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Hypertrophic pachymeningitis (HP) is a rare disorder marked by thickening of the dura mater due to diverse etiologies. MPO-ANCA-positive HP represents a variant of AAV confined to the central nervous system, distinguished by the presence of serum MPO antibodies. Distinguishing HP triggered by MPO-ANCA from other causes can be challenging.In this study, we present two cases of MPO-ANCA-positive HP initially misdiagnosed as intracranial infections. Case 1 underwent surgery for chronic suppurative otitis media, with histopathological findings revealing inflammatory changes without definitive suppuration. He was presumed to have a secondary intracranial infection resulting from the surgery. However, his condition deteriorated despite two weeks of antibiotic and antiviral treatment. Case 2 presented with headache and was initially suspected of having intracranial Brucellosis given his serum Brucella positivity. Despite treatment for brucellosis, his symptoms persisted, and he developed visual and hearing impairments. Both patients were ultimately diagnosed with MPO-ANCA-positive HP, exhibiting serum MPO antibody positivity. Their symptoms showed improvement with glucocorticoid and immunosuppressive therapy.Based on these observations, we propose that MPO-ANCA-positive HP may initially present as intracranial infection. For HP patients presenting with headache, mastoiditis, otitis media, and visual loss, it is imperative to conduct ANCA antibody-related tests to enhance diagnostic precision.

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of vasculitis sharing a common pathophysiology, which affects small and medium blood vessels. There are three categories of AAV: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). As a systemic disease, AAV can affect basically every organ. The goal of this publication is to sum up and underline the problem of the aural manifestation of AAV; it details the definition of Otitis Media with Antineutrophil Cytoplasmic Antibody Associated Vasculitis (OMAAV) and allows for a better understanding of the specific tasks of medical professionals taking part in the diagnostic and therapeutic process. Among others, this publication is directed to otolaryngologists who may encounter patients with AAV and often are the first specialists who see patients with early symptoms of AAV. This publication presents brief characteristics of AAV, descriptions of aural manifestations and symptoms, differential diagnosis, and both pharmacological and surgical treatment options, based on current recommendations and information found in the literature and clinical databases.

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PURPOSE: To describe four cases of non-infectious hypertrophic pachymeningitis (HP)-associated with orbital inflammatory disease (OID). This study summarises the clinico-radiological features, outcomes, and management of HP-associated OID. METHODS: Retrospective case-series of patients with radiological evidence of HP and OID. Comprehensive literature review of all published English-language non-infectious causes of HP-associated OID. Reference lists were screened for inclusion of relevant articles. RESULTS: Thirty-seven cases of HP-associated OID (Mean age: 49.2 ± 17.4 years old; Male: 15) were identified, including four cases from our institution. Aetiologies included ANCA-associated vasculitis (12/37), non-specific/idiopathic (11/37), IgG4/multifocal fibrosclerosis (11/37), neurosarcoidosis (1/37), inflammatory myofibroblastic tumour (1/37), and giant cell arteritis (1/37). Orbital pain, headache, visual deterioration, and cranial nerve palsies were common clinical presentations. Both "focal" and "diffuse" HP were observed, with the most common sign of orbital involvement being an inflammatory orbital mass, typically with orbital apex involvement. Orbital myositis and dacryoadenitis were less common. The cavernous sinus was the most common site of extra-orbital inflammation. There was no single differentiating specific radiological feature between non-specific and specific forms of HP-associated OID. CONCLUSION: The clinico-radiological manifestations of HP-associated OID differ from those described in isolated HP or OID. There is no single specific radiological marker differentiating non-specific/idiopathic disease from secondary causes; however, the co-existence of HP in OID should prompt suspicion of an underlying cause. The disease may be refractory or resistant to initial treatment, although guidelines surrounding its management and the long-term prognosis remain to be determined.

