Hypertrophic Pachymeningitis Research Articles

IgG4-related hypertrophic pachymeningitis with cerebral venous thrombosis.

IgG4-related hypertrophic pachymeningitis with cerebral venous thrombosis.

IgG4-related Hypertrophic Pachymeningitis with Serum ANCA Positivity.

IgG4-related Hypertrophic Pachymeningitis with Serum ANCA Positivity.

Immunoglobulin G4-Related Hypertrophic Pachymeningitis Presenting with Multiple Lower Cranial Nerve Palsies

Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ condition that infrequently exhibits neurological involvement. Here, we describe a case of IgG4-related hypertrophic pachymeningitis with multiple lower cranial nerve palsies. A 65-year-old man presented with a 5-month history of hoarseness and dysphagia. A neurologic examination revealed multiple lower cranial nerve palsies. Brain magnetic resonance imaging indicated diffuse dural involvement along the left tentorium and temporal fossa. Serum IgG and IgG4 levels were found to be elevated. Ethmoid polyp biopsy revealed chronic inflammation with moderate lymphoplasmacytic and some eosinophilic infiltration. Up to 60 IgG4-positive cells were observed per high-power field, and the measured IgG4/IgG ratio was approximately 10% to 20%. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography indicated abnormally high FDG uptake in the left posterior fossa, tentorium, and left temporal fossa, with additional involvement of the lung, iliac artery, and renal artery. Based on these findings, we diagnosed the patient with IgG4-related hypertrophic pachymeningitis and successfully treated the condition with steroids and rituximab. IgG4-RD should be differentiated from distinct conditions presenting with multiple cranial nerve palsies accompanied by pachymeningitis.

Two Cases of Perinuclear Anti-neutrophil Cytoplastic Antibodies Associated Hypertrophic Pachymeningitis Presented with Visual Loss

Pachymeningitis is an inflammatory process mainly involving the dura mater and is possibly associated with infectious, neoplastic, or autoimmune disease. However, in most cases, the etiology remains unknown. Clinical manifestations of pachymeningitis can vary, ranging from headache, cranial neuropathy, cerebellar dysfunction, to seizure. Here we report two cases of perinuclear anti-neutrophil cytoplasmic antibodies associated with pachymeningitis, in both the visual loss attributed to optic neuropathy was the primary sign.

Clinical Observation of Various Types of Idiopathic Hypertrophic Cranial Pachymeningitis

To assist doctors in making better treatment decisions and improve patient prognosis, it is important to determine which therapy modalities are suitable for various forms of idiopathic hypertrophic cranial pachymeningitis (IHCP). All cases were received from the hospital medical record system, and some follow-up information was gathered through telephone follow-up. A total of 26 patients, 14 men and 12 women, with ages ranging from 20 to 73 years and a mean of 47.42 years, were included in the research. Regular types were less likely to recur than irregular and nodular types, focal types were less likely to recur than diffuse types, and corticosteroid-refractory types were more likely to recur than corticosteroid-sensitive types. The extent and shape of the lesion and susceptibility to corticosteroids are potential factors that could influence recurrence. Futhermore, this paper also proposes the fibroblasts as a new therapeutic target which may improve the quality of prognostic survival of patients.

A Rare Case of Hypertrophic Pachymeningitis after Surgery for Chronic Subdural Hemorrhage: A Case Report

Hypertrophic pachymeningitis (HP) is a rare type of inflammatory fibrosis characterized by diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. Progressively worsening headache is the usual symptom, along with ataxia and various cranial nerve palsies. This article describes a rare case of HP after burr hole trephination for a chronic subdural hemorrhage (CSDH). A 64-year-old man was admitted to the emergency room with a headache that had started approximately 2 weeks prior. After the diagnosis of CSDH on imaging, burr hole trephination was performed. Six months later, the patient visited the hospital again with a headache. He was diagnosed with a recurrence of CSDH, and a craniotomy was performed. A solid subdural granuloma was suspected and a pathological examination revealed acute and chronic inflammation. Tissue biopsy and laboratory blood tests did not reveal any abnormalities. Three months later, he visited the hospital again with a severe headache. Magnetic resonance imaging showed no significant difference in the area of the previous operation; however, focal venous congestion and localized venous ischemic or inflammatory changes were observed in the tentorium. A craniotomy was performed for biopsy, and the dura mater was very thick. The pathology was diagnosed as HP. After taking steroids and immunosuppressive agents, the patient's symptoms improved. HP is a rare chronic inflammatory condition of the dura mater that causes neurological deficits. If the diagnosis is not definitively established based on an imaging examination, it should be made through a surgical biopsy. Steroid and immunosuppressive agents can improve the prognosis after diagnosis.

