Syringobulbia associated with posterior fossa meningioma: a review of the literature.

PURPOSE: Syringomyelia can be defined as a degenerative, progressive and chronic spinal cord disease. Its association with tumors of the posterior cranial fossa (PCF) is a rare condition. METHODS: The authors report a rare case of syringobulbia consequent to a meningioma originating from PCF in a 17-year-old female, discussing the pathogenetic mechanism of development and the resolution of the syrinx cavity after surgical procedure. RESULTS: The postoperative period was uneventful without complications. At 6-month follow-up, MRI revealed complete tumor removal with resolution of the syrinx cavity. CONCLUSIONS: In cases of syringomyelia and tonsillar herniation associated with PCF meningioma, the tumor resection allows to eliminate the mass effect and increases the size of the posterior fossa with the progressive ascent of the cerebellar tonsils and the consequent reduction of their downward movement with systolic pulsation. The re-establishment of a normal anatomical condition led to the gradual disappearance of syrinx and hydrocephalus.

Extracranial Jugular Foramen Schwannomas Treated with the Extreme Lateral Juxtacondylar Approach: Surgical Technique and Our Experience.

BACKGROUND: Schwannoma that arises in the jugular foramen (JF) represents an important challenge for neurosurgeons for its precise location, extension, and neurovascular relationship. Nowadays, different managements are proposed. In this study, we present our experience in the treatment of extracranial JF schwannomas (JFss) with the extreme lateral juxtacondylar approach (ELJA). OBJECTIVE: To present our experience in the treatment of extracranial JF schwannomas (JFss) with the ELJA. METHODS: Between January 2013 and January 2017, 12 patients with extracranial JFs underwent surgery by ELJA. All lesions were type C of the Samii classification. Indocyanine green videoangiography was used to evaluate the relationship between the internal jugular vein and the tumor and to control the presence of spasm in the vertebral artery. RESULTS: A complete exeresis was achieved in 9 patients while in 3 patients, it was subtotal. The complete regression of symptoms was obtained in 7 patients with a total resection. The remaining cases experienced a persistence of symptoms. CONCLUSION: The success of this surgery is achieved through a management that starts from the patient's position. We promote an accurate evaluation of JFs through the Samii classification: Type C tumors allow the use of ELJA that reduces surgical complications. Furthermore, we recommend the use of indocyanine green videoangiography to preserve the vessels and prevent vasospasm.

Acute stress alters transcript expression pattern and reduces processing of proBDNF to mature BDNF in Dicentrarchus labrax.

BACKGROUND: Stress involves alterations of brain functioning that may precipitate to mood disorders. The neurotrophin Brain Derived Neurotrophic Factor (BDNF) has recently been involved in stress-induced adaptation. BDNF is a key regulator of neuronal plasticity and adaptive processes. Regulation of BDNF is complex and may reflect not only stress-specific mechanisms but also hormonal and emotional responses. For this reason we used, as an animal model of stress, a fish whose brain organization is very similar to that of higher vertebrates, but is generally considered free of emotional reactions. RESULTS: We provide a comprehensive characterization of BDNF gene in the Dicentrarchus labrax and its transcriptional, translational and post-translational regulation following acute stress. While total BDNF mRNA levels are unchanged, BDNF transcripts 1c and 1d resulted down regulated after acute stress. Acute stress induces also a significant increase in proBDNF levels and reduction in mature BDNF suggesting altered regulation of proBDNF proteolytic processing. Notably, we provide here the first evidence that fishes possess a simplified proteolytic regulation of BDNF since the pro28Kda form, generated by the SKI-1 protease in mammals, is absent in fishes because the cleavage site has first emerged in reptilians. Finally, we show that the proBDNF/totBDNF ratio is a highly predictive novel quantitative biomarker to detect stress in fishes with sensitivity = 100%, specificity = 87%, and Negative Predictive Value = 100%. CONCLUSION: The high predictivity of proBDNF/totBDNF ratio for stress in lower vertebrates indicates that processing of BDNF is a central mechanism in adaptation to stress and predicts that a similar regulation of pro/mature BDNF has likely been conserved throughout evolution of vertebrates from fish to man.

Deep brain stimulation for intractabile epilepsy.

