1/5. Progressive intracranial vascular disease with strokes and seizures in a boy with progeria.progeria, a rare genetic disorder, is characterized by severe growth failure, premature aging, and very early atherosclerosis with coronary artery and cerebrovascular disease. There has been no detailed description of progressive cerebrovascular changes in progeria or any attempted neurologic correlation of those changes. A 5-year-old boy developed signs of progeria at 4 months and hypertension at 4 years, treated with atenolol and dipyridamole. Left-sided seizures with a left hemiparesis occurred at 5 years. magnetic resonance imaging (MRI) showed bilateral acute, subacute, and chronic cerebral infarctions. magnetic resonance angiography disclosed severe stenosis of the left internal carotid artery. The child was also found to have an aortic valve vegetation and was anticoagulated. He subsequently developed right-sided seizures, and treatment with gabapentin was started. Later, severe stenosis also of the right internal carotid artery was found. MRI showed new left cerebral infarction. The child's neurologic symptoms almost certainly were caused by cerebral infarctions from progressive atherosclerosis of major intracranial vessels, but clinical-neuroradiologic correlations were imprecise. There were multiple cerebral infarctions of different ages, some asymptomatic, others ipsilateral to the child's neurologic findings. No therapy has halted progression of the child's cerebrovascular disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/5. Smooth muscle cell depletion and collagen types in progeric arteries.review of two autopsy cases of progeria confirms severe smooth muscle cell (SMC) depletion in the atherosclerotic aortic media and the presence of collagen types I, III, IV, V, and VI in the aorta and renal vessels as is consistent with atherosclerotic disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/5. Epidural hematomas in a child with Hutchinson-Gilford progeria syndrome.INTRODUCTION. Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder. It is characterized by severe growth failure, premature aging, and very early atherosclerosis with coronary artery disease and cerebrovascular disease. CASE REPORT. A 10-year-old boy with HGPS was admitted to our department because of progressive deterioration after a mild head injury. The CT scans revealed epidural hematoma in posterior fossa and another one in the temporal region on the left side. On admission the child was given an estimated score of 10 on the GCS. Neurological examination revealed right hemiparesis. The boy was operated on, and both hematomas were evacuated. In a few days the neurological symptoms disappeared, and he was discharged from the hospital with only residual, minimal right hemiparesis. CONCLUSION. Intracranial pathology was certainly caused by the head trauma, but was more severe than would have been expected had the trauma been the sole cause. We suggest that progressive atherosclerosis of intracranial vessels was responsible for formation of the hematomas.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/5. Idiopathic arterial calcification of infancy.A description is given of a 15-month-old girl with idiopathic arterial calcifications, detected during life time by X-ray. Radiological examination revealed calcifications in medium-sized arteries, histopathological examination showed distinct abnormalities of small vessels. The patient also had a metageria-like outward and retarded mental and motor development with myolysis. No cardiac failure was present.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/5. Juvenile systemic granulomatosis manifesting as premature aging syndrome and renal failure.We describe a case of juvenile systemic granulomatosis in a 22-year-old woman. The rash consisted of purple papules and first appeared at the age of one year. She had persistent symmetrical painless boggy tenosynovitis with minimal roentgenographic changes and chronic granulomatous symptoms. uveitis resulted in visual impairment. She also had granulomatous changes in her vessels. Renal impairment developed; however, neither renal artery stenosis nor hypercalcemia was found. Clinical features included the development of premature aging with alopecia, which differed from the previously reported progeria syndrome. Poikiloderma may cause a prematurely aged appearance. Our report expands the clinical spectrum of systemic granulomatosis to include the development of premature aging with alopecia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |