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Abstract Background To report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis. Case presentation A 40-year-old female patient with tetraparesis, dysphagia and dysphonia was evaluated with clinical examination, magnetic resonance imaging, and meningeal biopsy. Magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement involving the cranial, cervical, thoracic, and lumbar segments with spinal cord compression and cystic lesions. CSF immunology was initially positive for cysticercus cellulosae. After disease progression a meningeal biopsy was compatible with IgG4 related disease. The patient had partial response to rituximab and needed multiple surgical procedures for spinal cord decompression and CSF shunting. Conclusions This case highlights the possibility of IgG4-related disease in patients with diffuse pachymeningitis causing spinal cord compression, even with cystic lesions on MRI. Diagnosis of IgG4-related pachymeningitis is paramount due to the possibility of treatment response to immunotherapy, particularly to anti-CD20 agents.

This case represents the first diagnosis of pachymeningitis due to granulomatosis with polyangiitis (GPA) in an elderly Iranian man who initially presented with persistent daily headaches. PCR tests of cerebrospinal fluid for tuberculosis, brucellosis, and fungal infections all yielded negative results. Given the pachymeningitis pattern observed on brain MRI and the absence of infectious and lymphoma diseases, along with positive anti‐PR3 and proteinuria (793 mg in a 24‐h urine sample), a diagnosis of GPA was established. The patient was treated with five doses of pulse methylprednisolone and one dose of pulse cyclophosphamide (1 g). Additionally, prednisolone 60 mg daily, monthly pulse cyclophosphamide, a daily calcium‐D tablet, and alendronate 70 mg weekly were prescribed. Subsequently, the patient's headaches, hearing loss, and vision loss were completely resolved. GPA should be considered in older individuals with persistent daily headaches, especially when pachymeningitis is evident. The use of contrast‐enhanced brain MRI is an essential diagnostic tool in such cases. [ABSTRACT FROM AUTHOR]

Background: To report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis. Case presentation: A 40-year-old female patient with tetraparesis, dysphagia and dysphonia was evaluated with clinical examination, magnetic resonance imaging, and meningeal biopsy. Magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement involving the cranial, cervical, thoracic, and lumbar segments with spinal cord compression and cystic lesions. CSF immunology was initially positive for cysticercus cellulosae. After disease progression a meningeal biopsy was compatible with IgG4 related disease. The patient had partial response to rituximab and needed multiple surgical procedures for spinal cord decompression and CSF shunting. Conclusions: This case highlights the possibility of IgG4-related disease in patients with diffuse pachymeningitis causing spinal cord compression, even with cystic lesions on MRI. Diagnosis of IgG4-related pachymeningitis is paramount due to the possibility of treatment response to immunotherapy, particularly to anti-CD20 agents. [ABSTRACT FROM AUTHOR]

Introduction: Rheumatoid pachymeningitis and optic neuritis are rare complications of rheumatoid arthritis (RA) and are a diagnosis of exclusion. Case description: A 75-year-old male with a history of seronegative RA presented to the emergency department with left eye pain and blurry vision lasting two days. He had been diagnosed with seronegative RA around nine months previously. His blood pressure was elevated at 204/75 mmHg upon arrival. Physical examination revealed left conjunctival injection, mild ptosis, painful extraocular movements and tenderness over the orbit and sinuses. Initial treatments included painkillers and intravenous labetalol, which alleviated his symptoms and decreased his blood pressure. Laboratory tests showed a C-reactive protein of 2.5 mg/dl and an erythrocyte sedimentation rate of 32 mm/h, with other blood work unremarkable. A computed tomography (CT) angiogram of the head and neck showed no high-grade stenosis. Given his RA history, initial concerns included scleritis. A magnetic resonance imaging (MRI) of the brain and orbit revealed inflammation around the left optic nerve, and pachymeningitis at the left cerebral convexity and interhemispheric fissure, suggesting hypertrophic pachymeningitis. An ophthalmologic examination was unremarkable. Treatment was adjusted to include pulse doses of intravenous methylprednisolone for optic neuritis, resulting in significant pain relief. Though inadequate for complete testing, a lumbar puncture indicated an inflammatory disorder with elevated glucose (199 mg/dl), protein (109 mg/dl), and unremarkable WBC/RBC and Gram staining. Cytology and culture were unremarkable. The most likely diagnosis at this point was rheumatological meningitis and rheumatological optic neuritis. The patient improved markedly with high-dose steroids over four days and was discharged on prednisone. Conclusion: In cases of optic neuritis and pachymeningitis, RA should remain on the differential in patients with or without a prior diagnosis.

IgG4-related diseases (IgG4-RD) are a group of chronic progressive autoimmune diseases of unknown etiology that are increasingly recognized as an important pathophysiological basis for a variety of systemic diseases. It is thought to involve almost any organ of the body, but the involvement of the central nervous system is relatively rare. We report the case of a 56-year-old male patient admitted to the hospital d recurrent dizziness and nausea for more than 3 months. The preoperative imaging was misdiagnosed as a meningioma, with this lesion demonstrated localized inhomogeneous thickening of the meninges in the left parietal region on T2-weighted and T2 fat suppression sequences with localized nodular changes. The patient's final pathologic diagnosis was IgG4-associated sclerosing thick encephalitis. The diagnosis of IgG4-associated hypertrophic pontine meningitis is challenging. Clinically, IgG4-associated sclerosing diseases usually present as mass-like lesions, which can be easily misdiagnosed as neoplastic lesions due to their similar appearance. These fundamentally recognized autoimmune disorders respond well to corticosteroid therapy. Therefore, accurate detection of IgG4-related disease is critical to prevent patients from undergoing unnecessary surgery.

