Parageusia: an unusual presentation of multiple sclerosis.
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Biomedical subjects
Publications and source records attributed to A Castriota-Scanderbeg.
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In an attempt to clarify whether T1 relaxation time mapping may assist in characterizing the pathological brain tissue substrate of multiple sclerosis (MS), we compared the T1 relaxation times of lesions, areas of normal-appearing white matter (NAWM) located proximal to lesions, and areas of NAWM located distant from lesions in 12 patients with the relapsing-remitting and 12 with the secondary progressive (SP) subtype of disease. Nine healthy volunteers served as controls. Calculated mean T1 values were averaged across all patients within each clinical group, and comparisons were made by means of the Mann-Whitney U-test. Significant differences were found between all investigated brain regions within each clinical subgroup. Significant differences were also detected for each investigated brain region among clinical subgroups. While T1 values of NAWM were significantly higher in patients with SP disease than in normal white matter (NWM) of controls, no differences were detected when corresponding brain areas of patients with RR MS were compared with NWM of controls. T1 maps identify areas of the brain that are damaged to a different extent in patients with MS, and may be of help in monitoring disease progression.
To study the sensitivity of intermolecular double quantum coherences (iDQc) imaging contrast to brain microstructure and brain anisotropy, we investigated the iDQC contrast between differently structured areas of the brain according to the strength and the direction of the applied correlation gradient. Thus diffusion-weighted imaging (DWI) and diffusion tensor imaging (DTI) maps have been obtained. This procedure, which consists of analyzing both iDQc and DWI images at different gradient strength and gradient direction, could be a promising tool for clinical brain investigations performed with higher than 1.5 T magnetic fields.
We studied five patients with multiple sclerosis with one plaque of demyelination more than 2 cm in diameter, using conventional and diffusion-weighted MRI, soon after the onset of symptoms and over 1-36 months. The orientationally averaged diffusion coefficient was increased in all the acute lesions, and increased further during follow-up in three. There was a strong correlation between and the degree of low signal on T1-weighted images. The quantitative information provided by allowed delineation of different diffusion patterns in large MS lesions, that may reflect heterogeneity of the anatomical substrate.
In vivo multiple spin echoes (MSE) images of bone marrow in trabecular bone were obtained for the first time on a clinical 1.5 T scanner. Despite of a reduced sensitivity of the MSE trabecular bone images with respect to the cerebral matter ones, it is possible to observe some features in the MSE trabecular bone images that may be useful in the diagnosis of osteopenic states. Two different CRAZED-type MSE imaging sequences based on spin-echo and EPI imaging modalities were applied in phantom and in vivo. Preliminary experimental results indicate that EPI imaging readout seems to conceal the MSE contrast correlated with pore dimension in porous media. However it is still possible to detect anisotropy effects related to the bone structure in MSE-EPI images. Some strategies are suggested to optimize the quality of MSE trabecular bone images.
We report on a 5(1/2) year-old Italian girl with a distinctive form of metaphyseal chondrodysplasia simulating cartilage hair hypoplasia. The pattern of metaphyseal changes and the associated bony abnormalities differentiate this patient from all the recognized forms of metaphyseal chondrodysplasia.
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We present a girl with short stature, growth hormone neurosecretory dysfunction, severe hypoplastic/aplastic changes of the bones of the hands and feet with dysharmonic ossification, severely delayed bone age, microcrania, and fibular hypoplasia. Parental consanguinity suggests autosomal recessive inheritance. An additional three cases [Eiken et al., 1984: Eur J Pediatr 141: 231-235] sharing some of the radiographic manifestations of this patient have been reported. However, distinctive findings in the present case seem to outline a separate entity.
PURPOSE: Computed tomography (CT) and, more recently, ultrasound (US), have proved excellent tools for quantifying adipose tissue distribution. Body fat distribution is an important factor in the treatment of obesity and its complications. We investigated the correlation between CT and US measurements in pediatric obesity. MATERIAL AND METHODS: Forty obese children and adolescents aged 4.1-14.8 years were submitted to CT and US. Intra-abdominal, subcutaneous and total body fat were calculated (in cm2), with the CT image analysis software. The rectus muscle-spine and rectus muscle-aorta distances, as indicative of visceral fat thickness, were measured on US images with(out) compression. The distance between skin-fat and fat-rectus muscle interfaces was measured as subcutaneous fat thickness. We also compared US-CT findings with other morphometric variables--i.e., patient's (ideal) body weight and skin fold measures. RESULTS: At US, the rectus muscle-aorta and rectus muscle-spine distances ranged 2.4-7.5 cm (mean: 4.47 cm) and 3.6-8.9 cm (mean: 5.79 cm), respectively. The skin-rectus muscle distance ranged 1.2-7.5 cm (mean: 3.14 cm). A statistically significant correlation was found between the CT measurement of visceral fat and the aorta-rectus muscle and rectus muscle-spine distances (r = 0.80 and 0.74, respectively). The US measurements of subcutaneous fat were correlated with CT subcutaneous fat area (r = 0.82). No correlation was found between overweight, as calculated by body mass index, and CT or US fat. CONCLUSION: Our findings indicate that US is as useful as CT in evaluating body fat distribution in pediatric obesity.
