| Background and ObjectiveAnti N-methyl-D-aspartate receptor encephalitis(for short: anti-NMDA receptor encephalitis)is caused by antibodies against neuronal cell surface antigen or synapse proteins of a new type of autoimmune encephalitis,because its can affect limbic system in the temporal lobe,hippocampus,insular lobe and can be with or are not associated with tumor,so always said limbic encephalitis.Diversity of clinical manifestations of the disease,mainly for the memory,spirit,behavior,seizures,and a series of clinical manifestation,easily misdiagnosed as intracranial infection,mental illness and other diseases.The pathogenesis is unknown at present,most scholars think is closely related to virus infection,cancer,etc.Most of the disease early diagnosis,prognosis is good.If not receive timely diagnosis and treatment,but the legacy sequela,even resulting in death.Since 2007,foreign scholars put forward the concept of anti-NMDA receptor encephalitis,the number of clinical case reports increased year by year.Our country the first confirmed case is reported of Chunling Xu from Beijing friendship hospital in 2010,but limited to the test conditions and clinicians understanding of this disease is not comprehensive,domestic report the number of cases is still less,there are still the majority of patients failed to get early diagnosis and active treatment.This study was to analysis the anti-NMDA receptor encephalitis of onset age,clinical manifestation,characteristics of cerebrospinal fluid,brain magnetic resonance image(MRI),treatment and prognosis of such information,to provide early diagnosis and treatment of clinical reference.Materials and Methods1.The clinical data of 50 cases with anti-NMDA receptor encephalitis between October 2014 and December 2016 from the first affiliated hospital of Zhengzhou university were collected.Inclusion criteria: using Graus in February 2016[1] and the diagnosis standard of NMDAR encephalitis,must meet the following three conditions:(1)rapid onset,course of < 3 months),the clinical manifestation has at least one of the following 6 kinds of symptoms:(1)mental symptoms or cognitive dysfunction;(2)language dysfunction(mandatory language,words or less silence);(3)epilepsy;(4)movement disorders,different movement disorder,stiffness or position;(5)consciousness levels drop;(6)Autonomic nerve dysfunction or central ventilation.(2)the NMDAR-resistant IgG antibody positive(antibody testing should include cerebrospinal fluid,such as serum samples,only need to do after the serum antibody positive verification test shall be confirmed as positive).(3)to rule out other possible causes(viral encephalitis,herpes simplex viral encephalitis,hashimoto’s disease,mental illness).(4)in this study have been in our hospital ethics committee approval,all patients were signed informed consent.2.into the group of 50 cases of anti-NMDA receptor encephalitis patients to general information,history,clinical manifestation,auxiliary examination were retrospectively analyzed.Patients admitted to hospital after routine examination and serum,cerebrospinal fluid anti-NMDA receptor antibody detection(indirect immunofluorescence),electrocardiogram(ECG)and routine electroencephalography or ambulatory electroencephalography,magnetic resonance imaging(when it is necessary to enhance inspection),chest CT,abdominal CT and abdominal basin of colour to exceed examination.Results1.This group of patients,24 cases were male and 26 female cases,female: male = 1.1:1.Onset age: minimum 3 years old,biggest 74 years old,the median age of 26 years old;Morbidity peak age 20 to 25 years old.2.This group of patients,nonspecific prodromal symptoms in 18 cases;20 patients with mental behavior as the initial symptoms 40%(20/50),12 patients with epilepsy onset 24%(12/50),6 patients with cognitive dysfunction have disease of 12%(6/50),2 cases with disturbance of consciousness,speech disorder,sleep disorder,involuntary movement have disease of 4%(2/50),4 cases with other neurological symptoms(onset limb dysfunction,decreased vision,or fuzzy)onset of 8%(4/50).3.This group of patients,accounted for 86%(43/50)of abnormal psychiatric behavior;Seizures of 86%(38/50);Appears involuntary movement accounted for 52%(26/50);A disturbance of consciousness accounted for 32%(16/50);A speech disorders accounted for 22%(11/50);Sleep disorders was 16%(8/50);Cognitive dysfunction was 14%(7/50);Other neurological symptoms(onset limb dysfunction,decreased vision,or fuzzy)accounted for 8%(4/50).4.This group of patients,41 patients blood tumor series examination,12 cases were abnormal(29.3%);35 cases patients blood rheumatoid immune series of inspection,abnormal in 3 patients(8.6%);46 cases of thyroid function,abnormal 7 cases(15.2%);46 patients serum virology smears,36 cases(78.3%).5.This group of patients,all line CSFNMDA receptor antibody detection;Line 41 patients serum NMDA receptor antibody detection.The CSFNMDA receptor antibody are positive in 50 cases(100%),29 cases(71%)in the same period of NMDA receptor antibody positive serum.6.This group of patients,no specificity,cerebrospinal fluid pressure is normal,most brain white blood cell count can rise,no more than 200 x106 / L,the lymphocyte change,proteins can be increased,and sugar and chloride were normal.Cerebrospinal fluid immunoglobulin IgG,Ig generated index,24-hour CSF IgG synthesis rate range can be increased.CSF oligoclonal can be positive.7.This group of patients,tumor may merge to ovarian teratomas.8.This group of patients,36 patients EEG examination,24 cases(66.7%),normal EEG 12 patients characterized by abnormal(33.3%).More widely slow-wave or diffuse abnormal,visible diffuse epileptic EEG.9.This group of patients,50 cases were MRI scan,abnormality of 17 cases(34%).Lesions involving the 8 cases of dorsal thalamus,involving frontal lobe 7 cases,involving the hippocampus,6 cases by the lateral ventricle,involving the parietal lobe in 5 cases,involving the insula and basal ganglia region 4 cases,involving the occipital lobe,3 cases of corpus callosum,involving the brain stem,cerebellar hemisphere in 1 case.36 regular brain MRI enhancement scanning,9 cases,abnormal visible varying degrees of dura mater,limitation of pia mater line sample or samples to strengthen or bilateral cerebral hemisphere pia mater.Conclusion1.The anti-NMDA receptor encephalitis in young people,a peak age is 20 to 25 years old,women slightly more than men.2.Clinical and major clinical manifestations of anti-NMDA receptor encephalitis starting resistance is almost more memory decline,mental behavior,epileptic seizures.3.The serum virology,rheumatoid immune index,tumor markers,is valuable for the differential diagnosis of disease.4.Anti-NMDA receptor encephalitis patients CSF no specificity,the early formed a lymphocyte change;The main diagnosis basis is CSF NMDA receptor antibody positive.5.EEG examination on disease severity assessment and differential diagnosis has important significance.6.Anti-NMDA receptor encephalitis MRI can show the unilateral or bilateral temporal lobe T2 Flair high signal,a few performance of multiple sclerosis-like demyelinating change and pseudotumor demyelinating change.7.Anti-NMDA receptor encephalitis patients should actively screening for cancer and immune therapy.After treatment with immune most prognosis is good,the recurrence rate is low. |