Background and purpose: Rheumatoid meningitis (RM) is a meningeal inflammation rarely occurring during the course of rheumatoid arthritis (RA). Anti-citrullinated protein antibodies (ACPA), which are highly specific biomarkers of RA, may allow RM sine arthritis (RMSA) to be diagnosed as an extra-articular early lesion of RA. MRI typically displays pachymeningitis and/or leptomeningitis predating convexity, and CSF diffusivity is focally restricted. Blood ACPA is a strong clue and intrathecal synthesis of ACPA is characteristic although rarely reported. Methods: We report two cases of RMSA that were confirmed by meningeal biopsy and intrathecal synthesis. Results: RMSA abated after steroids alone or associated with rituximab. No arthritis occurred during the follow-up. Conclusion: RM can occur in the absence of RA.

Idiopathic hypertrophic pachymeningitis is a rare, fibrosing inflammatory disease that causes localized or diffuse thickening of the dura mater in the brain and/or spinal cord. It may be cranial, spinal, and/or craniospinal pachymeningitis depending on the place of involvement. In our case, a 34-year-old woman presented with sixth cranial nerve involvement and headache and was diagnosed with idiopathic hypertrophic pachymeningitis as a result of the exclusion of other causes and central nervous system imaging. Clinical and radiological follow-up occurred for 1 year, and treatment is presented. [ABSTRACT FROM AUTHOR]

IgG4-related disease (IgG4-RD) is an immune-mediated multi-system disorder. The nervous system (IgG4-RND) is rarely affected. We describe a short case series. We performed an ambispective analysis of IgG4-RND patients admitted at our centre between January 2016 and December 2022. Eight patients (M: F-2:6) were included with a mean age at presentation of 40.63 ± 17.88 years and disease duration of 5.16 ± 4.08 years. The common diseased sites were pachymeninges (7), orbits (4), paranasal sinuses (3), frontal lobe (1), hypophysis (1), leptomeninges (1), and middle ear (1). Common symptoms were headache and cranial neuropathy. The common nerves involved were the optic nerve, followed by the third, fifth, sixth, and seventh. Cerebrospinal fluid showed lymphocytic pleocytosis. Histopathology showed lymphoplasmacytic infiltrate (8), fibrosis (5), >10 IgG4 + cells (7), and IgG4/IgG >40% (6). Six had a relapsing course. The rituximab-based treatment regimen showed a favourable response. [ABSTRACT FROM AUTHOR]

Objective: Our understanding of IgG4‐RD and pachymeningitis has grown substantially, but the optimal approach for diagnosis, management, and long‐term outcomes is still an area of uncertainty. Methods: HUVAC is a database for IgG4‐RD patients, this database was retrospectively evaluated for pachymeningeal disease. Demographic, clinical, serological, imaging, histopathological data, and treatment details were re‐interpreted in patients with pachymeningitis. Results: Among 97 patients with IgG4‐RD, 6 (6.2%) had pachymeningitis. None of these patients had extracranial features, and also, in most of the patients, serum IgG4 levels were normal. Tentorium cerebelli and transverse sinus dura were the most commonly involved in the posterior fossa. During 18 months of median follow‐up on steroid+‐rituximab, none of them relapsed as pachymeningitis. Conclusion: Our patients were mainly older males with sole neurological involvement. Non‐specific headache was the most common manifestation, and serum IgG4 levels were not useful for diagnosis. Typical radiology and tentorial thickening should suggest IgG4‐RD and prompt an early biopsy. Moreover, accompanying hypophysitis could also be a clue. With steroids+ rituximab treatment, no relapse related to meningeal involvement was seen in long‐term follow‐up. [ABSTRACT FROM AUTHOR]

ObjectiveWe describe three rare cases of spontaneous intracranial hypotension (SIH) presenting with symptoms of endolymphatic hydrops (EH) and perform a literature review to bring attention to a rare link between SIH and EH.PatientA 59-year-old female presented with postural headache, aural fullness, vertigo, hearing loss, and abnormal electrocochleography after being diagnosed with SIH by magnetic resonance imaging. The site of cerebrospinal fluid leak was identified in this individual. Two additional patients with vertigo, hearing loss, and SIH were identified by retrospective chart review.InterventionAll patients underwent blood patches. One patient also had diuretic treatment while another had fibrin glue injection.Main outcome measuresThe outcomes of interest were resolution of headache, vertigo, aural fullness, and hearing loss.ResultsAll patients eventually improved with time. Literature review suggests that overall outcome is excellent.ConclusionsSIH may be an under-recognized cause of EH. We support the theory that negative intracranial pressure transmitted through the cochlear aqueduct and perilymph leads to EH. Despite the variations in treatments, the overall prognosis is excellent.

IgG4-related disease (IgG4-RD) is an emerging immune-mediated chronic fibrotic disease characterized by tumour-like mass formation. Reports of brain parenchymal involvement in IgG4-RD are rare and complete treatment-related remission of lesions has never been reported. Here, we present a woman in her mid-50s who developed headache and seizures. Brain magnetic resonance imaging revealed frontal bilateral pachymeningitis and a left frontal lobe parenchymal lesion, and pathologic findings were consistent with an IgG4-RD central nervous system manifestation. She had a history of tumour-like growth around the right optic nerve, orbital and maxillary cavities treated successfully with corticosteroids 28 years ago, and was receiving infliximab as a maintenance therapy for uveitis for the last 14 years. After initial high-dose corticosteroid treatment, the patient was treated with rituximab, and after 3 months, the patient presented with complete remission of IgG4-RD lesions and associated symptoms. This case illustrates the chronic, decades-spanning nature of IgG4-RD, and a complete response to rituximab even with intracerebral mass lesions that had emerged despite the use of infliximab, a therapy previously reported successful in IgG4-RD. [ABSTRACT FROM AUTHOR]

