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Med Oral Patol Oral Cir Bucal-AHEAD OF PRINT Oral findings in Rett syndrome Journal section: Odontostomatology for the disabled or special patientsPublication Types: Review Oral findings in Rett syndrome: A systematic review of the dental literature
María-Cristina Fuertes-González 1, Francisco-Javier Silvestre 2, José-Manuel Almerich-Silla 3
1 Staff dentist of the Red Cross Dental Clinic for Special Patients, Valencia. Professor of the Master in Hospital Odontology and Special Patients2 Assistant Professor of Odontology in Special Patients. Department of Stomatology, University of Valencia. Director of the Master in Hospital Odontology and Special Patients3 Assistant Professor of Preventive and Community Care Odontology. Department of Stomatology, University of Valencia (Spain) Correspondence:Departament d’EstomatologiaClinica OdontològicaUniversitat de ValènciaGascó Oliag 146010 – Valencia (Spain)[email protected] ------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------ Abstract
Rett syndrome (RS) is a chromosome X-linked genetic neurological disorder characterized by de-velopmental
regression, particularly in relation to expressive language and use of the hands, together with profound mental
retardation, that almost exclusively affects females. The present review describes the 7 cases of RS published
in the indexed literature (Medline) – the first corresponding to 1985 and the last to the year 2007. Certain oral
manifestations of the disease are derived from the drug treatment prescribed to control the disease, while others
are common to other clinical conditions characterized by convulsion activity, difficulties for correct oral hygiene,
walking problems and/or an excess of oral / digital-manual habits. In any case, bruxism is the oral habit most fre-
quently associated with RS – the treatment of which remains the subject of controversy.
Key words: Rett syndrome, oral manifestations, bruxism, review.
describing the clinical features of the syndrome was Rett syndrome (RS) is a neurological development dis- published by Hagberg et al. in 1983 (3).
order characterized by the regression of psychomotor RS almost exclusively affects females, since it involves development with autistic manifestations, decelerated a dominant X chromosome-linked mutation (MECP2 growth of the head, seizures, and stereotyped repeti- gene, region Xq28) (4). Although initially regarded as tive movements of the hands (1). Although initially de- a neurodegenerative disease, it is now known that RS scribed by Rett in 1966 (2), the compilation of 35 cases Med Oral Patol Oral Cir Bucal-AHEAD OF PRINT Oral findings in Rett syndrome Table 1. Reviewed diagnostic criteria in Rett syndrome (6).
Required criteria
An apparently normal pre- and perinatal period. Mostly normal psychomotor development up to 6 months of age. Loss of manual functional skills at age 0.5-2.5 years. Stereotypic movements of the hands, such as clenching, applause, washing movements or taking the hands to the mouth. Social retraction, communication dysfunction, forgetting of learned words, and cognitive disability. Gait apraxia or failure to learn to walk. Supporting criteria
Respiratory dysfunction: apneas, hyperventilation, sharp expulsion of air and saliva, aerophagia. Abnormal muscle tone associated to dystonia Small, hypotrophic and cold feet; small and thin hands. Exclusion criteria
Enlarged internal organs or other signs of storage disease. Retinopathy, optic atrophy or cataracts. Evidence of peri- or postnatal brain damage. Presence of identifiable metabolic disorders or progressive neurological conditions. Development of neurological disorders as a result of serious infections or head injuries. The diagnosis of classical RS is based on a series of clinical criteria (table 1) (6), since MECP2 gene muta- Oral manifestations of RS have been only briefly de- tions alone are not equivalent to a diagnosis of RS, and scribed in the medical literature, and the dental litera- can also be seen in other conditions such as autism, for ture offers few articles on the oral pathology of these patients. The present study offers a systematic review A study of RS in the Spanish population published in of the literature on the oral manifestations of this rare 1999 (7) documented a total of 207 cases, though the true figure must be greater, since the epidemiologi-cal studies made in different countries yield a preva- Search Method
lence of between 1/12,000 (0.65/10,000) and 1/15,000 A Medline/PubMed search (http://www.ncbi.nlm.nih.
