João Alberto Assirati Junior

Amygdala gene expression of NMDA and GABAA receptors in patients with mesial temporal lobe epilepsy

Temporal lobe epilepsy (TLE) is the most common form of partial epilepsy and affects 40% of the patients. Seizures arising from the mesial temporal lobe structures (i.e., amygdala and hippocampus) are common, whereas neocortical seizures are rare. In recent years, many studies aimed to identify the pattern of gene expression of neurotransmitters involved in molecular mechanisms of epilepsy. We used real‐time PCR to quantify the expression of GABAA (subunits α1, β1, β2) and NMDA (subunits NR1, NR2A, and NR2B) receptor genes in amygdalae of 27 patients with TLE and 14 amygdalae from autopsy controls. The NR1 subunit was increased in patients with epilepsy when compared with controls. No differences were found in expression of NMDA subunits NR2A and NR2B or in α1, β1, and β2 subunits of GABAA receptors. Our results suggest that the NR1 subunit of NMDA receptors is involved in the amygdala hyperexcitability in some of the patients with TLE.

Olfactory groove meningiomas: surgical technique and follow-up review

BACKGROUND Olfactory groove meningiomas comprise 4-10% of the intracranial meningiomas. Generally they give signs of brain compression due to great size they reach before diagnosis. In this study, the clinical outcome of patients with olfactory groove meningiomas surgically treated was analyzed. METHOD 17 patients operated on from 1988-2006. Female: 16, Male: 1. Age: 19-76 years-old (mean=53.12 +/- 13.11). FOLLOW-UP 1-209 months (mean=51.07 +/- 12.73. Bifrontal/bifrontal-bi-orbital approaches were used. Outcome was analyzed using survival/recurrence-free Kaplan-Mayer curves. RESULTS 16 had WHO grade 1; one grade 2 meningiomas. Resection Simpsons grade 1 was in achieved in 64.7%, grade 2 in 29.4% and grade 3 in 5.9%. There was no recurrence during the follow-up. Global and operative mortality were 11.8%. Main postoperative complications were osteomyelitis (11.8%) and pneumonia (5.9%). CONCLUSION Extensive approaches allowed total resection of most olfactory groove meningiomas with no recurrence during the follow-up, but operative mortality and local complications were high.

Ultra-sonografia cerebral em crianças no primeiro ano de vida: método para o diagnóstico e acompanhamento das dilatações ventriculares

The ultrasonography (Mode B--Real Time) experience was analyzed to evaluate neurological diseases in children during their first year of life. Forty-two examination were accomplished in twenty-eight children with the following diagnosis: hydrocephalus (22), normal (15), subdural hygroma (3), intracranial cyst and hydrocephalus (1), giant encephalocele (1). The technique consists of positioning the transducer in the coronal, sagital and axial direction and selecting dynamically the images to be photographed. In the coronal position, the height of the lateral ventricle and the width of the third ventricule were obtained. In the axial position, the ventricular ratio-lateral ventricle width cerebral hemisphere width was obtained. Although it was a small group of patients, those indexes can objectify the ventricular size variation in children with well or poor functioning shunts. The importance of this method was the possibility to follow the development of hydrocephalus in cases of myelomeningocele and to analyze the etiology and features of hydrocephalus with or without shunts. In conclusion, this test is very usefull, mainly because it is very brief (about 20 minutes), the patient does not need sedation, it is innocuous, very precise even when compared with computerized tomography and for its low cost.

Foramen magnum meningiomas: surgical treatment in a single public institution in a developing country

OBJECTIVE To analyze the clinical outcome of patients with foramen magnum (FM) meningiomas. METHOD Thirteen patients (11 Feminine / 2 Masculine with FM meningiomas operated on through lateral suboccipital approach were studied. Clinical outcome were analyzed using survival (SC) and recurrence-free survival curves (RFSC). RESULTS All tumors were World Health Organization grade I. Total, subtotal and partial resections were acchieved in 69.2%, 23.1% and 7.7%, respectively, and SC was better for males and RFSC for females. Tumor location, extent of resection and involvement of vertebral artery/lower cranial nerves did not influence SC and RFSC. Recurrence rate was 7.7%. Operative mortality was 0. Main complications were transient (38.5%) and permanent (7.7%) lower cranial nerve deficits, cerebrospinal fluid fistula (30.8%), and transient and permanent respiratory difficulties in 7.7% each. CONCLUSIONS FM meningiomas can be adequately treated in public hospitals in developing countries if a multidisciplinary team is available for managing postoperative lower cranial nerve deficits.

