Wegener granulomatosis: MR imaging findings in brain and meninges

PURPOSE: To determine the spectrum of intracranial magnetic resonance (MR) imaging appearances of Wegener granulomatosis. MATERIALS AND METHODS: MR imaging studies in 19 patients with Wegener granulomatosis and possible central nervous system involvement were reviewed by two neuroradiologists. Intermediate-weighted and T2-weighted fast spin-echo MR images of the brain had been acquired in all patients, and spin-echo T1-weighted nonenhanced and gadolinium-enhanced images had been acquired in 18 patients. RESULTS: MR imaging findings included diffuse linear dural thickening and enhancement (n = 6); focal dural thickening and enhancement contiguous with orbital, nasal, or paranasal disease (n = 5); infarcts (n = 4); nonspecific white matter areas of high signal intensity on intermediate-weighted and T2-weighted images (n = 10); enlarged pituitary gland with infundibular thickening and enhancement (n = 2); a discrete cerebellar lesion that was probably granulomatous in origin (n = 1); and cerebral (n = 8) and cerebellar atrophy (n = 2). CONCLUSION: MR imaging demonstrated the wide spectrum of findings of central nervous system involvement in patients with Wegener granulomatosis and was particularly useful for the evaluation of direct intracranial spread from orbital, nasal, or paranasal disease.     

MR imaging findings of spinal dural involvement with Wegener granulomatosis

Involvement of the brain and meninges is rare in cases of Wegener granulomatosis, occurring in 2% to 8% of cases. Meningeal involvement in association with Wegener granulomatosis has scarcely been reported as being confined to the dura mater of brain on images and is thought to represent granulomatous infiltration. There are a few reported cases of Wegener granulomatosis that document involvement of dura at the level of the spinal cord. We present the case of a 52-year-old man with Wegener granulomatosis involving the cervical spinal dura and include detailed MR imaging findings.     

Wegener granulomatosis: CT features of parenchymal lung disease

Chest CT from eight patients with pulmonary Wegener granulomatosis were reviewed. The CT features of parenchymal lung involvement included multiple nodules or masses (seven of eight; 88%), ranging in size from 0.3 to 5.0 cm. Lung nodules demonstrated distinct feeding vessels in seven of eight patients (88%); and lesions similar to pulmonary infarcts, i.e., peripheral wedge-shaped lesions abutting the pleura, were identified in seven cases (88%). Scarring, spiculation, and pleural tags emanating from pulmonary nodules were prominent features (seven of eight; 88%). Other findings included cavitation (four of eight; 50%), air bronchograms through nodules (two of eight; 25%), and pleural effusions (two of eight; 25%). One patient on immunosuppressant therapy also demonstrated scattered ground glass infiltrates due to complicating pneumocystis pneumonia. In comparison to corresponding conventional chest radiography, CT examinations revealed more parenchymal lesions in five of eight cases (63%) and bilateral disease instead of unilateral disease in one of eight cases (13%). Feeding vessels were only identified on CT. Wegener granulomatosis is a primary necrotizing granulomatous vasculitis, and when it affects the lung it demonstrates CT features that are similar to other vessel-related disorders of the lung such as septic emboli, pulmonary infarcts, and tumor emboli of hematogenous metastases. We believe that these CT features--nodules with feeding vessels and wedge-shaped lesions abutting the pleura--are related to the necrotizing angiitis that affects pulmonary arteries and veins in this disease.     

Wegener granulomatosis of the colon: CT and histologic correlation

Wegener granulomatosis is a systemic vasculitis with distinct clinical and histologic features often characterized with involvement of the upper airway, lung, and kidneys. The reported incidence of gastrointestinal involvement is relatively rare, with histologic confirmation often lacking. We report a case in which gastrointestinal involvement was the principal feature, with CT and histologic correlation.     

MR imaging of cardiac mass in Wegener granulomatosis

Wegener granulomatosis is a disorder of unknown etiology characterized by a necrotizing granulomatous vasculitis that primarily affects the paranasal sinuses, lungs, and kidneys. It is usually a disease of adults with infrequent cardiac involvement. We report the unusual presentation of this disorder in an adolescent with a cardiac mass. The findings on two-dimensional echocardiography and MR imaging are discussed.     

鼻部韦格纳肉芽肿的CT诊断

目的：探讨鼻部韦格纳肉芽肿的CT表现及其诊断价值。方法：回顾性分析8例经活检或术后病理证实的鼻部韦格纳肉芽肿，均经过CT检查，主要临床表现包括慢性鼻窦炎症状、鼻粘膜干燥结痂和鼻出血。结果：3例韦格纳肉芽肿局限于鼻部，其余5例为全身性韦格纳肉芽肿。CT表现：①鼻窦炎表现。②鼻腔内充以软组织影，鼻甲、鼻中隔破坏。③早期上颌窦内壁破坏，晚期鼻窦骨质增生、硬化，窦壁增厚，出现“双线”征。④2例随访观察鼻甲、鼻中隔破坏较前明显，4例鼻甲、鼻中隔改变未见加重，6例上颌窦壁骨质明显增厚，出现不规则“双线”征。窦腔变小。结论：鼻甲、鼻中隔破坏。鼻腔充以软组织影，鼻窦壁“双线”征是诊断本病有价值的征象；CT可准确显示鼻部韦格纳肉芽肿侵及范围；也能够帮助与其他病变进行鉴别。     

MR Imaging in Wegener granulomatosis of the spinal cord

Wegener granulomatosis is a multisystemic disorder characterized by necrotizing vasculitis that primarily involves the respiratory tract. The orbits, heart, skin, joints, and nervous system are frequently involved. We describe the MR imaging findings of Wegener granulomatosis in the cervical spine and correlate them with the histopathologic features. MR imaging showed epidural liquid masses surrounded by granulomatous masses that compressed the cervical myelin from behind.     

