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Background Evidence of immune-mediated neurological syndromes associated with the severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection is limited. We therefore investigated clinical, serological and CSF features of coronavirus disease 2019 (COVID-19) patients with neurological manifestations. Methods Consecutive COVID-19 patients with neurological manifestations other than isolated anosmia and/or non-severe headache, and with no previous neurological or psychiatric disorders were prospectively included. Neurological examination was performed in all patients and lumbar puncture with CSF examination was performed when not contraindicated. Serum anti-gangliosides antibodies were tested when clinically indicated. Results Of the 349 COVID-19 admitted to our center between March 23rd and April 24th 2020, 15 patients (4.3%) had neurological manifestations and fulfilled the study inclusion/exclusion criteria. CSF examination was available in 13 patients and showed lymphocytic pleocytosis in 2 patients: 1 with anti-contactin-associated protein 2 (anti-Caspr2) antibody encephalitis and 1 with meningo-polyradiculitis. Increased serum titer of anti-GD1b antibodies was found in three patients and was associated with variable clinical presentations, including cranial neuropathy with meningo-polyradiculitis, brainstem encephalitis and delirium. CSF PCR for SARS-CoV-2 was negative in all patients. Conclusions In SARS-Cov-2 infected patients with neurological manifestations, CSF pleocytosis is associated with para- or post-infectious encephalitis and polyradiculitis. Anti-GD1b and anti-Caspr2 autoantibodies can be identified in certain cases, raising the question of SARS-CoV-2-induced secondary autoimmunity.

Introduction: Uni- or bi-lateral fourth cranial nerve palsy due to hydrocephalus and/or after VPS placement is a very rare oculomotor manifestation. We report a case of relapsing bilateral fourth nerve palsies demonstrating recurring hydrocephalus. We reviewed the literature (table1) in order to inform the clinician about the clinical assessment, the past medical history and the radiological findings that prompt research for this peculiar entity and to avoid misdiagnoses like palsies of the sixth cranial nerve. Diagnosis, intervention and outcome: The patient presented with recurrence of diplopia in reading position, partially resolved after a second VPS placement. A diagnosis of bilateral fourth nerves palsies was done after complete neuro-ophthalmological evaluation. A close follow-up demonstrated fluctuating level of diplopia by changing VPS valve resistance. An optimal placement of the VPS offered reduction and stability of diplopia. A final strabismus surgery was necessary to obtain complete symptoms release.

Mucormycosis is an opportunistic fungal infection involving among others the paranasal sinuses, nasal fossa and brain parenchyma. Mucor can invade the brain parenchyma by either contiguous spread from the paranasal sinuses or through vascular invasion. We report a case of fatal rhino-cerebral mucormycosis in whom cytotoxic edema at magnetic resonance diffusion-weighted imaging was symmetrically restricted to both neocortical and paleocortical primary areas of olfactory projection at earliest phase of the disease process. Shortly later tissue damage extended into the whole brain. This undescribed observation raised the hypothesis of preferential way of brain invasion by Mucor through the olfactory tract.

Object Several scientific papers report clinical symptoms, indications, complications and outcomes of brainstem cavernous malformation (BSCM) surgery without reporting on the occurrence of postoperative Holmes tremor (HT). Our purpose is to report our experience with HT in a monocentric series of resected brainstem cavernomas. Methods We reviewed all the BSCM surgical records between 2002 and 2018 at Saint-Luc University Hospital's Department of Neurosurgery, Brussels and selected patients developing HT postoperatively. Patients’ demographics, symptoms, pre- and postoperative imaging, recurrence and complications were analysed. A PubMed literature review was performed to compare our results with those in the existing literature. Results In a total series of 18 resected BSCM, 5 patients: 1 male and 4 females, with a median age of 51 years (range 29–59 years), developed HT. The median preoperative mRS score was 2 (range 1–4). GTR was achieved in all patients without surgery-related death. BSCM were located in the mesencephalon in 4 patients (80%) who developed HT. Tremor was noticed between ten days and one year after surgery. One patient saw significant improvements to the point of stopping treatment. The median follow-up period was 2 years (range 1–14 years). At the last follow-up, 40% of our patients showed a worse mRS score, 40% stayed unchanged, and 20% improved. Conclusion We are reporting an original single-center series of patients suffering from HT after BSCM surgery. The risk for HT after surgery is significant for midbrain BSCM. A spontaneous favorable evolution is possible.

Invasive fungal sinusitis can lead to dramatic complications in immunocompromised patients and requires prompt diagnosis. Here we report three cases with ophthalmological complications secondary to invasive fungal sinusitis in immunocompromised patients. From an ophthalmological point of view, these cases illustrate different clinical presentations, evolutions, complications, treatments, prognoses, and highlight different pathophysiological mechanisms. Diagnoses were delayed in all cases. In none of the cases did patients recover better vision than counting fingers at 24 months follow up, and two patients died. This case series highlights key points useful for quickly recognising this highly morbid infection in immunocompromised patients.