gov/PubMed) of the literature on the subject published Girls with RS are born apparently healthy and with nor- up until 2009 november has been made, based on the mal psychomotor development until the first or second following keywords: Rett syndrome AND oral manifes- year of life, when brain functional regression begins, tation OR dental OR bruxism. The articles were exam- with a loss of manual motor skills and the appearance of ined on an independent basis by two reviewers (CFG, stereotyped movements, gait apraxia, loss of verbal and FJS). The inclusion criteria were: articles in English nonverbal communication skills, and the association of generated by the Medline database, contributing at least episodes of hyperventilation, aerophagia and epileptic one clinical case with oral manifestations, and describ- ing the buccodental characteristics of the disorder and There is no specific treatment for RS, apart from physi- the dental treatment provided. Of the 363 articles found, cal and occupational therapies to develop and maintain muscle tone and function, together with a range of drugs Med Oral Patol Oral Cir Bucal-AHEAD OF PRINT Oral findings in Rett syndrome time of the exploration, 6 of the 17 children presented The first article on RS published in the dental litera- deciduous dentition, 10 mixed dentition and one perma- ture (10) described the case of a 12-year-old girl seen for nent dentition. As regards the oral habits, all patients endodontic treatment of the upper central incisors fol- presented digital-manual sucking or nibbling activities, lowing damage as the result of a fall in the course of an 82% suffered bruxism, 41% showed oral breathing, 29% epileptic attack. The patient had previously undergone exhibited tongue thrusting, 29% suffered dribbling, and dental treatment under general anesthesia. In this case 12% showed lateralization of the mandible. In relation dental treatment was likewise carried out under general to the oral manifestations, gingivitis with important anesthesia with nasotracheal intubation. This was fol- plaque accumulation was observed in all the patients, lowed by dental revision every 6 months.
probably as a result of parent difficulties in maintaining Pagnacco et al. (11) in turn presented the case of a adequate oral hygiene in the children. Non-physiologi- 5-year-old girl with caries of the deciduous dentition, cal dental wear was noted in 71% of the cases, and was dental wear and abundant sialorrhea. General anesthe- more common in the anterior deciduous teeth, though sia was used for dental treatment, due to the impossibil- in contrast only 2.7% of the explored dental surfaces ity of providing care on an outpatient basis.
showed caries. A high-arched palate was present in 53% Buccino et al. (9) reported the case of a 4-year-old girl of the girls, and a high prevalence of open bite was noted presenting stereotyped movements of the hands, hyper- (80%) – this possibly being related to the digital-manual ventilation, autistic behavior and motor development sucking and oral breathing. However, the authors of the problems. There were no particular oral features apart mentioned series concluded that with the exception of from a high-arched palate and the consequences of the digital-manual sucking or nibbling activities, there bruxism, with generalized wear of the deciduous denti- were no pathognomonic oral manifestations of RS. tion (particularly of the upper and lower anterior sector Nevertheless, certain characteristics did prove char- teeth). In this context, the right and left maxillary inci- acteristic, such as bruxism (dental wear), open bite, a sors showed class II mobility apparently related to the existing bruxism and chronic digital sucking habit. The The clinical case published by Alpoz et al. (14) in turn X-ray study in turn showed external root reabsorption corresponded to a 5-year-old girl with day-time brux- of both incisors, periodontal widening and signs of cal- ism as the sole oral manifestation. This habit resulted cium metamorphosis. A Molt mouth prop was used for in intense dental wear, with almost complete loss of dental exploration of the patient, who presented autis- the dental crown, though without pulp exposure. Ra- tic-type behavior with episodes of eye contact with the diographically, enlargement of the temporomandibular dental surgeon. The prevention of oral disease was the joint space was noted, probably as a result of the brux- only recommendation made, establishing the control of ism. The patient was subjected to nitrous oxide seda- bruxism and traumatisms, improving oral hygiene with tion, and alginate impressions were obtained in order to the help of the parents, the daily application of topical prepare a soft splint for the upper jaw, to be worn only fluor gel or rinses, and frequent follow-up visits, in or- when awake and controlled by the parents, since the der to avoid the need for sedation or general anesthesia patient did not suffer nocturnal bruxism. This was the only treatment of choice, since it proved impossible to Peak et al. (12) published the case of a 5-year-old girl apply other options such as management of the occlusal with RS who presented important wearing of the de- interferences or the placement of stainless steel crowns ciduous dentition, as a result of constant bruxism. At (due to scant retention of the deciduous molars).
