Meningioma with rhabdoid, papillary and clear cell features: case report and review of association of rare meningioma variants.

Meningiomas are common central nervous system tumors with a wide range of morphological variants, assigned World Health Organization (WHO) Grades I - III. We report an extremely rare rhabdoid, papillary and clear cell meningioma (WHO Grade III) in a 29-year-old female, who presented with diplopia and headache over a few days, 2 years ago. Magnetic resonance imaging showed a well-circumscribed, lobulated, predominantly solid and contrast-enhancing lesion in the right temporal, parietal and occipital lobes. On routine staining, the tumor did not display classical meningioma features. A wide immunohistochemical panel ruled out metastasis and endorsed the meningothelial nature of the lesion (positivity for epithelial membrane antigen and vimentin). Electron microscopy did not show usual hallmarks of meningioma but was helpful in excluding other tumors. Even though the three variants are associated with aggressive behavior, the patient is currently asymptomatic. The concurrent use of different techniques was essential for diagnosis.

Lung disease features in Brazilian children with HIV infection before HAART era.

Lower respiratory tract diseases are major causes of morbidity and mortality in HIV infected children. We studied the lung disease features associated with AIDS in children and adolescents, in an era of ineffective antiretroviral therapy, between January 1996 and October 1998. This prospective, descriptive, longitudinal and historical medical chart review included 48 vertically HIV infected patients, receiving mono or double antiretroviral therapy, who had developed pulmonary disease. Those who presented acute pneumonia were classified into group 1; radiological changes for >or=3 months into group 2; those from group 1 and 2 who underwent lung biopsy into group 3. A rapidly progressive clinical course was found in 70.7% of the children and 37.5% younger than 6 months old. Bacterial pneumonia was diagnosed in all patients. High resolution chest computer tomographic scans (HRCT) from 27 patients showed a reticulonodular pattern in 8, ground-glass in 3, reticular in 3, nodular in 3, airspace consolidation in 3, mediastinal adenopathy in 3, pulmonary air cystic in 2 and air-trapping in 1. In five patients the HRCT were normal. Histopathology revealed: lymphoid interstitial pneumonitis in 5 patients, pulmonary lymphoid hyperplasia in 9, tuberculosis in 1, interstitial pneumonia in 1, diffuse alveolar damage in 1. Two patients had Cryptococcus neoformans and Mycobacterium tuberculosis. We conclude that lung diseases were the major risk factor for high morbidity, and an invasive diagnostic procedure may clarify the main cause for similar radiologic images of infectious and non-infectious processes.

Neuroimaging in pineal tumors.

BACKGROUND AND PURPOSE: The authors report radiological findings in 11 tumors in the pineal region, which were histologically diagnosed as germinomas, pineocytomas pineoblastomas, ependymomas, teratomas, and astrocytomas. METHODS: Computed tomography (CT) was performed in seven patients and magnetic resonance imaging (MRI) was performed in all patients. RESULTS: CT showed a solid or solid/cystic mass with variable contrast enhancement. MRI showed a heterogeneous mass, with hypointense signal on T1 and iso/hyperintense signal on T2-weighted images (WI) and gadolinium enhancement. Extension to adjacent structures occurred in five patients and spread through the cerebral spinal fluid (CSF) in two. CONCLUSIONS: Pineal region tumors have no pathognomonic imaging pattern. MRI and CT are complementary in diagnosis and are important to determine localization, extension, and meningeal spread.

