Disease progression in blepharospasm: a 5-year longitudinal study.

BACKGROUND AND PURPOSE: The clinical manifestation of dystonic spasms in blepharospasm (BSP) patients may be heterogeneous. Whether the varying phenomenology of eyelid spasms becomes manifest sequentially during the course of the disease or aggregates in separate clusters according to different disease courses is still unclear. For this purpose, the clinical features in BSP patients were evaluated longitudinally over a 5-year period and also the blink reflex recovery cycle was tested in a subgroup of BSP patients. METHODS: Sixty BSP patients were videotaped at time 0 and after approximately 5 years of follow-up. Two experts in movement disorders, who were blinded to the video order, reviewed the videotapes and scored the severity of BSP using the Blepharospasm Severity Rating Scale. Changes in the R2 recovery index were also evaluated in 18 patients twice, i.e. upon enrolment and at the follow-up. RESULTS: The severity of BSP worsened significantly over the 5-year follow-up period owing to the appearance or the increased duration and frequency of prolonged spasms. It was also found that the blink reflex recovery cycle worsened at follow-up in comparison with the baseline. CONCLUSIONS: This study shows that the disease progression of BSP is characterized by the appearance or worsening of prolonged spasms. Prolonged spasms are accompanied by changes in the excitability of brainstem interneurons. Aging-related effects may exacerbate the pathophysiological mechanisms underlying spasms.

Re-emergent tremor in Parkinson's disease: the effect of dopaminergic treatment.

BACKGROUND AND PURPOSE: Patients with Parkinson's disease (PD) with resting tremor may be affected by a tremor that appears after a varying latency while a posture is maintained, a phenomenon referred to as re-emergent tremor (RET). The aim of the study was to evaluate the occurrence and clinical features of RET in patients with PD tested off and on treatment, and to compare the effect of dopaminergic treatment on RET with the effect on resting and action tremor. METHODS: We consecutively enrolled 100 patients with PD. Patients were clinically evaluated 24 h after withdrawal of therapy (off-treatment phase) and 60 min after therapy administration (on-treatment phase). We collected the demographic and clinical data of patients with PD. The severity of the disease was assessed by means of the Hoehn and Yahr scale and Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale part III. We evaluated the latency, severity and body side affected both off and on treatment in patients with RET. RESULTS: Re-emergent tremor was present in 24% of the patients with PD off treatment and in 19% of the patients on treatment. Dopaminergic treatment reduced the clinical severity of RET. Dopaminergic treatment increased the number of patients with unilateral RET and reduced the number of those who had bilateral RET. RET and resting tremor responded similarly to dopaminergic treatment, whereas action tremor was less responsive. Patients with RET had milder motor symptoms than patients without RET both off and on treatment. CONCLUSIONS: Dopaminergic treatment modified RET occurrence, severity and body distribution. Dopaminergic depletion plays a role in the pathophysiology of RET.

Spread of dystonia in patients with idiopathic adult-onset laryngeal dystonia.

BACKGROUND AND PURPOSE: Adult-onset laryngeal dystonia (LD) can be isolated or can be associated with dystonia in other body parts. Combined forms can be segmental at the onset or can result from dystonia spread to or from the larynx. The aim of this study was to identify the main clinical and demographic features of adult-onset idiopathic LD in an Italian population with special focus on dystonia spread. METHODS: Data were obtained from the Italian Dystonia Registry (IDR) produced by 37 Italian institutions. Clinical and demographic data of 71 patients with idiopathic adult-onset LD were extracted from a pool of 1131 subjects included in the IDR. RESULTS: Fifty of 71 patients presented a laryngeal focal onset; the remaining subjects had onset in other body regions and later laryngeal spread. The two groups did not show significant differences of demographic features. 32% of patients with laryngeal onset reported spread to contiguous body regions afterwards and in most cases (12 of 16 subjects) dystonia started to spread within 1 year from the onset. LD patients who remained focal and those who had dystonia spread did not show other differences. CONCLUSIONS: Data from IDR show that dystonic patients with focal laryngeal onset will present spread in almost one-third of cases. Spread from the larynx occurs early and is directed to contiguous body regions showing similarities with clinical progression of blepharospasm. This study gives a new accurate description of LD phenomenology that may contribute to improving the comprehension of dystonia pathophysiology.

