The Experts below are selected from a list of 537 Experts worldwide ranked by ideXlab platform
Diego Franciotta - One of the best experts on this subject based on the ideXlab platform.
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cerebrospinal fluid analysis in immunoglobulin g4 related hypertrophic pachymeningitis
The Journal of Rheumatology, 2013Co-Authors: Emanuel Dellatorre, Gabriella Passerini, Luisa Roveri, R Chieffo, Nicoletta Anzalone, Elisabetta Zardini, Roberto Furlan, Maria Grazia Sabbadini, Claudio Doglioni, Diego FranciottaAbstract:To the Editor: Immunoglobulin G4-related disease (IgG4-RD) is characterized by fibrous swelling of affected organs, elevations in serum IgG4 concentrations, and responsiveness to glucocorticoid treatment1. Affected tissues display similar histological features: diffuse lymphoplasmacytic infiltration by numerous IgG4-positive plasma cells, occasional eosinophils, storiform fibrosis, and obliterative phlebitis2. IgG4-related hypertrophic pachymeningitis (IgG4-HP) has been identified as a characteristic central nervous system (CNS) manifestation of IgG4-RD, but comprehensive cerebrospinal fluid (CSF) analyses are substantially lacking3,4. Recently, we demonstrated an intrathecal IgG and IgG4 synthesis in the CSF of a patient with IgG4-HP and suggested IgG4 Indices as safe potential diagnostic tools for IgG4-HP5. Here, we describe 2 new cases of IgG4-HP with CSF evaluation at diagnosis and in response to treatment. In case 1, March 2007, a 56-year-old man was admitted with a 1-year history of right frontal headache. Magnetic resonance imaging (MRI) showed right frontal pachymeningitis. Granulomatous Meningeal inflammation was suspected and oral prednisone (1 mg/kg body weight/day) was started with clinical and radiological improvement. Prednisone was discontinued after 8 months, but in July 2008 the headache recurred. Blood and CSF analyses are shown in Table 1. MRI evidenced a right frontotemporal progression of the pachymeningitis (Figure 1A). A Meningeal Biopsy … Address correspondence to Dr. E. Della-Torre, Department of Medicine and Clinical Immunology, San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy. E-mail: dellatorre.emanuel{at}hsr.it
Gary M Miller - One of the best experts on this subject based on the ideXlab platform.
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syndrome of orthostatic headaches and diffuse pachyMeningeal gadolinium enhancement
Mayo Clinic Proceedings, 1997Co-Authors: Bahram Mokri, David G Piepgras, Gary M MillerAbstract:Objective To characterize clinical and imaging features, Biopsy findings, etiologic factors, and outcome in the syndrome of intracranial hypotension, headaches, and diffuse pachyMeningeal gadolinium enhancement on magnetic resonance imaging (MRI). Material and Methods We describe our experience with 26 consecutive patients with orthostatic headaches and diffuse pachyMeningeal gadolinium enhancement, for all of whom clinical, imaging, and follow-up data were available. For 10 patients who had undergone Meningeal Biopsy, slide material was also reviewed. Results The 15 men and 11 women ranged from 24 to 76 years of age. All 26 patients had postural headaches; in 22 patients, the headaches were completely alleviated by recumbency. Nausea or emesis, neck pain, horizontal diplopia, changes in hearing, photophobia, upper limb pains or paresthesias, visual blurring, or dysgeusia was noted in some of the patients. Cardinal MRI features were diffuse pachyMeningeal gadolinium enhancement (100%), subdural collections of fluid (69%), and evidence of descent of the brain (62%) that sometimes resembled type I Chiari malformation. Cerebrospinal fluid (CSF) opening pressures were 40 mm or less in only 46%. In three patients, CSF pressures were consistently no less than 90 and as high as 130 mm of water. A variable pleocytosis of 5 or more cells/mm3 was noted in 15 patients (more than 40 cells/mm3 in 4 patients). A variable increase in CSF protein was noted in at least one spinal tap in 23 patients. Six patients had overdraining CSF shunts; CSF leak was documented in another 11 patients. Shunt revision or ligation and surgical correction of the leak led to a resolution of the clinical and MRI abnormalities in all cases thus treated. Improvement occurred with epidural blood patch in four patients. Three of the 12 patients treated supportively have remained symptomatic. Histologically, a thin subdural zone of fibroblasts and thin-walled vessels was noted in an amorphous matrix. Two patients with prolonged symptoms had a more pronounced proliferative reaction. Conclusion The syndrome of low-pressure headaches and pacbyMeningeal gadolinium enhancement is being recognized with increasing frequency. The source of the CSF leak can be demonstrated in many patients. Meningeal abnormalities are likely attributable to decreased CSF volume and hydrostatic CSF pressure changes. The prognosis is typically good.
