Unusual bifrontal cerebral infarction related to pituitary apoplexy. An uncommon presentation and literature review.

Background: Pituitary apoplexy (PA) is a syndromic condition described in 1950. The main symptoms are headache, visual impairment, ophthalmoplegia, and hypopituitarism. The relationship between stroke and PA is uncommon and two mechanisms are described: vascular compression and vasospasm. Case Report: A 65-old-year man presented with severe headache, vomiting, ophthalmoplegia, and somnolence. Radiological examinations showed an expansive sellar and suprasellar lesion with a heterogeneous signal, besides Diffusion-weighted imaging (DWI) restriction in the bifrontal area was present. The findings were compatible with PA and stroke. Conclusion: PA leading to cerebral infarction is a rare condition that presents high morbidity and mortality levels. There are two main mechanisms related: direct arterial compression and arterial vasospasm. The cases must be conducted as neuroendocrinological emergencies and surgical management is a key point to better the prognosis of patients.

Pure endoscopic transsphenoidal treatment of skull base ameloblastoma with intracranial extension: Case report and literature review.

BACKGROUND: Ameloblastoma is a benign locally invasive lesion that represents 1% of all oral tumors. Epidemiological characteristics are variable in the literature. The most common origin sites are mandible and maxilla. Rarely presents metastasis, but the skull base, lymph nodes, and the lung are described as metastatic sites. Low recurrence rates were reported by the authors when surgical treatment achieved complete resection. CASE DESCRIPTION: A female patient, 19 years old presenting moderate headache associated with nausea, vomiting, left facial hypoesthesia, and low visual acuity. Resonance image showed a heterogeneous expansive solid formation in sphenoid bone and clivus with neoplastic aspect. Signs of dissemination due to contiguity and invasion of skull base structures, especially cavernous sinus and internal carotid artery, determining also compression of the brainstem. First, an endoscopic biopsy was performed with otorhinolaryngology service. The pathological study showed histological characteristics of ameloblastoma. After, the patient was submitted to endoscopic surgery for resection of tumor. CONCLUSION: Ameloblastoma is a rare tumor with benign behavior and slow growing. It arises from odontogenic epithelium and accounts 1% of all oral tumors. The mandible and maxilla are the most common sites of origin. Ameloblastoma with intracranial involvement is a rare presentation with few literature reviews. A long time illness course and multiple surgeries are characteristics present in the majority of cases described. Total resection surgery is the treatment of choice and endoscopic transnasal resection is a viable option.

Intraparenchymal Epidermoid Cyst Close to Broca Area-Awake Craniotomy and Gross Total Resection.

BACKGROUND: Epidermoid tumors, or epidermoid cysts (ECs), are benign, slow-growing, congenital, and rare lesions that represent approximately 0.2%-1.8% of all intracranial tumors. Intraparenchymal ECs are very rare lesions that may account for 1.5% of all intracranial epidermoid tumors; frontal lobe involvement is found in 39.2% of intraparenchymal ECs. We present a case using awake craniotomy to achieve maximal safe gross total resection of a rare intraparenchymal EC close to Broca area in a bilingual patient. CASE DESCRIPTION: A 45-year-old man presented with a generalized seizure episode. He was initially treated with levetiracetam, which led to renal failure. Imaging findings demonstrated an intraparenchymal left frontal EC with peripheral coarse calcifications at Broca area. As the patient was bilingual and had a normal neurologic examination, we performed a left frontal awake craniotomy under local anesthesia so as to map both languages, using the motor task and a test for language monitoring, alternating a naming task in Portuguese and English and a semantic task in Portuguese. A gross total resection was achieved with no neurologic deficits. Histopathologic examination confirmed the diagnosis of an epidermoid cyst. After 1 year, the patient is still seizure-free. CONCLUSIONS: Awake surgery proved to be a useful tool for complete resection of the capsule even in a very eloquent language area. In multilingual patients with benign intra-axial lesions, intraoperative mapping should be performed for all the languages in which the patient is fluent to avoid postoperative neurologic deficits.

IDH1-mutant primary intraventricular gliosarcoma: Case report and systematic review of a rare location and molecular profile.

