Russell Bodies Research Articles

Mide adenokarsinomu ile ilişkili Russel body gastriti : Nadir bir olgu sunumu

Russel Body Gastritis (RBG) is a rare entity, the exact etiology of which is unknown. About 50 cases have been reported in literature so far. In literature, especially H. pylori infection, HIV, and multiple myeloma have been associated with diseases such as gastric adenocarcinoma and with alcohol use. RBG has rarely been reported to be associated with gastric adenocarcinoma. We will present the determination of Russell body gastritis in the control biopsy of a 60-year-old female patient diagnosed with stomach adenocarcinoma six months ago. This study aimed to present this rare case with literature review.

Should we perform cytokeratin immunostaining in cases of Russell body gastritis?

Should we perform cytokeratin immunostaining in cases of Russell body gastritis?

Rheumatoid Arthritis Morning Stiffness Is Associated With Synovial Fibrin and Neutrophils.

Morning stiffness is a hallmark symptom of rheumatoid arthritis (RA), but its etiology is poorly understood. This study was undertaken to determine whether any histologic features of synovium are associated with this symptom. Data on patient-reported morning stiffness duration and severity, and Disease Activity Score in 28 joints (DAS28) were collected from 176 patients with RA undergoing arthroplasty. Synovium was scored for 10 histopathologic features: synovial lining hyperplasia, lymphocytes, plasma cells, Russell bodies, binucleate plasma cells, fibrin, synovial giant cells, detritus, neutrophils, and mucin. Fibrinolysis of clots seeded with various cell types was measured in turbidimetric lysis assays. Stiffness severity and morning stiffness duration were both significantly associated with DAS28 (P = 0.0001 and P = 0.001, respectively). None of the synovial features examined were associated with patient-reported stiffness severity. The presence of neutrophils and fibrin in RA synovial tissue were significantly associated (P < 0.0001) with patient-reported morning stiffness of ≥1 hour, such that 73% of patients with both synovial fibrin and neutrophils reported morning stiffness of ≥1 hour. Further, neutrophils and fibrin deposits colocalized along the synovial lining. In in vitro analyses, fibrin clots seeded with necrotic neutrophils were more resistant to fibrinolysis than those seeded with living neutrophils or no cells (P = 0.008). DNase I treatment of necrotic neutrophils abrogated the delay in fibrinolysis. In RA, prolonged morning stiffness may be related to impaired fibrinolysis of neutrophil-enmeshed fibrin deposits along the synovial membrane. Our findings also suggest that morning stiffness severity and duration may reflect distinct pathophysiologic phenomena.

Histopathology of Post Kala-azar Dermal Leishmaniasis.

The various lesions seen in the clinical presentation of post kala-azar dermal leishmaniasis (PKDL) are reflected in the histopathology of the type of lesion biopsied. The cells that form the dermal infiltrate include lymphocytes, histiocytes, and plasma cells in varying proportions. The infiltrate, which is mild and confined to the superficial dermis in macular lesion becomes denser with the increasing severity of the lesion. Leishman–Donovan bodies (LDB) in general are rarely demonstrable in macules and somewhat infrequently in the rest, though at times they may be numerous; mucosal lesions offer a greater chance of visualizing LDB than biopsies from the skin. A characteristic histomorphology in nodules is prominent follicular plugging with a dense plasma cell-rich lymphohistiocytic dermal infiltrate that shows an abrupt cut-off in the lower dermis, an appearance highly suggestive of PKDL even in the absence of LDB. Russell bodies within plasma cells, vascular changes, and xanthoma-like hue have been seen in plaques from chronic PKDL. The histopathologic picture in some may also mimic that seen in tuberculoid and lepromatous leprosy, and other granulomatous dermatoses. In contrast to Indian PKDL, epithelioid cell granulomas with giant cells are more common in African PKDL, and vascular changes are rare though neuritis showing LDB has been described.

