| Reversible posterior leukoencephalophy syndrome (RPLS) is a newly recognized clinical-radiologic syndrome, characterized by signs of posterior cerebral edema upon radiographic examination. RPLS is often associated with an abrupt and significant elevation in blood pressure, as well as the clinical features, including headache, nausea and vomiting, seizures, altered mental stations, visual disturbances, and occasionally focal neurologic deficit. The causes of RPLS are diverse, but the common ones include hypertensive encephalopathy, pre-eclampsia/eclampsia, immunosuppressive and cytotoxic drugs. The classically neuroimaging findings of RPLS are associated with subcortical vasogenic edema, patchy symmetrical bilateral involvement with preferential involvement of the posterior head regions. CT scans and MR imaging are the favorite techniques used for detecting the lesions, especially the fluid-attenuated inversion recovery (FLAIR) imaging. Diffuse-weighted imaging (DWI) and apparent diffusion coefficient (ADC) map images are helpful in distinguishing vasogenic edema, the predominant abnormality in RPLS, from cytotoxic edema. The patients usually have a well prognosis, early recognition of this condition is of paramount importance because prompt and effective therapy will cause reversal of the syndrome, and the clinical sign and symptoms will fully disappear. Delay in the diagnosis and treatment can result in permanent damage, even death.Objective and Methods:In order to observation of the etiologies, pathophysiology, clinical manife- stations, imaging features, diagnosis and differential diagnosis, therapy and prognosis, raise the doctor's attention to RPLS and build the basis for the clinic. A series of 15 patients, whose clinical manifestations and neuroimagings fulfill the diagnosis criteria of RPLS, consecutively admitted to our hospital between March 2000 and March 2008 were reviewed retrospectively.Results:(1) There are there children and twelve adults in our study, whose ages ranged from 12 to 67 years (the average age was 31.9 years), and the ratio of gender was 1:2.75 (four males vs. 11 females). (2) Etiology : In our study, there were 6 patients presented with eclampsia, including postpartum eclampsia (n=3), eclampsia (n=2), and a thirty-two week's pregnant female with fetal death (n=1); 6 patients presented with hypertensive encephalopathy, including essential hypertension (n=3) and secondary hypertension (n=3), which secondary to polyarteritis and acute glomerulonephritis; one patient with non-small cell lung cancer commenced on systemic chemotherapy with cyclophosphamide, carboplatin and pharmorubicin; one patient with essential hypertension accepted steroid and cyclophosphamide; one postpartum female followed by hemorrhagic shock, HELLP syndrome and acute renal failure. (3) Clinical manifestations: In this group, 13 patients had acute onset; all of the patients had an elevation of blood pressure, the mean systolic blood pressure was 190 mmHg (ranged from 150mmHg to 240mmHg), the average mean arterial pressure was 143.1 mmHg (ranged from 116.7 mmHg to 186.7mmHg); clinical seizures occurred in all of the patients, of which 4 patients presented with status epilepticus; headache occured in 13; 12 patients presented with various levels of consciousness dysfunction ranged from lethargy to deep coma, or mental behavior abnormality; visual disturbance happened in 6, including blurred visualization and cortical blindness; there were still other clinical presentations, including dizziness, nausea and vomiting, declined memory and response, pupil abnormal and ophthalmoplegia, hemiplegia or pamplegia and positive Babinski sign; one patient was dead because of cerebral hernia. (4) Neuroimaging: CT scan and MRI are the favorite techniques used for detecting the lesions, especially a better visualization of subtle lesions obtain in MRI. 11 patients had CT scan in early onset of symptoms, and the hypodensity lesions were found in 8; MRI were performed in all 15 patients and the characterized neuroimagings were found, with low signal intensity or isointense on T1-weighted images and high signal intensity or little high on T2-weighted images and FLAIR images. Most RPLS lesions locat predominantly in the white matter of parieto-occipital lobes, and are not enhanced on T1-weighted images. Sometimes they are regarded as atypical, such as involvement of frontal lobe, basal ganglia, thalami, brainstem, cerebella, splenium of corpus callosum, cortical lesions, and unilaterality, occasionally foci of hemorrhage and infarction into lesions. In our group, the neuroimaging features included white matter of posterior circulation in 15 patients, gray matter/cortical involvement in 8 patients, parietal lobe lesions in 15 patients, occipital lobe lesions in 14 patients, frontal lobe lesions in 4 patients, and temporal lobe lesions in 5 patients. Basal ganglia involvement in RPLS occurred in 3 patients, brainstem and cerebella neuroimaging involvement were present in 2 and 4 patients separately. Splenium of corpus callosum lesion occurred in 1 patient. Unilateral lesions were seen in 1 patient. Hemorrhage lesions were presented in 4 patients. No gadolinium enhancement was seen in 2 patients who received contrast. The brain vessels were often seen in normal appearance in MRA and MRV. There was no abnormality in 2 patients who received MRA. MRV were taken into 7 patients, 6 of which were normal and 1 were suspect to experience venous thrombosis formation. Complete reversibility is generally regarded as a defining feature of RPLS. In our group, except for the dead woman, all of other 14 patients received repeated brain imaging. The lesions were obviously disappeared in 3 patients, and completely reversible in 11 patients. (5) Tightly relied on the clinical manifestations and neuroimagings was helpful for diagnosing RPLS. The differential diagnosis of RPLS included various acute neurological conditions such as TOBS, encephalitis, cerebral venous thrombosis, SAH, and demyelinating disorders. (6) 15 patients had given dehydration, lowering blood pressure, treatment of seizures, termination of pregnancy, removal or significant reduction of the causative immunosuppressive/cytotoxic drugs, allopathic and supporting treatments, clinical manifestations disappeared in 9 patients, clinical manif- estations almost disappeared in 5 patients, and 1 died. The repeated neuroimag- ings were obviously improved or disappeared in 14 patients.Conclusions:1. RPLS is a newly recognized clinical-radiologic syndrome, which can occur in both children and adult, and with a higher morbidity in female.2. The common causes of RPLS include hypertensive encephalopathy, pre- eclampsia/eclampsia.3. With acute onset, RPLS mainly present as seizures, headache, altered mental stations, visual disturbance, and an abrupt and significant elevation in blood pressure.4. The characterized neuroimaging features of RPLS are associated with subcortical vasogenic edema, patchy symmetrical bilateral involvement with preferential involvement of the posterior head regions. The lesions show hypodensity on CT scan, usually isointense to hypointense on T1WI, hyperintense on T2WI, and hyperintense on FLAIR images.5. The common differential diagnosis of RPLS includes TOBS, encephalitis, cerebral venous thrombosis, SAH, and demyelinating disorders.6. The patients usually have a good prognosis, Control of blood pressure and termination of seizures are the key point of treatment. Early recognition of this condition is of paramount importance because prompt and effective therapy will cause reversal of the syndrome, and the clinical sign and symptoms will fully disappear.
|
PDF Full Text Request