PURPOSE: To assess the predictive value for functional recovery of Ganglion Cell Complex Layer (GCC) and Retinal Nerve Fiber Layer (RNFL) measurements obtained by Optical Coherence Tomography (OCT) in patients undergoing chiasmal decompression and to define potential OCT thresholds for visual recovery. METHODS: We measured preoperative GCC and RNFL thickness in patients with a sellar and/or perisellar tumor compressing the optic chiasm. Visual recovery was defined as recovery of mean deviation (MD) and pattern standard deviation (PSD) using Humphrey visual field testing after 12 successful decompressions (24 eyes). Receiver operating characteristic curve (ROC) analysis was used to identify the best thresholds. RESULTS: Robust global and focal OCT thresholds were found. Superior GCC≥63μm had the best functional prognostic value (AUC=1) for visual improvement. Mean GCC ≥ 67μm and mean RNFL≥75μm also had excellent predictive values (AUC>0.9). CONCLUSION: In this preliminary study, significant preoperative OCT thresholds for early visual recovery after chiasmal decompression were identified, mainly regarding GCC measurements. Further studies on larger cohorts with closely scheduled follow-up could refine our results. [Valeur pronostique fonctionnelle de la tomographie par cohérence optique dans les décompressions du chiasma optique : une étude préliminaire] BUT : Déterminer la valeur prédictive de récupération fonctionnelle des couches des cellules ganglionnaires (GCC) et des fibres nerveuses rétiniennes (RNFL) mesurées à l’OCT (Optical Coherence Tomography) dans les décompressions du chiasma optique, en particulier identifier des seuils de récupération visuelle. MÉTHODES : Les valeurs préopératoires des GCC et RNFL des patients présentant une tumeur sellaire et/ou péri-sellaire comprimant le chiasma optique ont été mesurées. La récupération visuelle a été définie par la récupération en termes de déviation moyenne (MD) et de déviation individuelle (PSD) sur champs visuels Humphrey après 12 décompressions efficaces (24 yeux). Une analyse par courbe ROC (Receiver Operating Characteristic) a été utilisée pour identifier les meilleurs seuils de récupération. RÉSULTATS : Des seuils robustes de récupération fonctionnelle ont pu être été identifiés: un GCC supérieur ≥ 63 μm a montré la meilleure valeur pronostique fonctionnelle (AUC = 1). Un GCC moyen ≥ 67 μm et un RNFL moyen ≥ 75 μm ont également montré d’excellentes valeurs prédictives de récupération fonctionnelle (AUC > 9). CONCLUSION : Cette étude préliminaire a permis d’identifier pour la première fois des seuils pronostiques significatifs de récupération visuelle, principalement pour les mesures de GCC, obtenus à l’OCT préopératoire avant décompression chiasmatique. De futures investigations complémentaires menées dans une plus grande cohorte de patients pourraient affiner nos résultats.

BACKGROUND: Microstructure analyses are gaining interest in cancer MRI as an alternative to the conventional apparent diffusion coefficient (ADC), of which the determinants remain unclear. PURPOSE: To assess the sensitivity of parameters calculated from a double diffusion encoding (DDE) sequence to changes in a tumor's microstructure early after radiotherapy and to Compare them with ADC and histology. STUDY TYPE: Cohort study on experimental tumors. ANIMAL Model: Sixteen WAG/Rij rats grafted with one rhabdomyosarcoma fragment in each thigh. Thirty-one were imaged at days 1 and 4, of which 17 tumors received a 20 Gy radiation dose after the first imagery. FIELD STRENGTH/SEQUENCE: 3T. Diffusion-weighted imaging, DDE with flow compensated, and noncompensated measurements. ASSESSMENTS: 1) To compare, after irradiation, DDE-derived parameters (intracellular fraction, cell size, and cell density) to their histological counterparts (fraction of stained area, minimal Feret diameter, and nuclei count, respectively). 2) To compare percentage changes in DDE-derived parameters and ADC. 3) To evaluate the evolution of DDE-derived parameters describing perfusion. STATISTICAL tests: Wilcoxon rank sum test. RESULTS: 1) Intracellular fraction, cell size, and cell density were respectively lower (-24%, P < 0.001), higher (+7.5%, P < 0.001) and lower (-38%, P < 0.001) in treated tumors as compared to controls. Fraction of stained area, minimal Feret diameter, and nuclei count were respectively lower (-20%, P < 0.001), higher (+28%, P < 0.001), and lower (-34%, P < 0.001) in treated tumors. 2) The magnitude of ADC's percentage change due to irradiation (16.4%) was superior to the one of cell size (8.4%, P < 0.01) but inferior to those of intracellular fraction (35.5%, P < 0.001) and cell density (42%, P < 0.001). 3) After treatment, the magnitude of the vascular fraction's decrease was higher than the increase of flow velocity (33.3%, vs. 13.3%, P < 0.001). DATA CONCLUSION: The DDE sequence allows quantitatively monitoring the effects of radiotherapy on a tumor's microstructure, whereas ADC only reveals global changes. EVIDENCE LEVEL: 2. TECHNICAL EFFICACY: Stage 4. J. Magn. Reson. Imaging 2020;52:941-951.

Primary cutaneous apocrine adenocarcinoma (PCAC) is an extremely rare neoplasm involving the sweat glands. Due to a lack of cases, there is no consensus for the systemic treatment of locally advanced or metastatic PCAC. Anti‐androgen therapy may have activity in inoperable or metastatic PCAC with high androgen receptor (AR) expression.

Background Amyloid infiltration of endocrine glands has been reported, mostly in the thyroid, pancreas, adrenals, and testes, but affected patients do not frequently exhibit overt endocrine insufficiency. Here we report the case of a patient with complete anterior hypopituitarism probably due to a known systemic amyloidosis. Case presentation Our male Caucasian patient was diagnosed with Crohn’s disease at the age of 22 years. At the age of 37, he developed secondary renal amyloidosis, which resulted in end-stage renal failure. He received a living-donor kidney transplant at the age of 57, without initial complication. Two months later, he developed extreme fatigue, weight loss, and dyspnea. A hormonal evaluation demonstrated complete anterior pituitary insufficiency. A pituitary magnetic resonance imaging was performed and showed a diffusely hypointense anterior gland on both T1-weighted and T2-weighted images with reduced gadolinium enhancement, highly suggestive of amyloid infiltration of the pituitary. Treatment was initiated with levothyroxine, orally administered hydrocortisone, and testosterone enanthate, rapidly allowing progressive marked clinical improvement and nearly complete resolution of symptoms. Conclusions Pituitary amyloid infiltration should be considered in patients with a known systemic amyloidosis who develop symptoms of hypopituitarism and magnetic resonance imaging features compatible with protein deposits.

