Glandular odontogenic cyst (sialo-odontogenic cyst): report of two cases and literature review of 45 previously reported cases

The clinical, radiological, histopathological and immunohistochemical (cytokeratin) features of two cases of glandular odontogenic cyst (GOC) are presented and discussed in a review of 45 cases of GOC hitherto reported. Of cases with available information, 34 occurred in the mandible and 6 in the maxilla; the male:female ratio was 19:28, and the mean age was 46.7 years in males and 50.0 years in females. Six cysts recurred once after 2-8 years (mean 2 years 8 months) and 2 (5.3%) recurred twice after 2 and 5 years and after 3 and 5 years, respectively, giving a rate of recurrence of 21%. The identification of osteodentin in one of the present cases and the co-expression of cytokeratins (CK) 13, 19 and 8 strongly support the concept of odontogenic differentiation in the GOC. Careful surgical removal of the lesion succeeded by a 5-year follow-up period is recommended.     

The nephrotic syndrome (a follow up study of 32 cases)

Groups of children with different IQ levels were trained to discriminate between stimulus cards containing two or more pictures. In subsequent testing the pictures were presented singly in order to determine how many pictures the child had responded to during discrimination training. The data showed that the lower the IQ, the fewer pictures the child responded to. We speculated that intellectually retarded functioning may be related to problems in overselective attention; the lower a person's IQ level, the less of the environment becomes functional in controlling his behavior.     

Acromesomelic dwarfism: description of a patient and comparison with previously reported cases

SPF-male rats were treated intraperitoneally with phenobarbital (30 mg/kg/day) or 0.9% sodium chloride for 4 days. Endogenous creatinine clearance in conscious rats and inulin clearance in nondiuretic rats under inactin anesthesia were measured 24 hr following the last injection. In an additional group of treated rats diuresis was induced by sodium chloride and mannitol and then inulin- and PAH clearance were measured simultaneously. Following 4 days treatment with phenobarbital, endogenous creatinine clearance and inulin clearance were not significantly different in control and phenobarbital-treated animals. In contrast significant changes were found in urine volume and PAH clearance in the phenobarbital-treated animals. These results do not reflect any change in glomerular filtration rate, but might be attributed to an increase in renal plasma flow or an activated tubular transport system following phenobarbital administration.     

Acute brachial neuritis (Parsonage-Turner syndrome): MR imaging appearance--report of three cases

Magnetic resonance (MR) imaging findings in three patients with acute onset of neuritic shoulder pain and weakness included high signal intensity in supra- and infraspinatus muscles (n = 2), partial involvement of infraspinatus muscle (n = 1) and of deltoid muscle (n = 1), and atrophy of supra- and infraspinatus muscles (n = 2). Clinical diagnosis of acute brachial neuritis (Parsonage-Turner syndrome) correlated with MR imaging results in all cases.     

Orbital phlebography evaluation in 8 cases of Tolosa-Hunt syndrome

Tolosa-Hunt syndrome (THS), or painful ophthalmoplegia is associated to a non-specific granulomatosis of unknown etiology, that involves the superior orbital fissure and its nervous and vascular structures. The clinical picture that responds to steroid therapy, is variable and is always associated with pain. Inflammatory conditions, tumors and aneurysms can produce similar symptoms. Computed tomography, cerebral angiography and orbital phlebography are the imaging methods of choice for making the diagnosis. We revised the results of these radiological examinations of eight patients seen at the Hospital S?o Paulo from 1989 to 1991, with the diagnosis of THS according to Hunt and Hannerz criteria. The analysis of orbital phlebographic changes based upon Hannerz et al. systematization showed non-specific features, but those were able to help the diagnosis.     

