other co-morbid conditions include anxiety disorder, depression, explosive outbursts, and learning disorders.1-5 Self-injurious behavior2-5 and autism spectrum disorders5 have also been associated with TS. Tics and the co-morbid conditions frequently require medical treatment. Treatment of tics most commonly involves alfa-adrenergic agonists or antidopaminergic drugs. Other drugs, including anticonvulsant (topiramate), benzodiazepines, and botulinum toxin A © 2014 Special Care Dentistry Association and Wiley Periodicals, Inc. DOI: 10.1111/scd.12098
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may also be used. Attention deficit hyperactivity disorder is most often treated with stimulants (methylphenidate and dextroamphetamine), alternatively norepinephrine re-uptake inhibitor (atomoxetine). Obsessive-compulsive disorder may be treated with a selective serotonin re-uptake inhibitor, rarely an atypical neuroleptic.1,3,4 Patients with TS pose several challenges to the dentist. Tics themselves can be a hazard to the patient if they occur during a dental procedure, and both tics and some of the co-morbid conditions could be expected to be aggravated by the stress and anxiety associated with undergoing dental treatment. These are all factors which can complicate dental procedures and may entail a need for the use of sedation of patients with TS.
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Table 1. Search terms for the systematic literature search. PubMed
Embase
Tourette Syndrome (TS)
“Tourette Syndrome” [Mesh]
“gilles de la tourette syndrome”/exp
Dental treatment
Dental OR Dentist OR Dentists OR Tooth OR Teeth [Title/Abstract]
dental:ab OR dental:ti OR dentistry:ab OR dentistry:ti OR dentist:ab OR dentist:ti OR dentists:ab OR dentists:ti OR tooth:ab OR tooth:ti OR teeth:ab OR teeth:ti
Sedation or General anaesthesia (GA)
“Anesthesia” [Mesh] OR “Anesthesia Recovery Period” [Mesh] OR “Conscious Sedation” [Mesh] OR “Deep Sedation” [Mesh] OR “Anesthetics” [Mesh] OR “Postoperative Complications” [Mesh] OR “Perioperative Care” [Mesh] OR “Perioperative Period” [Mesh] NOT “Deep Brain Stimulation” [Mesh]
“anesthesia”/exp OR “anesthetic recovery”/exp OR “conscious sedation”/exp OR “deep sedation”/exp OR “anesthetic agent”/exp OR “postoperative complication”/exp OR “perioperative period”/exp NOT “brain depth stimulation”/exp
Search 1: Tourette Syndrome search AND Dental treatment search. Search 2: Tourette Syndrome search AND Sedation or general anaesthesia search.
Furthermore, possible interactions between the patient’s daily medication and drugs used in relation to the dental procedure must be taken into consideration.6 The purpose of this study was to systematically review this literature regarding the dental treatment of patients with TS, including sedation and GA and if possible provide clinical guidelines.
Mater ial and me tho ds
The study was performed as a systematic review of original publications reporting perioperative procedures, including the use of sedation, and/or GA in patients diagnosed with TS.
Search strategy The term “Tourette Syndrome” was searched for as a controlled search term. The search strategy was developed for PubMed and was revised appropriately for Embase to comply with differences in MeSH and Emtree terms. The MeSH and EMTREE terms were “exploded.” We conducted two separate searches. Search 1 focused on TS and dental treatment by combining “Tourette syndrome” with words like “dental” OR “teeth”, among others. Search 2 focused on TS in combination with sedation and GA by
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combining the search term “Tourette Syndrome” with MeSH terms like anaesthesia OR premedication (for detailed search strings see Table 1). Both databases were searched from 1980 to April 2014. Additionally, reference lists were hand-searched to identify the additional eligible studies. The eligibility criteria were clinical studies (follow-up, case-control, or crosssectional) or case reports involving humans of all ages that focused on the association between TS and complications in relation to dental treatment, sedation, and/or GA. Only papers written in English were included.