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Immunoglobulin G4 (IgG4) related hypertrophic pachymeningitis of the spinal cord is a rare condition, characterized by infiltration of the spinal meninges with IgG4-producing plasma cells and subsequent hypertrophic fibrosis. Here, we report on a 65-year-old woman with IgG4 associated hypertrophic spinal pachymeningitis, in whom cerebrospinal fluid (CSF) analysis was a decisive diagnostic tool. Not only could we demonstrate an intrathecal IgG4 production, but also IgG4 positive plasma cells in CSF. Following decompressive surgery, diagnosis of IgG4 associated hypertrophic pachymeningitis was confirmed histologically. Surgery and immunosuppressive therapy with rituximab were associated with clinical improvement. This case highlights CSF analyses as diagnostic tool for detection of IgG4 related hypertrophic pachymeningitis.

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IgG4-related disease is an immune-mediated fibroinflammatory condition. Isolated manifestation in the spine as hypertrophic pachymeningitis is very rare and the mass-like lesion on MRI often mimic tumour or infection. Patients would present with symptoms that result from mass effect or neurovascular compression. Studies showed that serum and CSF IgG4 levels are rarely informative, and therefore, tissue biopsy is crucial for accurate diagnosis. Apart from supporting the diagnosis, MRI is helpful in delineating the extent of disease and follow-up after treatment. A 18F-FDG PET/CT scan is useful in detecting systemic manifestations of IgG4-related disease. Although IgG4-related disease generally responds well to corticosteroid at inflammatory state, relapse is not uncommon. Current treatment strategies for IgG4-related hypertrophic pachymeningitis are high dose corticosteroid therapy and early decompressive surgery to avoid chronic neurological complications. We described a case of a 27-year-old gentleman complaining of lower limb weakness and numbness. MRI showed a mass-like epidural lesion at the thoracic spine causing cord compression. Open biopsy of the epidural mass demonstrated histopathological characteristics of IgG4-related disease. Patient responded well to early surgical decompression of the spinal cord and corticosteroid as evidenced by symptom improvement and resolving mass on subsequent MRI study. However, a follow-up MRI revealed disease recurrence years later.

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Introduction and importance Hypertrophic pachymeningitis (HP) is an uncommon disorder with varied etiological origins and heterogeneous clinical presentation. Establishing the etiological diagnosis poses a challenge, but prompt identification provides a treatment window, potentially leading to a reversal of symptoms. MRI is the reference examination, allowing not only the early diagnosis of pachymeningitis but also the assessment of its extent and importance, detection of possible complications, and suggestion of etiology. Case presentation We conducted a retrospective study involving 24 patients recruited over 5 years for who brain imaging had revealed the presence of pachymeningitis. The average age of the patients was 40 years, with a male-to-female ratio of 0.6. Clinical discussion Headache was present in 54.17% of patients. All the patients underwent MRI examinations utilizing different sequences, with subsequent Gadolinium injection showing localized and asymmetrical meningeal thickening in 13 cases, and diffuse in the rest. The cerebrospinal fluid study unveiled an inflammatory fluid characterized by a lymphocytic predominance and hyperproteinorrhea, noted in 50% of the patients. The histopathological analysis of a stereotactic biopsy conducted on an individual patient revealed non-diagnostic results. The etiological investigation was dominated by tuberculosis, which was detected in 33.3% of cases. Idiopathic origin was identified in 16.7% of patients. Conclusion Meningeal thickening is rare, and the multitude of potential causes makes the etiological investigation challenging unless they fall within the scope of secondary meningeal disorders; otherwise, a dural biopsy becomes necessary, and the prompt initiation of treatment, along with determining the etiology influences the prognosis.