Atypical and anaplastic meningiomas in a public hospital in São Paulo State, Brazil

UNLABELLED Atypical/anaplastic (World Health Organization (WHO) grades II and III) are less common and have poorer outcomes than benign meningiomas. This study aimed to analyze the outcome of patients with these tumors. METHOD Overall/recurrence-free survivals (RFS) and the Karnofsky Performance Scale of 52 patients with grades II (42) and III (9) meningiomas surgically treated were analyzed (uni/multivariate analysis). RESULTS Total/subtotal resections were 60.8%/35.3%. Patients <60 years-old and grade II tumors had longer survival. Grade II tumors, total resection and de novo meningioma had better RFS (univariate analysis). Patients >60 years-old, de novo meningioma and radiotherapy had longer survival and patients <60 years-old and with grade II tumors had longer RFS (multivariate analysis). Recurrence rate was 51% (39.2% Grade II and 66.7% Grade III). Operative mortality was 1.9%. CONCLUSION Age <60 years-old, grade II tumors and de novo meningiomas were the main predictors for better prognosis among patients with grades II and III meningiomas.

Tratamento cirúrgico dos aneurismas intracranianos: comparação entre cirurgias precoce e tardia

The clinical course of patients with subarachnoid hemorrhage (SAH) due to rupture of cerebral aneurysm admitted during the last five years is analysed: 157 patients were treated by direct surgical approach of the aneurysm, 58 localized in the anterior communicating artery (ACoA), 48 in the internal carotid artery (ICA), 43 in the middle cerebral artery (MCA), and 8 in the posterior circulation. Fourty-four patients were operated on during the first 72 hours (early surgery), 40 during the 4th and 7th days, 16 during the 8th and the 10th, and 57 after the 10th (late surgery). According to main localizations, the outcome of patients with aneurysms in the ACoA was good in 79.1%, in the ICA in 69.7%, and in the MCA in 69.7%. Patients treated in Hunt & Hess grade I and II had both good results in 77.5%, grade III patients had good results in 71.3%, and grade IV in 56.2%. According to timing of surgery good results were observed in 61.4% for patients submitted to early surgery, in 80% for patients treated during the 4th and 7th days, in 81.2% for patients treated during the 8th and the 10th days, and in 70.2% for that submitted to late surgery. The overall mortality was 14.6%. For grade I patients mortality was 6.4%, for grade II was 12.2%, for grade III was 15.2%, for grade IV was 25%, and all patients operated on in grade V died.(ABSTRACT TRUNCATED AT 250 WORDS)

