Comorbidity of amyotrophic lateral sclerosis and progressive supranuclear palsy: a case report and literature review
10.3760/cma.j.cn113694-20200505-00327
- VernacularTitle:肌萎缩侧索硬化合并进行性核上性麻痹一例并文献复习
- Author:
Yixuan ZHANG
1
;
Yuanjin ZHANG
;
Xiangyi LIU
;
Dongsheng FAN
Author Information
1. 北京大学第三医院神经内科，北京市神经退行性疾病生物标志物及转化研究重点实验室 100191
- From:
Chinese Journal of Neurology
2020;53(12):1040-1044
- CountryChina
- Language:Chinese
-
Abstract:
The co-existence of different neurodegenerative diseases in the same case has received increasing attention and reports. A rare comorbidity of amyotrophic lateral sclerosis (ALS) and progressive supranuclear palsy (PSP) in a sporadic patient was reported. The patient presented with pseudobulbar palsy, freezing gait, and developed muscle weakness and fasciculation. Brain magnetic resonance imaging, positron emission computed tomography and whole exomesequencing showed no evident abnormality. The patient had no response towards the treatment of levodopa, and received supportive treatment and gastrostomy. He is in stable condition by now. Totally 23 cases of ALS with PSP were reviewed, and found that the clinical manifestations were related to the main distribution of pathological inclusion bodies and the location of neuron loss, and the deposition of the same pathological protein in multiple systems may lead to the coexistence of different neurodegenerative diseases. Traditional treatment is generally ineffective, thus support treatment is pivotal. The genetic background and pathogenesis of this comorbidity should be studied in the future.
