Tumefactive Primary Central Nervous System Vasculitis: Dynamic Susceptibility Contrast Perfusion-Weighted Magnetic Resonance Imaging Findings With Histological Correlation.

Purpose: Primary central nervous system vasculitis (PCNSV) is a rare inflammatory disease affecting the central nervous system. In some cases, it presents with large, solitary lesion with extensive mass effect that mimic intracranial neoplasms. This condition results in a diagnostic confusion for neuroradiologists because the differentiation is almost impossible on conventional MRI sequences. The aim of this study is to reveal the significance of dynamic susceptibility contrast (DSC) perfusion-weighted imaging in differentiating of tumefactive PCNSV (t-PCNSV) lesions from intracranial neoplasms such as glio-blastomas and metastasis. Methods: In this retrospective study, DSC of 8 patients with biopsy-proven t-PCNSV has been compared with DSC obtained in 10 patients with glioblastoma, 10 patients with metastasis, who underwent surgery and histopathological confirmation. The ratio of relative cerebral blood volume (rrCBV) was calculated by rCBV (lesion) / rCBV (controlateral normal-appearing white matter) in the gadolinium-enhancing solid areas. Results: The mean rrCBV was 0.86±0.7 (range: 0.76-0.98) in the patients with t-PCNSV, 5,16±0.79 in patients with glioblastoma (range: 3.9-6.3), and 4.27±0.73 (range: 2.8-5.3) in patients with metastases. Conclusion: DSC-PWI seems to be useful in the diagnostic work-up of t-PCSNVs. A low rrCBV, i.e. a rCBV similar or lower to that of the contralateral normal white matter, seems to be consistent with the possibility of t-PCSNV.

Reduction of intratumoral brain perfusion by noninvasive transcranial electrical stimulation.

Malignant brain neoplasms have a poor prognosis despite aggressive treatments. Animal models and evidence from human bodily tumors reveal that sustained reduction in tumor perfusion via electrical stimulation promotes tumor necrosis, therefore possibly representing a therapeutic option for patients with brain tumors. Here, we demonstrate that transcranial electrical stimulation (tES) allows to safely and noninvasively reduce intratumoral perfusion in humans. Selected patients with glioblastoma or metastasis underwent tES, while perfusion was assessed using magnetic resonance imaging. Multichannel tES was applied according to personalized biophysical modeling, to maximize the induced electrical field over the solid tumor mass. All patients completed the study and tolerated the procedure without adverse effects, with tES selectively reducing the perfusion of the solid tumor. Results potentially open the door to noninvasive therapeutic interventions in brain tumors based on stand-alone tES or its combination with other available therapies.

Detection of calcifications in retinoblastoma using gradient-echo MR imaging sequences: comparative study between in vivo MR imaging and ex vivo high-resolution CT.

BACKGROUND AND PURPOSE: Intratumoral calcifications are very important in the diagnosis of retinoblastoma. Although CT is considered superior in detecting calcification, its ionizing radiation, especially in patients with hereditary retinoblastoma, should be avoided. The purpose of our study was to validate T2*WI for the detection of calcification in retinoblastoma with ex vivo CT as the criterion standard. MATERIALS AND METHODS: Twenty-two consecutive patients with retinoblastoma (mean age, 21 months; range, 1-71 months) with enucleation as primary treatment were imaged at 1.5T by using a dedicated surface coil. Signal-intensity voids indicating calcification on T2*WI were compared with ex vivo high-resolution CT, and correlation was scored by 2 independent observers as poor, good, or excellent. Other parameters included the shape and location of the signal-intensity voids. In 5 tumors, susceptibility-weighted images were evaluated. RESULTS: All calcifications visible on high-resolution CT could be matched with signal-intensity voids on T2*WI, and correlation was scored as excellent in 17 (77%) and good in 5 (23%) eyes. In total, 93% (25/27) of the signal-intensity voids inside the tumor correlated with calcifications compared with none (0/8) of the signal-intensity voids outside the tumor. Areas of nodular signal-intensity voids correlated with calcifications in 92% (24/26), and linear signal-intensity voids correlated with hemorrhage in 67% (6/9) of cases. The correlation of signal-intensity voids on SWI was better in 4 of 5 tumors compared with T2*WI. CONCLUSIONS: Signal-intensity voids on in vivo T2*WI correlate well with calcifications on ex vivo high-resolution CT in retinoblastoma. Gradient-echo sequences may be helpful in the differential diagnosis of retinoblastoma. The combination of funduscopy, sonography, and high-resolution MR imaging with gradient-echo sequences should become the standard diagnostic approach for retinoblastoma.

