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Brain Biopsy (brain + biopsy)
Selected AbstractsFebrile infection,related epilepsy syndrome (FIRES): A nonencephalitic encephalopathy in childhoodEPILEPSIA, Issue 7 2010Andreas Van Baalen Summary Encephalitis is generally presumed, even when seizures follow banal febrile infection, and pathogen detection in cerebrospinal fluid fails. This retrospective multicenter case series reports on 22 previously healthy children aged 3,15 years (median 6.5 years) with prolonged or recurrent seizures occurring 2,14 days (median 5 days) after fever onset (19 children with respiratory or nonspecific infections). Cerebrospinal fluid studies revealed 2,42 cells/,l (median 5 cells/,l) and no pathogens. Electroencephalography showed diffuse slowing or multifocal discharges. Neuroimaging demonstrated normal findings in 10 children. Brain biopsies were performed in seven children showing gliosis but no inflammation. Anesthetic barbiturates were used in 14 children with refractory status epilepticus, and immunotherapy in 9. Two children died, eight remained in a state of impaired consciousness, eight developed therapy-refractory epilepsies, two had behavioral disturbances, and two recovered. The lack of evidence for encephalitis suggests another infection-related pathogenesis of this disastrous epileptic encephalopathy. Therefore, we propose the term "febrile infection,related epilepsy syndrome" (FIRES). [source] Viral meningoencephalitis: a review of diagnostic methods and guidelines for managementEUROPEAN JOURNAL OF NEUROLOGY, Issue 8 2010I. Steiner Background:, Viral encephalitis is a medical emergency. The prognosis depends mainly on the pathogen and host immunologic state. Correct immediate diagnosis and introduction of symptomatic and specific therapy has a dramatic influence upon survival and reduces the extent of permanent brain injury. Methods:, We searched the literature from 1966 to 2009. Recommendations were reached by consensus. Where there was lack of evidence but consensus was clear, we have stated our opinion as good practice points. Recommendations:, Diagnosis should be based on medical history and examination followed by CSF analysis for protein and glucose levels, cellular analysis, and identification of the pathogen by polymerase chain reaction amplification (recommendation level A) and serology (level B). Neuroimaging, preferably by MRI, is essential (level B). Lumbar puncture can follow neuroimaging when immediately available, but if this cannot be performed immediately, LP should be delayed only under unusual circumstances. Brain biopsy should be reserved only for unusual and diagnostically difficult cases. Patients must be hospitalized with easy access to intensive care units. Specific, evidence-based, antiviral therapy, acyclovir, is available for herpes encephalitis (level A) and may also be effective for varicella-zoster virus encephalitis. Ganciclovir and foscarnet can be given to treat cytomegalovirus encephalitis, and pleconaril for enterovirus encephalitis (IV class evidence). Corticosteroids as an adjunct treatment for acute viral encephalitis are not generally considered to be effective, and their use is controversial, but this important issue is currently being evaluated in a large clinical trial. Surgical decompression is indicated for impending uncal herniation or increased intracranial pressure refractory to medical management. [source] Viral encephalitis: a review of diagnostic methods and guidelines for managementEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2005I. Steiner Viral encephalitis is a medical emergency. The spectrum of brain involvement and the prognosis are dependent mainly on the specific pathogen and the immunological state of the host. Although specific therapy is limited to only several viral agents, correct immediate diagnosis and introduction of symptomatic and specific therapy has a dramatic influence upon survival and reduces the extent of permanent brain injury in survivors. We searched MEDLINE (National Library of Medicine) for relevant literature from 1966 to May 2004. Review articles and book chapters were also included. Recommendations are based on this literature based on our judgment of the relevance of the references to the subject. Recommendations were reached by consensus. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. Diagnosis should be based on medical history, examination followed by analysis of cerebrospinal fluid for protein and glucose contents, cellular analysis and identification of the pathogen by polymerase chain reaction (PCR) amplification (recommendation level A) and serology (recommendation level B). Neuroimaging, preferably by magnetic resonance imaging, is an essential aspect of evaluation (recommendation level B). Lumbar puncture can follow neuroimaging when immediately available, but if this cannot be obtained at the shortest span of time it should be delayed only in the presence of strict contraindications. Brain biopsy should be reserved only for unusual and diagnostically difficult cases. All encephalitis cases must be hospitalized with an access to intensive care units. Supportive therapy is an important basis of management. Specific, evidence-based, anti-viral