Analysis Of Diagnosis And Treatment Of137Clinical Patients With Reversible Posterior Leukoencephatopathy Syndrome (RPLS)

Objective:Reversible posterior leukoencephalopathy syndrome(RPLS) is a group of clinical and radiologic syndrome with multipleetiologies，and closed with a variety of clinical medicine (Neurology，Medical imageology，Obstetrics and Gynaecology，Tumor immunology，Organ transplantation science，etc.) and Basic medicine. With thedevelopment of the imaging technology and the accumulation of clinicalexperience, and its pathogenesis, clinical manifestations, neuroimagingcharacteristics, diagnosis and treatment which have also been a newdiscovery and understanding. The disease has complex etiologies，changeable clinical-radiological features and involved many medicaldisciplines， which make clinical or imaging physicians easy tomisdiagnosis or missed diagnosis， and treatment time delay.It isnecessary of analyszing its etiologies pathogenesis, examinationmethods, diagnostic criteria, treatment and prognosis to improvescientific research and the diagnosis and treatment of RPLS.Methods: Retrieved articles about RPLS in Chinese academicliterature library (CNKI)，VIP Chinese Journal Full-text Database，Chinese Journal Full-text Database，and Chinese Biomedical Database， from September2008to January2014. There were all46related articles.After removed7Summaries,1recording meeting,2articles ofincomplete clinical data, and3duplicate articles, there were a total of126patients of RPLS. Combined with the11patients which werediagnosed and cured in The Second Hospital of Jilin University．Wesummarized and analyszed clinical data of137patients of RPLS.Results: All of137patients included36(27.28%) males and101(73.12%) females, the ratio of gender was1:2.81. Their agesranged from6to68years and the averaged age is30.43.There were11(8.02%)Children and126(91.98%) adults, the proportion of them was1:11.5.58patients were Secondary to preeclampsia/eclampsia；32cases were to hypertensive encephalopathy, and in addition to othersecondary causes, such as systemic lupus erythematosus，uremia，acutenephritis， nephrotic syndrome， taking immunosuppressive drugs(cyclophosphamide combined hormone，smooth platinum，BLM，etc.)，blood diseases after chemotherapy,，epilepsy，scleroderma,，thrombocytopenia（SLE after taking hormone therapy)，renal arterystenosis， cerebellar artery abnormalities， renal transplantation.Intracranial hypertension, seizures, unconsciousness, abnormal visionare the common clinical symptoms, some patients had limitedneurological symptoms. The classic imaging features of lesions aremostly symmetry vasogenic edema of white matter based back of the brain. But it can also affect the brain stem，periventricular (deep whitematter)，the corpus callosum，the internal capsule atypical and so on. Itsearly lesions showed no abnormalities or low-density on CT; Lesions onTI-weighted imaging showed low or equal signal, and high signal onT2-weighted images or Flair images. It showed slightly lower or equalsignals on DWI images in patients of doing DW1and ADC images,minority was slightly higher signal; while ADC showed high signal.After active treatment, symptoms and signs of135cases recovered tothe onset before. Lesions that reviewing head CT or MRI disappearcompletely or mostly disappeared and patients had no clinical symptomsfollowed up for3months.One patient treated properly heavier，and headMRI reviewed showed infarction; One patient dead due to brainherniation.Conclusion: RPLS is a complex syndrome, acute or subacuteonset, and most patients have high blood pressure. Clinical symptomsinclude intracranial hypertension symptoms, visual disturbances,seizures, unconsciousness and mental disorders, limited neurologicalsymptoms. Women have high incidence than men, children and adultsmay be the patients. More and more causes is constantly beingdiscovered, which of domestic and foreign patients were different. Themost common cause is eclampsia/pre-eclampsia and hypertensiveencephalopathy for domestic patients. The classic lesion is bilateral symmetry and diffuse vasogenic edema in the white matter of the brain-based rear.But lesion can also be combined or alone, such as thebrainstem, basal ganglia, corpus callosum, periventricular and cortex.Characteristic of signal lesions on CT showed a low density; It showedlow signal or equal signals on T1WI，high signal on T2WI，high signalon FLAIR too;Lesions is mostly low signal or equal signals on DWI, afew slightly higher signal; ADC showed high signal. Combined withreview of recent literature data，it was recommended that standardizedclinical diagnosis of RPLS should be based risk factors， clinicalmanifestations (before and after treatment) and maging mode (beforeand after treatment， especially MRI).Early diagnosis and earlytreatment must be emphasized to prevent irreversible brain damage.