The role of fluid biomarkers in the diagnosis of Alzheimer′s disease / 中华神经科杂志

The rapid advancement of biomarker research in Alzheimer′s disease (AD) has improved the accuracy of AD diagnosis, facilitated its early diagnosis and intervention, and has significant implications for disease-modifying therapy research and evaluation. Since its introduction, the AT(N) system has grown in importance in both the clinical and research aspects of AD. However, the fluid biomarkers in the AT(N) system do not yet adequately reflect the complex pathophysiology of AD. Other fluid biomarkers associated with neuroinflammation, synaptic dysfunction, and vascular dysfunction should be a valuable addition to the AT(N). In recent years, the development and application of peripheral blood biomarkers have greatly enriched the fluid biomarker system for AD and are expected to greatly facilitate early screening and diagnosis of the disease. Here, a brief overview of current research on AT(N) and other pathogenesis-related fluid biomarkers is presented, and the article proposes that fluid biomarkers for AD should be studied from a variety of pathophysiological perspectives in order to improve their role in disease diagnosis. This article also discusses the role and limitations of fluid biomarkers in the diagnosis of AD, as well as the future development and application of fluid biomarkers in AD.

The value of Facial Emotion Recognition Test in the early diagnosis of behavioral variant frontotemporal dementia / 中华神经科杂志

Objective:To evaluate the value of Facial Emotion Recognition Test (FERT) in the early diagnosis of behavioral variant frontotemporal dementia (bvFTD).Methods:A total of 27 patients with mild bvFTD, 27 patients with mild Alzheimer′s disease (AD) and 54 normal controls were successively collected in the Memory Clinic of Department of Neurology,Xuanwu Hospital, Capital Medical University from January 2018 to July 2019. The subjects were assessed by the FERT and a battery of neuropsychological background tests including Mini-Mental State Examination, Frontal Assessment Battery (FAB), and Auditory Verbal Learning Test (AVLT), etc. The discriminatory power of the FERT was evaluated using the receiver operating characteristic curve (ROC).Results:The average FERT scores of bvFTD, AD and control groups were 18.00 (14.00, 21.00), 25.00 (21.00, 28.00) and 28.00 (26.00, 30.00) respectively. The FERT scores of bvFTD group were significantly lower than those of control groups ( H=-55.278, P<0.001) and AD groups ( H=-28.407, P=0.002). ROC results showed that the FERT had a high discriminatory power for differentiating bvFTD from the controls, with an area under the curve (AUC) value of 0.969 (95% CI 0.931-1.000, P<0.001). Both the sensitivity and specificity were 92.6% with a cut-off value at 24. For differentiating bvFTD group from AD group, the AUC value for the FERT was 0.850 (95% CI 0.749-0.951, P<0.001), with sensitivity of 81.5% and specificity of 71.4% with a cut-off value of 22. Compared with the AUC values for the FAB and AVLT-Delayed Recall (0.776 and 0.714), the AUC value for the FERT was slightly higher, though the differences among them were not statistically significant. Conclusions:Disturbance of emotion processing is presented in the early stage of bvFTD. FERT is one of the sensitive and specific neuropsychological indicators for the early diagnosis of bvFTD.

Research progress on the effect of single nucleotide polymorphism on drug efficacy of Alzheimer′s disease / 中华神经科杂志

Alzheimer′s disease is a progressive neurodegenerative disease that requires medication to improve patient symptoms, but there is an individual difference in the efficacy. In this paper, the correlation between single nucleotide polymorphism and the drug efficacy of Alzheimer′s disease (AD) in the past 20 years was searched through the databases of China National Knowledge Infrastructure, VIP Database, Wanfang Database, Pubmed, Springer Link and Cochrane Library with key words of Alzheimer′s disease, drug efficacy, single nucleotide polymorphism. The correlation between AD drug efficacy difference and gene single nucleotide polymorphism was reviewed, including ABCA1, ApoE, ChAT, CHRNA7, IL-6, A2M, CYP2D6, BChE, 5HT2a, PON-1 and ESR1 genes, so as to provide a reference basis for clinicians to select drugs in the treatment of AD.

The present and future of China's dementia / 中华神经科杂志

At present,the prevalence and incidence of dementia in China are high,bringing heavy economic burden to society and families.At the same time,the level of diagnosis and treatment of dementia varies greatly from hospital to hospital.Although there has been great progress in recent years,it still can not completely meet the clinical needs.In terms of treatment,it is symptomatic treatment at present,and multi-target intervention is needed in the future.In view of the problems and shortcomings in the diagnosis and treatment of dementia in China,the authors put forward their own views and suggestions on the future research strategies.

