RESUMO
【Objective】 To investigate cortical thickness changes in the face-head region of the primary motor cortex (PMC) and its effect on survival in amyotrophy lateral sclerosis (ALS) patients. 【Methods】 A retrospective analysis was performed on 105 ALS patients who underwent head MRI scan at the same time. The A4hf (face-head) region of PMC was used as the region of interest (ROI). According to clinical symptoms, patients were divided into two groups: bulbar involvement and non-bulbar involvement. The differences of clinical features and cortical thickness in ROI were analyzed. According to the symptoms of bulbar palsy, physical examination of nervous system and EMG of tongue muscle, the patients with bulbar palsy were divided into lower motor neuron (LMN), upper motor neuron (UMN) and LMN+UMN groups. The differences of bulbar subgroup score and ROI of cortical thickness were analyzed. Age at onset, body mass index, delayed time of diagnosis, bulbar subgroup score, and ROI cortical thickness were included in survival analysis. 【Results】 ① The ROI cortical thickness was significantly lower in bulbar involvement group than non-bulbar involvement group (-0.198±0.87 vs. 0.235±0.95, P=0.017). ② There were no significant differences in the bulbar subgroup scores or cortical thickness of ROI between LMN, UMN and LMN+UMN groups (P>0.05). ③ Survival analysis showed age of onset (HR=3.296, 95% CI:1.63-6.664, P=0.001), delayed time of diagnosis (HR=0.361, 95% CI:0.184-0.705, P=0.003), bulbar subgroup score (HR 0.389, 95% CI:0.174-0.868, P=0.021), and ZRE_ROI cortical thickness (HR=2.309, 95% CI:1.046-5.096, P=0.038) were independent influencing factors of ALS survival. 【Conclusion】 Cortical thickness in A4hf (face-head) region can more objectively reflect UMN signs of region bulbar. In addition to age of onset and delayed time of diagnosis, bulbar subgroup score and cortical thickness of face-head region are also independent influencing factors, and cortical thinning in face-head region is a protective factor for survival of ALS patients.
RESUMO
Objective: To explore the effect of bulbar involvement time on survival time of patients with spinal-onset amyotrophic lateral sclerosis (ALS). Methods: We followed up 168 patients with spinal-onset ALS admitted to our hospital from January 2, 2011 to December 31, 2017 until December 31, 2018. Univariate and multivariate analyses were performed to evaluate the affecting factors of the ALS patients' survival time. Kaplan-Meier analysis was made to evaluate the effects of bulbar involvement time on survival time. Results: COX multivariate analysis showed that the risk of death in age-onset <55 y patients was 0.72 times that in age-onset ≥55 y (P=0.09), the risk of death in diagnosis delay time <10.98 m patients was 2.64 times that ≥10.98 m (P<0.001); the risk of death in bulbar involvement time ≥11.5 m and bulbar uninvolvement was 0.30 and 0.32 times respectively that bulbar involvement time <11.5 m (P<0.001). Kaplan-Meier analysis showed differences among bulbar involvement time <11.5 m, ≥11.5 m and bulbar uninvolvement groups (median survival time 20.37 m vs. 40.6 m vs. 39.60 m, Test statistic =39.96, P<0.001). The 2-year, 3-year and 5-year survival rates were 32.17%, 10.80% and 0%, respectively, in bulbar involvement time <11.5 m patients; 89.20%, 57.24% and 10.53% in bulbar involvement time ≥11.5 months patients; and 62.16%, 38.39% and 10.53% in bulbar uninvolvement patients. Conclusion: Similar to the diagnosis delay time and whether to have taken riluzole, the occurrence of bulbar involvement at 11.5 month after onset was an independent risk factor affecting survival time in spinal-onset ALS. The median survival time in patients with bulbar involvement time <11.5 months was significantly shorter than that in patients with bulbar involvement time ≥11.5 months and bulbar uninvolvement.
RESUMO
La miastenia gravis (MG) es una enfermedad autoinmune que afecta a la unión neuromuscular, y que se caracteriza por la presencia de autoanticuerpos dirigidos contra los receptores de membrana postsinápticos, bloqueando así la transmisión neuromuscular, y logrando de esta forma un descenso en el número de receptores en la placa neuromotora. Se presenta clínicamente como una debilidad muscular focalizada, de algunos grupos musculares, o generalizada. En el contexto ORL las presentaciones más clásicas afectan a la musculatura bulbar, presentándose como disfonía, disfagia, debilidad de la musculatura facial o la combinación de todas ellas. Presentamos 2 casos clínicos de pacientes con manifestaciones totalmente distintas de la misma enfermedad. En el primer caso se presenta a una paciente con compromiso leve de la musculatura bulbar y en el segundo, un paciente con una miastenia generalizada que es internado grave en la UCI por una crisis miasténica. Realizamos una revisión bibliográfica de las últimas pruebas diagnósticas y tratamientos para la MG, y tratamos de definir los signos y síntomas que nos ayudarán como otorrinolaringólogos a un diagnóstico y tratamiento oportuno.
Myasthenia gravis is an autoimmune disease of the neuromuscular junction, characterized by the presence of autoantibodies directed against the postsynaptic membrane receptors, blocking neuromuscular transmission in skeletal striated muscles, leading to a decrease in the number of receptors at the motor neuroplate. Clinically, myasthenia gravis presents as focalized or generalized muscle weakness. For the ENT the most frequent presentation is the bulbar involvement which presents as dysphonia, dysphagia, weakness of facial musculature or a combination of all. We report 2 cases of patients with completely different manifestations of the same disease. The first case is a patient with mild bulbar musculature compromise and the other, is a patient with severe generalized myasthenia who had to be admitted to the ICU for a myasthenic crisis.