therapy, acyclovir, is available for herpes encephalitis (recommendation level A). Acyclovir might also be effective for varicella-zoster virus encephalitis, gancyclovir and foscarnet for cytomegalovirus encephalitis and pleconaril for enterovirus encephalitis (IV class of evidence). Corticosteroids as an adjunct treatment for acute viral encephalitis are not generally considered to be effective and their use is controversial. Surgical decompression is indicated for impending uncal herniation or increased intracranial pressure refractory to medical management. [source] Combined Use of F-18 Fluorocholine Positron Emission Tomography and Magnetic Resonance Spectroscopy for Brain Tumor EvaluationJOURNAL OF NEUROIMAGING, Issue 3 2004Sandi A. Kwee MD ABSTRACT Background. Choline metabolism is often abnormal in malignant brain tumors.Methods. Brain positron emission tomography (PET) imaging with F-18 fluorocholine (FCH) was performed on 2 patients with intracranial lesions suspected to be high-grade malignant gliomas on the basis of magnetic resonance (MR) imaging and multivoxel 1H-MR spectroscopic imaging (MRSI) findings. Standardized uptake value (SUV) measurements on PET were compared with measurements of choline/creatine metabolite ratio on MRSI in corresponding regions. Brain biopsy revealed glioblastoma multiforme (GBM) in one case and demyelinating disease in the other.Results. In the case of GBM, the tumor demonstrated increased FCH uptake on PET. The mean and maximum SUV in areas of the tumor correlated with regional choline/ creatine ratio measurements (r= 0.76,P < .001;r= 0.83,P < .001, respectively). In the case of tumefactive demyelinating lesions, the lesion demonstrated low FCH uptake, which did not correlate with choline/ creatine ratio measurements.Conclusions. Assessments of choline metabolism may aid in evaluating intracranial mass lesions. [source] Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons,ANNALS OF NEUROLOGY, Issue 6 2009Christian Wüthrich PhD The polyomavirus JC (JCV) is the causative agent of progressive multifocal leukoencephalopathy and of JCV granule cell neuronopathy. We present a human immunodeficiency virus,negative patient who experienced development of multiple cortical lesions, aphasia, and progressive cognitive decline after chemotherapy for non,small-cell lung cancer. Brain biopsy and cerebrospinal fluid polymerase chain reaction demonstrated JCV, and she had a rapidly fatal outcome. Postmortem analysis showed diffuse cortical lesions and areas of necrosis at the gray,white junction. Immunostaining showed a productive JCV infection of cortical pyramidal neurons, confirmed by electron microscopy, with limited demyelination. This novel gray matter syndrome expands the scope of JCV clinical presentation and pathogenesis. Ann Neurol 2009;65:742,748 [source] Primary necrotizing lymphocytic central nervous system vasculitis due to perforin deficiency in a four-year-old girlARTHRITIS & RHEUMATISM, Issue 3 2007Despina Moshous We report the case of a 4-year-old girl who presented with headaches, ataxia, and visual disturbances. Cranial magnetic resonance imaging showed multiple supra- and infratentorial lesions with peripheral contrast enhancement and central necrosis. Brain biopsy revealed necrotizing lymphocytic vasculitis of undetermined etiology. Perforin expression was found to be significantly reduced in the patient's peripheral blood cells, and sequence analysis of the patient's perforin gene showed a compound heterozygous state with 1 nonsense mutation and 2 missense alterations in exon 2. Central nervous system (CNS) vasculitis was thus attributed to the perforin deficiency, and the patient was successfully treated by transplantation of stem cells from an HLA-identical brother. The findings described herein indicate that, even in the absence of classic non-neurologic symptoms of hemophagocytic lymphohistiocytosis, measurement of perforin expression should be one of the diagnostic tests used to identify the cause of unexplained CNS vasculitis, since this may have profound implications regarding therapy. [source] Rheumatoid Leptomeningitis: Magnetic Resonance Imaging and Pathologic Findings,A Case ReportJOURNAL OF NEUROIMAGING, Issue 2 2010Alessandro Cianfoni MD ABSTRACT BACKGROUND AND PURPOSE Rheumatoid arthritis (RA) is a chronic inflammatory multisystem disease with articular and extra-articular manifestations. Intracranial manifestations of RA are rare. Purpose of this article is to report on a rarely described leptomeningeal involvement in RA, and on its neuroimaging features, including diffusion-weighted imaging (DWI). METHODS The authors describe the case of a 74-year-old woman with a 5-year history of RA presenting with progressive left-side weakness and hypoesthesia. The patient underwent laboratory investigation and brain contrast-enhanced MRI, also with DWI, before undergoing brain biopsy. RESULTS Neuroimaging revealed abnormal high T2-signal in right frontal and parietal lobes, restricted diffusion in the subarachnoid space, and diffuse thick linear leptomeningeal contrast-enhancement. These findings were interpreted as rheumatoid leptomeningitis, and brain biopsy confirmed this diagnosis. CONCLUSIONS