We investigated changes on 2'-deoxy-2'-[18F]fluoro-D-glucose positron emission tomography (18FDG-PET), diffusion-weighted magnetic resonance imaging (DW-MRI), and choline spectroscopy as early markers of cetuximab activity in squamous cell carcinoma of the head and neck (SCCHN). SCCHN patient-derived tumor xenografts models were selected based on their cetuximab sensitivity. Three models were resistant to cetuximab and two were sensitive (one was highly sensitive and the other one was moderately sensitive). Cetuximab was infused on day 0 and 7. Maximal standardized uptake values (SUVmax), apparent diffusion coefficient (ADC), and total choline pool were measured at baseline and at day 8. To investigate the possible clinical relevance of our pre-clinical findings, we also studied the SUVmax and ADC modifications induced by cetuximab in five patients. Cetuximab induced a significant decrease in SUVmax and an increase in ADC at day 8 compared to baseline in the most cetuximab-sensitive model but not in the other models. At day 8, in one resistant model, SUVmax was decreased compared to baseline and was significantly lower than the controls. Choline spectroscopy was not able to predict cetuximab activity. The five patients treated with cetuximab had a 18FDG-PET partial response. One patient had a partial response according to RECISTv1.1. Interestingly, this last had also an increase in ADC value above 25%. Our preclinical data support the use of PDTX to investigate imaging techniques to detect early treatment response. Our pre-clinical and clinical data suggest that DW-MRI and 18FDG-PET should be further investigated to predict cetuximab activity. ispartof: Oncotarget vol:9 issue:47 pages:28572-28585 ispartof: location:United States status: Published online

Neurological involvement of sarcoidosis is a rare condition, and its occurrence in the context of transplantation is exceptional. Moreover, treatment can be challenging. We report the unusual case of a patient transplanted with a kidney for end-stage renal disease secondary to sarcoidosis who experienced a neurological recurrence of the disease under immunosuppressive treatment, translating in behavioural aggressiveness, social withdrawal and weight loss. He relapsed thrice under corticosteroids but responded finally to infliximab. This case highlights the potential of sarcoidosis to recur neurologically after kidney transplantation despite immunosuppressive treatment. Also, treatment of relapsing neurosarcoidosis can be challenging and may benefit from TNF-α blockers.

Disseminated abscesses due to group Gβ-hemolyticStreptococcus dysgalactiaewere observed in a 57-year-old cirrhotic patient with the skin being the putative way of entry for the pathogen.S. dysgalactiaeis a rare agent in human infections responsible for acute pyogenic meningitis. The mortality rate associated withS. dysgalactiaebacteraemia and meningitis may be as high as 50%, particularly in the presence of endocarditis or brain abscesses. In our patient, main sites of infections were meningitis and ventriculitis, spondylodiscitis, septic arthritis, and soft-tissue infections. In contrast, no endocarditis was evidenced. Cirrhosis-related immune suppression was considered as a pathophysiological cofactor for the condition. Fortunately, clinical status improved after long-term (3 months) antimicrobial therapy.

Herein we report a patient with a rare delayed-onset Holmes head titubation after midbrain cavernoma hemorrhage that dramatically responded to levodopa treatment. To our knowl- edge, the levodopa responsiveness of a Holmes head titubation has not yet been reported with accompanying video footage.

Background: With the increasing age of acute stroke patients being admitted to hospitals, more data are needed on indications, complications and outcome of endovascular treatment (EVT) in the very elderly. Methods: Retrospective observational study with data collection from Belgian, Swiss, Canadian comprehensive stroke centers and Swedish EVT National database. All patients with acute ischemic stroke were eligible if aged older than or >= 90 years and treated with EVT +/- pretreatment with intravenous thrombolysis (IVT). Safety assessment comprised presence of peri-procedural complications, hemorrhagic transformation or other adverse events (= 2b), favorable clinical outcome (modified Rankin Score (mRS) 0-2) and 3-months mortality. Results: Inclusion of 112 nonagenarians (mean age 93.3 +/- 2.5 years; 76.8% women; pre-mRS = 2b) was achieved. Favorable outcome (mRS

A 90-year-old female patient was brought to our hospital by ambulance after her husband found her somnolent and mute upon waking. Upon arrival, neurological and physical examinations showed spastic flexion of the right arm and deviation of gaze to the left. A so-called ‘wake-up stroke procedure’ was triggered and brain magnetic resonance imaging (MRI), including fluid-attenuation inversionrecovery (FLAIR) and diffusion weighted imaging (DWI) acquisitions, was immediately performed (Figure 1). Axial FLAIR images showed only leukoaraiosis (Figure 1A). DWI demonstrated high signal intensity in the territory of left middle cerebral artery (MCA) at high b-value (Figure 1B) with decreased apparent diffusion coefficient values. MRangiography was precluded by patient’s movements. Non-contrast CT (NCCT), CT-angiography (CTA), and CT-perfusion (CTP) were thereafter performed for angiographic assessment including carotid arteries, circle of Willis, and intracranial collateral circulation (Figure 2).

PURPOSE: To present a double diffusion encoding MRI sequence on a clinical scanner to analyze micro-structure and micro-vasculature of tumors. METHODS: The sequence was tested on phantoms, asparaguses, and 2 tumors allografts in a rodent. Results were analyzed using an adapted VERDICT model to estimate microstructural parameters. The technical feasibility of the sequence on a 3T clinical system was assessed on a water phantom. The accuracy of cell size estimation was assessed on asparaguses by comparison with light microscopy. Cell size estimations were also validated when limiting relative angles of diffusion encodings to 0 and 180°. Sensitivities to restricted diffusion and incoherent flow from the vasculature were investigated in experimental tumor models. Values of microstructural parameters in viable and decaying tumor tissue were compared with those obtained from histological analysis. RESULTS: Measurements on the water phantom revealed no significant sequence artifacts and accurate apparent diffusion coefficient values within a 4% relative error. In asparaguses, quartiles and medians of pore size distributions typically deviated less than 6% from light microscopy regardless of whether the full or reduced set of relative angles was used. Signal analyses in tumors showed mixed effects of both blood flow and diffusion restriction. Microstructural parameter estimations in tumors were consistent with histology and allowed clear and histology-proven distinctions between decaying and viable tumor tissue. CONCLUSIONS: Double diffusion encoding with clinical gradients and scan times allows characterization of restricted diffusion and micro-circulation flow in tumors. Our estimated microstructural parameters are promising for further investigations in assessing microstructural evolutions in tumors.