Selection of studies and data extraction The search produced 82 references, which were reduced to 70 references after screening for duplicates. Reports concerning perioperative complications in relation to sedation or GA were included whether they were combined with dental treatment or not. Subsequent screening of the title and the abstract identified 13 articles in which the term “Tourette Syndrome” was the diagnostic term applied by the authors of the paper, thereby making the publication relevant for this review. In case of disagreement, the abstract was discussed in plenum by
all authors until consensus as to the inclusion or exclusion was obtained. Reviews were excluded. Thirteen full-length articles were studied, and after excluding reviews, brief communications, etc., five publications, all case reports, were identified and formed the basis for the subsequent data extraction. One additional publication was identified by hand-searching the reference list of the included papers. The inclusion of publications is illustrated in Figure 1. The data extracted from the published reports reviewed are shown in Table 2 (demographics, various information, and the outcomes). It was impossible to pre-specify all the variables presented in Table 2 because of the heterogeneity of the information in the included studies. The data extraction form was revised by all authors to facilitate accuracy and clarity in the collection of data. The final data extraction form was used to evaluate all the included articles independently by two reviewers (JKH and PEJ).
R es ul t s Basic results In total, seven TS cases were identified in six studies.7-12 All the included studies
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Outcomes Dental treatment Of the seven cases described, four had oral or dental procedures performed. One underwent dental treatment because of caries,12 and three underwent oral or dental procedures because of self-mutilation. One patient had performed self-extraction of teeth,7 one presented with self-mutilation of the tongue,8 and one presented with self-mutilation of the upper lip,11 all reported under oral-facial problems in Table 2. In two of these cases, the oral or dental self-mutilation was the presenting symptom that eventually led to a diagnosis of TS.7,11
Sedation Sedation was used in four of the seven cases. Three were sedated with a benzodiazepine (midazolam, diazepam),10-12 and one with morphine sulphate in combination with droperidol.8 Of these four cases, one case received no daily medication,10 and three received haloperidol either as mono therapy (one case) or in combination with other drugs (two cases).8,11,12 No interactions or unexpected adverse effects were described regardless of the type of sedation or the use of daily medication. Benzodiazepines used for the sedation were found efficient in controlling tics,10,12 and in one case, it was specified that no delayed arousal or exacerbation was seen.12 Figure 1. Flow diagram illustrating the inclusion of literature.
were case reports or case series and involved patients living on three continents (Asia, Europe, and North America; Table 2). The year of publication varied by 20 years, with two papers from 1986,8,9 one from 2002,12 one from 2003,11 one from 2005,7 and one from 2006.10
Clinical presentation In one case, a positive family history of disorders predisposing to TS was reported.7 In three cases, no family his-
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tory was found,8,10,11 and in three cases the family history was not mentioned.9,12 Information on whether the diagnosis of TS had been made according to generally applied criteria was not given in any of the described cases. Co-morbidity was described in five cases, and included obsessive-compulsive disorder/behavior, hyperactivity, selfmutilation, speech problems, emotional outbursts, and emotional lability.7–9,11 In two cases, no co-morbid conditions were reported.10,12
General anaesthesia Five patients underwent GA in relation to the procedure or surgery performed,8–10,12 but no complications in relation to GA was reported. The anaesthetics used varied greatly (Table 2). Three of the five patients were on daily medication.8,9,12 Regardless of which combinations of daily medication the patients were on and which anaesthetic medications were used, no drug interactions were reported. One paper did not mention which anaesthetics were used.8
Perioperative complications No perioperative complications were reported in the four patients undergoing
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Turkey
Woman, 21 yrs, pregnant
No
No
No
Not described
Midazolam
Succinylcholine, Not described Propofol 50% nitrous oxide in oxygene Desflurane 3% Cisatracurium Mepridine Oxycontin (for uterine contraction) Metoclopramide (antiemetic) Diclofenac potassium
No
No
No
Country/State
Gender, age
Family history
Daily medication against tics or co-morbid condition
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Specification of the diagnostic criteria of TS
Co-morbidity
Sedation
General anesthesia
