11/30. A case of rapidly progressive Rosai-Dorfman disease restricted to the central nervous system.Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is an idiopathic condition typically associated with cervical lymphadenopathy, fever and hypergammaglobulinaemia. Extranodal involvement has been reported in diverse sites such as the skin, upper respiratory tract, orbit and the central nervous system. We document a case of intracranial RDD in a 40-year-old woman with rapid evolution over a period of three months. Clinically, the patient suffered from headache. The MRT showed a left parietal tumour with dural attachment. Histologically, the lesion consisted of pale-staining histiocytes with emperipolesis, neutrophilic granulocytes and scattered lymphocytes. Focally, the granulocytes dominated the histological picture. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein, CD68 and FXIIIa, but negative for CD1a. No Birbeck-granula were detectable in electron microscopic analysis. granulocytes showed a positive Anti-HHV6b immunoreaction. The tumour was diagnosed asA'an intracranial manifestation of RDD primary to the CNS with an unusual preponderance of neutrophilic granulocytes and with only scattered lymphocytes. The postoperative clinical staging showed no other manifestations of the disease. On postoperative MRI the lesion had been completely resected. No further therapy has been applied and the patient has had an unremarkable clinical course for the last ten months.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
12/30. A severe case of chronic infantile neurologic, cutaneous, articular syndrome treated with biologic agents.In this report we describe a case of severe chronic infantile neurologic, cutaneous, articular (CINCA) syndrome with a novel G307V cryopyrin mutation and all of the characteristic clinical and laboratory features of this autoinflammatory disease. There was no clear response to standard therapies, including human interleukin-1 (IL-1) receptor antagonist (anakinra) and soluble tumor necrosis factor receptor (etanercept). The patient finally had a partial clinical response (reduction in fever and irritability) and complete laboratory response (improved c-reactive protein and serum amyloid A levels) to humanized anti-IL-6 receptor antibody (MRA), but died from congestive heart failure and interstitial pneumonia 2 months after initiation of therapy. We serially measured the serum cytokine levels and expression of NF-kappaB activation in the patient's peripheral blood mononuclear cells before and during consecutive therapies. Pathologic examination of autopsy specimens was also performed. This case illustrates the continued difficulty in management of patients with CINCA syndrome and the complexity of the inflammatory pathways in this disorder.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
13/30. Neuro-Behcets/neuro-Sweets disease presents simultaneously with severe tonsillitis, and features mimicking bacterial meningitis with skin lesions.The patient was hospitalized due to rapidly undulant fever and sore throat. Empirical antibiotic therapy was started, however, headache also occurred. Lumbar puncture disclosed polynuclear leukocyte-predominant pleocytosis indicating that the patient suffered from bacterial meningitis. The antibiotics therapy was increased, however, consciousness became impaired and erythema multiforme-like skin lesions appeared. T2-weighted brain magnetic resonance imaging showed high signal intensity in the brainstem. HLA testing revealed B54 and Cw1. The patient presented futures of Behcets disease at the same time as those of Sweets syndrome and it was difficult to distinguish between the two diseases. Administration of prednisolone showed remarkable effect.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
14/30. Neurologic abnormalities in a patient with human ehrlichiosis.Human ehrlichiosis is a tick-borne rickettsial disease characterized by fever, headache, myalgias, anorexia, and occasionally rash. In our patient, changes in mental status, upper motor neuron signs, cerebrospinal fluid pleocytosis, and increased serum protein levels were found in association with serologically confirmed ehrlichiosis and were most likely due to vasculitis involving the central nervous system. Intraleukocytic inclusions, although observed in our case, have been infrequently found in other reported cases of ehrlichiosis.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
15/30. brucellosis of the central nervous system. A case report of an infected infant.brucellosis in early infancy is unusual and reports of congenitally acquired infection are extremely rare. The patient presented at the age of 8 months with high fever and signs of meningitis. He had a previous history of hydrocephalus undergoing shunt alleviation at 1 month of age. A diagnosis of central nervous system (CNS) brucellosis was subsequently made. Transplacental transmission is offered as a possible explanation for the acquisition of this child's brucellosis infection.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
16/30. Extra-intestinal manifestations of salmonella infections.While salmonellosis is often considered to affect primarily the gastrointestinal tract, infection at other sites may occur, producing characteristic clinical syndromes. We reviewed cases from our institutions and the literature on focal manifestations of salmonella infections. In the past, most extra-intestinal salmonella infections were caused by S. choleraesuis; however, we found S. typhimurium to be the predominant serotype. The mortality rate for patients in our series was considerably lower than the rate described for focal infections in other reviews. This may in part be due to lower proportion of infections due to S. choleraesuis, improved microbiologic and diagnostic techniques, increased use of ampicillin, and improved surgical techniques. Salmonella endocarditis usually occurs in patients with preexisting heart disease. Unlike other salmonella infections, S. choleraesuis is the most frequent serotype. Salmonella endocarditis is often very destructive, with