Cases reported "Wegener Granulomatosis"

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1/12. P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature.

    An autopsy case of hypertrophic pachymeningitis and multiple cranial neuropathies is reported. A 53-year-old woman with paraplegia and various neurological signs which developed over a 2 year period was diagnosed as having an epidural mass with thickened dura mater extending from the lower cervical to the thoracic spinal cord. In addition, bilateral episcleritis, blephaloptosis, and blindness of the right eye with various cranial nerve deficits were found to be caused by the mass lesions involving the paranasal sinuses, orbit, and the cavernous sinus. Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) was positive, but cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) was negative by enzyme-linked immunosorbent assay. The partially removed epidural mass with hypertrophied dura mater and biopsy of the paranasal lesions showed chronic granulomatous inflammation with vasculitis. The remaining lesions resolved with steroid therapy with remarkable neurological improvement. The positive p-ANCA test, paranasal involvement, the report of a similar histopathological case and a review of the literature on granulomatous pachymeningitis suggest the presence of p-ANCA-positive Wegener's granulomatosis with central nervous system involvement characterized by hypertrophic pachymeningitis and/or multiple cranial neuropathies.
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ranking = 1
keywords = pachymeningitis, meningitis
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2/12. Cerebral and oculorhinal manifestations of a limited form of Wegener's granulomatosis with c-ANCA-associated vasculitis.

    The authors report on cerebral and oculorhinal manifestations in a patient with a cytoplasmic pattern of antineutrophil cytoplasmic autoantibody (c-ANCA)-associated vasculitis. Recurrent tolosa-hunt syndrome, cavernous sinus syndrome, Raeder's paratrigeminal neuralgia, and seizures were the major clinical manifestations. brain MRI showed localized enhancing lesions initially in the cavernous sinus and later in the convexity pachymeninges. The lesions disappeared following 9 months of oral prednisolone (15 mg/day) and cyclophosphamide (100 mg/day) therapy. The presence of c-ANCA, demonstration of vasculitis, and depositions of immunoglobulin g (IgG) and fibrinogen in the vessel walls of pachymeninges of the patient confirmed an immune-mediated cause of the vasculitis. Cranial pathology without renal and pulmonary involvement suggests a variant of Wegener's granulomatosis, which is called the "limited" form of Wegener's granulomatosis. MRI, Raeder's paratrigeminal neuralgia, localized pachymeningitis.
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ranking = 0.14285714285714
keywords = pachymeningitis, meningitis
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3/12. wegener granulomatosis manifesting as meningitis. Case report.

    The authors describe a unique presentation of wegener granulomatosis (WG) manifesting predominantly as meningitis. magnetic resonance imaging demonstrated diffuse meningeal enhancement, including the pia mater, in a 28-year-old man with meningitis. A diagnosis of atypical WG was based on the findings of a dural biopsy sample and an elevated cytoplasmic antineutrophil cytoplasmic antibody (cANCA) titer, although the patient did not have any of the lesions common to WG. Immunosuppressive therapy was quite effective. With treatment, the meningeal enhancement resolved and the cANCA titer normalized. Meningeal granulomatosis as the sole lesion in WG has never been reported in the literature. This atypical course of WG should be noted.
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ranking = 0.0028526689222957
keywords = meningitis
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4/12. Intracranial granuloma and skull osteolysis: complication of a primary cutaneous cryptococcosis in a kidney transplant recipient.

    cryptococcosis is the third most common invasive fungal infection in organ transplant recipients after candidiasis and aspergillosis. It occurs almost exclusively in the late posttransplantation period (>6 months after the initiation of immunosuppression). Subclinical onset of meningitis is the usual clinical presentation. Despite initiation of therapy, the mortality rate associated with this infection in this patient population remains high. To the best of our knowledge, this report describes one of the first cases of a rare entity: a primary cutaneous cryptococcosis in a renal transplant recipient disclosed by skull osteomyelitis and pseudotumoral intracranial extension. Surgical debridement and azole antifungal therapy were performed. Ten months after the onset of treatment, the patient feels good, clinical examination findings are normal, and no sign of evolutive cryptococcosis is noted.
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ranking = 0.00047544482038262
keywords = meningitis
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5/12. central nervous system involvement in wegener granulomatosis.

