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Malignant rhabdoid tumors (MRT) are aggressive neoplasms generally of the infant kidney, although a few extrarenal, central nervous system-located, were reported. We describe two cases of such tumors arising intracerebrally in patients aged 16 and 31 years, hence older than the usually reported, with survival times of 16 and 4 months, respectively. Besides, in the youngest, the mass seemed to have developed from a low grade glioma, an occurrence not reported earlier, although the coexistence of morphological features of glioma and rhabdoid tumor is well known. The broad immunoprofile spectrum found in these two tumors seems to reflect the lack of specific differentiation of MRTs. As in the case of other MRTs, our patients had short survivals despite the aggressive post-surgical treatment. In conclusion, MRTs should be suspected also in intracerebral neoplasms of adult patients, and the medical treatment remains disappointing.

Anti-glomerular basement membrane (anti-GBM) disease and antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are the main causes of pulmonary-renal syndrome (PRS). The concurrence of both ANCA -myeloperoxidase (MPO) and proteinase 3 (PR3) -and anti-GBM antibodies has been described, although positivity for all three antibodies has rarely been reported. The natural history of triplepositive patients as well as the best therapeutic approach remains unknown. We describe a case of an 80-year-old woman that presented to the emergency department with a 3-month history of progressive fatigue, malaise and anorexia, and 5 weeks of cough with bloodstreaked sputum and progressive peripheral edema. Through the complementary study, a rare diagnosis of PRS with triple-seropositive for both ANCA (MPO and PR3) and anti-GBM antibodies was made in a patient with untreated chronic hepatitis B virus infection. She was treated with glucocorticoid, cyclophosphamide, plasma exchange and entecavir, with pulmonary recovery. Renal function did not improve. After 2 years, the patient is still in dialysis, but did not have relapse of alveolar hemorrhage and ANCA and anti-GBM antibody titers remain negative. The authors intend to warn to PRS, in particular this rare cause, since delaying diagnosis can lead to significant morbidity and mortality for patients.

For successful surgery for drug-resistant epilepsy the site of the seizure focus needs to be known exactly. The purpose of this study was to compare the evaluation of the regional cerebral blood flow (rCBF) (localization and degree of disturbances) by 99mTc-hexamethylpropyleneamineoxime (HMPAO) single photon emission computed tomography (SPECT) with computerized electroencephalographic topography (CET) and transmission computed X-ray tomography (CT) in partial epilepsy. The study included 20 patients with medically refractory complex partial seizures. Of the 20 patients included, 15 were studied interictally, four ictally and one in both states, interictally and ictally. 99mTc-HMPAO SPECT detected rCBF changes in 95% of the patients. Interictal studies demonstrated focal areas of hypoperfusion in 93% of the patients. Ictal studies demonstrated an area of hyperperfusion in all patients. Blood flow disturbances in deeper structures of the brain, such as basal ganglia, could be detected. The areas of abnormal 99mTc-HMPAO uptake were concordant, in localization, with CET in 85% of the patients. Abnormal data with CT scans were found in only 45% of the patients. Focal lesions were found in 20% of the patients by CT scans. 99mTc-HMPAO SPECT combined with CET may be a useful screening procedure prior to referral for invasive diagnostic procedures in future management with medically refractory complex partial seizures.

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