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Meningeal Biopsy in intracranial hypotension Meningeal enhancement on mri
Neurology, 1995Co-Authors: Bahram Mokri, Joseph E Parisi, Bernd W Scheithauer, David G Piepgras, Gary M MillerAbstract:Intracranial hypotension is a cause of diffuse enhancement of the pachymeninx with gadolinium, which often is associated with subdural fluid collections. We reviewed the results of Meningeal Biopsy in six patients with intracranial hypotension and diffuse pachyMeningeal enhancement to correlate the MRI findings with histopathologic observations and to explain the abnormalities seen on MRI. Grossly, the dura mater was unremarkable in all patients, as were the leptomeninges, except for one patient with prolonged (18 months) intracranial hypotension in whom the arachnoid was thickened and opaque. Microscopically, the dura mater was entirely normal on its epidural aspect; however, a fairly thin zone of fibroblasts and thin-walled small blood vessels in an amorphous matrix was noted on the subdural aspect. In the patient with longstanding symptoms, diffuse benign arachnoidal cell proliferation was also noted, probably a reaction triggered by longstanding changes in the subdural area, as noted in the five other patients. There was no evidence of inflammation, infection, or metastatic neoplasia. These findings suggest that in intracranial hypotension, the dural-Meningeal abnormalities probably represent reactive secondary phenomena, likely related to hydrostatic changes in the CSF, and not a primary Meningeal process.
Emanuel Dellatorre - One of the best experts on this subject based on the ideXlab platform.
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cerebrospinal fluid analysis in immunoglobulin g4 related hypertrophic pachymeningitis
The Journal of Rheumatology, 2013Co-Authors: Emanuel Dellatorre, Gabriella Passerini, Luisa Roveri, R Chieffo, Nicoletta Anzalone, Elisabetta Zardini, Roberto Furlan, Maria Grazia Sabbadini, Claudio Doglioni, Diego FranciottaAbstract:To the Editor: Immunoglobulin G4-related disease (IgG4-RD) is characterized by fibrous swelling of affected organs, elevations in serum IgG4 concentrations, and responsiveness to glucocorticoid treatment1. Affected tissues display similar histological features: diffuse lymphoplasmacytic infiltration by numerous IgG4-positive plasma cells, occasional eosinophils, storiform fibrosis, and obliterative phlebitis2. IgG4-related hypertrophic pachymeningitis (IgG4-HP) has been identified as a characteristic central nervous system (CNS) manifestation of IgG4-RD, but comprehensive cerebrospinal fluid (CSF) analyses are substantially lacking3,4. Recently, we demonstrated an intrathecal IgG and IgG4 synthesis in the CSF of a patient with IgG4-HP and suggested IgG4 Indices as safe potential diagnostic tools for IgG4-HP5. Here, we describe 2 new cases of IgG4-HP with CSF evaluation at diagnosis and in response to treatment. In case 1, March 2007, a 56-year-old man was admitted with a 1-year history of right frontal headache. Magnetic resonance imaging (MRI) showed right frontal pachymeningitis. Granulomatous Meningeal inflammation was suspected and oral prednisone (1 mg/kg body weight/day) was started with clinical and radiological improvement. Prednisone was discontinued after 8 months, but in July 2008 the headache recurred. Blood and CSF analyses are shown in Table 1. MRI evidenced a right frontotemporal progression of the pachymeningitis (Figure 1A). A Meningeal Biopsy … Address correspondence to Dr. E. Della-Torre, Department of Medicine and Clinical Immunology, San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy. E-mail: dellatorre.emanuel{at}hsr.it
Vani Santosh - One of the best experts on this subject based on the ideXlab platform.
