No abstract

PALAVRAS-CHAVEDeslocamento do disco intervertebral, neoplasias da medula espinal, neoplasias da bainha neural, vértebras lombares.

-Pituitary carcinomas are rare neoplasms characterized by craniospinal and/or systemic metastases originated from the pituitary. Their histopathology is frequently indistinguishable from that of benign adenomas. The development of markers that better reflect their behavior is desirable. We present the case of a 47 year-old man with a pro l a c t i n -s e c reting macroadenoma who was submitted to surgeries, cranial radiation therapy, and bromocriptine treatment, but evolved to a fatal outcome after the disclosure of int r a c r anial metastases. Tumor samples underwent p53 and Ki-67 immunohistochemical assessment. p53 was absent in all samples, a rare finding among pituitary carcinomas. Ki-67 proliferative index was 2.80% in the original tumor, 4.40% in the relapse, and 4.45% in the metastasis. The figure in the relapse is higher than the expected for a noninvasive adenoma. In conclusion, p53 staining is not positive in all pituitary carc i n o m a s . A high Ki-67 proliferative index in a pituitary adenoma might indicate a more aggressive behavior.KEY WORDS: prolactinoma, pituitary carcinoma, metastasis, p53, Ki-67. P rolactinoma metastático: relato de caso com avaliação imuno-histoquímica para os antígenos p53 e Ki-67 RESUMO -Carcinomas pituitários são neoplasias raras caracterizadas pela presença de metástases cranioespinhais e/ou sistêmicas originadas da hipófise. Sua histopatologia é freqüentemente indistinguível daquela dos adenomas benignos. O desenvolvimento de marc a d o res que melhor reflitam o seu comport a m e nto é desejável. Apresentamos o caso de um homem de 47 anos com um macroadenoma secretor de pro l a c t i n a que foi submetido a procedimentos cirúrgicos, radioterapia e tratamento com bromocriptina, mas que e v o l u i u para o óbito após o descobrimento de metástases intracranianas. Amostras do tumor foram submetidas à a n á-lise imuno-histoquímica para os antígenos p53 e Ki-67. A coloração para p53 foi negativa em todas as amostras, um achado raro entre os carcinomas pituitários. O índice proliferativo Ki-67 foi 2,80% no tumor origin a l , 4,40% na recidiva e 4,45% na metástase. O valor obtido na recidiva é maior que o esperado para um adenoma não-invasor. Concluindo, a coloração para p53 não é positiva em todos os carcinomas pituitários. Um í n d i c e proliferativo Ki-67 alto em um adenoma pituitário poderia indicar um comportamento mais agressivo. PALAVRAS-CHAVE: prolactinoma, carcinoma pituitário, metástase, p53, Ki-67.The prevalence of clinically overt pituitary tumors is around 0.02% of the population

Paracoccidioidomycosis is the most prevalent endemic mycosis in Latin America, with a great incidence in Brazil. Although a common disease, its medullary form is rare. We present a case report of medullary paracoccidioidomycosis presenting with subacute, progressive, spinal cord symptoms, and with multiple expansive lesions into the cervical and thoracic spinal cord. The patient was treated with itraconazole and showed good clinical and radiologic recovery upon six months of follow-up.

-Down syndrome is the most frequent genetic cause of mental retardation. Although usually presenting dysmorphic features and organ malformations, it is rarely associated with congenital hydrocephalus. The case of male neonate whose hydrocephalus was detected since the pregnancy and was discovered to have the syndrome at birth is reported. Chromosomal analysis confirmed the genetic disorder, and hydrocephalus was treated with ventriculoperitoneal shunt because of abnormal increase of head circumference. The patient has been accompanied and his development is considered normal when compared to the expected for those affected by the syndrome.

