Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
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PMID:A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis? 1546 68

A 68-year-old man was diagnosed as drug-induced hypersensitivity syndrome (DIHS) with erythema and liver dysfunction three weeks after an administration of phenobarbital. Three weeks after the appearance of skin eruptions, the patient developed disturbed consciousness and status epilepticus. MRI findings revealed bilateral symmetrical abnormalities localized in the amygdala, hippocampi, parahippocampal gyri, and insula regions, which showed high intensities on diffusion weighted images and decreased apparent diffusion coefficient (ADC) values on an ADC map. We considered that the MRI abnormalities were indicative of cytotoxic edema. Increased serum anti-human herpesvirus 6 (HHV-6) IgG antibody titer and presence of HHV-6 DNA in peripheral blood, revealed by polymerase chain reaction (PCR) analysis, suggested reactivation of HHV-6. However, PCR analysis performed with DNA extracted from the CSF was negative for HHV-6. We concluded that limbic encephalitis in the present case might have been caused by an autoimmune inflammatory mechanism associated with drug-induced hypersensitivity syndrome, as well as a direct infection of HHV-6 to the central nervous system.
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PMID:[Limbic encephalitis associated with drug-induced hypersensitivity syndrome due to phenobarbital--a case report]. 1611 31

Human parvovirus B19 is the agent that causes erythema infectiosum (fifth disease), a disease that is generally benign and self-limiting. This virus also is associated with severe disease in hemolytic or immunocompromised patients. It rarely causes meningoencephalitis in healthy children. Herein we present the case of a 10-year-old healthy girl with refractory status epilepticus possibly owing to human parvovirus B19 encephalitis who was successfully treated with high-dose corticosteroids. To our knowledge, this is the first report of human parvovirus B19 encephalitis complicated by refractory status epilepticus in a child.
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PMID:Refractory status epilepticus owing to human parvovirus B19 encephalitis in a child. 1697 Aug 96

Japanese spotted fever (JSF), first reported in 1984, is a rickettsial disease characterized by high fever, rash, and eschar formation. A 61-year-old man was admitted to a local hospital in Nagasaki City, Japan, after several days of high fever and generalized skin erythema. His condition deteriorated and laboratory findings indicated disseminated intravascular coagulation (DIC). The patient was transferred to our hospital with mental disturbance and status epilepticus. Treatment included minocycline, and new quinolone. Definitive diagnosis was made with a serological test showing increased antibody levels against Rickettsia japonica. Rickettsial infections are rare, but should be seriously considered for the differential diagnosis of aseptic meningitis and encephalitis, as they show no response to conventional antibiotic treatment.
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PMID:A case of Japanese spotted fever complicated with central nervous system involvement and multiple organ failure. 2246 39

A 73-year-old man presented in status epilepticus. He had a long history of epilepsy for which he was treated with regular phenytoin and phenobarbitone. On admission, his phenytoin level was found to be subtherapeutic and was treated with rectal diazepam and intravenous lorazepam. He was later started on an infusion of phenytoin in preparation to restart his oral medication. The medication was delivered via a peripheral cannula in his forearm. Five days later, he developed redness and swelling of this arm followed by the appearance of a large ulcer. After ruling out infective causes and a compartment syndrome it was felt that the patient had developed a delayed purple glove syndrome secondary to intravenous phenytoin. Following 7 days of regular saline irrigation and dressing changes, the erythema improved and the ulcer showed signs of healing, and eventually resolved.
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PMID:An atypical case of purple glove syndrome: an avoidable adverse event. 2277 51