INTRODUCTION: Deep brain stimulation (DBS) is currently considered a promising neuromodulation therapy for refractory epilepsy not suitable for resective surgery. Several anatomical targets and different stimulation approaches have been proposed in order to obtain satisfactory seizures reduction. As expected, according with different patterns of neural pathways involvement, the efficacy of each anatomical target stimulation in reducing seizure frequency varies among the different epileptic syndromes. EVIDENCE ACQUISITION: We reviewed the current literature on this topic to provide an overview of the clinical efficacy of the main stimulation targets (anterior nucleus of the thalamus [ANT], hippocampus formation [HF] and centromedian nucleus of the thalamus [CMT]) related to the different epileptic syndromes. We also summarized the available data concerning side effects, neuropsychological outcome, quality of life and future perspective of DBS in intractable epilepsy. EVIDENCE SYNTHESIS: Encouraging results were reported for each target stimulation. However, only in ANT and responsive neurostimulation (RNS) the efficacy and safety were tested on large series of patients and achieving class I evidence level. CONSLUSIONS: We concluded that responsive stimulation could be more appropriate in focal epilepsy, while ANT stimulation could be properly performed also in multifocal seizures with predominant limbic involvement. Despite the small samples size, HF stimulation reduces seizure frequency in (bi) temporal lobe epilepsy, as well as CMT stimulation is able to reduce, seizure frequency, in generalized epilepsy.

Image-guided transoral biopsy in a boy with Grisel's syndrome.

Grisel's syndrome is a disease characterized by an atlanto-axial rotatory subluxation following acute inflammation of the upper respiratory tract. The syndrome has a good prognosis as it usually heals with antibiotics, despite the delayed serious complications that have been reported. When neuroradiological investigation does not allow an accurate differential diagnosis between a tumor and osteomyelitis, an image-guided transoral biopsy is a safe, fast, minimally invasive, as well as effective, procedure.

Endoscope-assisted microneurosurgery for intracranial aneurysms.

BACKGROUND: The endovascular techniques has widely changed the treatment of intracranial aneurysms. However surgery still represent the best therapeutic option in case of broad-based and complex lesions. The combined use of endoscopic and microsurgical techniques (EAM) may improve surgical results. OBJECTIVE: The purpose of our study is to evaluate the advantages and limits of EAM for intracranial aneurysms. METHODS: Between January 2002 and December 2012, 173 patients, harboring 206 aneurysms were surgically treated in our department with the EAM technique. One hundred and fifty-seven aneurysms were located in the anterior circulation and 49 were in the posterior circulation. Standard tailored approaches, based on skull base surgery principles, were chosen. The use of the endoscope included three steps: initial inspection, true operative time, and final inspection. For each procedure, an intraoperative video and an evaluation schedule were prepared, to report surgeons' opinions about the technique itself. In the first cases, we always used the endoscope during surgical procedures in order to get an adequate surgical training. Afterwards we became aware in selecting cases in which to apply the endoscopy, as we started to become familiar with its advantages and limits. RESULTS: After clipping, all patients were undergone postoperative cerebral angiography. No surgical mortality related to EAM were observed. Complications directly related to endoscopic procedures were rare. CONCLUSION: Our retrospective study suggests that endoscopic efficacy for aneurysms is only scarcely influenced by the preoperative clinical condition (Hunt-Hess grade), surgical timing, presence of blood in the cisterns (Fisher grade) and/or hydrocephalus. However the most important factors contributing to the efficacy of EAM are determined by the anatomical locations and sizes of the lesions. Furthermore, the advantages are especially evident using dedicated scopes and holders, after an adequate surgical training to increase the learning curve.

Altered serum content of brain-derived neurotrophic factor isoforms in multiple sclerosis.

