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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]

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]

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]

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Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic inflammatory disease, primarily involving the upper and lower respiratory tract and kidneys. It is a relatively uncommon condition, characterized by necrotizing granulomatous vasculitis of small- and medium-sized vessels and the presence of anti-neutrophil cytoplasmic antibodies in the serum as defined in the Chapel Hill Consensus Conference 2012. GPA presents a wide spectrum of manifestations and remains one of the most challenging diagnostic dilemmas in clinical medicine. From common respiratory and neurological symptoms to infrequent cardiac complications, this fatal systemic illness is difficult to distinguish from infectious etiologies, and it is often mistaken for an isolated complaint. We report a case of granulomatosis with polyangiitis in a patient with persistent frontal headache, for a very long time considered as migraine versus tension-type headache. [ABSTRACT FROM AUTHOR]

Highlights • Pachymeningitis can cause optic neuropathy as onset symptom. • A patient with extra-pulmonary TB presented with optic neuropathy due to pachymeningitis of the orbit. • Worsening on anti-TB treatment with recovery on steroids suggests that the pachymeningitis was due not to primary TB infection but to antiphospholipid syndrome secondary to the TB. • Antiphospholipid antibodies may provoke inflammatory changes in dura microvessels. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA) is a rare immune-mediated disease characterized by granulomatous inflammation involving upper and lower respiratory tract, kidneys and peripheral nervous system. However, central nervous system involvement is uncommon and frequently refractory to classical therapy. Rituximab has emerged as promising alternative, but published reports are scarce. We report a case of pachymeningitis and cerebral granuloma in a patient with a history of severe generalized GPA, treated with rituximab. This case illustrates the complexity of the management of neurologic manifestations and provides insight into the potential utility of rituximab in this condition. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4 (IgG4)-related disease is a well-known disorder characterized by an inflammatory reaction with an increase in the number of IgG4-positive plasma cells associated with sclerosis. IgG4-related disease often affects the dura mater with a pattern of diffuse thickening when the central nervous system is involved. However, some nodular dural thickening requires discrimination from tumors because of obviously different treatment options. We report of a case of IgG4-related disease with tumefactive dural involvement. [ABSTRACT FROM AUTHOR]

Intracranial dural arteriovenous fistulas (DAVFs) are abnormal arteriovenous communications within the dura locating near a major venous sinus and are supplied by pachymeningeal arteries. DAVFs represent 10–15% of all intracranial arteriovenous malformations. The natural history and clinical manifestations are determined by location of the DAVFs and their angioarchitecture. Aggressive DAVF is usually associated with leptomeningeal venous drains or reflux. It may present with hemorrhagic or nonhemorrhagic stroke. The goal of embolization of DAVFs is total fistula occlusion without interfering with the normal dura–venous drains. Embolization can be performed by transarterial and/or transvenous routes or direct puncture of affected dural sinus. Selection of embolic materials depends on access route and angioarchitecture of the fistula. With the involution of endovascular devices, embolic materials, and high-quality angiography, endovascular embolization of DAVFs has been proved a safe and effective method of treating these complex cerebrovascular lesions. [ABSTRACT FROM AUTHOR]

Histoplasma capsulatum infection is fairly common in the United States in areas endemic to infected bats and birds. For most individuals, the illness is minor and self-limited; however, immunocompromised individuals have a significant risk of developing disseminated disease. Central nervous system (CNS) infection is recognizable in 5% to 10% of individuals with disseminated disease, but it is rare in individuals with no other evidence of dissemination. The case described in this report is unusual because an immunocompetent individual developed CNS histoplasmosis that presented with neurological symptoms of headaches and dizziness. Subsequent investigation yielded a diagnosis of pachymeningitis. The pertinent medical literature is reviewed, including reported CNS histoplasmosis cases that utilize the newer antifungal medications and steroid-sparing therapies to manage pachymeningitis. [ABSTRACT FROM AUTHOR]

Cranial vault involvement in primary lymphoma is extremely rare in immunocompetent subjects. However, it should be considered as a differential diagnosis in the presence of a lesion involving all three compartments of the cranial vault, including the scalp, skull, and pachymeninges. We report a case of primary cranial vault lymphoma involving all three compartments of the cranial vault in an immunocompetent patient. [ABSTRACT FROM AUTHOR]

We report the case of a 84-year-old man, with history of rheumatoid arthritis, admitted the Hospital for a fall and complaining of dysaesthesia and pain located to the cervical spine and arms. Within a few hours after admission, fever and acute, progressive, ascendant quadriplegia became evident. Magnetic resonance imaging (MRI) of cervical spine showed spinal canal stenosis between C4-C6 with spinal cord compression. Hemocultures resulted positive for Staphylococcus aureus. The clinical picture rapidly evolved to sepsis with a fatal multi-organ failure. An autopsy found a osteosclerosis narrowing the neurocanal at the level of C3-C6, and recent cervical medulla infarction. A histological exam revealed the presence of a suppurative pachymeningitis with local phenomenas of periradiculitis, vasculitis and thrombosis of the anterior medullar artery, associated with coagulative necrosis of the neural tissue. [ABSTRACT FROM AUTHOR]