The present and future of China's dementia / 中华神经科杂志

At present, the prevalence and incidence of dementia in China are high, bringing heavy economic burden to society and families. At the same time, the level of diagnosis and treatment of dementia varies greatly from hospital to hospital. Although there has been great progress in recent years, it still can not completely meet the clinical needs. In terms of treatment, it is symptomatic treatment at present, and multi-target intervention is needed in the future. In view of the problems and shortcomings in the diagnosis and treatment of dementia in China, the authors put forward their own views and suggestions on the future research strategies.

Features of connected speech in patients with mild Alzheimer's disease / 中华神经科杂志

Objective To explore the features of connected speech produced by Chinese mild Alzheimer's disease (AD) patients.Methods Thirty Chinese mild AD patients (eight males and 22 females,and the age was (72.73 ± 7.78) years) and 30 age and education matched normal controls from three communities were recruited in Xuanwu Hospital from January 2018 to May 2018.All subjects completed tasks of picture naming,semantic fluency (animal),and Cookie Theft picture description.Picture description was analyzed for speech production (total word output,speech rate,sound errors),lexical content (number of nouns and verbs,proportion of pronouns and verbs,semantic errors),information content (information unit,idea density,efficiency),syntactic structure and complexity (mean length of utterance,words in sentences,syntactic errors).Differences between groups were calculated.Association between picture naming,semantic fluency,and measures in picture descriptions was analyzed.Results Compared with the controls,mild AD patients achieved lower scores on the picture naming task (74.45±8.04 vs 79.60± 1.73,t=-2.800,P=0.010) as well as on the semantic fluency task (10.35±3.08 vs 18.50±4.66,t=-6.520,P＜0.01) and produced lower speech rate (131.80±36.40 vs 149.64±26.69,t=-2.095,P=0.041),less number of nouns (7.52±2.55 vs 9.67±3.58,t=-2.509,P=-0.015) and verbs (4.40±2.24 vs 5.73±1.92,t=-2.375,P=0.021),increased proportion of pronouns (0.286 (0.319) vs 0.039 (0.122),Z=3.602,P＜0.01) and semantic errors (0.629 (1.396) vs 0 (0.779),Z=2.386,P=0.017),less information units (11.00±3.63 vs 13.70±3.53,t=-2.787,P=0.007),decreased idea density (0.11±0.04 vs 0.16±0.05,t=-3.946,P＜0.01),decreased efficiency (14.23±7.56 vs 22.95±7.14,t=-4.738,P＜0.01),shorter mean length of utterance (6.64± 1.27 vs 8.25± 1.70,t=-3.928,P＜0.01),and less words in sentence (0.84±0.10 vs 0.95±0.06,t=-4.625,P＜0.01).Group differences in total word output,sound errors,proportion of verbs,and syntactic errors did not reach statistical significance.Scores on picture naming task were negatively associated with the proportion of pronouns (r=-0.737,P=0.003),and positively associated with the idea density (r=0.540,P=0.046) and efficiency (r=0.592,P=0.026) in the AD group.Scores on semantic fluency task were negatively associated with the proportion of pronouns (r=-0.554,P=0.011).Conclusions Connected speech in mild AD patients was characterized by decreased naming accuracy,decreased sematic fluency,decreased content words,information units,conciseness,efficiency,and syntactic complexity,but motoric aspects of speech and sentence structure remained relatively preserved.Decreased content words and increased pronouns in mild AD may relate to semantic impairment.

Analysis of clinical and neuroimaging features in a Chinese family with hereditary diffuse leukoencephalopathy with neuroaxonal spheroids / 中华神经科杂志

Objective To explore the clinical and neuroimaging features in a Chinese family with hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) caused by mutation of the colony stimulating factor 1 receptor gene (CSF1R). Methods The proband and another patient from a HDLS pedigree were assessed respectively through standardized clinical evaluation (medical history inquiry, physical examination),neuropsychology assessment,MRI,genetic sequencing, as well as brain PET imaging with carbon11-labelled Pittsburgh compound-B(11C-PIB). Results A HDLS pedigree with three patients was recruited to this study. Apathy, memory decline, slow behavior were the first symptoms for two of the patients. Being bedridden, urinary incontinence and epilepsy were developed at the later stage. A missense mutation c. 2381T>C(p. I794T) in exon 18 of the CSF1R gene of chromosome 5 was identified in the proband. The brain DWI illustrated multiple patchy high signal in periventricular white matter and centrum semiovale which was characterized by persistence, and the corpus callosum was affected in the early stage. Conclusion The multiple patchy high signal with persistence in periventricular white matter and centrum semiovale of DWI is helpful for the early diagnosis of HDLS.

