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Progressive multifocal leukoencephalopathy (PML) may develop in follicular lymphoma patients treated with bendamustine-rituximab. In this report, treatment with pembrolizumab successfully inhibited the clinical progression of PML by promoting radiologically demonstrated immune restoration inflammatory syndrome (IRIS), allowing complete clearance of the virus. These findings may further support the use of pembrolizumab in PML with special consideration for the potential occurrence of IRIS.

Introduction Central diabetes insipidus is a heterogeneous condition characterized by decreased release of antidiuretic hormone by the neurohypophysis resulting in a urine concentration deficit with variable degrees of polyuria. The most common causes include idiopathic diabetes insipidus, tumors or infiltrative diseases, neurosurgery and trauma. Temozolomide is an oral DNA-alkylating agent capable of crossing the blood-brain barrier and used as chemotherapy primarily to treat glioblastoma and other brain cancers. Cases Two men (aged 38 and 54 years) suddenly developed polyuria and polydispsia approximately four weeks after the initiation of temozolomide for a glioblastoma. Plasma and urine parameters demonstrated the presence of a urinary concentration defect. Management The clinical and laboratory abnormalities completely resolved with intranasal desmopressin therapy, allowing the continuation of temozolomide. The disorder did not relapse after cessation of temozolomide and desmopressin and relapsed in one patient after rechallenge with temozolomide. Discussion Our report highlights the importance of a quick recognition of this exceptional complication, in order to initiate promptly treatment with desmopressin and to maintain therapy with temozolomide.

Magnetic resonance imaging is of paramount importance in the presurgical evaluation of drug resistant epilepsy. Detection of a potentially epileptogenic lesion significantly improves seizure outcome after surgery. To optimize the detection of subtle lesions, MRI post-processing techniques may be of essential help. In this study, we aimed to evaluate the detection rate of the voxel-based morphometric analysis program (MAP) in a prospective trial. We aimed to study the MAP+ findings in terms of their clinical value in the decision-making process of the presurgical evaluation. We included, prospectively, 21 patients who had negative MRI by visual analysis. In a first step, results of the conventional non-invasive presurgical evaluation were discussed, blinded to the MAP results, in multidisciplinary patient management conferences to determine the possible seizure onset zone and to set surgical or invasive evaluation plans. Thereafter, MAP results were presented, and the change of initial clinical plan was recorded. All MAP detections were reaffirmed by a neuroradiologist with epilepsy expertise. For the 21 patients included, mean age at the time of patient management conference was 26 years (SD 15 +/- years, range: 5-54 years). In total, 4/21 had temporal lobe epilepsy and 17/21 had extra-temporal lobe epilepsy. MAP was positive in 10/21 (47%) patients and in 6/10 (60%) a diagnosis of focal cortical dysplasia was confirmed after neuroradiologist review, corresponding to a 28% detection rate. MAP+ findings had a clear impact on the initial management in 7/10 patients (7/21, 33% of all patients), which included an adaptation of the intracranial EEG plan (6/7 patients), or the decision to proceed directly to surgery (1/7 patients). MRI post-processing using the MAP method yielded an increased detection rate of 28% for subtle dysplastic lesions in a prospective cohort of MRI-negative patients, indicating its potential value in epilepsy presurgical evaluation.

Susac syndrome (SS) is a central nervous system vasculitis characterized by the clinical triad of encephalopathy, sensorineural hearing loss, and visual disturbance caused by branch retinal artery occlusion. It is considered as an inflammatory disorder, and an autoimmune etiology is suggested. A 29-year-old man with a history of recent cocaine abuse developed the clinical features of SS. Toxicological analysis including hair testing revealed that cocaine had been adulterated with levamisole. After an initial clinical improvement following corticosteroid therapy, the introduction of mycophenolate mofetil was justified a few weeks later by the progression (or relapse) of the retinal injury, followed by complete recovery. The presence of levamisole has been documented in patients with multifocal inflammatory leukoencephalopathy (MIL). Further investigations are needed to determine if levamisole as an adulterant of cocaine could also play a role in the development of rapidly progressive leukoencephalopathy in young men, with Susac or Susac-like syndromes as possible variants of MIL.

The role of neuroinflammation in epileptogenesis is extensively investigated, but short-term effects of seizures on established CNS pathologies are less studied and less predictable. We describe the case of a woman with previous recurrent episodes of focal cerebral haemorrhage of unknown cause who developed a pseudo-tumoural oedema triggered by provoked focal status epilepticus. A brain biopsy revealed that the underlying condition was primary angiitis of the CNS. Ictal-induced blood-brain barrier dysfunction allows the entry of water and inflammatory molecules that, in the context of CNS inflammatory diseases, may trigger a self-reinforcing process. Caution should be observed when tapering antiepileptic drugs in patients with such conditions.

Case report : A 30-year-old woman with a history of bilateral sensorineural deafness since the age of 14 years, a right occipital ischemic stroke with left hemianopia and epilepsy was admitted to the neurology department with speech disorders and a new epileptic insult. MRI revealed extensive cytotoxic edema of the left temporo–parieto–occipital cortex and mirrored right-sided sequelae (Fig. 1a, b). MR-angiography and MR-venography (not shown) were normal. [...]

