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The added value of patient-reported outcome measures (PROMs) in addition to standard clinical outcome tools in the assessment of relapsing-remitting multiple sclerosis (RRMS) patients' status is increasingly recognized. PROMs facilitate the detection of hidden aspects of MS and help to integrate the patient's subjective experience of health-related quality of life (HRQoL) status and treatment satisfaction in a holistic way. However, the relationship between PROMs and clinical and cognitive status has been scarcely investigated up to now. To investigate the association of PROMs with physical and cognitive disability in a cohort of RRMS patients at initiation of a new disease-modifying treatment. In this cross-sectional bicenter study, 59 consecutive RRMS patients underwent neurological examination with EDSS assessment, comprehensive cognitive tests (BVMT-R, SDMT, CVLT-II) and a set of self-reported questionnaires. Lesion and brain volumes were analyzed and processed by the automated MSmetrix software (Icometrix, Leuven, Belgium). Spearman's correlation coefficient was used to evaluate the association of collected variables. A cross-sectional logistic regression analysis was performed to find baseline correlates of cognitive impairment. Of the 59 RRMS patients (mean age 39 ± 9.8 years, 79.7% female, median EDSS 2.0), 33 (56%) had cognitive impairment. While almost all dimensions of health, explored by PROMs, were impacted in the overall sample, no significant difference was observed in patients with and without cognitive impairment. All PROMs were significantly associated with EDSS (R = 0.37-0.55; p

Background Chronic active lesions (CAL) in multiple sclerosis (MS) have been observed even in patients taking high-efficacy disease-modifying therapy, including B-cell depletion. Given that CAL are a major determinant of clinical progression, including progression independent of relapse activity (PIRA), understanding the predicted activity and real-world effects of targeting specific lymphocyte populations is critical for designing next-generation treatments to mitigate chronic inflammation in MS. Methods We analyzed published lymphocyte single-cell transcriptomes from MS lesions and bioinformatically predicted the effects of depleting lymphocyte subpopulations (including CD20 B-cells) from CAL via gene-regulatory-network machine-learning analysis. Motivated by the results, we performed in vivo MRI assessment of PRL changes in 72 adults with MS, 46 treated with anti-CD20 antibodies and 26 untreated, over ~2 years. Findings Although only 4.3% of lymphocytes in CAL were CD20 B-cells, their depletion is predicted to affect microglial genes involved in iron/heme metabolism, hypoxia, and antigen presentation. In vivo, tracking 202 PRL (150 treated) and 175 non-PRL (124 treated), none of the treated paramagnetic rims disappeared at follow-up, nor was there a treatment effect on PRL for lesion volume, magnetic susceptibility, or T1 time. PIRA occurred in 20% of treated patients, more frequently in those with ≥4 PRL (p=0.027). Interpretation Despite predicted effects on microglia-mediated inflammatory networks in CAL and iron metabolism, anti-CD20 therapies do not fully resolve PRL after 2-year MRI follow up. Limited tissue turnover of Bcells, inefficient passage of anti-CD20 antibodies across the blood-brain-barrier, and a paucity of Bcells in CAL could explain our findings.

Cerebral infarction is a very rare complication of diabetic ketoacidosis (DKA) which is a metabolic disorder caused by insulin deficiency. A previously healthy 6-year-old boy with a newly diagnosed Type 1 diabetes mellitus presented with a severe DKA. The patient, who tested positive for SARS-CoC-2 nasopharyngeal PCR, developed about 72 hours after admission a Parinaud’s syndrome (PS), also known as dorsal midbrain syndrome, which is described as an up-gaze saccadic paresis, a convergence-retraction nystagmus, a light-near dissociation of the pupils and occasionally a lid retraction. The brain magnetic resonance imaging revealed an ischemic infarction in the left thalamus and the thalamo-mesencephalic junction with a slight extension in the midbrain tegmentum. His symptoms improved gradually and at 3-weeks follow-up he had a full neuro-ophthalmological recovery. By describing a Parinaud syndrome as a neuro-ophthalmologic complication in diabetic ketoacidosis (DKA) crisis, which, to our best knowledge, has not been described yet, our case expands the knowledge of the neurological manifestations occurring in children during diabetic ketoacidosis and reiterates the importance to keep those patients under strict neurological monitoring for at least 72 hours, especially in severe DKA and to request early brain imaging for any child with neurological deterioration.

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.