Prognostic Factors Affecting Hearing in Otitis Media With ANCA-Associated Vasculitis Patients: A Systematic Review and Meta-Analysis.

Objectives: To conduct a systematic review and meta-analysis of clinical studies describing the possible prognostic factors affecting hearing outcomes in Otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) patients. To provide guidance for clinical work, avoiding profound irreversible hearing loss affecting patients' lives. Methods: A literature search was performed in PubMed, MEDLINE, EMBASE, Cochrane, Scopus, and Web of Science to identify English articles published before December 1, 2022. After screening the articles, the Newcastle-Ottawa Scale (NOS) was used to assess the risk of bias of the extracted literature, and studies with high quality (score > 6) were included. Results: Four studies were included: 1 was a retrospective cohort study, and 3 were case-control studies. We performed a meta-analysis of 4 factors: facial palsy, hypertrophic pachymeningitis, ANCA-negative status, and the period from onset to diagnosis. The results showed that there was a significant association between facial palsy [odds ratio (OR) 1.51; 95% confidence interval (CI) 1.07-2.15; I2 = 0%; P = .02], hypertrophic pachymeningitis (OR 1.73; 95% CI 1.18-2.53; I2 = 24%; P = .005), ANCA negativity (OR 1.75; 95% CI 1.11-2.77; I2 = 33; P = .02), and poor hearing prognosis in OMAAV patients. However, the period from onset to diagnosis (SEM ± SD 2.54; 95% CI -1.56 to 6.64; I2 = 98%; P = .22) of OMAAV was not significantly associated with poor hearing outcomes. Conclusion: We found that OMAAV patients with facial palsy, hypertrophic pachymeningitis, and ANCA negativity have a significant association with poor hearing prognosis, which provides diagnosis and treatment guidance in protecting patients' hearing.

Hypertrophic Pachymeningitis as an Unusual Cause of Headache and Sphincter Dysfunction in Systemic Lupus Erythematosus.

Hypertrophic pachymeningitis is a rare condition of diverse aetiologies.A workup including search for infectious, autoimmune and neoplastic aetiologies should be performed.It is an extremely rare complication in systemic lupus erythematosus and might be the initial and sole clinical manifestation.

Quantitative evaluation of the thickened dura mater impacting clinical signs in immune-mediated hypertrophic pachymeningitis

ObjectiveThis study evaluated the volume of thickened dura mater lesions and their impact on clinical findings in immune-mediated hypertrophic pachymeningitis (HP). MethodsThe volume of contrast-enhanced dura mater on magnetic resonance imaging was evaluated using the imaging feature quantification system in 19 patients with immune-mediated HP, including 12 with antineutrophil cytoplasmic antibody-related, 4 with IgG4-related, and 3 with idiopathic HP, as well as 10 with multiple sclerosis (MS) as controls. The implications of HP volume on neurological manifestations and cerebrospinal fluid (CSF) laboratory markers were statistically analyzed in patients with immune-mediated HP. ResultsThe volumes of the contrast-enhanced dura mater in the convexity, cranial fossa, and tentorium cerebelli were significantly higher in patients with immune-mediated HP than in those with MS. Among patients with immune-mediated HP, those with cranial nerve (CN) VIII neuropathy had a significantly higher volume of the contrast-enhanced dura mater in the cranial fossa than those without CN VIII neuropathy. The volume of the contrast-enhanced dura mater in the tentorium cerebelli was positively correlated with CSF protein levels. ConclusionQuantification of the thickened dura mater is useful for elucidating the relationship with the clinical findings in immune-mediated HP. Thickened dura mater lesions in the cranial fossa may be implicated in the development of CN VIII neuropathy. The enlargement of HP lesions in the tentorium cerebelli can increase CSF protein levels.