extraoral level she presented bilateral masseter muscle Magalhaes et al. (15) studied 13 patients with a mean hypertrophy, while at intraoral level the deciduous in- age of 9 years, and with daytime bruxism as the sole cisors and canines showed wear. The proposed dental oral manifestation in all but two cases. This habit result- management coincides with the techniques used in ap- ed in intense dental wear, and according to the mothers plication to the disorders seen in autism: the establish- was associated with increased stereotypic movements ment of a routine, minimization of the patient waiting of the hands. Clenching began by opening of the mouth, time before entering the dental office, and the avoid- followed by mandibular thrusting and closing of the ance of noise that might frighten the patient during the mouth. During bruxism, the tongue was projected to- exploration. Likewise, due to the difficulty of providing wards the anterior teeth, giving rise to anterior open dental treatment under local anesthesia, the use of an- bite in the older patients, in addition to excessive den- algesia, sedation and even general anesthesia is recom- tal wear and muscle dysfunction. For the treatment of bruxism, the authors prepared modified splints placed The largest RS patient series presented in one same in the upper jaw so that the cuspids of the deciduous study in the literature comprised 17 cases (13), with a lower molars showed centric occlusion with the splint. mean age of 7.33 years (range 2.7-12.7 years). At the The margin of the splint extended to the hard palate, Med Oral Patol Oral Cir Bucal-AHEAD OF PRINT Oral findings in Rett syndrome and the use of denture adhesive was recommended to manifestations derived from the specific conditions of increase retention. A groove measuring 5 mm in width and 1.5 mm in depth was made in the occlusal surface The drug-related (anxiolytic and anticonvulsivant) of the splint, and was filled with soft and resilient mate- orofacial manifestations of RS are the following (16): rial, in order to avoid anterior mandibular movement. xerostomia, stomatitis, glossitis, erythema multiforme, This treatment in turn was combined with acupuncture sialorrhea, dysgeusia, gingivitis, parotid gland swelling, (reported to be effective in treating temporomandibular periodontal abscesses, sinusitis, dysphagia and tongue dysfunction and facial pain) performed on a weekly ba- paralysis. However, these manifestations are very dif- sis for an average of three years and four months. The ficult to evaluate in patients with RS, unless they prove parents reported that bruxism did not reappear until 7 evident in the course of the clinical exploration. This days after the acupuncture sessions, and was moreo- is particularly due to the communication problem, the ver of lessened intensity. This combination of modified lack of language in most cases, and a heightened pain splints and acupuncture for the treatment of bruxism threshold that can mask the presence of pain-producing proved effective in three patients with RS. Likewise, for conditions. On the other hand, consideration is required dental treatment, the authors suggested brief visits, the of the possible interactions between the anxiolytic and avoidance of physical restraints, and the use of mouth anticonvulsivant medication and analgesic drugs widely props – since the patients had little or no muscle con- used in dental practice, such as paracetamol, which at high doses and over the long term lessens the effect of Among the RS studies published to date, we identified lamotrigine. Likewise, acetylsalicylic acid (aspirin) can 5 articles involving a single clinical case (9-15), two ar- inhibit the metabolism of valproic acid, which in turn ticles respectively reporting samples of 17 (13) and 13 increases the bleeding tendency associated with the patients (15), and another article on the orofacial effects administration of aspirin and other nonsteroidal antiin- and possible interactions of the drugs used to treat the flammatory drugs. The use of antibiotics must also be taken into considera-tion. In this sense, carbamazepine The mean age of the patients described in the reviewed accelerates the metabolism of doxycycline, and its own clinical cases and series (9-15) was almost 7 years (spe- metabo-lism can be inhibited by erythromycin and clar- cifically 6.8 years), with a range of 2.7-21 years. In the ithromycin. Therefore, a detailed clinical history must reviewed dental literature, only 35 children with this be compiled, including all the drugs used by the patient rare syndrome were subjected to oral exploration – this for control of the disease, with a view to avoiding pos- sample being too small to draw firm conclusions regard- ing the degree of oral pathology and the problems posed The orofacial manifestations and habits of patients with RS found in the literature are described in (Table 2). Evaluation of these oral manifestations indicates that the Discussion
oral findings in RS are not exclusive of the syndrome, Two groups of oral manifestations of Rett syndrome since they can also be seen in other diseases character- (RS) can be distinguished: those derived from the med- ized by the presence of excessive oral / digital-manual ication used to treat the disease on one hand, and the habits, walking problems, seizures, and difficulties for Table 2. Oral manifestations of Rett syndrome found in the dental literature.
Oral manifestations
High-arched palate, bruxism, digit sucking Digital-manual sucking / nibbling, bruxism, oral breathing, tongue thrusting, dribbling, mandibular lateralization, gingivitis, caries, Med Oral Patol Oral Cir Bucal-AHEAD OF PRINT Oral findings in Rett syndrome In conclusion, the present review shows that the delete-rious digital-oral habits and orofacial motor functional disorders of these patients must be taken into account, since in the early stages of the disease they can give rise to malocclusions such as anterior open bite and a high-arched palate. However, the most characteristic clinical feature of RS is bruxism, of an uncertain etiopathogen-esis, and difficult to treat (Fig. 1).
1. Chahrour M, Zoghbi HY. The story of Rett syndrome: from clinic to
neurobiology. Neuron. 2007; 56:422-37.
