ABSTRACT
Cerebral amyloid angiopathy (CAA) is a common cerebral small vessel disease, mainly caused by β-amyloid deposition on the small vessels less than 200 μm in diameter in cortex and leptomeninges. CAA is a major cause of spontaneous intracerebral hemorrhage in the elderly, especially lobar location. Early symptoms are insidious, and as the disease progress, they manifest as cerebral hemorrhage, cognitive decline, transient focal neurological episodes, cerebral infarction, epilepsy, headache, etc. MRI revealed that CAA is a disease in which bleeding and ischemia coexist, and even inflammation and immune responses are involved. MRI findings of CAA include cerebral hemorrhage, cerebral microbleeds, convexity subarachnoid hemorrhage and cortical superficial siderosis, cortical microinfarcts, CAA-associated inflammation, white matter hyperintensities, enlarged perivascular spaces, cerebral atrophy and lacune, etc. The same patient often has several of the above manifestations, and each manifestation has different specificity for the diagnosis of CAA. The rapid development of MRI technology has led to the improvement of the diagnostic level of CAA, and it is of great clinical significance to understand these imaging findings. This article reviews the MRI findings of sporadic CAA.
ABSTRACT
Objective:To analyze the efficacy and safety of enzyme therapy in late-onset Pompe disease (LOPD) patients, so as to provide reference for the treatment and prognosis of LOPD.Methods:The effect of α-glucosidase (GAA) on a patient diagnosed with LOPD in the Affiliated Hospital of Jining Medical University was observed and analyzed. Besides, literature related to enzyme therapy in LOPD patients were searched in PubMed, Web of Science, Medline databases. Twenty-one studies containing clinical data from 910 LOPD patients related to enzyme therapy were finally included for analysis.Results:The patient developed muscle weakness since he was 16 years old. The GAA activity in peripheral blood was 0. Electromyography suggested myogenic lesions in both lower extremities. Compound heterozygous mutations of GAA gene were found by next- generation sequencing. Muscle biopsy revealed characteristic vacuolar changes. After eight years of diagnosis, the patient was given enzyme therapy for 18.5 months, 20 times in total. The symptoms of muscle weakness were slightly improved in the early stages of treatment without obvious adverse reactions. Most of the 910 LOPD patients were stabilized or had improved muscular and (or) respiratory function following treatment with GAA.Conclusion:GAA treatment is effective and well tolerated. In patients with advanced severe LOPD, enzyme replacement therapy remains effective even years after onset.
ABSTRACT
Objective:To explore the effects of behavior intervention on patients with ischemic cerebrovascular disease recurrence, carotid artery stenosis recurrence, and quality of life after carotid artery stenting implantation.Methods:Sixty patients with carotid stenosis who underwent stenting surgery between January 2017 and July 2018 in Affiliated Hospital of Jining Medical University were recruited.The subjects were randomly divided into behavioral intervention group and control group.The control group was routinely followed up after carotid artery stenting implantation.The behavioral intervention group added home visit, education, guidance of the control and detection of risk factors for cerebrovascular disease of stent implantation.Results:After 12 months of intervention, the incidence of ischemic cerebrovascular disease and the recurrence rate of carotid restenosis in the behavioral intervention group(6.7%, 3.3%) were significantly lower than those in the control group(30.0%, 20.0%) (both P<0.05). Six months after the intervention and 12 months after the intervention, the self-management ability score (intervention group: six months after the intervention (171.20±18.43), 12 months after the intervention (179.90±14.34); control group: six months after the intervention (160.77±13.43); 12 months after the intervention (164.27±14.85)) and quality of SS-QOL score (intervention group: 6 months after intervention (188.47±16.25), 12 months after intervention (203.17±13.84); control group: 6 months after intervention (170.67±15.82); 12 months after intervention (183.80±18.19)of the intervention group were higher than that of the control group, the difference is statistically significant (all P<0.05). Conclusion:Behavioral intervention after carotid artery stenting implantation can effectively reduce the incidence of stroke and the recurrence rate of carotid stenosis and improve the prognosis of patients.The mechanism may be related with that the behavioral intervention can improve the self-management ability and the quality of life of patients with carotid stenosis.