Compound Charcot-Marie-Tooth disease may determine unusual and milder phenotypes

Compound forms of Charcot-Marie-Tooth (CMT) disease have been recently associated with unusually severe neuropathies, an observation that prompted the proposition that the additive effects of two mutations should be searched in patients whose clinical severity falls outside the common CMT phenotypes. In this report, we present a father and a daughter with a very mild and unusual disease that segregates with two mutations in PMP22 gene, the 17p11.2-p12 duplication and a Ser72Leu point mutation. We propose that the deleterious effects of each mutation are partially compensated by the functional effect of the other.

Toothache referred from auriculotemporal neuralgia: case report

P>Aim To present a 52-year-old male patient who complained of intense pain of short duration in the region of the left external ear and in the ipsilateral maxillary second molar that was relieved by blockade of the auriculotemporal nerve in the infratemporal fossa. Summary Extra- and intraoral physical examination revealed a trigger point that reproduced the symptoms upon finger pressure in the ipsilateral auriculotemporal nerve and in the outer auricular pavilion. The patient`s medical history was unremarkable. The maxillary left second molar tooth was not responsive to pulp sensitivity testing and there was no pain upon percussion or palpation of the buccal sulcus. Periapical radiographs revealed a satisfactory root filling in the maxillary left second molar. On the basis of the clinical signs and symptoms, the auriculotemporal was blocked with 0.5 mL 2% lidocaine and 0.5 mL of a suspension containing dexamethasone acetate (8 mg mL(-1)) and dexamethasone disodium sulfate (2 mg mL(-1)), with full remission of pain 6 months later. The diagnosis was auriculotemporal neuralgia. Key learning point Auriculotemporal neuralgia should be considered as a possible cause of nonodontogenic toothache and thus included in the differential diagnoses. The blockade of the auriculotemporal nerve in the infratemporal fossa is diagnostic and therapeutic. It can be achieved with a solution of lidocaine and dexamethasone.

Characterizing the phenotypic manifestations of MFN2 R104W mutation in Charcot-Marie-Tooth type 2

Mutations of the mitofusin 2 (MFN2) gene have been reported to be the most common cause of the axonal form of Charcot Marie Tooth disease (CMT). The aim of this study was to describe a de novo MFN2 p.R104W mutation and characterize the associated phenotype. We screened the entire coding region of MFN2 gene and characterized its clinical phenotype, nerve conduction studies and sural nerve biopsy. Neuropsychological tests and brain MRI were also performed. A de nova mutation was found in exon 4 (c.310C > T; p.R104W). In addition to a severe and early onset axonal neuropathy, the patient presented learning problems, obesity, glucose intolerance, leukoencephalopathy, brain atrophy and evidence of myelin involvement and mitochondrial structural changes on sural nerve biopsy. These results suggest that MFN2 p.R104W mutation is as a hot-spot for MFN2 gene associated to a large and complex range of phenotypes. (C) 2011 Elsevier B.V. All rights reserved.

Dorsal root ganglionectomy for the diagnosis of sensory neuropathies. Surgical technique and results

Background: Inflammatory diseases stand out among sensory neuronopathies because, in their active phase, they can be treated with immunosuppressive agents. Immunosuppressive therapy may present severe adverse effects and requires previous inflammatory activity confirmation. Sensory neuronopathies are diagnosed based on clinical and EMG findings. Diagnostic confirmation and identification of inflammatory activity are based on sensory ganglion histopathological examination. We describe the surgical technique used for dorsal root ganglionectomy in patients with clinical/EMG diagnosis of sensory neuronopathies. Methods: The sensory ganglion was obtained from 15 patients through a small T7-T8 hemilaminectomy and foraminotomy to expose the C7 root from its origin to the spinal nerve bifurcation. In 6 patients, the dural cuff supposed to contain the ganglion was resected en bloc; and in 9 patients, the ganglion was obtained through a longitudinal incision of the dural cuff and microsurgical dissection from the ventral and dorsal roots and radicular arteries. All ganglia were histopathologically examined. Results: No ganglion was found in the dural cuff in 2 patients submitted to en bloc removal, and the ganglion was removed in all patients who underwent microsurgical dissection. All but 2 patients that had ganglion examination presented a neuronopathy of nerve cell loss, 3 with mononuclear inflammatory infiltrate. These patients underwent immunosuppressive therapy, and 2 of them presented clinical improvement. No surgical complications were observed. Conclusions: Microsurgical dorsal root ganglionectomy for diagnosing inflammatory sensory ganglionopathies was effective and safe. Although safe, en bloc resection of the proximal dural cuff was not effective for this purpose. (c) 2008 Published by Elsevier Inc.

