Improving Outcome in Severe Myasthenia Gravis and Guillain-Barré Syndrome.

When progressive and severe, myasthenia gravis and Guillain-Barré syndrome may have the potential for fatal and unfavorable clinical outcomes. Regardless of important differences in their clinical course, the development of weakness of oropharyngeal muscles and respiratory failure with requirement of mechanical ventilation is the main driver of poor prognosis in both conditions. The need for prolonged mechanical ventilation is particularly relevant because it immobilizes the patient and care becomes extraordinarily complex due to daily risks of systemic complications. Additionally, patients with myasthenia gravis often require long-term immunosuppressive treatments with associated toxicity and infectious risks. Unlike myasthenia gravis, the recovery period is prolonged in Guillain-Barré syndrome, but often favorable, even in the more severely affected patients. Outcome, for a large part, is determined by expert neurocritical care.

West Nile Neuroinvasive Disease Presenting as Elsberg Syndrome.

INTRODUCTION: Elsberg syndrome (ES) is a rarely recognized cause of cauda equina syndrome and lower thoracic myelitis, mainly linked to reactivation, or occasionally primary, infection with herpes simplex virus type 2. West Nile virus neuroinvasive disease is rarely considered in the differential diagnosis of patients with ES. CASE REPORT: A 63-year-old man with pancreatic cancer in remission and polymyalgia rheumatica on low-dose prednisone presented with a 10-day history of low-back pain and a viral-type illness with low-grade fever, nausea, and vomiting. Days later, he developed left leg monoparesis, neurogenic bladder, and bowel. Magnetic resonance imaging of the lumbar spine revealed a hyperintense signal abnormality within the central spinal cord and conus medullaris with mild swelling of the conus. Cells, proteins, and glucose in cerebrospinal fluid were 67/mm, 70 mg/dL, and 58 mg/dL, respectively. Serology was positive for West Nile virus IgM. Nerve conduction studies and electromyography showed an acute motor neurogenic process affecting left lumbosacral segments. CONCLUSIONS: West Nile virus neuroinvasive disease is an uncommon condition that should be considered in patients with ES. Determining the etiology of ES in the acute setting may avoid unnecessary diagnostic investigations and treatments.

West Nile virus induces a post-infectious pro-inflammatory state that explains transformation of stable ocular myasthenia gravis to myasthenic crises.

West Nile virus (WNV) infection has been reported to promote myasthenia gravis (MG) and various other diseases that have a presumed autoimmune pathogenesis. Molecular mimicry between WNV proteins and host proteins has been postulated as the major mechanism for WNV-triggered breaking of immunological self-tolerance. We present a patient with stable ocular MG and positive anti-acetylcholine receptor antibodies who progressed to myasthenic crisis after WNV neuroinvasive disease. In this case of stable autoimmune disease with proven auto-antibodies, transformation to generalized disease cannot be attributed to molecular mimicry, which requires that an immune response first be generated against an infectious agent. Rather, the evidence supports the concept of a post-infectious pro-inflammatory state that may contribute to the amplification and promotion of autoimmune disease in some WNV survivors.

Headache and Treatment of Unruptured Intracranial Aneurysms.

BACKGROUND AND PURPOSE: The relationship between unruptured intracranial aneurysms (UIAs) and chronic headache and the impact of aneurysm treatment on headache outcome are controversial. The aim of this study was to determine clinical features of a supposedly primary headache in patients with UIA. We also assessed changes in headache characteristics after UIA treatment. METHODS: We examined clinical and imaging data of patients in whom a UIA was diagnosed during diagnostic workup of a suspected primary headache. Medical records were reviewed and personal telephone follow-ups were performed after UIA treatment to assess changes in the frequency and intensity of the headache. RESULTS: Forty-two patients (76%) reported a substantial improvement in headache frequency and intensity after UIA treatment. Forty-five patients (81%) reported a decrease in headache frequency from a median of 8 days/month before treatment to 1 day/month after treatment (95% confidence interval [CI] 81-83, P < .001). The average intensity in an analog pain scale was 7.7 ± 1.6 before treatment and 5.6 ± 2.4 after treatment (P < .001). Higher headache frequency was associated with a greater odd of improvement after treatment (odds ratio 1.12, 95% CI 1.0-1.26, P = .03). No associations were found between the type of headache, type of treatment (endovascular versus surgical), number, size, or localization of the aneurysms and the response to treatment. CONCLUSIONS: The treatment of UIA had a robust beneficial effect on previous headache. Although a "placebo" effect of aneurysm treatment cannot be ruled out, these results suggest a potential association between UIA and certain chronic headaches usually considered to be primary.