Correction to: The Italian Dystonia Registry: rationale, design and preliminary findings.

In the original article, Gina Ferrazzano was affiliated to Department of Neurology and Psychiatry, Neuromed Institute IRCCS, Sapienza University of Rome, Pozzilli, Italy.The corrected affiliation should be: Neuromed Institute IRCCS, Pozzilli, IS, Italy.

Dental Sealants. Part 2: Who should get dental sealants and when.

BACKGROUND: Despite the remarkable achievements in recent decades in oral prevention, sealants are still underused even if their efficacy is well documented and clinical practice guidelines are available in almost every developed country. The clinician should know who needs dental sealants and when, with particular focus on high risk patients and pre-school children.

Dental Sealants Part 4: Bisphenol A: What dentists should know.

BACKGROUND: Bisphenol A (BPA) is a synthetic chemical resin used worldwide to produce plastic products. It is also a component of the bisphenol A diglycidylether methacrylate (Bis-GMA), which is a monomer found in dental resin-based materials (including resin-based dental sealants, RBSs). The controversy about its possible toxicity begins around the early '30s. Even if the amount of BPA released by dental sealants is well below the limit proposed by the U.S. Environmental Protection Agency and the European Food Safety Authority, we can reduce the risk of exposure, particularly for children, following precautionary measures.

Paediatric oral surgery: endoscopic approach in ameloblastic fibroma management. A preliminary report.

AIM: Ameloblastic fibroma (AF) is a rare benign odontogenic tumor of slow-growing behaviour characterised by proliferation of both epithelial and mesenchymal odontogenic tissues. Treatment of AF is usually conservative by means of enucleation, curettage and long term follow-up. CASE REPORT: This report describes a case of AF in a 12-year-old boy. Examination of the oral cavity revealed absence of tooth 3.7 and mild swelling in the same mandibular area. Orthopantomography and Cone Beam Computed Tomography (CBCT) showed a wide multilocular radiolucent lesion at the left mandibular angle, extending from the first molar to the lower part of the mandibular branch; agenesis of tooth 3.7 and displacement of tooth 3.8. Surgical excision was performed under general anaesthesia. A fiberscope was used to perform a more conservative bone removal and extraction of tooth 3.8. Furthermore, an accurate curettage of the bone site was performed. The histological investigation showed an AF pattern: an epithelial cell component arranged in nests and tubules immersed in a stroma of low differentiated mesenchymal tissue. No sign of recurrence has been observed during the 6-month follow-up period. CONCLUSION: Although AF is a rare tumor, it is more prevalent in children's jaw. A new intra-operative visual technique could provide a conservative treatment by minimising bone deformities and permitting an accurate bone curettage.

Is there an association between various aspects of oral health in Southern Italy children? An epidemiological study assessing dental decays, periodontal status, malocclusions and temporomandibular joint function.

AIM: To describe the current oral health status of primary school children in Southern Italy, to investigate if there is an association between malocclusions and temporomandibular disorders (TMDs), and to investigate if there is an association between dental caries and periodontal disease. MATERIALS AND METHODS: Study design: Transversal epidemiological study. We examined 1086 children, considering them of the same socioeconomic status (medium). The Decayed, Missing, Filled (DMF) index and the Community Periodontal Index (CPI) were used to assess decay and periodontal status. Moreover, orthodontic and gnathologic evaluations were performed. RESULTS: Children had a mean age of 10.3±0.72 yrs, 41.6% had active dental caries in permanent teeth and 54.8% experienced periodontal problems. The 54.3% of patients had Angle Class I, 40.1% Class II and 5.5% Class III. The 13.8% of children had a deviated opening pattern of the mandible, and 2.2% of them had TMJ pain. DMF indices greater than 0 were associated with positive CPI. Males were more susceptible to periodontal disease, compared to females. TMJ pain was found associated with a decreased overbite. STATISTICS: Descriptive statistics for continuous data, and frequencies and percentages for categorical and ordinal data were calculated. Univariate linear and logistic regression model, with ? calculations, was used to assess associations between dental caries status and CPI, and between malocclusions and gnathologic aspects. CONCLUSIONS: This study found a noticeable prevalence of oral diseases among children of Southern Italy and an association between malocclusions and TMDs, periodontal disease and dental decay. Thus, a higher number of preventive interventions are recommended in the area.