Hemiparetic cerebral palsy: clinical data compared with neuroimaging

OBJETIVO: Este estudo correlacionou o desempenho da função motora fina (FMF) e Sensorial (FS) na paralisia cerebral hemiparética (PC-H) à classificação da ressonância magnética (RM). MÉTODO: Utilizaram-se os protocolos específicos para avaliar FMF, FS e lesões encefálicas quanto à sua extensão e localização no hemisfério. Foram avaliados 46 sujeitos sendo 23 com PC-H, grupo 1, e 23 crianças normais, grupo 2. A idade variou entre 07 a 16 anos, idade média de 12 anos e 8 meses. RESULTADOS:O desempenho das FMF e FS demonstrou ser significantemente pior no grupo 1 quando comparado ao grupo controle. PC-H que apresentavam lesões atingindo uma única estrutura encefálica demonstraram melhores resultados que aqueles com comprometimento atingindo duas estruturas maiores que 10 mm. O desempenho dos sujeitos com comprometimento cortical e subcortical, uni ou bilateral, foi inferior quando comparados aos com lesões subcorticais.CONCLUSÃO: As disfunções motoras e sensoriais necessitam ser identificadas e compreendidas para prover melhor treinamento de rotina e cuidados especiais a essas crianças.

Lipidized giant-cell glioblastoma of cerebellum.

Glioblastoma multiforme is recognized rarely in the cerebellum. We describe a peculiar case with lipid accumulation in giant tumor cells, possibly the second example so far reported in this unusual location. A 46-year-old man with a 5-month history of headache, vomiting, dizziness and instability of gait, was found to have on magnetic resonance imaging an expanding mass situated deep in the left cerebellar hemisphere. The lesion was hypointense in T 1- and hyperintense in T2-weighted images, had poorly defined borders, peripheral edema and annular foci of contrast enhancement. Eight months after subtotal removal and radiotherapy, control MRI showed tumor recurrence with aggressive features. The patient was alive 15 months after operation but follow-up was eventually lost. Histologically, the tumor showed marked pleomorphism, with many giant cells characterized by finely vacuolated cytoplasm strongly suggestive of lipid accumulation. There were few, sometimes atypical mitotic figures and foci of endothelial proliferation. The tumor cells were strongly positive for GFAP, vimentin and S100 protein, all of which stressed the foamy appearance of the giant cells. About 15% of nuclei were positive for Ki-67. We considered the case to be a so-called lipidized glioblastoma, first recognized as a subtype by Kepes and Rubinstein [1981]. Differential diagnosis with anaplastic pleomorphic xanthoastrocytoma is discussed.

Cerebral atrophy related to corticotherapy in systemic lupus erythematosus (SLE).

The aim of this study was to evaluate the frequency and intensity of cerebral atrophy using CT scanning and the possible relation to corticosteroid therapy or disease in systemic lupus erythematosus (SLE) and to analyse the relationships between cerebral atrophy and activity disease and neuropsychiatric manifestations in lupus patients. We studied 107 consecutive SLE patients (American Rheumatology Association 1982 criteria) who were taking steroid drugs at the time and not selected for any particular manifestation (group 1). A complete clinical, neurological and laboratory evaluation was performed. The American College of Rheumatology's classification for neuropsychiatric manifestations and SLE disease activity index for activity were employed. Group 2 comprised 39 non-SLE patients with oral chronic steroid use (1 mg/k/day for more than 3 consecutive months); 50 normal individuals were the controls (group 3). There were no demographic differences between the groups. Brain CT was performed in all individuals and the frequency and the intensity (minimal, moderate and severe) of atrophy analysed, through well-defined measures and indices, by two neuroradiologists. Cerebral atrophy was significantly more frequent in groups 1 and 2 than in group 3, but with no significant difference between groups 1 and 2. The severity of cerebral atrophy was significantly higher in SLE patients (p<0.05), independent of steroid dose or duration of disease. In both groups no patient presented severe atrophy. Lupus patients with and without cerebral atrophy presented neuropsychiatric manifestations and activity disease in a similar proportion. The more frequent neuropsychiatric manifestation in lupus patients with cerebral atrophy was seizures (p<0.05). Chronic glucocorticoid therapy was responsible for cerebral atrophy, with a comparable incidence in both lupus and non-lupus patients compared to age and gender-matched normal subjects untreated with glucocorticoids. The disease activity was not related to cerebral atrophy in group 1 and seizures were the neurologic manifestation related to cerebral atrophy. The severity of the cerebral atrophy was independent of steroid dose, or duration of treatment. Moreover, the disease itself contributes to the severity of this process, but not to the development of cerebral atrophy.