Corticospinal tract integrity after stroke has been widely investigated through the evaluation of fibres descending from the primary motor cortex. However, about half of the corticospinal tract is composed by sub-pathways descending from premotor and parietal areas, to which damage may play a more specific role in motor impairment and recovery, particularly post-stroke. Therefore, the main aim of this study was to investigate lesion load within corticospinal tract sub-pathways as predictors of upper limb motor impairment after stroke. Motor impairment (Fugl-Meyer Upper Extremity score) was evaluated in 27 participants at one week and six months after stroke, together with other clinical and demographic data. Neuroimaging data were obtained within the first week after stroke. Univariate regression analysis indicated that among all neural correlates, lesion load within premotor fibres explained the most variance in motor impairment at six months (R2 = 0.44, p 2 = 0.81; p

Introduction L’infarctus bithalamique par occlusion de l’artere de Percheron est une entite rare dont le diagnostic precoce est souvent difficile. Objectifs Decrire les caracteristiques demographiques et cliniques et le devenir a 3 mois des patients presentant un AVC de Percheron. Patients et methodes Etude retrospective multicentrique menee incluant 25 patients admis pour AVC de Percheron aux cliniques universitaires Saint-Luc et au CHU UCL Namur site Godinne, Belgique, entre 2001 et 2019. Resultats Parmi les 25 patients (âge moyen 73 ans ; femme = 52 %), les manifestations cliniques les plus frequentes etaient : somnolence/coma (18/25), paresie oculomotrice (10/25), anisocorie (9/25). Seuls 4 patients ont beneficie d’une imagerie cerebrale dans les 6 h suivant l’apparition des symptomes. Le scanner etait normal chez 84 %. L’IRM etait positive chez tous les patients. Seuls 3 patients ont recu un traitement par thrombolyse IV. A 3 mois, 6/25 etaient decedes, 8 avaient un bon resultat fonctionnel (mRS 0–2), et 11 presentaient un handicap significatif (mRS 4–6). Discussion Notre serie illustre que la fenetre therapeutique est frequemment depassee dans l’AVC de Percheron, ce qui est associe a de mauvais resultats fonctionnels. Une demarche diagnostique rapide et rigoureuse est requise afin de ne pas retarder une prise en charge therapeutique optimale, mais la complexite et la variabilite de la semiologie expliquent probablement les difficultes a en etablir le diagnostic precoce. Conclusion L’IRM cerebrale a un role majeur dans l’etablissement du diagnostic positif et doit etre consideree rapidement devant tout coma d’origine indeterminee, en particulier lorsque des signes neurologiques focaux sont presents.

PURPOSE: To determine the ideal implantation site for selective tongue neurostimulation in obstructive sleep apnea, anatomy of the distal branching of the hypoglossal nerve (HGN) was revisited. METHODS: The HGN distal course and intramuscular distribution to the tongue muscles were studied in 17 embalmed and 5 fresh heads (age 60-98, BMI 20-35). Medial branches supplying selectively the genioglossus (GG) muscle were identified. Then, the distinct bundles entering the oblique (GGo) and horizontal (GGh) parts of the GG were located. Morphometric data were compared to similar measurements made on MRI sections from 12 patients (age 43-71, BMI 18-47). RESULTS: The key facts relevant to optimize stimulation and electrode design are the following: the mean width of both GG muscles in embalmed and fresh cadavers was 20.7 ± 2.9 and 21.4 ± 5 mm, respectively; it is significantly (p < 0.05) superior to the MRI value of 18.26 ± 2.0 mm. Selective nervous branches for GGh and GGo were located at 52 ± 8% of hyoid bone-mandibular symphysis distance and at 5.8 ± 1.1 mm from the inferior border of the GG muscle. The surface of stimulation is a 4.4 ± 1.1 × 6.9 ± 3.8 mm ellipse. CONCLUSIONS: According to our observations, the optimal selective or supra-selective stimulation of the tongue protractor muscles can be performed on the lateral surface of the GG at roughly equal distance between the mandibular symphysis and the hyoid bone, at a depth of about 0.6 cm above the GG lower border

Two patients were admitted to the Emergency Department with fever and altered consciousness. The diagnosis of Streptococcus pneumoniae meningitis was rapidly obtained from cerebrospinal fluid (CSF) culture. Their neurological condition rapidly improved after the introduction of antimicrobial therapy plus dexamethasone. The brain's computed tomography on admission did not reveal any specific lesion. By the second week of treatment, while CSF was sterile, their neurological condition gradually worsened and both died from extensive brain ischaemic lesions. While persisting inflammation could be the underlying mechanism, these observations illustrate that such complications are not fully prevented by a short course of high dose steroids.

Availability of an innocuous and repeatable technique for monitoring tumor oxygenation throughout therapeutic course should be a key factor for adaptative therapeutic strategies. We previously qualified lipids R1 as a marker of oxygen level on experimental tumor models. The objectives of the present study were to assess the applicability of measuring lipids R1 in primary central nervous system malignancies in a clinical setting as well as to compare lipids R1 with global (water+lipids) R1 and R2* which are also sensitive to the oxygen environment. 25 patients with brain neuroepithelial tumors were examined on a clinical 3T MR system. Values obtained within regions of interest contouring contrast-enhanced tumor (C+), unenhanced tumor (C-), peritumoral edema, and normal appearing white matter (NAWM) were compared to those obtained for the normal brain parenchyma of 17 healthy volunteers. Global R1 and lipids R1 values were significantly lower in tumors than in NAWM of patients or healthy brain of normal volunteers. In contrast, R2* values were not significantly different in tumors compared to NAWM or healthy brains. None of them showed significant difference between C+ and C- tumors. Global R1 values within NAWM were significantly different from that of both tumor and peritumoral edema, but lacked sensitivity to differentiate between tumor and peritumoral edema. In turn, lipids R1 measurements enabled discrimination between tumor areas and peritumoral edema. In conclusion, global R1 and lipids R1 deserve further attention as potential markers of tumor hypoxia in primary brain tumors.