Lesões múltiplas não neoplásicas na tomografia computadorizada do crânio

Multiple intracranial lesions observed in the computerized tomography ever cause worry due to the possibility of neoplastic etiology, although granulomatous diseases (parasitic or not) and vascular diseases can produce this type of lesions. Five patients with clinical picture that sugested expansive intracranial masses and that had multiple lesions in the computerized tomography that sugested neoplastic diseases are presented. In all patients the clinical evolution and/or other complementary diagnostic tests showed the non neoplastic etiology of the lesions. In three patients the lesions were due to parasitic diseases (intracranial tuberculomas in one case and neurocysticercosis in two cases) and in the other two cases the lesions were due to vascular disturbs (cerebral infarcts). The knowledge of the several diseases that can cause multiple intracranial lesions as seen in the computerized tomography allows to direct the etiologic diagnostic that is essential to introduce the correct treatment avoiding irradiation of non neoplastic lesions and unnecessary surgical procedures. In our country, granulomatous parasitic diseases, mainly neurocysticercosis, tuberculosis and fungal diseases must be considered in the differential diagnosis of multiple intracranial lesions observed in the computerized tomography, together with cerebral infarcts and neoplasms.Multiple intracranial lesions observed in the computerized tomography ever cause worry due to the possibility of neoplastic etiology, although granulomatous diseases (parasitic or not) and vascular diseases can produce this type of lesions. Five patients with clinical picture that suggested expansive intracranial masses and that had multiple lesions in the computerized tomography that suggested neoplastic diseases are presented. In all patients the clinical evolution and/or other complementary diagnostic tests showed the non neoplastic etiology of the lesions. In three patients the lesions were due to parasitic diseases (intracranial tuberculomas in one case and neurocysticercosis in two cases) and in the other two cases the lesions were due to vascular disturbs (cerebral infarcts). The knowledge of the several diseases that can cause multiple intracranial lesions as seen in the computerized tomography allows to direct the etiologic diagnostic that is essential to introduce the correct treatment avoiding irradiation of non neoplastic lesions and unnecessary surgical procedures. In our country, granulomatous parasitic diseases, mainly neurocysticercosis, tuberculosis and fungal diseases must be considered in the differential diagnosis of multiple intracranial lesions observed in the computerized tomography, together with cerebral infarcts and neoplasms.

Occurrence of neoplasms in individuals with congenital, severe GH deficiency from the Itabaianinha kindred

Growth hormone (GH) and the insulin-like growth factor I (IGF-I) have cell proliferative and differentiation properties. Whether these hormones have a role in mutagenesis is unknown. Nevertheless, severe IGF-I deficiency seems to confer protection against the development of neoplasms. Here, we report five cases of adult patients with severe and congenital isolated GH deficiency (IGHD) due to the c.57+1G>A mutation in the GHRH receptor gene, who developed tumors. Four GH-naïve subjects presented skin tumors: a 42-year-old man with a fibroepithelial polyp, a 53-year-old woman and two men (59 and 56 years old) with epidermoid skin cancers. One of these died from it after three surgeries and radiotherapy. The fifth patient was a 25-year-old woman, who had intermittently received GH replacement therapy (GHRT) from age 11 to 18, who developed an ependymoma extending from the fourth ventricle to the end of the thoracic spine. She underwent three surgical procedures, without obvious evidence of tumor recurrence during the six years follow up. These observations suggest that severe IGHD does not protect completely from development of tumors.

Extra and intradural spinal Hemangioblastoma

Hemangioblastomas do sistema nervoso central sao lesoes de baixo grau de malignidade, altamente vascularizadas, que podem se apresentar esporadicamente ou associadas com a doenca de Von Hippel-Lindau. Hemangioblastomas extradurais sao incomuns e os extra e intradurais sao ainda mais raros. Este estudo usa um caso ilustrativo e revisao da literatura para discutir as dificuldades de considerar o diagnostico correto e selecionar a melhor abordagem cirurgica. Um paciente do sexo masculino, branco, com 57 anos de idade apresentou-se com mielopatia e radiculopatia de C5 a direita. As imagens mostraram lesao extra-intradural lobulada, em forma de ampulheta, com alta impregnacao apos contraste, que ocupava o canal vertebral e estreitava o forame intervertebral de C4-C5 a direita. A resseccao total da lesao intradural foi alcancada atraves de abordagem posterior, mas a porcao extradural so pode ser parcialmente removida. Melhora total dos sintomas foi observada apos quatro meses e o tumor residual tem sido seguido clinica e radiologicamente. Embora a impressao pre-operatoria tenha sido de um schwannoma espinal, o exame histopatologico revelou hemangioblastoma grau I, segundo a OMS. Apesar de sua raridade, exames complementares atuais permitem o correto diagnostico pre-operatorio. Isto e essencial para melhor programacao cirurgica, tendo em vista as caracteristicas particulares desta lesao.