| Objective: Reversible posterior leukoencephalopathy syndrome (RPLS) is a new clinical neuro-radiological entity. It was firstly reported by Hinchey in 1996. Although numerous cases have been reported subsequently, its etiology, pathogenesis, clinical and neuro-radiological features are controversial so far. In addition, due to its etiological complexity, diversity of clinical manifestations, and the lack of standardized diagnostic criteria for clinical diagnosis, clinical physicians may have a lack of knowledge about this disease, which may cause clinical misdiagnosis and mistreatment of the disease, resulting in irreversible brain dysfunction or threatening life. Therefore, it is nessceray to further study its clinical and neurological features and establish its standardized diagnostic criteria for clinical diagnosis, which benefits to enhance the physicians'new knowledge of this disease.Methods: By retrospective analysis of the etiology, clinical symptoms, neuroimaging features, treatment, and prognosis in eight patients with RPLS, and reviewing the domestic and foreign literature, we sought to further investigate the etiology, clinical symptoms, neuroimaging features of this disease, establish its standardized diagnostic criteria for clinical diagnosis, and provide a new proposal for renaming this disorder.Results: A total of 8 patients with RPLS, including 5 females and 3 males, were studied. Their etiology included 2 systematic lupus erythematosis, 2 eclampsia, 1 hypertension, 1 fungal meningitis, 1 multiple myeloma, 1 kidney transplantation, 3 usage of immunosuppressive agents, 1 usage of chemotherapeutic drugs, 1 usage of antifungal agents. Their main clinical symptoms exhibited 6 headache, 6 visual disturbances, 7 seizures, 5 disturbance of consciousness (i.e., lethargy, drowsiness, and coma). Brain MRI revealed that the lesions involved in the cerebral cortex, subcortical structure, cerebellum and brain stem, particularly the posterior region of the brain; among them, 6 cases with bilateral symmetrical lesions, and 2 cases with unilateral lesions; 6 in occipital lobe, 6 in frontal lobe, 5 in parietal lobe, 3 in cerebellum hemisphere, 2 in basal ganglia, 1 in thalamus, 3 in periventricular white matter, 1 in brain stem. After controlling blood pressure, decreasing the edema, and symptomatic treatments for 1 to 3 weeks, all patients'symptoms completely disappeared, and all or most of the lesions on the MRI disappeared. Based on our findings and the results of the related literature, we provided a new proposal for renaming this disorder as"reversible encephalopathy syndrome (RES)", and for standardized diagnostic criteria for clinical diagnosis of this disease:①Induced factors: most cases have physical illnesses or other risk factors, such as hypertension, eclampsia, immune diseases, tumor, kidney insufficiency, usage of chemotherapeutic drugs or immunosuppressive agents.②Onset of the disease: acute or subacute;③Clinical manifestations and neurologic signs: headache, seizures, and visual disturbances are common symptoms of these patients, with or without positive neurologic signs. There is a"separation"phenomenon, known as severe lesions on neuroimages as opposed to less clinical symptoms and positive neurologic signs.④Neurologic images (Brain MRI): The lesions may involve in the cerebral cortex, subcortical structure, cerebellum or brain stem, particularly the posterior region of the brain, presenting with bilateral (or unilateral), symmetric (or asymmetric) vasogenic edema.⑤Prognosis: After treatments for 1 to 3 weeks, the patient's symptoms and positive signs completely disappeared, and all or most of the lesions on the MRI disappeared.⑥Ruling out other diseases.Conclusions: Headache, seizures and visual disturbances are common syptoms of these patiens, with or without positive neurologic signs; the lesions on neuroimaging may involve in the cerebral cortex, subcortical structure, cerebellum or brain stem, particularly the posterior region of the brain, presenting with bilateral or unilateral, symmetric or asymmetric changes. We firstly put forward the standardized diagnostic criteria for clinical diagnosis of this disease, and provide a new proposal for renaming this disorder as"reversible encephalopathy syndrome (RES)". |