Teaching Point: Central nervous system adverse effects of cytarabine treatment include aseptic meningitis, myelopathy, and more rarely, encephalopathy, seizures, and cerebellar dysfunction. This case illustrates a cytarabine-induced encephalitis with predominant cerebellar involvement.

A 27-year-old female patient with no significant medical history presented with persistent headache. Clinical examination and blood tests (including normal calcium levels) were unremarkable. CT examination revealed an osteolytic lesion of the squamous part of the left temporal bone eroding the two tables and extending inwards into the middle cranial fossa and outwards into the upper part of the temporo-mandibular joint. [...]

Background Type IIIb dysplasia is a subtype of focal cortical dysplasia associated with a tumor, most frequently with gangliogliomas then with dysembryoplastic neuroepithelial tumors (DNETs). Their preoperative diagnosis often remains equivocal since specific features are missing. The functional results (i.e., seizure free) is good with 81%–87% of Engel Ia at 5-year follow-up. Case Description A 4-year-old boy presented with a 1-year history of severe, invalidating, drug-resistant epilepsy. Imaging workup demonstrated a huge left limbic lesion, of which diagnosis remained speculative. Because of worsening neurological status, resective surgery was recommended after multidisciplinary discussion. The resection was performed through left transtemporal approach under neuronavigation (C.R.). Postoperative magnetic resonance imaging assessed uncomplicated near-total resection. Histopathological analysis showed combined features of a DNET of nonspecific type and a focal cortical dysplasia. Conclusion We describe a rare condition of type IIIb dysplasia combining a focal cortical dysplasia with a DNET. Preoperative diagnosis of the lesion was of utmost difficultly, thereby rendering mandatory a thorough histopathological examination of resected specimen in the vast majority of cases. Increased recognition of the condition brings up the hypothesis of a genetic continuum or linkage between the 2 conditions. Functional results on seizure activity after ablative surgery are good and maximal safe resection should be the goal.

It can be challenging to disentangle human immunodeficiency virus (HIV)-related infectious optic neuropathy and secondary triggered auto-immune disease when an HIV positive patient presents with vision loss. We report a 44-year-old untreated HIV positive Congolese woman who presented with two episodes of vision loss associated with pain in first her left eye and then her right eye and was diagnosed with a relapsing optic neuropathy. A correlation was observed between the clinical activity and cerebrospinal fluid viral load, CD4-count in the blood and magnetic resonance imaging signs of blood - optic nerve barrier breakdown. CD4 cell counts and viral loads are great clinical features to identify the type of acute optic neuropathy since differential diagnosis between an infectious optic neuropathy or an auto-immune induced optic neuropathy such as neuromyelitis optica spectrum disorder or immune reconstitution inflammatory syndrome can be puzzling.

Teaching Point: Contrast-enhanced FLAIR images have unsurpassed value for the radiological depiction of hypertensive papilledema. FLAIR acquisition should therefore be performed after intravenous contrast, especially in the of work-up of intracranial hypertension and/or tumor.

Dear Editors, Bing–Neel syndrome (BNS) is a rare form of Waldenström’s Macroglobulinemia (WM), accounting for about 1% of the WM patients which is characterized by the infltration of the central nervous system (CNS) by tumor cells [...]

Dear Editor, Asymptomatic striatocapsular slit-like hemorrhage (SSH) has been recently described as a potential new radiological marker for hypertensive cerebral small vessel disease (cSVD). We herein report a case of a young patient with a left acute putaminal hemorrhage associated with no other cSVD marker than a contralateral asymptomatic SSH. [...]

We report a clinical case of an isolated cortical cerebral venous thrombosis (CVT), without any history of genetic or acquired thrombophilia, associated with COVID-19. Up to now, a few cases of sinus CVT have been reported to be possibly linked to a SARS-CoV-2 infection [...]

Dear editor, Burned-out testicular tumors are characterized by spontaneous regression of the tumor at the primary site [1]. This spontaneous regression has been described in various types of cancer and involve autoimmune mechanisms similar to those induced by immune check-points inhibitors [2]. We herein report a case of a paraneoplastic encephalomyelitis leading to the diagnosis of a burned-out seminoma. [...]

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.

Among other autonomic dysfunctions complicating acute spinal cord injury, deep hypothermia is rare but may induce serious cardiovascular complications. There are few pharmacological options to influence hypothermia. A 66-year-old woman was transferred to the intensive care unit (ICU) for serious cardiac arrhythmias (atrial fibrillation and asystole) in the context of a deep hypothermia (axillary temperature below 32°C). She had been admitted to the hospital two months before for an acute L4-L5 infectious spondylodiscitis without any initial neurological deficit. After surgery for epidural abscess drainage, she became paraplegic due to spinal cord infarction (from C7 to T6 levels) in the upper territory of the anterior spinal artery. In the ICU, the patient experienced several episodes of asystole and hypotension associated with a core body temperature below 35°C. Common causes of hypothermia (drugs, hypothyroidism, etc.) were excluded. A definitive pacemaker had to be inserted, but hypotension persisted. The prescription of oral progesterone (200 mg·d−1) helped to maintain a core temperature higher than 35°C, with a withdrawal of vasopressors. This case report illustrates that patients with incomplete spinal cord injury may present with delayed and deep hypothermia leading to serious cardiovascular complications. Progesterone could be able to influence positively central and peripheral thermal regulation.

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).

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

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.