Fulminant intravascular lymphomatosis mimicking acute haemorrhagic leukoencephalopathy.

BACKGROUND: Intravascular lymphomatosis (IVL) is a rare non-Hodgkin's lymphoma, usually of B cell lineage, characterized by massive angiotropic growth. The clinical presentation of IVL may include changes in mental status, non-localizing neurological deficits, seizures, fever of unknown origin and skin changes. Because of its rarity and the absence of specific diagnostic procedures except for cerebral biopsy, diagnosis is often postmortem. Brain MRI usually shows non-specific abnormalities. The purpose of this case report is to increase the knowledge of clinical and neuroimaging features of IVL by describing the findings observed in a 71-year-old patient. CASE REPORT: A 71-year-old male was admitted for right hemiparesis, acute cognitive impairment and febricula. A bone marrow biopsy resulted normal. He then developed a rapid progressive impairment of his mental status and left hemisoma motor seizures. Brain CT and MRI were interpreted as consistent with acute haemorrhagic leukoencephalopathy (AHLE), including multiple areas of restricted diffusion without gadolinium enhancement and a small focal area of gadolinium enhancement in the left temporal lobe white matter. The patient died within a few days and the autopsy led to the diagnosis of IVL. CONCLUSION: IVL may present with a variety of clinical signs and symptoms, including stroke and hemiparesis. IVL may mimic AHLE at brain MRI. However, the evidence of multiple areas of restricted diffusion without gadolinium enhancement and of a small area of gadolinium enhancement could have led to the correct diagnosis. IVL should be added to the differential diagnosis of AHLE at brain MRI.

Calcifying Pseudoneoplasm of the Neuraxis. Two Case Reports and Review of CT and MR Findings.

Calcifying pseudoneoplasm is rarely encountered along the neuraxis, and only few cases have been reported to involve the spine. Its exact pathogenesis is unknown, and has been regarded as an unusual reactive process which must be differentiated from infection or malignancy. This rare entity carries a good prognosis after surgical resection. We report on the computed tomography and magnetic resonance imaging and diffusion-weighted features of calcifying pseudoneoplasm of the spine by describing two patients with involvement of the craniocervical junction, and thoracic spine.

Cranial osteolytic metastases as the only recurrence of gastric carcinoma.

The purpose of this case report is to increase the knowledge about bone metastatic pattern in gastric cancer. A 59-year-old man presented with headache three years after a total gastrectomy for signet-ring cell carcinoma. Head computed tomography and magnetic resonance imaging showed multiple osteolytic lesions of the cranial vault and base, consistent with metastatic or haematological disease. Bone scintigraphy confirmed areas of accumulation only in the skull. An extensive search didn't show any other tumor. Bone biopsy revealed metastatic signet-ring cell carcinoma. In gastric cancer, bone metastases are generally associated with metastases in lymph nodes, liver, and lung, and have a higher frequency in the thoracolumbar spine. However, cranial bone metastases presenting with headache may be the only manifestation of gastric cancer recurrence.

Supero-lateral orbitotomy for resection of spheno-orbital meningioma: a case report.

Spheno-orbital meningioma have traditionally been defined as secondary tumors of the orbit originating from the dura of the sphenoid wing bone. Nevertheless, pathologic findings reveal a distinct periorbital component as a defining feature of these lesions. These tumors are characterized by an intraosseous mass growth leading to a significant hyperostosis involving the sphenoid wing, the orbital roof, the lateral orbital wall and the middle fossa cranial base and to a thin, usually soft-tissue growth at the dura. We report here on the extension of the primary tumor into the orbital cavity and present the surgical approach performed.

Wernicke encephalopathy after gastrointestinal surgery for cancer: causes of diagnostic failure or delay.