Le dosage des IgG totales sur sérum est un examen courant de biologie médicale. Plus spécialisé, le dosage des sous- classes d'IgG sur sérum trouve sa place en deuxième ligne lors d'un bilan de déficit immunitaire ou pour la maladie associée aux IgG4 (MAG4). Dans le liquide céphalorachidien, leur évaluation permet la distinction entre un transsudat et une synthèse intrathécale. Concernant les sous-classes d'IgG dans le LCR, les laboratoires de biologie médicale commencent à recevoir des demandes, afin de mettre en évidence la présence d'IgG4. Dans le LCR elles seraient liées à la pachyméningite hypertrophique associée à la MAG4 mais, en l'absence d'étude clinique de grande envergure, leur dosage ne semble être pertinent que lorsque la biopsie tissulaire est impossible. La technique de dosage validée sur sérum est quantitative en milieu liquide (néphélémétrie/turbidimétrie). L'utilisation de ces techniques sur LCR doit être validée par chaque laboratoire, en l'absence de validation des kits sur LCR. Cette revue actualisée de la littérature revient sur les différentes indications des dosages d'IgG et des sous-classes d'IgG sur sérum, sur l'intérêt du dosage des sous- classes d'IgG sur le LCR et les techniques associées. Serum 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. [ABSTRACT FROM AUTHOR]

IgG4-related disease-associated hypertrophic pachymeningitis (IgG4RD-HP) is a fibroinflammatory autoimmune disorder in which diagnosis is difficult without biopsy. Guidance on management of disease refractory to glucocorticoids and intravenous rituximab is limited. We present the case of a 68-year-old woman with IgG4RD-HP who developed sensorineural hearing loss with associated bulky basilar pachymeningeal enhancement. Her cerebrospinal fluid was inflammatory and had an elevated IgG4 concentration, strongly suggestive of IgG4RD-HP. Biopsy of involved meninges was not possible due to surgical risk. Over years she developed bilateral optic neuropathies and hydrocephalus, requiring intravenous rituximab and ventriculoperitoneal shunt. Her disease was refractory to glucocorticoids. Despite maintenance intravenous rituximab, she developed slowly progressive symptoms of intracranial hypertension and hydrocephalus with persistently inflammatory spinal fluid. Switching to intrathecal rituximab therapy led to dramatic improvement in gait and headache and reduced pachymeningeal bulk and metabolic activity. In patients with IgG4RD-HP refractory to glucocorticoids and intravenous rituximab, intrathecal rituximabmay be an efficacious therapy. [ABSTRACT FROM AUTHOR]

A 52-year-old male patient presented with longstanding non-specific symptoms of ocular redness and irritation. Clinical examination not only revealed bilateral anterior scleritis but also bilateral optic disc swelling. Additional history taking revealed headaches and tinnitus, both starting around the same time as the eye redness, as well as a prior episode of swelling and redness of both ears. The lumbar puncture opening pressure was 29 cm of cerebrospinal fluid (CSF). There were 11 white blood cells/µl in the CSF. Subsequent magnetic resonance imaging showed focal thickening of the dura mater over the left cerebral convexity, suggestive of focal pachymeningitis. 18F-fluorodeoxyglucose positron emission tomography demonstrated hypermetabolic abnormalities located at the auricles, the nostrils, the anterior part of the eyes, and the dura mater over the left cerebral convexity, suggestive of relapsing polychondritis (RPC). RPC is a rare systemic immune-mediated condition; diagnosis can sometimes be delayed or missed due to insidious disease onset with non-specific symptoms. Nevertheless, sight-threatening or even life-threatening complications may occur. Given the high prevalence of ocular involvement, one should be suspicious when faced with patients with recurrent ocular inflammation. Optic disc swelling is a more uncommon finding, and while different mechanisms have been reported, it has rarely been associated with raised intracranial pressure. Nevertheless, intracranial hypertension arising from inflammation of the CSF and/or surrounding meninges caused by the newly diagnosed RPC was identified as the most likely underlying mechanism for the bilateral optic disc swelling in our patient. [ABSTRACT FROM AUTHOR]

To report a patient with concomitant aceruloplasminemia (with a novel mutation) and IgG4-related pachymeningitis and to hypothesize on the possible relation between the two diseases. Clinical, radiological, and laboratory features of a 56-year-old lady with chronic headache, bifacial palsy, and cerebellar signs are described. Pathophysiology of aceruloplasminemia leading to hyperferritinemia and consequent immune activation is elucidated. A coherent explanation of IgG4-related pachymeningitis resulting from aceruloplasminemia and hyperferritinemia is given. The patient has aceruloplasminemia with a novel nonsense mutation. She also suffers from biopsy-proven IgG4 related pachymeningitis as per standard criteria. These two seemingly unrelated illnesses are linked by hyperferritinemia. This case is a fine example of Occam's razor. Immune dysfunction and autoimmune disorders in aceruloplasminemia need to be explored through further studies to look for causal associations. [ABSTRACT FROM AUTHOR]

IgG4-related disease-associated hypertrophic pachymeningitis (IgG4RD-HP) is a fibroinflammatory autoimmune disorder in which diagnosis is difficult without biopsy. Guidance on management of disease refractory to glucocorticoids and intravenous rituximab is limited. We present the case of a 68-year-old woman with IgG4RD-HP who developed sensorineural hearing loss with associated bulky basilar pachymeningeal enhancement. Her cerebrospinal fluid was inflammatory and had an elevated IgG4 concentration, strongly suggestive of IgG4RD-HP. Biopsy of involved meninges was not possible due to surgical risk. Over years she developed bilateral optic neuropathies and hydrocephalus, requiring intravenous rituximab and ventriculoperitoneal shunt. Her disease was refractory to glucocorticoids. Despite maintenance intravenous rituximab, she developed slowly progressive symptoms of intracranial hypertension and hydrocephalus with persistently inflammatory spinal fluid. Switching to intrathecal rituximab therapy led to dramatic improvement in gait and headache and reduced pachymeningeal bulk and metabolic activity. In patients with IgG4RD-HP refractory to glucocorticoids and intravenous rituximab, intrathecal rituximab may be an efficacious therapy.