Clinical and neuroimaging features of frontotemporal dementia with parkinsonism linked to chromosome 17 / 中华神经科杂志

Objective To explore the clinical and neuroimaging features of a frontotemporal dementia with parkinsonism linked to chromosome 17 ( FTDP-17 ) pedigree caused by mutation of microtubule-associated protein tau ( MAPT) gene.Methods The proband and one patient from a FTDP-17 pedigree were assessed through standardized clinical evaluation , neuropsychology assessment , video-electroencephalogrom ,MRI, genetic sequencing , as well as 18 F fludeoxyglucose ( FDG) SPECT for brain metabolism and 11 C 2β-carbomethoxy-3β-( 4-fluoro ) tropane ( CFT ) PET for dopamine transporter ( DAT ) distribution, respectively.Results A FTDP pedigree with 15 patients (6 still alive) was recruited to this study.The proband and one affected patient were genotyped and confirmed as MAPT c .1788T>G mutation. Parkinsonism was the first symptom for both two patients . Personality, speech changes and dementia accompanied with brain atrophy were developed at the later stage in one patient .The 18 F FDG SPECT studies illustrated asymmetric hypometabolism of the temporal , frontal lobes and basal ganglia in two patients . Regarding to the 11 C CFT PET, one affected patient showed asymmetric decreased uptake of tracer in basal ganglia regions.Conclusions FTDP-17 can display a confusingly broad clinical phenotype , with the parkinsonism as the first symptom . Brain glucose metabolism and DAT distribution could be potential biomarkers in early diagnosis of FTDP-17.

Analysis of relation between the development of study and literatures about benign positional paroxysmal vertigo published international and domestic / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#Benign paroxysmal positional vertigo (BPPV) is a common vestibular disorder that causes vertigo. Study of BPPV has dramatically rapid progress in recent years. We analyze the BPPV growth@*METHOD@#We searched the international data quantity year by year in database of PubMed, ScienceDirect and WILEY before 2014 respectively, then we searched the domestic data quantity year by year in database of CNKI, VIP and Wanfang Data before 2015 by selecting "Benign paroxysmal positional vertigo" as the keywords. Then we carried out regression analysis with the gathered results in above databases to determine data growth regularity and main factors that affect future development of BPPV. Also, we analyzes published BPPV papers in domestic and international journals.@*RESULT@#PubMed database contains 808 literatures, ScienceDirect contains 177 database and WILEY contains 46 literatures, All together we collected 1 038 international articles. CNKI contains 440 literatures, VIP contains 580 literatures and WanFang data contains 449 literatures. All together we collected 1 469 domestic literatures. It shows the rising trend of the literature accumulation amount of BPPV. The scattered point diagram of BPPV shows an exponential growing trend, which was growing slowly in the early time but rapidly in recent years.@*CONCLUSION@#It shows that the development of BPPV has three stages from international arical: exploration period (before 1985), breakthrough period (1986-1998). The deepening stage (after 1998), Chinese literature also has three stages from domestic BPPV precess. Blank period (before the year of 1982), the enlightenment period (1982-2004), the deepening stage (after the year of 2004). In the pregress of BPPV, many outsantding sccholars played an important role in domestic scitifction of researching, which has produced a certain influence in the worldwide.

A new classification of extensions of the sphenoid sinus of Chinese adult by CT / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To examine various pneumatized extensions of the sphenoid sinus of Chinese people.@*METHOD@#The sphenoid sinus and its surrounding structures were examined from 100 computed tomography images of the sinus. The type of the sphenoid sinus was classified according to the various extensions of the sinus.@*RESULT@#The type of the sphenoid sinus was classified into the following 6 basic types based on the direction of pneumatization: sphenoid body, lateral, clival, lesser wing, anterior, and combined.@*CONCLUSION@#The variations in the extensions of pneumatization of the sphenoid sinus may facilitate entry into areas bordering the sphenoid sinus.