Age Related Changes in the Cellular Population and Lymphoid Tissue in the Harderian Gland of Turkey

The aim of the current investigation was to identify the cellular changes of the cell populations positioned within the connective tissue of the harderian glands in Turkeys at different ages extended from one day hatched offspring till the age of seven months. To conduct such research, 50 specimens of harderian glands from the above-dated ages of turkey were collected. Post euthanasia and dissection of the birds, specimens were cut and fixed in 10% neutral buffered formalin and Bouin’s solution. The subsequent routine histological technique was made on these specimens such as dehydration, clearing, paraffin embedding, block preparation, and sectioning. Tissue sections were obtained and stained with different stains such as hematoxylin-eosin, Masson’s trichrome, periodic acid-Schiff (PAS) and Alcian blue (AB). Microscopic findings showed connective tissue of the harderian gland was populated by three types of cells that were heterophils, lymphocytes and plasma cells. The incidence of each of these cells was changed with advance of the age. During the growth of the birds, a gradual shift was taking place in the domination of these three cell types. The lymphoid nodules were detected only in 40 days old poults, whilst in adult birds the harderian glands were contained the largest number of mature plasma cells. Some plasma cells enclosed distinctly Russell bodies with different sizes and shapes. In conclusion, 4 weeks aged turkeys developed lymphoid tissue in the harderian gland which is a critical age for vaccination in this avian species.

A Diagnosis to Wrestle With: Intraventricular Angiomatoid Fibrous Histiocytoma

Abstract We report a case of a 15-year-old boy who presented with an intraventricular angiomatoid fibrous histiocytoma. The patient suffered a concussion during a wrestling match and underwent a head and spine CT, which prompted an MRI. MRI revealed an enhancing 2.3-cm mass in the posterior horn of the right lateral ventricle with no hydrocephalus. The patient subsequently admitted to having had headaches with associated appetite suppression but no visual problems or weakness. The tumor was resected. It was composed of sheets and cords of histiocytoid cells with focal pleomorphism. Interspersed throughout was a robust lymphoplasmacytic infiltration with Russell bodies and lymphoid follicles. The histiocytoid neoplastic cells were desmin, EMA, and sparsely S100 protein positive. They were negative for CD31, CD34, Cam 5.2, CD163, CD1a, HMB45, Melan-A, progesterone, and ERG. The abundant plasma cell population was confirmed by CD138 reactivity and was polyclonal by kappa and lambda immunohistochemistry. Fluorescence in situ hybridization revealed an EWSR gene rearrangement. The patient was radiographically free of disease 3 years after surgery with headaches as residual sequela. The differential diagnosis for this case included a lymphoplasmacyte-rich meningioma or a plasmacytoma. The EWSR rearrangement made both of these unlikely. The polyclonal nature of the plasma cell component and the age of the patient also argued against plasmacytoma. A literature search revealed one other ventricular-based angiomatoid fibrous histiocytoma in a pediatric patient.

Russell-Like Bodies in Plant Seeds Share Common Features With Prolamin Bodies and Occur Upon Recombinant Protein Production.

Although many recombinant proteins have been produced in seeds at high yields without adverse effects on the plant, endoplasmic reticulum (ER) stress and aberrant localization of endogenous or recombinant proteins have also been reported. The production of murine interleukin-10 (mIL-10) in Arabidopsis thaliana seeds resulted in the de novo formation of ER-derived structures containing a large fraction of the recombinant protein in an insoluble form. These bodies containing mIL-10 were morphologically similar to Russell bodies found in mammalian cells. We confirmed that the compartment containing mIL-10 was enclosed by ER membranes, and 3D electron microscopy revealed that these structures have a spheroidal shape. Another feature shared with Russell bodies is the continued viability of the cells that generate these organelles. To investigate similarities in the formation of Russell-like bodies and the plant-specific protein bodies formed by prolamins in cereal seeds, we crossed plants containing ectopic ER-derived prolamin protein bodies with a line accumulating mIL-10 in Russell-like bodies. This resulted in seeds containing only one population of protein bodies in which mIL-10 inclusions formed a central core surrounded by the prolamin-containing matrix, suggesting that both types of protein aggregates are together removed from the secretory pathway by a common mechanism. We propose that, like mammalian cells, plant cells are able to form Russell-like bodies as a self-protection mechanism, when they are overloaded with a partially transport-incompetent protein, and we discuss the resulting challenges for recombinant protein production.

Histomorphological Study of Harderian Gland in Japanese Quail (Coturnix coturnix japonica)

Harderian gland acts as a peripheral lymphoid organ in avian species, which involved in activation and terminal differentiation of B lymphocytes and proliferation of plasma cells. The Harderian gland samples were collected from different age groups of Japanese quail birds and the tissues were processed through routine techniques for studying the histomorphological details of the organ. The Harderian gland was a compound tubulo acinar gland comprised of secretory acini in the periphery of the gland with ductular system composed of tertiary, secondary and primary ducts. The interstitial space of the gland contained large population of age dependent plasma cells, lymphocytes, Russell bodies, myoepithelial cells and mast cells. The number of plasma cells and Russell bodies increased from first week of age to adult age, with maximum population observed between 2nd and 3rd months of age in Japanese quail.