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Background: Hypertrophic pachymeningitis (HP) is a disease with diverse aetiologies, including the autoimmune one, either associated with antineutrophil cytoplasmic antibodies or immunoglobulin G4. Case description: A 65-year-old woman with a history of systemic arterial hypertension, presented with intense progressive headaches. HP and hemispheric vasogenic oedema were observed by nuclear magnetic resonance (NMR) study. During the six months before the headache, she had developed progressive hearing loss which she attributed to age. A biopsy of dura mater showed necrotising vasculitis with peripheral inflammatory infiltrate, made up of accumulations of epithelioid cells and multinucleated giant cells, and abundant eosinophils. A final diagnosis of HP with eosinophilic granulomatosis with polyangiitis (EGPA) was made. Discussion: The patient had eosinophilic granulomatosis with polyangiitis (EGPA) histology, ANCA-negative serology and HP. This case is important because it shows that EGPA seems to have a spectrum of clinical diseases, including HP with negative serology, and bilateral sensorineural hearing loss. Conclusion: We are facing a wide spectrum of EGPA, breaking the paradigm of only systemic involvement. LEARNING POINTS: Hypertrophic pachymeningitis (HP) has several aetiologies; if the systemic investigation is not contributory to a diagnosis, a meningeal biopsy is necessary.This is the first case report of HP, associated with eosinophilic granulomatosis with polyangiitis (EGPA), and ANCA-negative serology.EGPA is probably a spectrum of diseases with predominant systemic involvement, but there may be cases where there is histological evidence, without the systemic context or positive serology.

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Hypertrophic pachymeningitis (HP) is a rare inflammatory disease characterized by thickening of the dura mater. HP develops with several inflammatory diseases. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4 related disease are reported as 2 major causes. With hematologic diseases, only 3 cases have been reported. We report the case of myelodysplastic syndrome (MDS) developing HP. Our case provides a thought-provoking hypothesis regarding the potential relationship between MDS and HP.

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Rosai-Dorfman disease (RDD) is a rare form of non-Langerhans cell histiocytosis. Although 20% of patients with RDD have spontaneous remission, some cases with central nervous system (CNS) involvement require surgery or systemic treatment. We encountered a case of RDD in which hypertrophic pachymeningitis was diffuse, eliminating the need for surgical intervention. A 72-year-old Japanese man was diagnosed with RDD based on pathological lymph node findings. Repeated intravenous methylprednisolone (IVMP) administration resolved and stabilized the hypertrophic pachymeningitis without any sequelae. If surgery or anticancer medications are contraindicated, repeated IVMP may be a good therapeutic option for CNS-associated RDD.

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Hypertrophic pachymeningitis (HP) is a rare inflammatory disease of the central nervous system. It typically manifests in the cranium; cases involving the spinal cord are rare (8.6%). This report includes two cases of spinal HP encountered among 666 spinal operative cases. The purpose of this study is to present the initial imaging findings, final diagnosis, and course of treatment in these two cases of spinal HP and to present the possible risk of misdiagnosis with a literature review. In case 1, a 69-year-old female presented with back pain. The initial radiological diagnosis with magnetic resonance imaging (MRI) was a meningioma. However, her blood test showed a mild elevation of C-reactive protein level (3.16 mg/dL), with positive IgG4 and myeloperoxidase anti-neutrophil cytoplasmic antibody results, suggesting an autoimmune disease. We performed a biopsy of the thickened dura and an expansive duraplasty. Serological and pathological diagnosis suggested IgG4-related HP. In case 2, a 67-year-old male presented with bilateral thigh pain. MRI revealed a mass resembling a disc hernia at the L2/3 intervertebral level. The mass was surgically removed. Pathological examination and cerebrospinal fluid analysis confirmed the diagnosis of HP associated with IgG4-related disease. In both cases, immunosuppressive therapy was administered, and follow-up MRI scans revealed the disappearance of the mass. The study concludes that a spinal HP can potentially be misdiagnosed when its images resemble those of tumors or disc hernias owing to its rarity.