Wernicke encephalopathy (WE) is a neurological emergency due to thiamine deficiency. We aimed to identify clinical course and causes of diagnostic delay or failure of WE in a group of patients who underwent surgery for gastrointestinal tumors. A retrospective review of clinical, laboratory, neuroimaging, and therapeutic features of 10 patients with WE following abdominal surgery for cancer was carried out. Four patients died; in these subjects, diagnosis was delayed and supplementation of vitamin was absent or likely inadequate. Diagnostic delay or failure was also related to the coexistence of several medical complications at presentation masking typical symptoms of WE. In the surviving patients, outcome was influenced by promptness and type of therapy. Postoperative abdominal bleeding and number of subsequent operations may also had an effect. Postsurgical patients with gastrointestinal tumors may develop a subtle WE. The number of subsequent operations and the severity of postoperative complications may increase the risk of unrecognized WE. The disease should be suspected in postsurgical patients who have unexpected mental status changes, even under prophylactic treatment with vitamins. We suggest that prophylaxis with high doses of thiamine should be undertaken in patients with gastrointestinal tumors before surgery.

Papillary glioneuronal tumor of the fourth ventricle: case report and review of the literature.

Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle is a relatively new entity recently described. Neuronal and mixed neuronal-glial tumours have been incorporated in the same category in the WHO Classification of Nervous System Tumours. This category comprises heterogeneous neoplasms composed of neuronal and glial cells with various grades of differentiation. We present a case of papillary glioneuronal tumor occurring in a 44-year-old man with an unusual hemorrhagic onset. The clinical, morphological and immunohistochemical features are discussed and the published literature is reviewed.

Pterygomaxillary extension of orbital pseudotumor. Case report.

Idiopatic pseudotumor (IP) is a non-specific, non-neoplastic, inflammatory process without identifiable local or systemic causes. IP most commonly affects the orbit, comprising 5% to 8% of all orbital masses with occasional extension to other areas of the skull base. The ophthalmology literature has detailed reviews of IP as a space-occupying intraorbital lesion, but lesion involving only the skull base and sparing the orbit is exceptional. Understanding the presentation, work-up, and characteristics of the lesion is important for proper diagnosis and treatment. We report a rare case of orbital IP with extension into infratemporal fossa. Clinical and radiological findings and surgical approach are discussed.

Vasospasm and cerebral infarction from pituitary apoplexy. A case report.

Pituitary apoplexy is a potentially life-threatening acute or subacute clinical syndrome occurring from enlargement of the pituitary gland, and pituitary insufficiency, from hemorrhage or ischemia from an unknown pituitary lesion, most frequently being a non-functioning macroadenoma. A close, and multidisciplinary management is required. The purpose of this case report is to increase awareness to pituitary apoplexy presentation and management by reporting clinical features and neuroradiological findings observed in a 70-year-old patient with an unknown pituitary lesion. He presented with pituitary apoplexy and brain ischemia at magnetic resonance imaging (MRI) including diffusion-weighted imaging (DWI), and apparent diffusion coefficient (ADC) maps. MR angiography (MRA) showed diffuse vasospasm of anterior and posterior circulation. Both MRI and cytochemical examination of the cerebrospinal fluid ruled out subarachnoid hemorrhage. Due to concomitant diseases, and absence of visual deficit, the management was conservative by medical and substitutive therapy, without surgery. Clinical follow-up showed clearcut improvement, and this was consistent with MRI and MRA evidence of vasospasm regression, and clearcut pituitary lesion shrinkage. Pituitary lesions with hemorrhagic infarction presenting with pituitary apoplexy may be associated with vasospasm and brain ischemia at diagnosis, also in the absence of subarachnoid hemorrhage. A correct MR evaluation of patients with PA should include DWI, ADC maps, and MRA. Notably, early diagnosis of PA-associated vasospasm and cerebral ischemia avoids the possibility of their detection only after neurosurgery.

Is CT still useful in the study protocol of retinoblastoma?