Large intracranial supratentorial tuberculoma imitating as a malignant lesion: a case report

BackgroundCNS TB roughly correlates to 1–10% of all intracranial tumors. The spectrum of CNS-TB is wide and can present as tuberculous meningitis, tubercular abscess, tuberculoma, or hypertrophic pachymeningitis. Tuberculomas are usually located at the corticomedullary junction and periventricular region, as expected for hematogenous dissemination. They are mostly infratentorial in children and supratentorial in adults. They may present months to years after infection.ObservationsHere we have a 55-year-old female who presented with a history of headaches, sudden loss of consciousness, and seizures. Initial imaging showed a right parietal mass lesion appearing to be malignant on initial imaging which had a significant mass effect and midline shift. The patient was operated on for the same and histopathology showed chronic granulomatous lesion and tissue staining s/o tuberculosis.LessonsSo a diagnostic option for infective etiology should be kept even for an intracranial supratentorial mass lesion.

A selected case series of idiopathic hypertrophic pachymeningitis in a single center: Pathological characteristics and case-oriented review

Hypertrophic pachymeningitis (HP) is a relatively rare disease of the central nervous system characterized by local or diffuse fibrous thickening of the dura mater. At present, there is still insufficient research on the pathogenesis and treatment strategies of this disease. We reported a continuous case series of seven patients with idiopathic HP (IHP), and also details one case of immunoglobulin G4-related HP requiring surgical intervention. Early diagnosis and appropriate surgical intervention for IHP could prevent the progression of permanent neurological damage and spinal cord paraplegia.

Clinical case of hypertrophic convexital pachymeningitis

A clinical analysis of a patient with undifferentiated granulomatous systemic vasculitis with a predominant lung lesion and its complication in the form of chronic hypertrophic convexital pachymeningitis of fungal (candidal) etiology is presented. A differential diagnosis with granulomatosis with polyangiitis was carried out. Modern literature data on diagnostic criteria and methods of treatment of this rare pathology are given.

Hypertrophic Pachymeningitis Associated with Myeloperoxidase-anti-neutrophil Cytoplasmic Antibodies Induced by Propylthiouracil: A Case Report.

A 71-year-old man with hyperthyroidism complained of headache lasting two months. He had been using propylthiouracil (PTU) for 14 years. Treatment intensification did not improve the symptoms. Blood tests detected a positive myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA). Diffuse dural thickening was identified by magnetic resonance imaging. The patient was diagnosed with hypertrophic pachymeningitis (HP) due to ANCA-associated vasculitis (AAV). He received methylprednisolone pulse therapy followed by prednisolone and methotrexate, which improved his headache. PTU-induced AAV-related HP is a rare and indiscernible disease. Therefore, the possibility of the disease should be proactively considered when a PTU user experiences refractory headaches.

Les sous-classes d’IgG dans le LCR présentent-elles un réel intérêt ?

AbstractSerum total IgG measurement is a common lab act in medical biology. More specialized, serum IgG subclasses measurement plays a key role in IgG4-Re- lated Disease but is also useful for second-line diagnosis in humoral immune deficiencies. In the cerebrospinal fluid (CSF), an index is used as an indicator of the relative amount of CSF IgG compared to serum. Any increase in the index reflects IgG production in the central nervous system. Regarding IgG subclasses in the CSF, medical laboratories are now getting demands, actually to find out if there is any IgG4 subclass. These IgG4 in CSF could be associated with IgG4-related hypertrophic pachymeningitis but without appropriate studies corroborating this, their measurement should be done only when tissue biopsy cannot be performed. Quantitative measurement on serum is validated with nephelometry and turbidimetry assays. Each laboratory has to validate the assays on CSF before giving results. In this mini review, we present the indications of total IgG and IgG subclasses on serum as well as the benefit of IgG subclasses in CSF and the techniques available in a medical laboratory.