Meningioangiomatosis: CT and MR findings.

Meningioangiomatosis (MA) is a rare hamartomatous lesion of the cerebral cortex; to date only 18 cases with imaging findings have been reported in the English literature. The origin of MA is probably malformative, with possible association with neurofibromatosis. These lesions frequently cause seizures in young patients. We report two new cases seen at our institution and present their CT and MR findings clearly illustrating MA cortex infiltration. Gd-DTPA used in one of the two cases failed to cause enhancement.     

Kimura disease: CT and MR findings.

The lesions of Kimura disease showed slightly high and very high intensity on T2-weighted MR, and low and intermediate intensity, respectively, on T1-weighted images. The degree of enhancement also differed between the two cases. These discrepancies may be attributable to differing degrees of fibrosis and vascular proliferation.     

Kikuchi disease: CT and MR findings.

Two cases of Kikuchi disease showed variable nodal enhancing features, including homogeneous enhancement and focal or extensive nodal necrosis on contrast-enhanced CT scans. At MR imaging, the area of central necrosis was isointense or hypointense on T1-weighted images and had a lower signal than nonnecrotic areas on T2-weighted images. The CT appearance of Kikuchi disease can be variable and can mimic not only lymphoma but various nodal diseases with nodal necrosis, including metastasis and tuberculosis.     

Retroperitoneal neurilemoma: CT and MR findings.

OBJECTIVE. Our objective was to characterize the CT and MR imaging findings of retroperitoneal neurilemomas. MATERIALS AND METHODS. We reviewed the CT and MR imaging findings in six women with retroperitoneal neurilemomas. RESULTS. Retroperitoneal neurilemomas were round, 5-13 cm in diameter, and located in the presacral pelvic retroperitoneum in four patients and adjacent to the kidney in two patients. CT findings of the tumors were well-demarcated round masses showing prominent cystic changes and oriented in a somewhat radial fashion. Medium and heavily T2-weighted MR images showed high-intensity necrotic areas and nonnecrotic areas of various signal intensity. CONCLUSION. CT findings of a round mass with prominent cystic degeneration, along with certain MR imaging characteristics, may be helpful in the preoperative diagnosis of retroperitoneal neurilemomas.     

Neonatal hypoglycemia: CT and MR findings.

A case of neonatal hypoglycemia with extensive occipital cortical loss is presented. Imaging studies revealed a predominance of brain parenchymal loss in the occipital lobes bilaterally with nearly complete absence of cortex in the posterior parietal and occipital regions and generalized thinning of the cortex throughout the brain.     

Glioependymal cysts: CT and MR findings.

Four patients with intracranial glioependymal cysts were evaluated in our institution in the last 7 years. All underwent surgical drainage and biopsy of the cyst wall. Cranial CT revealed a uniformly hypodense lesion with no contrast enhancement in all cases. Magnetic resonance imaging studies revealed a well defined cyst that was isointense to CSF on T1-weighted images and iso- or mildly hyperintense to CSF on proton density and T2-weighted images. In one case, a fluid-fluid level was demonstrated within the cavity, indicating the presence of fluid with a high protein content. A diagnosis of glioependymal cysts can be suggested based on CSF-like intensity patterns on T1-weighted images and iso- or mild hyperintensity on T2-weighted images.     

Intracerebral schwannoma: CT and MR findings.

A case of intracerebral schwannoma in a 19-year-old man is reported. CT and MR findings were nonspecific, suggesting a primary glioma. The histologic features were of schwannoma.     

Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation

Wegener granulomatosis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis. However, chest computed tomography (CT) has superior sensitivity and specificity for evaluation of the airways, lung parenchyma, and mediastinum, particularly with the use of multiplanar reformatted and three-dimensional images. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually characterized by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusions are the most common manifestation of pleural disease and can result from primary involvement or be secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with Wegener granulomatosis.     

Cerebellomedullary ganglioglioma: CT and MR findings.

We present a case of cerebellomedullary ganglioglioma in a young child with indolent clinical symptoms. CT demonstrated a region of hypodensity with central contrast enhancement in the right cerebellar hemisphere and the inferior peduncle. On MR the lesion was iosintense on T1-weighted images with fairly homogeneous enhancement with gadolinium, and of high signal intensity on T2-weighted images.     

Elastofibroma: CT and MR findings

Two cases of elastofibroma of the chest wall are reported. Each patient was investigated with CT and, in 1 case, MR imaging was also performed. Elastofibroma appears like a mass in the subscapular region and often presents problems of differential diagnosis. The contribution of MR imaging is reported.     

Chest radiograph in lymphomatoid granulomatosis: comparison with Wegener granulomatosis

A form of angiitis and granulomatosis principally involving the lung was identified as a variant form of Wegener granulomatosis only 15 years ago. Recent experience indicates that the most common form of pulmonary angiitis and granulomatosis is lymphomatoid granulomatosis. Chest radiographs of 16 patients with this condition were reviewed and the findings compared with those of Wegener granulomatosis. The frequency and distribution of nodular masses, cavitation, and migratory lesions were similar in the two disorders, but reticulonodular infiltrates occurred only in lymphomatoid granulomatosis. The study suggests that lymphomatoid granulomatosis and Wegener granulomatosis occasionally can be distinguished by radiographic criteria. Although radiologic patterns will suggest the diagnosis of pulmonary angiitis and granulomatosis, the specific diagnosis of lymphomatoid granulomatosis must rely on clinical, immunologic, and pathologic evidence. Accurate differentiation is essential since treatment of Wegener granulomatosis with cyclophosphamide is highly effective while treatment of lymphomatoid granulomatosis is infrequently successful.