Joubert syndrome: a report of 5 cases

OBJECTIVE: To review clinical features, radiological findings and prognosis in Joubert syndrome. MATERIAL AND METHODS: We report 5 children (3 male and 2 female) with the diagnosis of Joubert syndrome by clinical and radiological findings. They were diagnosed in the first year of life, in the Hospital Infantil La Paz (Madrid, Spain), from 1971 to 1996. Three patients have already been published, and here, we report two new cases. RESULTS: Partial absence of the cerebellar vermis, hypotonia and developmental delay were seen in all patients. Other cardinal findings were episodic hyperpnoea (5/5) with periods of apnoea (2/5), abnormal eye movements (2/5) and strabismus (3/5), tongue protrusion (2/5), seizures (1/5), hemifacial spasms (1/5) and occipital meningocele (2/5). Clinical manifestations were first noticed soon after birth. Two patients died in the first 5 years of life, and the rest of the cases actually show severe mental retardation. CONCLUSIONS: Joubert syndrome is a rare and probably underdiagnosed syndrome with bad prognosis. This inherited condition is characterized by agenesis of the cerebellar vermis, mental retardation, hypotonia, episodic hyperpnoea and abnormal eye movements. Additional manifestations have been reported since the original cases were described.     

Wallenberg syndrome: a review of 25 cases

INTRODUCTION: Wallenberg's syndrome is one of the most common clinically recognized conditions due to brain stem infarct, which can nowadays be identified thanks to modern neuro-imaging techniques. We present a retrospective study of 25 cases admitted to our hospital in the past eight years, to evaluate epidemiological aspects and clinical findings and to correlate these with magnetic resonance topography. MATERIAL AND METHODS: Patients diagnosed in the Neurology Section of Hospital Xeral-Calde in Lugo between January 1989 and December 1997 as having Wallenberg's syndrome. RESULTS: There was a predominance of middle aged men presenting at 55.06 years of age (range 30- 78). Arterial hypertension was the main risk factor (52%). There was a progressive form of onset in most cases. The commonest symptom was dysphonia followed by dysphagia. The commonest finding on physical examination was ataxic gait (24 patients) MR was positive in 22 of the 23 cases in which this was done. There were different clinical findings depending on the site of the lesion, whether rostral, caudal or medial. This is considered in the discussion. In most cases the prognosis was good. The commonest sequel was ataxia. CONCLUSIONS: The results are similar to those in the literature. We emphasize the excellent correlation of clinical data and neuroimaging findings.     

Nondiagnostic CT-guided stereotactic biopsies in a series of 407 cases: influence of CT morphology and operator experience

Nondiagnostic biopsies were analyzed in a consecutive series of 407 patients undergoing computerized tomography (CT)-guided stereotactic biopsies. These were categorized as either negative biopsies, when normal tissue or nonspecific pathology was found, or inconclusive, when a definitive diagnosis could not be made although representative tissue was obtained. Nineteen biopsies (4.7%) were negative and 10 (2.4%) were inconclusive, giving an overall nondiagnostic biopsy rate of 7.1% (29 of the 407 cases). Suspected neoplastic masses (390 cases) were classified on the basis of their CT morphology into four groups: Group 1 included purely hypodense nonenhancing masses; Group 2 included isodense nonenhancing masses; Group 3 included ring-enhancing masses; and Group 4 included mixed-density enhancing masses. Although a higher proportion of hypodense nonenhancing masses (six of 56, or 10.7%) yielded a negative result, there was no statistically significant difference in the negative biopsy rates for the different CT categories (p = 0.06). The negative biopsy rates for the 6 years of the study, 1987 to 1992 (1987 being an incomplete year) were as follows: 13.3%, 6%, 3.2%, 3%, 5.8%, and 2.7%. There was no significant decrease in the negative biopsy rate as experience with this procedure increased (p = 0.20). A total of eight surgeons independently performed the biopsies. There was no significant difference (p = 0.24) in the negative biopsy rate of the surgeon with the most experience (124 biopsies, 2.4% negative biopsy rate) compared with that of the seven other surgeons combined (283 biopsies; 5.7% negative biopsy rate). These findings suggest that the yield in a stereotactic biopsy is independent of the CT appearance of the mass. Adherence to certain basic principles in patient and target selection will ensure a reasonable percentage of positive yield with stereotactic biopsy procedures even if the surgeon is relatively inexperienced. There does not appear to be a learning curve in the performance of CT-guided stereotactic biopsies. The management of patients with nondiagnostic biopsies is discussed.     

