Apert syndrome: Analysis of associated brain malformations and conformational changes determined by surgical treatment

Apert Syndrome, also called acrocephalosyndactylia type 1, is characterized by craniostenosis with early fusion of sutures of the vault and/ or cranial base, associated to mid-face hypoplasia, symmetric syndactylia of the hands and feet and other systemic malformations. CNS malformations and intracranial hypertension are frequently observed in these patients. Early surgical treatment aims to minimize the deleterious effects of intracranial hypertension. Fronto-orbital advancement, the usual surgical technique, increases the intracranial volume and improves the disposition of encephalic structures previously deformed by a short skull. This study analyzes CNS alterations revealed by magnetic resonance in 18 patients presenting Apert Syndrome, and the conformational alterations in the encephalic structures after surgical treatment. The patients' age in February 2001 ranged from 14 to 322 months (m=107). Image study included brain magnetic resonance showing ventricular enlargement in five cases (27.8%), corpus callosum hypoplasia in five cases (27.8%), septum pellucidum hypoplasia in five cases (27.8%), cavum vergae in two cases (11.1%) and, arachnoid cyst in the posterior fossa in two cases (11.1%). Absence of CNS alterations was noted in 44.4% of cases. A corpus callosum morphologic index was established by dividing its height by its length, which revealed values that ranged from 0.4409 to 1.0237. The values of this index were correlated to the occurrence or absence of surgical treatment (p=0.012; t=2.83). Data analysis allowed the conclusion that the corpus callosum morphologic measure quantified the conformational alterations of the cerebral structures determined by the surgical treatment.

Acute coronary syndrome associated with continuous 5-fluorouracil infusion in a patient with metastatic colorectal cancer-a case report with a discussion on this clinical dilemma

Introduction: 5-Fluorouracil (5-FU) is considered to be the backbone of colorectal cancer (CRC) systemic therapy since the great majority of recommended regimens include its administration. A clinical picture consisting of chest pain, sometimes cardiac enzyme elevation, electrocardiogram abnormalities consistent with myocardial ischemia, and normal coronary angiogram associated with 5-FU administration have been infrequently reported. The clinical dilemma is: Which chemotherapy regimen should we use in CRC patients with a previous acute coronary syndrome (ACS) associated with 5-FU? Case Report: We describe the case of a 55-year-old otherwise healthy woman with metastatic colon adenocarcinoma who presented an ACS probably secondary to arterial vasospasm while receiving continuous intravenous 5-FU infusion (mFOLFOX6 regimen). After the ACS, the patient was treated with raltitrexate plus oxaliplatin (TOMOX) and subsequently with irinotecan plus cetuximab with no other cardiac event. Conclusion: The risk of cardiotoxicity associated with 5-FU is low but real. The probable mechanism is arterial vasospasm, as suggested by our case report. Both the use of the TOMOX regimen and irinotecan plus cetuximab seems to be safe regimens to be considered in this clinical scenario. © 2009 Humana Press Inc.

Neurocysticercosis: The enigmatic disease

Neurocysticercosis (NCC) is an infection of the central nervous system (CNS) caused by the metacestode larval form of the parasite Taenia sp. Many factors can contribute to the endemic nature of cysticercosis. The inflammatory process that occurs in the tissue surrounding the parasite and/or distal from it can result from several associated mechanisms and may be disproportionate with the number of cysts. This discrepancy may lead to difficulty with the proper diagnosis in people from low endemic regions or regions that lack laboratory resources. In the CNS, the cysticerci have two basic forms, isolated cysts (Cysticercus cellulosae = CC) and racemose cysts (Cysticercus racemosus = CR), and may be meningeal, parenchymal, or ventricular or have a mixed location. The clinical manifestations are based on two fundamental syndromes that may occur in isolation or be associated: epilepsy and intracranial hypertension. They may be asymptomatic, symptomatic or fatal; have an acute, sub-acute or chronic picture; or may be in remission or exacerbated. The cerebrospinal fluid (CSF) may be normal, even in patients with viable cysticerci, until the patients begin to exhibit the classical syndrome of NCC in the CSF, or show changes in one or more routine analysed parameters. Computed tomography (CT) and magnetic resonance imaging (MRI) have allowed non-invasive diagnoses, but can lead to false negatives. Treatment is a highly controversial issue and is characterised by individualised therapy sessions. Two drugs are commonly used, praziquantel (PZQ) and albendazole (ABZ). The choice of anti-inflammatory drugs includes steroids and dextrochlorpheniramine (DCP). Hydrocephalus is a common secondary effect of NCC. Surgical cases of hydrocephalus must be submitted to ventricle-peritoneal shunt (VPS) immediately before cysticidal treatment, and surgical extirpation of the cyst may lead to an absence of the surrounding inflammatory process. The progression of NCC may be simple or complicated, have remission with or without treatment and may exhibit symptoms that can disappear for long periods of time or persist until death. Unknown, neglected and controversial aspects of NCC, such as the impaired fourth ventricle syndrome, the presence of chronic brain oedema and psychic complaints, in addition to the lack of detectable glucose in the CSF and re-infection are discussed. © 2011 Bentham Science Publishers.