BACKGROUND AND PURPOSE: Intralesional calcium deposition is considered a key element for differentiating retinoblastoma from simulating lesions. Our aim was to assess whether MR imaging associated with ophthalmologic investigations (ophthalmoscopy and ultrasonography) could replace CT in the detection of diagnostic intralesional calcifications in retinoblastoma. MATERIALS AND METHODS: Ophthalmoscopic findings, MR images, CT scans, and histologic examination of 28 retinoblastomas from 23 consecutive children (11 males, 12 females; age range at admission, 1-35 months; mean age, 11 months; median age, 9 months) were retrospectively evaluated. Ultrasonography was performed in 18 patients with 21 retinoblastomas. MR imaging included T2-weighted spin-echo and gradient-echo images, fluid-attenuated inversion recovery images, and T1-weighted spin-echo images with and without contrast enhancement. Clinical data were integrated with MR imaging data to evaluate the utility of both approaches to discover calcifications; particularly, a correlation between intralesional signal-intensity void spots on MR imaging and hyperattenuating areas on CT scans was performed. RESULTS: Ophthalmoscopy detected calcifications in 12 of 28 eyes (42.85%). Ultrasonography detected calcifications in 20 of 21 eyes (95.23%). CT showed hyperattenuating intralesional areas consistent with calcifications in 27 of 28 eyes (96.42%). MR imaging showed intralesional signal-intensity void spots in 25 of 28 eyes (89.28%). All spots detected with MR imaging matched the presence of calcifications on CT scans. Gradient-echo T2*-weighted and fast spin-echo T2-weighted images showed the highest degree of correlation with CT. When we put together ophthalmoscopy, ultrasonography, and MR imaging data, no calcifications detected on CT were missed, and the differential diagnosis was thorough. CONCLUSIONS: A combination of clinical data and MR images may remove potentially harmful ionizing radiation from the study protocol of retinoblastoma.

Total occlusion of a conus medullaris pial arteriovenous malformation obtained with one session of superselective embolization.

The authors report about a case of the endovascular treatment of a pial arteriovenous malformation (AVM). The lesion was located on the conus medullaris. This injury is a rare spinal AVM. The diagnostic management and surgical treatment was chosen with a collaboration between neurosurgeons and neuroradiologists. The diagnostic management was based on clinical validation and magnetic resonance with angiographic technique as a gold standard. With regard to the surgical treatment of spinal AVM, endovascular and radiotherapy is a decision which should be taken multidisciplinarily. The treatment is crucial in resolving this lesion. The authors describe the case of a 38-year-old girl with clinical findings of progressive radiculomedullary ischemic process caused by the presence of spinal AVM. The angiographic images showed a pial AVM of the conus medullaris fed by an anterior radiculomedullary artery (Adamckiewiz artery) originated from a left T11 dorsospinal artery and by a posterior radiculopial artery originated from the left L1 artery. The draining veins were posterior pial veins, and accessory anterior subpial veins. Even if the first treatment of a pial arteriovenous malformation (AVM) of conus medullaris can be the surgical treatment for posterior localization, a neurointerventional angiographic and modern materials make it possible to reach pial AVMS of conus medullaris avoiding surgery. The authors describe a successful treatment of conus medullaris arteriovenous malformation with a one session of superselective embolization.

Cerebral abscesses and necrotic cerebral tumours: differential diagnosis by perfusion-weighted magnetic resonance imaging.

PURPOSE: This study was undertaken to evaluate the usefulness of perfusion-weighted imaging (PWI) in the differential diagnosis of ring-enhancing cerebral lesions, including abscesses, high-grade gliomas and metastases. MATERIALS AND METHODS: Nine cerebral abscesses (five pyogenic, four from Toxoplasma gondii), ten glioblastomas and five cerebral metastases in 19 patients were studied with gadolinium-enhanced magnetic resonance imaging, diffusion-weighted imaging (DWI) including calculation of mean apparent diffusion coefficient (ADC) of the lesion core, and PWI. At PWI, the mean of the maximum regional cerebral blood volume (rCBV) was calculated in the gadolinium-enhancing peripheral solid areas and compared with that of the contralateral normal-appearing white matter [ratio=rCBV (lesion)/rCBV (contralateral normal-appearing white matter)]. RESULTS: DWI achieved the differential diagnosis in all cases except for the four Toxoplasma abscesses. At PWI, the mean ratio of the rCBV of the capsular portion was 0.72+/-0.08 (range 0.60-0.82) in the pyogenic abscesses, 0.84+/-0.07 (range 0.75-0.91) in the Toxoplasma abscesses, 4.45+/-1.5 (range 2.9-8.0) in the high-grade gliomas and 3.58+/-0.68 (range 3.28-4.27) in the metastases. CONCLUSIONS: PWI seems to be useful in the differential diagnosis of ring-enhancing cerebral lesions. High rCBV values in the peripheral areas appear to indicate the possibility of a necrotic tumour, whereas low values tend to indicate an abscess.

