A 55-year-old mildly hypertensive woman died after having developed a subcortical dementia during the past 9 years, with focal neurological signs. She presented at the age of 46 years with short episodes of dizziness and diplopia, suggesting that transient ischemic attacks involved the posterior fossa structures. Over the next 8 years, she developed difficulty in walking, urinary incontinence and seizures. On examination in 1989, she was severely demented. There was tetraparesis, bilateral arm and leg spasticity with hyperreflexia and bilateral Babinski signs. She showed epilepsia partialis continua involving the eyes, left hemiface and limbs. CT showed hypodensity of the white matter and lacunes in the basal ganglia and centrum semiovale, moderate hydrocephalus with cerebellar and cortical atrophy. Clinical and radiological features were similar to those of Binswanger's disease. Similar cases had occurred in the family affecting the patient's grandfather, father and two brothers, suggesting an autosomal dominant hereditary disease. Postmortem examination disclosed a Binswanger type of leukoencephalopathy caused by a peculiar microangiopathy characterized by a slightly basophilic small arterial granular degeneration of the medial sheath associated with the presence of ballooned smooth muscle cells with clear cytoplasm. Electron microscopic study revealed degenerative changes in the parietal vessels with notable increase of basal-membrane-type material and electron-dense granular deposits. These lesions could correspond to a specific familial pathology of the small arteries of the brain. They are identical to those reported in some patients with autosomal dominant inheritance. For other patients with similar clinical features and the same familial pattern, reported as "hereditary multi-infarct dementia'' and "chronic familial vascular encephalopathy'', there are no sufficient objective pathological facts to consider that they have the same disease.(ABSTRACT TRUNCATED AT 250 WORDS)
A newborn boy presented with a progressively infiltrating and painful congenital ulcerated plaque on the back of his left foot. A partial excision was performed and histopathologic examination confirmed a diagnosis of a plexiform fibrohistiocytic tumor. This rare tumor usually appears in children and adolescents, with congenital presentations even more uncommon. This case details the exceptional presentation of a congenital ulcerated plexiform fibrohistiocytic tumor with a review of the current literature.
African tick-bite fever (ATBF), a tickborne disease endemic in rural areas of sub-Saharan Africa and the West Indies caused by Rickettsia africae, has been recognized as an emerging health problem in recent years. ATBF has been reported as the second most commonly documented etiology of fever, after malaria, in travelers who return ill from sub-Saharan Africa. Most cases reported in the literature occurred in middle-aged adults, so the incidence of ATBF in children is unclear. We report a cluster of three cases of ATBF that occurred in children ages 7 to 16 years after returning from a game-hunting safari in South Africa.
Background. The diagnostic imaging techniques in dermatology, such as skin ultrasound, could improve the diagnosis of non-melanocytic benign tumor lesions, especially those of intradermal and subcutaneous nature. Objective. The general objective of this study was to evaluate the validity and diagnostic safety of high-frequency ultrasound as a diagnostic test for non-melanocytic benign skin tumor detection. The specific objectives were to measure the sensitivity and specificity of ultrasound as a measure of the validity relative to the gold-standard test – diagnostic-therapeutic biopsy. Methods. This study was carried out between January 2017 and June 2019. The patients (n = 641) included in this study comprised of 318 women and 323 men who were between 48 and 74 years. All of them underwent an ultrasound examination of the skin tumor in the dermatology clinic, the removal of the non-melanocytic tumor, and a final histopathological examination. Results. The skin ultrasound has a sensitivity and specificity of 93% and 88%, respectively. Furthermore, the positive and negative predictive values of using skin ultrasound are 84% and 94%, respectively. Conclusion. Skin ultrasound is a diagnostic technique with high sensitivity and specificity in the diagnosis of non-melanocytic skin tumors.
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