Chez les adultes, le spectre des maladies démyélinisantes associées aux anticorps MOG (MOG-SD) constitue une entité décrite récemment. Il englobe près de 25 % des patients présentant un trouble du ...spectre de la neuro-myélite optique (NMO-SD) négatif pour les anticorps anti-aquaporin-4 (AQP4-Abs). La manifestation la plus fréquemment observée est la névrite optique (ON). L’objectif de cette communication est de résumer la littérature sur le sujet et de compléter la description clinique et radiologique des atteintes du nerf optique en s’appuyant sur une cohorte française de près de 50 patients multicentrique.
Le syndrome de Balint correspond à l’association d’une ataxie optique, d’une simultagnosie et d’une apraxie du regard ; il traduit un dysfonctionnement du cortex pariétal postérieur, le plus souvent ...par atteinte bilatérale. Depuis ses premières descriptions ce syndrome a été soumis à de nombreuses réinterprétations et controverses. Une première partie développera l’aspect clinique, les causes, les descriptions des symptômes, les méthodes d’examens et enfin les corrélations neuroanatomiques. Une seconde partie traitera de l’intégration de ce syndrome dans le cadre des neurosciences visuelles, en particulier via l’attention visuelle. Nous aborderons le phénomène du remapping et des travaux récents pouvant contribuer à mieux comprendre et approcher la physiopathologie des manifestions de l’ataxie optique, de la simultagnosie ou de l’apraxie du regard.
Balint's syndrome corresponds to the combination of optic ataxia, simultanagnosia and gaze apraxia. It generally results from a bilateral dysfunction of the posterior parietal cortex. Since its early descriptions the syndrome has been subject to many interpretations and controversies. In this article we will reconsider the current concept of Balint's syndrome. A first part will develop the clinical aspects, causes, description of symptoms, examination techniques and neuroanatomical correlations. In a second part, we will discuss how this syndrome can be included in the background of visual neurosciences, particularly through a visual attentional aspect. We will discuss the phenomenon of remapping and some recent data that may contribute to explain the pathophysiology of manifestations as optic ataxia, simultanagnosia or gaze apraxia.
Today, first-line treatments for multiple sclerosis include injectable immunomodulators – some of which have been on the market for nearly 25 years – as well as teriflunomide and dimethyl fumarate, ...which are more recent, but have opened the way for oral treatments. These drugs are considered similar in effectiveness, and their safety and side-effect profiles are generally reassuring. These treatments have been associated with a reduction in radiological and clinical disease activity, and a positive effect on patient quality of life, especially when introduced early in the disease process. This article will discuss data on first-line treatments currently available in France, their effectiveness and safety, and their place in pediatric patients and in woman who plan to become pregnant.
Les ostéoarthropathies neurogènes sont des complications peu fréquentes mais graves des déficits sensitifs chroniques en particulier des neuropathies diabétiques. Il s’agit d’une ostéoarthrite ...destructrice souvent peu douloureuse, consécutive à la perte de la sensibilité profonde et au défaut de protection contre les microtraumatismes des articulations touchées. Nous rapportons l’observation d’une arthropathie neurogène du genou consécutive à une pathologie médullaire dysraphique chez une femme de 55
ans et discutons l’intérêt du traitement par les bisphosphonates malgré l’absence de recommandations consensuelles.
Neurogenic arthropathy is a severe complication of chronic sensitive deficits that occurred commonly in diabetic neuropathies. It is a destructive and painless osteoarthritis associated with a loss of the deep sensitivity and a defect of protective reactions against chronic articular microtraumatisms. We report a 55-year-old woman with neuroarthropathy of the knee resulting from a spina bifida. Bisphosphonate use is an effective but non-consensual treatment.
Late-onset neutropenia (LON) after anti-CD20 therapy is a poorly described side effect in inflammatory disorders of the CNS. In this prospective study, patients treated with Rituximab or Ocrelizumab ...for MS, neuromyelitis optica spectrum disorders or MOG antibody-associated disease (MOGAD) were asked to perform complete blood count (CBC) every two weeks for six months, with the aim of identifying LON. Out of 152 patients, two (1,32%) had an absolute neutrophil count <1,000/mm3: one patient with MOGAD had agranulocytosis and one patient with MS had grade 3 neutropenia. Both were asymptomatic. These results confirm that LON after anti-CD20 therapy in inflammatory disorders of the CNS is not exceptional. Nevertheless, this biological complication remains too infrequent to justify close systematic CBC follow-up.