    wegener granulomatosis (WG) is an antineutrophil cytoplasmic antibody (ANCA)-associated granulomatous vasculitis of small and medium-sized vessels. This vasculitis involves mainly the upper and lower respiratory tracts and kidneys, although WG may affect any organ. central nervous system (CNS) involvement is an uncommon manifestation of WG, reported in 7%-11% of patients. Three major mechanisms have been incriminated as causing CNS disease in WG: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma, and CNS vasculitis. Herein we describe 6 patients with WG-related CNS involvement, 2 of whom had chronic hypertrophic pachymeningitis, 3 with pituitary involvement, and 1 with cerebral vasculitis. CNS involvement was present at disease onset in 2 patients and occurred 5-18 years after WG diagnosis in the remaining 4. Based on these observations and a review of the literature, we discuss the pathogenic mechanisms, clinical features, imaging findings, treatment, and outcome of meningeal, pituitary, and vascular involvement, with an emphasis on differential diagnoses, prognosis, and therapeutic management of WG-related CNS involvement.
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ranking = 0.14285714285714
keywords = pachymeningitis, meningitis
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6/12. Bacterial meningitis associated with infliximab.

    We report an episode of bacterial meningitis in a 45 year-old woman, who was treated with infliximab for Wegener's granulomatosis. This patient presented with the classic clinical presentation of acute meningitis: the triad of fever, neck stiffness, and an altered mental state that appeared 6 months after the infliximab initiation. A computed tomographic (CT) scan of the head showed cerebral edema and streptococcus pneumoniae was isolated from blood and CSF cultures. Prompt diagnosis and early treatment improved the outcome of this patient.
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ranking = 0.0028526689222957
keywords = meningitis
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7/12. Subacute meningitis heralding a diffuse granulomatous angiitis: (Wegener's granulomatosis?).

    Subacute meningitis was the first manifestation of illness in a young man with multisystem disease involving lung, kidney, eye, and central nervous system. Necrotizing granulomatous angiitis was found in tissue obtained at thoracotomy and the diagnosis of Wegener's granulomatosis was suggested. This patient had an excellent functional recovery with cyclophosphamide and prednisone.
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ranking = 0.0023772241019131
keywords = meningitis
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8/12. Case report: Wegener's granulomatosis accompanied by communicating hydrocephalus.

    A case of Wegener's granulomatosis (WG) accompanied by communicating hydrocephalus is described. An elderly woman with rapidly progressive renal failure was referred to the authors' hospital. Renal histologic study showed necrotizing granulomatous glomerulonephritis with some multinucleated giant cells, which suggested a diagnosis of WG. After admission, a gait disturbance, incontinence, and dementia developed in the patient. Diagnostic procedures including lumbar puncture, computed tomography (CT), and scintigraphy showed findings compatible with communicating hydrocephalus with a normal cerebrospinal fluid (CSF) pressure. Removal of 20 mL of CSF led to a marked improvement in symptoms. Because the presence of subarachnoid hemorrhage, meningitis, and brain tumor was excluded, the final diagnosis was communicating hydrocephalus secondary to WG.
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ranking = 0.00047544482038262
keywords = meningitis
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9/12. Chronic meningitis with cranial neuropathies in Wegener's granulomatosis. Case report and review of the literature.

    We report the case of a 41-year-old man with chronic meningitis and cranial neuropathies in whom leptomeningeal biopsy demonstrated Wegener's granulomatosis. The literature on meningitis in this disease is reviewed. This case illustrates that Wegener's granulomatosis can cause chronic meningitis.
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ranking = 0.0033281137426783
keywords = meningitis
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10/12. An autopsy case of Wegener's granulomatosis with pachymeningitis.

    A 61-year-old woman presented with high fever, headache and left facial palsy with diplopia. Histopathological examination of the biopsied specimens taken from nasal mucosa and kidney revealed a granulomatous angiitis with giant cell infiltration. Ga-DTPA-enhanced magnetic resonance imaging (MRI) revealed a thickening of dura mater in the middle cranial fossa and tentorium cerebelli. The observed left facial and occulomotor palsy was considered to be caused by pachymeningitis associated with Wegener's granulomatosis (WG). cyclophosphamide combined with prednisolone effectively improved the symptoms. However, the patient died of acute interstitial pneumonitis, presumably caused by cyclophosphamide. The pathohistology obtained in the autopsy revealed a fibrous thickening of the dura mater in the left meningen with a segmental scarring of the arteries and a necrotizing arteritis in the kidney.
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ranking = 0.71428571428571
keywords = pachymeningitis, meningitis
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