To report a patient with concomitant aceruloplasminemia (with a novel mutation) and IgG4-related pachymeningitis and to hypothesize on the possible relation between the two diseases. Clinical, radiological, and laboratory features of a 56-year-old lady with chronic headache, bifacial palsy, and cerebellar signs are described. Pathophysiology of aceruloplasminemia leading to hyperferritinemia and consequent immune activation is elucidated. A coherent explanation of IgG4-related pachymeningitis resulting from aceruloplasminemia and hyperferritinemia is given. The patient has aceruloplasminemia with a novel nonsense mutation. She also suffers from biopsy-proven IgG4 related pachymeningitis as per standard criteria. These two seemingly unrelated illnesses are linked by hyperferritinemia. This case is a fine example of Occam's razor. Immune dysfunction and autoimmune disorders in aceruloplasminemia need to be explored through further studies to look for causal associations.

Rosai-Dorfman disease is characterized by dilated lymph node sinuses filled with lymphocytes, plasma cells, and histiocytes. Many of these histiocytes classically exhibit emperipolesis of lymphocytes and plasma cells. Abundant immunoglobulin G4+ plasma cells occur in some cases, and a potential relationship with immunoglobulin G4-related disease has been suggested. Here, we report 3 cases of immunoglobulin G4-associated Rosai-Dorfman disease. Immunoglobulin G4-related disease was suspected based on immunoglobulin G4+ plasma cell infiltration, but the final diagnosis was immunoglobulin G4-associated Rosai-Dorfman disease. At present, the evidence does not support a link between immunoglobulin G4-associated Rosai-Dorfman disease and immunoglobulin G4-related disease, and one condition should not be considered part of the spectrum of the other. We believe it is of paramount importance to increase the awareness of immunoglobulin G4-associated Rosai-Dorfman disease for pathologists who interpret the biopsies and clinicians who integrate the diagnosis and treat such patients to not overdiagnose immunoglobulin G4-related disease. [ABSTRACT FROM AUTHOR]

Abstract Background Lymphoplasmacyte-rich meningioma (LPRM) is a rare form of meningioma characterized by prominent lymphoplasmacytic infiltrates into the tumor. Report of flat growth of LPRM mimicking pachymeningitis is rare in the literature. Case presentation A 55-year-old female who suffered from episodes of headache and seizures has been diagnosed with pachymeningitis for 4 years because post contrast brain MRI demonstrated enhanced carpet-like dura lesion in the left frontal lobe. The lesion kept unchanged on yearly follow-ups until a recent brain MRI found the lesion grew significantly into a mass. The lesion was resected and pathology suggested LPRM. Conclusion LPRM may present as carpet-like growth pattern on MRI. Long-term follow-up in patients with pachymeningitis is necessary.

A 55-year-old woman with a history of severe endometriosis, followed at our center for 16 years due to multiple episodes of horizontal binocular diplopia, was diagnosed with recurrent paresis of the VI cranial nerve of her right eye. Magnetic resonance imaging revealed localized pachymeningitis in her right cavernous sinus. Initial episodes were treated with botulinum toxin. Subsequently, she declined treatment during inter-crisis periods because she remains asymptomatic. Idiopathic hypertrophic pachymeningitis is a rare condition of unknown cause, characterized by chronic inflammation of the meninges resulting in localized or diffuse dural thickening. Clinical manifestations vary widely, from generalized processes with complex neurological symptoms to localized processes with monosymptomatic expression, as presented in this case., Competing Interests: Declaration of competing interest None declared., (Copyright © 2024 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: Rheumatoid pachymeningitis and optic neuritis are rare complications of rheumatoid arthritis (RA) and are a diagnosis of exclusion., Case Description: A 75-year-old male with a history of seronegative RA presented to the emergency department with left eye pain and blurry vision lasting two days. He had been diagnosed with seronegative RA around nine months previously. His blood pressure was elevated at 204/75 mmHg upon arrival. Physical examination revealed left conjunctival injection, mild ptosis, painful extraocular movements and tenderness over the orbit and sinuses. Initial treatments included painkillers and intravenous labetalol, which alleviated his symptoms and decreased his blood pressure. Laboratory tests showed a C-reactive protein of 2.5 mg/dl and an erythrocyte sedimentation rate of 32 mm/h, with other blood work unremarkable. A computed tomography (CT) angiogram of the head and neck showed no high-grade stenosis. Given his RA history, initial concerns included scleritis. A magnetic resonance imaging (MRI) of the brain and orbit revealed inflammation around the left optic nerve, and pachymeningitis at the left cerebral convexity and interhemispheric fissure, suggesting hypertrophic pachymeningitis. An ophthalmologic examination was unremarkable. Treatment was adjusted to include pulse doses of intravenous methylprednisolone for optic neuritis, resulting in significant pain relief. Though inadequate for complete testing, a lumbar puncture indicated an inflammatory disorder with elevated glucose (199 mg/dl), protein (109 mg/dl), and unremarkable WBC/RBC and Gram staining. Cytology and culture were unremarkable. The most likely diagnosis at this point was rheumatological meningitis and rheumatological optic neuritis. The patient improved markedly with high-dose steroids over four days and was discharged on prednisone., Conclusion: In cases of optic neuritis and pachymeningitis, RA should remain on the differential in patients with or without a prior diagnosis., Learning Points: Rheumatoid arthritis (RA) is a systemic disease but can rarely cause neurological involvement.Pachymeningitis is an inflammation of the dura mater, rarely caused by RA.Optic neuritis can also rarely be caused by RA and responds to steroids., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2024.)