Drug interactions
Peri-operative complications
Oro-facial problem
Self-extraction of teeth
Not described
Not described
Not described
Obsessive-compulsive behavior Hyperactivity Self-mutilation
No
No
Mother: Tics in childhood Father: Attention and concentration problems in school age
Girl, 4 yrs
Sweden
Case report (1)
Case report (1)
Design (no. of cases described in the report)
Leksell & Edvardson 20057
Sener et al. 200510
Reference
Stomatitis, decayed teeth
No
No
Atropine sulphate Thiamyl Vecuronium 30% nitrous oxide and 0.6-1% sevoflurane in oxygen
Midazolam
Not described
No
Haloperidol Fluvoxamine Milnacipran hydrochloride
Not described
Boy, 13 yrs
Japan
Case report (1)
Yoshikawa et al. 200212
Self-mutilation of the tongue
Not described
No
The patient had general anesthesia, but no information regarding which drugs was used
Morphine sulphate Droperidol
Hyperactivity Speech problem Self-mutilation
No
Haloperidol
No
Boy, 16 yrs
USA/California
Case report (1)
Lowe 19868
Table 2. Summary of findings in seven Tourette Syndrome cases in six studies.
Self-mutilation of the upper lip
No operation performed
No
Not relevant
Diazepam
Obsessive-compulsive disorder Self-mutilation
No
Haloperidol Bromazepam Fulnitrazepam
No
Boy, 13 yrs
Japan
Case report (1)
Shimoyama et al. 200311
No
Case 1: No Case 2: No
Case 1 + 2: No
Case 1: Morphine sulphate, fentanyl, hydroxyzine pamoate, thiopental, d-tubocurarine, succinylcholine, nitrous oxide in oxygen, enflurane Droperidol Case 2: Thiopental, halothane, nitrous oxide in oxygen
Not used
Case 1: Emotional outburst Case 2: Hyperactivity, emotional lability
No
Case 1: Haloperidol and clonidine Case 2: No daily medication
Case 1: Not described Case 2: Not described
Case 1: Male, 28 years Case 2: Boy, 12 years
USA/Colorado
Case report (2)
Morrison & Lockhart 19869
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The specific anesthetic chosen is less important than the general approach to the patient, taken by the operating room team. Intensification of sedation may temporarily be effective in order to prevent further selfmutilation. Dental care should be planned as soon as possible when signs of oral tics occur. Metoclopramide, ondansetron, midazolam and opioids may be used safely for anesthesia in TS. Conclusions
Cooperation between the dentist and the child neurologist is recommended in order to allow early diagnosis and treatment of TS.
Sedation or GA as described efficiently prevented tics during the procedures.
Morphine sulphate and droperidol were used without complications.
Morrison & Lockhart 19869 Sener et al. 200510 Reference
Table 2. Contined.
Leksell & Edvardson 20057
Yoshikawa et al. 200212
Lowe 19868
Shimoyama et al. 200311
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oral or dental procedures or the three patients subjected to other types of surgery (caesarean delivery,10 retinal surgery, herniotomy9).
Di scus s ion
The present review revealed only a few studies regarding procedures related to the treatment of patients with TS in a clinical dental setting. Furthermore, the total number of reported cases was low. These factors weakened the possibility of providing general recommendations or guidelines regarding dental treatment of patients with TS. The present review was methodologically carried out as a systematic review, however, the low evidence of the existing literature, leads to a more descriptive reporting of the listed outcomes (Table 2). On the other hand, the sparse number of papers facilitated the opportunity to include all interesting findings and thereby revealing the importance of the dental teams to be aware of the early symptoms of TS. The studies were published over a time span of 20 years and originated from three different continents. Thus, a great variety of drugs and combinations of drugs had been used. Accordingly, the conclusions of this study should be generally applicable. The method followed in the present review was in accordance with the PRISMA statement, and thus aimed to secure a systematic selection and evaluation of all relevant material on the topic. Due to the low level of evidence of the papers identified, it was not relevant to use quality score assessment instruments as NOS or GRADE. Although the search strategy was developed for a specific database, the number of references identified in the GA search in Embase was three times larger than in PubMed. Embase has a more detailed pharmacological profile, and the search result contained a large number of publications with a medical approach, and thus not relevant for the purpose of this review. The majority of these publications were later excluded in the data selection. In the seven cases reported on in the present literature, it was not specified
whether the diagnosis of TS fulfilled the diagnostic criteria according to DSM-IV or ICD-10.7-12 How the diagnosis was reached in each case was described in variable detail. However, based on a thorough review of the presented cases, it is reasonable to expect that the cases identified in the case reports correspond to the focus of this study.
