Extreme sensitivity of hearing to decreases of ICP in Menière's disease

We report the case of a Menière's disease patient affected by normal pressure hydrocephalus (NPH) who presented a cerebrospinal fluid (CSF) pressure-dependent hearing impairment after shunting. This side-effect was not only reversible and reproducible but occurred at a high opening pressure when the valve setting was lowered by only 0.7mmHg (10mmH2O). This observation suggests that hearing in Menière's disease might be very sensitive to small reductions of intracranial pressure (ICP) and that these patients should be informed of this potential risk, which can compromise the efficacy of the shun

Hidden aqueductal stenosis associated to bilateral idiopathic foramina of Monro stenosis mimicking a Chiari I malformation? Case report

A 39-year old man came to our outpatient clinic with long history of unspecific symptoms and signs. Cerebral MRI showed herniation of the cerebellar tonsils of more than 1cm below the foramen magnum and a triventricular hydrocephalus. A diagnosis of Chiari I malformation was retained. After an osteo-dural decompression of the posterior fossa, post-operative MRI revealed an aqueductal stenosis with triventricular hydrocephalus. An endoscopic-third- ventriculostomy showed an idiopathic stenosis of the right foramen of Monro. Residual symptoms and persistence of biventricular hydrocephalus justified a ventriculo-peritoneal shunt. Aqueductal and foramina of Monro stenosis can mimick a Chiari I malformatio

Occipital extra- and intracranial lipoencephalocele associated with tectocerebellar dysraphia

Objects: Tectocerebellar dysraphism was first described by Padget and Lindenberg, together with occipital encephaloceles, cerebellar midline defects, tectal beaking, posterior kinked and displaced brainstem, and inverted cerebellum. We present a unique case of lipoencephalocele associated with tectocerebellar dysraphia and discuss the etiologies of both pathologies. The importance of the developmental comprehension of posterior fossa malformations is stressed. Case report: A 9-month-old girl in good health was referred for a huge occipital mass. On local examination, the patient had a rounded mass about 10×12cm in size, covered by alopecic skin, with a small eccentric dimple without any discharge. Magnetic resonance imaging revealed a lipoencephalocele with an intra- and an extracranial part, associated with tectocerebellar dysraphia with no aspect of inverted cerebellum as originally described in the tectocerebellar dysraphia. Resection of the extracranial part of the lipoma was performed for esthetic reasons. The intracranial part remained in situ. Conclusion: The association of lipoencephalocele and tectocerebellar dysraphia is extremely rare. The functional prognosis seems to be less severe for this combination than for the isolated tectocerebellar dysraphia. This association favors the dysraphic theory for the embryogenesis of the lipoma. It seems safe to restrict the surgery of this bizarre malformation to its external par

Neuropsychological outcome after extra-temporal epilepsy surgery

Background: The neuropsychological results of temporal lobe epilepsy surgery are well reported in the literature. The aim of this study was to analyse the neuropsychological outcome in a consecutive series of patients with extra-temporal epilepsy. Methods: We retrospectively analysed the data of patients operated between 1996 and 2008 for extra-temporal epilepsy. Standard neuropsychological tests were applied. We assessed the neuropsychological outcome after surgery and the correlation of the neuropsychological outcome with (1) side and localisation of surgery, (2) Engel scale for seizure outcome and (3) timing of surgery. Findings: Patients had a better neuropsychological outcome when undergoing non-frontal resection [χ2 (2) =6.66, p = 0.036]. Subjects who had undergone left or right resection showed no difference in outcome [χ2 (2) =0.533, p = 0.766]. The correlation between the Engel scale for seizure re-occurence and the neuropsychological scores showed only a tendency for better outcome (Spearman ρ = −0.437; p = 0.069). The global measure of change did not correlate significantly with delay of surgery (Spearman ρ = −0.163; p = 0.518). Conclusions: Resective epilepsy surgery improves neuropsychological status outcome in patients with extra-temporal epilepsy even if the patient did not become seizure free. The outcome is better for non-frontal localisatio

Transformation of a Microprolactinoma into a Mixed Growth Hormone and Prolactin-Secreting Pituitary Adenoma

Combined prolactin (PRL) and growth hormone (GH) secretion by a single pituitary tumor can occur in approximately 5% of cases. However, in all previously reported patients, combined secretion of both hormones was present at the time of diagnosis. Here we describe a patient initially diagnosed with a pure prolactin-secreting microadenoma, who experienced the progressive apparition of symptomatic autonomous GH secretion while on intermittent long term dopamine agonist therapy. She was operated on, and immunohistochemical analysis of tumor tissue confirmed the diagnosis of pituitary adenoma with uniform co-staining of all cells for both GH and PRL. This patient represents the first documented occurrence of asynchronous development of combined GH and PRL secretion in a pituitary adenoma. Although pathogenic mechanisms implicated remain largely speculative, it emphasizes the need for long term hormonal follow up of patients harboring prolactinomas

Finite element analysis of normal pressure hydrocephalus: influence of CSF content and anisotropy in permeability

Hydrocephalus is a cerebral disease where brain ventricles enlarge and compress the brain parenchyma towards the skull leading to symptoms like dementia, walking disorder and incontinence. The origin of normal pressure hydrocephalus is still obscure. In order to study this disease, a finite element model is built using the geometries of the ventricles and the skull measured by magnetic resonance imaging. The brain parenchyma is modelled as a porous medium fully saturated with cerebrospinal fluid (CSF) using Biot’s theory of consolidation (1941). Owing to the existence of bundles of axons, the brain parenchyma shows locally anisotropic behaviour. Indeed, permeability is higher along the fibre tracts in the white matter region. In contrast, grey matter is isotropic. Diffusion tensor imaging is used to establish the local CSF content and the fibre tracts direction together with the associated local frame where the permeability coefficients are given by dedicated formulas. The present study shows that both inhomogeneous CSF content and anisotropy in permeability have a great influence on the CSF flow pattern through the parenchyma under an imposed pressure gradient between the ventricles and the subarachnoid spaces

Antigenic expression and spontaneous immune responses support the use of a selected peptide set from the IMA950 glioblastoma vaccine for immunotherapy of grade II and III glioma.

