The application of computerized tomography (CT) to the dental ageing of children and adolescents.

INTRODUCTION: Following a mass disaster, the aim of the Disaster Victim Identification process is to establish the identity of the victims. The ageing screening process on victims in Victoria may now be complemented with the use of computerized tomography (CT), where previously any dental ageing analysis was performed using conventional radiographs. The aim of this study was to assess the accuracy of age estimation using the dental ageing method proposed by Moorrees, Fanning and Hunt (MFH) using CT images. Intra- and inter-rater variability between two raters, one experienced and one inexperienced, was also assessed. MATERIALS AND METHODS: The two raters were blinded to the ages of 96 deceased Australian children aged up to 15 years. Using three-dimensional (3D) shaded surface displays (SSD) and reformatted CT images, the age was first estimated based on prior experience alone, followed at a later date by the age estimation utilizing the MFH method. These estimates were then compared to the known chronological age. The results were statistically analyzed in a one-sample t-test, using the mean log-ratio of the estimated age to the chronological age. RESULTS: Our findings show that the experienced rater was more accurate in age estimation than the less experienced when using prior experience (p<0.0001). The use of reformatted CT images to perform an ageing estimate using the MFH method was found to systematically underestimate the chronological age by 10% by both raters (p=0.784). There was no significant difference between the two raters. Intra-rater reliability was high (p=0.135). CONCLUSIONS: CT can provide accurate estimates of dental ages. Prior experience with dental ageing and/or CT improves the accuracy. However, with the use of validated ageing charts, inexperienced raters can also achieve accurate age estimates using CT images.

Assessing the new general competencies for resident education: a model from an emergency medicine program.

The Accreditation Council for Graduate Medical Education (ACGME) has promoted six areas that should be addressed during graduate medical training, or "general competencies" (GCs). According to the ACGME, these GCs should be reflected in the educational processes of all residency programs. In promulgating these competencies, however, the ACGME has not provided examples of core content, methods of implementation, or methods of evaluation. The authors propose a practical method for modifying an existing evaluation format, providing a template other programs could use in assessing residents' acquisition of the knowledge, skills, and attitudes reflected in the GCs.

Parkinson's disease with late Pick's dementia.

We report a case in which typical clinical features of idiopathic Parkinson's disease existed for seven years prior to the development of significant behavioral and cognitive changes and severe dementia. The patient presented with right-sided resting tremor, bradykinesia, and rigidity, which were highly responsive to levodopa. Serial neuropsychological evaluation revealed no evidence of dementia until late in the disease. The patient deteriorated rapidly eight years into the disease, requiring full care. She died 16 years after symptom onset and post-mortem neuropathological analysis revealed Lewy body Parkinson's disease and Pick's disease. To our knowledge, this is the first non-familial case with this combination of clinical history and pathologically confirmed disease to be reported in the literature. The absence of a family history of any neurological disease sets this case apart from the recently described genetic cases of frontotemporal dementia with Parkinsonism linked to chromosome 17. In addition, the relatively late onset of dementia in frontotemporal dementia is atypical. While there is considerable debate regarding the cause of dementia in idiopathic Parkinson's disease, our case illustrates that Pick's disease is one such cause.

Effect of calcium source, dietary calcium concentration, and gestation phase on various bone characteristics in gestating gilts.

Sixty gravid crossbred gilts were allotted to a 2 x 3 x 2 factorial arrangement of treatments: two Ca sources (sun-cured alfalfa meal and CaCO3), three dietary concentrations of Ca (50, 75, and 100% of NRC requirements), and two phases of gestation (55 and 105 d). The objectives were to determine the effect of Ca source, dietary Ca concentration, and gestation phase on bone characteristics (bone breaking strength, bone ash percentage, bone density, and bone ash density in the rib, thoracic, and coccygeal bones), to correlate bone responses to determine relative bone activity, and to determine reliability of the coccygeal bones as indicators of Ca status in the body. At 55 d, rib strength and coccygeal ash content were lower (P < .01) than at 105 d of gestation. A gestation phase x Ca concentration (P < .05) interaction occurred. As Ca concentration increased, thoracic strength and rib ash responded quadratically during each gestation phase, for which at 55 d a minima and at 105 d a maxima was produced at 75% of NRC. A Ca source x Ca concentration (P < .05) interaction occurred. Gilts fed alfalfa had the lowest rib bone and ash density when fed 75% of NRC for Ca, whereas gilts fed CaCO3 were highest at this level of Ca compared with the other concentrations. Generally, all bones were positively correlated with respect to their response to dietary Ca concentration. Few negative correlations were observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Mitochondrial encephalomyopathy: variable clinical expression within a single kindred.