Clinical analysis of 14 cases with nasal respiratory enithelial adenomatoid hamartom / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To describe and analyze the clinical features of nasal respiratory epithelial adenomatoid hamartom and improve the levels of diagnosis and treatment.@*METHOD@#Fourteen cases of nasal respiratory epithelial adenomatoid hamartom confirmed by pathology were collected and analyzed.@*RESULT@#In 14 cases, primary complaint of Hyposmia(or Anosmia)and nasal obstruction were occured in 13 and 11 cases respectively. Four cases had past history of endoscopic sinus surgery because of the diagnosis of nasal polyps. Polypoid neoplasms could be seen in the bilateral olfactory clefts. Sinus CT showed soft tissue shadows in bilateral nasal cavity and mucous membrane thickening in different sinus. Endoscopic sinus surgery were utilized to eliminate focus in all cases. All cases weren t recurred after 2-20 months' following-up visitings.@*CONCLUSION@#Nasal respiratory epithelial adenomatoid hamartom is so rare that clinical and pathological doctors have limited knowledge of it. It has its own characteristics from the clinical symptoms, signs to sinus CT although they are nonspecific. So we should improve the understanding about it to avoid misdiagnosis or missed diagnosis.

Association between Presenilin 1 gene methylation and sporadic Alzheimer's disease / 中华老年医学杂志

Objective To investigate the correlation between Presenilin 1 gene methylation (PSEN1) and sporadic Alzheimer's disease (SAD).Methods Massarray method was used to perform an analysis of DNA methylation in the promoters of PSEN1 gene in lymphocytes from 35 SAD patients,33 mild cognitive impaired (MCI)patients and 22 age-and gender-matched controls.Reporter gene plasmid of PSEN1 was built by genetic recombination methods,and then transiently transfected into the SH-SY5Y and HeLa cells.After that,cells were treated with several agents including serum deprivation,Aβ25-35,5-aza-2'-deoxycytidine (5-aza Cdr) and S-adenosyl-Lmethionine (SAM).A dual-luciferase reporter assay system was used to calculate the relative luciferase activity (RLA).Results Some CpG sites of PSEN1 (Cytosines at-173,95 and +76) showed hypomethylation patterns in SAD cases as compared with MCI and control group (P＜0.01).A dual-luciferase reporter assay showing significant differences in PSEN1 transcription activities were found in both cell lines under SAM treatment [SY5Y,SAM:(6.40±0.81),control:(9.13±1.67),P=0.000; Hela,SAM:(2.06±0.34),control:(2.99±0.59),P=0.000].Conclusions DNA hypomethylation of PSEN1 gene promoter is significantly associated with SAD susceptibility.PSEN1 may change the gene transcription level by methylation,further affecting the activity of amyloid protein-like γ-secretase,leading to the onset of AD.

Individual identification research of amnestic mild cognitive impairment based on support vector machine / 医学研究生学报

Objective In recent years , multivariate pattern analysis ( MVPA) method was proposed and considered to be a promising tool for automated identification of various neuropsychiatric populations .Support vector machine ( SVM) is one of the most widely used methods of MVPA .Using SVM classifier for MVPA of amnestic mild cognitive impairment (aMCI) and normal control (NC) group, the present study aims to build an individual diagnostic model with significant discriminative power and investigate the gray matter abnor-malities of aMCI patients . Methods Fifty-one aMCI patients and 68 normal controls were scanned on the 3-Tesla magnetic resonance imaging (MRI) for high-resolution T1-weighted images.Gray matter volume map was calculated for each subject and used as features for subsequent discriminative analysis .We first applied feature selection to remove redundant information and reduce feature dimension , and then trained an SVM classifier . Leave-one-out cross validation ( LOOCV) was used to estimate the performance of the classifier , and finally the most discriminative features were identified . Results The proposed classifier achieved a classification accuracy of 83.19%with a sensitivity of 76.47%and a specificity of 88.24%.In ad-dition, the area under the receiver operating characteristic (ROC) curve was 0.8368.Further analysis revealed that the most discrimi-native features for classification included bilateral parahippocampal gyri , bilateral hippocampi , bilateral amygdala , bilateral thalamus , right cingulate , right precuneus , left caudate , left superior temporal gyrus , left middle temporal gyrus , left insula and left orbitofrontal cortex. Conclusion The proposed classification model has achieved significant accuracy for aMCI prediction , and it also displayed the whole brain gray matter atrophy pattern in aMCI patients .It suggests that the proposed method may have important implications for early clinical diagnosis of aMCI patients .

