RESUMEN
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.
Asunto(s)
Esclerosis Múltiple , Neuromielitis Óptica , Neutropenia , Autoanticuerpos , Humanos , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/tratamiento farmacológico , Glicoproteína Mielina-Oligodendrócito , Neuromielitis Óptica/tratamiento farmacológico , Neutropenia/inducido químicamente , Estudios ProspectivosRESUMEN
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.
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Factores Inmunológicos/administración & dosificación , Inmunosupresores/administración & dosificación , Esclerosis Múltiple Recurrente-Remitente/terapia , Vías de Administración de Medicamentos , Esquema de Medicación , Francia , Humanos , Factores Inmunológicos/efectos adversos , Inmunosupresores/efectos adversos , Calidad de Vida , Resultado del TratamientoRESUMEN
AIM: To determine the incidence and main characteristics of cerebrovascular events as the presenting manifestations of myeloproliferative neoplasm (MPN). METHODS: The Hematology in Lyon (HEMILY) registry is a prospective database (763 patients) of all cases of MPN diagnosed since 2005 in the Rhône-Alpes district of France. The MPN cases were divided into four groups: polycythemia vera (PV); essential thrombocythemia (ET); myelofibrosis (MF); and atypical MPN. The ischemic stroke subtype was classified according to TOAST criteria. RESULTS: A stroke history revealed MPN in 35 (4.3%) patients: 22 (63%) had an ischemic stroke; eight (23%) had a transient ischemic attack; four (11%) had cerebral venous thrombosis; and one (3%) had hemorrhagic stroke. All patients had hemoglobin and/or platelet count abnormalities. In addition, 12 (34%) patients had PV, 21 (60%) had ET, one (3%) had MF and one (3%) had atypical/unclassified MPN. The JAK2 V617F mutation was found in 83% of patients. In 18 (51%) patients, an additional mechanism of stroke was present (atherosclerosis in 10 patients, atrial fibrillation in one patient and dissection in another). The median NIHSS score at entry was 2, and the median modified Rankin Scale score at 3 months was 0. Compared with the general MPN population, stroke-MPN patients presented with significantly higher levels of hemoglobin (P<0.001) and were more frequently positive for the JAK2 V617F mutation (P=0.044). CONCLUSION: Stroke revealing MPN is rare. However, careful attention should still be paid to blood counts even in patients with obvious stroke etiologies, as early diagnosis permits prompt treatment and decreases the risk of recurrence, thus limiting morbidity and mortality.
Asunto(s)
Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/diagnóstico , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/etiología , Adulto , Anciano , Estudios de Cohortes , Diagnóstico Diferencial , Femenino , Francia/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Trastornos Mieloproliferativos/epidemiología , Sistema de Registros , Estudios Retrospectivos , Accidente Cerebrovascular/epidemiologíaRESUMEN
We report a 41-year-old woman with rapidly progressive left hemiparesis, revealing an inflammatory reactivation of a previously known parietal Baló's concentric sclerosis lesion. The first attack occurred five years before. After a slow recovery following high-dose steroid infusions the patient stabilized. Because of recurrent ataxia and left hemiparesis a new magnetic resonance imaging was performed showing an extension of the initial lesion with a peripheral gadolinium enhancement on T1-weighted images. Such a reactivation pattern of an isolated Baló's concentric sclerosis lesion, occurring some years later, is described for the first time.
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Esclerosis Cerebral Difusa de Schilder/patología , Adulto , Antiinflamatorios/uso terapéutico , Esclerosis Cerebral Difusa de Schilder/complicaciones , Esclerosis Cerebral Difusa de Schilder/tratamiento farmacológico , Femenino , Humanos , Inmunoglobulina G/líquido cefalorraquídeo , Inflamación/etiología , Inflamación/patología , Imagen por Resonancia Magnética , Paresia/etiología , Esteroides/uso terapéuticoRESUMEN
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.