Hypertrophic pachymeningitis due to IgG4-related disease (RD-IgG4). A case report

IntroductionHypertrophic pachymeningitis (HP) is a clinico-radiological entity characterized by a thickening of the dura mater that may be focal or diffuse and manifested by a variety of neurological syndromes. Aetiologically, it is classified as infectious, neoplastic, autoimmune, and idiopathic. Many of these formerly idiopathic cases have been shown to fall into the spectrum of IgG4-related disease. ObjectiveTo describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. CaseA 25-year-old woman with neurological symptoms of 3 years’ evolution characterized initially by right hypoacusis, evolving with headache and diplopia. Magnetic resonance imaging (MRI) of the encephalon showed pachymeningeal thickening with involvement of vasculo-nervous structures in the tip of the cerebellum, cavernous sinus, ragged foramen, and optic chiasm. The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour.Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested. Biopsy reviewNon storiform fibrosis, predominantly lymphoplasmacytic infiltrate, histiocytes, and polymorphonuclear infiltrate in sectors, without granulomas or atypia. Staining for germs negative. Immunohistochemistry with 50–60 IgG4+/HPF cells and range of 15%–20%, CD68+ in histiocytes, CD1a−, S100−.The patient presented deterioration of visual acuity due to ophthalmic nerve involvement, so glucocorticoid treatment was started in pulses and rituximab with regression of symptoms and imaging improvement of the lesions. ConclusionHP is a clinical imaging syndrome with variable symptoms and aetiologies that poses a diagnostic challenge.In this case the initial diagnosis was inflammatory myofibroblastic tumour, which is a neoplasm of variable behaviour, locally aggressive, and can metastasize; it is one of the main differential diagnoses of IgG4-related disease because they share anatomopathological features, including storiform fibrosis.IgG4-RD is an immune-mediated condition that can have single or multiple involvement. Its diagnosis is complex when it presents with single organ involvement or in non-typical organs (CNS, meninges) in which data are scarce, as in the case of our patient with single organ involvement of the CNS.Although there are classification criteria to guide non-specialists in the diagnosis, the sum of the clinical picture, imaging, laboratory, pathological anatomy, and immunohistochemistry will always be evaluated together for a definitive diagnosis.

Retrospective histomorphologic study of meningeal masses in Benin City, Nigeria

Background: The rarity of many histologic entities and the common discordance between histologic appearance and biologic behaviour make the diagnosis of meningeal masses challenging. Except for a few common entities like meningioma and meningeal metastases, data on the prevalence of meningeal mass lesions is limited, and most exist as case reports and small case series. This study presents the pattern of meningeal masses in our environment, in the hope of adding to knowledge and awareness of the differential diagnoses of meningeal masses in our environment.
 Materials and methods: A retrospective study of meningeal masses histologically diagnosed at the the University of Benin Teaching Hospital, Benin city, Nigeria, based on clinical, demographic and histomorphologic data obtained over a 15-year period.
 Results: Total of 140 meningeal masses; 137 neoplastic and 3 non-neoplastic mass lesions. Ages ranged from 3-86 years. Mean age was 45.85 ± 18.16 years. There were 52 males and 88 females with an overall male-female ratio of 1:1.7; but non-meningothelial tumours were more common amongst males. Most common tumour was Meningioma (78.6%), then metastatic tumours (15%), non-meningothelial mesenchymal tumors (4.3%), and hypertrophic pachymeningitis (2.1%). Meningioma, hemangioblastoma and embryonal rhabdomyosarcoma were the most common tumours in children.
 Conclusion: Rare histologic entities arising in the meninges, can easily be overlooked in the differential diagnosis of meningeal mass lesions, while the common ones like meningioma and meningeal metastases, are at risk of becoming ‘waste basket’ or ‘safety net’ diagnoses for meningeal tumours. There is need for continuous evaluation and audit of histological diagnoses of meningeal tumours to avoid missing the rare entities that may occasionally be encountered.

