Miguel Farage Filho

Hemiatrofia facial de Romberg: relato de caso

The authors report the case of a 45-years-old female patient, with progressive hemiatrophy of the left face and tongue. The laboratorial and image studies were normal, including routine blood exams, cerebral-spinal fluid, hepatic function tests, renal and rheumatic; besides X ray, computerized tomography and magnetic resonance of skull. The electroneuromyography showed peripheral neurogenic alterations of motor trigeminal and hypoglossal nerves, markedly on the left side. Those findings are compatible with Rombergs facial hemiatrophy diagnosis, motivating discussion of several aspects of this rare disease according to the account and literature.

Sindrome de russel tumor diencefálico em uma criança

The case of a male child with Russels syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th month of life, when he started losing weight By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nistagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/l) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).

Condrossarcoma de ossos da órbita: relato de um caso

Os autores registram um caso de condrossarcoma primitivo de orbita que se apresentou inicialmente com sintomas de dificuldade visual, dor e exoftalmia, registrando a raridade desta localizacao, que constitui o primeiro caso da literatura nacional. Sao discutidas as possibilidades de diagnostico diferencial mediante exame radiologico com carcinoma, meningioma e sarcoma osteogenico. Na histopatologia procura-se mostrar nao tanto a dificuldade diagnostica, mas principalmente a determinacao do tipo do tumor, com seu grau de diferenciacao. O prognostico sera tanto melhor quanto mais precoce for o diagnostico, quanto mais ressecavel for a lesao.A case of chondrosarcoma of the orbit is reported. The patient complained of marked deterioration of her vision, pain and presented exophatalmus of the left eye. Chondrosarcoma of the orbit are rare and this is the first case reported in the Brazilian literature. Diagnostic and prognostic are discussed.

Efeitos tardios na função hipotálamo-hipófise após tratamento de tumores parasselares

O acompanhamento a longo prazo de pacientes submetidos a tratamento de tumores da regiao parasselar e importante para detectar complicacoes tardias da terapeutica. Neste estudo avaliamos 6 pacientes com craniofaringioma, 1 com meningioma, 1 com germinoma e 1 com cisto epidermoide, localizados na regiao parasselar. Eles haviam sido tratados, em media, 3,8±3,2 anos antes, por cirurgia e radioterapia (6 casos) ou somente cirurgia (3 casos). Cinco pacientes eram do sexo feminino e a media de idade era de 24,3±18,8 anos. A avaliacao consistiu na infusao endovenosa de TRH (200 mg), GnRH (100 mg) e insulina regular (0,1 UI Kg/peso), bem como na dosagem dos hormonios hipofisarios antes (0) e apos 20, 40, 60 e 80 minutos. Encontramos os seguintes resultados: (a) resposta deficitaria do GH e do cortisol era todos pacientes; (b) 7/9 pacientes nao tiveram respostas adequadas do FSH e 3/9 do L.H; (c) 4/9 tiveram respostas inadequadas da prolactina e 2/8 do TSH. Concluimos que: (a) o deficit de GH e cortisol sao os mais frequentes nestes pacientes; (b) a lesao apos radioterapia pode localizar-se tanto no hipotalamo quanto na hipofise ou, ainda, em ambos; (c) a sensibilidade das celulas hipofisarias e hipotalâmicas a irradiacao e diferente de acordo com os hormonios que produzem; (d) e necessario o acompanhamento endocrinologico frequente dos pacientes submetidos a tratamento de tumores parasselares, a fim de detectar deficits hormonais tardios.Long term follow-up of patients submitted to treatment of parasellar tumours region is important for the detection of late therapeutic complications. In this study the authors conducted an evaluation of six patients with craniopharyngioma, one with germinoma, one with meningioma, and one epidermoid cyst. All above tumours were localized at parasellar region. Six out of nine patients had been treated both by surgery and by radiotherapy and the other three surgically only, on an average 3.8 +/- 3.2 years before this observation was carried out. Five patients were female with their ages average 24.3 +/- 18.8 years old. Evaluation consisted: in the first place, an intravenous infusion of thyrotropin-releasing hormone (TRH, 200 micrograms), gonadotropin-releasing hormone (GnRH, 100 micrograms), and insulin tolerance test (0.1 IU/Kg, regular insulin); and secondly, in measurements of pituitary hormones secretion at different time points--0, 20, 40, 60 and 80 minutes. We found both diminished response of growth hormone and cortisol in all the patients. Seven out of nine patients did not have adequate response to follicle-stimulating hormone. Three out of nine responded unsatisfactory to luteinizing hormone. Four out of nine showed inadequate responses to prolactin as well as, two out of eight to thyrotropin. We concluded that: (a) growth hormone and cortisol deficiency are the most frequent finding in these patients; (b) post-radiotherapy lesions can be located in the hypothalamus or pituitary, or even in both; (c) hypophysial and hypothalamic cells sensitivity to irradiation is different, according to their respective hormones; and (d) it is necessary a frequent endocrinologic follow-up of patients to detect late hormonal deficiencies.