The Italian Dystonia Registry: rationale, design and preliminary findings.

The Italian Dystonia Registry is a multicenter data collection system that will prospectively assess the phenomenology and natural history of adult-onset dystonia and will serve as a basis for future etiological, pathophysiological and therapeutic studies. In the first 6 months of activity, 20 movement disorders Italian centres have adhered to the registry and 664 patients have been recruited. Baseline historical information from this cohort provides the first general overview of adult-onset dystonia in Italy. The cohort was characterized by a lower education level than the Italian population, and most patients were employed as artisans, builders, farmers, or unskilled workers. The clinical features of our sample confirmed the peculiar characteristics of adult-onset dystonia, i.e. gender preference, peak age at onset in the sixth decade, predominance of cervical dystonia and blepharospasm over the other focal dystonias, and a tendency to spread to adjacent body parts, The sample also confirmed the association between eye symptoms and blepharospasm, whereas no clear association emerged between extracranial injury and dystonia in a body site. Adult-onset dystonia patients and the Italian population shared similar burden of arterial hypertension, type 2 diabetes, coronary heart disease, dyslipidemia, and hypothyroidism, while hyperthyroidism was more frequent in the dystonia population. Geographic stratification of the study population yielded no major difference in the most clinical and phenomenological features of dystonia. Analysis of baseline information from recruited patients indicates that the Italian Dystonia Registry may be a useful tool to capture the real world clinical practice of physicians that visit dystonia patients.

Multidisciplinary approach to the treatment of double bilateral upper permanent incisors in a young boy.

BACKGROUND: A double tooth is a rare developmental anomaly referring to the fusion of two adjacent tooth buds or the gemination of a single bud. CASE REPORT: This case report describes the multidisciplinary approach to an 11-year-old patient with two double upper permanent incisors. The clinical intraoral examination showed a mixed dentition with bilateral double maxillary central incisors, molar Class I malocclusion and palatal ectopy of two lateral upper incisors. Computed tomography of the upper dental arch revealed the presence of double central incisors with two distinct roots. The clinical choice consisted of an innovative treatment including surgical, endodontic, orthodontic and restorative treatments. This management protocol produced good aesthetic, healthy and functional results that were stable also two years post-treatment.

In vitro performance of ultrasound enamel preparation compared with classical bur preparation on pit and fissure sealing.

AIM: Fissure sealants are effective in preventing caries. The aim of this in vitro study was to evaluate the effects of two different enamel surface preparation techniques for pit and fissure sealing. MATERIALS AND METHODS: Sixty extracted sound third molars were used. For each tooth, the mesial half of the occlusal fissures was treated with ultrasound diamond tip T1 mounted on an ultrasonic handpiece, while the distal half with conventional diamond bur. The teeth were randomly divided into 2 groups (n = 30/each). Group 1 samples were stored in distilled water at 4 °C. For group 2 samples, sealing of occlusal fissures was performed according to standard procedures. Bucco-lingual cuts parallel to the long axis of the tooth were made in order to separate the two different types of preparations. The effects of the executed procedures were assessed with SEM. RESULTS: Surfaces prepared with ultrasound system showed the presence of residual debris and appeared more irregular than surfaces prepared with traditional bur system. Furthermore, images showed the presence of cracks on the bottom and on the walls of the ultrasound prepared fissures. CONCLUSION: Conventional bur surface treatment showed a better performance when compared to ultrasound preparation and could probably ensure superior sealant retention.

An experimental in vivo procedure for the standardised assessment of sealants retention over time.

