case of autoimmune GFAP astrocytopathy with eosinophils in the cerebrospinal fluid.

Cerebrospinal fluid (CSF) eosinophilia is associated with a narrow differential, primarily including parasitic and fungal infections, neoplasm, and chemical meningitis. It has rarely been reported in neuroinflammatory conditions including as a finding of CSF cytology in two autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy cases. Here we describe a case of autoimmune GFAP astrocytopathy with classic clinical and radiographic features as well as presence of eosinophils in the CSF. This case highlights a potential association of eosinophils in the CSF with autoimmune GFAP astrocytopathy, which may suggest its inclusion in the differential diagnosis of eosinophilic meningitis, encephalitis, or myelitis.

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.

IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma.

Hypertrophic pachymeningitis is a rare disorder of the dura mater of the spine or brain. It can be caused by inflammatory, infective or neoplastic conditions or can be idiopathic. We report a man with hypertrophic pachymeningitis and bilateral chronic subdural haematoma caused by IgG4-related disease. We highlight the diagnostic challenges and discuss possible underlying mechanisms of subdural haematoma formation in inflammatory conditions. Isolated IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma is very rare; previously reported cases have suggested a possible predilection for men in their sixth decade, presenting with headache as the dominant symptom. Given the rarity and complexity of the condition, it should be managed in a multidisciplinary team setting.

IgG4-related pachymeningitis-Long term follow up and outcome of six patients.

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.

Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.

Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment. Regarding the diagnosis workup, enhanced MRI is the most useful imaging method to evaluate dural thickening. This article addresses the MR imaging patterns of immune-mediated HP, including immunoglobulin G4-related disease, neurosarcoidosis, granulomatosis with polyangiitis, rheumatoid pachymeningitis, and idiopathic HP. The main infectious and neoplastic mimicking entities are also discussed with reference to conventional and advanced MR sequences.

Management of a rare case of intraventricular ruptured dermoid cyst and chemical meningitis.

INTRODUCTION: Intraventricular dermoid cyst are very rare benign tumour. Due to benign nature, it may go un-noticed for years and might present with sudden rupture. Ruptured cyst can cause chemical meningitis, hydrocephalus, seizures etc. Due to lack of data, there are no defined guidelines about its management. We are reporting an interesting case of ruptured intraventricular dermoid cyst and chemical meningitis, who was managed successfully and will review the literature. CASE REPORT: A 48 years male patient was brought with complaint of sudden mental deterioration, irritability, headache, vomiting for 3 days. He had history of seizures for 1 year. On examination, his higher mental functions were deteriorated, had neck rigidity and left lower limb weakness. CT/MRI brain confirmed diagnosis of right frontal horn ruptured dermoid cyst with scattered fat droplets, hydrocephalus and ventriculitis. Excision of cyst was done via right trans-sulcal (superior frontal sulcus) approach. Patient recovered well. DISCUSSION: Intraventricular dermoid cyst are a rare benign lesion. Due to potential of rupture, it must be surgically treated. Steroids should be used to treat chemical meningitis. Various individual case reports have shown good outcome after surgical management. CONCLUSION: We recommend early surgery, thorough ventricular wash with ringer lactate, post-operative extraventricular drain and steroid cover to manage ruptured cyst and chemical meningitis.

Meningitis as a complication of Sjögren's syndrome.

Sjögren's syndrome can be complicated by several neurological manifestations, including aseptic meningitis, which can be manifested with headache, flu-like symptoms, confusion, fever, signs of meningeal irritation, with or without focal neurological symptoms and cranial nerve palsy. Neuroimaging can reveal contrast enhancement in the lepto- or pachymeninges. Therefore, Sjögren's syndrome should be considered in the differential diagnosis of lepto- or pachymeningeal enhancement.

Neuroimaging Patterns of Intracranial Infections: Meningitis, Cerebritis, and Their Complications.

