Epileptogenic role of occult temporal encephalomeningocele: Case-control study.

OBJECTIVE: The purpose of this case-control study is to evaluate the prevalence of occult temporal encephalomeningocele (OTE) in patients with temporal lobe epilepsy (TLE) of unknown etiology presenting to an epilepsy center, independently from drug sensitivity. METHODS: We studied 95 patients with TLE (51 female, mean age 49.4 ± 17.1 years) and 151 controls (88 female, mean age 54.1 ± 21.0 years) using a 1.5T brain MRI, including balanced steady-state gradient echo sequences, targeted to the temporal lobes. RESULTS: OTE was found in 5.2% of the TLE population (9.5% of drug-resistant TLE) and in none of the controls (p = 0.008). Two patients with OTE and drug-resistant TLE became seizure-free after lesionectomy (follow-up 18-24 months). CONCLUSION: OTE is not a rare finding in unselected patients with TLE of unknown origin, provided that it is carefully searched. The absence of OTE in a large group of nonepileptic controls adds evidence to its epileptogenic role.

Direito ao patrimônio genético como direito transindividual: considerações sobre o caso Sangue Yanomami / Derecho al patrimonio genético como derecho transindividual: consideraciones sobre la controversia de la sangre Yanomami / Dret al patrimoni genètic com a dret transindividual: consideracions sobre la controvèrsia de la sang Yanomami / Right to Genetic Heritage as Transindividual Right: considerations about the Yanomami Blood Controversy

O presente artigo revisita a controvérsia sobre o Sangue Yanomami, no qual uma aldeia do povo yanomami exigiu a repatriação de amostras de sangue indígena coletada por pesquisadores estrangeiros nos anos 1960, quando eles estiveram na floresta amazônica. Ela é apresentada como um leading case no processo de reconhecimento tanto do direito individual à identidade genética (direito ao genoma) como do direito transindividual ao patrimônio genético da Humanidade (direito do genoma). O caso é relevante, dentre outros, porque permite que se compreenda a postulação dos yanomamis a partir (I) do direito ao genoma exercido coletivamente por uma comunidade e (II) do direito difuso que têm a Humanidade em exigir respeito ao material genético que possua um valor cultural (imaterial) específico (não científico) para aquela comunidade

Este artículo retoma la controversia de la Sangre Yanomami, en que una aldea yanomami exigió la repatriación de las muestras de sangre indígena recolectadas por investigadores extranjeros en la jungla amazónica en los años ’60. Se muestra como un caso destacado en el proceso de reconocimiento de dos derechos: a la identidad genética (derecho al genoma) y el derecho transindividual al patrimonio genético de la Humanidad (derecho genómico). Este caso es relevante, entre otros, porque permite entender la demanda yanomami del (I) derecho a genoma, ejercido colectivamente por la comunidad, y (II) el derecho de la Humanidad a exigir respeto por su material genético en virtud de sus valores culturales específicos (inmateriales) para la comunidad

Aquest article reprèn la controvèrsia de la Sang Yanomami, en la qual un poblat yanomami va exigir la repatriació de les mostres de sang indígenes recol·lectades per investigadors estrangers a la jungla amazònica en els anys ’60. Es tracta d’un cas remarcable en el procés de reconeixement de dos drets: el dret a la identitat genètica (dret al genoma) i el dret transindividual al patrimoni genètic de la humanitat (dret genòmic). Aquest cas és rellevant, entre d’altres coses, perquè permet entendre la demanda yanomami del (I) dret a genoma, exercit col·lectivament per la comunitat, i (II) el dret de la humanitat a exigir respecte pel seu material genètic en virtut dels seus valors culturals específics (immaterials) per la comunitat

This paper revisits the Yanomami Blood Controversy, in which a yanomami village demanded repatriation of indigenous blood samples collected by foreign researchers in ‘60s when they were in Amazon jungle. It is shown as a leading case in the process of recognition both individual right to genetic identity (right to genome) as well as transindividual right to genetic patrimony of Humanity (genome law). This case is relevant, among others, because it allows one to understand the yanomami demand from (I) right to genome collectivelly exercised by the community and (II) Humanity's right to demand respect to genetic material for its specific cultural values (immaterial) for that community

Combined thoracic endovascular aortic repair and neurosurgical intervention for injury due to posterior spine surgery.

Aortic injuries represent a rare but life-threatening complication of spinal surgery. Perforation of the aorta due to pedicle screw penetration or misplacement can lead to immediate bleeding with hemodynamic instability or to pseudoaneurysm development with delayed risk of rupture, which can occur weeks to months later. Recently, thoracic endovascular aortic repair (TEVAR) in aortic trauma has contributed to a reduction of both mortality and morbidity. The literature on this subject is reviewed.

Low-back pain as the presenting sign in a patient with a giant, sacral cellular schwannoma: 10-year follow-up.

Giant sacral tumors present unique challenges to surgeons because there is no established consensus regarding the best treatment options. The authors report on the care of and outcome in a patient presenting with low-back pain only, who underwent preoperative biopsy sampling and subsequent embolization of the feeding vessels of a giant, sacral cellular schwannoma. The main procedure was performed via a combined posterior-anterior approach with complete microsurgical removal of the tumor, without the use of instrumentation, bracing, or adjuvant radio- and chemotherapy. At the 10-year follow-up, no evidence of residual tumor, recurrence, or instability was recognizable. Giant, sacral cellular schwannomas can be aggressively completely removed without any significant morbidity, achieving long-term control of the disease.