The anatomical and functional correlates of the hand sensorimotor areas was investigated in a stroke patient with a malacic lesion in the left fronto-parieto-temporal cortex. The patient presented hemiplegia and motor aphasia 12 months earlier, followed by an excellent motor recovery. Transcranial magnetic stimulation mapping, functional magnetic resonance and magnetoencephalography were used as methods of functional imaging and all yielded consistent results. In particular, an asymmetrical enlargement and posterior shift of the sensorimotor areas localized in the affected hemisphere were found with all three techniques. Aspects related to brain 'plasticity' for functional recovery are discussed.
OBJECTIVE: To compare the performance of a new sonographic (US) method of bone age estimation with other methods currently in use. DESIGN AND PATIENTS: One hundred and fifteen subjects underwent left hand/wrist radiography and US examination of the hip for bone age assessment. For each patient, measurements of skeletal age were available based on Greulich-Pyle and Tanner and Whitehouse, the latter being presented in three subtypes (RUS, carpals, and B20) in addition to the US values. To assess agreement between methods, each method was compared with every other method. Differences between calculated skeletal age and chronological age were assessed, and the sensitivity, specificity, and predictive values of each method computed. RESULTS: Coupled B20/RUS values showed the best agreement, with 95% of observations within 2.45 years of each other, followed by carpals/B20, B20/GP, and GP/RUS. The US method agreed the least (difference of 4.19-5.13 years) with the other methods. The US method provided 85.8% (US vs RUS) to 91.3% (US vs GP) concordant results in recognizing differences between skeletal and chronological age, and showed a 72.5% sensitivity and a 56.8% specificity. CONCLUSION: Although the US method promises to permit a safe and cost-effective assessment of skeletal age, its low accuracy makes it currently unsuitable for clinical use.
A thalassaemic girl presented with agranulocytosis, arthritis of both ankles and clinical and laboratory features consistent with the diagnosis of systemic vasculitis, during oral iron chelator L1 (deferiprone) treatment. Changes in the humoral and cell-mediated immune function. including antinuclear antibodies (ANA), anti-DNA and extractable-nuclear antigens (ENA) antibodies positivity, increased immunoglobulin values, decreased T suppressor and the presence of circulating immune complexes, suggest a cause-and-effect relationship with the observed clinical manifestations. A careful monitoring of the immune function is recommended in patients who are receiving the oral iron chelator L1.
A newborn girl is described with an association of spondylo-acrodysplasia, mild short limbed dwarfism without significant metaphyseal changes, joint dislocations, and severe immune system dysfunction. This association is distinct from other known immuno-osseous dysplasias, including Schimke dysplasia, ADA deficiency with osseous changes, and Omenn phenotype with short limbed dwarfism.
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BACKGROUND: A simple, accurate, reproducible and noninvasive method of body iron overload assessment would be of great clinical use. Objective. The purpose of the study was the implementation of a 0. 5-T MRI method for liver iron overload measurement. MATERIALS AND METHODS: Thirty patients with thalassemia major took part in the study. Liver and paraspinal muscle signal intensity (SI) measurements were performed on T1-weighted images and normalized on a standard phantom, and a subjective hemochromatosis grading scale was made on both T1- and T2-weighted images. Serum ferritin levels and tissue iron from liver biopsy specimens were determined for comparison. RESULTS: A close correlation was found between bioptic liver iron and both the liver-to-phantom SI ratio (r = -0.88) and the subjective grading scale (rho = 0.89). Serum ferritin correlated poorly with liver iron deposition, whether assessed by biopsy (r = 0. 62) or MRI (r = -0.69). CONCLUSIONS: Both the subjective and the quantitative MRI methods proposed here are clinically valuable, with the former being adequate for a gross, the latter for an accurate estimation of tissue iron overload.
OBJECTIVE: To establish the precision of sonographic measurement of the thickness of the articular cartilage of the hip and knee in children. DESIGN: The precision was assessed by evaluating the intra- and interobserver variations in sonographic measurements. PATIENTS: A total of 65 healthy children were in the study. The articular cartilage of the right hip and knee of 40 subjects (mean age 10.3 years, range 4-16.9 years) was evaluated in masked fashion by two observers to assess the interobserver variability. The articular cartilage of the right hip and knee of 25 children (mean age 10.4 years, range 6.2-15.5 years) was examined twice by the same observer to assess the intraobserver variability. RESULTS AND CONCLUSIONS: Discrepancies between repeated measurements were expressed as data differences. The "limits of agreement" of data differences, i.e. the mean +2 SD and the mean -2 SD, ranged from -0.26 to +0.22 mm and from -0.56 to +0.48 mm for the interobserver analysis of the femoral head cartilage (FHC) and femoral condylar cartilage (FCC), respectively. The "limits of agreement" for the intraobserver analysis ranged from -0.16 to +0.15 mm and from -0.51 to +0.41 mm for the FHC and the FCC, respectively. The overall precision of the sonographic measurements was satisfactory. When used for determination of skeletal age, sonographic assessment of FHC thickness resulted in under/overestimation of skeletal age by about 7.5 months. In conclusion, sonographic measurement of articular cartilage is precise enough to be used in clinical practice.
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