Ehlers-Danlos syndrome type IV and multiple aortic aneurysms--a case report.

Beside atherosclerosis, aortic aneurysms can be part of the clinical spectrum of many systemic diseases, including infectious, inflammatory, genetic and, less often, congenital disorders. A 48-year-old white man presented with multiple large aneurysms of the aorta and its main branches. Medical history was unremarkable except for the presence of a softened abdominal mass since he was 28 years old. On the physical examination, an arterial murmur was heard over the left carotid artery and a palpable mass was noted in the whole right side of the abdomen. No skin or joint abnormalities were noted. Aortography, computed tomography, and magnetic resonance angiography showed multiple large aneurysms of the descending thoracic and abdominal aorta. Aneurysms of the innominate, left subclavian, and carotid arteries were also seen. This case resembles those previously reported, in which multiple aortic aneurysms were associated with abnormalities of the type III procollagen gene (COL3A1). Although the classic stigmas of the Ehlers-Danlos syndrome type IV were lacking, this genetic disease may be the cause of the multiple aneurysms in this patient.

Magnetic resonance imaging of femoral marrow cellularity in hypocellular haemopoietic disorders.

AIM: Magnetic resonance imaging (MRI) of the femora was used to investigate the marrow cellularity during the evolution of non-transplanted aplastic anaemia (AA) and hypocellular myelodysplastic syndrome (h-MDS) in order to investigate the relationship between this cellularity and disease progression. METHODS: Magnetic resonance imaging was performed in adult patients with pancytopaenia and hypocellular bone marrow. Coronal T1 weighted and STIR images were obtained, and analysed semiquantitatively. These data were compared with diagnosis, peripheral blood counts and bone marrow histology at diagnosis and at the time of the MRI examination. RESULTS: Patients were examined 2-84 months after diagnosis (median, 16 months). In AA, 11/13 patients showed a fatty, faint or nodular pattern. In h-MDS, the majority of the patients (10/14) had a scattered or uniform signal pattern. In AA, a significant correlation was found between the degree of femoral cellularity and disease duration. Only three cases had diffuse high signal on STIR: among them, one had paroxysmal nocturnal haemoglobinuria and the other developed acute leukaemia 3 months after MRI examination. Four patients have died: three with h-MDS presenting a scattered (two cases) or a uniform (one case) MRI signal and one with AA (with a nodular pattern) CONCLUSION: In AA, femoral haemopoiesis is usually not pronounced, and if present, does not contribute to the improvement of blood counts. In h-MDS, patients with discrete femoral haemopoiesis had an improvement in their blood counts with disease duration, similar to that found in AA. Conversely, in patients with pronounced femoral cellularity, blood counts remained stable or had deteriorated since diagnosis. This favours the hypothesis that, as is observed in MDS with a hypercellular marrow, scattered or uniform marrow patterns in femoral MRI are signs of more aggressive disease.

Childhood epilepsy due to neurocysticercosis: a comparative study.

PURPOSE: To assess the clinical profile of pediatric patients with epilepsy and neurocysticercosis (NC), and compare them with a group of pediatric patients with benign partial epilepsy to determine clinical differences, response to treatment, and prognosis. METHODS: We studied 28 patients (16 girls) with probable or definitive diagnosis of NC and epilepsy and 32 patients (16 girls) with partial benign epilepsy (BE). All patients had normal neurologic examination. We compared NC and BE patients looking for differences in demographics (age at first seizure, gender, family history); clinical presentation (type, frequency, duration, and total number of seizures, duration of epilepsy, status epilepticus, cluster, and postictal deficit); treatment [duration, number of antiepileptic drugs (AEDs), maximal dose, drug association, number of seizure-free patients, time to obtain control and recurrence after medication discontinuation]; complementary examinations (the first and the last EEG). RESULTS: The mean follow-up was 5.4 years for the 28 NC patients and 4.6 years for the 32 BE patients (p=0.98). We did not find statistical differences between NC and BE in gender, family history, types of seizures, frequency and length of seizures, previous status epilepticus, seizure clustering, and presence of postictal deficits. However, we found that NC compared with BE patients had significant longer AED treatment, more seizures after AED introduction, tried more AEDs and at maximal dose, and in 20%, required polytherapy. The recurrence rate in NC was 54.4% and this was not significantly associated with number of lesions and disease activity seen on CT scans or the presence of EEG abnormalities. CONCLUSIONS: NC presents with a mild form of epilepsy in terms of seizure severity; however, it is more challenging in regard to drug management and has a less favorable long-term prognosis in terms of seizure remission. The number of lesions or disease activity seen on computed tomography (CT) as well as EEG abnormalities have no prognostic value in childhood epilepsy due to NC.

