Dural enhancement detected by magnetic resonance imaging is a common finding in patients with cerebral venous sinus thrombosis （CVST） and is usually interpreted as a change secondary to CVST. We report two cases of ...Dural enhancement detected by magnetic resonance imaging is a common finding in patients with cerebral venous sinus thrombosis （CVST） and is usually interpreted as a change secondary to CVST. We report two cases of CVST with intense and diffuse dural enhancement that resulted from pachymeningitis in one patient and spontaneous intracranial hypotension in another. Pachymeningitis and spontaneous intracranial hypotension were also determined to be the underlying causes of CVST. The clinical data of these two patients are described. In patients with CVST, dural enhancement is not always a secondary change to CVST. It can be a manifestation of the underlying causes of CVST. When diffuse and intense dural enhancement is revealed, sufficient ancillary tests are warranted to rule out other potential patholooical chanoes of the dura mater those can result in CVST.展开更多
BACKGROUND Immunoglobulin G4 related disease(Ig G4-RD)is a fibroinflammatory disease with markedly elevated serum Ig G4 levels and fibrous tissue proliferation,accompanied by numerous plasma cells.Ig G4 related hypert...BACKGROUND Immunoglobulin G4 related disease(Ig G4-RD)is a fibroinflammatory disease with markedly elevated serum Ig G4 levels and fibrous tissue proliferation,accompanied by numerous plasma cells.Ig G4 related hypertrophic pachymeningitis(Ig G4-RHP)is relatively rare and indistinguishable from other phymatoid diseases before the operation.The risk of long-term immunosuppression needs to be balanced with disease activity.CASE SUMMARY A 40-year-old man presented with headache and bilateral abducent paralysis.He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same.Before the operation and steroid therapy,the patient was suspected of having tubercular meningitis at a local hospital.A clivus lesion was found via brain magnetic resonance imaging(MRI)at this presentation.He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery.Transnasal endoscopic resection was performed to treat deterioration of nerve function.Postoperative pathologic examination suggested Ig G4-RD.Moreover,the serum Ig G4 was elevated at 1.90 g/L(reference range:0.035-1.500 g/L).After steroid therapy for 2 mo,the lesion size diminished on MRI,and the function of bilateral abducent nerves recovered.CONCLUSION Ig G4-RHP is relatively rare and indistinguishable before the operation.Elevated serum Ig G4 levels and imaging examination help in the diagnosis of Ig G4-RHP.Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit.展开更多
Background:IgG4-related hypertrophic pachymeningitis is a relative newly recognized and rare manifestation of IgG4-related disease,an immune-mediated fibroinflammatory tumefactive disorder.Fewer than 80 patients have ...Background:IgG4-related hypertrophic pachymeningitis is a relative newly recognized and rare manifestation of IgG4-related disease,an immune-mediated fibroinflammatory tumefactive disorder.Fewer than 80 patients have been reported in the literature,and it can mimic common neurosurgical conditions.We describe the clinical presentation of two patients that were initially considered to have a subdural collection,tuberculous meningitis,and a cervical spinal meningioma,but were eventually diagnosed with this disease.Case presentation:Two ethnic Chinese men,86 and 62 years old,experienced a 4-week history of headache.Both patients had a history of autoimmune disease,namely glomerulonephritis and Grave’s disease,respectively.Magnetic resonance brain imaging revealed diffuse dural thickening with the latter patient exhibiting homogeneous and intense gadolinium-contrast enhancement.Since the 86-year-old patient also had progressive bilateral visual loss,giant cell arteritis was suspected and a 2-week course of glucocorticoid therapy was prescribed,but his symptoms failed to improve.The 62-year-old patient also had accompanying low-grade fever and was treated empirically as having tuberculous meningitis although there were no confirmatory microbiological findings.This patient further developed right hemiparesis,and additional imaging revealed a C4/5 intradural-extramedullary contrast-enhancing lesion resembling a meningioma causing cord compression.Both patients underwent neurosurgical intervention with the former undergoing a dural biopsy and the latter having the cervical lesion resected.The final diagnosis was IgG4-related hypertrophic pachymeningitis with the hallmark histological features of lymphoplasmacytic infiltration of IgG4+plasma cells,storiform fibrosis,and obliterative phlebitis.In addition,their serum IgG4 levels were elevated(i.e.,>135 mg/dL).Both patients received at least 6 months of glucocorticoid therapy while the latter also had azathioprine.Their symptoms improved significantly and recurrent lesions展开更多
Idiopathic hypertrophic pachymeningitis (IHP) is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. The main clinical features of IHP at presentation include headache, cranial ...Idiopathic hypertrophic pachymeningitis (IHP) is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. The main clinical features of IHP at presentation include headache, cranial nerve involvement, ataxia, transient ischemic attacks (TIA) and seizures. Intracranial hypotension (ICH) and distension of anterior internal vertebral venous plexus (IVVP) have not previously been reported as the main clinical manifestations. We report a unique case with these characteristics as the main clinical findings, and investigate the internal relationship between these characteristics. This study highlights the heterogeneity of IHP, in terms of clinical, imaging, and pathological features. We recommend that dural biopsies should be considered in patients with clinically typical orthostatic headache and (or) diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI) for the diagnosis of IHP.展开更多
文摘Dural enhancement detected by magnetic resonance imaging is a common finding in patients with cerebral venous sinus thrombosis （CVST） and is usually interpreted as a change secondary to CVST. We report two cases of CVST with intense and diffuse dural enhancement that resulted from pachymeningitis in one patient and spontaneous intracranial hypotension in another. Pachymeningitis and spontaneous intracranial hypotension were also determined to be the underlying causes of CVST. The clinical data of these two patients are described. In patients with CVST, dural enhancement is not always a secondary change to CVST. It can be a manifestation of the underlying causes of CVST. When diffuse and intense dural enhancement is revealed, sufficient ancillary tests are warranted to rule out other potential patholooical chanoes of the dura mater those can result in CVST.