Tolosa–Hunt syndrome (THS) remains a challenging diagnosis for many neurologists. Often believed to be a rare presentation, the classical presentation is known to involve cranial nerves and tissues surrounding the cavernous sinus. Traditionally, a diagnosis of THS is considered when all secondary conditions have been ruled out. Yet, newer findings have elaborated a complex pathogenetic process with some overlap from the IgG4 spectrum of disorders, with which it shares many phenotypic similarities. In this narrative review, we present an updated picture of the condition focusing on the latest developments in the pathogenesis, diagnosis, and clinical management of these two conditions and use illustrative examples to highlight the salient features of this rare presentation.

Background: Melioidosis is an endemic disease in South-East Asia and Northern Australia caused by a Gram-negative bacillus, Burkholderia pseudomallei. Manifestations are wide and neurological involvement have rarely been described. Methods: In this paper, we report a patient returning from Asia with an unusual infection including CNS involvement consistent with a melioidosis. Results: This diagnosis was challenging and complex to carry out with multiple considerations, mainly because of the atypical nature of the germ. Burkholderia pseudomallei can be easily misidentified with Burkholderia thailandensis (rarely pathogenic to humans) during bacterial culture because of their phylogenetic proximity. The main pitfall of the management was that the responsible infectious agent was not referenced in the MALDI-TOF (considered as a bioterrorism agent) and led to a wrong strategy. Conclusions: This case of melioidosis shows the difficulty regarding the diagnosis of this disease in a patient returning from an endemic zone and its frequent multiple organs involvement. Melioidosis is an emerging, potentially fatal disease which requires prolonged antibiotic treatment. Difficulties in clinical microbiology laboratories diagnosis of melioidosis, especially in non-endemic areas where clinical suspicion is low, may delay treatment and affect disease outcomes.

Objective: IgG4-related hypertrophic pachymeningitis is a rare fibroinflammatory disorder that may cause localized or diffused thickening of the dura mater. Misinterpretations of the clinical and imaging findings are common. Clinical manifestations depend on the location of the inflammatory lesion and on compression of neural structures leading to functional deficits. A dural biopsy is commonly needed for a definitive diagnosis. Immunomodulatory therapy is considered the therapy of choice. Methods: Four patients with IgG4-related hypertrophic pachymeningitis were identified over a 5-year period. Patient-related characteristics including age, preoperative workup, signs and symptoms of patients, and diagnostic procedures were evaluated. Furthermore, the surgical treatment and 5-year follow-up outcomes were analyzed. Results: There were two adults and two adolescents (mean age 32 years; range 15 to 67 years). Two patients were male, and two were female. No history of disease was known in any of the patients. Clinical symptoms were epilepsy (n = 2), ataxia and nausea (n = 1), and facial nerve palsy (n = 1). MR imaging studies showed contrast enhancing lesions in the temporal region in two patients, and in the cerebellar region in the other two patients. Subtotal resection was performed in two instances and a biopsy via a suboccipital retrosigmoid approach was obtained in the other two patients. Histochemical and immunohistochemical investigations revealed an IgG 4 disease in all of these patients. Immunomodulatorry therapy led to clinical stability during follow-up of 5 years in all four cases. Conclusion: The diagnosis of IgG4-related hypertrophic pachymeningitis is challenging, but is of great relevance as treatment differs significantly from other forms of pachymeningitis and a specific therapeutic approach may avoid long-term neurological complications. Our series contributes to a better clinical characterization of this rare disease. [ABSTRACT FROM AUTHOR]

Tolosa--Hunt syndrome (THS) remains a challenging diagnosis for many neurologists. Often believed to be a rare presentation, the classical presentation is known to involve cranial nerves and tissues surrounding the cavernous sinus. Traditionally, a diagnosis of THS is considered when all secondary conditions have been ruled out. Yet, newer findings have elaborated a complex pathogenetic process with some overlap from the IgG4 spectrum of disorders, with which it shares many phenotypic similarities. In this narrative review, we present an updated picture of the condition focusing on the latest developments in the pathogenesis, diagnosis, and clinical management of these two conditions and use illustrative examples to highlight the salient features of this rare presentation. [ABSTRACT FROM AUTHOR]

Nervous system involvement in IgG4‐related systemic disease (IgG4‐RD) is rarely reported and manifests as hypertrophic pachymeningitis and hypophysitis. In this report, a 33‐year‐old woman with neurological manifestations was diagnosed with IgG4‐RD by biopsy. The patient showed improvement in symptoms after treatment. [ABSTRACT FROM AUTHOR]

A 36-year-old male patient who originally presented with recurrent inflammations in the mastoid, otitis media and peripheral facial paralysis was diagnosed with sterile pachymeningitis, associated with high titres of antineutrophil cytoplasmic antibodies (cANCA) directed against proteinase 3 (PR3). Induction therapy with oral prednisolone 1 mg/kg body weight and cyclophosphamide (CYC) 750 mg/m 2 i.v. every 4 weeks was initiated. Due to side effects, increasing arthralgias and progressive meningitis after 5 doses of CYC, treatment was changed to rituximab (RTX), one cycle comprising two administrations of 1000 mg RTX i.v. two weeks apart, repeated every 6 months. After the very first cycle of RTX, we confirmed subjective improvement of the patient’s fitness as well as radiologic response. Methotrexate (MTX) was added to ease arthralgias. Painful bleeding ulcerations on finger tips were successfully treated with 22 iloprost infusions. Up to date, we have administered 7 cycles of RTX and achieved complete remission of the cANCA-associated vasculitis. After the induction therapy, maintenance treatment with MTX or rituximab will be performed for at least 18–24 months.We can demonstrate a complete remission with the use of RTX in a patient with cANCA-associated vasculitis and sterile pachymeningitis who failed to respond to the induction treatment with CYC. RTX has been well tolerated.