Russell Bodies and Russell Body Inflammatory Polyp in the Colorectum: A Review of Clinicopathologic Features.

Colorectal mucosa with Russell bodies is a reactive inflammatory lesion composed of mature plasma cells, known as Mott cells which contain multiple intracytoplasmic eosinophilic globules. To the best of our knowledge, 3 case reports of colorectal Russell body containing lesions have been reported in the English literature (searched from 1980 to date), including just one case report of Colonic Russell body inflammatory polyp. Their importance lies in being aware of this unusual entity, recognizing it as well as the clinical scenario in which this typically arises and differentiating it from its malignant mimics that come in the histologic differential. This review discusses the clinical and endoscopic presentation, histopathologic features, ancillary studies, pathogenesis, differential diagnosis, prognosis, and treatment of this rare lesion.

Castleman Disease and Rosai-Dorfman Disease.

This chapter describes the main features of two different diseases, Castleman Disease (CD) and Rosai-Dorfman Disease (RDD). Castleman disease (CD) is a clinical and histopathologically heterogeneous lymphoproliferative disorder that encompasses at least three distinct entities with some common overlapping morphological features: Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD and Multicentric CD. The most important feature of HVCD is the presence of abnormal germinal centers with hyaline-vascular transformation, sometimes showing multiple germinal centers within a single reactive lymphoid follicle, this outlining HVCD as a disorder of follicular dendritic cells. Unicentric and multicentric CD are, in contrast, lymphoproliferative lesions. Proinflammatory hypercytokinemia is an essential feature of multicentric CD, distinguished by a florid clinical presentation. Rosai-Dorfmann Disease is a histiocytic proliferative disorder diagnosed by the presence of tissue infiltration by S100-positive CD1a-negative histiocytes and plasma cell aggregates, often with Russell bodies. A typical, though not specific, characteristic of the disease is emperipolesis. Initially considered to be an inflammatory/reactive condition, molecular studies suggest that at least some cases of RDD could be considered as a low-grade histiocytic neoplastic process.

Ultrastructure of plasma cells in harderian gland of laying hens.

Ultrastructure of plasma cells in Harderian gland was investigated using the transmission electron microscopy. For this research, we examined the glands of 32 laying hens collected at 1, 7, 20 and 40days and 4, 6, 8 and 12months of the birds' ages. The research showed that the stroma of the gland contains a large number of lymphocytes and plasma cells. Most of the plasma cells are mature, but morphologically do not show productive activity. Only some individual plasma cells, situated under the secretory epithelium of primary and secondary ducts, have extremely dilated cisternae of rough endoplasmic reticulum which contain moderately dense, granular material. The morphology of these cells indicates that they are in active stage of immunoglobulin production. Also, we identified plasma cells with two types of Russell bodies. One type of these bodies was small, round or oval, while the other had irregular, angular shape. It was noted that one plasma cell never contains both type of Russell bodies at the same time. These cells were often affected by apoptosis. Among them, in deeper part of the stroma, were situated the small plasmablast cells.

Kappa restricted Russell body gastroenteritis in two pediatric patients

Kappa restricted Russell body gastroenteritis in two pediatric patients

Rare Gastric Lesions Associated with Helicobacter pylori Infection: A Histopathological Review.

Helicobacter pylori infection is associated with chronic gastritis, peptic ulcer disease, gastric adenocarcinoma, and mucosa-associated lymphoid tissue lymphoma. However, some rare gastric lesions exhibiting distinctive histological features may also be associated with H. pylori infection, including lymphocytic gastritis, granulomatous gastritis, Russell body gastritis, or crystal-storing histiocytosis. Although diverse factors can contribute to their development, there is convincing evidence that H. pylori infection may play a pathogenic role. These findings are mainly based on studies in patients with these lesions who exhibited clinical and histological improvements after H. pylori eradication therapy. Thus, H. pylori eradication therapy might be indicated in patients with no other underlying disease, particularly in countries with a high prevalence of H. pylori infection. This review describes the characteristic histological features of these rare lesions and evaluates the evidence regarding a causative role for H. pylori infection in their pathogenesis.

