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A case of tardive dyskinesia caused by metoclopramide.

Abnormal involuntary movements appeared in the mouth, tongue, neck and abdomen of a 64-year-old male patient after he took metoclopramide for gastrointestinal disorder in a regimen of 30 mg per day for a total of about 260 days. The symptoms exacerbated to a maximum in a month. When the metoclopramide administration was discontinued, the abnormal movements gradually improved to a considerable extent. Attention to the possible induction of specific tardive dyskinesia is called for in the use of this drug.

Dyskinesia, Drug-Induced↗

Trismus-pseudocamptodactyly syndrome (Hecht-Beals' syndrome): case report and literature review.

The trismus-pseudocamptodactyly syndrome (TPS) is a rare condition inherited as an autosomal dominant trait with variable expressivity. Clinically the syndrome is characterized by decreased ability to open the mouth and curvature of the fingers at the level of interphalangic joints while attempting dorsiflexion of the wrist (pseudocamptodactyly). A 6-year-old male patient from Guatemala with this syndrome is presented. The surgical treatment consisted of bilateral coronoid amputation to resolve the pseudotrismus. The procedure was successful and the patient achieved normal function.

Abnormalities, Multiple↗

[A case of hemifacial oculo-oral anomalies].

In the present work is described a female with an incomplete oral duplication. The occurrence of a supernumerary mouth is rare. Moreover, these cases present other anomalies that make each one relatively unique. In this context, in fact, the patient presented an association of oral manifestation and ocular anomalies. From a pathogenetic point of view, maxillary duplication probably arises from a partial doubling of growth centers around the stomodeal plate.

Abnormalities, Multiple↗

Temporomandibular joint abnormalities and bite force in a group of adults with rheumatoid arthritis.

Maximal mouth opening, translatory condylar motion (radiographically assessed) and bite force in sixteen adults with rheumatoid arthritis and radiographic temporomandibular joint abnormalities were compared with data from sixteen individuals without joint disease. Mean mouth opening in the arthritis group was reduced, but not statistically significant. Condylar translation and bite force showed significantly lower values than in the healthy individuals. Maximal mouth opening seemed to be the most uncertain parameter for the estimation of jaw function. In seven patients with a normal mouth opening (40-46 mm), both the condylar translation and the bite force were significantly impaired.

Adult↗

Medical management of disorders of swallowing.

Disorders of swallowing in a given patient can result from abnormalities of dentition, the oral cavity (mouth and pharynx), the cricopharyngeal muscle, esophageal skeletal (proximal) or smooth (distal) muscle, the lower esophageal sphincter, and the stomach. Recent advances include refinements in esophageal manometric techniques with reclassification of motility disorders; newer drugs to treat gastroesophageal reflux; recognition of more frequent infections of the esophagus in immunosuppressed patients; and adaptation of the use of medications such as nitrates and calcium-channel blockers, which were previously primarily cardiotonic, for investigative treatment of esophageal motility abnormalities.

Combined Modality Therapy↗

Gastrointestinal transit disorders in patients with insulin-treated diabetes mellitus.

Gastric emptying, mouth-to-cecum transit and whole gut transit of a solid-liquid meal were measured in 43 insulin-treated diabetics and in 30 control subjects by using scintigraphic techniques, the hydrogen breath test and stool markers. In the diabetics various parameters including duration of diabetes, gastrointestinal symptoms and complications such as autonomic neuropathy, peripheral neuropathy and proteinuria were determined and related to gastrointestinal transit times. Gastric emptying was significantly prolonged in diabetics as compared to the control group (p less than 0.05) with 35% of the diabetics disclosing abnormally delayed gastric emptying, whereas no significant overall differences were observed between diabetics and controls concerning mouth-to-cecum transit and whole gut transit time. However, abnormally prolonged mouth-to-cecum transit was detected in 23% and delayed whole gut transit in 26% of the diabetics (p less than 0.02 as compared to the control group). There was a significant correlation of dyspeptic symptoms and diarrhea with prolonged gastric emptying (p less than 0.001). Gastric emptying, but not mouth-to-cecum transit or whole gut transit was significantly related to autonomic nerve dysfunction (p less than 0.001) and peripheral neuropathy (p less than 0.02). Furthermore, gastric emptying and WGT were significantly correlated to proteinuria (p less than 0.03). Using a linear regression model, autonomic neuropathy, diarrhea and dyspeptic symptoms were the major parameters in predicting delayed gastric emptying. It is concluded that in diabetics different compartments of the gut are affected by gastrointestinal motor abnormalities and that these segments are probably regulated by independent or different control mechanisms.

