Background:
Fungal infections of the central nervous system usually affect immunocompromised patients. Primary Aspergillus myelitis has never been described.
Report:
A 45-year-old immunocompetent ...male with subacute paraplegia was treated for inflammatory myelitis before clinical deterioration requiring mechanical ventilation. Purulent meningitis preceded the formation of a paraspinal nodule biopsied by neurosurgery. Histopathological analysis revealed the presence of fungal hyphae, and polymerase chain reaction was positive for Aspergillus fumigatus. No cause of immunodeficiency was identified in this patient.
Discussion:
Primary Aspergillus myelitis may be confused with inflammatory myelitis and should be considered even in the absence of apparent immunosuppression.
Background:
Epidemiologic studies on coronavirus disease 2019 (COVID-19) in patients with multiple sclerosis (pwMS) have focused on the first waves of the pandemic until early 2021.
Objectives:
We ...aimed to extend these data from the onset of the pandemic to the global coverage by vaccination in summer 2022.
Methods:
This retrospective, multicenter observational study analyzed COVISEP registry data on reported COVID-19 cases in pwMS between January 2020 and July 2022. Severe COVID-19 was defined as hospitalization or higher severity.
Results:
Among 2584 pwMS with confirmed/highly suspected COVID-19, severe infection rates declined from 14.6% preomicron wave to 5.7% during omicron wave (p < 0.001). Multivariate analysis identified age (odds ratio (OR) = 1.43, 95% confidence interval (CI) = 1.25–1.64 per 10 years), male sex (OR = 2.01, 95% CI = 1.51–2.67), obesity (OR = 2.36, 95% CI = 1.52–3.68), cardiac comorbidities (OR = 2.36, 95% CI = 1.46–3.83), higher Expanded Disability Status Scale (EDSS) scores (OR = 2.09, 95% CI = 1.43–3.06 for EDSS 3–5.5 and OR = 4.53, 95% CI = 3.04–6.75 for EDSS ⩾6), and anti-CD20 therapies (OR = 2.67, 95% CI = 1.85–3.87) as risk factors for COVID-19 severity. Vaccinated individuals experienced less severe COVID-19, whether on (risk ratio (RR) = 0.64, 95% CI = 0.60–0.69) or off (RR = 0.32, 95% CI = 0.30–0.33) anti-CD20.
Discussion:
In pwMS, consistent risk factors were anti-CD20 therapies and neurological disability, emerging as vital drivers of COVID-19 severity regardless of wave, period, or vaccination status.
Background
Electroencephalography (EEG) is recommended for the practical approach to the diagnosis and prognosis of encephalitis. We aimed to investigate the prognostic value of standard EEG (
std
...EEG) in adult patients with severe herpes simplex encephalitis.
Methods
We performed a retrospective analysis of consecutive ICU patients with severe herpes simplex encephalitis in 38 French centers between 2006 and 2016. Patients with at least one
std
EEG study performed at ICU admission were included.
std
EEG findings were reviewed independently by two investigators. The association between
std
EEG findings (i.e., background activity, lateralized periodic discharges, seizures/status epilepticus, and reactivity to painful/auditory stimuli) and poor functional outcome, defined by a score on the modified Rankin Scale (mRS) of 3 to 6 (moderate to severe disability or death) at 90 days, were investigated.
Results
We included 214 patients with at least one available
std
EEG study. The first
std
EEG was performed after a median time of one (interquartile range (IQR) 0 to 2) day from ICU admission. At the time of recording, 138 (64.5%) patients were under invasive mechanical ventilation. Lateralized periodic discharges were recorded in 91 (42.5%) patients, seizures in 21 (9.8%) and status epilepticus in 16 (7.5%). In the whole population, reactivity to auditory/noxious stimuli was tested in 140/214 (65.4%) patients and was absent in 71/140 (33.2%) cases. In mechanically ventilated patients,
std
EEG reactivity was tested in 91/138 (65.9%) subjects, and was absent in 53/91 (58.2%) cases. Absence of reactivity was the only independent
std
EEG finding associated with poor functional outcome in the whole population (OR 2.80, 95% CI 1.19 to 6.58) and in the subgroup of mechanically ventilated patients (OR 4.99, 95% CI 1.6 to 15.59). Adjusted analyses for common clinical predictors of outcome and sedation at time of
std
EEG revealed similar findings in the whole population (OR 2.03, 95% CI 1.18 to 3.49) and in mechanically ventilated patients (OR 2.62, 95% CI 1.25 to 5.50).
Conclusions
Absence of EEG reactivity to auditory/noxious stimuli is an independent marker of poor functional outcome in severe herpes simplex encephalitis.
Des troubles du sommeil tels que et l’hypersomnie ont été décrits dans l’encéphalite à anti-NMDAr. L’agrypnie, stade le plus extrême du status dissociatus, n’a jamais été rapportée.
