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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A survey of 118 patients seen in the last twenty years in Newcastle upon Tyne forms the basis of this report. All of these 118 patients fulfilled clearly defined clinical, electrophysiological and pathological criteria for the diagnosis of polymyositis: muscle pain, weakness and characteristic EMG and/or muscle biopsy 55%; and characteristic muscle biopsy 17%; muscle weakness and characteristic EMG 7%; muscle weakness and pain, and raised serum CK activity in an established collagen-vascular disease 5%. A smaller group of 25 patients were selected in whom the clinical characteristics, EMG, muscle biopsy and serum enzyme levels were all completely diagnostic of polymyositis. The patients were followed for two months to twenty-six years, with a mean follow-up duration of six years. Analysis was made of the features at presentation and during the course of the illness, and of prognostic factors bearing upon the disability, response to treatment and mortality. Cases were classified according to the system of Rose and Walton (1966). Groups I, II, and III each constituted approximately one-third of the total cases, while only 8% of all cases were associated with carcinoma. The female to male ratio was 1.4:1. Though cases were seen in all age groups, the largest number was in the sixth decade. The sedimentation rate was raised in 55% of cases. Electromyography was characteristic of polymyositis in 45% of cases, and in only 11% was it normal. The serum creatine kinase activity was raised in 64% of cases. There was no correlation between the extent of these abnormalities and the degree of weakness or disability. 65% of muscle biopsies had changes with inflammatory infiltration virtually diagnostic of polymyositis. 17% of cases had a normal muscle biopsy. Most of the patients (89%) were treated with high-dose prednisone therapy, commencing with 30-100 mg/day, gradually reducing to a maintenance dose of 5-15 mg/day over two or three months. All clinical groups showed considerable improvement in average disability with time on "high dose" corticosteroid therapy, the maximum improvement occurring within the first three years. The degree of improvement in disability was considerably less in those inadequately treated, though the mortality rate was similar in the two groups. 66% of all survivors had essentially no functional disability at follow-up three or more years later, and in the majority of these cases the disease appeared to have burned itself out. 33% of cases had significant disability after three years, and in half of these the disease appeared to be still active.
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PMID:Polymyositis: its presentation, morbidity and mortality. 121 71

We describe conduction block as an unusual electrophysiologic manifestation in a patient with necrotizing angiopathy. The patient developed subacute symptoms over a 1-month period consisting of progressive pain, tingling, and weakness of the lower extremities. Physical examination revealed a pattern consistent with a polyneuropathy. Electrodiagnostic studies provided evidence of a conduction block in the left ulnar nerve. Pathologic studies confirmed the process to be a necrotizing angiopathy. This report establishes the role of conduction block in human nerve ischemia.
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PMID:Necrotizing angiopathy presenting with multifocal conduction blocks. 162 80

A 41-year-old man with PPP since in 1982 was admitted in May, 1987, because of the progressive asymmetric sensory disturbance in the hands and feet over 4 months, accompanied by an exacerbation of PPP. On admission, eruptions of PPP were observed in the palms and soles. Asymmetric and mildly decreased sensations of touch and pain were present in the distal part of the four extremities as well as in his trunk, accompanied by paresthesia and dysesthesia. Mild to moderate weakness was noted in the hand muscles, and slight muscular atrophy was present in the right lower leg. A work-up for collagen vascular disease was within normal limits. T lymphocyte subset showed a decreased ratio of OKT 4/OKT 8. Left sural nerve biopsy showed axonal degeneration and moderate decrease of myelinated fibers, and the vasculitis was not found. The neurological signs and symptoms as well as the skin eruptions improved with methylprednisolone 40 mg/day. A causal relationship between the multiple mononeuropathy and PPP of our patient was indicated by the almost simultaneous onset of the neuropathy and the exacerbation of PPP as well as the improvement of these two conditions with corticosteroid therapy. Such combination of multiple mononeuropathy and PPP has not so far been reported.
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PMID:[A case of multiple mononeuropathy associated with pustulosis palmaris et plantaris (PPP)]. 176 52

We report a 34-year-old woman with mixed connective tissue disease (MCTD) who developed severe pulmonary and neuromuscular complications. At presentation, pulmonary function tests and pulmonary mechanics were suggestive of pulmonary vascular disease, and she subsequently developed clinical signs of pulmonary hypertension. These noninvasive tests may be useful in the timing of more invasive hemodynamic studies. She initially had myasthenia gravis and then developed polymyositis, profound peripheral neuropathy, and ventilatory muscle failure. She died despite aggressive immunosuppressive therapy and plasmapheresis. Autopsy showed spinal cord changes secondary to a peripheral neuropathy and signs of neurogenic atrophy confined to the ventilatory muscles. Peripheral neuropathy may be an important cause of ventilatory muscle weakness that can be found in MCTD and systemic lupus.
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PMID:Pulmonary and neuromuscular complications of mixed connective tissue disease: a report and review of the literature. 231 66

Among the generalized chronic idiopathic inflammatory myopathies, inclusion body myositis (IBM) has emerged as a clinico-pathologic variant during the past two decades. It occurs primarily in elderly persons (in approximately the sixth decade of life), but young adults (in approximately the second decade of life) may also be affected. Slowly progressive weakness of distal as well as proximal muscle groups in IBM is usually not associated with skin rash, malignancy or collagen-vascular disease, and is refractory to treatment with steroids or other immunosuppressants. Exceptions to each of these general rules have been found. Muscle biopsy and electromyography may suggest a neurogenic process mixed with myopathic features. Rimmed vacuoles with basophilic granules in cryostat sections stained with hematoxylin-eosin are strongly suggestive of IBM if accompanied by the histopathologic triad of polymyositis. The presence of eosinophilic intranuclear or cytoplasmic inclusions in affected myofibers is further suggestive of IBM. The ultimate diagnosis, however, depends on ultrastructural demonstration of characteristic microtubular filaments resembling the nucleocapsids of the paramyxovirus group. Recent reports of immunostaining of the inclusions for mumps virus antigen strongly suggest a chronic persistent mumps virus infection as the cause of IBM. IBM is considered to be pathologically related to both distal myopathy (DM) and oculopharyngeal muscular dystrophy (OPMD).
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PMID:Inclusion body myositis: a chronic persistent mumps myositis? 301 64

Six patients with an aortoiliac vascular disease and a peripheral neurological deficit are presented. Clinical and electromyographic findings revealed lumbosacral plexus, sciatic and femoral nerve lesions. A correlation is made between the level of the vascular lesion (aortic, aortoiliac or distally) and the type of peripheral nerve deficit observed. In a patient complaining of pain, weakness, or numbness in a leg, the differential diagnosis should include aortoiliac vascular disease. The peripheral neurological symptoms may be the initial manifestation of the vascular disease or may appear in the early post-operative period.
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PMID:Peripheral neurological complications of aortoiliac vascular disease. 303 89

In seven patients with slowly progressive muscle weakness, inclusion body myositis (IBM) was diagnosed on biopsy. None had stigmata of collagen-vascular disease or malignancy. Serum creatine kinase levels were mildly or moderately increased. The six patients treated with prednisone did not improve. Needle electromyography showed a "myopathic" pattern in all patients, but four also had diffuse neurogenic changes with normal nerve conductions. Histologic study of muscle showed a mixture of small rounded fibers varying in size, atrophic angulated fibers forming small groups, and hypertrophic fibers. Variable amounts of inflammation, necrosis, and regeneration were seen in all specimens. All showed numerous intracytoplasmic vacuoles lined with purple-blue granules. Electron microscopy showed membranous whorls and masses of abnormal filaments measuring 14 to 18 nm in diameter. Although IBM seems to be a distinct type of inflammatory myopathy, its etiology and pathogenesis are not clear.
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PMID:Inclusion body myositis. A corticosteroid-resistant idiopathic inflammatory myopathy. 629 95

IBM has emerged as a clinicopathological entity during the past 25 years but with increasing complexity. It occurs primarily in elderly persons (over the sixth decade of life, with 3:1 male preponderance), but young adults or children may also be affected in some families. FIBM is by and large non-inflammatory though some autosomal dominant FIBM cases have inflammatory cell infiltrates. In IBM, slowly progressive weakness of proximal as well as distal muscle groups occurs and is usually not associated with skin rash or malignancy. The incidence of associated collagen-vascular disease is thought to be lower than in DM or PM but is reported to be as high as 15%. It is generally refractory to treatment with corticosteroids or other immunosuppressants. Muscle biopsy and electromyography may suggest a neurogenic process mixed with myopathic features. None of the histopathological features is specific enough to be a diagnostic criterion. The diagnostic criteria have to be collective, encompassing both clinical and pathological criteria in different combinations. The presence of eosinophilic intranuclear or cytoplasmic inclusions immunoreactive for both beta-amyloid and ubiquitin in affected myofibres may facilitate the diagnosis of IBM. The diagnosis no longer depends on the ultrastructural demonstration of characteristic microtubular filaments as previously thought. The identification of both beta-amyloid and ubiquitin may provide a new concept for the disease process in IBM. A chronic persistent intracytoplasmic synthesis of abnormal amyloid protein in IBM is suspected to be similar to that in Alzheimer's disease. IBM is considered to be intimately related to a heterogenous group of non-inflammatory IBMD, including DMY, OPMD, and both autosomal recessive and dominant FIBM. An inflammatory response has been seen, however, in muscles of both OPMD and autosomal dominant FIBM. The pathogenesis in IBM and in IBMD may not be the same. Unlike IBM, there is no abnormal sarcolemmal expression of MHC-I antigen in IBMD as a sign of T-cell-mediated cytotoxicity causing myofibre destruction. The prion theory derived from identification of amyloidogenic protein in the filament inclusions in the rimmed vacuoles is provocative. If one believes in the contention that the amyloidogenic filaments are the primary pathogen of either IBM or IBMD, one must account for the fact that these filaments are originally derived from sarcolemmal nuclei and not from autophagic vacuoles. Until this is clarified, the possibility that the filaments represent either abnormal or defective 'slow' virus nucleocapsids cannot be completely ruled out.
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PMID:Inclusion body myositis. 815 43

Transient ischemic attacks (TIA) are defined as acute, retinal or focal-cerebral neurological symptoms, resulting from vascular disease, which resolve in less than 24 hours. Typical clinical signs are transient visual obscuration, sudden weakness of one arm or leg, loss of speech, and dizzy spells. These patients run a considerable risk of stroke; hence rapid diagnosis and treatment are mandatory. Differential diagnosis includes transient global cerebral function loss, non-vascular transient focal attacks, as well as extracerebral causes of transient neurological symptoms. The following basic investigations are necessary for most patients with TIA: simple laboratory work-up, extra- and transcranial doppler/duplex-ultrasonography, cardiological examination and CT-scan. Angiography and MR-angiography are reserved for specific questions.
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PMID:[Assessment and diagnosis of transient ischemic attacks]. 853 77

The case of dermatomyositis complicated with cecum perforation and panniculitis occurred in a 62-year-old woman was reported. She was admitted to Keio University Hospital with a history of proximal muscular weakness, and dysphagia. Physical examination showed erythema over the face and shoulder. Serum level of muscle enzymes was remarkably increased. The diagnosis of dermatomyositis was made based on proximal muscular weakness, elevated serum level of muscle enzymes and myogenic change of electromyocardiogram. The treatment with 60 mg/day of prednisolone was started, and was a good response. However, 7 months later the disease became active again when the amount of prednisolone was reduced to 13 mg/day. Subsequently she complained of abdominal pain on the right lower quadrant. The surgical findings included peritonitis due to the perforation of the cecum and multiple ulcers of the cecum. After operation, azathioprine was added. Four years and 9 months later, she noticed skin erythema with ulceration and subcutaneous nodule. Skin biopsy indicated the findings of the panniculitis with membrano-cystic lesion. It was thought that both cecum perforation and panniculitis were caused by angiopathy which was often seen in childhood dermatomyositis.
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PMID:[An adult case of dermatomyositis complicated with cecum perforation and panniculitis]. 910 66


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