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Laurin-Sandrow syndrome with additional associated manifestations.

A Thai man with Laurin-Sandrow syndrome (LSS, MIM 135750), the ninth reported case, is described. He had an underdeveloped nasal bone, scar-like seams under the nose, large heads of mandibular condyles, and brachymesophalangy of toes as newly observed findings of the syndrome. He also had mental retardation. The patient had duplication of ulna, with triphalangeal thumbs, and polydactyly of one finger. The triphalangeal thumbs were non-opposable. Carpal bones were malformed. Mirror image polydactyly of the toes was present. There were nine toes on the right and eight on the left. Joint abnormalities were observed at his elbows, wrists, knees, ankles, fingers, and toes. Synostosis of severely malformed tarsal bones was noted. This appears to be the first case of LSS with anomalies not limited to the nose and limbs. The relationship between LSS, tibial hemimelia-polysyndactyly-triphalangeal thumbs syndrome, triphalangeal thumb-polysyndactyly syndrome, preaxial polydactyly types 2 and 3, and Haas-type syndactyly is discussed.

Abnormalities, Multiple↗

The human mandible: lever or link?

The mammalian mandible, and in particular the human mandible, is generally thought to function as a lever during biting. This notion, however, has not gone unchallenged. Various workers have suggested that the mandible does not function as a lever, and they base this proposition on essentially two assertions: (1) the resultant of the forces produced by the masticatory muscles always passes through the bite point; (2) the condylar neck and/or the temporomandibular joint is unsuited to withstand reaction forces during biting. A review of the electromyographic data and of the properties of the tissues of the temporomandibular joint do not support the non-lever hypothesis of mandibular function. In addition, an analysis of the strength of the condylar neck demonstrates that this structure is strong enough to withstand the expected reaction force during lever action. Ordinarily the human mandible and the forces that act upon it are analyzed solely in the lateral projection. Moments are then usually analyzed about the mandibular condyle; however, some workers have advocated taking moments about other points, e.g., the instantaneous center of rotation. Obviously it makes no difference as to what point is chosen since the moments about any point during equilibrium conditions are equal to zero. It is also useful to analyze the forces acting on the mandible in the frontal projection, particularly during unilateral biting. The electromyographic data suggest that during powerful unilateral molar biting the resultant adductor muscle force is passing between the bite point and the balancing (non-biting side) condyle. Therefore, in order for this system to be in equilibrium there must be a reaction force acting on the balancing condyle. This suggests that reaction forces are larger on the balancing side than on the working side, and possibly explains why individuals with a painful temporomandibular joint usually prefer to bite on the side of the diseased joint.

Biomechanical Phenomena↗

Craniofacial sequelae of lesions to facial and trigeminal motor nuclei in growing rats.

Unilateral electrolytic lesions were made in the left-side facial motor nucleus (FMNu) of six Sprague-Dawley rats at 35 days of age in order to correlate craniofacial sequelae with changed motoneuron function. Experimental and control rats were killed at 22, 32, 42, and 52 days postoperatively to provide muscle weight, brain histology, and dry skull preparations for analyses. Dissection, muscle weight, motoneuron count, and osteometric data revealed that lesion-side facial and masticatory muscles were affected by the lesions. Paired t-tests indicated that significant differences existed between weights of experimental lesion- and nonlesion-side anterior digastric, temporalis, masseteric complex, and medial pterygoid muscles, numbers of facial and trigeminal motoneurons, and several skeletal dimensions of the skull. Basi-cranial dimensions of experimental animals were least affected by the lesion, whereas zygomatic arch, dorsal facial region, and mandibular condyle dimensions were most affected. Statistical analyses also detected significant differences between experimental and control groups for several skeletal dimensions of the skull. Data indicated that damage to the trigeminal motor nucleus (TMNu) was secondary to the primary lesion in the FMNu. Motoneurons within the facial and trigeminal neuromuscular complexes (FNC and TNC) play an important role in craniofacial growth and development.

Animals↗

Temporomandibular joint osteoarthritis in a British skeletal population.

The potential role of dental function in TMJ osteoarthritis is examined in an ancient British population. Human skeletal remains from five archaeological sites in England (n = 369) were studied to assess the associations between the presence of osteoarthritis and dental function variables, in particular antemortem tooth loss and dental attrition. Individuals aged over 17 years with at least a portion of either the left or right TMJ were included in the study. Each individual was scored for the occurrence of arthritic lesions on the mandibular condyle and glenoid fossa, the level of dental attrition, and antemortem tooth loss. A loglinear analysis was performed with osteoarthritis as the dependent variable and tooth loss, attrition, sex, and age as the independent variables. Tooth loss and sex are not significantly associated with TMJ osteoarthritis when attrition and age are included in the analysis. Attrition is significantly associated with osteoarthritis even when the analysis corrects for age. The significant association of attrition with osteoarthritis suggests that dental functions may have an influence, separate from ageing, on the development of TMJ osteoarthritis.

Adolescent↗

Morphological adaptation to diet in platyrrhine primates.

Morphological features of the jaws and teeth are examined in eight species of platyrrhine monkeys that coexist in the Suriname rainforest. Z-scores calculated from geometric predictions for several features of the feeding apparatus thought to have some functional significance (e.g., tooth dimensions, jaw robusticity, leverage of primary jaw elevators) are compared to a profile of the naturalistic dietary behavior of these species (i.e., proportions of fruit mesocarp, seeds, leaves, and fauna eaten). Several features are found exclusively in those platyrrhines whose dietary preferences are the most limited. Such specializations appear to be associated with a particular protein source exploited by a species to supplement a largely frugivorous diet. Ateles paniscus, which feeds primarily on the mesocarp of ripe fruit, has an adaptive morphology that emphasizes broad incisors. Chiropotes satanas (and to a slightly lesser extent, Pithecia pithecia) is a frugivore/seed predator with large upper and lower canines and a robust mandible. The frugivore/folivore Alouatta seniculus has a relatively large total molar area and effective mandibular condyle height. In all four of these strictly vegetarian species, the leverage of the masseter muscle is greater than that of temporalis. Of the omnivorous species, Cebus apella and C. nigrivittatus exploit both fauna and seeds for protein and exhibit an array of many of the above features, such as large teeth and thick mandibles. Saimiri sciureus, not particularly known for seed predation, departs from Cebus in having less robust canines and a more gracile mandible. All three cebid omnivores have a temporalis with greater leverage than the masseter, indicating a requirement for resisting anteriorly directed forces, for example, using the jaws for vigorous foraging. The lack of any enlarged features, other than incisors, in the omnivorous Saguinus midas may be attributable to the functional constraints of small body size. Because the small size of the gape limits the size of the food parcel ingested, a requirement to enlarge other dentomandibular structures for trituration is alleviated.

Adaptation, Physiological↗

Comparison of the distribution patterns of tenascin and alkaline phosphatase in developing teeth, cartilage, and bone of rats and mice.

Tenascin is a glycoprotein of the extracellular matrix, which has been associated with differentiation of hard tissue forming cells. Alkaline phosphatase (AP) is involved in calcification, and it has also been suggested to function in cell differentiation. We have compared the distributions of tenascin and AP in the developing skull and teeth of embryonic and growing rats and mice. Tenascin was localized by immuno-Peroxidase and AP by enzyme histochemical staining of tissue sections. Both tenascin and AP were largely restricted to bone, cartilage, and teeth. In cartilage, tenascin was expressed in the perichondrium, whereas AP activity was detected only in the hypertrophic cartilage. In growing intramembranous bone, tenascin and AP were expressed in the periosteum and endosteum. AP activity was restricted to the inner layer of the periosteum, whereas tenascin expression extended to the more superficial layers. In bud-staged teeth tenascin but no AP activity was localized in the condensing mesenchymal cells around the epithelial bud. At the bell stage both tenascin and AP activity were localized in the cuspal mesenchyme, and the intensity of staining decreased towards the cervical region. In summary, tenascin was present at all sites of AP activity except in the epithelial cells of the enamel organ and the hypertrophic cartilage of the mandibular condyle. In mesenchymal tissues tenascin was more widely distributed than AP. It can be suggested that tenascin has functions at earlier stages of hard tissue formation than AP.

Alkaline Phosphatase↗

Functional movements of putative jaw muscle insertions.

BACKGROUND: The craniomandibular muscles control jaw position and forces at the teeth and temporomandibular joints, but little is known regarding their biomechanical behaviour during dynamic function. The objective of this study was to determine how jaw muscle insertions alter position during different jaw movements in living subjects. METHODS: Computer 3D reconstruction of MR images and jaw-tracking were combined to permit the examination of movement with six degrees of freedom. Maximum mandibular opening, protrusive and laterotrusive positions were recorded in four subjects, and the translation and rotation of the putative insertions of masseter, temporal, medial, and lateral pterygoid muscles were measured. RESULTS: The sizes and shapes of regional attachments varied markedly among subjects, and their displacement patterns were different in specific muscles. For instance, when the jaw closed to the dental intercuspal position from maximum gape, the region near the superior insertion site of the masseter moved backward and upward, whereas the region near the inferior insertion site displaced mainly forward. In three subjects, the jaw's rotational center during this act was approximately 26-34 mm below the mandibular condyles. CONCLUSIONS: Since the movements of each muscle part differ according to variations in the size and shape of insertion areas, individual musculoskeletal form, and patterns of jaw motion during function, the prediction of motion-related muscle mechanics in any one subject is unlikely to be possible without direct measurement of the motion of visualized muscle parts. The present study shows that this information can be obtained.

Adult↗

Expression of caveolin-1 in the rat temporomandibular joint.

This immunocytochemical study revealed the expression of caveolin-1, a major protein of caveolae, in the rat temporomandibular joint. In the synovial lining layer, immunoreactive products for caveolin-1 were detected on the cell membrane of the fibroblast-like type B cells, as confirmed by immunocytochemistry for heat shock protein 25. The cells in the articular disk, the articular layer, and zone of proliferation of the mandibular condyle also showed intense immunoreactions for caveolin-1.

Animals↗

Relationship between sex, age, and the minimum thickness of the roof of the glenoid fossa in normal temporomandibular joints.

This autopsy study investigates the minimum thickness of the roof of the glenoid fossa of grossly normal temporomandibular joints (TMJ) and correlates this to gender and age. This study was based on 49 TMJ specimens collected from 26 male and 23 female cadavers whose mean age at death was 73.8 years (range=50-96). No information was available about TMJ symptoms before death. A digital micrometer was used to measure the minimum thickness of the glenoid fossae. Comparisons were made based on gender and age. The mean value for the minimum roof thickness of all joint specimens examined was 0.8 mm. Mean values for male and female specimens were 0.8 and 0.7 mm, respectively, which were not significantly different. In addition, no age-related differences were observed. The average thickness was 0.7 mm for individuals in their fifties, 0.8 mm for those in their sixties and seventies, 0.6 mm for those in their eighties, and 0.8 mm for individuals in their nineties. Although these data indicate that the minimum thickness of the glenoid fossa of the TMJ is not significantly correlated with sex or age in patients 50 years of age and older, they nonetheless provide additional information about normal TMJ anatomy, particularly with regard to our knowledge of joint remodeling and function in the disease and non-disease states. Thickness of the glenoid fossa has also been implicated as a factor in facial trauma involving superior displacement of the mandibular condyle into the middle cranial fossa. The thickness of the glenoid fossa is also of potential interest during surgeries involving the glenoid fossa, such as TMJ arthroplasty or joint reconstruction.

Age Factors↗

Bony eminence on the middle cranial fossa corresponding to the temporomandibular joint.

We report a nameless bony eminence over the temporomandibular joint (TMJ) and its possible clinical significance. Forty-two half heads of 21 UK Caucasian cadavers (61-95 years old, mean 84.3 +/- 8.2 years, male:female = 11:10) were used to investigate the surface of the middle cranial fossa (MCF) over the TMJ. The thickness of the bony roof of the glenoid fossa was also measured. A bony eminence over the glenoid fossa was observed in half of the specimens. Some showed a complete oval bulge, which completely reflected the contour of the glenoid fossa. The others showed a bony bulge, which partially reflected that contour. The mean (+/-SD) thickness of the bone in the roof of glenoid fossa was 1.5 +/- 1.2 mm. The mean bony thickness of specimens showing the eminence was 0.8 +/- 0.5 mm, whereas it was 2.3 +/- 1.2 mm in specimens without an eminence. These differences were statistically significant (P < 0.01). The osteological features we describe may be relevant to certain clinical problems. Traumatic dislocation of mandibular condyle, for example, might relate to a weakness of the glenoid fossa.

Aged↗

Molecular cloning of a highly conserved mouse and human integral membrane protein (Itm1) and genetic mapping to mouse chromosome 9.

We have isolated and characterized a novel cDNA coding for a highly hydrophobic protein (B5) from a fetal mouse mandibular condyle cDNA library. The full-length mouse B5 cDNA is 3095 nucleotides long and contains a potential open reading frame coding for a protein of 705 amino acids with a calculated molecular weight of 80.5 kDa. The B5 mRNA is differentially polyadenylated, with the most abundant transcript having a length of 2.7 kb. The human homolog of B5 was isolated from a cDNA testis library. The predicted amino acid sequence of the human B5 is 98.5% identical to that of mouse. The most striking feature of the B5 protein is the presence of numerous (10-14) potential transmembrane domains, characteristic of an integral membrane protein. Similarity searches in public databanks reveal that B5 is 58% similar to the T12A2.2 gene of Caenorhabditis elegans and 60% similar to the STT3 gene of Saccharomyces cerevisiae. Furthermore, the report of an EST sequence (Accession No. Z13858) related to the human B5, but identical to the STT3 gene, indicates that B5 belongs to a larger gene family coding for novel putative transmembrane proteins. This family exhibits a remarkable degree of conservation in different species. The gene for B5, designated Itm1 (Integral membrane protein 1), is located on mouse chromosome 9.

Animals↗

Light and electron microscopic morphology of the temporomandibular joint in growing and mature crab-eating monkeys (Macaca fascicularis): the condylar calcified cartilage.