The clinical manifestations of mitochondrial encephalomyopathy are described in four generations of a single kindred. The age of onset of major neurological disturbance varied from 3-70 years. In some patients, deafness was the only manifestation; in others, recurrent bouts of status epilepticus associated with focal neurological deficits and headache, caused severe disability or death. Examples of all three adult forms of mitochondrial encephalomyopathy: MELAS, MERFF and Kearns Sayre syndrome, were represented within the kindred. Associated features included deafness, short stature, non-insulin-dependent diabetes mellitus, migraine, peptic ulceration and severe constipation. The nt 3243 A-G MELAS mutation was detected in two members of the kindred. This study highlights the diversity of clinical expression of a mitochondrial mutation within a single kindred.

Bioavailability of calcium in sun-cured alfalfa meal and effect of dietary calcium concentration on bone and plasma characteristics during two phases of gestation in gilts.

A study was conducted with gravid gilts to determine the bioavailability of Ca in sun-cured alfalfa meal (AM) and the effect of dietary Ca concentration on bone and blood characteristics during two phases of gestation. Two Ca sources (AM and CaCO3), three dietary concentrations of Ca (50, 75, and 100% of the NRC requirement), and two gestation phases (55 and 105 d) were used in a 2 x 3 x 2 factorial arrangement in a randomized design with five replications (60 gravid gilts). Response criteria were as follows: 1) plasma Ca, P, and alkaline phosphatase (AKP) measured at the onset and at 25-d intervals and 2) metacarpal (MC) and metatarsal (MT) bone breaking strength (kilograms), ash content (percentage), density (grams/cubic centimeter), and ash density (grams of ash/cubic centimeter) at the conclusion of the experiment. Plasma Ca, P, and AKP concentrations were similar between Ca sources. Because the response between Ca sources was similar, the data were pooled among sources. There was a linear decline in plasma P and AKP (P < or = .05) as dietary Ca concentration increased. As gestation progressed from 0 to 100 d, there was a decline (P < .05) in plasma Ca and P. There were no differences in bone breaking strength and ash between Ca sources in either the MC or MT. No differences in bone strength between gestation phases occurred. A gestation phase x dietary Ca concentration interaction (P < .05) was observed for bone ash in both bones.(ABSTRACT TRUNCATED AT 250 WORDS)

Goodley polyaxial cervical traction. A new approach to a traditional treatment.

A cervical traction system is discussed that offers advantages over traditional methods of applying cervical traction. The major advantage of this system is that it allows precise application of traction to only the restricted spinal segments. Because of this specificity, traction may be applied at reduced force, decreasing the risk of injury. The traction apparatus is adjusted easily to the individual and may be used in the clinic or at home. My experience with the apparatus has shown greater patient compliance, resulting in increased treatment effectiveness, when compared with traditional traction methods. Four representative case studies demonstrate the effectiveness of this traction system. Closely controlled studies are needed to substantiate the effectiveness and safety of this traction system.

Psychometric and cranial CT study in myotonic dystrophy.

Twenty-two adults with myotonic dystrophy were assessed for evidence of intellectual impairment and cranial CT abnormalities. Psychometric evaluation demonstrated significantly lower verbal and performance IQs in the MD subjects than in matched normal controls. The reduced performance IQ was not simply related to loss of fine motor skills. No significant differences were found in memory and personality assessments between the MD and control groups. Lateral ventricular surface area was measured on CT scans using a semiautomated image analysis technique and was found to have a positive correlation with age in a control group of 45 normal scans (r = 0.833, P less than 0.001). This correlation was lacking in the MD group of subjects (r = 0.345, P greater than 0.05), in eight of whom the ventricular surface area was above the upper limit for age found in the control group. Asymptomatic areas of focal atrophy were found in two subjects. There was no correlation between ventricular size and severity or duration of the disease or verbal or performance IQ.

Lymphocyte capping in myotonic dystrophy.

Lymphocyte capping with antihuman immunoglobulin was studied in 16 cases of myotonic dystrophy, including two cases with the congenital form. Percentage capping after 1 hour incubation was reduced and the time course of the capping sequence was apparently delayed. The significance of these findings remains to be determined.

Immunological studies in myotonic dystrophy.