Dentine caries inhibition through CO(2) laser (10.6 mu m) irradiation and fluoride application, in vitro

Objective: The purpose of the study was to investigate whether dentine irradiation with a pulsed CO(2) laser (10.6 mu m) emitting pulses of 10 ms is capable of reducing dentine calcium and phosphorus losses in an artificial caries model. Design: The 90 dentine slabs obtained from bovine teeth were randomly divided into six groups (n = 15): negative control group (GC); positive control group, treated with fluoride 1.23% (GF); and laser groups irradiated with 8 J/cm(2) (L8); irradiated as in L8 + fluoride 1.23% (L8F); irradiated with 11j/cm(2) (L11); irradiated as in L11 + fluoride 1.23% (L11F). After laser irradiation the samples were submitted to a pH-cycling model for 9 days. The calcium and phosphorous contents in the de- and remineralization solutions were measured by means of inductively coupled plasma optical emission spectrometer - ICP-OES. Additionally intra-pulpal temperature measurements were performed. The obtained data were analysed by means of ANOVA and Tukey`s test (alpha = 0.05). Results: In the demineralization solutions the groups L11F and GF presented significantly lower means of calcium and phosphorous losses than the control group; and in L11F means were significantly lower than in the fluoride group. Both irradiation parameters tested caused intrapulpal temperature increase below 2 degrees C. Conclusion: It can be concluded that under the conditions of this study, CO(2) laser irradiation (10.6 mu m) with 11J/cm(2) (540 mJ and 10 Hz) of fluoride treated dentine surfaces decreases the loss of calcium and phosphorous in the demineralization process and does not cause excessive temperature increase inside the pulp chamber. (C) 2010 Elsevier Ltd. All rights reserved.

Comparative analysis of root surface smear layer removal by different etching modalities or erbium:yttrium-aluminum-garnet laser irradiation. A scanning electron microscopy study

The purpose of this study was to evaluate the effect of erbium:yttrium-aluminum-garnet (Er:YAG) laser (2.94 mu m) irradiation on the removal of root surface smear layer of extracted human teeth and to compare its efficacy with that of citric acid, ethylenediamine tetra-acetic acid (EDTA), or a gel containing a mixture of tetracycline hydrochloride (HCl) and citric acid, using scanning electron microscopy (SEM). Thirty human dentin specimens were randomly divided into six groups: G1 (control group), irrigated with 10 ml of physiologic saline solution; G2, conditioned with 24% citric acid gel; G3, conditioned with 24% EDTA gel; G4, conditioned with a 50% citric acid and tetracycline gel; G5, irradiated with Er:YAG laser (47 mJ/10 Hz/5.8 J/cm(2)/pulse); G6, irradiated with Er:YAG laser (83 mJ/10 Hz/10.3 J/cm(2)/pulse). Electron micrographs were obtained and analyzed according to a rating system. Statistical analysis was conducted with Kruskal-Wallis and Mann-Whitney tests (P < 0.05). G1 was statistically different from all the other groups; no statistically significant differences were observed between the Er:YAG laser groups and those undergoing the other treatment modalities. When the two Er:YAG laser groups were compared, the fluency of G6 was statistically more effective in smear layer removal than the one used in G5 (Mann-Whitney test, P < 0.01). Root surfaces irradiated by Er:YAG laser had more irregular contours than those treated by chemical agents. It can be concluded that all treatment modalities were effective in smear layer removal. The results of our study suggest that the Er:YAG laser can be safely used to condition diseased root surfaces effectively. Furthermore, the effect of Er:YAG laser irradiation on root surfaces should be evaluated in vivo so that its potential to enhance the healing of periodontal tissues can be assessed.