Abstract: Wegener''s granulomatosis (WG) classically affects the upper and lower respiratory tracts as well as the renal system, while central nervous system (CNS) involvement is rare. We report a 17-year-old patient with WG who presented with a 2-month history of meningeal signs, left Horner''s syndrome, multiple cranial neuropathies and a left middle ear effusion. MRI demonstrated left middle ear and mastoid effusions, pachymeningitis with diffuse left temporal dural enhancement, and right sinus destructive changes. In this report, we will review diagnostic and therapeutic approaches to Wegener''s granulomatosis, with a particular emphasis on CNS manifestations and the adolescent population. [Copyright &y& Elsevier]

Purpose: To evaluate intraoperative effectiveness and postoperative outcome of intracameral, preservative-free triamcinolone acetonide in pediatric cataract surgery. Methods: A prospective, interventional, observational case series was performed in children undergoing congenital cataract surgery. Three standardized applications of triamcinolone acetonide were used. Postoperatively, IOP, pachymetry, glaucoma, posterior synechiae, cell deposits, and posterior capsule opacification were studied at 1 week, 1 month, and 12 months after surgery. Results: Mean age at the time of surgery was 14.8 ± 12.2 months. Of 43 eyes, 5 were kept aphakic and 38 received IOL implantation. After manual posterior continuous curvilinear capsulorhexis and the first injection of triamcinolone, the anterior vitreous face disturbance was visualized as free-floating vitreous strands going into the incision in 18.6% of eyes (8 of 43). Triamcinolone was injected a second time after anterior vitrectomy: residual vitreous strands were identified and removed in 7 of these 8 eyes. Triamcinolone was used for a third time after IOL implantation: residual vitreous strands were identified in 5.3% of eyes (2 of 38). Mean IOP was 12.2 ± 2.2 mm Hg preoperatively and was 13.6 ± 3.2 at the 1 week follow-up visit, 12.8 ± 2.8 at 1 month, and 12.4 ± 2.0 mm Hg at 12 months. Conclusions: Preservative-free triamcinolone acetonide improved visualization of the vitreous during pediatric cataract surgery, thereby ensuring thorough and complete anterior vitrectomy. IOP was not affected, and no adverse postoperative results were observed. [ABSTRACT FROM AUTHOR]

Abstract: Idiopathic hypertrophic cranial pachymeningitis (IHPM) is a clinicopathological entity characterized by thickening and fibrosis of the dura mater with resultant clinical symptoms. It is generally steroid responsive and has a tendency to remit and relapse. We present here two patients with IHPM with associated dural sinus occlusion and describe their clinicoradiological features and long-term course and outcome. [Copyright &y& Elsevier]

Diffuse pachymeningitis is an uncommon presentation of tuberculous meningitis (TBM). We present a 78-year-old woman patient with a 1-year history of progressive headache and MRI of the brain compatible with diffuse pachymeningitis. Without strong evidence to support a diagnosis, she subsequently underwent dural biopsy which revealed caseous granulomatous inflammation and was positive for Mycobacterium tuberculosis complex by PCR. The dura tissue culture subsequently confirmed the diagnosis of TBM. Successful treatment with antituberculous drugs and corticosteroid was observed without complications. Literature review on characteristics, diagnoses and treatment of central nervous system tuberculosis was also performed. [ABSTRACT FROM AUTHOR]

Abstract: We describe the clinical manifestations, radiological features and response to therapy of a 10-year-old child with idiopathic hypertrophic cranial pachymeningitis, a rare condition with potentially severe disabling neurological consequences. The child presented with progressive cranial polyneuropathies secondary to dural compression of the neural and vascular structures within the cavernous sinus. Corticosteroids and methotrexate proved effective in inducing near complete and sustained remission of the condition. To our knowledge, this is the first case reported of idiopathic hypertrophy cranial pachymeningitis in a child. The experience with oral methotrexate to treat this entity in a child has also never been reported. Idiopathic cranial hypertrophic pachymeningitis should be considered in children presenting with a progressive cranial polyneuropathies. [Copyright &y& Elsevier]

Abstract: Primary muscle tumors of the thyroid gland are exceedingly rare. We report on the case of a patient with primary leiomyosarcoma of the thyroid gland and review the literature. An 83-year-old woman complaining of neuropathic pain in her left arm and enlargement of her anterior neck underwent multiple surgical biopsies of the thyroid gland. The tumor was composed of interlacing fascicles of spindle-shaped cells that expressed smooth muscle actin and vimentin but were negative for cytokeratins and thyroglobulin. Ultrastructurally, bundles of myofilaments were present. Magnetic resonance imaging showed a thyroid tumor that directly extended to the adjacent vertebra with an associated pachymeningitis. The patient died 2 months after surgery. The diagnosis of primary leiomyosarcoma of the thyroid gland is difficult and requires numerous clinical, radiologic, and pathologic data. To our knowledge, this case is the first one with such a locoregional extension. [Copyright &y& Elsevier]