Une patiente de ...22 ans a présenté un tableau de bradypsychie et troubles du comportement, avec des anticorps anti-NMDAr positifs associés à un tératome ovarien droit. Après une amélioration initiale des symptômes sous traitement immunomodulateur, l’évolution a été marquée par une aggravation comportementale et cognitive, avec un déficit sévère en quantité de sommeil. Deux enregistrements en vidéo-EEG avec montage polysomnographique ont mis en évidence une destruction profonde de l’architecture du sommeil avec de courtes séquences de sommeil N1, des phases de mouvements oculaires rapides non physiologiques et une hyperactivité motrice et autonomique, confirmant un tableau d’agrypnia excitata. L’identification de l’agrypnie a permis la mise en évidence d’une rechute de l’encéphalite en relation avec le développement d’un second tératome ovarien controlatéral. Une seconde ligne d’immunothérapie a permis de normaliser le sommeil et les symptômes cliniques.
L’agrypnie, forme la plus sévère du status dissociatus, correspondant à un déficit subtotal de sommeil et sa déstructuration profonde sans stade physiologique identifiable, a été observée au décours d’une rechute d’encéphalite à anti-NMDAr. Une insomnie très sévère a été rapportée à la phase aiguë de l’ENMDAr sans donnée polysomnographique disponible, et pourrait correspondre à un tableau d’agrypnie.
Les enregistrements polysomnographiques manquent cruellement à la phase aiguë des encéphalites et permettraient une meilleure compréhension de la clinique et du pronostic de ces pathologies.
Sepsis-associated encephalopathy is a severe neurologic syndrome characterized by a diffuse dysfunction of the brain caused by sepsis. This review provides a concise overview of diagnostic tools and ...management strategies for SAE at the acute phase and in the long term. Early recognition and diagnosis of SAE are crucial for effective management. Because neurologic evaluation can be confounded by several factors in the intensive care unit setting, a multimodal approach is warranted for diagnosis and management. Diagnostic tools commonly employed include clinical evaluation, metabolic tests, electroencephalography, and neuroimaging in selected cases. The usefulness of blood biomarkers of brain injury for diagnosis remains limited. Clinical evaluation involves assessing the patient's mental status, motor responses, brainstem reflexes, and presence of abnormal movements. Electroencephalography can rule out non-convulsive seizures and help detect several patterns of various severity such as generalized slowing, epileptiform discharges, and triphasic waves. In patients with acute encephalopathy, the diagnostic value of non-contrast computed tomography is limited. In septic patients with persistent encephalopathy, seizures, and/or focal signs, magnetic resonance imaging detects brain injury in more than 50% of cases, mainly cerebrovascular complications, and white matter changes. Timely identification and treatment of the underlying infection are paramount, along with effective control of systemic factors that may contribute to secondary brain injury. Upon admission to the ICU, maintaining appropriate levels of oxygenation, blood pressure, and metabolic balance is crucial. Throughout the ICU stay, it is important to be mindful of the potential neurotoxic effects associated with specific medications like midazolam and cefepime, and to closely monitor patients for non-convulsive seizures. The potential efficacy of targeted neurocritical care during the acute phase in optimizing patient outcomes deserves to be further investigated. Sepsis-associated encephalopathy may lead to permanent neurologic sequelae. Seizures occurring in the acute phase increase the susceptibility to long-term epilepsy. Extended ICU stays and the presence of sepsis-associated encephalopathy are linked to functional disability and neuropsychological sequelae, underscoring the necessity for long-term surveillance in the comprehensive care of septic patients.
spp. myelopathies are very rare. We report a case of subacute longitudinally extensive transverse myelitis in an apparently immunocompetent 55-year-old man. After a negative infectious workup, ...corticosteroids and plasma exchange were initiated. Although there was a transient initial improvement, symptoms then worsened, and the lumbar puncture was repeated.
was isolated in the CSF, and a diagnosis of spinal cord candidiasis was made. Gene panel sequencing for inborn immune deficiencies identified a homozygous disease-causing
variant. Despite antifungal treatment, necrotic myelitis, meningoencephalitis, and cerebral vasculitis developed. Fungal spinal cord infections can mimic inflammatory myelitis, and beta-D-glucan testing of both serum and CSF may help narrow down the diagnosis. In cases of severe or unexpected invasive
infection, even adults and apparently immunocompetent patients should be screened for inborn immune deficiencies and CARD9 deficiency in particular.
Nous rapportons le cas d’une patiente de 35 ans sans antécédent notable, qui a présenté un épisode neurologique dans les suites d’une vaccination ChAdOx1 nCoV-19 (COVISHIELD AstraZeneca) contre le ...SARS-CoV-2.
Une semaine après la vaccination, la patiente a présenté des céphalées avec nausées et douleurs rétro-orbitaires sans anomalie à la consultation d’ophtalmologie, suivie une semaine plus tard d’une rétention aiguë d’urine spontanément résolutive.
Trois semaines après, la patiente est hospitalisée pour un syndrome confusionnel bref. La ponction lombaire retrouvait une méningite panachée (189 éléments, 64 % de lymphocytes, protéines 0,66g/L), d’amélioration spontanée en 2 semaines.