Analysis of Apical Root Transportation Associated with Protaper Universal F3 and F4 Instruments by Using Digital Subtraction Radiography

Introduction: The aim of this study was to assess the occurrence of apical root transportation after the use of Pro Taper Universal rotary files sizes 3 (F3) and 4 (F4). Methods: Instruments were worked to the apex of the original canal, always by the same operator. Digital subtraction radiography images were produced in buccolingual and mesiodistal projections. A total of 25 radiographs were taken from root canals of human maxillary first molars with curvatures varying from 23-31 degrees. Quantitative data were analyzed by intraclass correlation coefficient and Wilcoxon nonparametric test (P = .05). Results: Buccolingual images revealed a significantly higher degree of apical transportation associated with F4 instruments when compared with F3 instruments in relation to the original canal (Wilcoxon test, P = .007). No significant difference was observed in mesiodistal images (P = .492). Conclusions: F3 instruments should be used with care in curved canals, and F4 instruments should be avoided in apical third preparation of curved canals. (J Endod 2010;36:1052-1055)

Diode Laser Irradiation Effects on the Sealing Ability of Root Canal Sealers

This study aimed to test the hypothesis that dentine alterations induced by 810 nm-diode laser may affect the interaction between root canal sealers and the dentin wall. Seventy-two single root human teeth were selected and root canals were enlarged with K-files. Dentine was treated with 0.5% NaOCl and 17% EDTA-T and irradiated (laser group) by diode laser (810 nm/P = 2.5W/I = 1989 W/cm(2)) or remained non-irradiated (control group). Six samples per group were analyzed by scanning electron microscopy (SEM). The remaining samples of each group were divided into three subgroups (n = 10) and sealed with one of the tested sealers (N-Rickert/AHPlus (TM)/Apexit (R)). Apical leakage was estimated by evaluating penetration of 0.5% methylene-blue dye. SEM analysis revealed that dentine at the apical third in irradiated samples was melted and fusioned whereas non-irradiated samples exhibited opened dentinal tubules. Despite the morphological changes induced by irradiation, laser did not affect the sealing ability of N-Rickert and AHPlus (TM) sealers. However, the length of apical leakage in roots filled with Apexit (R) was lower in irradiated root canals than in non-irradiated samples (p < 0.05). Morphological changes of root canal walls promoted by diode laser irradiation may improve de sealing ability of Apexit (R), a calcium hydroxide-based sealer, suggesting that improved sealing promoted by irradiation may represent an additional factor contributing to the endodontic clinical outcome.

Morphological assessment of dentine and cementum following apicectomy with Zekrya burs and Er:YAG laser associated with direct and indirect Nd:YAG laser irradiation

Objectives. This study aimed to assess the apical surface morphology of maxillary central incisors resected 3.0 mm from the tooth apex using Zekrya burs or Er:YAG laser, with or without subsequent direct Nd:YAG laser irradiation (apical and buccal surfaces) and indirect irradiation (palatal surface). Study design. Forty maxillary central incisors were instrumented and obturated. The roots were divided into 4 groups according to the root resection method (Zekrya bur or Er: YAG laser -1.8 W, 450 mJ, 4 Hz, 113 J/cm(2)) and further surface treatment (none or Nd: YAG laser -2.0 W, 100 mJ, 20 Hz, 124 J/cm(2)). The teeth were prepared for SEM analysis. Scores ranging from 1 to 4 were attributed to cut quality and morphological changes. The data were analyzed by the Kruskal-Wallis test and by Dunn`s test. Results. SEM images showed irregular surfaces on the apical portions resected with Zekrya burs, with smear layer and grooves in the resected dentine and slight gutta-percha displacement and plasticization. On the other hand, apicectomies carried out with Er: YAG laser showed morphological changes compatible with ablated dentine, with rough surfaces and craters. In spite of the presence of plasticized gutta-percha, with the presence of bubbles, an irregular adaptation of the filling material to the root walls was also observed. Direct Nd: YAG laser irradiation of the apical and buccal surfaces of the resected roots resulted in areas of resolidification and fusion in the dentine and cementum, with a vitrified aspect; indirect Nd: YAG laser irradiation of the palatal surfaces yielded a lower number of changes in the cementum, with irregular resolidification areas. Conclusions. There were no differences in terms of cut quality between the use of burs and Er: YAG laser or between the 2 surfaces (apical and buccal) treated with Nd: YAG laser with direct irradiation. However, morphological changes were significantly less frequent on surfaces submitted to indirect irradiation (palatal) when compared with those directly irradiated. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010; 109: e77-e82)

In vitro analysis of inhibitory effects of the antibacterial monomer MDPB-containing restorations on the progression of secondary root caries

