Clinical analysis of 5 patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes
10.3760/cma.j.issn.1671-8925.2012.10.020
- VernacularTitle:五例MELAS综合征的临床分析
- Author:
Xu-En YU
1
;
Xun WANG
;
Yong-Guang SHI
;
Wei-Ming GAO
;
Yan YAN
;
Rao RAO
;
Wei WANG
;
Zhi-Hua ZHOU
;
Nan CHENG
;
Gang PENG
Author Information
1. 安徽中医学院神经病学研究所附属医院神经内科
- Keywords:
Mitochondrial myopathy;
Clinical manifestation;
Muscle pathology
- From:
Chinese Journal of Neuromedicine
2012;11(10):1050-1053
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical data,muscle pathological findings and recent follow-up results of 5 patients being diagnosed as having mitochondrial encephalopathy,lactic acidosis,and stroke-like episodes (MELAS).Methods A retrospective analysis was carried out on the clinical manifestations,sero-enzymology,electrophysiology,iconography and muscle biopsy pathology,and recent follow-up results of 5 patients with MELAS,who admitted to our hospital from December 2008 to June 2011.Results Headache as first symptom appeared in 3 patients,hemiparesis as first symptom in 1 patient and upper gastrointestinal bleeding in 1.The total body seizure in the course was noted in 4 patients and psychiatric symptoms in 2.The creatine kinase level was normal in 4 with one being mildly elevated.Four patients were observed no abnormal electromyography with one having myogenic lesions.Brain MRI showed significant abnormality in 5 patients,mainly presented as permanent cerebral infarction and cerebral atrophy.Five patients were found strongly SDH-reactive blood vessels (SSVs) in muscle tissue pathological staining and the SSVs of CCO (+).After mitochondrial protection treatment,follow-up for six months showed that 3 patients has been restored to normal work and mildly improvement was noted 2 patients.Conclusion The brain MR imaging of 5 patients with MELAS is significantly abnormal,and muscle biopsy pathology and tissue enzymatic staining are important methods in diagnosing the disease; generally,the disease is a benign course.