Forámenes emisarios del cráneo humano: Características anatómicas y sus relaciones con la neurocirugía clínica

The recognition of emissary foramens is important not only for understanding the regional neurovascular anatomy, but also to distinguish normal from potentially abnormal structures. Thus, the aim of this study was to review the literature on anatomical and clinical aspects of the mastoid, parietal and sphenoid emissary foramens. It was found that the emissary foramen presents importance in clinical practice because it acts as a route of spread of extracranial infection to the intracranial structures and also possible complications in neurosurgery, due to its influence in the performance of techniques such as radiofrequency rhizotomy for treatment of trigeminal neuralgia. The anatomical knowledge of the emissary foramens is important due to variability in their incidence in the human skull and its relation to the dura mater sinuses.

Paranasal sinus infection causing orbital subperiosteal abscess: Surgical management of this devastating entity

Background: Orbital infection is an uncommon devastating infection and is usually a complication of paranasal sinus infection. Without appropriate treatment, orbital infection may lead to serious complications, even death. Prompt treatment is mandatory to avoid visual loss or intracranial complications. The literature shows that initially, intravenous antibiotics should be administered, and after 48 h, if no improvement appears, the affected orbit and the sinuses must be surgically drained. The authors describe two cases of orbital cellulitis with a brief literature review. Case report: The authors describe two cases of orbital abscess caused by paranasal sinus infection. In case 1, the patient presented a decreased visual acuity associated with ophthalmoplegia of the right eye. In case 2, the patient presented a decreased visual acuity. Thus, administration of intravenous antibiotic combined with surgical drainage was performed. After surgical procedure, eye movements were normalized in case 1, and in both patients, the visual acuity returned to normal parameters. Discussion: The authors recommend early surgical drainage with parenteral antibiotic administration and careful postoperative observations by monitoring the signs and symptoms of the orbital complaint. © 2012 Springer-Verlag.

Espectros Clínicos da Trombose Venosa Cerebral

Introduction. Cerebral Venous Thrombosis has a highly variable clinical presentation. Four major syndromes had been described in patients with cerebral venous thrombosis: isolated intracranial hypertension, focal neurological deficits, focal or generalized seizures and disturbances of consciousness and cognitive dysfunction. Method. We describe five consecutive patients admitted to our service with a diagnosis of cerebral venous thrombosis, highlighting the different possibilities of clinical presentation and prognosis. Discussion. The diagnosis of cerebral venous thrombosis should be considered in patients with acute, subacute or chronic headache, with or without signs of intracranial hypertension or focal deficits, even in the absence of cerebrovascular risk factors. Treatment should be started as soon as the diagnosis is confirmed and consists of reversal of the underlying cause when known, control of seizures and intracranial hypertension, and antithrombotic therapy.

Odontogenic infection causing orbital cellulitis in a pediatric patient

Odontogenic abscess can become an orbital cellulitis, causing potentially serious intracranial and orbital complications. The full clinical complications from odontogenic orbital cellulitis in a pediatric patient are rarely seen daily in hospital emergency departments. Thus, odontogenic orbital cellulitis still remains a rarity, resulting in a medical challenge. With this in mind, this study aimed to describe a case of periorbital and orbital cellulitis resulting from odontogenic origin in a 6-year-old patient who was successfully treated by performing intravenous antibiotic administration combined with surgical drainage. Copyright © 2013 by Mutaz B. Habal, MD.

Metilação e expressão do gene BRCA1 em meningiomas

Pós-graduação em Pesquisa e Desenvolvimento (Biotecnologia Médica) - FMB

Estudo experimental de sistemas de drenagem externa do líquido cefalorraquidiano

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Simulação hidrodinâmica e caracterização experimental de mecanismos anti-sifão em sistemas de drenagem externa de líquido cefalorraquidiano

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Relação entre os sinais clínicos neurológicos e os achados tomográficos de 20 cães com suspeita de neoplasia intracraniana

Pós-graduação em Medicina Veterinária - FMVZ

Efeitos do sevofluorano e do desfluorano sobre variáveis intracranianas e hemodinâmicas em cães

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Infusão contínua de propofol ou de etomidato em cães normocapneicos: efeitos intracranianos e hemodinâmicos

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)