a fatality rate of 70%. Nonvalvular (mural) endocarditis occurs in one-fourth of patients and survival has not been reported. While antibiotic therapy should be tried initially, if response is not prompt the clinician should look for an associated site of infection (intra- or extra-cardiac abscess), which will often require surgery. Salmonella pericarditis often presents with cardiac or pulmonary symptoms, but typical signs of pericardial disease (pulsus paradoxus, friction rub) or characteristic electrocardiographic changes (low voltage, elevated ST segments) are uncommon. early diagnosis, before infection involves other areas of the heart, is crucial for survival. In addition to antibiotic therapy, pericardiocentesis or pericardiectomy is required. Salmonella may infect the peripheral or visceral arteries, but the abdominal aorta is the most frequent site of vascular infection. Most patients are men over age 50 with preexisting atherosclerosis of the aorta who do not have a previous history of gastroenteritis. About one-fourth of patients have associated lumbar osteomyelitis. No patients have been reported to survive with medical therapy alone. Specific guidelines for surgical removal of infected aneurysms have been proposed and these (in addition to increased use of ampicillin) may be responsible for higher survival rates in recent years. Due to the high incidence of relapses, postoperative blood cultures should be done routinely. Arterial infection should be considered in any elderly patient with salmonella bacteremia especially with prolonged fever or bacteremia after an "adequate course" of antibiotic therapy.(ABSTRACT TRUNCATED AT 400 WORDS)- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
17/30. Atypical presentation and neuropathological studies in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.A 6 1/2-month-old male offspring of consanguineous Egyptian parents was first seen because of fever, somnolence, vomiting, right focal motor seizures, right hemiparesis, elevated transaminase levels, hyperammonemia, and acidosis. A computed tomographic scan of the head suggested swelling of the left cerebral hemisphere, and an electroencephalogram indicated left frontotemporal abnormalities, but brain biopsy demonstrated diffuse white matter spongiosis and gliosis. Subsequently, urine organic acid analysis and enzyme assays were diagnostic of 3-hydroxy-3-methylglutaryl-coenzyme a lyase deficiency.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
18/30. Infantile multisystem inflammatory disease: a specific syndrome?We report two patients with infantile onset of evanescent rash, fever, arthropathy with severe deformities, periosteal changes, chronic meningitis, hydrocephalus, convulsions, developmental delay, papilledema, unusual uveitis, and lymphadenopathy. A few patients with similar findings have been previously reported. Although some similarity exists between findings in these patients and in others with systemic juvenile rheumatoid arthritis, they appear to differ both in regard to the nature and severity of the clinical and pathologic features. We suggest that this group of patients has a separate rheumatic disorder not yet included in the standard classifications of the childhood rheumatic diseases.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
19/30. Epidemic hemorrhagic fever in Hubei Province, The People's Republic of china: a clinical and serological study.Between July 1975 and April 1980, 71 patients were admitted to the Second Attached Hospital of Hubei Provincial Medical College in Wuchang with the diagnosis of epidemic hemorrhagic fever (EHF). The clinical course among these patients was similar to that described for patients with Korean hemorrhagic fever, and hemorrhagic fever with renal syndrome of the U.S.S.R. The overall mortality was 11.2 percent. Sera obtained from some of these patients as well as from patients admitted to the First Attached Hospital of Hubei Provincial Medical College were tested against an antigen associated with Korean hemorrhagic fever and showed exceedingly high antibody titers. We conclude that EHF in Central china represents the same or a closely related disease process as Korean hemorrhagic fever.- - - - - - - - - - ranking = 9keywords = fever (Clic here for more details about this article) |
20/30. Tuberculous spondylitis. A report of six cases and a review of the literature.Tuberculous spondylitis in the united states is a disease of decreasing incidence and, when the incidence is compared to that of developing nations, affects an older population. The thoracic and lumbar spine is most frequently involved, and in advanced disease several vertebrae may be destroyed with resulting significant morbidity and mortality. The usual clinical presentation consists of fever, back pain, and nonspecific systemic symptoms of varying duration. More advanced disease presents with neurological deficits, kyphotic deformities of the spinal column, and paravertebral cold abscesses. Treatment has changed in the past 50 years not only because of the introduction of highly effective anti-tuberculous chemotherapeutic agents, but also because of the development of new surgical approaches. Controlled studies comparing various surgical and more conservative therapeutic regimens have been conducted within the past decade. Despite these new data, controversy remains regarding the indications for surgical treatment, since anti-tuberculous chemotherapy alone is successful in a large proportion of cases. However, in situations where rapid loss of neurologic function is evident or in which progressive deterioration in spinal cord function continues in the face of apparently adequate drug therapy, it appears that anterior surgical decompression of the spinal cord is indicated to prevent irreversible neurologic deficits. Needle biopsy of bone lesions under fluoroscopic or computed tomographic control is essential in the initial evaluation of patients in order to obtain cultural confirmation of tuberculosis.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
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