Dental treatment Self-mutilation is a common co-morbid feature in patients with TS. The case reports by Lowe8 and Shimoyama et al.11 illustrate the risk of severe injury to the tongue or lip caused by biting during tics. In both cases, a splint was recommended to prevent further injury because the pain itself can have a self-reinforcing effect on the tics. The report by Leksel and Edvardson7 describes a 4-year old girl with unexplainable toothache. The dentition of the child was caries-free, but after a few consultations the primary canines suddenly showed extreme mobility. In this case, the dentist was the initial healthcare professional to observe clinical symptoms of TS, and the dentist’s referral to a neurologist resulted in a confirmed diagnosis of TS. The toothache was caused by extreme grinding during tics with tooth mobility and muscle tenderness as a result. As self-mutilation is a known co-morbid feature of TS2-5 and from the findings in this study, we would recommend that a diagnosis of TS should be considered and evaluated in patients presenting with dental or oral self-mutilation. In relation to TS patients, the dentist could be a relevant professional to include in the interdisciplinary diagnostic team. Tics occurring during dental or oral procedures can be a hazard to the patient. Tics themselves can be exacerbated by stress and anxiety,3,4,13 which could be elicited by the planned dental procedure(s). It has been recommended that time should be set aside for the anaesthesiologist to make a pre-anaesthetic consultation with the patient and his family prior to GA to reduce the patient’s anxiety, and thus reduce possible exacerbations of the patient’s motor symptoms.9 The study by Morrison and Lockhart9 specifically addressed pre-anaesthetic anxiety
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r eduction in patients with TS. This recommendation could also be applicable to the normal clinical dental setting where anxiety most commonly is reduced by thorough information and the acceptance of the procedures carried out. However, an individual approach is always necessary to identify the factors provoking the anxiety.14 On the basis of the reports in the literature and thus of the present review, it is not possible to provide general recommendations regarding stress reducing procedures in TS patients in a clinical dental setting with or without sedation or under GA.
Sedation Sedation may be necessary in order to reduce tics during the procedure. Benzodiazepines were used in three cases and appeared to be both safe and efficient in reducing tics.10-12 Morphine sulphate in combination with droperidol was used in one case with sedative effect and without adverse effects.8 The review shows that benzodiazepines are the most up-to-date and widely used drugs,10-12 and may be used to obtain sedation for dental or oral procedures in patients with TS. However, the data are too sparse to provide exact knowledge of the efficacy and safety in this setting.
General anaesthesia In five cases, GA was used.8–10,12 In all cases, tics were efficiently inhibited. No adverse effects or drug interactions were reported. However, it was not possible to compare the cases, because of differences in the anaesthetics used, and because some patients were on daily medications and some were not, and in some cases the anaesthesia was combined with sedation. Therefore, it is not possible to provide recommendations regarding GA in patients with TS undergoing dental treatment. In contrast, we cannot confirm the concerns stated by Friedlander and Cummings regarding interactions between the patient’s daily medication and the drugs used for conscious sedation or GA.6 In addition to tics, up to 90% of patients with TS suffer from co-morbid disorders.1,4 Co-morbidity was described in five cases, but its possible impact on the management of the patient was not described in any of the cases.7–9,11
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Therefore, many questions regarding dental treatment of patients with TS cannot be answered based on the published reports at present. Studies specifically addressing co-morbid conditions related to TS may provide useful information regarding handling of TS patients in a clinical dental setting, but this was beyond the scope of this study.