Rheumatoid meningitis (RM) presents with sufficiently wide-ranging, but non-specific, symptoms and neuroimaging features of pachy- and/or leptomeningeal thickening that it may be indistinguishable from subacute infectious meningitis. RA diagnosis variably antedates RM and serological confirmation by rheumatoid factor and anti-citrullinated peptide antibodies may not be present preoperatively. Thus, meningeal biopsy may be undertaken. Classic examples of RM show lymphoplasmacytic leptomeningeal inflammation and small vessel vasculitis, with rheumatoid nodules being less frequent. We reviewed our experience with 5 RM biopsies, as well as the literature, placing "classic" histological findings in perspective with biopsies showing less pathognomonic features. 5 RM cases were identified, 2 male: 3 female, ages 62–79 years. All patients had leptomeningeal enhancement by MRI and 2 had known negative RF serology prior to meningeal biopsy. In 1 case RF was initially negative, but on serological reassessment turned positive; 2 patients were diagnosed by clinical correlation. 4 leptomeningeal/superficial cortical biopsies manifested chronic lymphoplasmacytic inflammation with multinucleated giant cells, with discrete foci of deep blue/black necrosis with cellular debris ("dirty necrosis") surrounded by a variably- developed palisade of histiocytes (rheumatoid nodules). The 5th showed only non-specific mononuclear cell inflammation. All showed variable degrees of diffuse astrocytosis and microgliosis of the cortex without microglial clusters or compact granulomas. Stains for microorganisms were negative. Diagnosis of RM can be suspected by the pathologist if the "classic" features of rheumatoid nodules are present on biopsy, but in some cases, only non-specific inflammation is present. Diagnosis necessitates correlation between clinical, serological, and histological features. • Rheumatoid meningitis has wide-ranging neuroimaging features that can be non-specific. • All 5 rheumatoid meningitis cases were diagnosed by meningeal biopsy. • Biopsy revealed varying degrees of "classic" histologic findings for rheumatoid meningitis. • Leptomeningeal enhancement by MRI was identified in all 5 cases of rheumatoid meningitis. [ABSTRACT FROM AUTHOR]

Abstract Nervous system involvement in IgG4‐related systemic disease (IgG4‐RD) is rarely reported and manifests as hypertrophic pachymeningitis and hypophysitis. In this report, a 33‐year‐old woman with neurological manifestations was diagnosed with IgG4‐RD by biopsy. The patient showed improvement in symptoms after treatment.

Background: Lymphoplasmacyte-rich meningioma (LPRM) is a rare form of meningioma characterized by prominent lymphoplasmacytic infiltrates into the tumor. Report of flat growth of LPRM mimicking pachymeningitis is rare in the literature.Case Presentation: A 55-year-old female who suffered from episodes of headache and seizures has been diagnosed with pachymeningitis for 4 years because post contrast brain MRI demonstrated enhanced carpet-like dura lesion in the left frontal lobe. The lesion kept unchanged on yearly follow-ups until a recent brain MRI found the lesion grew significantly into a mass. The lesion was resected and pathology suggested LPRM.Conclusion: LPRM may present as carpet-like growth pattern on MRI. Long-term follow-up in patients with pachymeningitis is necessary. [ABSTRACT FROM AUTHOR]

Background: IgG4-related disease is a rare, recently recognized chronic inflammatory disease. IgG4-related hypertrophic pachymeningitis (IgG4-RHP) of the central nervous system predominantly involves the cranial meninges. Spinal involvement remains rare.Objective: We report a case of recurrent cervicothoracic IgG4-RHP and review the surgical literature.Methods and Materials: A 35-year-old woman presented with a 6-month history of neck and right shoulder pain, progressive right triceps weakness and paresthesias in the right C8 and T1 dermatomes. MRI demonstrated a T2 hypointense epidural soft tissue mass extending from C6-T1. The patient underwent C6-T1 laminoforaminotomy and partial resection with near complete symptom resolution. Histopathology was consistent with diagnosis of IgG4-RHP. Eighteen months postoperatively, she experienced symptom recurrence necessitating re-operation and adjuvant postoperative prednisone with complete resolution at 40-months' follow-up.Results and Conclusions: Of the now nineteen confirmed cases of IgG4-RHP, fifteen underwent surgery. A majority achieved partial resection. Three surgical patients did not receive adjuvant therapy with symptomatic recurrence between 2 and 18 months. [ABSTRACT FROM AUTHOR]

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an extremely rare clinical entity of unknown etiology, characterized by a chronic inflammation causing thickening of the dura mater.[1] ICHP can produce similar presentation to Tolosa–Hunt syndrome (THS) if it involves cavernous sinus.[2] THS might represent a focal manifestation of IHCP.[2] The THS is a rare syndrome with an estimated annual incidence of one case per million per year.[3] It typically presents with orbital pain associated with palsy of the third, fourth, or sixth cranial nerve. We present an interesting case of a patient with IHCP associated with THS in an elderly female who responded well to high-dose steroids and in a few days had significant improvement in her retro-orbital pain and ocular movements. The importance of appropriate neuroimaging and serological investigation in patients with suspected THS, role of erythrocyte sedimentation rate in follow-up of these patients, and the relationship between IHCP and THS are discussed.

Abstract IgG4‐related disease (IgG4RD) with intracranial involvement is rare. We report a 56‐year‐old male who had an excellent response to rituximab and dexamethasone after going undiagnosed for 5 years. After 3 years of rituximab maintenance, he has no evidence of disease on brain MRI.