In multiple sclerosis (MS), brain-derived neurotrophic factor (BDNF) provides neuroprotection, but can also promote disease through the maintenance of autoreactive T cells. One aspect that has not been explored yet in MS is related to the opposite functions of BDNF protein isoforms consisting of the pro-BDNF precursor, which has pro-apoptotic effects, and two proteolytic isoforms, the mature BDNF with pro-survival effects and truncated BDNF, with unknown functions. Using ELISA and semi-quantitative Western-blot we determined the relative serum levels of BDNF isoforms in 20 relapsing-remitting MS patients without any disease modifying therapy and 20 age and gender-matched healthy controls and searched for clinical correlates. Total serum BDNF was lower in MS than in HC. We demonstrate that the capture and detection antibodies of the ELISA kit from Promega are able to recognize all three isoforms but with different efficiency. Using Western-blot analysis, we show that the percentage of serum mature BDNF and pro-BDNF with respect to total serum BDNF was significantly decreased, while truncated BDNF was increased. No correlation between BDNF isoform percentage and clinical or demographic features was found. Serum Fas (sFas) was increased. These results support and expand the current hypothesis on the role of BDNF in multiple sclerosis, in that low pro-BDNF and high sFas might result in a failure to limit autoreactive T cells by apoptotic deletion and decreased mature BDNF may not provide enough neuroprotection, while truncated BDNF percent increase could be a compensatory mechanism. Hence, future studies on MS should take into account BDNF proteolytic processing.

A rare case of greater petrosal nerve schwannoma.

BACKGROUND: Facial nerve schwannomas include only 0.8% of all intrapetrous mass lesions, and schwannomas originating exclusively from the greater petrosal nerve (GPN) are extremely rare. To date, only 13 reports have been described. In this case, the tumor was thought to originate from the GPN on the basis of clinical, radiological, and operative findings. CASE DESCRIPTION: A 23-year-old girl presented an acute left facial palsy, a disturbance in tear secretion of the ipsilateral eye, and a left-sided conductive hypoacusia. Computed tomography (CT) scan and magnetic resonance imaging (MRI) showed an extradural mass in the left middle fossa. A subtemporal approach was performed and the lesion, originating from the proximal portion of the GPN, was excised. The post-operative course was satisfactory, except for a xerophtalmia, which was treated with artificial teardrops. CONCLUSION: GPN schwannomas can originate anywhere alongside the course of the nerve, from its proximal segment near the facial hiatus to its distal segment near the foramen lacerum. For these reasons, it requires differential diagnosis with trigeminal nerve schwannomas or with injuries arising from the geniculate ganglion, because it can be easily confused with those lesions. However, in less severe cases, an early diagnosis can be able to preserve the function of the facial nerve by reducing iatrogenic injuries caused by surgical maneuvers.

Low serum truncated-BDNF isoform correlates with higher cognitive impairment in schizophrenia.

Brain-derived neurotrophic factor (BDNF) is a key factor in learning and memory. Altered BDNF-signalling is thought to contribute to the pathogenesis of schizophrenia (SZ) especially in relation to cognitive deficits. However, analysis of serum BDNF as a potential biomarker in schizophrenia has provided controversial data. We hypothesized that these confounding results might be due to a differential regulation of BDNF precursor pro-BDNF (32 KDa) and proteolytic products mature (mat-BDNF; 14 KDa), and truncated-BDNF (28 KDa). Accordingly, we investigated the serum abundance of these BDNF isoforms and its relationship with cognitive impairment in schizophrenia. Schizophrenia was diagnosed with PANSS test. Abbreviated cognitive assessment included tests for attention, perceptual-motor skills, processing speed and memory. Using an ELISA assay, we found a slight reduction in serum BDNF levels in SZ patients (n = 40) with respect to healthy controls (HC, n = 40; p = 0.018). Western-blot analysis revealed increased serum pro-BDNF and mat-BDNF and reduced truncated-BDNF (p < 0.001) in SZ with respect to HC. Patients with an increase in pro-BDNF (n = 15/40) or mat-BDNF (n = 9/40) higher than the HC mean + 2 Standard Deviations (SD) also had >2SD reduction of truncated-BDNF (n = 27/40). Reduced truncated-BDNF correlated significantly with higher positive and lower negative PANNS scores and a worst performance in all cognitive assays but not with antipsychotic type. Measurement of serum truncated-BDNF abundance predicted for high cognitive deficits with sensitivity = 67.5%, specificity = 97.5%, Negative Predictive Value = 75% and Positive Predictive Value = 96.4%. These results suggest deficiency in pro-BDNF processing as a possible biological mechanism underlying schizophrenia with cognitive impairment.