Neuroimaging provides rapid, noninvasive visualization of central nervous system infections for optimal diagnosis and management. Generalizable and characteristic imaging patterns help radiologists distinguish different types of intracranial infections including meningitis and cerebritis from a variety of bacterial, viral, fungal, and/or parasitic causes. Here, we describe key radiologic patterns of meningeal enhancement and diffusion restriction through profiles of meningitis, cerebritis, abscess, and ventriculitis. We discuss various imaging modalities and recent diagnostic advances such as deep learning through a survey of intracranial pathogens and their radiographic findings. Moreover, we explore critical complications and differential diagnoses of intracranial infections.

Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-term Outcomes.

BACKGROUND AND OBJECTIVES: Meningeal inflammation is one of the most common manifestations of neurosarcoidosis, occurring in 16%-69% of affected patients. While the clinical and radiographic features of leptomeningitis in neurosarcoidosis are well known, those of pachymeningitis are far less clear. Our primary aim was to study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment. METHODS: Patients with a diagnosis of neurosarcoidosis seen at Emory University (January 2011-August 2021) were included if pachymeningeal involvement was evident by MRI and the patient's sarcoidosis was pathologically confirmed (from a CNS or non-CNS site). RESULTS: Twenty-six of 215 (12.1%) patients with neurosarcoidosis qualified for inclusion. Pathologic confirmation came from CNS tissue in 50%. The median age of onset was 43.5 years; most were male (16/26, 61.5%). Symptoms were primarily related to pachymeningitis in 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), and seizures (7/26, 26.9%) were the most common symptoms. All patients had cranial pachymeningitis; only a single patient undergoing spinal imaging (1/11, 9.1%) had spinal pachymeningitis. The falx cerebri (16/26, 61.5%) was the most commonly affected dural structure, but the anterior and middle cranial fossae and tentorium cerebelli were frequently involved (12/26 each, 46.2%). The pachymeningeal lesions were unifocal (11/26, 42.3%) or multifocal (15/26, 57.7%) in distribution, nodular morphologically (23/25, 92.0%), and homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred with steroids initially in 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation of steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually undergoing TNF inhibition. Pachymeningeal relapses occurred in 7/26 (26.9%). The median clinical follow-up was 48 months. The median modified Rankin scale score at last follow-up improved to 1.0 from 2.0 at presentation. DISCUSSION: Pachymeningitis due to sarcoidosis often presents with headaches, visual dysfunction, and seizures; it usually affects the dura of the falx cerebri, anterior and middle cranial fossae, and tentorium cerebelli and tends to require steroid-sparing immunosuppressants. It has the potential to relapse, but the prospect for recovery is good.

Evaluation of the epidemiologic, clinical, radiologic, and treatment methods of patients with subacute and chronic meningitis.

BACKGROUND: Meningitis is known as a meningeal inflammation accompanied by pleocytosis in the cerebrospinal fluid (CSF), and can be classified into acute, subacute, and chronic meningitis based on symptoms duration of ≤ 5 days, ≥ 5 days and ≥ 4 weeks, respectively. Subacute and chronic meningitis are caused mainly by indolent infectious agents and noninfectious causes such as autoimmune, and neoplastic. In this study, we investigated the characteristics, diagnosis, and treatment of subacute and chronic meningitis. METHODS: We extracted the medical records of patients with chronic and subacute meningitis who were referred to three tertiary centers from Jun 2011 to Jun 2021. Initially, 2050 cases of meningitis were screened, and then 79 patients were included in the study. RESULTS: Headache (87.3%), nausea and vomiting (74.7%), fever (56.4%), and visual impairments (55.7%) were the most prevalent symptoms. The most common signs were nuchal rigidity (45.3%), altered mental status (26.9%), and papillary edema (37.5%). Brain computed tomography (CT) was normal in 68.6% of the patients while 22.9% of the cases had hydrocephalus. Brain magnetic resonance imaging (MRI) was normal in 60.0% of the patients. The most common abnormal MRI findings were leptomeningeal enhancement (16.0%) and hydrocephalus (16.0%). We had a 44.3% definite diagnosis with bacterial (n:25, 31.6%) and neoplastic (n:8, 10.1%) being the most prevalent etiologies. Mycobacterium tuberculosis (60%) and Brucella spp. (12%) were the most prevalent bacterial pathogens. CONCLUSIONS: The most common etiologies include infectious, neoplastic, and immunologic. Due to insidious presentation and uncommon etiologies, establishing a proper diagnosis, and providing timely targeted treatment for patients with subacute and chronic meningitis remains a challenge for clinicians.