Dear Editor, We thank Dr. Bolek and colleagues (Bolek et al., 2018) for their appropriate comments about the case report paper we recently published (London et al., 2018) on a patient with lethal neuro-Behçet's disease (NBD) after repeated failure of currently available therapies (steroids, azathioprine, cyclophosphamide, TNF-alpha and IL-6 blockers). We fully agree with Dr Bolek and colleagues that interferon-alpha (IFN-α) could be a potential alternative treatment option for refractory NBD. As pointed out in the paper, controlled or comparative studies on NBD treatment are lacking, thereby rendering the choice of therapy based on expert opinions and small patients’ series. [...]

Spinal intradural extramedullary cavernous hemangiomas are very rare. Mixed intensities on T1- andT2-weighted images due to repeated hemorrhages and poor to absent contrast-enhancement are the most common imaging features of the disease allowing accurate differentiation from the far more frequent meningiomas and schwannomas of similar location.

BACKGROUND: Proportional motor recovery in the upper limb has been investigated, indicating about 70% of the potential for recovery of motor impairment within the first months poststroke. OBJECTIVE: To investigate whether the proportional recovery rule is applicable for upper-limb somatosensory impairment and to study underlying neural correlates of impairment and outcome at 6 months. METHODS: A total of 32 patients were evaluated at 4 to 7 days and 6 months using the Erasmus MC modification of the revised Nottingham Sensory Assessment (NSA) for impairment of (1) somatosensory perception (exteroception) and (2) passive somatosensory processing (sharp/blunt discrimination and proprioception); (3) active somatosensory processing was evaluated using the stereognosis component of the NSA. Magnetic resonance imaging scans were obtained within 1 week poststroke, from which lesion load (LL) was calculated for key somatosensory tracts. RESULTS: Somatosensory perception fully recovered within 6 months. Passive and active somatosensory processing showed proportional recovery of 86% (95% CI = 79%-93%) and 69% (95% CI = 49%-89%), respectively. Patients with somatosensory impairment at 4 to 7 days showed significantly greater thalamocortical and insulo-opercular tracts (TCT and IOT) LL ( P < .05) in comparison to patients without impairment. Sensorimotor tract disruption at 4 to 7 days did not provide significant contribution above somatosensory processing score at 4 to 7 days when predicting somatosensory processing outcome at 6 months. CONCLUSIONS: Our sample of stroke patients assessed early showed full somatosensory perception but proportional passive and active somatosensory processing recovery. Disruption of both the TCT and IOT early after stroke appears to be a factor associated with somatosensory impairment but not outcome. ispartof: NEUROREHABILITATION AND NEURAL REPAIR vol:32 issue:8 pages:691-700 ispartof: location:United States status: published

PURPOSE: Recent work supports the use of T2-weighted MRI intensity as a tool for treatment stratification in acromegaly. Our study aimed to establish if the pattern of T2 intensity could be a predictor of hormonal and/or tumoral response to dopamine agonists (DAs) in prolactinomas. METHODS: This was a retrospective study performed in two academic centers. We characterized the magnetic resonance T2-weighted aspect of prolactinomas (signal intensity and homogeneity in the whole tumors) before DA therapy and correlated this pattern to the prolactin (PRL) concentration at diagnosis and to hormonal and tumoral responses after 1 year of medical treatment. We separately analyzed a subgroup of prolactinomas visually very bright in more than 50% of the surface ("cystic" tumors). RESULTS: Out of 70 prolactinomas, 80% were T2 hyperintense and 40% were heterogeneous. At diagnosis, heterogeneous prolactinomas were more frequent in men (68% vs. 28.9%, p ≤ 0.011), larger (median area 304.5 mm2 vs. 56.5 mm2, p ≤ 0.021), taller (mean height 18.6 mm vs. 9.9 mm, p < 0.001), more secreting (median PRL ULN_area 23 µg/L/cm2 vs. 12.6 µg/L/cm2, p ≤ 0.032) and had poorer hormonal response to DA as compared with homogeneous prolactinomas. "Cystic" tumors were diagnosed almost exclusively in women and secreted less prolactin, but showed similar hormonal and tumoral response as "non-cystic" tumors. In homogeneous prolactinomas, the T2-weighted intensity ratio was correlated to prolactin secretion, although not significantly, and did not predict hormonal and tumoral response to DA. CONCLUSIONS: Our study confirms that hypo/isointense prolactinoma is a rare finding and suggests for the first time that the heterogeneity of prolactinoma T2 signal at diagnosis might be correlated with a different clinical behavior and could be used as a negative predictor factor of hormonal response to DA.

INTRODUCTION : A 56-year-old man was admitted to the intensive care unit (ICU) for refractory status epilepticus. His medical history started in September 2014 when the patient developed left retro-orbital pain with conjunctival chemosis, ptosis and paresthesia of the scalp. Visual acuity was preserved at this stage (80/100 at left eye). Ophthalmological examination revealed in addition to left conjunctival chemosis, a major dilation of the capillaries on the ocular surface. Intraocular pressure was 19 mmHg at the left eye and 16 mmHg at the right eye. Fundoscopic examination was consistent with a bilateral papilledema. Initial brain magnetic resonance imaging (MRI) examination revealed marked enlargement of the left ophthalmic vein and abnormally increased contrast enhancement of dilated occipitals veins and of the left portion of the lateral venous sinus. No parenchymal lesions were noted at the time. Angiography was performed to confirm the suspicion of left carotid-cavernous fistula (Fig. 1). During the first embolization session (January 2015), the main arterial feeders arising from the distal part of the left occipital artery were embolized with a histoacryl/lipiodol mixture. During the same session, partial occlusion of the right transverse and sigmoid sinus was diagnosed, contra-indicating embolization of arterial feeders. After the first embolization, the patient developed dysphagia, hiccup, diplopia, alternating hypoesthesia and progressive impairment of cognition. Episodes of tonic–clonic- and partial complex seizures were first noted in August 2015. The patient’s neurological status progressively worsened with impairment of speech fluency and bradypsychism. Left facial and right hemiparesia, left-sided retro-orbital pain with conjunctival chemosis, proptosis and distorsion of the visual field were noted at physical examination. [...]

Summary A 26-year-old woman presented with severe postpartum headaches. Magnetic resonance imaging (MRI) revealed a symmetric, heterogeneous enlargement of the pituitary gland. Three months later, she developed central diabetes insipidus. A diagnosis of postpartum hypophysitis was suspected and corticosteroids were prescribed. Six months later, the pituitary mass showed further enlargement and characteristics of a necrotic abscess with a peripheral shell and infiltration of the hypothalamus. Transsphenoidal surgery was performed, disclosing a pus-filled cavity which was drained. No bacterial growth was observed, except a single positive blood culture for Staphylococcus aureus, considered at that time as a potential contaminant. A short antibiotic course was, however, administered together with hormonal substitution for panhypopituitarism. Four months after her discharge, severe headaches recurred. Pituitary MRI was suggestive of a persistent inflammatory mass of the sellar region. She underwent a new transsphenoidal resection of a residual abscess. At that time, the sellar aspiration fluid was positive for Staphylococcus aureus and she was treated with antibiotics for 6 weeks, after which she had complete resolution of her infection. The possibility of a pituitary abscess, although rare, should be kept in mind during evaluation for a necrotic inflammatory pituitary mass with severe headaches and hormonal deficiencies. Learning points: The possibility of a pituitary abscess, although rare, should be kept in mind during evaluation for a necrotic inflammatory pituitary mass with severe headaches and hormonal deficiencies. In a significant proportion of cases no pathogenic organism can be isolated. A close follow-up is necessary given the risk of recurrence and the high rate of postoperative pituitary deficiencies.

Background To investigate the activity and safety of afatinib in the preoperative treatment of squamous cell carcinoma of the head and neck (SCCHN). Patients and methods This study was an open-label, randomized, multicenter, phase II window of opportunity trial. Treatment-naive SCCHN patients selected for primary curative surgery were randomized (5 : 1 ratio) to receive afatinib during 14 days (day –15 until day –1) before surgery (day 0) or no treatment. Tumor biopsies, 2-[fluorine-18]-fluoro-2-deoxy- d -glucose positron emission tomography (FDG-PET), and magnetic resonance imaging (MRI) were carried out at diagnosis and just before surgery. The primary end point was metabolic FDG-PET response (according to EORTC guidelines). Other end points included response assessment based on the Response Evaluation Criteria In Solid Tumors (RECIST) v1.1, dynamic contrast-enhanced (DCE)-MRI, diffusion weighted (DW)-MRI, safety, and translational research (TR). Results Thirty patients were randomized: 25 to afatinib and 5 to control arm. Of the 23 eligible patients randomized to afatinib, 16 (70%; 95% CI: 47% to 87%) patients had a partial metabolic FDG-PET response (PMR). Five patients (22%; 95% CI: 8% to 44%) showed a partial response by RECISTv1.1. Responses assessed via DCE-MRI and DWI-MRI did not show a strong association with PMR or RECIST. One patient discontinued afatinib after 11 days for grade 3 diarrhea with subsequent renal failure and 24 days delay in surgery. No grade 4 toxicities or surgical comorbidities related to afatinib were reported. TR results indicated that PMR was more frequent in the tumors with high Cluster3-hypoxia score expression and with TP53 wild type. Conclusion Afatinib given for 2 weeks to newly diagnosed SCCHN patients induces a high rate of FDG-PET partial metabolic response and partial response according to RECISTv1.1. Afatinib can be safely administered before surgery. Although exploratory, the hypoxic gene signature needs further investigations as a predictive biomarker of afatinib activity. Clinical trial registration ClinicalTrials.gov: NCT01538381

BACKGROUND: Aberrant functional connectivity in brain networks associated with motor impairment after stroke is well described, but little is known about the association with somatosensory impairments. AIM: The objective of this cross-sectional observational study was to investigate the relationship between brain functional connectivity and severity of somatosensory impairments in the upper limb in the acute phase post stroke. METHODS: Nineteen first-ever stroke patients underwent resting-state functional magnetic resonance imaging (rs-fMRI) and a standardized clinical somatosensory profile assessment (exteroception and higher cortical somatosensation) in the first week post stroke. Integrity of inter- and intrahemispheric (ipsilesional and contralesional) functional connectivity of the somatosensory network was assessed between patients with severe (Em-NSA< 13/32) and mild to moderate (Em-NSA> 13/32) somatosensory impairments. RESULTS: Patients with severe somatosensory impairments displayed significantly lower functional connectivity indices in terms of interhemispheric (p = 0.001) and ipsilesional intrahemispheric (p = 0.035) connectivity compared to mildly to moderately impaired patients. Significant associations were found between the perceptual threshold of touch assessment and interhemispheric (r = -0.63) and ipsilesional (r = -0.51) network indices. Additional significant associations were found between the index of interhemispheric connectivity and light touch (r = 0.55) and stereognosis (r = 0.64) evaluation. CONCLUSION: Patients with more severe somatosensory impairments have lower inter- and ipsilesional intrahemispheric connectivity of the somatosensory network. Lower connectivity indices are related to more impaired exteroception and higher cortical somatosensation. This study highlights the importance of network integrity in terms of inter- and ipsilesional intrahemispheric connectivity for somatosensory function. Further research is needed investigating the effect of therapy on the re-establishment of these networks. ispartof: PLOS ONE vol:13 issue:10 ispartof: location:United States status: published

A 75-year-old woman was admitted to ICU with coma following Streptococcus pneumoniae meningitis with bacteremia. Her Glasgow Coma Scale (GCS) score fluctuated around 4 to 6 over the next four weeks. There was no evidence of increased intracranial pressure (ICP). Electroencephalogram (EEG) showed only diffuse aspecific slowing. Impaired cerebral blood flow (CBF) autoregulation was suggested at transcranial Doppler (TCD). Repeated brain magnetic resonance imaging (MRI) examination failed to demonstrate venous thrombosis, arterial ischemic stroke, or brain abscesses but revealed diffuse but reversible cortical cytotoxic edema at diffusion-weighted (DW) sequences. The brain FDG-positron emission tomography (FDG-PET) showed diffuse cortical hypometabolism. The patient unexpectedly experienced a complete neuropsychological recovery the next few weeks. The suggested hypothesis to explain this unusual disease course could be a transient alteration of CBF autoregulation due to some degree of diffuse subcortical microangiopathy. A concomitant reduction of brain metabolism probably prevented the progression towards cortical irreversible ischemic damage.