Co nclus ion
In conclusion, the literature on TS and its impact on procedures in dental treatment are too sparse at present to provide general recommendations on the dental treatment of TS patients. Based on the findings of the present review and general knowledge on TS, it seems appropriate to suggest a reduction of the patient’s anxiety prior to and during the procedures carried out. The findings concerning sedation and GA are too limited to enable formulation of general guidelines concerning the use of sedatives or anaesthetics, including their safety and efficacy in patients with TS. Therefore, a thorough review of the patient’s daily medication and a consideration of possible interactions with medications planned for sedation and local or general anaesthesia should be done in each case. Original research reports are warranted to elucidate the unanswered questions regarding the treatment of patients with TS in a clinical dental setting. Previous studies have addressed general aspects of movement disorders in the dental setting,15,16 however, this was not the aim of the present systematic review. It is recommended that patients presenting to the dentist with oral or dental self-mutilation should be referred to a relevant specialist for further evaluation of possible TS.
Co nf lict s of Int er es t None to declare.
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2. Cavanna AE, Servo S, Monaco F, Robertson MM. The behavioral spectrum of Gilles de la Tourette syndrome. J Neuropsychiatry Clin Neurosci 2009;21:13-23. 3. Jankovic J, Kurlan R. Tourette syndrome: evolving concepts. Mov Disord 2011;26: 1149-56. 4. McNaught KS, Mink JW. Advances in understanding and treatment of Tourette syndrome. Nat Rev Neurol 2011;7:667-76. 5. Zinner SH, Coffey BJ. Developmental and behavioral disorders grown up: Tourette’s disorder. J Dev Behav Pediatr 2009;30:560-73. 6. Friedlander AH, Cummings JL. Dental treatment of patients with Gilles de la Tourette’s syndrome. Oral Surg, Oral Med, Oral Pathol. 1992;73:299-303. 7. Leksell E, Edvardson S. A case of Tourette syndrome presenting with oral self-injurious behaviour. Int J Paediatr Dent 2005;15:370-4. 8. Lowe O. Tourette’s syndrome: management of oral complications. ASDC J Dent Child 1986;53:456-60. 9. Morrison JE Jr, Lockhart CH. Tourette syndrome: anesthetic implications. Anesth Analg 1986;65:200-2. 10. Sener EB, Kocamanoglu S, Ustun E, Tur A. Anesthetic management for cesarean delivery in a woman with Gilles de la Tourette’s syndrome. Int J Obstet Anesth 2006;15:163-5. 11. Shimoyama T, Horie N, Kato T, Nasu D, Kaneko T. Tourette’s syndrome with rapid deterioration by self-mutilation of the upper lip. J Clin Pediatr Dent 2003;27:177-80. 12. Yoshikawa F, Takagi T, Fukayama H, Miwa Z, Umino M. Intravenous sedation and general anesthesia for a patient with Gilles de la Tourette’s syndrome undergoing dental treatment. Acta Anaesthesiol Scand 2002;46: 1279-80. 13. Lobbezoo F, Naeije M. Dental implications of some common movement disorders: a concise review. Arch. Oral Biol. 2007;52:395-8. 14. Porritt J, Marshman Z, Rodd HD. Understanding children’s dental anxiety and psychological approaches to its reduction. Int J Paediatr Dent 2012;22:397-405. 15. Browner NM, Frucht S. Movement disorders in dental practice. In: Lamster IB, Northridge ME, eds. Improving Oral Health for the Elderly: An Interdisciplinary Approach, New York: Springer; 2008:79-97. 16. Frucht SJ. Movement disorder emergencies in the perioperative period. Neurol. Clin. 2004;22:379-87.
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