Gliomas are lethal brain tumors that resist standard therapeutic approaches. Immunotherapy is a promising alternative strategy mostly developed in the context of glioblastoma. However, there is a need for implementing immunotherapy for grade II/III gliomas, as these are the most common CNS tumors in young adults with a high propensity for recurrence, making them lethal despite current treatments. We recently identified HLA-A2-restricted tumor-associated antigens by peptide elution from glioblastoma and formulated a multipeptide vaccine (IMA950) evaluated in phase I/II clinical trials with promising results. Here, we investigated expression of the IMA950 antigens in patients with grade II/III astrocytoma, oligodendroglioma or ependymoma, at the mRNA, protein and peptide levels. We report that the BCAN, CSPG4, IGF2BP3, PTPRZ1 and TNC proteins are significantly over-expressed at the mRNA (n = 159) and protein (n = 36) levels in grade II/III glioma patients as compared to non-tumor samples (IGF2BP3 being absent from oligodendroglioma). Most importantly, we detected spontaneous antigen-specific T cell responses to one or more of the IMA950 antigens in 100% and 71% of grade II and grade III patients, respectively (27 patients tested). These patients displayed T cell responses of better quality (higher frequency, broader epitope targeting) than patients with glioblastoma. Detection of spontaneous T cell responses to the IMA950 antigens shows that these antigens are relevant for tumor targeting, which will be best achieved by combination with CD4 epitopes such as the IDH1R132H peptide. Altogether, we provide the rationale for using a selective set of IMA950 peptides for vaccination of patients with grade II/III glioma

EEG source connectivity to localize the seizure onset zone in patients with drug resistant epilepsy

Visual inspection of the EEG to determine the seizure onset zone (SOZ) in the context of the presurgical evaluation in epilepsy is time-consuming and often challenging or impossible. We offer an approach that uses EEG source imaging (ESI) in combination with functional connectivity analysis (FC) to localize the SOZ from ictal EEG. Ictal low-density-scalp EEG from 111 seizures in 27 patients who were rendered-seizure free after surgery was analyzed. For every seizure, ESI (LORETA) was applied on an artifact-free epoch selected around the seizure onset. Additionally, FC was applied on the reconstructed sources. We estimated the SOZ in two ways: (i)the source with highest power after ESI and (ii)the source with the most outgoing connections after ESI and FC. For both approaches, the distance between the estimated SOZ and the resected zone (RZ) of the patient were calculated. Using ESI alone, the SOZ was estimated inside the RZ in 31% of the seizures and within 10mm from the border of the RZ in 42%. For 18.5% of the patients, all seizures were estimated within 10mm of the RZ. Using ESI and FC, 72% of the seizures were estimated inside the RZ, and 94% within 10mm. For 85% of the patients, all seizures were estimated within 10mm of the RZ. FC provided a significant added value to ESI alone (p<0.001). ESI combined with subsequent FC is able to localize the SOZ in a non-invasive way with high accuracy. Therefore it could be a valuable tool in the presurgical evaluation of epilepsy

Adjuvant or radical fractionated stereotactic radiotherapy for patients with pituitary functional and nonfunctional macroadenoma

Purpose: To evaluate the efficacy and toxicity of stereotactic fractionated radiotherapy (SFRT) for patients with pituitary macroadenoma (PMA).Methods and Materials: Between March 2000 and March 2009, 27 patients (male to female ratio, 1.25) with PMA underwent SFRT (median dose, 50.4 Gy). Mean age of the patients was 56.5 years (range, 20.3 - 77.4). In all but one patient, SFRT was administered for salvage treatment after surgical resection (transphenoidal resection in 23, transphenoidal resection followed by craniotomy in 2 and multiple transphenoidal resections in another patient). In 10 (37%) patients, the PMAs were functional (3 ACTH-secreting, 3 prolactinomas, 2 growth hormone-secreting and 2 multiple hormone-secretion). Three (11.1%) and 9 (33.3%) patients had PMA abutting and compressing the optic chiasm, respectively. Mean tumor volume was 2.9 +/- 4.6 cm(3). Eighteen (66.7%) patients had hypopituitarism prior to SFRT. The mean follow-up period after SFRT was 72.4 +/- 37.2 months.Results: Tumor size decreased for 6 (22.2%) patients and remained unchanged for 19 (70.4%) other patients. Two (7.4%) patients had tumor growth inside the prescribed treatment volume. The estimated 5-year tumor growth control was 95.5% after SFRT. Biochemical remission occurred in 3 (30%) patients with functional PMA. Two patients with normal anterior pituitary function before SFRT developed new deficits 25 and 65 months after treatment. The 5-year survival without new anterior pituitary deficit was thus 95.8%. Five patients with visual field defect had improved visual function and 1 patient with no visual defect prior to SFRT, but an optic chiasm abutting tumor, had a decline in visual function. The estimated 5-year vision and pituitary function preservation rates were 93.2% and 95.8%, respectively.Conclusions: SFRT is a safe and effective treatment for patients with PMA, although longer follow-up is needed to evaluate long-term outcomes. In this study, approximately 1 patient with visual field defect out of two had an improved visual