Objective: This study aimed to analyze in vitro inhibitory effects of restorative materials containing the antibacterial monomer 12-methacryloyloxydodecylpyridinium bromide (MDPB) on the formation of artificial secondary root caries lesions. Methods: Class V cavities (2 mm x 2 mm) were prepared in 75 human root fragments. Specimens were randomly divided into five groups (n = 15 fragments per group) and restored as follows: (I) MDPB-free adhesive system + MDPB-free composite (negative control); (II) resin modified glass ionomer (RM-GIC; positive control); (III) MDPB-free adhesive system + MDPB-containing composite (2.83% MDPB); (IV) MDPB-containing adhesive system + MDPB-free composite; M MDPB-containing adhesive system + MDPB-containing composite. Artificial secondary root caries lesions were produced by a biological artificial caries challenge. The restored specimens were immersed into a culture medium containing Streptococcus mutans and sucrose for 15 days. Histological slices (80 +/- 20 mu m) of the specimens were used for measuring the mean depths of the artificial lesions produced in both margins of the restorations using polarized light microscopy. Results were expressed in percentage related to the mean depth of the negative control, considered 100%. Data were compared by ANOVA followed by the Tukey`s test (p <= 0.05). Results: The depths of lesions adjacent to cavities filled with RM-GIC (GII; 85.17 +/- 15.2%) were significantly (p < 0.01) shallower than those adjacent to restorations with MDPB-free composite (GI; 100.00 +/- 10.04%), despite the presence of MDPB in the adhesive system (GIV; 101.95 +/- 21.32%). The depths of lesions adjacent to cavities restored with MDPB-containing composite (GIII; 82.68 +/- 12.81% and GV; 85.65 +/- 15.42%), despite the adhesive system used, were similar to those of RM-GIC (GII). Mean lesions depths in these groups decreased from 13% (GV) to 17% (GIII) in relation to the negative control (GI). Conclusions: MDPB-containing composite inhibits the progression of artificial secondary root caries lesions regardless of adhesive systems. (C) 2009 Elsevier Ltd. All rights reserved.

Does a final rinse to remove smear layer interfere on dentin permeability of root canals?

Objective. The objective of this study was to assess the influence of different final irrigating solutions on dentin permeability and smear layer removal using the same specimens and relate the results obtained. Study design. Forty anterior human teeth were instrumented and divided into 4 groups (n = 10) at the final rinse step, according to the irrigant used: G I (control) - 1% NaOCl; G II - 17% EDTA; G III - 17% EDTAT; and G IV - Biopure MTAD. The canals were filled with 0.5% methylene blue and maintained in bottles for 48 hours. The roots were transversally split in coronal, middle, and apical fragments. The specimens were photographed and analyzed regarding dye penetration. The fragments were then axially split and prepared for SEM. The photomicrographs were analyzed and qualified by scores. Results. Only the EDTA-T group exhibited statistical difference in which the apical third had less dentin permeability (P < .05). When a decalcifying agent was used, smear layer was removed, which did not happen in the NaOCl group. Regarding smear layer removal, differences were found only in the EDTA group in which the apical third presented more smear layer (P < .05). No correlation was found for both studies (r = 0.4207). Conclusions. There was not an even relationship between the results from both studies, which inferes that higher or lower dentinal permeability does not necessarily correspond to a higher or lower amount of smear layer. The analysis of dentin permeability and smear layer removal was shown to be a feasible procedure using the same specimens. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107: e47-e51)

Determination of pulp vitality in vivo with pulse oximetry

Aim To evaluate the use of pulse oximetry as a test for pulp vitality, by comparing in the same patient, the levels of oxygen saturation of the index finger and of the maxillary central incisor and canine teeth without clinically detectable pulp inflammation. Methodology Seventeen male and female patients aged between 26 and 38 years participated and a total of 32 maxillary central incisor and 32 canine teeth were analysed. Selection criteria required the teeth to have healthy crowns, or with restorations no more than 2 mm in diameter and no clinical and radiographical signs or symptoms of pulp or periapical inflammatory changes. The negative control group consisted of 10 root filled teeth. Measurements were first taken from the index finger of patients. Their teeth were then subjected to a thermal test with refrigerant gas and then to a vitality test with pulse oximetry. Data were analysed by Pearson`s and paired t-tests. Results There were no significant statistical correlations between blood oxygen levels in the index finger and in the teeth of the patient (P > 0.05). There was a statistically significant difference in the oxygen levels between the two tooth groups studied and the index finger (P <= 0.002). Mean oxygen values in the index finger of patients were 95% (SD = 1.6), oxygen values in the maxillary central incisor were 91.29% (SD = 2.61) and mean oxygen values in maxillary canine were 90.69% (SD = 2.71). Conclusion The method determined consistently the level of blood oxygen saturation of the pulp in maxillary central incisor and canine teeth and can therefore be used for pulp vitality testing. Further studies are required to assess the effectiveness and validity of pulse oximetry in determining pulp vitality in traumatized teeth.