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Agnosia/complicaciones , Apraxias/complicaciones , Ataxia/complicaciones , Oftalmopatías/complicaciones , Lóbulo Parietal/fisiopatología , Percepción Espacial/fisiología , Agnosia/diagnóstico , Agnosia/fisiopatología , Apraxias/diagnóstico , Apraxias/fisiopatología , Ataxia/diagnóstico , Ataxia/fisiopatología , Oftalmopatías/diagnóstico , Oftalmopatías/fisiopatología , Humanos , Modelos Biológicos , Síndrome , Percepción Visual/fisiologíaRESUMEN
INTRODUCTION: Cryptococcus spp. is a fungus responsible for 600,000 deaths per year worldwide, mainly in immunosuppressed subjects. However, 20% of cases occur in immunocompetent subjects. Neuropathic disorders involving the auditory nerve have been reported, but vestibular disorders have never been described in detail. We report the case of an immunocompetent man, who presented audiovestibular disorders leading to a diagnosis of cryptococcal meningitis. CASE REPORT: A 39-year-old man was referred for balance disorders and right sensorineural hearing loss. He presented right vestibulo-saccular impairment and bilateral absence of auditory brainstem responses. Brain MRI was suggestive of cryptococcal meningitis. A cystic lesion in the right flocculus compressed the vestibulocochlear nerve. During monthly follow-up, pure tone audiometry gradually improved and speech audiometry in silence returned to normal. Partial resynchronization of the auditory afferent pathways was observed only on the contralateral side to vestibulocochlear nerve compression, while complete recovery of saccular function was observed. DISCUSSION: Cryptococcal meningitis in immunocompetent subjects may be accompanied by lesions of the auditory and vestibular afferent pathways. Recovery of hearing and balance was observed in response to medical treatment and early vestibular rehabilitation.
Asunto(s)
Pérdida Auditiva Sensorineural , Meningitis Criptocócica , Adulto , Audiometría de Tonos Puros , Nervio Coclear , Potenciales Evocados Auditivos del Tronco Encefálico , Humanos , Masculino , Meningitis Criptocócica/diagnósticoRESUMEN
INTRODUCTION: 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. CASE REPORTS: 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. CONCLUSION: 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.
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Neoplasias Encefálicas/patología , Quiste Dermoide/patología , Metabolismo de los Lípidos/fisiología , Adulto , Encéfalo/patología , Neoplasias Encefálicas/metabolismo , Neoplasias Encefálicas/cirugía , Quiste Dermoide/metabolismo , Quiste Dermoide/cirugía , Cefalea/etiología , Humanos , Imagen por Resonancia Magnética , Masculino , Rotura , Adulto JovenRESUMEN
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.
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Enfermedades Autoinmunes Desmielinizantes SNC/tratamiento farmacológico , Enfermedades Autoinmunes Desmielinizantes SNC/inmunología , Factores Inmunológicos/efectos adversos , Neutropenia/inducido químicamente , Rituximab/efectos adversos , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Esclerosis Múltiple/tratamiento farmacológico , Esclerosis Múltiple/inmunología , Glicoproteína Mielina-Oligodendrócito/inmunología , Neuromielitis Óptica/tratamiento farmacológico , Neuromielitis Óptica/inmunologíaRESUMEN
BACKGROUND: 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. MATERIAL AND METHODS: 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). RESULTS: 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. CONCLUSION: tocilizumab appears to be an effective therapy for patients with refractory NMOSD or MOGAD. Subcutaneous and intravenous injections appear to be equally effective.
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Neuromielitis Óptica , Anticuerpos Monoclonales Humanizados , Acuaporina 4 , Autoanticuerpos , Francia , Humanos , Glicoproteína Mielina-Oligodendrócito , Neuromielitis Óptica/diagnóstico por imagen , Neuromielitis Óptica/tratamiento farmacológico , Uso Fuera de lo Indicado , Estudios RetrospectivosRESUMEN
INTRODUCTION: Striking developments in stroke epidemiology, initially based on the results of the Framingham study, have greatly improved our neuroepidemiological knowledge of the disease. STATE OF ART: The development of stroke registries has made it possible to evaluate the descriptive epidemiology of stroke and its evolution. With the increasing use of CT-scan, MRI, and either cardiac or vascular imaging, the diagnosis of stroke and its subtypes has been made easier. Over the last 20 years, a decrease in the incidence and mortality of stroke has been observed in Western countries. In contrast, in Dijon, which has the only population-based stroke registry in France, stable incidence rates have been reported. However, over the same period, age at stroke onset has risen by five years in men and eight years in women, which is probably related to both population aging and improvements in primary prevention and general health. The reported decrease in case-fatality rates suggests better acute management of stroke patients, and explains in part the increase in the prevalence of stroke. In addition, the assessment of vascular risk factors has demonstrated that high blood pressure remains the principal risk factor for both ischemic and hemorrhagic stroke, and that antihypertensive treatment is able to reduce stroke incidence. PERSPECTIVES: Epidemiology studies could make it possible to measure the impact of new therapeutic strategies applied in both primary and secondary prevention. CONCLUSION: Prevention, diagnosis, and acute treatment of stroke have considerably improved, but cerebrovascular diseases together with myocardial infarction remain the leading cause of death. Despite the absence of a rise in the incidence of stroke, its prevalence has increased. This is due to the decrease in case-fatality rates. As a consequence, there is an urgent need to organize health networks around stroke. Moreover, the rise in stroke-free life expectancy is a positive finding that reflects improvements in prevention.
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Accidente Cerebrovascular/epidemiología , Adulto , Anciano , Clima , Ambiente , Métodos Epidemiológicos , Femenino , Francia/epidemiología , Humanos , Hipertensión/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Sistema de Registros , Factores de Riesgo , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/mortalidadRESUMEN
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 be managed the same as transient ischemic attack involving the brain, in order to prevent a subsequent stroke.
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Amaurosis Fugax , Amaurosis Fugax/diagnóstico , Amaurosis Fugax/etiología , Amaurosis Fugax/fisiopatología , Estenosis Carotídea/complicaciones , Diagnóstico Diferencial , Técnicas de Diagnóstico Oftalmológico , Epilepsias Parciales/diagnóstico , Arteritis de Células Gigantes/complicaciones , Humanos , Isquemia/etiología , Ataque Isquémico Transitorio/complicaciones , Migraña con Aura/diagnóstico , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/diagnóstico , Neuropatía Óptica Isquémica/complicaciones , Oclusión de la Arteria Retiniana/complicaciones , Evaluación de Síntomas , Tromboembolia/complicacionesRESUMEN
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.
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Amaurosis Fugax/diagnóstico , Amaurosis Fugax/etiología , Enfermedades Vasculares/complicaciones , Enfermedades Vasculares/diagnóstico , Amaurosis Fugax/epidemiología , Ceguera/diagnóstico , Ceguera/epidemiología , Ceguera/etiología , Enfermedades de las Arterias Carótidas/complicaciones , Enfermedades de las Arterias Carótidas/diagnóstico , Diagnóstico Diferencial , Humanos , Ataque Isquémico Transitorio/complicaciones , Enfermedades Vasculares/epidemiología , Trastornos de la Visión/diagnóstico , Trastornos de la Visión/epidemiología , Trastornos de la Visión/etiologíaAsunto(s)
Adenocarcinoma/complicaciones , Antígenos de Neoplasias/inmunología , Encefalitis Límbica/etiología , Proteínas del Tejido Nervioso/inmunología , Trastornos de la Motilidad Ocular/etiología , Neoplasias Gástricas/complicaciones , Adenocarcinoma/patología , Autoanticuerpos/inmunología , Encéfalo/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasias Gástricas/patologíaRESUMEN
Migraine with visual aura is marked by recurrent episodes of transient visual disturbance, often followed by headaches. Its pathophysiology has not been fully understood, but visual auras might be related to a self-propagating wave of cortical depolarization called "cortical spreading depression", triggering a trigemino-vascular "storm" ultimately leading to headaches. The most specific visual symptom is the "fortification spectrum" consisting of glimmering jagged lines spreading from the center to the periphery, and leaving a transient scotoma in its wake. Other visual symptoms are numerous, ranging from elementary positive or negative visual phenomena to complex and elaborate hallucinations. The diagnosis can be made according to the International Classification of Headache Disorders revised in 2013. The main goal of the treatment is to relieve the patient's pain quickly and to decrease the frequency of the episodes.
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Migraña con Aura , Depresión de Propagación Cortical/fisiología , Cefalea/complicaciones , Cefalea/diagnóstico , Humanos , Trastornos Migrañosos/clasificación , Trastornos Migrañosos/diagnóstico , Trastornos Migrañosos/epidemiología , Trastornos Migrañosos/terapia , Migraña con Aura/diagnóstico , Migraña con Aura/epidemiología , Migraña con Aura/etiología , Migraña con Aura/terapia , Escotoma/complicaciones , Escotoma/diagnósticoAsunto(s)
Diplopía/microbiología , Neurosífilis/diagnóstico , Anciano , Humanos , Masculino , Oftalmoplejía/microbiologíaRESUMEN
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.