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PURPOSE OF REVIEW: To review the pathophysiology, presentation, and treatment of neuromyelitis optica spectrum disorder (NMOSD) and its association with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS). RECENT FINDINGS: NMOSD is an autoimmune disorder of the central nervous system that primarily targets astrocytes. Although the prevalence is unknown, the coexistence of NMOSD and SLE/SS is well-recognized. Patients with both NMOSD and SLE or SS require may require unique approaches to diagnosis and management. Coexistence of NMOSD and SLE/SS is important for the rheumatologist and neurologist to be able to recognize. For the rheumatologist, NMOSD and its neurologic symptoms represent a distinct disease process from neurologic complications of the patient's underlying connective tissue disease, and it requires distinct acute and chronic management. For the neurologist, the coexistence of SLE and SS can help to establish a diagnosis of NMOSD, or in some situations, the development of neurologic symptoms secondary to NMOSD can lead to the diagnosis of connective tissue disease.
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Enfermedades del Tejido Conjuntivo , Lupus Eritematoso Sistémico , Neuromielitis Óptica , Síndrome de Sjögren , Enfermedades del Tejido Conjuntivo/complicaciones , Humanos , Lupus Eritematoso Sistémico/complicaciones , Neuromielitis Óptica/complicaciones , Neuromielitis Óptica/diagnóstico , Reumatólogos , Síndrome de Sjögren/complicacionesRESUMEN
Nitrous oxide, also known as "laughing gas," is a naturally occurring gas that is colorless, odorless, nonflammable, and nontoxic. It has been used as an inhalant anesthetic in the medical field for more than 150 years for dental and surgical procedures. Due to its wide availability and ability to cause euphoria, its recreational use is on the rise. We present a case of subacute combined degeneration (SCD) due to nitrous oxide-induced vitamin B12 deficiency. The patient presented with bilateral lower extremity paresthesia, weakness, and ataxic gait. The patient was found to have vitamin B12 deficiency. An MRI of the cervical spine revealed an abnormal T2 signal within the cervical spinal cord extending from the level of C2-C6 affecting only the posterior column. On the fifth day of hospitalization, the patient reported that he had been inhaling nitric oxide from whipped cream cans for recreational use. According to his clinical presentation and laboratory and imaging findings, we concluded that the patient had SCD. The patient slowly improved after receiving vitamin B12 supplementation therapy. Patients presenting with paresthesia, weakness, and laboratory studies indicating vitamin B12 deficiency should be questioned about nitrous oxide recreational use since the incidence is increasing.
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Bilateral acute optic neuritis is a rare and challenging clinical presentation, often associated with conditions like multiple sclerosis or neuromyelitis optica spectrum disorder. We present the case of a 40-year-old woman with a complex medical history, including poorly differentiated squamous cell carcinoma of the cervix (stage IIIC), who presented with a swift and profound bilateral vision loss. Despite initial treatment with high-dose methylprednisolone and therapeutic plasma exchange, her optic nerve enhancement on MRI and negative autoantibody results raised suspicion of paraneoplastic optic neuritis. This prompted consultation with oncology, and the patient initiated chemotherapy. The rapid onset and progression of bilateral optic neuritis in the context of cervical carcinoma emphasize the importance of considering paraneoplastic syndromes in such cases. A multidisciplinary approach involving neurology, ophthalmology, and oncology specialists is vital for the diagnosis and management of these complex presentations. This case underscores the need for heightened awareness of paraneoplastic etiologies in patients with malignancies and unexplained neurological symptoms.
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Auditory manifestations from multiple sclerosis (MS) are not as common as the well-recognized sentinel exacerbations of optic neuritis, partial myelitis, motor weakness, vertiginous episodes, heat intolerance, and eye movement abnormalities. This paper discusses four cases of auditory changes, secondary to MS, and describes the first case, to our knowledge, of palinacousis, the perseveration of hearing, despite cessation of the sound stimulus. For each we characterize the initial complaint, the diagnostic work up, and ultimately, underscore the individualized treatment interventions, that allowed us to achieve a remission in all four cases. Individually codifying the treatment regimens served to mitigate, if not to abolish, the clinical derangements in hearing. Special attention is focused upon examination of the clinical manifestations and the pathophysiologic mechanisms which are responsible for them. We further emphasize the differential diagnostic considerations, and physical exam findings, along with the results of laboratory testing, neuro-imaging sequences, and lesion localization. Taken together, such information is germane to organizing cogently coherent strategic treatment plan(s). We believe that this small case series represents a clinically pragmatic example of 'precision medicine'; a principal theme and goal throughout this paper, the achievement of such in MS, but also as an illustration for the assessment and management schema for neuroimmunologic disorders in general.