The cerebral proliferative angiopathy (CPA) is an uncommon vascular malformation of the brain, representing approximately 3.4% of arteriovenous malformation (AVM) cases, with complex morphology and poorly understood origin. Thus, we present the case of a woman patient aged 27 years, who performed magnetic resonance imaging (MRI) of the brain because of a headache. Extensive malformation was identified vascular right frontoparietal, with the presence of the brain tissue mixed with the vessel. CPA identified multiple feeder arteries, with the absence of nidus and slowing the flow into the venous system, confirming that it was CPA. During evaluation at another hospital she underwent partial embolization of the lesion, without success. Currently, the patient is progressing with left hemiparesis. CPA has distinct characteristics, such as large dimensions, presence of functional brain tissue intermingled with the vessels, no nidus, multiple nourishing vessels, and small veins. It does not present well-defined nidus or flow-related aneurysms. These characteristics classify it as a disease itself within the group of AVMs. This case clinically presents with seizures, refractory headaches, focal neurologic deficits, and low risk of bleeding. Alternatives for curative treatment imply significant neurologic deficits and the most accepted options aimed at easing the symptoms and the partial embolization alternative that is best associated with risks and benefits. ResumoA angiopatia cerebral progressiva (ACP) é uma malformação vascular encefálica incomum, representando cerca de 3,4% dos casos de malformações arteriovenosas (MAV's), com morfologia complexa e origem pouco compreendida. Assim, apresentamos o caso de uma paciente de 27 anos, que realizou RNM de crânio para investigação etiológica de cefaleia, tendo sido identificado extensa malformação vascular frontoparietal direita, com a presença de tecido cerebral entremeado aos vasos. Exame de

-Fasciculations are symptoms present in a broad spectrum of conditions, ranging from normal manifestations to motor neuron diseases. They also represent the main picture of benign fasciculation syndrome. We report a case of such syndrome: a 48-years-old woman complaining about fasciculations for three decades who remained with the symptoms even after the compensation of a disclosed hyperthyroidism. The introduction of gabapentin rendered control of her fasciculations. The available data in the literature about the therapeutic approaches for fasciculations are revised, as long as the rare reports of evolution from patients with "benign" fasciculations to cases of amyotrophic lateral sclerosis, underlining the importance of following the patients with fasciculations.KEY WORDS: benign fasciculations, gabapentin, motor neuron disease, amyotrophic lateral sclerosis. Fasciculações benignas responsivas à gabapentinaRESUMO -Fasciculações são sintomas presentes em um amplo espectro de condições, desde manifestações normais até doenças do neurônio motor. Elas representam também o principal aspecto da síndrome de fasciculações benignas. Relatamos um caso desta síndrome: uma paciente de 48 anos com queixas de fasciculações por três décadas que, mesmo após a compensação de um quadro de hipertireoidismo, permaneceu com os sintomas. A introdução de gabapentina levou a controle das fasciculações. Os dados disponíveis na literatura sobre as abordagens terapêuticas para fasciculações são revisados, assim como os raros relatos de evolução de pacientes com fasciculações "benignas" para casos de esclerose lateral amiotrófica, salientando a importância do seguimento dos pacientes com fasciculações. PALAVRAS-CHAVE: fasciculações benignas, gabapentina, doenças do neurônio motor, esclerose lateral amiotrófica.

Tuberculosis is a chronic bacterial infection caused by Mycobacterium tuberculosis.Despite advances in treatment, resistant strains and unusual sites of involvement have been diagnosed. We present a case of a 13-year-old patient in treatment for tuberculous meningitis who presented with progressive paraparesis. The MRI showed two intramedullary nodular lesions at T4-T6 levels, isointense with annular hyperintensity on T1W, hypointense on T2W, becoming hypointense with ring enhancement after contrast. These characteristics differ from those usually described for intramedullary tuberculomas. Surgical excision was performed, confirming the diagnosis of intramedullary tuberculoma. The formation of intramedullary tuberculomas is rare, with a ratio of two cases per thousand diagnosed with CNS tuberculosis, and the thoracic spine is most frequently affected. The clinical picture is of progressive subacute spinal cord compression, and it may lead to paraplegia. At MRI, the lesion in early stage appears as hypointense rings on T1W and hyperintense on T2W, with homogeneous enhancement after contrast. After the formation of the solid caseous granuloma, it becomes isointense on T1W and hypointense on T2W with homogeneous enhancement after contrast. When the center of the granuloma becomes liquefied, it shows hypointense sign on T1W and hyperintense with peripheral enhancement on T2W. The treatment of choice is medical, with the current protocol including rifampin, isoniazid, pyrazinamide, and ethambutol. Surgery is reserved for cases of progressive neurologic