Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

BACKGROUND: Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. CASE PRESENTATION: A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented. CONCLUSIONS: The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and LSCS actually represent different spectra of the same disease

Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia

BACKGROUND: Sera from children with post-varicella infections have autoantibodies that react with centrosomes in brain and tissue culture cells. We investigated the sera of children with infections and post-varicella ataxia and related conditions for reactivity to five recombinant centrosome proteins: γγ-enolase, pericentrin, ninein, PCM-1, and Mob1. METHODS: Sera from 12 patients with acute post-varicella ataxia, 1 with post-Epstein Barr virus (EBV) ataxia, 5 with uncomplicated varicella infections, and other conditions were tested for reactivity to cryopreserved cerebellum tissue and recombinant centrosome proteins. The distribution of pericentrin in the cerebellum was studied by indirect immunofluorescence (IIF) using rabbit antibodies to the recombinant protein. Antibodies to phospholipids (APL) were detected by ELISA. RESULTS: Eleven of 12 children with post-varicella ataxia, 4/5 children with uncomplicated varicella infections, 1/1 with post-EBV ataxia, 2/2 with ADEM, 1/2 with neuroblastoma and ataxia, and 2/2 with cerebellitis had antibodies directed against 1 or more recombinant centrosome antigens. Antibodies to pericentrin were seen in 5/12 children with post-varicella ataxia but not in any of the other sera tested. IIF demonstrated that pericentrin is located in axons and centrosomes of cerebellar cells. APL were detected in 75% of the sera from children with post-varicella ataxia and 50% of children with varicella without ataxia and in none of the controls. CONCLUSION: This is the first study to show the antigen specificity of anti-centrosome antibodies in children with varicella. Our data suggest that children with post-varicella ataxia have unique autoantibody reactivity to pericentrin

Evaluation of a dichromatic X-ray source for dual-energy imaging in mammography

A novel X-ray system, providing dichromatic beams for dual-energy radiography, has been assembled. The source generates pairs of superimposed quasi-monochromatic beams, having energies E and 2E; with E tuneable in the 15–20 keV range. In this paper the characteristics of the radiation field in terms of energy resolution and fluence, for three dichromatic X-ray beams are reported. A study of the spectra attenuated by a 5 cm-thick phantom of breast equivalent tissue demonstrates that the optimal energy of the dichromatic beam for dual-energy application may be set as a function of the thickness of investigated tissue. A detailed topographic study of mean energy and flux shows the spatial superposition of the first and the second diffraction order beam, that is the main requirement for the application of a single exposure dual-energy radiography. The bidimensional mapping of the irradiated beam is also reported, showing the presence of energy and intensity gradients. We estimate that the observed gradients do not affect the results of dual-energy technique application in an appreciable way

Dichromatic source for the application of dual-energy tissue cancellation in mammography

A novel x-ray source, providing dichromatic beams for the application of dual-energy radiography, has been assembled and studied. The system works via Bragg diffraction, by monochromatizing the beam produced by a conventional W-anode x-ray tube with a mosaic crystal monochromator. The source generates a laminar beam (10 cm-high and 0.8 cm-wide), composed by two spatially superimposed quasi-monochromatic beams. The characteristics of the radiation field in terms of energy resolution and fluence have been reported, for three pairs of energies. A study of the spectra attenuated by several phantoms of breast equivalent tissue of different thicknesses shows that the optimal energy of the dichromatic beam for dual energy mammography application ranges between 18/36 keV and 18.6/37.2 keV and may be set as a function of the thickness or density of investigated tissue

Signal-to-noise ratio evaluation in dual-energy radiography with synchrotron radiation

Dual-energy radiography is an effective technique that allows removal of contrast between pairs of materials in order to display details of interest on a uniform background. In mammographic images the detection of small nodules is often impeded by obscuring background 'clutter' resulting from the contrast between normal tissues (glandular and adipose) in their neighbourhood. We consider whether it could be possible to apply dual-energy radiography to the breast, which is hypothetically principally composed of three tissues, glandular, adipose and cancerous, in order to remove the contrast due to the distribution of normal tissues and, as a consequence, to enhance the intrinsic contrast of the pathology. The purpose of this work is to test the limitations of dual-energy radiography on a three-component phantom under optimum conditions of the source and detector. We use a synchrotron monochromatic beam, produced at the ELETTRA synchrotron facility (Trieste, Italy), and an imaging plate detector, in order to acquire two images at low and high energies of a phantom composed of polyethylene, plexiglas and water. For evaluation of the potential of this procedure we studied the signal-to-noise ratio (SNR) of polyethylene and water on a set of images obtained by applying the dual-energy procedure. We found that the SNR of polyethylene and water is around the detectability threshold (according to Rose's criteria) at the contrast cancellation angles. Finally we evaluated the air entrance dose required for this double exposure, resulting in 0.81 mGy for the low-energy image and 0.01 mGy for the high-energy image. To obtain the same image quality for a standard breast of 5.5 cm, mean glandular doses of 3.50 mGy and 0.03 mGy at 17 keV and at 34 keV, respectively, are required