Giant cystic intraventricular cerebral cavernous malformation: MRI with pathologic correlation. A case report.

The purpose of this case report is to increase awareness of the spectrum of cerebral cavernous malformations (CCMs). We report the MRI findings and histological features of an uncommon case of a single giant (maximum diameter: >6 cm) cystic CCM of the left lateral ventricle occurring in a 26-year-old man who had undergone 30 Gy cranial irradiation for acute leukemia at the age of six years. Large cystic CCMs must be included in the neuroradiological differential diagnosis of intraventricular hemorrhagic cystic lesions.

Coats disease: smaller volume of the affected globe.

PURPOSE: To determine whether a significant smaller volume of the affected globe, compared with that of the normal globe, is an additional feature of Coats disease. MATERIALS AND METHODS: Ocular globe volume was assessed in 13 children (11 boys, two girls; age range, 0.6-14 years; mean age, 4.1 years) with Coats disease and in 18 (eight boys, 10 girls; age range, 0.5-12 years; mean age, 3.6 years) with unilateral retinoblastoma. Orbital computed tomographic scans were available for all children; magnetic resonance images were available for 11 children-seven with Coats disease and four with retinoblastoma. For volume estimation, anteroposterior and equatorial diameters of ocular globes were measured. Statistical analysis was conducted with univariate and multivariate methods. RESULTS: In children with Coats disease, the mean volume of the affected globe was 4,877.03 mm(3) (range, 2,951.47-6,284.70 mm(3)) and that of the normal globe, 6,018.00 mm(3) (range, 4,062.32-7,509.26 mm(3)). In children with retinoblastoma, the mean volume of the affected globe was 4,557.06 mm(3) (range, 1,612.01-7,463.00 mm(3)) and that of the normal globe, 4,402.11 mm(3) (range, 1,360.46- 7,463.00 mm(3)). The Coats disease population had a significantly smaller volume of the affected globe (z = -3.1009; P =.002); the retinoblastoma population did not have a statistically significant trend toward a bigger affected globe volume (z = -1.7064; P =.088). The difference between the affected globe volume and the normal globe volume in children with Coats disease was the only significant independent variable (P =.005). CONCLUSION: A significantly smaller volume of the affected globe is an additional feature of Coats disease.

Destructive spondyloarthropathy of the cervical spine in long-term hemodialyzed patients: a five-year clinical radiological prospective study.

OBJECTIVE: To describe the radiographic features and progression of cervical spine destructive spondyloarthropathy (DSA) in hemodialyzed patients, and to evaluate the relationship between this disease and patient characteristics, biochemical values, and hemodialysis duration. DESIGN AND PATIENTS: Standard radiographs, and lateral flexion and extension views of the cervical spine, were performed annually for 5 years in 31 hemodialyzed patients who were divided into three groups at the commencement of the study: those showing (I) DSA, (II) vertebral rim erosions (VRE) without DSA, and (III) absence of DSA and VRE. Magnetic resonance (MR) imaging and computed tomography (CT) were performed in seven and two patients respectively. The imaging features were evaluated for the presence and progression of spondyloarthropathy and correlated with clinical and biochemical variables. Statistical analysis was performed using one-way analysis of variance. RESULTS: The duration of hemodialysis appeared to be the main predictive factor (P=0.0003) for DSA, which was found in six patients (19%). DSA was found to correlate with higher levels of beta2-microglobulin (P<0.00001), parathyroid hormone (P<0.05), and alkaline phosphatase (P<0.05). Clinical symptoms were minimal. In two patients, MR imaging revealed changes mimicking spondylodiscitis. In another patient, CT of the craniocervical junction showed bone resorption due to a pseudotumor, and basilar invagination. CONCLUSIONS: DSA of the cervical spine is often clinically silent. Pathogenesis of DSA may be multifactorial but its progression is most influenced by the duration of hemodialysis. On MR imaging, DSA may mimic spondylodiscitis.