ABSTRACT Importance: Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. Observations: HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. Conclusion: HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

Inrtoduction: Granulomatosis with polyangiitis (GPA) is a systematic and necrotizing vasculitis with positive autoimmune antibodies. Some studies have reported the prevalence of eye involvement between 40%-50% of cases. Retro orbital granuloma is a rare complication of GPA which should be treated by surgical involvements, while pachymeningitis can be diagnosed by MRI and treated by medical management. In this study, we tried to present a case of GPA with optic neuritis and typical central nervous system (CNS) involvement, while there were no definite features of sinusitis or kidney injuries. Case Presentation: A 15-year-old girl was admitted because of blurred vision in her left eye. She was a known case of GPA three years ago with initial features, including left facial nerve paresis due to pan-sinusitis and pulmonary cavity. Neurologic evaluations, including sensory and motor features, were normal, too. Ophthalmologic examinations showed that visual acuity of the right eye was good, while the visual acuity in the left eye decreased to the point of finger counting at a distance of 20 cm. The left eye Marcus gunn test was positive (3+); anterior and posterior eye segments were normal. The patient was evaluated by brain MRI with gadolinium and a pathologic enhancement in the left cavernous was seen which had a pressure effect on the optic nerve. She was treated by intravenous methylprednisolone followed by rituximab. Conclusion: Reporting orbital mass in a patient who had GPA can be supposed as granuloma which needs a biopsy to confirm a diagnosis. In our case, the imaging manifestation was heterodox for granuloma, while neurosurgical consultation recommended drug treatment for pachymeningitis.

Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by articular and extra-articular manifestations. Among extra-articular involvement, rheumatoid meningitis (RM) is a rare condition, which may exhibit variable symptoms including headache, focal and/or generalized neurologic deficits. It may develop as the preceding manifestation of RA or occur at any time of the disease course. Some drugs used for the treatment of RA may give rise to aseptic meningitis or create a tendency to infectious meningitis due to their immunosuppressive effect. All these possibilities may lead to difficulties in the differential diagnosis. Achieving a diagnosis in a short time is crucial in terms of prognosis. Here, we would like to report a case with longstanding RA manifested by left-sided weakness and seizure shortly after initiating etanercept (ETA) therapy. ETA-induced meningitis was confirmed with appropriate diagnostic tools. Our aim with this case-based review is to attract the attention of this rare condition and discuss diagnostic challenges. [ABSTRACT FROM AUTHOR]

Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a rare and chronic progressive clinical entity, characterized by elevated serum IgG4 along with tissue infiltration by IgG4 + plasma cells. It is an immune-mediated fibro-inflammatory condition that can affect virtually any organ and tissue. IgG4-related lung disease (IgG4-RLD) occupies 14% of all IgG4-RD, with nonspecific symptoms and various abnormal radiographic patterns. Published data on IgG4-related hypertrophic pachymeningitis (IgG4-RHP), an increasingly recognized central nervous system manifestation of IgG4-RD, is also limited. Both lung and cranial dura involvement have not yet been reported until now. We further entail a review of the literature on the clinicopathologic features and differential diagnosis of this uncommon disease. We herein report an interesting case of a 70-year-old male patient admitted due to headache and fever. A magnetic resonance imaging (MRI) of the brain revealed extensive dural thickening with marked enhancement. Chest computed tomography (CT) scan showed nodular or mass-like consolidation and focal interstitial change. Thoracoscopic lung biopsy and lumbar puncture were conducted. After careful histopathological observation and consideration of alternative differential diagnoses, he was diagnosed with IgG4-related disease with lung and cranial dural involvement based upon significant elevation of serum and cerebrospinal fluid (CSF) IgG4 concentration. The patient was started on oral prednisolone 60 mg/day (1.0 mg/kg/day) for 14 days, and a tapering dose of 5 mg every 2 weeks followed by maintenance therapy at low dose for 3 months. His clinical manifestations, and serologic and imaging findings improved with steroid treatment. Currently, the patient remains well without disease progression. IgG4-RD should be considered as a differential when diagnosing other similar multisystemic lesions. Clinical examination, careful histological observation, and immunostaining for appropriate markers are essential in establishing the diagnosis. Clinicians should become familiar with this alternative differential diagnosis. [ABSTRACT FROM AUTHOR]

Abstract Background Hypertrophic pachymeningitis (HP) is a rare disorder that involves localized or diffuse thickening of the dura mater. HP is associated with various inflammatory, infectious, and malignant diseases, such as rheumatic arthritis, sarcoidosis, anti-neutrophil cytoplasmic antibody-associated vasculitis, IgG4-related disorders, syphilis, tuberculosis, bacterial and fungal infections, cancer, and idiopathic diseases, when evaluation fails to reveal a cause. Among them, chronic infection with Propionibacterium acnes is a rare etiology of HP, and its pathology remains unclear. Case presentation An 80-year-old man having refractory otitis media with effusion of the right ear presented with progressive right-sided headache and nausea. Post-contrast brain magnetic resonance imaging revealed right mastoiditis and remarkable thickening of the dura mater and enhancement of pia mater extending from the right middle cranial fossa to the temporal lobe. HP secondary to middle ear infection was suspected, and a biopsy of the right mastoid was performed. An anaerobic culture of the biopsied right mastoid showed the growth of P. acnes, and histopathological examination using P. acnes-specific monoclonal antibody (PAB antibody) revealed the infiltration of inflammatory cells with P. acnes. Moreover, using PAB antibody, P. acnes was detected in the biopsy specimen of the thickening dura mater. No granulomas were identified in either specimen. HP was resolved with long-term administration of antibiotics and steroids. Conclusion This is the first documentation of pathologically demonstrated chronic HP associated with P. acnes infection followed by refractory otitis media. This report showed that chronic latent P. acnes infection induces chronic inflammation.