Performance of techniques used for re-attachment of endodontically treated crown fractured teeth

Objectives: The aim of this study was to compare the fracture strength of three techniques used to re-attach tooth fragments in sound and endodontically treated fractured teeth with or without fiber post placement. Material and methods: Ninety human lower incisors were randomly divided into three groups of 30 teeth each. In group A teeth were not subjected to endodontic treatment; while teeth from groups B and C were endodontically treated and the pulp chamber restored with a composite resin. All teeth were fractured by an axial load applied to the buccal area in order to obtain tooth fragments. Teeth from each group were then divided into three subgroups, according to the re-attachment technique: bonded-only, buccal-chamfer and circumferential chamfer. Before the re-attachment procedures, fiber posts were placed in teeth from group C using dual cure resin luting cement (Duo-Link). All teeth (groups A-C) had the fragments re-attached using a same dual cure resin luting cement. in the bonded-only group, no additional preparation was made. After re-attachment of the fragment, teeth from groups buccal and circumferential chamfer groups had a 1.0 mm depth chamfer placed in the fracture line either on buccal surfaceor along the buccal and lingual surfaces, respectively. increments of microhybid composite resin (Tetric Ceram) were used in subgroups buccal chamfer and circumferential chamfer to restore the chamfer. The specimens were loaded until fracture in the same pre-determined area. The force required to detach each fragment was recorded and the data was subjected to a three-way analysis of variance where factors Group and Re-attachment technique are independent measures and Time of fracture is a repeated measure factor (first and second) and Tukey`s test (alpha = 0.05). Results: The main factors Re-attachment technique (p = 0.04) and Time of fracture (p = 0.02) were statistically significant. The buccal and circumferential chamfer techniques were statistically similar (p > 0.05) and superior to the bonded-only group (p < 0.05). The first time of fracture was statistically superior to second time of fracture (p < 0.001). Conclusions: The use of fiber post is not necessary for the reinforcement of the tooth structure in re-attachment of endodontically treated teeth. When bonding a fractured fragment, the buccal or circumferential re-attachment techniques should be preferable in comparison with the simple re-attachment without any additional preparation. None of the techniques used for re-attachment restored the fracture strength of the intact teeth. (C) 2008 Elsevier Ltd. All rights reserved.

Tooth Tissue Engineering: Optimal Dental Stem Cell Harvest Based on Tooth Development

Our long-term objective is to devise reliable methods to generate biological replacement teeth exhibiting the physical properties and functions of naturally formed human teeth. Previously, we demonstrated the successful use of tissue engineering approaches to generate small, bioengineered tooth crowns from harvested pig and rat postnatal dental stem cells (DSCs). To facilitate characterizations of human DSCs, we have developed a novel radiographic staging system to accurately correlate human third molar tooth developmental stage with anticipated harvested DSC yield. Our results demonstrated that DSC yields were higher in less developed teeth (Stages 1 and 2), and lower in more developed teeth (Stages 3, 4, and 5). The greatest cell yields and colony-forming units (CFUs) capability was obtained from Stages 1 and 2 tooth dental pulp. We conclude that radiographic developmental staging can be used to accurately assess the utility of harvested human teeth for future dental tissue engineering applications.

MMP1 and MMP20 contribute to tooth agenesis in humans

Objective: Variations in genes that are critical for tooth formation may contribute to the tooth agenesis. MMPs are potential candidate genes for dental alterations based on the roles they play during embryogenesis. The aim of this study was to investigate the possible association between MMP1, MMP3, and MMP20 and tooth agenesis. Methods: One hundred sixty-seven nuclear families from two different populations were analysed, 116 from Brazil and 51 from Turkey. Probands had at least one congenitally missing tooth. DNA samples were obtained from blood or saliva samples and genotyping was performed using TagMan chemistry. In addition, Mmp20 was selected for quantitative real-time polymerase chain reaction analysis with SYBR Green I Dye in mouse tooth development. Results: Associations between tooth agenesis and MMP1 (p = 0.007), and MMP20 (p = 0.03) were found in Brazilian families. In the total dataset, MMP20 continued to be associated with tooth agenesis (p = 0.01). Mmp20 was not expressed during the initial stages of tooth development. Conclusion: Our findings provide evidence that MMP1 and MMP20 play a role in human tooth agenesis. (C) 2010 Elsevier Ltd. All rights reserved.