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OBJECTIVES: We aimed to characterize the clinical and radiological features, and outcomes, of a large cohort of hypertrophic pachymeningitis (HP) patients from a single center. METHODS: We conducted a retrospective study at a tertiary referral center, encompassing patients diagnosed with HP between 2003 and 2022. The diagnosis of HP relied on the identification of thickening of the dura mater via magnetic resonance imaging (MRI) of the brain or spine. RESULTS: We included 74 patients with a mean age of 43.6 ± 14.2 years, of whom 37 (50%) were male. Among them, 32 (43.2%) had an immune-mediated origin, including 21 with granulomatosis with polyangiitis (GPA) (predominantly PR3-ANCA positive), four with systemic lupus erythematosus, three with IgG4-related disease, three with idiopathic HP, and one with rheumatoid arthritis. Non-immune-mediated HP accounted for 45 cases (56.8%). Within this category, 21 (28.4%) were infectious cases, with 14 being Mycobacterium tuberculosis infection (TB-HP), and 21 (28.4%) were malignancy-associated HP. Clinical and MRI characteristics exhibited variations among the four etiological groups. Hypoglycorrhachia was primarily observed in infectious and malignancy-associated HP. Immune-mediated HP was associated with a peripheral pattern of contrast enhancement and the Eiffel-by-night sign. MRI features strongly indicative of TB-HP included leptomeningeal involvement, brain parenchymal lesions, and arterial stroke. MPO-ANCA GPA was associated with a higher prevalence of spinal HP. CONCLUSIONS: Within our cohort, GPA and Mycobacterium tuberculosis emerged as the predominant causes of HP. We identified significant disparities in clinical and radiological features among different etiologies, which could have implications for diagnosis.

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Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. We present the case of a 28-year-old woman who presented with recurrent blurred vision in her right eye for 3 months. She developed blindness and atrophy in her left eye a decade prior to presentation. She subsequently developed headache, fever, and impaired mental status. Cranial magnetic resonance imaging indicated hypertrophic pachymeningitis (HP), and 18F-fluoro-2-deoxy-2-d-glucose (FDG) positron emission tomography/computed tomography revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibody levels. Incisional biopsy of the atrophic eyeball revealed RDD with marked polyclonal plasmacytosis. The patient was diagnosed with RDD accompanied by multisystem involvement, including Sjögren's syndrome (SS), panuveitis, and HP. Treatment with methylprednisolone for several weeks resulted in significant improvement. This is the first reported case of RDD presenting with SS in combination with panuveitis and HP. Although RDD is rarely diagnosed in young patients, interdisciplinary collaboration is essential to prevent a delayed diagnosis.

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OBJECTIVES: To describe a rare complication of cholesteatoma. METHODS: Case report with literature review. RESULTS: We report a case of a 37-year-old male who presented for evaluation of otorrhea, headache, and progressive left sensorineural hearing loss. Clinical and radiologic evaluation demonstrated a large recurrent attic cholesteatoma with erosion into the lateral and superior semicircular canals, and diffuse enhancement of the internal auditory canal and cerebellopontine angle suggestive of hypertrophic pachymeningitis secondary to cholesteatoma. After treatment with a course of antibiotics and canal wall down mastoidectomy surgery for cholesteatoma exteriorization, he experienced improvement of his symptoms and resolution of hypertrophic pachymeningitis. CONCLUSION: Hypertrophic pachymeningitis is a rarely described complication of cholesteatoma. In the context of cholesteatoma, treatment with antibiotics and surgical removal or exteriorization of cholesteatoma are effective treatments for HP.

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The article presents a case of idiopathic hypertrophic pachymeningitis of a 61-year-old male patient with severe cephalgia and progressive neuropathy of the oculomotor nerves. The diagnosis was confirmed by MRI with gadolinium, which revealed thickening of the dura mater with accumulation of paramagnetic in the convexital parts of the frontal and temporal regions, as well as on the base of the skull and tentorium. During the use of pulse therapy with glucocorticosteroids (GCS) the symptoms regressed, but when the therapy was stopped, there was a relapse of ptosis and oculomotor abnormalities on the other side followed by a slower effect of GCS therapy. The article also presents a brief review of current knowledge about this pathology.

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Cogan's syndrome (CS) is a rare chronic inflammatory disease, characterized by interstitial keratitis and vestibular auditory dysfunction. Hypertrophic pachymeningitis (HP) is a rare chronic aseptic inflammatory disease of the central nervous system. This article reports a patient with CS coexisting with HP. The patient was a 66-year-old male with fever, headache, red eyes, hearing loss, and significantly elevated inflammatory markers. Cerebrospinal fluid examination, blood culture, and tests for autoantibodies such as antinuclear antibodies were negative. Pure tone audiology (PTA) indicated bilateral sensorineural deafness. Both Positron emission tomography-computed tomography (PET/CT) and vascular color Doppler ultrasound suggest the presence of vasculitis. Considering Cogan's syndrome, the patient received 40 mg of methylprednisolone intravenously once daily. The brain's magnetic resonance imaging (MRI) revealed slightly thickened and enhanced dura mater, suggesting HP. The dose of methylprednisolone was increased to 40 mg intravenously every 8 hours, leading to the patient's improved symptoms and decreased inflammatory markers. Both CS and HP are rare chronic inflammatory diseases, and their coexistence is even rarer, with only two reported cases in literature up to date. The coexistence of CS and HP should be considered when the CS patients with headaches do not respond well to treatment.