Aneurisma gigante parasselar simulando tumor de hipófise

Seven cases of compromised pituitary fossa at the conventional skull X-ray, who had the final diagnosis of giant aneurysm of the intracavernous portion of the carotid artery (6 cases) and one of the anterior communicating artery, are reported. The main findings were: headache (7/7), complex ophthalmoplegia involving the III, IV and VI cranial nerves (5/7), compromised V cranial nerve (4/7) and eyeball pain (4/7). Other manifestations were: meningeal signs (2/7), unilateral blindness (1/7), hemiparesis (1/7), cacosmia (1/7) and inferior bitemporal quadrantanopsia (1/7). Five patients with intracavernous carotid artery aneurysm showed benefits with progressive occlusion of the internal carotid artery at the cervical level. One died before surgery. The case with anterior communicating artery aneurysm improved after its surgical clipping. Our data, in accord with the literature, support the conclusion that the differential diagnosis of aneurysms in the parasellar region remains a very difficult task. The accurate final diagnosis requires cerebral angiography and the surgical treatment with progressive occlusion at the cervical portion of the internal carotid artery has a relatively low risk with promising results.Seven cases of compromised pituitary fossa at the conventional skull X-ray, who had the final diagnosis of giant aneurysm of the intracavernous portion of the carotid artery (6 cases) and one of the anterior communicant artery, are reported. The main findings were: headache (7/7), complex ophtalmoplegia involving the III, IV and VI cranial nerves (5/7), compromised V cranial nerve (4/7) and eyeball pain (4/7). Other manifestations were: meningeal signs (2/7), unilateral blindness (1/7), hemiparesis (1/7), cacosmia (1/7) and inferior bitemporal quadrantanopsia (1/7). Five patients with intracavernous carotid artery aneurysm showed benefits with progressive occlusion of the internal carotid artery at the cervical level. One died before surgery. The case with anterior communicant artery aneurysm improved after its surgical clipping. Our data, in accord with the literature, support the conclusion that the differential diagnosis of aneurysms in the parasellar egion remains a very difficult task. The accurate final diagnosis requires cerebral angiography and the surgical treatment with progressive occlusion at the cervical portion of the internal carotid artery has a relatively low risk with promising results.

Instrumentação anterior da coluna em espondilite tuberculosa mal de pott: Relato de caso

We report a case of a surgical treatment with anterior instrumentation in tuberculous spondylitis (Potts disease), in a 71 years old woman, that was in treatment for pulmonary tuberculosis, with lumbar pain, progressive disability to walk, kyphotic deformity and vesical dysfunction. Magnetic resonance image presents a lesion in the bodies of T12 and L1, with paravertebral abscess. The patient was treated surgically by transthoracic-abdominal approach. The vertebral bodies were cut off and the spine were instrumented anteriorly with a mesh cage and a Z plate. This procedure permits a good arthrodesis and a immediately stabilization of the spine, without any complication of the infection. The patient was seen a year after the surgery and is free of infection, without motor deficit, pain or reminiscent kyphosis.We report a case of a surgical treatment with anterior instrumentation in tuberculous spondylitis (Potts disease), in a 71 years old woman, that was in treatment for pulmonary tuberculosis, with lumbar pain, progressive disability to walk, kyphotic deformit and vesical dysfunction. Magnetic resonance image presents a lesion in the bodies of T12 and L1, with paravertebral abscess. The patient was treated surgically by transthoracic-abdominal approach. The vertebral bodies were cut off and the spine were instrumented anteriorly with a mesh cage and a Z plate. This procedure permits a good arthrodesis and a immediately stabilization of the spine, without any complication of the infection. The patient was seen a year after the surgery and is free of infection, without motor deficit, pain or reminiscent kyphosis.

Sindrome do encarceramento (locked-in syndrome): registro de um caso e revisao de literatura.

The case of a 46 year-old man who had a locked-in syndrome is reported. The patient had a haemorragic vascular accident with tetraplegia, anartria, vertical ocular moviments and preservation of the conciousness. The EEG showed a projected disturbance possibly caused by a braim-stem lesion, that was confirmed by the CAT SCAN. Cerebral angiography showed high level of aterosclerosis. The lesion is located in the ventral part of the pons above the nucleous of the abducent nerve, without damaging the substantia reticularis of the mesencephalon and can be caused by trombosis of the basilar artery and tumor, myastenia gravis, polyneurites, heroin overdose, after swine-flu inoculation and post-infectous polyneurites. The patient is still alive 2 years after the stroke with a good recovery. He can walk and speak fluently, but with cordonal ataxy of the limbs.

Carcinoma de plexo coroideo: relato de um caso

The case of a three-years-old male with intracranial hypertension is reported. Without a clear clinical diagnosis, a gradual deterioration ocurred before his death, 8 days later. The neuropathological examination of the brain revealed a tumor with indistint borders at the right lateral ventricle, with granulous surface, pink-gray colour, irregular necrotic and hemorragic areas, invading the adjacent brain tissue. The histopathological study revealed a malignant variant of the choroid plexus tumor, of extreme rarity in the central nervous system. The biological and histological criteria accepted by Lewis, Rubinstein and Polak for the correct diagnosis of this type of tumour are discussed.