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rosai dorfman disease case with extensive dural involvement and cerebrospinal fluid pleocytosis
Journal of the Neurological Sciences, 2012Co-Authors: Atchayaram Nalini, Saini Jitender, Gudipati Anantaram, Vani SantoshAbstract:We report a young adult man who presented with chronic raised intracranial tension features and unusually progressive bilateral visual and hearing impairment of 18 months duration. MR imaging showed extensive dural involvement and contiguous orbital and spinal disease. Cerebrospinal fluid demonstrated persistent high lymphocytic pleocytosis. Dural Biopsy obtained from posterior cervical approach with C1 arch excision and Meningeal Biopsy revealed features of classical of Rosai-Dorfman disease. Histiocytes were strongly positive for CD-68 and S-100 proteins. The illness relentlessly progressed with patient developing total deafness and near total blindness at last follow-up.
Bahram Mokri - One of the best experts on this subject based on the ideXlab platform.
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syndrome of orthostatic headaches and diffuse pachyMeningeal gadolinium enhancement
Mayo Clinic Proceedings, 1997Co-Authors: Bahram Mokri, David G Piepgras, Gary M MillerAbstract:Objective To characterize clinical and imaging features, Biopsy findings, etiologic factors, and outcome in the syndrome of intracranial hypotension, headaches, and diffuse pachyMeningeal gadolinium enhancement on magnetic resonance imaging (MRI). Material and Methods We describe our experience with 26 consecutive patients with orthostatic headaches and diffuse pachyMeningeal gadolinium enhancement, for all of whom clinical, imaging, and follow-up data were available. For 10 patients who had undergone Meningeal Biopsy, slide material was also reviewed. Results The 15 men and 11 women ranged from 24 to 76 years of age. All 26 patients had postural headaches; in 22 patients, the headaches were completely alleviated by recumbency. Nausea or emesis, neck pain, horizontal diplopia, changes in hearing, photophobia, upper limb pains or paresthesias, visual blurring, or dysgeusia was noted in some of the patients. Cardinal MRI features were diffuse pachyMeningeal gadolinium enhancement (100%), subdural collections of fluid (69%), and evidence of descent of the brain (62%) that sometimes resembled type I Chiari malformation. Cerebrospinal fluid (CSF) opening pressures were 40 mm or less in only 46%. In three patients, CSF pressures were consistently no less than 90 and as high as 130 mm of water. A variable pleocytosis of 5 or more cells/mm3 was noted in 15 patients (more than 40 cells/mm3 in 4 patients). A variable increase in CSF protein was noted in at least one spinal tap in 23 patients. Six patients had overdraining CSF shunts; CSF leak was documented in another 11 patients. Shunt revision or ligation and surgical correction of the leak led to a resolution of the clinical and MRI abnormalities in all cases thus treated. Improvement occurred with epidural blood patch in four patients. Three of the 12 patients treated supportively have remained symptomatic. Histologically, a thin subdural zone of fibroblasts and thin-walled vessels was noted in an amorphous matrix. Two patients with prolonged symptoms had a more pronounced proliferative reaction. Conclusion The syndrome of low-pressure headaches and pacbyMeningeal gadolinium enhancement is being recognized with increasing frequency. The source of the CSF leak can be demonstrated in many patients. Meningeal abnormalities are likely attributable to decreased CSF volume and hydrostatic CSF pressure changes. The prognosis is typically good.
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Meningeal Biopsy in intracranial hypotension Meningeal enhancement on mri
Neurology, 1995Co-Authors: Bahram Mokri, Joseph E Parisi, Bernd W Scheithauer, David G Piepgras, Gary M MillerAbstract:Intracranial hypotension is a cause of diffuse enhancement of the pachymeninx with gadolinium, which often is associated with subdural fluid collections. We reviewed the results of Meningeal Biopsy in six patients with intracranial hypotension and diffuse pachyMeningeal enhancement to correlate the MRI findings with histopathologic observations and to explain the abnormalities seen on MRI. Grossly, the dura mater was unremarkable in all patients, as were the leptomeninges, except for one patient with prolonged (18 months) intracranial hypotension in whom the arachnoid was thickened and opaque. Microscopically, the dura mater was entirely normal on its epidural aspect; however, a fairly thin zone of fibroblasts and thin-walled small blood vessels in an amorphous matrix was noted on the subdural aspect. In the patient with longstanding symptoms, diffuse benign arachnoidal cell proliferation was also noted, probably a reaction triggered by longstanding changes in the subdural area, as noted in the five other patients. There was no evidence of inflammation, infection, or metastatic neoplasia. These findings suggest that in intracranial hypotension, the dural-Meningeal abnormalities probably represent reactive secondary phenomena, likely related to hydrostatic changes in the CSF, and not a primary Meningeal process.