Abnormalities of cellular and humoral immunity have been recognised in myotonic dystrophy but their significance is uncertain. To throw further light on this, a group of 22 patients with myotonic dystrophy was investigated, looking specifically for evidence of abnormalities of B lymphocyte function and of disturbed immunoregulation. The previously reported reduction in serum IgG levels was confirmed and, in addition, serum IgA levels were significantly reduced. Autoantibodies to muscle and non-muscle antigens were not found and there was no increase in autoimmune disease or malignancy, suggesting that immunoregulatory mechanisms are not disturbed. The proportions of T and B cells were not significantly different in patients and controls. B-lymphocyte capping with FITC- antihuman IgG was quantitatively altered, the percentage of capped cells after incubation being reduced and the time-course of the capping process being apparently prolonged in the myotonic dystrophy subjects. While there are a number of possible explanations for these findings, they may reflect an intrinsic abnormality of the lymphocyte membrane. This could be part of a more generalised membrane defect which has been suggested to underlie the myotonia and certain other manifestations of this multisystemic disorder.

A search for genetic influence in idiopathic inflammatory myopathy.

33 patients with idiopathic inflammatory myopathies (polymyositis or dermatomyositis) and 45 of their first-degree relatives were investigated in a search for any influence of genetic factors in these diseases. None of the relatives had evidence of an inflammatory myopathy but 13 had some other autoimmune disease. Mean serum IgG levels were reduced and serum C3c concentration increased both in patients and relatives. Levels of IgM were reduced and C4 increased in some groups of patients. The incidence of autoantibodies was increased in the patient group, particularly in those with isolated dermatomyositis or other systemic features, but not in the relatives. HLA, blood group and other genetic markers showed no deviation from normal population frequencies. Evidence favouring a genetic influence on the etiology of idiopathic inflammatory myopathy is restricted to disturbance of levels of some Ig and complement components, and for these there may be other explanations.

Hypercalcaemia associated with cerebral vasospasm causing infarction.

Central nervous system disorders are not uncommon in patients with hyperparathyroidism and hypercalcaemia. Usually these consist of neuropsychiatric disturbances but acute encephalopathies and seizures may occur. A rare manifestation is cerebral infarction. A patient is presented with neuroradiological evidence of infarction caused by cerebral arterial spasm which appears related to hypercalcaemia due to hypervitaminosis D. Arterial spasm is suggested as a possible aetiological factor in focal neurological lesions associated with hypercalcaemia.

Cerebral arteritis in cat-scratch disease.

Acute right hemiplegia and transient expressive aphasia occurred in a 7-year-old girl a few days after nonspecific constitutional symptoms and the appearance of a large right submandibular lymph node. Biopsy of this node and lack of other evident cause suggested a diagnosis of cat-scratch disease. Carotid arteriography showed a localized arteritis of the supraclinoid part of the left internal carotid artery and the left middle cerebral artery, involving also some lenticulostriate vessels. Computerized tomography demonstrated infarction in the left internal capsule. The size of this infarct and the angiographic abnormalities improved 6 weeks after onset, and coincided with clinical recovery. Cat-scratch disease may have caused the localized arteritis.

Progressive polyradiculoneuropathy: treatment with Azathioprine.

A case is presented of a 64-year-old male with chronic inflammatory polyradiculoneuropathy (CIP) relentlessly progressing (despite steroids) to virtually complete quadriplegia over seven months. Once commenced on Azathioprine he dramatically improved over a six week period. The truly progressive form of CIP is rare. It is, however, immunologically similar to relapsing CIP and to the more common acute inflammatory polyradiculoneuropathy (AIP). Although the place of steroid therapy is still in doubt it would seem that cytotoxic immunosuppressives have something definite to offer in these conditions where there is progression beyond 4-5 weeks. In the largest series of CIP available (where the patients were untreated, or treated with steroids alone) the mortality rate was 11%, and the "complete recovery" rate only 5%. Although only isolated case reports are available, it would seem that if more aggressive immunosuppressive therapy was used more frequently, the prognosis might be considerably improved.

Neurological features of polyarteritis nodosa.

A series of 20 cases of polyarteritis nodosa (PAN) is presented. Clinical features of 2 cases are described in more detail to illustrate the most common neurological complications. PAN is an uncommon disease but one which usually presents with some form of neurological disturbance, often a mononeuritis multiplex or a symmetrical polyneuropathy. Diagnosis requires a high degree of clinical suspicion as serological tests are at best non-specific, and absolute biopsy proof often requires examination of multiple sites. Early diagnosis is important, as treatment with corticosteriods usually induces symptomatic relief and provides a better chance of long term survival. If an acute remission is obtained the patient not only should have a good quality of life but in a proportion of cases, where the remission is prolonged, steroid therapy may be ceased.