Glassy droplet inclusions within the cytoplasm of Kupffer cells: A novel ultrastructural feature for the diagnosis of pediatric autoimmune hepatitis

Since Kupffer cells/macrophages (KCs/MPs) may be involved in the pathogenesis of autoimmune hepatitis (AIH), this pioneer study was undertaken to evaluate KCs/MPs in pediatric AIH in transmission-electron microscope. MethodsUltrastructural analyses were performed using liver biopsies from 14 children with clinicopathologically diagnosed AIH. ResultsIn all AIH children, ultrastructural findings revealed changes in the cells lining sinusoidal vessels, especially KCs/MPs and endothelial cells. KCs/MPs showed increased phagocytic activity and damaged mitochondria, frequently with accompanying intense fibrosis. In 10/14 AIH patients, the cytoplasm of sinusoidal KCs/MPs contained characteristic glassy droplet inclusions. They were round, sharply circumscribed, and contained homogeneous material and distinct translucent fields. Their ultrastructure was identical with the Russel bodies of plasma cells, which were also found in liver biopsies in the same patients. ConclusionUltrastructural identification of characteristic cytoplasmic droplets with glassy appearance in KCs/MPs, never before described in AIH, provides a useful novel morphological feature in the diagnosis of this disease.

A comparison of Mott cell morphology of three avian species. II. - Bad behavior by plasmacytes?

Mott cells are atypical plasmacytes recognized microscopically by endoplasmic reticulum (ER) distensions (Russell bodies) a result of retained secretory product (antibody). Originally associated with parasitism, they are observed in a broad spectrum of immunopathology, sometimes involving hypergammaglobulinemia. Few descriptions of Mott cells appear in avian literature. The purpose of the manuscript is to provide examples identified by light microscopy in three poultry species. Transmission electron micrographs (TEM) of plasmacytes from the turkey oviduct mucosa are included for comparison with Mott cell light microscopic images. Wright's stained blood and bone marrow from commercial and specific pathogen free (SPF) chickens, ducks, and turkeys are the sources. Mott cell positive samples commonly occurred with leukocytosis or leukemoid reactions, polymicrobial bacteremia, and fungemia. Atypical granulocytes and leukocytes regularly accompanied Mott cells. It is proposed that circulating Mott cells are “sentinels” indicative of stress, dyscrasia, and pathology. Moreover, Mott cells, like other atypia, complicate the interpretation of simple heterophil/lymphocyte (H/L) ratios. As Mott cells are defective plasmacytes these observations address hematology, immunology, pathology, and welfare issues.

THE POSSIBLE EFFECT OF OMEGA-3 FATTY ACIDS VERSUS INSULIN ON EXPRESSION OF ACTIVATED CASPASE-3 AND RUSSELL BODIES DEVELOPMENT IN BUCCAL