AIM: The elaboration of an experimental system to obtain reproducible and comparable photographs of the occlusal surface to monitor sealants retention. MATERIALS AND METHODS: An intraoral camera connected to a computer was used to obtain photos of the occlusal surfaces. A specific software was utilized to perform measurements on archived pictures. An experimental two-part system, consisting of a dental arch support and a camera support, connected to each other through holes and pins, was made to obtain a standardised and reproducible placement of the camera in the mouth. In the first part, to test the degree of reliability of the procedure and the percentage of image distortion, 120 first molars were sealed and for each molar ten photographs were taken, using the intraoral camera connected with the dental arch support, the camera support and the dedicated software. In the second part, 165 first molars were sealed and photographed, as above described, immediately after sealing (T0), 6 months (T1) and 1 year later (T2). With the software, the sealed areas were measured. The comparison of the selected sealed areas between T0 and T1, T0 and T2, T1 and T2 determined the percentage of sealant loss. RESULTS: In the first part, the experimental procedure showed a reliability of 96.85%. In the second part, the difference in the rate of lost sealant between T0-T1 and T1-T2 was statistically significant (p <0.001). STATISTICS: ANOVA analysis was made. CONCLUSIONS: Photographs, obtained through the experimental two-part system, allowing a reproducible positioning of the intraoral camera in oral cavity, could represent a standardised and useful method to monitor sealants retention over time.

New approach in paediatric dentistry: ultrasonic nondestructive evaluation of restorative dental materials. Experimental study.

AIM: The ultrasonic inspection is a non invasive method which is very developed in the industrial field, for the non-destructive evaluation of materials, and in the medical field, for the ultrasound diagnostic analysis. In paediatric dentistry the most widely used non- destructive evaluation is the X-ray technique. Radiographs are valuable aids in the oral health care of infants, children, adolescents, allowing dentists to diagnose and treat oral diseases that cannot be detected during a visual clinical examination. The aim of this in vitro study was to analyse the ultrasonic non-destructive evaluation (UT-NDE) technique to inspect both dental materials internal structure and the form and position of internal defects in order to obtain a diagnostic method, free of ionising radiations, in paediatric dentistry. Moreover the ultrasonic inspection (UT) could be a rapid method of diagnosis in uncooperative paediatric patients. MATERIALS AND METHODS: Study Design: Experimental samples were manufactured with the characteristics of a large composite or glass ionomer cement paediatric dental restoration, in terms of either size or operative technique used. Characteristics of the common restorations were analysed and reproduced in vitro, using the same operative conditions, also adding operative defects into some samples. All the samples were subjected to an innovative UT test using the pulse echo immersion scanning technique. Both C-scans and full volume scans were carried out during the experimental programme. To enhance the data obtained from the UT scan, a digital system (Ecus Inspection software) for signal detection, archiving, processing and displaying was used. RESULTS: UT images showed the presence of internal defects in the dental materials. It was also possible to inspect very thin discontinuity such as the one represented by the fluid resin. STATISTICS: In order to execute the statistical analysis, the values of electric voltage measured in five higher white points and in five higher grey points of the pictures pixels, were measured for each sample. Then, the average values and the standardised data were calculated. CONCLUSION: n In conclusion, the ultrasonic test could be a diagnostic non-invasive method in paediatric patients, capable to evaluate the quality of the restorative teeth filling, showing internal little defects. In vivo application of this diagnostic method should be developed.

An impacted central incisor due to supernumerary teeth: a multidisciplinary approach.

BACKGROUND: Hyperdontia is a disorder of odontogenesis characterised by excess teeth. Many complications can be associated with supernumerary teeth, such as crowding, tooth displacement, diastema, deep caries, retention or impaction, delayed eruption or ectopic eruption of adjacent teeth, aesthetic problems, neuralgic manifestations, root resorption of adjacent teeth, and dentigerous cyst formation with significant bone destruction. Treatment of hyperdontia depends on the area and number of supernumerary teeth, and also on the presence of pathologic processes that can affect them and/or the teeth of the normal series that could be erupted, retained or impacted. CASE REPORT: The present case report describes the clinical management of two impacted supernumerary teeth, which impeded the eruption of the maxillary right central incisor in a paediatric patient. A multidisciplinary treatment approach was planned, including the surgical removal of the supernumerary teeth and the orthodontic correction of the unerupted permanent maxillary right central incisor. Combined surgical and orthodontic treatment resulted in an aesthetically pleasant and balanced occlusion. Thus, early multidisciplinary treatment is required for greater hard and soft tissue preservation.