Intracranial Pachymeningitis: A Case-Series Describing Clinical Features, Imaging, Serological and Cerebrospinal Fluid Markers, Biopsy Findings and Management (P9-5.018)

<h3>Objective:</h3> We present a case-series of patients with intracranial pachymeningitis and outline their management for successful outcomes. <h3>Background:</h3> Hypertrophic pachymeningitis is characterized by focal/diffuse thickening of intracranial and/or intraspinal dura. <h3>Design/Methods:</h3> <h3>Case-1:</h3> 48-year-old-woman with Crohn’s disease, relapsing polychondritis, presented with persistent left-sided headaches. MRI brain revealed left-sided contrast-enhancing pachymeningeal thickening; CSF showed lymphocytic pleocytosis. Glucocorticoids, adalimumab, and methotrexate resulted in clinical and neuroimaging improvement. <h3>Case-2:</h3> 78-year-old-woman presented with intractable headache, bilateral sensorineural-hearing-loss and vision-impairment. MRI brain showed nodular, multifocal dural-thickening at clivus, parasellar region, cavernous-sinuses extension encasing major vessels at skull-base. CSF showed lymphocytic pleocytosis, protein and serology demonstrated IgG4-subclasses, attributing etiology to IgG4-related-disease. Steroids, methotrexate, and later IV-rituximab resulted in equivocal response. Intrathecal rituximab, resulted in clinical and imaging stabilization, from better CNS penetration. <h3>Case-3:</h3> 60-year-old woman, with sarcoidosis presented with severe headaches. MRI demonstrated contrast-enhancing dural thickening in left posterior-temporo-occipital regions. Brain-biopsy showed non-necrotizing granulomas involving dura, parenchyma consistent with sarcoidosis. Steroids and infliximab, lead to clinical and radiological improvement. <h3>Case-4:</h3> 76-year-old man with prior metastatic melanoma, post-chemotherapy (nivolumab+ipilimumab), presented with headaches. MRI showed diffuse intracranial contrast-enhancing pachymeningeal thickening. Chest CT-scan showed circumferential thickening, contrast-enhancing thoracic aorta indicating systemic vasculitis. Serologically, ANA 1:320, +SSA/SSB, ESR/CRP both &gt;100 and ANCA was negative. Meningeal biopsy demonstrated granulomatosis with polyangiitis; etiology considered paraneoplastic. Steroids and methotrexate demonstrated clinical and radiological response. <h3>Case-5:</h3> 56-year-old woman presented with headaches and CN-VII palsy. MRI brain revealed hypertrophic pachymeningitis causing mass-effect on right lateral ventricle. ANCA serological positivity and biopsy demonstrated AFB-stain-negative caseating granulomas, consistent with ANCA-vasculitis. Rituximab led to complete resolution of both pachymeningeal and CN-VII enhancement. <h3>Results:</h3> Clinical resolution and radiological stability was achieved for all 5 patients. <h3>Conclusions:</h3> Intracranial pachymeningitis presents in setting of systemic autoimmune disorders and sarcoidosis. Brain imaging, CSF-analysis, serological immune-markers, and biopsy aid in diagnosis. Immunosuppressive therapies provide for clinical and radiologic stabilization. <b>Disclosure:</b> Dr. Varma-Doyle has nothing to disclose. Dr. Manzano has nothing to disclose. Dr. Venna has nothing to disclose.

A case report: Diagnosis and treatment of idiopathic hypertrophic pachymeningitis

AbstractA 57‐year‐old man who suffered from a headache for 1 year, accompanied by blurred vision for 7 months and numbness in his left face for 1 week was admitted to the Affiliated Hospital of Zunyi Medical University on May 7, 2022. One year ago, the patient had no obvious precipitating factor of paroxysmal stabbing pain in the whole skull with dizziness, which could be relieved by oneself after lasting for 1–2 min each time, with about 20 episodes per day. The cranial magnetic resonance imaging revealed changes in bilateral frontal lobe ischemic foci, bilateral frontal, ethmoid, sphenoid and maxillary sinusitis, and retinal macular degeneration. After hormone shock treatment, the condition improved. He suffered from headaches again with blurred vision in the right eye 7 months ago and was initially diagnosed with multiple sclerosis. He then was discharged after improvement due to hormone shock therapy. Oral hormone therapy was continued outside the hospital, but he stopped it due to drug side effects (details remained unclear). After cutting off, he developed a headache and visited our hospital once more, the relevant tests were performed and the patient was diagnosed with idiopathic hypertrophic pachymeningitis (IHP). The symptoms were slightly abated after hormone therapy. We hope that through this case report, we can deepen the clinicians' understanding of IHP, and improve the diagnosis rate of the disease through relevant examinations in future clinical work, so that patients can receive timely treatment and the mental pressure and economic burden caused by the disease on patients are reduced.