Background: Damage of tissue repair and dysfunction of the oral mucosa are usually associated with the diabetic condition. Apoptosis plays a functional role in limiting diabetic repair. Russell bodies, an uncommon finding that may be associated with chronic inflammatory conditions. Although insulin is the mainstay of diabetes treatment, it has prominent side effects. Episodes of severe hypoglycemia and increased mortality rate are associated with insulin therapy. Cod liver oil (CLO) is an important source of long-chain omega-3 (ω-3) fatty acids that have antioxidant and anti-inflammatory properties.Objective: The present study was designed to determine the adaptive apoptotic alterations accompanying diabetes in the buccal mucosa with the possibility of occurrence of the so-called Russell bodies and the possible role of long-chain ω-3 fatty acids versus insulin supplementation in enhancement of the buccal mucosa in streptozotocin (STZ)-induced diabetic rats.Design: Sixty adult male Swiss albino rats (200-250 gm) were selected for this study. The animals were randomly divided into four groups (fifteen rats each): Group I (Control group), Group II (Diabetic untreated group), Group III (Insulin treated group) and Group IV (Cod liver oil treated group). At the end of the experimental period (four weeks), the rats were sacrificed and the specimens were obtained from the mucosa of the cheek of both sides. The sections were examined histologically, immunohistochemically and histomorphometrically. Statistical analysis: Data obtained from histomorphometric analysis were statistically described in terms of mean ± standard deviation (± SD). Results: Histopathologic examination of Group I revealed the normal histological features of the buccal mucosa. In Group II several histological changes in the epithelial layer of the buccal mucosa were noticed. These changes include; atrophy in the epithelium, evidences of cells undergo degeneration, nuclear changes, ill-defined cell membrane, cytoplasmic vacuolations, thickening of stratum granulosum (hypergranulosis) which was accompanied by hyperkeratosis. Moreover, the lamina propria showed differential orientation and arrangement of the collagen fiber bundles, areas of degeneration and hyalinization in addition to fibroblasts that showed signs of degeneration. Furthermore, the lamina propria was strongly infiltrated by inflammatory cells and invaded by multiple Russell bodies scattered throughout the connective tissue (C.T) and dilated blood vessels (BVs); lined by swollen endothelial cells and engorged with red blood cells (RBCs) were observed. Some of these changes were still observed in Group III that received insulin treatment. Treatment with CLO in Group IV resulted in histological features resembling nearly those of the control group (Group I). The least immuno-expressions for caspase-3 and CD-138 were detected in Group I, followed by Group IV, then Group III and subsequently Group II. The histomorphometric analysis supported the previous results as Group I showed the highest mean epithelium thickness, followed by Group IV, then Group III and the least value was for Group II. On the other hand, Group I showed the least mean keratin thickness and the least mean area percentage of both caspase-3 and CD-138 immunoreactivities, followed by Group IV, then Group III and the highest values were for Group II. There was statistically highly significant difference between the studied groups.Conclusions: Diabetes has deleterious effect on the structure of the buccal mucosa. The raised levels of capase-3 in diabetic buccal mucosa are related to increased Russell bodies (Mott cells) formation. Insulin can’t completely inhibit the complications of diabetes. However, ω-3 fatty acids present in CLO can inhibit to a great extent the abnormalities caused by diabetes.

Russell body cervicitis: A case report and literature review

Russell bodies are prominent eosinophilic inclusions seen in the cytoplasm of plasma cells and sometimes outside the cell. There are very few publications available in the English literature on Russell Body Cervicitis. Our case is a 40 year old female patient with a positive screening test for HPV DNA. The cervical surface epithelium in biopsy specimens obtained from the cervix completely have eroded intense vesicular eccentric nuclei and large eosinophilic cytoplasm containing focal Russell Body. The infiltration of dense plasma cells were observed. Our case is the fourth case in the English literature. The case is rare and the etiology has not yet been elucidated.

Correlation Between Prognosis and Morphological Abnormalities in Multiple Myeloma

BackgroundMultiple myeloma shows various morphological abnormalities such as plasmablastic morphology, cytoplasmic granules, vacuolation, and nuclear atypia. Although plasablastic morphology has been revealed as a poor prognostic factor, little is known about clinical significance of other abnormalities. Therefore we investigated a correlation between morphological abnormalities and clinical outcome.MethodsPatient SelectionA total of 76 multiple myeloma patients who hospitalized in National Center for Global Health and Medicine (NCGM), Japan, between 2010 and 2015 were enrolled in this study.Morphological EvaluationBone marrow smears were obtained from each patient before initiation of treatments. We evaluated Greipp's morphological classification and morphological abnormalities of myeloma cells including cytoplasmic granules, vacuolation, and nuclear atypia. We also evaluated morphological abnormalities of other hemocytes. All specimens were observed at 400x magnification with an optical microscope.Clinical InformationClinical information obtained from medical records of NCGM includes gender, age, overall survival, Durie-Salmon staging system (DS), international staging system (ISS), types of M protein and light chain. The results of G-banded chromosomal analysis and fluorescent in situ hybridization (FISH) analysis were also collected from medical records.Statistical AnalysisWe performed survival analysis by generalized Wilcoxon test with EZR (version 1.31).ResultsClinical DataOf all the patients, 46 (60.5%) are male. Mean age of the patients is 63.5 years old.Morphological Abnormality of Myeloma CellsAccording to Greipp's morphological classification, we identified 13 cases as mature type (17.1%), 28 cases as intermediate type (36.8%), 31 cases as immature type (40.8%), 4 cases as plasmablastic type (5.3%). We found cytoplasmic granules in 20 cases (26.3%), vacuolation in 8 cases (10.5%), atypical myeloma cells in 54 cases (71.1%), Russell bodies in 5 cases (6.6%), and Dutcher bodies in 3 cases (3.9%).Morphological Abnormality of Other HematocytesAs for myeloid cells, we found Auer rods in 1 case (1.3%), pseudo-Pelger-Huet anomaly in 2 cases (2.6%), neutrophils with ring-shaped nuclei in 2 cases (2.6%), megakaryocytes with multiple separated nuculeus in 3 cases (3.9%).Statistical AnalysisWe revealed that overall survival of granular group is significantly longer than non-granular group (p=0.04, Fig.1). Median overall survivals are 66.2 months (granular group) vs 54.5 months (non-granular group). However, there are no significance of vacuolation and nuclear atypia on overall survival.ConclusionsA variety of morphological abnormalities were identified in multiple myeloma. We found that presence of cytoplasmic granules in myeloma cells correlates with good prognosis.DiscussionPrevious reports have shown that cytoplasmic granules of myeloma cells contain immunoglobulin however pathophysiological roles of them remain unclear. Further studies with molecular and cellular analysis should be carried out to elucidate roles of cytoplasmic granules. [Display omitted] DisclosuresHagiwara:Takeda: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees.