Blood homocysteine and vitamin B levels are not associated with cognitive skills in healthy normally ageing subjects.

Increased plasma total homocysteine (tHcy) levels are a known risk factor for vascular disease and have been reported in association with cognitive impairment of old age. Alternatively, however, increased tHcy levels may simply be an indicator of B vitamin deficiency. We evaluated the relationship between plasma tHcy levels, serum vitamin B12 and folate levels, and the scores at a battery of neuropsychological tests in 54 healthy cognitively normal subjects aged 65 years and over. Hyperhomocysteinemia prevalence (plasma tHcy>15 micromol/L) was about 24%. In univariate analysis, vitamin B12 levels were associated with both verbal memory and visuo-spatial skills, whereas no association was found between psychometric test scores and folate levels or tHcy levels. However, none of the univariate associations of neuropsychological test scores and serum B12 vitamin levels was confirmed when adjusting for age, education and other confounding variables. In conclusion, although a relationship between homocysteine, B vitamins and poor cognitive skills in the elderly is plausible, this study does not suggests that such relationship is biologically important.

Acute cervical epidural hematoma: case report.

A 74 year-old patient with a nocturnal onset of neck and chest pain was brought to an emergency clinic. Physical examination and cardiac assessment were normal. Three hours after the addmittance, a flaccid paralysis of the four limbs supervened. Suspecting of an unusual onset of central nervous system infection, a lumbar puncture was performed, yielding 20 ml of normal cerebrospinal fluid. Thirty oinutes after the puncture, the patient completely regained neurological funcion. He was then referred to a General Hospital where a computed tomography (CT) scan was done showing a large cervical epidural bleeding in the posterolateral region of C4/C5 extending to C7/Th1, along with a C6 vertebral body hemangioma. A magnetic resonance imaging revealed the same CT findings. A normal selective angiography of vertebral arteries, carotid arteries and thyreocervical trunk was carried out. Spontaneous spinal epidural hematoma (ASSEH) is a rare but dramatic cause of neurological impairment. In this article we report a fortunate case of complete recovery after an unusual spine cord decompression. We also review the current literature concerning diagnosis and treatment of ASSEH.

[Dose-dependent relationship of chronic use of phenytoin and cerebellar atrophy in patients with epilepsy]. / Relação dose-dependente do uso crônico de fenitoína e atrofia cerebelar em pacientes com epilepsia.

The chronic treatment with phenytoin or the acute intoxication by this drug may cause permanent cerebellar injury with atrophy of cerebellum vermis and hemispheres, which can be detected by neuroimaging studies. The aim of the present study was to investigate the correlation between the dosage and duration of treatment with phenytoin and the occurrence of cerebellar atrophy. Sixty-six patients were studied and had their tomographies analyzed for cerebellar atrophy. Of the 66 patients studied, 18 had moderate/severe atrophy, 15 had mild atrophy and 33 were considered to be normal. The patients with moderate/severe atrophy were those with higher exposure to phenytoin (longer duration of treatment and higher total dosage) showing statistically significant difference when compared to patients with mild atrophy or without atrophy (p=0. 02). Further, the patients with moderate/severe atrophy had serum levels of phenytoin statistically higher than those of patients with mild atrophy or without atrophy (p = 0.008). There was no association between other antiepileptic drugs dosage or duration of treatment and degree of cerebellar atrophy. We also found that older patients had cerebellar atrophy more frequently, indicating that age or duration of the seizure disorder may also be important in the determination of cerebellar degeneration in these patients. We conclude that although there is a possibility that repeated seizures contribute to cerebellar damage, long term exposure to phenytoin, particularly in high doses and toxic serum levels, cause cerebellar atrophy.

