1/33. Influenza virus-induced encephalopathy: clinicopathologic study of an autopsied case.BACKGROUND: Rapid progressive encephalopathy with a high fever, consciousness loss and recurrent convulsions has been occasionally reported in children during influenza pandemics in japan since 1995. We examined a 2-year old girl with hemorrhagic shock and encephalopathy syndrome associated with acute influenza a virus infection (A/Nagasaki/76/98; H3N2), to answer several questions for which no histologic or virologic data exist. methods: A clinicopathologic study using immunohistochemical staining and viral genome detection by reverse transcriptase polymerase chain reaction (RT-PCR) was performed with this autopsied case. RESULTS: The virus antigen was positive in CD8 T lymphocytes from the lung and spleen. The virus infected a very limited part of the brain, especially Purkinje cells in the cerebellum and many neurons in the pons, without inducing an overt immunologic reaction from the host. The RT-PCR used for detecting the hemagglutinin gene demonstrated positive bands in all frozen tissues and cerebrospinal fluid taken at autopsy and not in samples obtained on admission. CONCLUSIONS: The pathologic change induced by the direct viral invasion cannot be responsible for all of the symptoms, especially for the rapid and severe clinical course of the disease within 24-48 h after the initial respiratory symptoms. Together with the rapid production of several inflammatory cytokines, the breakdown of the blood-brain barrier may induce severe brain edema and can be a major pathologic change for the disease. Any therapeutic strategy to control this multistep progression of the disease could be effective.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
2/33. influenza a virus encephalopathy with symmetrical thalamic lesions.During an epidemic of influenza A infection in japan, a 7-year-old boy was admitted to our hospital because of high fever, convulsions, coma, and liver dysfunction on the 2nd day of a cold-like illness. His serum CPK was markedly elevated, but there was no hyperammonaemia or hypoglycaemia. His CSF showed an increased protein level, but the cell count and glucose level were normal. CT and MRI of the brain showed symmetrical thalamic lesions, and he was diagnosed with acute necrotizing encephalopathy in childhood. He had a significant increased in antibodies to influenza A H1N1 in serum and CSF, but the CSF was negative for influenza virus using virus isolation and a polymerase chain reaction assay. CONCLUSION: Antibody production without detectable levels of influenza virus in cerebrospinal fluid suggests that virus infection occurred, but the virus did not replicate in sufficient numbers in his central nervous system. The thalamic lesion, the hallmark of acute necrotizing encephalopathy in childhood, may be initiated by a local virus infection and develop with subsequent local changes such as breakdown of the blood-brain barrier and the extravasation of blood.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
3/33. A novel mutation, P126R, in a Japanese patient with HHH syndrome.Mitochondrial ornithine transporter deficiency, or HHH syndrome, is a metabolic disorder resulting in various neurologic symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia. Several mutations have been reported in the ORNT1 gene encoding mitochondrial ornithine transporter of patients with this disorder. In this article, we report a new patient, a male 15 years of age, who had typical clinical features of HHH syndrome. Because the patient did not have any of the three mutations previously described in other Japanese patients with HHH syndrome, and the only material available from the patient was peripheral leukocytes, we established a genomic polymerase chain reaction method using intronic primers to amplify every exon of the ORNT1 gene, and we directly sequenced the polymerase chain reaction products. Using this method, we documented a novel mutation in this patient, P126R, and demonstrated that HHH syndrome is genetically heterogeneous, even in the Japanese population.- - - - - - - - - - ranking = 2keywords = lyme (Clic here for more details about this article) |
4/33. Acute transient encephalopathy after paclitaxel infusion: report of three cases.paclitaxel (Taxol) is a diterpene plant product and antineoplastic agent that promotes the assembly of microtubules as well as stabilizing their formation by preventing depolymerization. Myelosuppression was found to be dose-limiting, but peripheral neurotoxicity is also a well known side-effect. central nervous system toxicity is rare, probably because paclitaxel does not cross the blood-brain barrier. We observed three patients who presented with acute encephalopathy within 6 h after infusion of paclitaxel at normal doses. All patients had received prior whole brain irradiation (WBI) and one patient had prior brain metastasectomy. Computer tomography and magnetic resonance imaging showed no evidence of cerebral metastases. An effect from other organ toxicities was excluded in all patients. All recovered spontaneously within 4-6 h. From this we can conclude that paclitaxel can cause severe acute transient encephalopathy, which may occur more frequently after prior WBI and/or surgery due to alteration of small vessel function.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
5/33. Ocular and central nervous system paracoccidioidomycosis in a pregnant woman with acquired immunodeficiency syndrome.PURPOSE: To describe an atypical case of central nervous system and ocular paracoccidioidomycoses simulating ocular toxoplasmosis in a pregnant woman with acquired immunodeficiency syndrome (AIDS). DESIGN: Interventional case report. methods: Case report. RESULTS: A 25-year-old pregnant woman with AIDS, presented with a severe ocular inflammation in the right eye involving the choroid, retina, and the optic disk, which rapidly progressed to retinal detachment, iris neovascularization, and neovascular glaucoma. The left eye was normal. magnetic resonance imaging (MRI) showed a focal hypodense contrast-enhanced ring lesion in the brain. Serum antibody titers were negative for toxoplasma gondii, but the polymerase chain reaction was positive for the parasite in the vitreous sample. The patient responded partially to specific treatment for toxoplasmosis, and there was a small reduction in size of the brain lesion. She progressed to a blind painful eye, which was enucleated. paracoccidioides brasiliensis was found in the histopathological studies of the eye and oropharynx. With the diagnosis of disseminated ocular paracoccidioidomycoses, the patient was treated with trimethoprim-sulfamethoxazole with a satisfactory outcome and reduction in size of the brain lesion. CONCLUSION: Although ocular infection with ocular paracoccidioidomycoses is rare, this diagnosis should be considered when investigating ocular inflammation in a patient with AIDS.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