Perception of stroke symptoms and utilization of emergency medical services / Percepción de los síntomas del accidente cerebrovascular y utilización de los servicios de emergencias médicos

ABSTRACT Lack of stroke awareness and slow activation of emergency medical services (EMS) are frequently reported reasons for delayed arrival to the hospital. We evaluated these variables in our population. Methods Review of hospital records and structured telephone interviews of 100 consecutive stroke patients. Forward stepwise logistic regression was used for the statistical analysis. Results Seventy patients (75%) arrived at the hospital 4.5 hours after stroke symptoms onset. The use of EMS did not improve arrival times. Most patients who recognized their symptoms did not use EMS (p < 0.02). Nineteen patients (20%) were initially misdiagnosed. Eighteen of them were first assessed by non-neurologist physicians (p < 0.001). Conclusions Our population showed a low level of stroke awareness. The use of EMS did not improve arrival times at the hospital and the non-utilization of the EMS was associated with the recognition of stroke symptoms. There was a concerning rate of misdiagnosis, mostly by non-neurologist medical providers.

RESUMO La falta de reconocimiento de los síntomas del accidente cerebrovascular (ACV) y la lenta activación de los servicios de emergencias médicos (SEM) son causas frecuentes de demoras en el arribo hospitalario. Nuestro objetivo fue evaluar ambas variables en nuestra población. Métodos Revisión de registros hospitalarios y entrevista telefónica estructurada de 100 pacientes consecutivos internados por ACV. El análisis estadístico se realizó mediante un modelo de regresión logística multivariada por pasos. Resultados Setenta pacientes (75%) arribaron al hospital luego de 4.5 horas del comienzo de los síntomas. El uso de los SEM no mejoró los tiempos de arribo al hospital (p < 0.02). Inicialmente, 19 pacientes (20%) recibieron un diagnóstico erróneo. Dieciocho de ellos fueron evaluados por médicos no neurólogos. (p < 0.001). Conclusiones El reconocimiento de los síntomas de ACV en nuestra población fue bajo. El uso de los SEM no mejoró los tiempos de arribo hospitalario y la no utilización de los mismos se asoció con el correcto reconocimiento de los síntomas por parte de los pacientes. La proporción de diagnósticos erróneos fue preocupante, fundamentalmente entre médicos no neurólogos.

Left atrial appendage occlusion with Amplatzer Cardio Plug is an acceptable therapeutic option for prevention of stroke recurrence in patients with non-valvular atrial fibrillation and contraindication or failure of oral anticoagulation with acenocumarol / La oclusión de la orejuela izquierda con Amplatzer Cardio Plug es una terapia aceptable para prevención del ACV isquémico en pacientes con fibrilación auricular y contraidicación o falla terapéutica con acenocumarol

ABSTRACT Left atrial appendage occlusion (LAAO) appears as a therapeutic option for some atrial fibrillation patients not suitable for oral anticoagulation because an increased hemorrhagic risk or recurrent ischemic events despite anticoagulant treatment. Methods Report of consecutive atrial fibrillation patients treated with LAAO with Amplatzer Cardio Plug because contraindication or failure of oral anticoagulation with acenocumarol. CHA2DS2VASC, HAS-BLED, NIHSS, mRS, procedural complications and outcome were assessed. Seven patients (73 ± 6 year-old) were treated after intracerebral (n = 5) and gastrointestinal (n = 1) hemorrhages or ischemic stroke recurrence while on acenocumarol (n = 1). Results Mean follow up was 18 months. Baseline CHA2DS2Vasc y HAS-BLED scores were 5.6 ± 0.7 and 4.1 ± 0.3 respectively. There were no strokes or deaths. There was only one non-serious adverse event. Conclusion LAAO with ACP appears as a feasible therapeutic option for stroke prevention in patients with atrial fibrillation and failure or contraindication to acenocumarol.

