Clinical Study Of Posterior Reversible Encephalopathy Syndrome In Children With Hematological Diseases

Purpose:1.To investigate the precipitating factors,pathogenesis,clinical manifestations,imaging features,and prognosis of posterior reversible encephalopathy syndrome(PRES),and to improve the understanding of the disease.2.Analysis of the possible mechanism of PRES induced by remission chemotherapy regimen in acute lymphoblastic leukemia combined with literature.3.Analysis the possible mechanisms of PRES induced by immunosuppressant cyclosporine A combined with literatur.Method:Retrospective study;Chose 9 cases of PRES occurred during the hospitalization of the pediatric central blood group in our hospital in the last 5 years,combined with the literature data to analysis the the precipitating factors,clinical manifestations,magnetic resonance imaging(MRI)features and prognosis of children with posterior reversible encephalopathy syndrome(PRES)during the treatment of hematological diseases.Combined with literature,analyzing the possible mechanism of PRES induced by Chemotherapeutic drugs in acute lymphoblastic leukemia and immunosuppressive agent cyclosporine A.Results:1.There were 9 cases in this group,five women and four men.The age ranged from 1 years and 7 months to 12 years and 5 months,with an average age of 7 years.2.In 6 cases of acute lymphoblastic leukemia,PRES occurred in the induction chemotherapy stage.All patients were treated with VDLD chemotherapy.The interval between initiation of chemotherapy and PRES was 15d-32d(mean 2 1d).3.3 patients had PRES during the course of oral cyclosporine A.The time of taking cyclosporine A before onset was 3d-20d,with an average of 14d.When PRES occurred,the plasma concentration of cyclosporine A was 132ng/ml,160ng/ml and 22.3ng/ml.4.The main clinical manifestations of this disease are epileptic seizures and hypertension5.The increase of serum lactate dehydrogenase and electrolyte disturbance are the main abnormal indexes in laboratory examination.6.Five patients underwent lumbar puncture examination within one weeks after the onset of the disease.Cerebrospinal fluid pressure increased in three cases,and two cases of cerebrospinal fluid protein were slightly elevated.7.Electroencephalogram(EEG)was performed in eight patients within one weeks after onset,7 cases showed abnormal EEG examination,among which 5 cases showed increased 8activity and high amplitude,and 2 cases showed epileptic discharge.8.Seven patients completed the first cranial nuclear magnetic resonance(MRI)examination within one weeks,Cranial MRI indicates that the lesion is mainly located in the subcortical white matter of the top and occipital lobe.Most of the lesions are bilateral,and the unilateral lesions are rare.Bilateral incomplete symmetry,it shows long T1,long T2 signal,FLAIR high signal,the focus can also be located outside the occipital region.The head MRI was reexamined after three months of onset,six cases were restored to normal,three cases were narrowed,but not completely disappeared.9.After active treatment,7 patients had good prognosis,and the other 2 had sequelae.Conclusion:1.The conclusion of a retrospective study:1)Cytotoxic drugs such as methotrexate and immunosuppressants such as cyclosporine A are important inducements for PRES in children with hematologic diseases.2)The blood concentration of cyclosporin A has no definite relationship with the occurrence and severity of PRES.3)Electroencephalogram(EEG)is not only an important tool for differential diagnosis,but also may be important for early intervention and prognosis.4)Cranial MRI is the first choice and the main imaging evidence for diagnosing PRES.5)Aminophylline may be used to treat PRES associated with methotrexate.6)PRES is not completely reversible.Early recognition and timely treatment can significantly improve the prognosis.2.The conclusion of the retrospective study combined with the literature:.1)The etiology of PRES is complex.Hematological disease is a common cause of PRES in children.2)The pathogenesis of PRES is not clear.The mechanism of PRES caused by different Precipitating factors may be different.3)Vascular brain edema is considered to be the basic pathophysiological change of PRES.4)The mechanism of chemotherapeutic drugs and immunosuppressants to induce PRES is complex.5)The occurrence of PRES is the result of a comprehensive effect of many factors.