Context: Neurosarcoidosis (NS) is a chronic disease with a diverse clinical spectrum, therapeutic response, and outcome. There is scarce literature from our country regarding the same. Aims: The aim of this study was to evaluate the clinical spectrum, therapeutic responses, and outcomes of NS in an Indian cohort. Settings and Design: In a cross-sectional study, we included all patients with NS treated at a quaternary care teaching hospital in India from January 2007 to October 2019. Subjects and Methods: Patients older than 18 years of age fulfilling the diagnostic criteria for NS from the Neurosarcoidosis Consortium Consensus Group were included in the study. The therapeutic response and the degree of disability at last follow-up were assessed. Results: We identified 48 patients, among them 3 were categorized as having definite NS, 30 probable NS, and 15 possible NS. Cranial neuropathy was the most common presentation (47.9%), followed by myelopathy (25%). Systemic involvement was identified in 95.83% and mediastinal lymph nodes were the most common site. Clinical improvement was seen in 65.8% and disease stabilized in 28.9%, while 5.26% deteriorated. Fifty percent recovered without any residual disability, while 26.3% had minor and 23.7% had major residual sequelae. Conclusions: NS is a diverse illness, with a heterogeneous spectrum of clinical presentation, treatment response, and outcome. Cranial neuropathy is the most common presenting feature and has a good prognosis while myelopathy has an unfavorable prognosis. Meningeal and brain parenchymal disease is difficult to diagnose accurately unless systemic involvement is present. The diagnosis of NS should be clinically suspected in the appropriate clinical setting, the presence of systemic involvement should be investigated, and histologic confirmation should be attempted.

Introduction: Rheumatoid arthritis (RA) is a common illness with many extraarticular manifestations. Rheumatoid pachymeningitis is a rare neurologic complication of this common disease. Here in we present a patient with longstanding RA who developed this uncommon complication. Case description: A 75-year-old woman with longstanding RA presented to the clinic with multiple seizure-like spells per day. Upon admission to the hospital, brain MRI showed enhancement of the meninges. After an extensive workup for possible other infectious or inflammatory causes, the patient was diagnosed with rheumatoid pachymeningitis. Conclusion: Rheumatoid pachymeningitis is a rare complication of RA that usually occurs late in the disease course. It may have many neurologic manifestations include mimicking seizure or stroke and must be considered in patients with RA presenting with neurologic symptoms. Pachymeningitis is treated distinctly from articular RA, so early recognition can lead to appropriate treatment.

Introduction: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare pathological entity which is diagnosed after the exclusion of secondary reasons. Most frequently, IHCP is related to rheumatological diseases such as rheumatic arthritis, sarcoidosis, anti-neutrophil cytoplasmic antibody-associated vasculitis, and IgG4-related disorders. The inflammatory process concerns dura mater and manifests with symptoms of cranial nerve damage or compression. Case description: A 59-year-old Caucasian man presented with transitional episodes of tinnitus and an unspecific headache. Imaging studies (magnetic resonance imaging - MRI) demonstrated a thickened dura mater. Lymphoma was suspected due to an abnormal flow cytometric analysis of a cerebrospinal fluid (CSF) sample. Hematological diagnostics including bone marrow biopsy, whole body computed tomography (CT) and digestion tract examination were performed leading to the exclusion of hematological malignancy. The patient underwent surgery, and a specimen from the lesion was obtained. On this basis, intracranial idiopathic hypertrophic pachymeningitis was diagnosed. First line treatment strategy was introduced with corticosteroids with a gradual dose reduction. After the 48-month follow-up, we observed an alleviation of symptoms with a gradual significant decrease in the thickening of dura matter with almost complete resolution. Conclusions: Idiopathic hypertrophic pachymeningitis should be considered in the differential diagnosis of a thickened dura mater. Administration of steroids appears reasonable as a first line treatment of IHCP. [ABSTRACT FROM AUTHOR]

At times, spinal lesions are part of systemic manifestations of autoimmune disease. Awareness regarding their clinicopathological spectrum, particularly the lesions which usually respond to steroids/immunotherapy, is essential to avoid unwanted surgical morbidity. We discuss a case of a young-man presenting with thoracic spinal epidural compressive lesion which was indeed a manifestation of IgG4-related hypertrophic pachymeningitis. The mass was firmly adherent to the dura and extended into left neural foramen/paravertebral space which precluded complete excision. Frozen sections suggested fibro-inflammatory stroma with large areas of fibrosis and lymphoplasmacytic infiltrate. After subtotal excision, the patient improved with medical therapy at 1-year follow-up. Although uncommon, the case highlights the need to consider spinal presentation of this rare entity, especially in the context of autoimmune disorders or even in isolation. In this regard, intraoperative frozen section can hint the underlying inflammatory/autoimmune pathology, guide further course of surgery as well as limit unwarranted operative morbidity. [ABSTRACT FROM AUTHOR]

IgG4‐related disease (IgG4RD) with intracranial involvement is rare. We report a 56‐year‐old male who had an excellent response to rituximab and dexamethasone after going undiagnosed for 5 years. After 3 years of rituximab maintenance, he has no evidence of disease on brain MRI. [ABSTRACT FROM AUTHOR]

IgG4-related disease (IgG4-RD) is a complex multi-system inflammatory disorder that can affect various organs in the body. This condition is characterized by elevated levels of immunoglobulin G subclass 4 (IgG4) and the presence of specific histopathological features. While neurological involvement is not as common as in other organs, when it occurs, it can lead to hypertrophic pachymeningitis and hypophysitis. Here, we present a case of a 53-year-old male with right-sided hemicranial headache and diplopia. Computed tomography revealed a soft tissue density lesion in the middle ear cavity and mastoid antrum with the destruction of the mastoid septae. Magnetic resonance imaging revealed a lesion in the right middle ear cavity associated with pachymeningitis and right sigmoid and transverse sinus thrombosis. Tissue pathology revealed dense plasma cell-rich chronic inflammation with storiform fibrosis. Immunohistochemistry was positive for IgG4. Hence, a diagnosis of IgG4-related disease causing mastoiditis, pachymeningitis and cerebral venous thrombosis was made. The patient was successfully operated and treated with steroids. IgG4-RD remains a rare but serious condition. It is crucial to identify and treat this condition promptly as it can lead to permanent organ damage. When patients continue to experience middle ear symptoms after an infection has been treated and cancer has been ruled out, it is important to consider inflammatory conditions as a differential diagnosis., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Khaladkar et al.)