Oral health status in liver transplant Italian children.

AIM: The aim of this study was to assess the oral health status in children submitted to liver transplantation in order to evaluate the need to promote suitable dental caries prevention programmes. MATERIALS AND METHODS: Thirty-eight children submitted to liver transplantation (4-5 years) were selected and their data were compared to those of an age-matched control healthy group of 78 children. Clinical examinations were carried out and X-ray bitewings were taken, in order to record caries prevalence, caries experience, periodontal health and dental enamel defects. A questionnaire investigating demographic and oral health behaviour data was completed by parents. RESULTS: Caries prevalence was 78.9% in the liver transplantation group and 39.7% in the healthy control group. The dmft mean value was 2.26±2.25 in the liver transplantation group and 0.69±1.51 in the healthy group. The difference in the mean dmft between the two groups was statistically significant (p<0.0001). From the elaboration of the data on periodontal health it resulted that 23.7% of the liver transplantation subjects and 48.7% of controls had a healthy periodontal status, respectively; 39.5% of the liver transplantation children and 23.6% of the controls had plaque and calculus. In addition, 44.7% of the liver transplantation patients and 28.2%% of the control subjects showed bleeding on probing. In the liver transplantation subjects there was a higher prevalence (65.8%) of dental enamel defects with respect to the healthy group (21.8%). CONCLUSION: The high prevalence of caries and gingival diseases showed the need to promote specific dental caries prevention programmes in liver transplant children.

Prevalence of sealant in an Italian paediatric population: an epidemiological study.

AIM: The use of sealants is an effective measure for the prevention of pit and fissure caries in children and it has been well documented by several studies In order to plan and establish a preventive national programme, it is important to know the epidemiological pattern in an Italian paediatric population, correlated to the risk of caries, DMFT and other sociodemographic factors. METHODS: This study was conducted on 2,442 children aged between 6 and 12 years attending the paediatric dentistry department of the University of L'Aquila, Italy. In addition to the oral examination, a questionnaire was administered on bad habits and the family perception of sealing. For descriptive analysis, the sample was stratified into two groups based on the presence/absence of at least one tooth with sealant. The differences between discrete and nominal variables, reported as absolute and percentage frequencies, were assessed by applying the χ2 test or the Fisher's exact test, as appropriate. Continuous variables were expressed in terms of mean values and their standard deviation(±DS) and the differences between the two groups under consideration were analysed through Student's t-test. The tests used are two-way and a significance level of 5% was applied. The statistical analysis was carried out using the statistical package STATA/IC 15.0 (StataCorp LLC, Texas, USA). CONCLUSION: The application of sealants to healthy occlusal surfaces is the best aid in preventing the development of caries in these areas, and this is especially important in childhood and adolescence, when the incidence of this pathology is particularly high.

Dental enamel defects in Italian children with cystic fibrosis: an observational study.

OBJECTIVE: The relationship between cystic fibrosis (CF) and caries experience has already been explored, but relatively little information is available on dental enamel defects prevalence among children affected by cystic fibrosis. The aim of this study was to investigate this issue in deciduous and permanent teeth of children with CF resident in southern Italy. BASIC RESEARCH DESIGN: This cross sectional observational study was undertaken between October 2009 and March 2010. PARTICIPANTS: 88 CF patients and 101 healthy age-matched participated in this study. METHODS: The prevalence of dental enamel defects was calculated using a modified Developmental Defects of Enamel (DDE) index. The comparison of dental enamel defects prevalence among groups was carried out using regression binary logistic analysis. RESULTS: In the CF subjects there was a higher prevalence (56%) of enamel defects in comparison to the healthy group (22%). The most prevalent enamel defect was hypoplasia with loss of enamel (23% of CF patients vs 1 1/2% of control group) in permanent teeth. CONCLUSION: This study confirms that children with cystic fibrosis are at increased risk of developing hypoplastic defects on their permanent teeth.