基金Supported by 1·3·5 Project for Disciplines of Excellence–Clinical Research Incubation Project,West China Hospital,Sichuan University,No.2019HXFH018。
文摘BACKGROUND Immunoglobulin G4 related disease(Ig G4-RD)is a fibroinflammatory disease with markedly elevated serum Ig G4 levels and fibrous tissue proliferation,accompanied by numerous plasma cells.Ig G4 related hypertrophic pachymeningitis(Ig G4-RHP)is relatively rare and indistinguishable from other phymatoid diseases before the operation.The risk of long-term immunosuppression needs to be balanced with disease activity.CASE SUMMARY A 40-year-old man presented with headache and bilateral abducent paralysis.He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same.Before the operation and steroid therapy,the patient was suspected of having tubercular meningitis at a local hospital.A clivus lesion was found via brain magnetic resonance imaging(MRI)at this presentation.He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery.Transnasal endoscopic resection was performed to treat deterioration of nerve function.Postoperative pathologic examination suggested Ig G4-RD.Moreover,the serum Ig G4 was elevated at 1.90 g/L(reference range:0.035-1.500 g/L).After steroid therapy for 2 mo,the lesion size diminished on MRI,and the function of bilateral abducent nerves recovered.CONCLUSION Ig G4-RHP is relatively rare and indistinguishable before the operation.Elevated serum Ig G4 levels and imaging examination help in the diagnosis of Ig G4-RHP.Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit.
文摘Background:IgG4-related hypertrophic pachymeningitis is a relative newly recognized and rare manifestation of IgG4-related disease,an immune-mediated fibroinflammatory tumefactive disorder.Fewer than 80 patients have been reported in the literature,and it can mimic common neurosurgical conditions.We describe the clinical presentation of two patients that were initially considered to have a subdural collection,tuberculous meningitis,and a cervical spinal meningioma,but were eventually diagnosed with this disease.Case presentation:Two ethnic Chinese men,86 and 62 years old,experienced a 4-week history of headache.Both patients had a history of autoimmune disease,namely glomerulonephritis and Grave’s disease,respectively.Magnetic resonance brain imaging revealed diffuse dural thickening with the latter patient exhibiting homogeneous and intense gadolinium-contrast enhancement.Since the 86-year-old patient also had progressive bilateral visual loss,giant cell arteritis was suspected and a 2-week course of glucocorticoid therapy was prescribed,but his symptoms failed to improve.The 62-year-old patient also had accompanying low-grade fever and was treated empirically as having tuberculous meningitis although there were no confirmatory microbiological findings.This patient further developed right hemiparesis,and additional imaging revealed a C4/5 intradural-extramedullary contrast-enhancing lesion resembling a meningioma causing cord compression.Both patients underwent neurosurgical intervention with the former undergoing a dural biopsy and the latter having the cervical lesion resected.The final diagnosis was IgG4-related hypertrophic pachymeningitis with the hallmark histological features of lymphoplasmacytic infiltration of IgG4+plasma cells,storiform fibrosis,and obliterative phlebitis.In addition,their serum IgG4 levels were elevated(i.e.,>135 mg/dL).Both patients received at least 6 months of glucocorticoid therapy while the latter also had azathioprine.Their symptoms improved significantly and recurrent lesions
文摘Idiopathic hypertrophic pachymeningitis (IHP) is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. The main clinical features of IHP at presentation include headache, cranial nerve involvement, ataxia, transient ischemic attacks (TIA) and seizures. Intracranial hypotension (ICH) and distension of anterior internal vertebral venous plexus (IVVP) have not previously been reported as the main clinical manifestations. We report a unique case with these characteristics as the main clinical findings, and investigate the internal relationship between these characteristics. This study highlights the heterogeneity of IHP, in terms of clinical, imaging, and pathological features. We recommend that dural biopsies should be considered in patients with clinically typical orthostatic headache and (or) diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI) for the diagnosis of IHP.