The necessity of post-maneuver postural restriction in treating benign paroxysmal positional vertigo / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To evaluate the necessity of postural restrictions after repositioning maneuvers in posterior canal benign paroxysmal positional vertigo (BPPV).@*METHOD@#Sixty-eight consecutive patients diagnosed of posterior canal BPPV with a positive Dix-Hallpike test. Thirty-two patients were instructed to follow postural restrictions after repositioning maneuvers, and 36 patients did not receive any postural restriction after treatment. All the patients were reevaluated at 1 week and 3 months later respectively.@*RESULT@#There was no statistical difference in number of maneuvers needed to resolve symptoms between two groups.@*CONCLUSION@#Epley maneuver is effective to treat patients with posterior canal BPPV, and postural restrictions does not improved the efficacy. Above all, we do not recommend any postural restrictions to patients with posterior canal BPPV.

Review and reflect on the development of national programming textbook Neurology for five-year undergraduates / 中华医学教育探索杂志

This paper reviewed and analyzed the whole 29 years' compiling and revising histo-ry of Neurology the 5-year programming textbook for national undergraduates. The textbook has been becoming a high-quality textbook with novelty, practicability, readability and systematicness, conform-ing to the need of national education reform and reflecting the characteristics of neurology. Through the review of textbook history, we know that material construction can deepen the educational reform, improve the teaching level and discipline development, promote quality education and personnel train-ing.

Clinical, neuroimage, and neuropsychological profiles of semantic dementia / 中华神经科杂志

Objective To explore the clinical,neuroimage,and neuropsychological profiles of semantic dementia (SD).Methods Detailed medical history were collected on 18 SD patients.Brain MRI scans were administered.Neuropsychological evaluation taping semantic memory (things naming),overall cognitive function ( Mini-mental State Examination,MMSE),visuoconstructive skill ( Clock Drawing Test,CDT), daily functional ability (Activities of Daily Living, ADL), neuropsychiatric symptoms (Neuropsychiatric Inventory,NPI),and disease severity (Frontotemporal Lobar Degeneration Modified Clinical Dementia Rating Scale,FTLD-CDR) were performed on all patients.Results The mean age at onset was (60.6 ± 8.5 ) years,with 5 cases over 65. All patients developed progressive word-finding difficulty and anomic speech.Ten patients complained significant memory impairment and 14 experienced behavioral disturbance.Five patients were previously diagnosed as Alzheimer' s disease (AD) and 1 as schizophrenia.All patients developed marked semantic memory impairment both for living things and nonliving things.The mean score on MMSE was 10.94 ± 8.86,with 16 cases performed incorrectly on naming.Mean score on CDT copy was 4.61 ±0.85,with 14 cases scored normally.Mean score on ADL was 29.72 ± 8.75.Cases with a 5-year course showed a significant overall decline.Fourteen cases presented behavior symptoms and scored 8.00 ± 7.22 on NPI.All patients scored worst on language domain among all the domains evaluated in FTLD-CDR.Atrophy,typically involving the left anterior temporal was shown on MRI scans.However,predominantly right temporal atrophy was observed in one patient.Atrophy confined to the temporal lobe in patients with early stage and extended to the contralateral temporal,frontal lobe,and parietal lobe as disease progression. Conclusions Current study suggested that SD tend to develop in presenile age.However,about 1/3 cases develop the disease after 65 years. Deficit in language is the earliest and most prominent symptom. Behavior change is prevalent as well. Patients are commonly misdiagnosed as AD or lack a definite diagnosis.Visuoconstructive skill and other abilities are relatively preserved in the early stage.With progression into the 5th years,overall decline comes inevitably.Brain scans can reflect the disease characteristics and progression. Of note,there exists individual with right dominant atrophy.

Association between promoter polymorphisms of a disintegrin and metalloproteinase 10 gene and sporadic Alzheimer's disease / 中华神经科杂志

