A 64-year-old woman with a liver abscess, Clostridium perfringens sepsis, progressive sensorimotor neuropathy, and abnormal serum proteins, case 42-1989

Article Abstract:

A 64-year-old black woman was first seen at the hospital with a severe and persistent headache. Her laboratory results also showed that she had an elevated erythrocyte sedimentation rate, which is the rate at which red blood cells settle out of anti-coagulated blood. This is an indication that an inflammatory process is occurring somewhere in the body. She underwent biopsies of her temporal arteries on both sides of the scalp, which appeared to be inflamed. She was placed on prednisone, an anti-inflammatory steroid, and had a bone scan, which showed some abnormalities. She returned to the hospital several months later with complaints of tingling and weakness of her right leg. On examination no compression of the nerves could be found. Muscle biopsies to evaluate arteries of the muscle and tests for rheumatoid arthritis were negative. Thirty-five days earlier the patient had been admitted to another hospital for a generalized infection by the bacteria Clostridium perfringens, at which time a liver abscess was treated with antibiotics and a drain tube was inserted into the affected part of the liver. Isolated and numerous neural problems were experienced and confirmed by laboratory electrophysiologic tests. The patient's widespread and multisystemic disease was suggestive of systemic arteritis, inflammation of the arteries. The differential diagnosis is discussed, as are the clinical findings and anatomic findings which led to the final diagnosis, polyarteritis nodosa. This condition is a multisystemic process which destroys small and medium-sized muscular arteries, which have a relatively thicker surrounding layer of smooth muscle than do so-called elastic arteries. Fever, fatigue, and weight loss are frequent symptoms of this disease. The average age at onset is 45 years, and headache, abdominal pain, muscle pain are often what bring the patient to the healthcare provider. If untreated the progress of the disease is unrelenting and the outcome is poor; the five-year survival rate is only 15 percent of afflicted patients.

Practice, Erythrocyte disorders, Cryoglobulinemia, Periarteritis nodosa, Polyarteritis nodosa, Yood, Robert A, Fienber, Robert, Mueller, Peter

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A 46-year-old woman with progressive dementia

Article Abstract:

A 46-year-old woman was admitted to the hospital with cognitive impairment and cerebrospinal fluid containing a high white blood cell count. One month prior to entering this hospital the patient was treated at a substance-abuse program and diagnosed with organic brain syndrome secondary to chronic alcoholism. She subsequently underwent two magnetic resonance (MR) brain scans. The first brain scan showed white matter-abnormality, while the second MR scan ,taken almost four months later, revealed a generalized decrease in brain size. A disorder confined to the central nervous system was diagnosed as the cause of the dementia, which became progressively worse. A tissue biopsy taken from the right frontal lobe of the brain and the overlying meninges established the diagnosis of granulomatous angiitis . The patient has been treated on an outpatient basis with high doses of methylprednisolone on alternating days. She slowly improved but continued to experience difficulty with short term memory and judgment and required assistance with basic activities and mobility six months after her last discharge. When seen again five months later the patient was able to care for herself but still had impaired short term memory, and continued to take the steroid medication.

Research, Causes of, Complications and side effects, Dementia, Inflammation, Brain, Central nervous system diseases, column, Brain biopsy, Levine, David N., Richardson, Edward P., Jr.

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A 19-year-old man with progressive proximal muscle weakness, contractures, and cardiac abnormalities

Article Abstract:

A 14 1/2-year-old boy admitted for muscle weakness was examined again when at age 19 complaining of accelerated muscle weakness. The diagnosis of Emery-Dreifuss muscular dystrophy was based partially on a biopsy of the right quadriceps muscle. This disorder has symptoms of progressive muscle weakness, muscle contractures and progressive atrial conduction problems. The patient must be monitored for indications of pacemaker implantation. Corticosteroids help in the short term because of the inflammatory component of muscular dystrophy. The negative side effects of long-term use as well as disease evaluation problems indicate these drugs should not be considered for long-term use. The patient's mother was an obligate carrier of the disease. There have been two other cases reported with X-linked disease and typical clinical picture.

Care and treatment, Muscular dystrophy, Muscle hypotonia

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