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Esclerosis Múltiple , Estimulación Acústica , Vías Auditivas , Audición , Humanos , Esclerosis Múltiple/diagnóstico , Esclerosis Múltiple/diagnóstico por imagenRESUMEN
OBJECTIVE: To determine whether the punctuated administration of low-dose rituximab, temporally linked to B-cell hyperrepopulation (defined when the return of CD19+ B cells approximates 40%-50% of baseline levels as measured before alemtuzumab treatment inception), can mitigate alemtuzumab-associated secondary autoimmunity. METHODS: In this hypothesis-driven pilot study, 10 patients received low-dose rituximab (50-150 mg/m2), a chimeric anti-CD20 monoclonal antibody, after either their first or second cycles of alemtuzumab. These patients were then routinely assessed for the development of autoimmune disorders and safety signals related to the use of dual monoclonal antibody therapy. RESULTS: Five patients received at least 1 IV infusion of low-dose rituximab, following alemtuzumab therapy, with a mean follow-up of 41 months. None of the 5 patients developed secondary autoimmune disorders. An additional 5 patients with follow-up over less than 24 months received at least 1 infusion of low-dose rituximab treatment following alemtuzumab treatment. No secondary autoimmune diseases were observed. CONCLUSIONS: An anti-CD20 "whack-a-mole" B-cell depletion strategy may serve to mitigate alemtuzumab-associated secondary autoimmunity in MS by reducing the imbalance in B- and T-cell regulatory networks during immune reconstitution. We believe that these observations warrant further investigation. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for people with MS, low-dose rituximab following alemtuzumab treatment decreases the risk of alemtuzumab-associated secondary autoimmune diseases.
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Alemtuzumab/farmacología , Enfermedades Autoinmunes/inducido químicamente , Enfermedades Autoinmunes/prevención & control , Linfocitos B/efectos de los fármacos , Factores Inmunológicos/farmacología , Esclerosis Múltiple/tratamiento farmacológico , Rituximab/farmacología , Adulto , Alemtuzumab/administración & dosificación , Alemtuzumab/efectos adversos , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Factores Inmunológicos/administración & dosificación , Factores Inmunológicos/efectos adversos , Masculino , Persona de Mediana Edad , Evaluación de Resultado en la Atención de Salud , Proyectos Piloto , Rituximab/administración & dosificación , Rituximab/efectos adversosRESUMEN
Toxic leukoencephalopathy (TL) is characterized by white matter disease on magnetic resonance imaging (MRI) and evidence of exposure to a neurotoxic agent. We describe a case of cocaine-induced TL in which extensive white matter disease did not preclude full recovery. A 57-year-old man with substance abuse disorder presented with a 5-day history of strange behavior. On admission, he was alert but had difficulty concentrating, psychomotor retardation, and diffuse hyperreflexia. Brain MRI revealed confluent subcortical white matter hyperintensities with restricted diffusion in some but not in other areas. Electroencephalography (EEG) showed mild diffuse slowing. Blood tests were normal except for mild hyperammonemia. Urine screen was positive for cocaine and benzodiazepine but quantitative analysis was significant only for cocaine. Prednisone 60-mg qd was initiated on day 4, tapered over a 5-day period, and discontinued on day 9. He was discharged after 3 weeks. Cognitive function returned to normal 2 weeks after discharge. Five months later, neurologic exam and EEG were normal and MRI showed near-100% resolution of white matter lesions. TL has been attributed to white matter ischemia/hypoxia resulting in demyelination/axonal injury. The clinical, EEG, and MRI findings and time course of recovery of our patient suggest that cocaine-induced inflammation/edema resulted in TL but not in ischemic/hypoxic injury. While inflammation/edema may have regressed when cocaine was discontinued, we cannot exclude a role for prednisone in protecting the patient from the ischemic/hypoxic sequelae of inflammation/edema. MRI is indispensable for diagnosing TL but EEG may provide additional useful diagnostic and prognostic information.
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Edema Encefálico/inducido químicamente , Trastornos Relacionados con Cocaína/complicaciones , Cocaína/toxicidad , Leucoencefalopatías/inducido químicamente , Edema Encefálico/diagnóstico por imagen , Electroencefalografía , Humanos , Leucoencefalopatías/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , NeuroimagenRESUMEN
Refractory seizures or status epilepticus (RS/SE) continues to be a challenge in the inpatient setting. Failure to abort a seizure with antiepileptic drugs (AEDs) may lead to intubation and treatment with general anesthesia exposing patients to complications, extending hospitalization, and increasing the cost of care. Studies have shown a key role of inflammatory mediators in seizure generation and termination. We describe 4 patients with RS/SE that was aborted when dexamethasone was added to conventional AEDs: a 61-year-old female with temporal lobe epilepsy who presented with delirium, nonconvulsive status epilepticus, and oculomyoclonic status; a 56-year-old female with history of traumatic left frontal lobe hemorrhage who developed right face and hand epilepsia partialis continua followed by refractory focal clonic seizures; a 51-year-old male with history of traumatic intracranial hemorrhage who exhibited left-sided epilepsia partialis continua; and a 75-year-old female with history of breast cancer who manifested nonconvulsive status epilepticus and refractory focal clonic seizures. All patients continued experiencing RS/SE despite first- and second-line therapy, and one patient continued to experience RS/SE despite third-line therapy. Failure to abort RS/SE with conventional therapy motivated us to administer intravenous dexamethasone. A 10-mg load was given (except in one patient) followed by 4.0- 5.2 mg q6h. All clinical and electrographic seizures stopped 3-4 days after starting dexamethasone. When dexamethasone was discontinued 1-3 days after seizures stopped, all patients remained seizure-free on 2-3 AEDs. The cessation of RS/SE when dexamethasone was added to conventional antiseizure therapy suggests that inflammatory processes are involved in the pathogenesis of RS/SE.