1/37. Anticoagulation therapy for radiation-induced myelopathy.OBJECTIVE: To report the use of heparin and enoxaparin for radiation-induced myelopathy. CASE SUMMARY: A 48-year-old White woman with presumed metastatic lung cancer presented with worsening numbness and weakness of both legs. The neuro-oncology service was consulted and determined that the symptoms were consistent with radiation-induced myelopathy. The patient briefly responded to steroid treatment. A trial of intravenous heparin therapy was initiated by the primary team and managed by the clinical pharmacy services. Her symptoms improved when heparin was begun. She was able to walk and was subsequently discharged home on enoxaparin. DISCUSSION: spinal cord injury is one of the known adverse effects of radiation. The onset of symptoms can be acute or delayed. The clinical signs and symptoms of delayed neurologic injury are related to the narrowing and occlusion of the vessel lumen, ischemia, edema, and cell death in the surrounding nervous tissue. Treatment often consists of corticosteroids and/or hyperbaric oxygen; however, the outcomes are often disappointing. In addition to the inhibition of serum protein-mediated coagulation, heparin inactivates or prevents the release of mediators of vascular injury inflammation, permeability, and edema. Therefore, patients with radiation-induced spinal cord injury may benefit from anticoagulant therapy. CONCLUSIONS: heparin and/or enoxaparin may be considered as potential treatments for patients with radiation-induced myelopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/37. nephrogenic fibrosing dermopathy with systemic involvement.BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/37. Immune-mediated neuropathy and myopathy in post-streptococcal disease: electron-microscopical, morphometrical and immunohistochemical studies.A 22-year-old man suffered from a complete flaccid tetraparesis and an immune complex-mediated rapid progressive glomerulonephritis after group A streptococcal infection. serum creatine kinase was excessively elevated and myoglobinuria occurred. Nerve conduction studies revealed evidence of axonal neuropathy. Recovery was satisfactory within 18 months. sural nerve and peroneus muscle biopsies were performed in the 4th and 14th week of the disease. light microscopy of the sural nerve showed an incipient axonal type of neuropathy in the first biopsy. Ultrastructurally, wallerian degeneration and endoneurial inflammatory cells were present. In the muscle biopsy, few atrophic fibers and altered blood vessels without further anomalies were found. In the second sural nerve biopsy, macrophages were numerous, some of which were immunoreactive for HLA-DR, and only a few myelinated and some unmyelinated nerve fibers remained. Muscle fibers in the second biopsy showed high-grade atrophy and myofibrillar abnormalities. immunohistochemistry revealed diffuse endoneurial immunoglobulin deposition in the first sample, while in the later biopsy specimen, deposits of IgG, and kappa and lambda light chains were visible in circumscribed endoneurial areas. Immune-mediated neuropathy and myopathy are not well-known complications of streptococcal disease. This is, to our knowledge, the first detailed report on morphological findings in muscle and nerve in such a disorder.- - - - - - - - - - ranking = 11.332238445151keywords = blood vessel, vessel (Clic here for more details about this article) |
4/37. Fetal abdominal cavernous hemangioma diagnosed by duplex Doppler velocimetry.A fetal abdominal wall mass was detected by ultrasound and analyzed by duplex Doppler showing a low resistance to blood flow through its vessels. The antenatal diagnosis of cavernous hemangioma was confirmed after birth.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/37. Amyloid myopathy presenting with distal atrophic weakness.Amyloid myopathy is a rare complication of primary amyloidosis usually presenting with proximal muscle weakness. We report a woman with multiple myeloma in whom marked atrophy and weakness of finger flexor muscles were the first manifestations of systemic amyloidosis. Muscle biopsy revealed amyloid angiopathy with deposits of lambda light chains in vessel walls. The recognition of amyloid myopathy is important because clinical symptoms may respond to chemotherapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/37. Necrotizing myopathy with microvascular deposition of the complement membrane attack complex.Necrotizing myopathy without prominent inflammatory changes is increasingly being recognized as a form of inflammatory myopathy, different from dermatomyositis, polymyositis and sporadic inclusion-body myositis. We report a patient with a chronic steroid-responsive myopathy and an ill-defined overlap syndrome. The muscle biopsy showed thickened capillaries and arterioles and deposition of the membrane attack complex in the replicated mural elements of some vessels. The surface of all muscle fibers showed major histocompatibility class I immunoreactivity. Similar patients have rarely been reported, either suffering from an undifferentiated connective tissue disorder or a carcinoma. The link between the muscle fiber necrosis and the microangiopathy is unclear. Absence of prominent inflammatory changes in a diagnostic muscle biopsy does not exclude the diagnosis of a treatable autoimmune inflammatory myopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/37. Amyloid myopathy: characteristic features of a still underdiagnosed disease.A 62-year-old man with progressive proximal weakness underwent extensive evaluation including muscle biopsy without a clear diagnosis being established. A repeat muscle biopsy including congo red-stained sections revealed infiltration of blood-vessel walls and endomysium with amyloid protein, as well as an unusual pattern of pathologic changes to muscle fibers. From a review of 79 cases of amyloid myopathy reported in the English-language literature, the characteristic features of this disorder are described. congo red-stained sections of muscle biopsy viewed under fluorescent or polarized optics, and serum or urine protein immunoelectrophoresis, play an important role in the evaluation of myopathy. Amyloid myopathy should be a consideration in adults with progressive neuromuscular weakness of uncertain cause.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/37. Amyloid myopathy presenting with rhabdomyolysis: evidence of complement activation.At age of 57 years, a man experienced an episode of rhabdomyolysis. On that occasion muscle biopsy was not performed, however monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. Further he developed a moderate proximal muscle weakness with CK level persistently elevated (1000-1200U/l). When he came to our observation, at age 67, a muscle biopsy revealed an amyloid myopathy and multiple myeloma was at the same time disclosed. Terminal complement complex C5b9 (membrane attack complex) deposits were found in the vessel walls and muscle fibers surface depicted by amyloid. Our case suggests to keep in mind the possibility that amyloid myopathy may begin as an isolate episode of rhabdomyolysis. The detection of complement complex C5b9 suggests that complement cascade is implicated in the muscular damage of amyloid myopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/37. Cryoprotein complexes and peripheral neuropathy in a patient with chronic active hepatitis.A cryoprotein complex was isolated and characterized from a patient with chronic active hepatitis and a severe peripheral neuropathy. This cryoprotein was composed of IgM, IgG, and hepatitis b surface antigen (HBsAg) and had a concentration of approximately 36 mg per 100 ml of serum. Electron microscopic examination of the cryoprotein demonstrated aggregates of Dane particles in close association with the antigenically related tubular and spherical forms of HBsAg. HBsAg, IgM, and IgG were detected by immunofluorescent staining in the intima of small arteries and veins. The association of a high serum level of cryoprotein and deposition of the cryoprotein components in small blood vessels suggests a role for the cryoprotein in the pathogenesis of peripheral neuropathy in this patient with chronic active hepatitis.- - - - - - - - - - ranking = 11.332238445151keywords = blood vessel, vessel (Clic here for more details about this article) |
10/37. MELAS of infantile onset: mitochondrial angiopathy or cytopathy?An 83-day-old male infant had convulsions, hypertrophic cardiomyopathy, and lactic acidosis. Cranial computed tomography revealed low-density areas in both parieto-occipital lobes and in the left temporal lobe. Muscle biopsy did not reveal ragged-red fibers, but abnormal mitochondria were found in the capillary endothelial cells as well as in the muscle fibers. At 5 months of age, the patient developed purpura on the soles and palms. skin biopsy showed degeneration of the endothelial cells with abnormal mitochondria in the arterioles and capillaries. Myelinated nerves in the skin had vacuolated axons with swollen mitochondria, and their myelin sheaths showed vacuolation. At 9 months of age, he died of heart failure, and autopsy revealed abnormal mitochondria in the myocardium but not in the coronary vessels. Our findings indicate that the symptoms of the mitochondrial encephalopathy, myopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome cannot be fully explained by the mitochondrial angiopathy alone.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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