Adult↗

[The role of orthodontic surgery in mouth rehabilitation].

The surgical treatment of malformations of jaws offers actually many possibilities. Basic principles of this specialized surgery are exposed: an exact diagnosis, the problem of incisions which must be hidden, age upon operation and association with other specialties. In the oral rehabilitation, this type of surgery permits total correction of complex malformations, which are impossible to treat completely by other methods.

Adult↗

Role of magnetic resonance imaging in the clinical diagnosis of the temporomandibular joint.

Temporomandibular joint (TMJ) abnormalities cannot be reliably assessed by a clinical examination. Magnetic resonance imaging (MRI) may depict joint abnormalities not seen with any other imaging method and thus is the best method to make a diagnostic assessment of the TMJ status. In patients with temporomandibular joint disorder (TMD) referred for diagnostic imaging the predominant TMJ finding is internal derangement related to disc displacement. This finding is significantly more frequent than in asymptomatic volunteers, and occurs in up to 80% of patients consecutively referred for TMJ imaging. Moreover, certain types of disc displacement seem to occur almost exclusively in TMD patients, namely complete disc displacements that do not reduce on mouth opening. Other intra-articular abnormalities may additionally be associated with the disc displacement, predominantly joint effusion (which means more fluid than seen in any asymptomatic volunteer) and mandibular condyle marrow abnormalities (which are not seen in volunteers). These conditions seem to be closely related. Nearly 15% of TMD patients consecutively referred for TMJ MRI will have joint effusion, of whom about 30% will show bone marrow abnormalities. In a surgically selected material of joints with histologically documented bone marrow abnormalities nearly 40% showed joint effusion. Disc displacement is mostly bilateral, but joint effusion seems to be unilateral or with a lesser amount of fluid in the contralateral joint. Abnormal bone marrow is also mostly unilateral. Many patients have unilateral pain or more pain on one side. In a regression analysis the self-reported in-patient TMJ pain side difference was positively dependent on TMJ effusion and condyle marrow abnormalities, but negatively dependent on cortical bone abnormalities. Of the joints with effusion only one fourth showed osteoarthritis. Thus, there seems to be a subgroup of TMD patients showing more severe intra-articular pathology than disc displacement alone, and mostly without osteoarthritis. It should, however, be emphasized that patients with TMJ effusion and/or abnormal bone marrow in the mandibular condyle seem to constitute only a minor portion (less than one fourth) of consecutive TMD patients referred for diagnostic TMJ imaging. The majority of patients have internal derangement related to disc displacement, but without accompanying joint abnormalities. In patients with rheumatoid arthritis and other arthritides TMJ involvement may mimick the more common TMDs. Using MRI it is possible, in most cases, to distinguish these patients from those without synovial proliferation.

Adult↗

Craniopagus parasiticus: a case report of a parasitic head protruding from the right side of the face.

An extremely rare case of parasitic head protruding from the right side of the face is presented. It differs from all the other cases previously reported in the literature. The patient had a parasitic head with an abnormal brain, two eyebrows, two underdeveloped eyes, nose, mouth, 12 teeth, a tongue and plenty of hair. The parasitic head was excised. The nature of the embryological abnormalities is discussed and the literature of polygnathism reviewed.

Abnormalities, Severe Teratoid↗

Third case of Pfeiffer-type cardiocranial syndrome.

We report on a third child with a newly recognized craniosynostosis syndrome involving the sagittal suture. Additional findings not related to the abnormal head growth include micrognathia with limited mouth opening, tracheobronchial anomalies, congenital heart defects, microphallus, cryptorchidism, and growth and mental retardation.

Abnormalities, Multiple↗

[Duplication of mouth and mandible. Apropos of a case].

We report a case of duplication of the mouth, tongue, mandible, and cervical spine in a female neonate. Findings in this patient were somewhat different from those in previously reported cases. Clinical, radiological and therapeutic features in this localized duplication are reviewed. Embryologic hypotheses include defective midline development, such as in the split notochord syndrome, and development of totipotent cells from the first branchial arch.

Abnormalities, Multiple↗

Brachmann-de Lange syndrome. Delineation of the clinical phenotype.

A total of 31 cases previously diagnosed as having Brachmann-de Lange syndrome were ascertained and examined, of which 11 were thought to have been misdiagnosed. Of those correctly diagnosed, there appeared to be a phenotypic dichotomy with classical and mild cases. Those facial findings of greatest diagnostic value were the combination of the characteristic eyebrows, long philtrum, thin lips and crescent-shaped mouth. The characteristic eyebrows were neat, well defined and arched as though they had been pencilled. This combination of anomalies was absent in postpubertal males but not in postpubertal females. Facial abnormalities most likely to lead to incorrect use of the eponym were hypertrichosis, synophrys, and bushy eyebrows.

Adolescent↗

Agnathia-holoprosencephaly: a developmental field complex involving face and brain. Report of 3 cases.

Agnathia-holoprosencephaly (A-H) is a developmental field complex involving a graded series of defects in the jaws, mouth, tongue, ears, eyes, and brain. Two general groups can be recognized: agnathia with holoprosencephaly (more severe) and agnathia without holoprosencephaly (less severe). This report describes three new cases of agnathia without holoprosencephaly and reviews the recent literature. By combining published cases with those ascertained through a survey of genetic centers in the United States, it appears that there have been at least 24 occurrences of A-H in the past 25 years. An inductive defect of the prechordal mesoderm that also affects neural crest cells is presented as the cause for this developmental field complex. Because of the etiologic heterogeneity associated with developmental field defects, the genetic counselor must provide a wide range of recurrence risks when dealing with the A-H complex.

Abnormalities, Multiple↗

Congenital oral mucosal abnormalities in true umbilical cord knots.

OBJECTIVE: The pathogenesis and clinical significance of true umbilical cord knots remain controversial. Here, we tested the hypothesis of the presence of congenital oral mucosal changes in newborns with true umbilical cord knots. STUDY DESIGN: Seven consecutive infants with true umbilical cord knots and 50 gestational age- and sex-matched controls were enrolled. The proportion of oral frenulum abnormalities and the two-dimensional vascular network geometry [fractal dimension, D, at two scales: D(1-46), and D(1-15), with the relative Lempel-Ziv complexity, (L-Z)], were analyzed. RESULTS: Infants with true umbilical cord knots showed significantly higher proportions of mandibular frenulum agenesis compared to controls (p = 0.000006). The oral vascular networks of these infants exhibited a significantly higher D(1-46) and D(1-15) (p < 0.0001, respectively), and higher L-Z values (p < 0.0001) than control networks. CONCLUSION: These findings indicate the presence of significant congenital oral mucosal changes in newborn infants with true umbilical cord knots, thus suggesting a previously unrecognized association between true umbilical cord knots and a subclinical extracellular matrix disorder.

Female↗

Dental findings and rehabilitation in familial osteodysplasia (Anderson type): a case report.

Familial osteodysplasia is a disorder of osteogenesis with an autosomal recessive pattern of inheritance which predominantly affects facial bones. No recent case had been reported, particularly from a dental point of view since the syndrome was first described by Anderson et al (JAMA 1972;220:1687-93). A 23-year-old male with familial osteodysplasia was presented in maxillofacial and dental aspects with clinical and radiological manifestations including malocclusion, abnormal teeth alignment, impacted teeth, shape disturbances including uncompleted coronal formation, root shortening with bulbous form, high angled mandible and elongation of the corpus of mandible. Recognition of the syndromal features prior to any dental intervention is of paramount importance because of increased inclination to spontaneous mandibular fractures. Hence, no surgical intervention was performed for impacted teeth. Following the extractions of severely mobile teeth, a definitive restoration was fabricated as distal-extension removable partial dentures with conus crown telescopic system. The aesthetic and functional outcome was satisfactory for the patient. In conclusion, dentists appear to play an important role in the recognition of familial osteodysplasia, based on maxillofacial and dentoalveolar findings. Awareness of the syndromal features, especially of spontaneous fractures, would detect the limitations for dental interventions and treatment planning.

Adult↗

Dup(1q)(q42-->qter) syndrome: case report and review of literature.

We report on a patient with primordial growth retardation, mental retardation, and minor anomalies (triangular face, open sagittal suture, frontal bossing, telecanthus, upturned nose, micrognathia, and small mouth with downturned corners). The diagnosis of Russell-Silver syndrome (RSS) had been considered but was abandoned when cytogenetic evaluation showed a partial trisomy 1q or duplication 1q (46,XY,15, + der(15)t(1;15)(q42;qter). Data from another 5 reports of dup(1)(q42-->qter) do not allow delineation of a typical syndrome. However, individuals with dup(1q), del(15q), and Russell-Silver syndrome share common manifestations (i.e., low birth weight, growth retardation, triangular face, low set/abnormal ears, micrognathia, renal anomalies.

Abnormalities, Multiple↗