2. Rett A. On a unusual brain atrophy syndrome in hyperammonemia
in childhood. Wien Med Wochenschr. 1966;116:723-6.
Fig. 1. Clinical characteristics in Rett síndrome: Gingivitis, bruxism
3. Hagberg B, Aicardi J, Dias K, Ramos O. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett’s syndrome: report of 35 cases. Ann Neurol. 1983;14:471-9.
4. Raizis AM, Saleem M, MacKay R, George PM. Spectrum of MECP2 mutations in New Zealand Rett Syndrome patients. N Z Med correct oral hygiene (9,12,13). Nevertheless, there are some features, such as bruxism, anterior open bite, a 5. Guy J, Gan J, Selfridge J, Cobb S, Bird A. Reversal of neurological de-fects in a mouse model of Rett syndrome. Science. 2007;315:1143-7.
high-arched palate and gingivitis, that are more prev- 6. Hagberg B, Hanefeld F, Percy A, Skjeldal O. An update on clini- alent in RS. Although bruxism is the oral habit most cally applicable diagnostic criteria in Rett syndrome. Comments to frequently associated with RS (9,11-16), its treatment Rett Syndrome Clinical Criteria Consensus Panel Satellite to Euro- remains the subject of controversy. Some authors con- pean Paediatric Neurology Society Meeting, Baden Baden, Germany, 11 September 2001. Eur J Paediatr Neurol. 2002;6:293-7.
traindicate the use of splints in RS due to the important 7. Pineda M, Aracil A, Vernet A, Espada M, Cobo E, Arteaga R et mental retardation of the affected patients, their defi- al. Rett´s syndrome in the Spanish population. Rev Neurol. 1999; cient oral motor control and excessive oral-digital habits (9). In turn, Peak et al. (12) consider that splints should 8. Budden SS, Dorsey HC, Steiner RD. Clinical profile of a male with Rett syndrome. Brain Dev. 2005; 27 Suppl 1:S69-S71.
not be used due to the young age of the patients, who 9. Buccino MA, Weddell JA. Rett syndrome: a rare and often misdiag- still present deciduous dentition. On the other hand, Al- nosed syndrome: case report. Pediatr Dent. 1989;11:151-7.
poz et al. (14) have treated bruxism with soft splints, 10. Di Bona MC. Hospital dentistry for a child with Rett’s syndrome. and special designs have even been developed in order Spec Care Dentist. 1985;5:62-3. 11. Pagnacco A, Ferrara M, Vangelisti R, Randon C.Rett syndome: a to avoid the anterior mandibular movement that accom- “new” and rare disease. A clinical case. Dent Cadmos. 1988;56:93-5.
panies bruxism, combining such splints with acupunc- 12. Peak J, Eveson JW, Scully C. Oral manifestations of Rett’s syn- ture (15) - though the success obtained is limited. The literature describes the use of mouth props to se- 13. Ribeiro RA, Romano AR, Birman EG, Mayer MP. Oral manifesta-tions in Rett syndrome: a study of 17 cases. Pediatr Dent. 1997;19:349- cure a degree of stability and oral opening, in order to facilitate dental exploration and/or treatment (9,15). As 14.Alpoz AR, Ergul N, Oncag O. Bruxism in Rett syndrome: a case regards behavioral management, some authors (12) have report. J Clin Pediatr Dent.1999;23:161-3.
suggested the use of certain techniques used in autism 15. Magalhâes MH, Kawamura JY, Araújo LC. General and oral char-acteristics in Rett syndrome. Spec Care Dentist. 2002;22:147-50.
(routines and the avoidance of noise). In this context, 16. Friedlander AH, Yagiela JA, Paterno VI, Mahler ME. The patho- consideration is required of the important alteration physiology, medical management and dental implications of fragile X, and limitation in patient communication capacity, the Rett, and Prader- Willi syndromes. J Calif Dent Assoc. 2003;31:693- profound mental retardation, and lack of attention. As 702.
17. Escribano Hernández A, Hernández Corral T, Ruiz-Martín E, Por- a result, the dental management techniques must be teros Sánchez JA. Results of a dental care protocol for mentally handi- adapted to each individual case. Some authors have capped patients set in a primary health care area in Spain. Med Oral used sedation with nitrous oxide (14), while others com- Patol Oral Cir Bucal. 2007;12:E492-5.
bine it with sublingual midazolam (16). In turn, some investigators use general anesthesia (10,11), due to the medical risk of the patient and the lack of cooperation needed for correct dental treatment (17). In any case, all authors agree that prevention, with diet control, the application of fluor and frequent revisions are crucial for the management of these patients, and can limit the use of sedation and general anesthesia to only the most extreme cases.


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