BACKGROUND: Gliosarcoma (GS) is classified as an IDH-wild-type variant of glioblastoma (GBM). While GS is already an unusual presentation of GBM, IDH1-mutant cases are especially rare. We present an IDH1-mutant primary intraventricular GS case report and a systematic review of the molecular profile in GS correlating to the prognostic and pathogenesis of IDH1/2 mutations. CASE DESCRIPTION: A 44-years-old man presented with ongoing fatigue symptoms and a new-onset intense occipital headache. The patient complained of memory loss, dyscalculia, and concentration difficulties. An MRI revealed a bihemispheric intraventricular mass crossing the midline through the corpus callosum and infiltrating the trigone of the lateral ventricles, hypointense, and hyperintense on the T1- and T2-weighted image. We performed a microsurgical resection with a transparietal transsulcal approach; however, the contralateral mass was attached to vascular structures and we decided to reoperate the patient in another moment. The histopathological study showed a Grade IV tumor and the immunohistochemistry confirmed the diagnosis of GS. The patient presented progressive neurologic decline and died 45 days after the surgical approach. CONCLUSION: We did two systematic reviews studies from PubMed, EMBASE, MEDLINE, Cochrane, and SCOPUS databases, and included molecular and intraventricular studies of GS. We performed further meta-analysis using OpenMetaAnalyst™ software. We conducted a forest plot with the molecular profile of GS. When correlated IDH1 mutation versus tp53 mutation, we found an odds ratio (OR) of 0.018 (0.005-0.064) and P < 0.001. Moreover, we compared IDH1 mutation versus MGMT methylation (P = 0.006; OR = 0.138 [0.034-0.562]). The studies evaluating the molecular profile in GS prognostics are often extended from all GBMs despite specifics GBM variants (i.e., GS). We found a correlation between IDH1 mutation expression with tp53 and MGMT expression in GS, and future studies exploring this molecular profile in GS are strongly encouraged.

Tenth case of bilateral hemifacial spasm treated by microvascular decompression: Review of the pathophysiology.

BACKGROUND: Bilateral hemifacial spasm (BHFS) is a rare neurological syndrome whose diagnosis depends on excluding other facial dyskinesias. We present a case of BHFS along with a literature review. METHODS: A 64-year-old white, hypertense male reported involuntary left hemiface contractions in 2001 (aged 50). In 2007, right hemifacial symptoms appeared, without spasm remission during sleep. Botulinum toxin type A application produced partial temporary improvement. Left microvascular decompression (MVD) was performed in August 2013, followed by right MVD in May 2014, with excellent results. Follow-up in March 2016 showed complete cessation of spasms without medication. RESULTS: The literature confirms nine BHFS cases bilaterally treated by MVD, a definitive surgical option with minimal complications. Regarding HFS pathophysiology, ectopic firing and ephaptic transmissions originate in the root exit zone (REZ) of the facial nerve, due to neurovascular compression (NVC), orthodromically stimulate facial muscles and antidromically stimulate the facial nerve nucleus; this hyperexcitation continuously stimulates the facial muscles. These activated muscles can trigger somatosensory afferent skin nerve impulses and neuromuscular spindles from the trigeminal nerve, which, after transiting the Gasser ganglion and trigeminal nucleus, reach the somatosensory medial posterior ventral nucleus of the contralateral thalamus as well as the somatosensory cortical area of the face. Once activated, this area can stimulate the motor and supplementary motor areas (extrapyramidal and basal ganglia system), activating the motoneurons of the facial nerve nucleus and peripherally stimulating the facial muscles. CONCLUSIONS: We believe that bilateral MVD is the best approach in cases of BHFS.

Long-Term Follow-Up of Anterior Pituitary Deficiency after Aneurysmal Subarachnoid Hemorrhage: Prospective Cohort.