RESUMEN La oclusión de la orejuela auricular izquierda (OOAI) es una opción terapéutica en pacientes con fibrilación auricular y alto riesgo hemorrágico o recurrencia de accidente cerebrovascular isquémico (ACVi) a pesar del tratamiento anticoagulante. Métodos Reporte de pacientes con fibrilación auricular y contraindicación o fallo terapéutico con acenocumarol tratados con OOAI. Se evaluaron escalas CHA2DS2VASC, HAS-BLED, NIHSS y mRS, complicaciones procedurales y resultados. Resultados Siete pacientes (73 ± 6 años) fueron tratados luego de sufrir hemorragia cerebral (n = 5), gastrointestinal (n = 1) o ACVi recurrente a pesar del tratamiento con acenocumarol. Las escalas CHADS2VASC y HAS-BLED fueron 5.6 ± 0.7 y 4.1 ± 0.3 respectivamente. Luego de un seguimeinto promedio fue de 18 meses (3-50) no se registraron ACVi o muertes. Se registró sólo un evento adverso no serio. Conclusión La OOAI es una opción terapéutica factible para prevenir ACVi en pacientes con fibrilación auricular y fallo o contraindicación para recibir acenocumarol.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) in Argentina.

CADASIL is the most common cause of hereditary stroke and vascular dementia. Published information about this disease in South America is scant. We describe clinical and demographic characteristics of 13 patients (10 families) with CADASIL from Argentina.Methods Medical records, diagnostic tests and family history of patients with CADASIL were reviewed.Results Thirteen patients with CADASIL (10 families) were included. All patients had European ancestry. Initial presentation was stroke in most patients (n = 11). Stroke patients later developed cognitive complaints (n = 9), migraine with aura (n = 1), apathy (n = 4) and depression (n = 6). External capsule and temporal lobe involvement on MRI were characteristic imaging findings. Two patients died after intracerebral hemorrhage.Conclusion This is the first report of non-related patients with CADASIL in South America addressing ancestry. Since European ancestry is not highly prevalent in all South American countries, there may be variable incidence of CADASIL within this region.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) in Argentina / Arteropatia cerebral autosomal dominante com infartos subcorticais e leucoencefalopatia (CADASIL) na Argentina

CADASIL is the most common cause of hereditary stroke and vascular dementia. Published information about this disease in South America is scant. We describe clinical and demographic characteristics of 13 patients (10 families) with CADASIL from Argentina.Methods Medical records, diagnostic tests and family history of patients with CADASIL were reviewed.Results Thirteen patients with CADASIL (10 families) were included. All patients had European ancestry. Initial presentation was stroke in most patients (n = 11). Stroke patients later developed cognitive complaints (n = 9), migraine with aura (n = 1), apathy (n = 4) and depression (n = 6). External capsule and temporal lobe involvement on MRI were characteristic imaging findings. Two patients died after intracerebral hemorrhage.Conclusion This is the first report of non-related patients with CADASIL in South America addressing ancestry. Since European ancestry is not highly prevalent in all South American countries, there may be variable incidence of CADASIL within this region.

CADASIL é a causa mais frequente de acidente vascular cerebral e demência hereditários. São poucas as publicações sobre esta doença na América do Sul. Aqui descrevemos dados clínicos e demográficos de 13 pacientes (10 famílias) da Argentina com CADASIL.Métodos Prontuários médicos testes diagnósticos e história familiar de pacientes com CADASIL foram revisados.Resultados Treze pacientes com CADASIL (10 famílias) foram incluídos. Todos os pacientes tinha ancestralidade europeia. A apresentação inicial foi acidente vascular cerebral na maioria dos pacientes (n = 11). Pacientes com acidente vascular cerebral depois desenvolveram alterações cognitivas (n = 9), enxaqueca com aura (n = 1), apatia (n = 4) e depressão (n = 6). Os achados de imagem característicos da RM foram na cápsula externa e no lobo temporal. Dois pacientes morreram por hemorragia intracerebral.Conclusão Este é o primeiro relato de série de casos de pessoas não relacionadas entre si que apresentavam CADASIL na América do Sul, discutindo ancestralidade. Uma vez que a ascendência europeia tem prevalência variada em diferentes países da América do Sul, é possível que esta seja uma variável de incidência de CADASIL nesta região.