Analysis Of12Patients With Reversible Posterior Leukoencephalopathy Syndrome

Objective:To explore and analysis the clinical features of reversible posterior leukoencephalopathy syndrome(RPLS),compare the clinical manifestation and imaging features with different etiologies, and through literature review, describe the etiology, pathological and physiological mechanism, clinical manifestation, laboratory examination and imaging features, diagnosis and differential diagnosis, therapy and prognosis, thus improving the clinical understanding of RPLS.Method:A series of12patients recruited from The Second Affiliated Hospital of Zhejiang University, School of Medicine were reviewed retrospectively, which were admitted from January1st2009to January1st2015. The patients were divided into2groups(hypertension group and eclampsia group) according to different etiologies. And describe the etiology, pathological and physiological mechanism, clinical manifestation, imaging features, therapy and prognosis of RPLS.Results:(1) There were12patients in our study,2males and10females, the ratio of gender was1:5. The ages ranged from16to40years old, average age is28.25years old. (2)Etiology:2presented with hypertension,10presented with eclampsia(2antepartum eclampsia,8postpartum eclampsia)(3)Clinical manifestation:all of them had acute onset.9presented with seizure,7presented with headache,4presented with vision problem,2presented with consciousness dysfunction,1presented with weakness of both inferior limbs,1presented with dizzy. The main clinical manifestations of eclampsia group were seizure and headache, while hypertension group were high blood pressure and vision problem.(4)Laboratory examination:12had tested the serum level of LDH,11of which showed the high level. Lumbar puncture showed6of8patients had increase of pressure, with the normal of cerebrospinal fluid examination.(5) Imaging features:7involved occipital lobe,7involved occipital lobe,6involved parietal lobe,5involved temporal lobe,6involved frontal lobe,1involved basal ganglia,3involved cerebellum,3involved the brain stem,5involved lateral ventricle and semi oval center and1involved thalamus.The main location of eclampsia group were occipital lobe, parietal lobe, temporal lobe, frontal lobe, while hypertension group were basal ganglia, cerebellum, the brain stem and lateral ventricle and semi oval center. The MRI features were low signal intensity on T1WI, high signal intensity on T2WI and FLAIR images, low or isointense signal intensity on DWI and high signal intensity on ADC map.(6)Treatment:7were given dehydration and lowering the intracranial pressure,6were lowering the blood pressure,4were treated of seizures,3were given anticoagulation.(7) All the12patients recovered within1-2weeks.8received repeated MRI images,4of which showed the reduce of the number and size of lesions and the other4showed the lesions had disappeared.Conclusions:1. The etiology of RPLS is complex. Hypertension, eclampsia and pre-eclampsia are the most common causes of RPLS. Besides the use of immunosuppressant and cytotoxic drugs,post-transplantation, autoimmune diseases can also induce RPLS. 2. With acute or subacute onset, RPLS present as four characteristics:seizure, headache, visual disturbance and consciousness dysfunction.The main clinical manifestations of RPLS with eclampsia were seizure and headache,while with hypertension were high blood pressure and vision problem.3. Laboratory examination finds the lever of LDH in serum is high. Lumbar puncture shows the increase of pressure, with the normal of cerebrospinal fluid examination.4. The imaging features of RPLS are associated with vasogenic edema of posterior leukoencephalophy, which are symmetrical and reversible. The location of RPLS with hypertension were more easier involved atypical positions like basal ganglia, cerebellum. The MRI shows low signal intensity on T1WI, high signal intensity on T2WI,low or isointense signal intensity on DWI and high signal intensity on ADC map.5. Early diagnosis and treatment are important to the prognosis. Control blood pressure, terminate seizure, lower the intracranial pressure, terminate pregnant and stop the associated immunosuppressant and cytotoxic drugs are the key point of treatment. The disease is usually reversible and the clinical symptoms and the lesions on images can disappear. However, delayed diagnosis and treatment can produce irreversible damage.