Comments on a commentary: statistical evaluation of split mouth caries trials

When results of split mouth caries trials are reported, the most frequently used outcome measures are "effectiveness" and "net gain". In a recent review by Riordan & FitzGerald (Community Dent Oral Epidemiol 1994;22:192-7) of the statistical analysis of data from such trials, the close connection between effectiveness and the traditional epidemiological outcome measure, relative risk, was pointed out and the latter measure was recommended. The confidence intervals for these parameters were, however, incorrect. This note provides valid confidence intervals for net gain, effectiveness and relative risk based on data from split mouth trials and presents examples of the calculations.     

Methodological and statistical issues of quality of life (QoL) and economic evaluation in cancer clinical trials: report of a workshop

In recent years, quality of life (QoL) and economic evaluations have become increasingly important as additional outcome measures in cancer clinical trials. However, both fields of research are relatively new and in need of finding solutions to a substantial number of specific methodological problems. This paper reports on the proceedings of a symposium aimed at summarising and discussing some of the most contentious methodological and statistical issues in QoL and economic evaluations. In addition, possible solutions are indicated and the most pertinent areas of research are identified. Issues specific to QoL evaluations that are addressed include clinically meaningful changes in QoL scores; how to analyse QoL data and to handle missing and censored data and integration of length of life and QoL outcomes. Issues specific to economic evaluations are the advantages and disadvantages of various outcome measures; statistical methods to analyse economic data and choice of decision criteria and analytical perspective. How to perform QoL and economic evaluations in large and simple trials and whether the gap between QoL and utility measures can be bridged are also discussed.     

Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases

Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed.     

Mucopolysaccharidosis III (Sanfilippo syndrome) type B: cranial imaging in two cases

We present the imaging findings in two patients with mucopolysaccharidosis III (Sanfilippo syndrome) type B, both with arachnoid cysts. We postulate that the deposition of glycosaminoglycans in the meninges may impair CSF flow and explain the development of arachnoid cysts also noted in patients with other forms of mucopolysaccharidoses.     

Nodular cutaneous lupus mucinosis: report of a case and review of previously reported cases

OBJECTIVE: The study was designed for the analysis on data of 51 patients with macular branch retinal vein occlusion (McBRVO) and the comparison between them and 210 patients (214 eyes) with major branch retinal vein occlusion (MjBRVO). METHODS: Fluorescein angiography, ophthalmoscope and slit-lamp were used for the examination of the venous obstructive site and the locations of artery and vein. RESULTS: (1) The incidence of McBRVO was 19.5% (51/261) in branch retinal vein occlusion. (2) The site of occlusion: the incidence of McBRVO at supero-temporal branches was 72.5% (37/51) and the incidence of MjBRVO was 73.4% (157/214). (3) The involved branch at the first macular branch accounted for 39.6%, the second branch 58.3%, and the third branch 2.1%. The artery anterior to the vein at arteriovenous crossing at the obstructive site accounted for 93.0% in the McBRVO group and 95.0% in the MjBRVO group. (4) The complications and visual prognosis: The incidence of cystoid macular edema was 20.0% in McBRVO group and that was 28.5% in the MjBRVO group. No neovascularization was found in the McBRVO group, while 37.9% neovascular formation was found in the MjBRVO group. The visual acuity recovered to 0.5 or better was in 88.2% in the McBRVO group, while 57.0% in the MjBRVO group (P < 0.005). CONCLUSION: There are no significant differences in sex, age, laterality, pathogenesis as well as occlusion site between the McBRVO and MjBRVO groups, but the visual prognosis is better in the former than that in the latter group.     

Congenital lower lip pits (Van der Woude syndrome): presentation of 10 cases

Congenital lower lip pits are a rare developmental malformation of the lower lip. Clinically they present as bilateral depressions in the vermilion zone of the lower lip. It is important to be aware of this disorder because lower lip pits have also been reported in a variety of other congenital disorders and are associated with other malformations. Ten cases of this entity are presented. Nine of the 10 patients are members of two related families. The clinical and pathologic picture as well as some of the genetic and therapeutic aspects of this peculiar condition are discussed.     

The Peutz-Jeghers syndrome: a report of 3 cases

Three cases of the Peutz-Jeghers syndrome are reported and the literature is reviewed. The difficulties in the management of these patients are described and the importance of radiology and endoscopy is emphasised.