Non-H. pylori-Associated Russell Body Gastritis

Case: A 62-year-old woman presented with weakness and rectal bleeding. She was found to be anemic. She denied dysphagia, hematemesis or prior history of gastrointestinal bleeding. She underwent an upper endoscopy that showed mild antral erythema. Biopsies were obtained that showed numerous Mott cells in the lamina propria. These cells have large eosinophilic cytoplasmic inclusions indicating Russell Body Gastritis. Plasma cells, lymphocytes, and neutrophils were also present in the superficial lamina propria. Of note, no Helicobacter pylori bacteria were seen. In situ hybridization kappa and lambda chains showed a polyclonal pattern with a kappa/lambda ratio of 2-3:1. There was no intestinal metaplasia, cellular atypia or evidence of carcinoma. Duodenal biopsies were normal. Discussion: We are reporting this case not only due to the rarity of the diagnosis, but also because in this case of Russell body gastritis, H. Pylori was not identified. A majority of the cases of Russell body gastritis are associated with H. Pylori. Other conditions associated with Russell body gastritis include certain autoimmune conditions like Hashimoto's thyroiditis, rheumatoid arthritis, and also malignancies like MALT lymphoma and plasma cell tumors. Proper identification of Russell Body Gastritis is important for early recognition of these conditions, and also for exclusion of malignancy, which can result in significant reduction in morbidity and mortality.

IgG4-related meningeal disease

Objective To explore the clinical and pathological characteristics, diagnosis and differential diagnosis of IgG4-related meningeal disease. Methods and Results A 49-year-old male patient suffered from headache for nearly 2 years and the symptom was aggravated progressively for over one month. MRI revealed space-occupying lesion in left parietal lobe, with irregular signal and clear borderline. Contrast-enhanced MRI showed homogeneous enhancement, and obvious meningeal thicking just like dural tail sign. The patient underwent operation, and the lesion was totally removed. Histologically, it showed a large amount of hyperplastic collagen fibrous tissue with inflammatory cells, including a large number of plasmocytes, as well as scattered lymphocytes and a small number of eosinophilic granulocytes. Russell bodies which were homogenously positive for eosin could be seen in some plasmocytes. Focal necrosis, and proliferation of interstitial fibroblasts and small vessels were found. The lesion had no capsules and invaded surrounding tissues. Immunohistochemical staining showed the plasmocytes were diffusely positive for IgG and IgG4 (> 60%), and were positive for CD38 and CD138 on the membrane. Lymphocytes were positive for CD3, CD4 or CD20 on the membrane. The serum IgG4 level was 1.05 g/L. Final pathological diagnosis was IgG4-related meningeal disease in left parietal lobe. After operation, the patient received anti-infectious, anti-epileptic and nutrition support treatment, and the symptoms were markedly improved. The patient was discharged after 26 d, but was lost to follow-up. Conclusions IgG4-related meningeal disease is a rare disease. Due to the atypical imaging features, it may be difficult to differentiate IgG4-related meningeal disease from other diseases with prominent inflammatory cells and stromal fibrosis. The elevated serum IgG4 level may provide diagnostic cues. However, a definite diagnosis depends on characteristic histological and immunohistochemical features. DOI: 10.3969/j.issn.1672-6731.2016.03.010