OBJECTIVE: The aim of this study was to evaluate the prevalence of hypopituitarism in the acute stage after aneurysmal subarachnoid hemorrhage (SAH) as well at the chronic stage, at least 1 year after bleeding, to assess its implications and correlation with clinical features of the studied population. PATIENTS AND METHODS: This was a prospective cohort study that evaluated patients admitted between December 2009 and May 2011 with a diagnosis of SAH secondary to cerebral aneurysm rupture. Clinical and endocrine assessment was performed during the acute stage after hospital admission and before treatment at a mean of 7.5 days (SD ± 3.8) following SAH, and also at the follow-up visit at a mean of 25.5 months (range: 12-55 months) after the bleeding. RESULTS: Out of the 119 patients initially assessed, 92 were enrolled for acute stage, 82 underwent hormonal levels analysis, and 68 (82.9%) were followed up in both acute and chronic phases. The mean age and median age were lower among patients with dysfunction in the acute phase compared to those without dysfunction (P < .05). The prevalence of dysfunction in the acute phase was higher among patients with hydrocephalus on admission computed tomography (57.9%) than among those without it (P < .05). At chronic phase, there was an association between dysfunction and Hunt & Hess scale score greater than 2 (P < .05). CONCLUSIONS: We believe that there is not enough literature evidence to incorporate routine endocrinological evaluation for patient victims of SAH, but we should always keep this differential diagnosis in mind when conducting long-term assessments of this population.

Surgical treatment of the intracranial pial arteriovenous fistula.

BACKGROUND: Pial arteriovenous fistula (PAVF) is a rare vascular condition comprising of one or more arterial vessels that are in direct communication with the draining veins. The condition is also characterized by the absence of a nidus. Due to high blood flows, varicose systems adjacent to the fistula appear. The key characteristic of the arteriovenous direct transition is that it offers a treatment option in which interruption of the blood flow can occur without removing the entire lesion. This study presents two cases of PAVF. CASE DESCRIPTION: The first case is of a 59-year-old male with lesions in the frontal region, fed by a branch of the right anterior cerebral artery and drained by the frontal basal vein to the sphenoparietal sinus. The second case is of a 3-year-old child with a lesion in the right anterior frontal lobe, fed by a branch of the right middle cerebral artery, which drains into the Trolard vein and was associated with large a venous varix. CONCLUSION: PAVF is a disease characterized by its rarity, and knowledge of PAVF's clinical presentation is of vital importance in early diagnosis. The treatment of the condition consists of an occlusion of the supply vessel, which can be done by endovascular, microsurgical, or both procedures. Both the cases were successfully treated by microsurgical procedure.

Multiple supratentorial intraparenchymal hemorrhage after posterior fossa surgery.

BACKGROUND: The intraparenchymal supratentorial hemorrhages after interventions of the posterior fossa is a very rare complication, with very little literature and its precise incidence is unknown (range of 0.4-1.6%). It possesses potentially an etiology diverse from that associated with other postoperative bleeding. CASE DESCRIPTION: A white, 23-year-old female, with no history of coagulation disorders or other diseases, was referred to our hospital with a large ependymoma, which extended from the floor of the fourth ventricle, emerged from the foramen of Magendie and descended to the C2 level. The patient was submitted to surgical treatment and during resection of the lesion, when near the vagal trigone, the patient presented great pressure lability. In the immediate postoperative period, the patient did not have a level of consciousness sufficient to tolerate extubation. Brain computed tomography (CT) was carried out, which showed multiple supratentorial hemorrhages. On the ninth day of the postoperative period, there was a sudden neurological worsening and anisocoria. A new brain CT was carried out [Figure 4], which demonstrated a diffuse cerebral edema. In spite of the introduction of clinical measures for the control of diffuse cerebral edema, the patient evolved to brain death. CONCLUSIONS: The principal measures in the management of these cases include early diagnosis, detection of possible coagulation disorders, continual monitoring, and maintenance of adequate cerebral perfusion. Surgical treatment is recommended in cases of the presence of mass effect or diffuse edema not yielding to clinical treatment. High rates of mortality and morbidity are observed.

E-learning for neurosurgeons: Getting the most from the new web tools.

As open access resource, the role of Internet has been increasing in our professional life. There are several emergent new tools that can facilitate and make it more efficient to get accurate and reliable information. In this article, we discuss how we can manage to get the most from these new instruments, like blogs, Facebook, Twitter, and LinkedIn, in order to improve clinical practice. With good sense and some caution, these can turn to be of valuable help in our careers.

Open-ring enhancement in pseudotumoral multiple sclerosis: important radiological aspect.