Few cases of late onset neutropenia after RITUXIMAB treatment (LONART) have been reported in patients with neuroinflammatory disorders. We conducted a retrospective analysis of patients treated with ...RITUXIMAB for neuromyelitis optica spectrum disorders (NMOSD), MOG-antibody-associated disease (MOGAD) and multiple sclerosis (MS) at the Toulouse University Hospital from November 2007 to October 2019. Ten patients with LONART were identified in a total of 385 patients: 4/25 were MOGAD patients, 2/20 were NMOSD patients and only 4/340 were MS patients (p < 0,05). Six required intravenous antibiotics whereas four were asymptomatic. Eight patients received new infusions of RITUXIMAB after resolution of their neutropenia. Neutropenia recurred in one patient.
The objective of the study was to evaluate the indication, efficacy and safety of tocilizumab, a humanized anti-interleukin-6 receptor antibody, in patients with neuromyelitis optica spectrum ...disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody associated diseases (MOGAD) encountered in current neurological practice.
We conducted a retrospective analysis of an exhaustive cohort of patients with inflammatory CNS disorders at Toulouse University Hospital, France, from 2014 to 2020. Efficacy was evaluated with clinical outcome by the Annual Relapse Rate, and radiological outcome with MRI data. The other outcomes were adverse events and effectiveness according to the form of injection (intravenous or subcutaneous).
Seven patients were treated with tocilizumab: four patients had NMOSD with AQP4+ antibodies (57%) and three had MOGAD (43%). Tocilizumab was administered in the presence of persistent clinical activity and/or severe side effects with other immunosuppressant medications. The median follow-up on tocilizumab was 23 months (4-50 months). All patients started with monthly intravenous injection, then three switched to a subcutaneous form. All patients were relapse-free throughout the duration of treatment with tocilizumab, and one presented with a new cervical lesion on MRI. Four patients had no adverse effect, two had a significant increase in infection rate, and one had dyslipidemia.
tocilizumab appears to be an effective therapy for patients with refractory NMOSD or MOGAD. Subcutaneous and intravenous injections appear to be equally effective.
Les kystes dermoïdes sont des tumeurs bénignes rares du système nerveux central, d’évolution lente. Ils se développent au cours de la vie embryonnaire, à partir d’une anomalie de fermeture du tube ...neural et sont essentiellement situés sur l’axe médian du système nerveux central. Ils sont responsables de nombreux symptômes aspécifiques dont les plus fréquents sont les céphalées et les crises convulsives, et peuvent se rompre, surtout de façon spontanée. La rupture est également de symptomatologie variable, allant du caractère totalement asymptomatique jusqu’au décès dans les cas extrêmes.
Nous rapportons deux cas de rupture de kyste dermoïde intracérébral. La rupture du kyste chez un patient, totalement asymptomatique, a été découverte lors d’IRM successives. Chez l’autre patient, la rupture du kyste a été diagnostiquée au cours d’un bilan pour des céphalées quotidiennes. Ces kystes dermoïdes n’ont pas été traités chirurgicalement. Une simple surveillance par IRM a été préconisée devant la bonne évolution spontanée des deux patients.
La chirurgie d’exérèse totale ou sub-totale des kystes dermoïdes n’est pas la seule alternative thérapeutique. Un traitement conservateur avec une surveillance régulière par IRM cérébrale peut être suffisant et doit être discuté si les symptômes de rupture ne mettent pas en jeu le pronostic vital.
Dermoid cysts are rare slow-growing benign tumors of the central nervous system generally diagnosed in the third to fifth decade. They are formed from inclusion of ectodermal elements during neural tube closure, and are mostly located along the cranial or spinal midline axis. They cause many non specific symptoms such as headache and seizures, and may spontaneously rupture spreading fatty droplets into the ventricles and subarachnoid spaces. Rupture of dermoid cysts causes sequelae which may vary from no symptoms to death. In general, subtotal surgical removal is required for ruptured dermoid cysts.
We report two cases of ruptured intracranial dermoid tumor with non-specific clinical presentations. The first rupture was asymptomatic and discovered on brain magnetic resonance imaging (MRI) performed for other purposes. The second case was identified on brain imaging performed because of daily headache. These dermoid cysts were not surgically treated. Surveillance was advised because of the spontaneously favourable outcome observed in both cases.
Surgical removal is not the only treatment of ruptured dermoid cyst. Monitoring with brain MRI can be sufficient if the rupture has no severe clinical impact.
Transient monocular blindness is an acute episode of ischemic origin in which one eye has profound visual loss, followed by full recovery within one hour. Transient monocular blindness most often ...occurs in the setting of retinal ischemia secondary to carotid embolism, but other mechanisms have been reported, including thrombosis (most often in the setting of giant cell arteritis), hemodynamic disorders (secondary to severe carotid stenosis) or vasospasm. Transient monocular blindness is considered a transient ischemic attack originating in the carotid arteries and must benefit from the same management as transient ischemic attack involving the brain, in order to prevent a subsequent stroke.