Long-term response to immunotherapy in patients with hypertrophic pachymeningitis.

In this study, we aimed to clarify the relationship between initial treatment response, prednisolone (PSL) dosage, clinical type, and recurrence in patients with hypertrophic pachymeningitis (HP). The study cohort comprised eight patients with HP who had been admitted to our hospital from April 2015 to June 2020. Diagnostic criteria for HP included neurological abnormalities and dural thickening on magnetic resonance gadolinium-enhanced T1-weighted images. Relevant characteristics of the eight study patients are as follows. There were two men and six women. The average age at onset was 58.3 (range: 29-79) years. Three of them had myeloperoxidase-antineutrophil cytoplasmic antibody-related vasculitis, one immunoglobulin G4-related disease, and one ulcerative colitis. The remaining three patients had idiopathic HP. The average maximum dosage of PSL was 0.79mg/kg/day, and the average daily maintenance dosage 0.18mg/kg/day. Three patients needed additional immunosuppressive drugs. Both idiopathic and secondary HP initially responded well to PSL, with improvement in activities of daily living. Six patients had some sequelae related to cranial nerve involvement. No relapses occurred while the patients were taking moderate doses of PSL; however, all patients with idiopathic HP had recurrences when their PSL dosage was reduced. Patients with idiopathic HP and HP associated with immune disorders respond to steroids and immunosuppressive drugs and recover well. However, there is a high rate of relapse after reduction of PSL dosage, mainly in those with idiopathic HP.

The Spectrum of Intracranial Hypertrophic Pachymeningitis at an Eastern Indian Tertiary Care Center.

Hypertrophic pachymeningitis (HP) is a treatable, rare inflammatory disease, either primary or secondary to systemic causes. To characterize the etiology, clinical manifestations, and treatment outcomes of HP patients and determine the factors influencing the radiological resolution of the pachymeningeal enhancement and recurrence of symptoms within the follow-up period. We collected data for this prospective observational study between March 1, 2021 and May 31, 2022, at the Bangur Institute of Neurosciences, Kolkata, and the patients were followed for a 6-month period. Demographic, clinical, laboratory, and treatment-related data were collected. A univariate logistic regression model was used for comparison between patients with and without radiological resolution of pachymeningitis and between patients with and without symptom recurrence. Among 44 patients, the male: female ratio was 1.2:1. The median age at disease onset was 35.5 (28.5-49.5) years. The etiologies were idiopathic (56.8%), tuberculosis (22.8%), immunoglobulin G subtype 4 (IgG4) disease (9.2%), other infections (6.8%), and neoplastic (4.4%). Headache was the most common presentation (95.4%), followed by cranial neuropathies (68.2%). Optic and oculomotor neuropathies were the most common. In terms of radiological features, 27.27, 29.54, and 43.18% of patients had diffuse, focal regular, and focal irregular enhancement, respectively. Temporal (50%), followed by cavernous sinus (38.63%) enhancement, was the most common. Recurrence occurred in 36 and 50% of idiopathic and IgG4-related HP cases, respectively. Mycophenolate mofetil was added to their steroid regimen with no further recurrences. The cohort had a marked absence of (antineutrophil cytoplasmic antibodies) ANCA-associated HP. The severity of clinical manifestations or distribution of pachymeningitis did not differ significantly among the etiological groups. The presence of idiopathic etiology and focal regular enhancement had a significantly higher chance of radiological resolution. The response to therapy was satisfactory. Recurrence was significantly related to shorter steroid courses (<median duration of 5.2 months).