Introduction. Observation of open-ring enhancement in magnetic resonance imaging (MRI) is considered a specificity marker for diagnosing pseudotumoral multiple sclerosis (MS). This finding is of great value in the differential diagnosis of tumefactive lesions. Case Report. We describe a 55-year-old white woman, with previous history of ovarian cancer and recent history of fatigue and bilateral retroorbital pain. Important bilateral visual impairment evolved over one month. Physical examination detected the presence of right homonymous hemianopia. Cranial MRI showed an expanding lesion with open-ring enhancement. Given the range of diagnostic possibilities, a stereotactic biopsy was performed, and histopathological examination was consistent with an active demyelinating disease. The patient was treated with 1 g of methylprednisolone and symptoms improved following a significant reduction in the lesion. Conclusions. We highlight the MRI results suggestive of pseudotumoral MS, especially open-ring enhancement, which is an important radiologic aspect to diagnosis and can assist in avoiding unnecessary biopsies.

Vitamin B12 extensive thoracic myelopathy: clinical, radiological and prognostic aspects. Two cases report and literature review.

The myelopathy caused by vitamin B12 deficiency is known as subacute combined degeneration. It is rare, but a well known cause of demyelination of the dorsal columns of the spinal cord. The magnetic resonance imaging is characterized by an increased signal on T2-weighted images involving the posterior columns of cervical and thoracic cord. There have been few cases in literature with extensive lesions (more than seven levels) of the thoracic spinal cord. The clinical and radiological improvements are possible if the replacement of vitamin B12 is initiated precocious. We present two rare cases of extensive thoracic myelopathy due to vitamin B12 deficiency. The first is a young woman with complete clinical recovery and important radiologic improvement after early treatment. In addition, the second case is an older man with partial response to the treatment. Those cases illustrate the importance of considering vitamin B12 deficiency in any patient, who presents with myelopathy.

Skin closure in vascular neurosurgery: A prospective study on absorbable intradermal suture versus nonabsorbable suture.

BACKGROUND: The craniotomy performed with minimal hair removal and closure with intradermal suture alone is an option in neurosurgical procedures, which can help faster psychological recovery of the patient, as it allows a better cosmetic result. This study is aimed at evaluating if such method is safe and effective, compared with continuous skin sutures with 2-0 nylon. METHODS: We analyzed the sutures in 117 patients undergoing craniotomies for cerebral aneurysm clipping. In the case group (n = 49), closure of the scalp was performed only with intradermal absorbable sutures using wire Monocryl(®) 2-0. In the control group (n = 68), closure was performed with continuous suture using 2-0 nylon. RESULTS: The case group was composed of 49 patients in whom just intradermal suture was performed. One (2.2%) patient developed wound infection and was given proper medical treatment. No cases of dehiscence or cerebrospinal fluid leaks were observed. The control group was composed of 68 patients in whom the skin was closed with 2-0 nylon continuous suture. Three (5.3%) patients developed wound infection and were given proper medical treatment. There were no cases of wound dehiscence. The overall infection rate in the control group was 4%. There was no statistically significant difference in the number of wound infections between the two groups (P = 0.73). CONCLUSION: The closure with intradermal suture alone in craniotomies is as safe as the traditional skin closure with nylon sutures, besides eliminating the need for suture removal and providing a cosmetic advantage.

Blogs for neurosurgeons.

Blogs are useful tools to research and to disseminate information. As they allow people who do not have specific knowledge on the building of sites to post content on the internet, they turned out to be very popular. In the past years, there has been a rapid expansion of blogs on several subjects and nowadays there are over 156 million blogs online. Neurosurgery was not out of this wave, and several blogs related to it can be found on the internet. The objective of this paper is to describe, in general, the functions of a blog and to provide initial guidance for the creation and the adequate use of neurosurgical blogs. Some interesting blogs and their features are also listed as examples.

Importance of recognizing sentinel headache.

BACKGROUND: Sentinel headache (SH) is a kind of secondary headache and is characterized as sudden, intense, and persistent, preceding spontaneous subarachnoid hemorrhage (SAH) by days or weeks. METHODS: Eighty-nine consecutive patients with a diagnosis of spontaneous SAH were evaluated following admission to the Neurosurgical Service at Santa Casa Hospital, Belo Horizonte, between December 2009 and December 2010. RESULTS: Out of the 89 patients, 64 (71.9%) were women. Mean age was 48.9 years (SD ± 13.4, ranging from 18 to 85 years). Twenty-four patients (27.0%) presented SH, which occurred, in average, 10.6 days (SD ± 13.5) before a SAH. No statistically significant differences were observed between the presence of SH and gender, arterial hypertension and migraine (P > 0.05), Glasgow Comma Scale (GCS) and World Federation of Neurological Surgeons (WFNS) scale at admission. CONCLUSION: The prevalence of SH was 27% in this study but no related factors were identified. Therefore, further clarification of this important entity is required so as to facilitate its recognition in emergency services and improve the prognosis of patients with cerebral aneurysms.