Thrombosis of the internal carotid artery secondary to soft palate injury in children and childhood. Report of two cases.

Trauma to the soft palate is a uncommon event during childhood. Stroke following intraoral trauma is also rare, but has been well documented by the current literature as a potentially serious complication. In this article, we report 2 cases of posttraumatic internal carotid artery thrombosis depicted by imaging studies. We discuss pathogenesis, and the literature is reviewed.

Magnetic resonance imaging in the evaluation of patients with aseptic meningoencephalitis and connective tissue disorders.

OBJECTIVE: To describe the role of magnetic resonance imaging (MRI) in the evaluation of patients with chronic and recurrent aseptic meningitis. METHOD: A retrospective study of five patients with aseptic meningoencefalitis diagnosed by clinical and CSF findings. CT scans showed without no relevant findings. RESULTS: MRI showed small multifocal lesions hyperintense on T2 weighted images and FLAIR, with mild or no gadolinium enhancement, mainly in periventricular and subcortical regions. Meningoencephalitis preceded the diagnosis of the underlying disease in four patients (Behçet's disease or systemic lupus erythematosus). After the introduction of adequate treatment for the rheumatic disease, they did not present further symptoms of aseptic meningoencephalitis. CONCLUSION: Aseptic meningoencephalitis can be an early presentation of an autoimmune disease. It is important to emphasize the role of MRI in the diagnosis and follow-up of these patients.

Tomografia computadorizada de torax de alta resolucao em paracoccidioidomicose (blastomicose sul-americana) / Resolution thoracic computed tomography for the diagnosis of south american blastomycosis

A blastomicose sul americana (BSB) e doenca causada pelo fungo Paracoccidioides brasiliensis.Ela acomete principalmente lavradores do sexo masculino, por via inalatoria,acometendo os pulmoes em mais de 80 por cento dos casos na forma cronica do adulto.Foram estudados 30 pacientes com DSA durante tes anos com radiogramas de torax e tomografia computadorizada de torax de alta resolucao(TCTAR) para correlacionar seus achados no sentido de auxiliar o diagnostico.O radiograma confirmou os achados mais comuns:infiltrados reticular e nodular,mais nos campos medios,alguns com consolidacao(19=63 por cento)hiperinsuflacao(20=66 pr cento)e bolhas subpleurais(10=33por cento).A TCTAR mostrou a historia natural da doenca: 1. Via inalatoria:com multiplas areas parenquimatosas semelhates a vidro fosco que podem progredir ou regredir(3=10 por cento) 2. Lesoes parenquimatosas progressivas com nodulos(25=83,3 por cento) consolidacoes irregulares(20=66 por cento, as vezes com insuflacao(22=73 por cento e bronquiectasias de tracao( 4=13 por cento)cavitacoes podem ocorrer por necrose(9=30 por cento) 3.Via linfatica com espessamento intersticial axial na zona medular com distorcao(25=83,3 por cento)4.Doenca progressiva com componente fibrotico e insuflacao(4=13 por cento).Conclusoes:As lesoes podem predominar em uma das duas regioes pulmonares.Temos:a padrao predominando lesoes parenquimatosas;padra predominando lesoes interticiais axiais;padrao predominando lesoes escavadas;e padrao combinado das lesoes

[Thrombosis of the internal artery secondary to soft palate injury: case report]. / Trombose da artéria carótida interna devida a trauma penetrante no pálato mole. Relato de caso.

Stroke following intraoral trauma is a rare complication of a common childhood injury. In the literature these complications have been well documented, however this condition is still infrequent. In order to alert the physicians about this possible injury we report our experience with one case. Computer tomography and magnetic resonance imaging evidenced complete occlusion of the internal carotid artery. Pathogenesis of this oral trauma is discussed.