Immunoglobulin G4-related spinal pachymeningitis:A case report

BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a complex immune-mediated condition that causes fibrotic inflammation in several organs.A significant clinical feature of IgG4-RD is hypertrophic pachymeningitis,which manifests as inflammation of the dura mater in intracranial or spinal regions.Although IgG4-RD can affect multiple areas,the spine is a relatively rare site compared to the more frequent involvement of intracranial structures.CASE SUMMARY A 70-year-old male presented to our hospital with a two-day history of fever,altered mental status,and generalized weakness.The initial brain magnetic resonance imaging(MRI)revealed multiple small infarcts across various cerebral regions.On the second day after admission,a physical examination revealed motor weakness in both lower extremities and diminished sensation in the right lower extremity.Electromyographic evaluation revealed findings consistent with acute motor sensory neuropathy.Despite initial management with intravenous immunoglobulin for presumed Guillain-Barrésyndrome,the patient exhibited progressive worsening of motor deficits.On the 45th day of hospitalization,an enhanced MRI of the entire spine,focusing specifically on the thoracic 9 to lumbar 1 vertebral level,raised the suspicion of IgG4-related spinal pachymeningitis.Subsequently,the patient was administered oral prednisolone and participated in a comprehensive rehabilitation program that included gait training and lower extremity strengthening exercises.CONCLUSION IgG4-related spinal pachymeningitis,diagnosed on MRI,was treated with corticosteroids and a structured rehabilitation regimen,leading to significant improvement.

Clivus-involved immunoglobulin G4 related hypertrophic pachymeningitis mimicking meningioma:A case report

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.

Idiopathic hypertrophic pachymeningitis with intracranial hypotension and distension of anterior internal vertebral venous plexus are associated with positional headaches but not sufficient in the diagnosis

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.

The protean manifestations of central nervous system IgG4-related hypertrophic pachymeningitis:a report of two cases

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 were not detected on follow-up imaging.Conclusions:A high index of suspicion for this condition is suggested when a male patient with a history of autoimmune disease and compatible radiological findings,experiences subacute headache that is disproportionate to the degree of dural involvement.Neurosurgeons should consider early meningeal biopsy to establish a definitive histological diagnosis in order for early effective immunosuppressive treatment to be initiated and to avoid unnecessary morbidity.

Pachymeningeal en Plaque Tuberculoma in Children: A Case Report

Pachymeningeal en plaque tuberculoma is a rare manifestation of tuberculosis and has a poor prognosis if not diagnosed early and treated adequately. The aim of the present paper is to collect the reported clinical and radiological signs in the literature and compare it with a personal observation to evaluate the possibility of a presumptive diagnosis. A 4-year-old boy presented at the pediatric emergency department with 7 days history of severe headache, vomiting blurred vision and had one partial seizure. His neurologic examination was notable for decreased visual acuity with bilateral papilledema and intact extra-ocular muscles. His neck was rigid. Neuroimaging revealed a multifocal dural-based mass on the fronto-parietal convexity with dense heterogeneous contrast enhancement and hydrocephalus. He has undergone a Ventriculoperitoneal shunt and surgical excision of the mass the next day. Outcome was favorable with antituberculosis therapy and corticosteroids. The clinical presentation and outcome were similar to the reported cases in the literature.

Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis

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.