Pseudotumoral form of cerebral Schistosomiasis mansoni.

OBJECTIVE: To describe published cases of cerebral mansoni schistosomiasis and three others and discuss the diagnosis and treatment of cerebral pseudotumoral schistosomiasis. CASE DESCRIPTIONS: In case 1, a 20-year-old man presented with occipital headache, intense dizziness, visual alterations, nausea, decreased appetite, and asthenia. Cranial computed tomography (CT) revealed an expansive cerebellar lesion in the right hemisphere with no contrast enhancement. The patient had complete resection of the lesion. Anatomicopathological examination revealed a schistosomal granuloma. In case 2, a 22-year-old man presented with generalized tonic-clonic seizure. Cranial CT and magnetic resonance imaging (MRI) revealed an expansive bilateral middle frontal lesion, with contrast uptake close to the cingulate gyrus and corpus callosum. The patient underwent left frontal craniotomy, and an interhemispheric approach was used to resect part of the lesion. In case 3, a 32-year-old man presented with generalized tonic-clonic seizures. Cranial CT showed a hyperdense intense intracranial expansive lesion that presented contrast uptake in the left temporal region. The patient had complete resection of the lesion. CONCLUSIONS: A surgical approach with lesion resection or stereotaxic biopsy is warranted to determine the diagnosis definitively. Antiparasitic drugs must be administered to complete treatment.

Wegener's granulomatosis: prevalence of the initial clinical manifestations--report of six cases and review of the literature.

OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG) in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF), as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study identified 49 patients, 15 (31%) males and 34 (69%) females. Systemic disease was observed in 35 patients (73%): 28 adults, 5 children, and 2 teenagers. Limited disease was observed in 13 adults and 1 child. The average age of onset in adults was 42.2 years (18 to 65 years). Acute clinical manifestations, with the onset of symptoms less than three months before the diagnosis, were observed in 41% (20/49) of the patients, and the insidious presentation in 59% (29/49) of the patients. The prevalence of the initial clinical manifestations in adults with systemic disease (n = 28) was 64% (18/28), upper airways, 36% (10/28), lungs, 18% (5/28), kidneys, 25% (7/28), eyes, 11% (3/28) skin, 25% (7/28), musculoskeletal, and 7% (2/28), neurological. In adults (n = 13) with limited disease, prevalent symptoms included: upper airway, 84% (10/13), eyes, 23% (3/13), and lungs, 15% (2/13). CONCLUSION: The prevalence of the initial clinical manifestations of WG in Brazil was similar to that reported in the literature. The lack of specific symptoms may delay diagnosis cases with insidious presentation of the disease and increase the morbidity and mortality in acute disease.

Headache associated with pituitary tumors.

The objective of this study is to analyze the presence of headache in pituitary tumors and their characteristics, the relationship between pituitary tumor size, biological type, local extension and intrasellar pressure (ISP). This is a prospective study, of 64 consecutive patients presenting with primary pituitary masses at Neuroendocrinological Department of General Hospital of Fortaleza from October 2005 to December 2006. We analyzed sex, age, headache (laterality, site, severity, quality, frequency, duration, associated symptoms, time of onset, trigger, alleviating factors and familial history) and tumor characteristics (type, size, quiasmatic compression, cavernous sinus invasion, sella turcica destruction, cystic or solid mass and ISP). We observed a statistic significant factor between pituitary tumor and tumor size, optic compression, sellar destruction, cavernous sinus invasion and ISP. Biochemical-neuroendocrine factors, mainly in prolactinomas, seem to be an important factor in the determination of headache. The presence of headache in pituitary tumor is related to a combination of factors, including ISP, tumor extension, relationship with the sellar structures, patient predisposition, familial history, and functional disturbance within the hypothalamo-pituitary axis.