In an attempt to show maturational alterations in the calcified cartilage, mandibular condyles of four growing and four adult male monkeys (Macaca fascicularis) were studied using light microscopy as well as transmission and scanning electron microscopy. All specimens were initially fixed by perfusion in the presence of ruthenium red. For examination of the hard tissue surfaces in the scanning electron microscope, uncalcified tissues were removed with sodium hypochlorite. In growing animals, almost the entire hard tissue surface in the joint region of the condyle was formed by calcified cartilage, while in adult animals, calcified cartilage was confined to load-bearing regions. In growing animals, the appearance of the calcified cartilage surface suggested a continuously advancing mineralizing front similar to that seen in the epiphyseal plate. Chondrocytes mostly exhibited a terminal stage of hypertrophy, and seemed to die and get lost through vascular invasion and subsequent endochondral ossification. In adult animals, most of the calcified cartilage surface appeared comparatively stable, and resembled the tidemark of articular cartilage. Chondrocytes were usually small and appeared viable. However, on the adult condyles, there were always circumscribed islands where chondrocytes and the pattern of mineralization resembled those seen in growing animals. In these regions, prominent chondroclastic activity indicated extensive articular remodelling. These observations suggest that at the end of somatic growth, condylar calcified cartilage undergoes considerable maturation from a type reminiscent of hyaline growth cartilage to a type resembling articular cartilage. Concomitantly, chondrocytes appear to change their developmental program, in that they stop enlarging and lose their commitment to death. However, they may be able to retain, or switch back to, a more immature stage, in case there is need for extensive articular remodelling.

Animals↗

Growth and differentiation of murine cartilage cells in vitro following a short-term exposure to triamcinolone acetonide.

Measurements of 3H-thymidine incorporation, quantitative autoradiography and morphometry were used to evaluate cell behavior during the recovery of mandibular condylar cartilage cultures following short-term exposure to a corticosteroid hormone in vitro. Apical segments of mandibular condyles of newborn mice were initially incubated in the presence of the hormone triamcinolone acetonide (10(-6) M) for 24 h and were thereafter cultured for additional 6 days in hormone-free medium. The present results indicated that the treatment led to a decrease in the rate of incorporation of 3H-thymidine, a feature that lasted for 48 h following the removal of the hormone. Quantitative 3H-thymidine autoradiography of explants that were labeled in the presence of the hormone further substantiated the initial suppressive effect of the hormone on cellular proliferation, a feature that was followed by a recovery. Differences were noted in the pattern of distribution of labeled cells: in control explants, labeled cells progressively moved from the chondroprogenitor compartment into the differentiated portion of the cartilage; in hormone-treated explants, 3H-thymidine labeled cells were confined to the progenitor layer up to 5 days after the treatment and only then appeared in the chondrocytic compartment. The hormone's adverse effect upon differentiation was manifested by both morphology, and by causing a significant increase in the size of the progenitor layer (up to 50.5% on 4th post-treatment day) along with a 70.5% reduction in the size of chondroblastic layer. We conclude that a short-term exposure to a glucocorticoid hormone in vitro interferes with proliferation of chondroprogenitor cells and their subsequent differentiative pathway.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

A tridimensional study using cuts at a low temperature of the infratemporal region.

Although the infratemporal region is well defined anatomically, its complex topography has been the subject of numerous, and sometimes, opposite works. That is the reason why it appeared necessary for the authors to re-evaluate this topic using the original method of Combelles and Boyer, allowing to define three referential planes, and thereby, a tridimensional shape and the volume of this region. This study allows to conclude that the infratemporal region is a triangular prism with an horizontal main axis 47 mm long. Its anterior base has a mean area of 733 mm2. The posterior top consists of the Juvara slot and has a mean area of 490 mm2. This infratemporal prism contains another one, the pterygomandibular space, prismatic too. It widens out from coronoïd plane (93 mm2) to mandibular foramen plane (169 mm2) before ending as a narrow groove between the neck of the mandibular condyle and the interpterygoïd fascia. The volume of the pterygomandibular space is quite superior to the value usually reported in the dental literature. It is of 4.8 ml to 5.8 ml according to denture. These results point out the opportunity to accomodate more important volumes of anesthesic solutions, than the 1.8 ml usually performed, without any leak out of the infratemporal region.

Aged↗

Dysphagia and micrognathia in a patient with juvenile rheumatoid arthritis.

This report describes the case of a 35-year-old man with juvenile rheumatoid arthritis. The patient experienced swallowing difficulties that were initially thought to be a result of the disease affecting the cervical spine. A maxillofacial examination, however, showed a total loss of the mandibular condyles, short rami and bodies, and a retrusion of the jaw. This was considered to be the main cause of the dysphagia, and not a glossopharyngeal nerve compression. The surgical-orthodontic management of these difficult cases is discussed.

Adult↗