Difficulties in diagnosing slowly progressive mucopolysaccharidosis VI: A case series.

An Erratum for this article can be found here: http://iospress.metapress.com/content/e16437020701m0u5/?p=df8dd6709cf44367a0c0e5d917aaeddf&pi=11We describe the cases of two adult sisters recently diagnosed with the attenuated form of mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome). MPS VI is a rare, clinically heterogeneous lysosomal storage disorder that is characterized by a deficiency in the glycosaminoglycan-degrading enzyme arylsulfatase B. Both cases had been misdiagnosed for over 30 years despite the presence of several characteristics of the disease, including short stature (mild), coarse facial features, skeletal dysmorphisms, carpal tunnel syndrome, heart valve disease, and spinal cord compression, which together are suggestive of a lysosomal storage disease. Awareness about the clinical features of MPS VI should be communicated amongst treating neurologists, rheumatologists and other specialists who are involved in the healthcare decisions of these patients with presenting symptoms, so they can refer them to specialized centers for proper diagnosis and treatment.

Atypical meningioma of the sylvian fissure.

Meningiomas are meningothelial cell neoplasms that account for approximately 25% of all primary intracranial tumors. Most meningiomas are slow-growing benign lesions, and they are usually attached to the inner surface of the dura mater. Nevertheless, since the first description by of Cushing and Eisenhardt, many meningiomas without dural attachment have been reported. A subgroup located in the sylvian fissure (also called deep sylvian meningiomas) has been described, and these represent a radiological and neurosurgical challenge. We describe an atypical sylvian fissure meningioma in a 23-year-old male with a brief history of headache and mild hemiparesis. We also review the pertinent literature defining the major epidemiological, clinical, radiological and surgical characteristics of these rare tumors.

Renal cell carcinoma and intradural spinal metastasis with cauda equina infiltration: case report.

STUDY DESIGN: A case report and clinical discussion. OBJECTIVE: To describe the diagnosis, pathophysiology, and treatment of a papillary renal cell carcinoma that developed an intradural spinal mass with cauda equina infiltration. SUMMARY OF BACKGROUND DATA: Cancers that metastasize intradurally to the spinal canal are uncommon, accounting for the 6% of all spinal metastases. Those from renal cell carcinoma are especially unusual. Only 3 reports that describe the spread of metastatic renal cell carcinoma to the cauda equina have been published to our knowledge. METHODS: A female patient had undergone nephrectomy for the treatment of the papillary renal cell carcinoma 2 years before, and only 1 localization (at the genital tract) was previously diagnosed. The patient presented with lower back pain irradiated to both legs, vesical incontinence, and severe paraparesis. RESULTS: Preoperative magnetic resonance images of the lumbar spine showed an intradural spinal mass from L3-L5 with infiltration of the cauda equina; the lesion measured 62 mm craniocaudally and 16 mm anteroposteriorly, and thus occupied the majority of the intrathecal space at that level. The magnetic resonance images, surgical treatment, and related pathophysiology are reviewed. CONCLUSIONS: The majority of cauda equina tumors are primary tumors, and metastases are very rare. To our knowledge, this is the fourth case described of metastasis of renal cell carcinoma at the cauda equina and the first of papillary type.

Renal cell carcinoma and intradural spinal metastasis with cauda equina infiltration: case report--part II.

STUDY DESIGN: A case report and clinical discussion. OBJECTIVE: To describe the diagnosis, pathophysiology, and treatment of a papillary renal cell carcinoma that developed an intradural spinal mass with cauda equina infiltration. SUMMARY OF BACKGROUND DATA: Cancers that metastasize intradurally to the spinal canal are uncommon, accounting for the 6% of all spinal metastases. Those from renal cell carcinoma are especially unusual. Only 3 reports that describe the spread of metastatic renal cell carcinoma to the cauda equina have been published to our knowledge. METHODS: A female patient had undergone nephrectomy for the treatment of the papillary renal cell carcinoma 2 years before, and only 1 localization (at the genital tract) was previously diagnosed. The patient presented with lower back pain irradiated to both legs, vesical incontinence, and severe paraparesis. RESULTS: Preoperative magnetic resonance images of the lumbar spine showed an intradural spinal mass from L3-L5 with infiltration of the cauda equina; the lesion measured 62 mm craniocaudally and 16 mm anteroposteriorly, and thus occupied the majority of the intrathecal space at that level. The magnetic resonance images, surgical treatment, and related pathophysiology are reviewed. CONCLUSIONS: The majority of cauda equina tumors are primary tumors, and metastases are very rare. To our knowledge, this is the fourth case described of metastasis of renal cell carcinoma at the cauda equina and the first of papillary type.

Impairment of executive functions in a patient with a focal lesion in the anterior cingulate cortex. Evidence from neuropsychological assessment.

Patients with naturally occurring lesions involving the anterior cingulate cortex are rare and there thus exist very few reports of focal lesions in this area. We report a longitudinal study of a new case of selective anterior cingulate damage due to the presence of an angiocavernoma at the junction of the anterior third with the middle third of the right gyrus cinguli. Before surgery, the results of several, different tests suggested a significant impairment of executive functions, including deficits in planning, monitoring of ongoing behavior, and strategy shifting, as well as an exaggerated susceptibility to retroactive interference. Most of these symptoms disappeared completely or almost completely after the surgical removal of the angiocavernoma, although exaggerated susceptibility to interference was found to persist four months after surgery.