Cauda equina syndrome is a serious condition resulting from dysfunction of the lumbosacral nerve roots and characterized by impairment of bladder, bowel, sexual and lower limb functions. We report the case of a 48-year-old woman who had Crohn's disease for more than twenty years. The patient was undergoing immunotherapy with infliximab and developed a partial cauda equina syndrome after an uneventful minimally invasive microdiscectomy (L5-S1) that completely cured her sciatica. A postoperative magnetic resonance imaging examination showed root clumping but no compressive lesion. We discuss a possible relationship between the cauda equina syndrome and the patient's active Crohn's disease, treatment and surgery.

OBJECTIVE: To report a single-center experience with a 3T intraoperative magnetic resonance imaging (iMRI) to assess transsphenoidal microsurgery on pituitary macroadenomas. METHODS: In a dual, independent operating room (OR) magnetic resonance imaging (MRI) suite, the operating table with the anesthetized patient was moved on rail tracks once a supposed maximized resection was reached to the MRI room for intraoperative image acquisition and interpretation. After the assessment of the iMRI images, the neurosurgeon evaluated whether additional resection was still possible. The resection rates were assessed on iMRI and postoperative MRI at 3 months. RESULTS: A total of 73 macroadenomas benefited from an iMRI from March 2006 to October 2011. The gross total resection (GTR) rate at the time of the first iMRI was 58.9% (n=43). Based on the iMRI, eight patients (10.9%) underwent a second surgical resection. In 3 cases, the intraoperative imaging results were suspicious for a minor residue but not convincing enough for further surgery. Fortunately, the 3 months postoperative MRI control did not disclose any residual tumor in these cases. Finally, the GTR rate at the 3-month postoperative MRI increased to 72.6% (n=53). CONCLUSIONS: 3T intraoperative MRI offered excellent quality images. Its use during transsphenoidal microsurgery on pituitary macroadenomas led to an increase not only in the extent of tumor resection (in 8 patients) but also in the rate of radical resections (69% instead of 60%). No complications due to the iMRI procedure were observed.

INTRODUCTION: Pituitary gangliocytomas are uncommon neuronal tumours that may present with endocrine disorders, the most frequent being acromegaly caused by growth hormone hypersecretion. Cushing's syndrome is very rarely seen with gangliocytomas. MATERIAL AND METHODS: We report the unique case of a 62 year-old woman whose clinical picture and endocrine testing clearly demonstrated adrenocorticotropin (ACTH)-dependent Cushing's syndrome. Pituitary magnetic resonance imaging showed a 12-mm homogeneous, infra- and retrosellar mass first diagnosed as pituitary macroadenoma. Transsphenoidal surgery was performed and allowed complete resection of the tumour with sparing of normal anterior pituitary. Very low postoperative serum cortisol and ACTH levels were observed in the early postoperative period and the patient is still in remission 18 months after surgery, thus demonstrating that the resected lesion was entirely responsible for the clinical picture. RESULTS: Histological and immunocytochemical analyses demonstrated a benign tumour composed of mature neuronal cells suggestive of a gangliocytoma, expressing both ACTH and corticotropin-releasing hormone (CRH). The tumour was surrounded by a rim of pituitary tissue containing ACTH-producing endocrine cells. Careful analysis of the resected lesion did not reveal any pituitary microadenoma. We search literature for similar cases and retraced only nine cases of gangliocytomas associated with Cushing's syndrome. In most of them, the tumour was combined with either pituitary corticotroph adenoma or hyperplasia. CONCLUSIONS: Our case represents a unique case of an infrasellar pituitary gangliocytoma which was able to cause Cushing's syndrome by both direct ACTH production and CRH-induced stimulation of neighbour normal corticotroph cells.

BACKGROUND: Gross total or near total resection (GTR/NTR; resection ≥95%) of glioblastoma (GBM) seems correlated with a longer survival. Intraoperative MRI (ioMRI) is one method to evaluate the extent of resection (EOR) in order to improve it during the same anesthesia. We compared GBM resections using a 3.0T ioMRI and then without considering the EOR, safety, survival and discussed the indications for using this expensive modality. METHODS: Between March 2006 and November 2011, 56 GBM resections were performed using an ioMRI, and 38 without (control group). The only criterion in order to have access to the ioMRI was its availability. We compared the variables EOR, Karnofsky Performance Scale scores and survival in both groups. RESULTS: In the ioMRI group, 15 patients (26.8%) underwent an immediate second resection increasing the GTR rate of 10.7% and the GTR/NTR rate of 8.9%. There was a significant difference between the use of an ioMRI and the control group in reaching a larger EOR (P=0.049, Fisher's exact test). The effect of using the ioMRI or not on the overall survival, with EOR as covariate, was not significant (P=0.147, Likelihood ratio test). However, the EOR alone had a significant effect on survival (P=0.049, Wald test), with a shorter survival for the patients with a partial resection (PR) than a GTR/NTR (Hazard ratio=1.6, 95% CI HR: 1.00-2.69), with a median overall survival of 15.26 months (95% CI: 12.34-19.08) for the GTR/NTR subgroup versus 10.26 months (95% CI: 6.64-15.82) for the PR subgroup. Multivariate regression analysis also identified age, sex and adjuvant chemotherapy as factors significantly associated with overall survival. CONCLUSIONS: A 3.0T ioMRI improved the quality of resection by 17.8% and increased the GTR/NTR rate by 8.9% up to 73.2% without additional morbidity. A GTR/NTR improves survival duration by about 50%. Thus, it remains reasonable to increase the EOR to reach GTR/NTR using an intraoperative control. However, ioMRI should be limited to the cases for which a GTR/NTR seems preoperatively possible.