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Hypertrophic pachymeningitis (HP) is a rare condition characterized by inflammation and thickening of the dura mater. It can be idiopathic or secondary to various causes, including infections, tumors, or systemic inflammatory diseases. Diagnosis is challenging due to its rarity and the overlap of symptoms with other conditions. We present the case of a 42-year-old Hispanic woman with diabetes mellitus type 2 and end-stage kidney disease who presented with chest pain, dry cough, mild dyspnea, and chronic occipital headaches. Physical examination revealed cranial VI nerve palsy. Imaging showed pulmonary cavitary lesions and mediastinal lymphadenopathy. Elevated inflammatory markers and positive autoimmune tests, including rheumatoid factor and antineutrophil cytoplasmic antibody (ANCA), led to further investigation. Brain imaging revealed dural thickening, confirming HP. The patient's medical history revealed double ANCA positivity and a lung biopsy confirmed granulomatous pneumonitis. A diagnosis of ANCA-associated vasculitis (granulomatosis with polyangiitis (GPA)) was established, and treatment with rituximab and high-dose corticosteroids led to symptom improvement. GPA rarely involves meningeal inflammation, but severe and persistent headaches are common early symptoms. Inflammatory markers are often elevated, and around two-thirds of HP cases related to GPA have positive serum ANCA. MRI is the primary diagnostic tool, with characteristic findings of dural thickening and contrast enhancement. This case highlights HP as a rare cause of chronic headaches and the importance of a comprehensive medical history in diagnosis. Early recognition and treatment are crucial for improving outcomes in GPA-related HP.

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In this case report, we discuss and explore the clinical, laboratory, and imaging findings, as well as the treatment options and follow-up measures, in an 83-year-old patient with idiopathic hypertrophic pachymeningitis (IHP), a rare disorder characterized by fibrosing, hypertrophic inflammation that thickens the dura mater. An 83-year-old female with a medical history of hypertension and hyperlipidemia presented with speech arrest and was taken to the emergency department, where she received a stroke code, a CT scan, and an MRI. The MRI results showed a temporal lobe meningioma and a pan-cranial pachymeningitis encasing the entire brain and cerebellum and extending into the upper cervical spine. Multiple unsuccessful attempts at a lumbar puncture were made, so a dural biopsy specimen was obtained, which revealed no malignant process. A cerebral spinal fluid specimen (CSF) from the biopsy showed minimal white blood cells (WBCs) which ruled out infection. Idiopathic hypertrophic pachymeningitis was the given diagnosis based on the apparent MRI findings. The patient was treated in the hospital for four days with IV methylprednisolone and discharged on oral methylprednisolone for four to six weeks.

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Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder associated with fibroinflammatory conditions that can affect multiple organs. Hallmark histopathological findings of IgG4-RD include lymphocytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. However, little is known about central nervous system involvement of IgG4-RD. Hypertrophic pachymeningitis (HP) has recently been reported as a manifestation of IgG4-RD, which may have previously been demonstrated in a significant percentage of idiopathic cases. Herein, we report a rare case of a 63-year-old male who presented with a scalp mass that mimicked a brain tumor. He was diagnosed with IgG4-related HP (IgG4-RP) after surgery. This case suggests that awareness of a possibility of IgG4-RP in patients with isolated scalp masses, even in the absence of systemic symptoms, is crucial. A combination of careful history taking, evaluation of serum IgG4-levels and imaging as an initial work-up, followed by tissue biopsy, is important for the differential diagnosis of IgG4-RP, malignancy, and other infectious diseases.