SEM investigation on casein phosphopeptides capability in contrasting cola drinks enamel erosion: an in vitro preliminary study.

AIM: Erosion of dental hard tissues induced by acidic dietary components is a high-prevalence finding, especially among children and adolescents. Acidic soft drinks are frequently implicated in dental erosion. The aim of this in vitro study was to assess if CPP-ACP preparation is capable of reducing enamel erosion caused by a cola-type drink. MATERIALS AND METHODS: Twenty-five sound human permanent premolars, extracted for orthodontic reasons in patients of 12-16 years old, were used. The roots were removed and the crowns were sectioned in order to obtain 3 enamel sections from each tooth. The specimens were immersed in: (A) cola-type drink; (B) cola-type drink plus CPP-ACP; (C) deionised water (control) for: 48 h, 24 h, 12 h, 6 h and 3 h, respectively. pH values were constantly monitored. Statistical analysis was performed using ANOVA. The enamel samples were evaluated for surface changes using scanning electron microscopy (SEM). RESULTS: Specimens subjected to cola-type drink (treatment A) showed wide areas of enamel dissolution, while the treatment B specimens showed a few areas of little enamel erosion, different from control samples. Adding CPP-ACP to the cola-type drinks influenced pH levels of the solutions, but always in the acidity range. CONCLUSION: CPP-ACP provides protection against dental erosion from cola-type drinks in vitro. Therefore, further studies are necessary to evaluate if adding casein phosphopeptide-stabilised amorphous calcium phosphate complex to acidic cola drinks could reduce their erosive potential in vivo as well.

Recurrent partial seizures with ictal yawning as atypical presentation of Hashimoto's encephalopathy (steroid-responsive encephalopathy associated with autoimmune thyroiditis).

Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare condition whose pathogenesis is unknown, though autoimmune-mediated mechanisms are thought to be involved. The prevalent neurological manifestations of this disorder are epileptic seizures and psychocognitive disorders associated with EEG alterations. High anti-thyroid antibody titers (particularly in cerebrospinal fluid) and the effectiveness of steroid therapy are usually considered to be crucial elements in the diagnostic process. We describe a 19-year-old female patient who had been referred to the psychiatric unit because of behavioral disorders characterized predominantly by delirium with sexual content. She developed recurrent focal seizures characterized by atypical ictal semiology (repetitive forceful yawning) and a rare EEG pattern (recurrent seizures arising from the left temporal region without evident "encephalopathic" activity). The presence of anti-thyroperoxidase antibodies in her cerebrospinal fluid and a good response to steroids confirmed the diagnosis of HE. The atypical presentation in the case we describe appears to widen the electroclinical spectrum of HE and highlights its importance for differential diagnosis purposes in the neuropsychiatric setting.

Instrumental Timed Up and Go test discloses abnormalities in patients with Cervical Dystonia.

Background Cervical dystonia is a movement disorder characterized by involuntary and sustained contraction of the neck muscles that determines abnormal posture. The aim of this study was to investigate whether dystonic posture in patients with cervical dystonia affects walking and causes postural changes. Methods Patients with cervical dystonia and a group of age-matched healthy controls underwent an instrumental evaluation of the Timed Up and Go Test. Findings All the spatio-temporal parameters of the sub-phases of the Timed up and go test had a significantly higher duration in cervical dystonia patients compared to the control group while no differences in flection and extension angular amplitudes were observed. Indeed, we found that Cervical Dystonia patients had abnormalities in turning, as well as in standing-up and sitting-down from a chair during the Timed up and go test than healthy controls. Interpretation Impairment in postural control in cervical dystonia patients during walking and postural changes prompts to develop rehabilitation strategies to improve postural stability and reduce the risk of fall in these patients.