Cranial pachymeningitis is typically a diffuse granulomatous disease that involves the dura mater extensively without intra-axial involvement. We report a rare case of focal idiopathic hypertrophic pachymenin-goencephalitis (IHP) focally affecting the right parietal dura mater and adjacent parietal lobule. A 43-year-old male suffered from acute onset of rhythmic twitching over the left abdominal muscles that had persisted for more than 4 hours. Neurologic examination revealed mild weakness and impaired sensation in the left lower limb. Electroencephalography disclosed active focal spikes in the right parietal region and brain magnetic resonance imaging showed a well-enhanced lesion involving the right parietal lobe and its overlying dura mater. Surgical removal of the lesion revealed infiltration by abundant chronic inflammatory cells without granuloma formation, caseous necrosis or vasculitis. After surgery, the patient was treated with steroid, which was tapered off 5 months later. Serial magnetic resonance imaging follow-up revealed that the parenchymal mass and perifocal edema had completely disappeared 6 months after the operation. We suggest that early recognition of this rare IHP, together with proper surgical intervention and concomitant steroid therapy, may be beneficial for long-term remission. [Copyright &y& Elsevier]

Summary: Idiopathic hypertrophic cranial pachymeningitis is a rare condition. A case of idiopathic hypertrophic cranial pachymeningitis presenting as Tolosa-Hunt syndrome is being reported. The importance of neuroimaging in patients with suspected Tolosa-Hunt syndrome is discussed. Tolosa-Hunt syndrome might represent a focal manifestation of Idiopathic hypertrophic cranial pachymeningitis. Future studies are necessary to further clarify the relationship between these two conditions. [Copyright &y& Elsevier]

We describe a 52-year-old man with hypertrophic pachymeningitis (HP) who was both seropositive for antineutrophil cytoplasmatic antibodies (ANCA) against myeloperoxidase, and had an immunoglobulin G4 (IgG4) positive fibroinflammatory response in meningeal biopsy. HP is a chronic inflammatory thickening of the dura mater which typically presents with headache, cranial nerve dysfunction and other neurological deficits. While first-line treatment with corticosteroids is recommended, many patients relapse and need additional immunosuppression. One recently described etiology is IgG4-related disease and in a subgroup of idiopathic patients, evidence suggests a crucial role of ANCA. To our knowledge, the simultaneous occurrence of IgG4-related disease and ANCA has not been reported so far. This man suffered life-threatening disease progression despite the administration of high dose steroids, cyclophosphamide and azathioprine. Treatment with rituximab was initiated which led to disappearance of clinical symptoms and decrease of dural thickening within weeks. This patient presents a possible disease overlap of IgG4-related and ANCA-associated HP and illustrates the effectiveness of rituximab in refractory IgG4-related HP. [ABSTRACT FROM AUTHOR]

A 50-year-old woman with a history of synovitis-acne-pustulosis-hyperostosis osteomyelitis (SAPHO) syndrome was admitted for left unilateral neglect, dysarthria, and left hemiparesis. Brain MRI showed multiple infarctions in the territory of the right middle cerebral artery and gadolinium enhancement of the thickened frontotemporal dura mater on the right side. MR angiography showed significant narrowing of the cavernous segment of the right internal carotid artery. The right internal carotid artery stenosis was thought to originate from hypertrophic pachymeningitis associated with SAPHO syndrome. This is the first report of brain infarction due to internal carotid artery stenosis caused by hypertrophic pachymeningitis associated with SAPHO syndrome. [ABSTRACT FROM AUTHOR]

Abstract: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare inflammatory disease which is sometimes difficult to diagnose and can lead to misinterpretations of the clinical and imaging findings. The main clinical manifestations are headache, ataxia and cranial nerve palsy. In most of the reported patients continuous medication is needed to avoid disease recurrence. We present a female patient with an 8-year follow-up, no clinical regression and no need for any further medical treatment. Even though most patients with IHCP experience recurrence after diagnosis and initial treatment there were no clinical or imaging signs of relapse in our patient. Our patient is still not under any medical or surgical treatment due to the lack of any significant symptoms. [Copyright &y& Elsevier]

Abstract: Hypertrophic pachymeningitis is rarely observed in inflammatory bowel disease. We report a woman with ulcerative colitis whose biopsy-confirmed hypertrophic pachymeningitis was complicated by cerebral venous sinus thrombosis and intracranial hypertension and required ventriculostomy and steroid therapy. This report highlights the challenges of the diagnosis and management of hypertrophic pachymeningitis from an unusual primary cause. [ABSTRACT FROM AUTHOR]

Abstract: Hypertrophic pachymeningitis is a rare inflammatory process that causes thickening of the dura mater. Most cases are idiopathic, but it can result from many inflammatory and infective conditions. We present a case of pachymeningitis of the cavernous sinus, the aetiology of which may have been dental. [Copyright &y& Elsevier]