1/4. autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases.OBJECTIVE: To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc). methods: We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms. RESULTS: We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found. CONCLUSION: Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/4. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review.Systemic sclerosis is a multi-system disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Although resorption of the mandible has been commonly observed and reported, we found no report of resorption leading to a painful neuropathy of the inferior alveolar nerve. We report a case of a patient with systemic sclerosis, severe resorption of the angles, and inferior alveolar border of the mandible, resulting in a compression neuropathy of the inferior alveolar nerve. Diagnostic tests, medical treatment, and surgical treatment are discussed, and the relevant literature is reviewed.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
3/4. Cranial nerve VIII involvement in a patient with progressive systemic sclerosis.Systemic sclerosis is a multisystem disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Cranial nerve involvement is an uncommon feature of this connective tissue disorder, and when it occurs it is the trigeminal nerve that is primarily affected. We report an elderly woman who presented with sensorineural hearing loss and was then diagnosed with the crest syndrome of progressive systemic sclerosis (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia). Involvement of the eighth cranial nerve with scleroderma and crest syndrome is rare, but appears to be the cause of sensorineural hearing loss in our patient.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
4/4. association of an irregularly shaped anterior choroidal aneurysm with crest syndrome. Case report.The authors present the case of a 50-year-old woman with a history of crest syndrome (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia), a variant of scleroderma, who was incidentally found to have an irregular intracranial aneurysm. The patient presented with migraine headaches. A magnetic resonance image of the brain obtained during the headache workup revealed a right posterior carotid artery wall aneurysm in the region of the anterior choroidal artery (AChA). On digital subtraction angiograms, the lesion measured 3.5 mm at its largest diameter. Because of the irregular shape of the aneurysm, the patient's relatively young age, and the potential for further aneurysm growth due to collagen disease, surgical clip application was recommended following a discussion of available treatment options. At surgery, the aneurysm was identified as bilobed and broad based, and the AChA was found to be associated with the aneurysm neck. Satisfactory clipping of the aneurysm was achieved with preservation of the parent vessels. An association of crest syndrome with intracranial aneurysms has only been reported once before. This case is presented to draw attention to the possibility of a pathophysiological connection between crest syndrome and intracranial aneurysms and to postulate a possible mechanism whereby this condition may result in aneurysm formation. The association of aneurysms with other pathological collagen-related conditions is well known, and literature relevant to a possible connection between crest syndrome and aneurysms is reviewed and discussed.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |