Medicine
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Case Reports
Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients.
We studied 71 patients with "paraneoplastic" encephalomyelitis, sensory neuronopathy, or both associated with the presence of the anti-Hu antibody in their serum. Most (78%) had small-cell lung cancer. In 9 patients no tumor was detected. ⋯ Autonomic and respiratory failure, either of central origin or secondary to neuromuscular weakness, were the principal causes of death. Patients with rapidly developing sensory neuropathy or symptoms of encephalomyelitis should be studied for the presence of the anti-Hu antibody; if the antibody is found, the possibility of small-cell lung cancer should be investigated. If a tumor is not found in the initial search, one may become evident in several months.
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The IIM are a heterogeneous group of systemic rheumatic diseases which share the common features of chronic muscle weakness and mononuclear cell infiltrates in muscle. A number of classification schemes have been proposed for them, but none takes into consideration the marked immunologic, clinical, and genetic heterogeneity of the various clinical groups. We compared the usefulness of myositis-specific autoantibodies (anti-aminoacyl-tRNA synthetases, anti-SRP, anti-Mi-2 and anti-MAS) to the standard clinical categories (polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, and inclusion body myositis) in predicting clinical signs and symptoms, HLA types, and prognosis in 212 adult IIM patients. ⋯ These findings suggest that myositis-specific autoantibody status is a more useful guide than clinical group in assessing patients with myositis, and that specific associations of immunogenetics, immune responses, and clinical manifestations occur in IIM. Thus the myositis-specific autoantibodies aid in interpreting the diverse symptoms and signs of myositis patients and in predicting their clinical course and prognosis. We propose, therefore, that an adjunct classification of the IIM, based on the myositis-specific autoantibody status, be incorporated into future studies of their epidemiology, etiology, and therapy.
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Review Case Reports
Purpura fulminans and symmetrical peripheral gangrene caused by Capnocytophaga canimorsus (formerly DF-2) septicemia--a complication of dog bite.
We review the syndrome of Capnocytophaga canimorsus (DF-2) septicemia after exposure to dogs or cats. We describe a previously healthy patient who developed diffuse intravascular coagulation and symmetrical peripheral gangrene as a result of C. canimorsus septicemia after a dog bite. To our knowledge, this patient was the first to receive combined plasmapheresis and leukapheresis for C. canimorsus septicemia. ⋯ Other complications include arthritis, meningitis, and endocarditis. These manifestations can also be found in previously healthy patients, although a prior splenectomy or other causes of impaired host defense clearly enhance the risk. Prompt treatment with penicillin G is required.
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We reviewed 153 episodes of cobalamin deficiency involving the nervous system that occurred in 143 patients seen over a recent 17-year period at 2 New York City hospitals. Pernicious anemia was the most common underlying cause of the deficiency. Neurologic complaints, most commonly paresthesias or ataxia, were the first symptoms of Cbl deficiency in most episodes. ⋯ The extent of neurologic involvement after treatment was strongly related to that before therapy as well as to the duration of symptoms. The percent improvement over baseline neurologic status after treatment was inversely related to duration of symptoms and hematocrit. Some evidence of response was always seen during the first 3 months of treatment.(ABSTRACT TRUNCATED AT 400 WORDS)
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Review Case Reports
Pyogenic infection of the sacroiliac joint. Case reports and review of the literature.
Three cases of pyogenic sacroiliitis are described, and the English literature from 1878 to 1990 reviewed, for a total of 166 cases. In 1 patient the source of infection was identified at the site of an intravenous line; 1 patient had 2 risk factors for developing the disease (pregnancy and intravenous drug use); and a third patient had no source of infection and no associated risk factors. The diagnosis of pyogenic sacroiliitis was made in each patient by history, physical examination, and positive skeletal scintigraphy or computed tomography of the sacroiliac joint. ⋯ In patients whose blood cultures fail to reveal a causative organism, fluoroscopic guided fine-needle aspiration of the sacroiliac joint under general anesthesia may help to identify the organism. If all cultures are negative, open biopsy of the sacroiliac joint may be required. Open biopsy should also be done if sequestration or an abscess is formed, or if the patient fails to respond to antibiotic therapy.(ABSTRACT TRUNCATED AT 400 WORDS)