Contains fulltext : 282718.pdf (Publisher’s version ) (Closed access) BACKGROUND AND PURPOSE: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by clinical manifestations involving the brain, eye and skin. SWS is commonly caused by somatic mutations in G protein subunit Alpha Q (GNAQ). Five cases of subunit Alpha 11 (GNA11) mutations have been reported. We studied phenotypic features of GNA11-SWS and compared them with those of classic SWS. METHODS: Within two European multidisciplinary centers we looked for patients with clinical characteristics of SWS and a GNA11 mutation. Clinical and radiological data were collected retrospectively and prospectively. RESULTS: We identified three patients with SWS associated with a somatic GNA11 mutation. All had disseminated capillary malformation (CM) and hyper- or hypotrophy of an extremity. At birth, the CMs of the face, trunk and limbs were pink and patchy, and slowly darkened with age, evolving to a purple color. Two of the patients had glaucoma. All had neurological symptoms and moderate brain atrophy with a lower degree of severity than that classically associated with SWS. Susceptibility-weighted imaging (SWI) and contrast-enhanced fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging demonstrated the best sensitivity to reveal the pial angiomas. CONCLUSIONS: We have differentiated two distinct clinical/radiological phenotypes of SWS; GNAQ- and GNA11-SWS. The classic GNAQ-SWS is characterized by a homogeneous dark-red CM, commonly associated with underlying soft tissue hypertrophy. The CM in GNA11-SWS is more reticulate and darkens with time, and the neurological picture is milder. SWI and post-contrast FLAIR sequences appear to be necessary to demonstrate leptomeningeal angiomatosis. Anti-epileptic medication or future targeted therapies may be useful, as in classic SWS.

Background The efficacy of the subthalamic nucleus (STN) stimulation for Parkinson’s disease has been widely established. The microlesion effect (MLE) due to deep brain stimulation (DBS) electrode implantation has been reputed to be a good predictor for long-term efficacy of the procedure but its analysis in asleep implantation is still unclear. We thus analyzed MLE rate in our strategy of targeting the STN on MRI under general anesthesia and its correlation with our long-term results. Method We retrospectively analyzed 32 consecutive parkinsonian patients implanted with a DBS targeting the STN bilaterally under general anesthesia between October 2013 and December 2020. Targeting was performed after head frame and localizer placement using a stereotactic peroperative robotic 3D fluoroscopy (Artis Zeego, Siemens) fused with preoperative CT and MRI data. We collected intraoperative data, postoperative occurrence of MLE, modification of Unified Parkinson Disease Rating Scale item III (UPDRS III) postoperatively and at subsequent visit, as well as reduction of medication. Results The mean operative time was 223 min. No permanent complication occurred. MLE was observed in 90.7%. The mean follow-up time was 17 months. The UPDRS III for the off medication/on stimulation condition improved by 64.8% from baseline. The mean dose reduction of Prolopa after the surgical procedure was 31.3%. Conclusions Direct targeting of STN under general anesthesia based on preoperative CT and MRI data fused with a preoperative 3D fluoroscopy is safe. It allows for a high rate of postoperative MLE (90.7%) and results in prolonged clinical improvement. Keywords Deep brain stimulation · STN · General anesthesia · Parkinson’s disease · Microlesion effect

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. [...]

Different surgical approaches have been described for selective amygdalohippocampectomy in patients with pharmacoresistant temporal lobe epilepsy. The aim of this study was to report the results of the innovative anterior trans-superior temporal gyrus approach in a single-center series. Patients' characteristics, postoperative outcomes, and complications were reviewed in a series of 8 consecutive patients with temporal lobe epilepsy operated on using the anterior trans-superior temporal gyrus approach between November 2015 and April 2017. Over a mean 2.5-year follow-up, 7 of 8 patients (87.5%) remained seizure-free (Engel class I). Only 1 patient (12.5%) was not cured (Engel class III) with no clear explanation for treatment failure. Mean operative time was 237 minutes, which was 80 minutes shorter compared with the classic transsylvian approach. No perioperative deaths were recorded and there were no visual field defects or visual acuity impairments secondary to the approach. One patient experienced a left posterior thalamocapsular stroke. The anterior trans-superior temporal gyrus approach is feasible, fast, and safe for selective amygdalohippocampectomy in patients with drug-refractory temporal lobe epilepsy. This approach allows preservation of the optic radiation but cuts part of the uncinate fasciculus and potentially the anterior aspect of the anterior bundle of the middle longitudinal fasciculus.

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.