L’IRM cérébromédullaire a révélé une myélite en C2, des hyper signaux du corps calleux, du tronc cérébral et du diencéphale mais également des signes évocateurs d’hypertension intracrânienne avec une dilatation des gaines des nerfs optiques.
L’examen ophtalmologique a mis en évidence un œdème papillaire bilatéral asymétrique avec une acuité visuelle conservée. Dans ce contexte, la ponction lombaire a été re-contrôlée avec une pression d’ouverture à 31cmH2O.
Des anticorps anti-MOG ont été retrouvés à la fois dans le sérum et dans le LCR. La ponction lombaire évacuatrice et l’introduction d’un traitement par acetazolamide a permis une amélioration rapide des symptômes.
À six mois du début des symptômes, la patiente a présenté une baisse de l’acuité visuelle à 9/10 dans un contexte de céphalées, faisant découvrir une névrite optique droite. Les anti-MOG étaient toujours détectables dans le sérum. Un traitement par trois bolus de methylprednisolone a été débuté avant un relais par azathioprine. À quatre mois de l’introduction d’azathioprine, aucune récidive n’a été observée.
Au total, il s’agit d’un épisode inflammatoire récidivant polyfocal du système nerveux central avec myélite cervicale, méningoencéphalite et hypertension intracrânienne associée à des anticorps anti-MOG dans les suites d’une vaccination COVID-19.
Malgré leur impact négatif sur la qualité de vie dans les encéphalites auto-immunes, les troubles du sommeil associés aux encéphalites à anticorps anti-glutamic acid décarboxylase (GAD65) n’ont ...jamais été étudiés.
Décrire les phénotypes neurologiques du sommeil associés à l’encéphalite à anti-GAD65 par enregistrements polysomnographiques.
Six patients avec une encéphalite à anti-GAD65 (quatre avec encéphalite limbique, deux avec syndrome de la personne raide, SPR) et 12 témoins sains ont bénéficié d’interrogatoire du sommeil et de vidéo-polysomnographie (V-PSG) sur une première nuit, suivi de cinq tests itératifs de latence d’endormissement (TILE, évaluant la propension à s’endormir en journée), puis une V-PSG de 18h avec sommeil ad libitum (évaluant le besoin excessif de sommeil).
Par rapport aux témoins, les latences d’endormissement aux TILE étaient plus courtes dans le groupe patients (médiane IQR 5,84,5, 6,0 vs 17,716,3, 19,7 min, p=0,001), et toutes≤8minutes. L’index de micro-éveils nocturnes était plus bas (2,52,3, 3,0/h vs 22,313,8, 30,0/h, p=0,002), mais le temps total de sommeil (TTS) similaire entre les groupes (621464,651min vs 542,5499,582min, p=0,51). Aucune parasomnie ou trouble respiratoire du sommeil n’a été détecté.
Tous les patients présentaient une hypersomnie centrale avec des latences aux TILE réduites et un TTS normal, indépendamment de la phase (subaiguë ou rémission), du phénotype (encéphalite limbique ou SPR) et des traitements reçus. Ce sommeil continu associé à un faible index de microéveils suggère que l’encéphalite touche plus les systèmes d’éveil que les générateurs du sommeil.
L’hypersomnie centrale à temps de sommeil total non allongé est une caractéristique des encéphalites à anticorps anti-GAD65.
Natalizumab is a high-efficacy therapy for recurrent multiple sclerosis (RMS) with a four-week administration interval. Controlled trials have shown that extending this interval to six weeks led to ...better safety without increasing the risk of relapse. We aimed to analyze the safety of extending the natalizumab interdose interval from 4 to 6 weeks in a real-life setting.
This monocentric retrospective self-controlled study included adult patients with RMS treated with natalizumab with a four-week interval between infusions for a minimum of six months, before switching to a six-week interval. The main outcomes were the incidence of MS relapse, new MRI lesions, and MRI activity signs during the two periods, with patients being their own controls.
Fifty-seven patients were included in the analysis. The mean (95%CI) annualized relapse rate (AAR) before natalizumab introduction was 1.03 (0.52; 1.55). During the four-week interval dosing period, no patient presented with an MS relapse, and seven (13.5%) patients had new MRI lesions. During the six-week interval dosing period, no relapse was observed and two (3.6%) patients had new MRI lesions.
We did not observe more relapses or signs of MRI activity when extending the interval between natalizumab infusions from four to six weeks.
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•Extending intervals between natalizumab (NTZ) infusions decreases the risk of progressive multifocal leukoencephalopathy.•57 patients treated with NTZ with an interval dosing of 4 weeks (SID) were switched to an interval dosing of 6 weeks (EID).•During SID and EID, no clinical reactivations were observed, EDSS scores and radiological activity were similar.•Natalizumab 6 weeks EID does not increase the risk of disease activity while reducing costs and improving quality of life.