6/33. Intracranial plasma cell granuloma with genetic analysis.BACKGROUND: Plasma cell granuloma of the central nervous system is rare and less than 30 cases have been reported in literature. METHOD/FINDINGS: A 64-year-old woman complained of headache, and computed tomography (CT) and magnetic resonance (MR) imaging of the brain revealed a tumour at the right anterior-third parasagittal region which led us to diagnose a parasagittal meningioma. We performed total resection of the tumour and the pathological diagnosis was plasma cell granuloma. polymerase chain reaction (PCR) analysis of immunoglobulin heavy chain (IgH) gene rearrangements produced no monoclonal band. This patient underwent no supplementary treatment and no recurrent tumour was observed for 3 years. INTERPRETATION: A case of plasma cell granuloma which is diagnosed with genetic analysis is presented. An analysis of the clonality using the PCR procedure would play an important role in supporting the pathological and clinical diagnosis.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
7/33. Whipple's disease with destructive arthritis, abdominal lymphadenopathy, and central nervous system involvement.We describe a patient with Whipple's disease who had an unusual erosive and destructive polyarthritis, massive abdominal lymphadenopathy, asymptomatic central nervous system involvement, and rare manifestations of orbital pseudotumor and orchitis with epididymitis. Taking oral therapy with trimethoprim-sulfamethoxazole he had recurrent flares of orbital pseudotumor, an episode of orchitis with epididymitis, and persistent polymerase chain reaction T. whipplei-positive cerebrospinal fluid. Resolution was achieved with a one month course of intravenous ceftriaxone and a 6 month course of azithromycin, and no relapse occurred during 24 months of followup.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
8/33. Detection of human immunodeficiency virus type 2 in brain tissue.infection due to human immunodeficiency virus (HIV) type 2 is believed to cause a clinical picture similar to that of hiv-1, although extensive data are not available. In 2 patients with West African exposure and neurologic symptoms, hiv-2 was detected in the central nervous system using dna and rna polymerase chain reaction, in situ hybridization, and immunohistology. In the first patient, the neurologic disease was most likely due to productive infection with hiv-2. In the second, a combination of neuropathologic abnormalities (including the presence of hiv-2) explained the clinical features. Thus hiv-2, like hiv-1, can be readily detected in brain tissue in patients with neurologic abnormalities, although the exact role of hiv-2 in pathogenesis of AIDS-associated neurologic disease requires further study.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
9/33. mitochondrial encephalomyopathies with the mutation of the mitochondrial tRNA(Leu(UUR)) gene.Four families with mitochondrial encephalomyopathy are described. Probands of three families had typical clinical presentations of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), but the proband of family 4 lacked strokelike episodes. The mitochondrial dna mutation of tRNA(Leu(UUR)) (transfer ribonucleic acid specific to leucine (UUR codon)) found in MELAS was examined in muscle dna obtained from biopsy samples of the probands of four families and the maternal relatives of family 2. The mutation was detected in all muscle samples, and the degree of the mutated dna was 68% to 84% by Southern blot analysis. However, the clinical patterns of the maternal relatives of family 2 were mild and distinctly different from MELAS. The same mutation was also detected in blood-derived dna samples of all family members examined, including healthy mothers but not fathers, although the degree of mutation did not correlate with the clinical severity. These results confirmed the maternal inheritance of this disease and suggested that the mitochondrial dna mutation (tRNA(Leu(UUR))) may cause clinical symptoms other than MELAS. The clinical findings of mitochondrial encephalomyopathy should be reinvestigated in terms of the mitochondrial gene mutation; the polymerase chain reaction method will be useful for screening for this mutation of mitochondrial dna in blood samples.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
10/33. Amyloidoma of the CNS. II. Immunohistochemical and biochemical study.We present the immunohistochemical and biochemical identification of an amyloidoma localized to the cerebral white matter in a patient who shows no evidence of systemic or extracranial localized amyloid deposits. Immunohistologic and immunochemical studies, using antibodies against biochemically different amyloid fibrils and amyloid-associated proteins, showed reactivity with antibodies only to lambda light chain and serum amyloid p-component. amino acid sequence analysis of the purified amyloid fibrils extracted from the brain tumor indicates that the amyloid protein is an unusual immunoglobulin lambda light chain, starting at residue five of the variable domain. These fibrils consist of lambda chain fragments of different lengths (10 to 30 kd) very likely arising by polymerization of the amyloid subunit or sequential proteolytic cleavage of the light chain, or both. After exposure to denaturing agents, the 10-kd subunit retains the characteristics of native amyloid fibrils by electron microscopy.- - - - - - - - - - ranking = 1keywords = lyme (Clic here for more details about this article) |
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