In summary, rheumatoid meningitis is a rare neurological complication of RA, but it should be considered in the proper clinical setting when patient presentation and laboratory results fail to support the other differential diagnostic possibilities proposed by the MR imaging findings. [source] An Unusual Presentation of Rheumatoid MeningitisJOURNAL OF NEUROIMAGING, Issue 3 2005Vaidehi Chowdhry MD ABSTRACT Background. Central nervous system involvement in rheumatoid arthritis can rarely occur in the absence of systemic disease. Rheumatoid meningitis has not been reported to present as spells of neurologic dys-function. Patient and Methods. The authors describe a woman with a history of well-controlled rheumatoid arthritis who presented with headaches and spells of focal neurological dysfunction. Brain magnetic resonance imaging, brain biopsy, and temporal artery biopsy were required to make the diagnosis of rheumatoid meningitis with arteritis. Results. Neuroimaging revealed abnormal leptomeningeal enhancement. Necrotizing granulomatous inflammation was seen on meningeal and brain biopsy. A temporal artery biopsy showed evidence of arteritis without giant cells. Conclusions. The possibility of central nervous system involvement by rheumatoid arthritis should be considered in patients with a history of rheumatoid arthritis even in the absence of systemic symptoms. Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive. [source] Cure of Acanthamoeba cerebral abscess in a liver transplant patientLIVER TRANSPLANTATION, Issue 3 2008Konrad Tang-Tat Fung Acanthamoeba-related cerebral abscess and encephalitis are rare but usually fatal, being caused by free-living amoebic infections usually occurring in immunocompromised patients. In patients receiving transplants, a literature review showed that the infection is universally fatal. The diagnosis is often missed despite appropriate investigations including lumbar puncture, computerized tomography, and brain biopsy. We present the first reported liver transplant patient with Acanthamoeba cerebral abscess. The diagnosis was made in brain tissue removed at decompressive frontal lobectomy. He was successfully treated with a 3-month course of co-trimoxazole and rifampicin. There was no recurrence of the disease after 11 years of follow-up. Liver Transpl 14:308,312, 2008. © 2008 AASLD. [source] Transmission of Anaplastic Large Cell Lymphoma via Organ Donation After Cardiac DeathAMERICAN JOURNAL OF TRANSPLANTATION, Issue 1 2008J. W. Harbell Recently, donation after cardiac death (DCD) has been encouraged in order to expand the donor pool. We present a case of anaplastic T-cell lymphoma transmitted to four recipients of solid organ transplants from a DCD donor suspected of having bacterial meningitis. On brain biopsy, the donor was found to have anaplastic central nervous system T-cell lymphoma, and the recipient of the donor's pancreas, liver and kidneys were found to have involvement of T-cell lymphoma. The transplanted kidneys and pancreas were excised from the respective recipients, and the kidney and pancreas recipients responded well to chemotherapy. The liver recipient underwent three cycles of chemotherapy, but later died due to complications of severe tumor burden. We recommend transplanting organs from donors with suspected bacterial meningitis only after identification of the infectious organism. In cases of lymphoma transmission, excision of the graft may be the only chance at long-term survival. [source] Primary central nervous system vasculitis: analysis of 101 patientsANNALS OF NEUROLOGY, Issue 5 2007Carlo Salvarani MD Objective To analyze the clinical findings, response to therapy, outcome, and incidence of primary central nervous system vasculitis (PCNSV) in a large cohort from a single center Methods We retrospectively studied 101 patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 21-year period. This was a collaborative study by five departments at a large multispecialty clinic. Clinical findings and outcomes were compared among patients categorized by method of diagnosis, response to therapy, survival, and degree of disability. An annual incidence rate was calculated Results Seventy patients were diagnosed by angiography and 31 by central nervous system biopsy. Three histological patterns were observed during biopsy. Although most patients responded to therapy, an increased mortality rate was observed. Relapses occurred in one fourth of patients. Mortality rate and disability at last follow-up were greater in those who presented with a focal neurological deficit, cognitive impairment, cerebral infarctions, and angiographic large-vessel involvement but were lower in those with prominent gadolinium-enhanced lesions when evaluated by magnetic resonance imaging. The annual incidence rate of PCNSV was 2.4 cases per 1,000,000 person-years Interpretation PCNSV is a rare disease that may result in serious neurological outcomes or death. Angiography and brain biopsy may complement each other when determining the diagnosis. Early recognition and treatment may reduce poor outcomes. PCNSV is a variable syndrome that appears to consist of several subsets of heterogeneous diseases. Ann Neurol 2007 [source] | ||||||||||||||||||||||