ObjectiveTo investigate the relationship between the polymorphisms of the promoter of a disintegrin and metalloproteinase 10(ADAM10) gene and sporadic Alzheimer's disease (SAD).Methods The promoter of ADAM10 gene in 10 controls and 10 SAD patients was sequenced.Three variations were found,then these variations in 298 SAD patients (SAD group) and 315 healthy controls (control group)were genotyped by using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP).ResultsThree polymorphisms were found in the promoter of ADAM10 gene: -279G/A (rs653765),- 630G/T( rs514049 ) and - 921GAGA/- ( rs33926666 ).For - 921GAGA/-,there were significant differences in genotype ( GAGA/GAGA:138 (46.3％ ),GAGA/-:155(52.0％),-/-:5(1.7％))and allele frequencies (GAGA:431 (73.6％),-:165 (27.7％) ) between SAD and control (genotype:x2 =34.130,P =0.000; allele:x2 =25.972,P =0.000). For - 279G/A,there were significant differences in genotype and allele frequencies between SAD and control in the subjects without ApoEε4 allele (genotype:x2 =8.734,P=0.013; allele:x2 =5.129,P=0.024). -279G and -921GAGA were relatively protective allele types for SAD,and they were not in linkage disequilibrium.ConclusionThe polymorphisms - 279G/A and - 921GAGA/- of ADAM10 are associated with SAD.Allele G or genotype G/G of -279G/A and the GAGA/GAGA genotype or the GAGA allele of -921GAGA/- might have a protective effect on SAD.

Risk factors and clinical features of mild vascular cognitive impairment due to subcortical small vessel disease / 中华神经科杂志

Objective To determine the risk factors and clinical features of mild vascular cognitive impairment due to subcortical small vessel disease (mVCI-SSVD).Methods Detailed demographic data,vascular risk factors, past and present history were collected and carefully neurological examination, National Institutes of Health Stroke Scale (NIHSS), as well as Hachinski ischemic score (HIS) were performed on 56 mVCI-SSVD patients.Further, the demographic data and vascular risk factors of mVCI-SSVD patients were compared with those of 80 normal control subjects.Results Proportions of smoking (39.3% (22/56)), hypertension (67.9% (38/56)), and diabetes (25.0% (14/56)) were higher in the patient group than in the normal control group (21.3% (17/80) , 47.5% (39/80), 11.3% (9/80)).Odds ratio (2.32(95% CI 1.05-5.13),2.15 (95% CI 1.02-4.54),2.26(95% CI 0.86-5.92)) between the two groups was statistically significant (P value: 0.039, 0.045, 0.047).There was no difference in terms of hyperlipidemia and cardiac disease between groups.Fifty percent (28/56) mVCI-SSVD patients had a clear stroke history.Twenty-six point eight percent (15/56) patients developed the cognitive impairment with an acute onset.Neurological focal signs presented in 20 patients (35.7%).Twenty four (42.9%) patients with HIS ≤ 4 points.Thirty eight cases (67.9%) scored 0 on NIHSS.Conclusions Current study suggested that smoking, hypertension, and diabetes may be risk factors for mVCI-SSVD.While hyperlipidemia and cardiac disease do not increase the risk of mVCI-SSVD.Unlike mVCI caused by large vessel disease, about half mVCI-SSVD patients lack of stroke history.Most patients show a relatively insidious onset and free of significant neurological focal signs.

Clinical study of a rare case of primary central nervous system natural killer/T cell meningeal lymphoma / 中华神经科杂志

Objective To explore the clinical features and diagnostic method of primary natural killer( NK)/T cell meningeal lymphoma. Methods An unusual case of a 19-year-old male with primary NK/T cell meningeal lymphoma was reported. His clinical presentation and laboratory findings were discussed. The related literatures have been reviewed. Results The patient presented with diplopia,headache, vomiting and facial drooping at the onset, followed by progressive pain and weakness of the four limbs. Cerebrospinal fluid showed significant increase in pressure, leukocytes number, levels of protein,normal glucose and adenosine deaminase, negative tuberculosis antibody and sterile staining. In cerebrospinal fluid cytological analysis, May-Grunwald-Gimsa staining showed large number of atypical lymphocytes with irregular nucleus and nuclear fission, Ki-67 immunostaining showed extensive proliferative activity of the lymphoid cells. Flow cytometric immunophenotypic analysis of cerebrospinal fluid indicated 97. 98 percent of cells expressed surface CD3, CD7, CD56, CD2, CD5, and partially expressed CD8. This was a rare immunophenotype for NK/T-cell. Cranial MRI with gadolinium showed thickening of the trigeminal nerve with slight enhancement and diffuse leptomeningeal enhancement. CT of the chest and abdomen and bone marrow biopsies were negative. He was diagnosed as primary NK/T cell meningeal lymphoma based on the clinical features and related examination. Conclusions Primary NK/T cell meningeal lymphoma is a rare type of primary central nervous lymphomas which has special immunophenotype. The clinical features include progressive raised intra-cranial pressure, multiple cranial and spinal nerve involvements. Cerebrospinal fluid cytological analysis and flow cytometric immunophenotypic analysis are key work-up for diagnosis. It has poor response to chemotherapy and radiotherapy.