Locations and Predictive Factors of Hypertrophic Pachymeningitis in Otitis Media With Antineutrophil Cytoplasmic Antigen-Associated Vasculitis.

OBJECTIVE: To determine the locations and predictive factors of hypertrophic pachymeningitis (HP), a serious central complication of otitis media with antineutrophil cytoplasmic antigen-associated vasculitis (OMAAV). STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Thirty-six patients with OMAAV underwent contrast-enhanced magnetic resonance imaging (CE-MRI) of the brain, of whom 18 and 18 had or did not have HP, respectively. MAIN OUTCOME MEASURES: The location of HP lesions, which were detected as dural thickening on CE-MRI, was reviewed. Clinical characteristics, laboratory data, and audiometric data before treatment were correlated with the occurrence of HP. RESULTS: HP lesions were most frequently observed in the middle cranial fossa, followed by the internal auditory meatus, tentorium cerebelli, and posterior cranial fossa, which were adjacent to the middle ear primary lesion. Headache was more frequently observed in patients with HP than without HP. The neutrophil-to-lymphocyte ratio (NLR) of the HP (+) group (median, 3.91; interquartile range, 2.50-6.06) was higher than that of the HP (-) group (median, 2.40; interquartile range, 2.01-3.03). The area under the receiver operating characteristic curve for the NLR was 0.741. An NLR of 3.46 had the highest sensitivity (61.1%) and specificity (94.4%) for predicting HP. Other markers of systemic inflammation and comorbidities of systemic diseases were not different between the groups. CONCLUSIONS: HP may not be a systemic manifestation of OMAAV but may be related to local lesions in the adjacent brain. A high NLR may be a predictive factor for comorbid HP. Therefore, CE-MRI should be considered for patients with a high NLR.

Clinical, imaging features and treatment response of idiopathic hypertrophic pachymeningitis.

BACKGROUND: Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease that causes focal or diffuse thickening of the dura mater. However, longitudinal follow up studies are still lacking for these patients. OBJECTIVE: To investigate the clinical characteristics, neuroimaging findings, treatment response and outcome of IHP. METHOD: A retrospective case series of 30 patients admitted Beijing Tiantan Hospital were screened via Hospital Information System from January 1st, 2011, to January 31st, 2021. All patients' clinical symptoms, imaging, and treatment response were collected via a standardized form. We compared the effects of high-dose and low-dose corticosteroids on headache, impaired vision, and MRI remission during acute onset. The effects of different immunosuppressants on preventing relapses were also compared. RESULTS: Headache (93.3%) and multiple cranial neuropathy (66.7%) were the most common symptoms of IHP. Cerebral spinal fluid test showed that protein levels were elevated in 17 (56.7%) patients, and white blood cells were increased in seven patients. MRI demonstrated that diffuse (60%) and focal (40%) enhancement occurred in the dura mater, especially in the tentorium cerebellum (80%). High-dose and low-dose corticosteroids reduced headache and dural enhancement during the acute phase. The high dose corticosteroid significantly relieved the headache than the low dose group (p = 0.041). Patients treated with mycophenolate mofetil and cyclophosphamide might achieve longer remission (months, p = 0.428). CONCLUSION: Headache and multiple cranial neuropathy are the most common clinical manifestations of IHP. In this study, almost all patients had a good initial response to corticosteroid therapy during the acute phase. Mycophenolate mofetil and cyclosporine may be effective for preventing relapses.