The authors describe a rare case of central auditory dysfunction induced by cerebral vasospasm after aneurysmal subarachnoid hemorrhage (SAH). A 55-year-old woman who was admitted after aneurysmal SAH developed cerebral vasospasm on Day 3 affecting mainly the right middle cerebral artery (MCA) and partly the left MCA. The vasospasm became refractory to conventional therapy and was ultimately improved by intraarterial infusion of nimodipine in the right MCA and angioplasty. Severe auditory dysfunction was apparent from Day 10 as the patient was not reactive to speech or environmental sounds. Brain MRI on Day 17 demonstrated infarcted areas mainly in the right hippocampus, medial occipital lobe, and thalamus. The patient underwent further examination using audiometry, speech testing, auditory evoked potentials, functional MRI, and cerebral PET. The initial diagnosis was extended nonverbal agnosia and total pure word deafness. The central auditory dysfunction improved over 6 months, with persisting hyperacusis, tinnitus, and amusia. Central auditory dysfunction is a rare complication after SAH. While cortical deafness may be associated with bilateral lesions of the temporal cortex, partly reversible central auditory dysfunction was observed in this patient after prominently unilateral right temporal lesions. The role of the interthalamic connections can be discussed, as well as the possibility that a less severe vasospasm on the left MCA could have transiently impaired the left thalamocortical auditory pathways.

To investigate the safety and activity of cetuximab in the pre-operative treatment of squamous cell carcinoma of the head and neck (SCCHN).Cetuximab was administered for 2 weeks before surgery to 33 treatment-naïve patients selected for primary surgical treatment. Tumour biopsies, 2-[fluorine-18]-fluoro-2-deoxy-d-glucose positron emission tomography ((18)FDG-PET) and imaging were carried out at baseline and before surgery. The primary aim of the study was safety and the secondary aims included metabolical, radiological and pathological tumour response. Five untreated patients were included as controls.Cetuximab given 24 h before surgery was safe. Ninety percent of patients had (18)FDG-PET partial response (EORTC guideline) in the cetuximab group versus 0% in the control group. Delta maximal standardized uptake values (ΔSUVmax) were correlated with tumour cellularity on the surgical specimens (P0.0001). For patients with ΔSUVmax less than -25% or less than -50%, Ki67 was significantly decreased by cetuximab (P = 0.01 and 0.003). Cetuximab induced down-regulation of pEGFR (P = 0.0004) and pERK (P = 0.003).Short-course pre-operative administration of cetuximab is safe and shows a high rate of (18)FDG-PET response. (18)FDG-PET response was correlated with residual tumour cellularity suggesting that (18)FDG-PET deserves further investigation as a potential early marker of cetuximab activity in SCCHN.

Venous dysfunction has recently been hypothesized to contribute to the pathophysiology of multiple sclerosis (MS). 2D phase-contrast (PC) magnetic resonance imaging (MRI) is a non-invasive and innocuous technique enabling reliable quantification of cerebrospinal fluid (CSF) and blood flows in the same imaging session. We compared PC-MRI measurements of CSF, arterial and venous flows in MS patients to those from a normative cohort of healthy controls (HC). Nineteen MS patients underwent a standardized MR protocol for cerebral examination on a 3T system including Fast cine PC-MRI sequences with peripheral gating in four acquisition planes. Quantitative data were processed using a homemade software to extract CSF and blood flow regions of interest, animate flows, and calculate cervical and intracranial vascular flow curves during the cardiac cycle (CC). Results were compared with values obtained in 21 HC using multivariate analysis. Venous flow patterns were comparable in both groups without signs of reflux. Arterial flows ( P = 0.02) and cervical CSF dynamic oscillations ( P = 0.01) were decreased in MS patients. No significant differences in venous cerebral and cervical outflows were observed between groups, thereby contradicting the recently proposed theory of venous insufficiency. Unexpected decrease in arterial perfusion in MS patients warrants further correlation to volumetric measurements of the brain.

We report on two cases of transient akinetic mutism after massive subarachnoid haemorrhage due to the rupture of an intracranial aneurysm of the anterior cerebral artery (ACA). In the two cases, vasospasm could not be demonstrated by imaging studies throughout the clinical course. Both patients shared common radiological features: a hydrocephalus due to haemorrhagic contamination of the ventricular system and a mass effect of a subpial hematoma on the borders of the corpus callosum. Patients were also investigated using auditory event-related evoked potentials at acute stage. In contrast to previous observations of akinetic mutism, P300 wave could not be recorded. Both patients had good recovery and we hypothesized that this unexpectedly favourable outcome was due to the absence of permanent structural damage to the ACA territory, with only transient dysfunction due to a reversible mass effect on cingulate gyri.

A 75-year-old man was admitted to the Department of internal medicine because of a 2-month history of neurological deterioration. During the previous year, he complained of recurrent sinusitis, asthma, arthralgias, myalgias and asthenia. Later on, an oculomotor palsy, weakness and disturbance of the sensibility of the right upper limb appeared. Blood sample showed 6510 eosinophils per microlitre. The cerebral magnetic resonance demonstrated bilateral frontal and left parietal subcortical lesions from which the most voluminous presented large haemorrhagic areas. A cerebral biopsy showed small vessel's vasculitis, fibrinoid necrosis and extravascular eosinophilic encroachment. A diagnosis of oculomotor palsy secondary to eosinophilic granulomatosis with polyangeitis was then made, which was successfully treated with corticosteroids and cyclophosphamide.

OBJECTIVE: This study aimed to determine whether the shape of the thyroid cartilage and gender influence voice outcomes after a Montgomery thyroplasty implant system (MTIS). METHODS: A retrospective cohort study was performed on 20 consecutive patients who underwent MTIS. Voice outcome variables were the relative decrease in Voice Handicap Index (%) and the absolute increase in maximum phonation time (MPT) (in seconds). Material variables were the angle between the thyroid cartilage laminae (α-angle), the size of the prosthesis, and a combination of both (the α-ratio). Continuous variables were analyzed using medians and were compared between groups using the Mann-Whitney U test. Factors associated with the outcome variables were assessed by multivariable linear regression. A Pearson coefficient was calculated between material variables. RESULTS: The absolute increase in MPT between the pre- and postoperative period was significantly different between men and women, with a median absolute increase of 11.0 seconds for men and of 1.3 seconds for women (P