Objective To investigate the clinical, pathological and imaging features of sporadic Creutzfeldt-Jakob disease (CJD). Methods The clinical, pathological, electroencephalogram (EEG) and imaging data of 12 cases of CJD were analyzed retrospectively.Results (1) 7 cases of the enrolled patients were male and 5 were female. The average onset age of them was 49 years old. 3 cases were acute onset by visual loss and 9 cases were subacute onset by hypophrenia, mental and behavior disorder or cerebellar ataxia. All the cases showed dementia, myoclonus and extrapyramidal sign. (2) 9 cases showed typical triphasic wave on EEG and 1 case presented atypical triphasic wave. (3) 5 cases showed abnormal hyperintense lesions in basal ganglia on T 2-weighted images (T 2WI). 8 cases in occipital and/or frontal cortex showed the abnormal hyperintense lesions on diffusion-weighted images (DWI), and 5 of them accompanied symmetric diffuse hyperintense lesions in basal ganglia. (4) 1 case with postmortem and 6 cases with biopsy of brain showed classic pathological features of CJD.Conclusion On the base of typical clinic manifestations, EEG and DWI examinations are useful in early diagnosis of sporadic CJD.

Objective To observe the therapeutic the effect and safety of high dose intravenous gamma globulin (GG) treating Guillain Barre syndrome (GBS) and multiple sclerosis (MS).Methods 17 patients with GBS and 12 patients with MS were treated with IVIG in a dose of 0.2 g/kg/d for 4 consecutive days (in a dose of 0.4g/kg for the first day), and 25 patients with GBS and 41 patients with MS were treated with steroid. Their therapeutic effectiveness was compared. Results In GBS and MS group, clinical grading scale and time of appearing effect of IVIG treated groups were superior to that of steroid treated groups ( P

Objective To explore the characteristics of neuroimaging and electroencephalogram (EEG) features of sporadic Creutzfeldt-Jakob disease (sCJD). Methods CT scan was performed at the 6th week, MRI was performed at the 7th, 9th, 17th and 64th week, regular EEG recording was performed at the 9th week, contineous 24-hour EEG recording was performed at the 11th, 14th, 16th, 37th and 64th week after onset in a patient with the pathological diagnosis of CJD. Regular pattern was analyzed according to the neuroimaging results and EEG. Results a) Diffusion-weighted imaging (DWI) was more sensitive in diagnosis of sCJD than cranial CT, T1-weighted MRI (T1WI), T2-weighted MRI (T2WI), fluid-attenuated inversion recovery (FLAIR) MRI or contrast-enhanced MRI. Abnormalities would not be found in the early stage of CJD in CT, T1-weighted MRI, T2-weighted MRI, FLAIR or contrast-enhanced MRI, but they could be found in the middle stage of CJD. The CT scan and routine MRI might not show any specific feature for the sCJD. b) DWI abnormalities appeared in bilateral cortex and basal ganglia, and changed along with the progression of disease. In late stage of the disease, abnormal aignals in the cortex would disappear, but it was still present in basal ganglia. c) The appearance of periodic discharge of sharp wave complexes (PSD), which could be mathematically described by a sine curve, might be absent in the early and late stage of the disease. Along with the progression of CJD, PSD would be present in a part of a lobe at first and then involve more lobes, and finally the whole brain would be involved. Conclusion Repeated monitoring of DWI and EEG is significant for early diagnosis of CJD.

300mmH2O), non-purulent CSF with pleocytosis up to 2700?106/L, increased polykaryocytes in the early stage, normal or obviously high total protein level, normal glucose and chloride level, and negative laboratory examinations (such as culture-and smear-examination of Mycobacterium tuberculosis, adenosine deaminase, anti-TB antibody, immuno-dot-blot assay MycoDot and PPD assay). In this series of 167 patients, 7 of them died (3.7%). Conclusions The findings in CSF examination in most patients with tuberculous meningitis may show atypical findings, therefore it should be emphasized to repeat the analysis of CSF in order to obtain an accurate diagnosis of tuberculous meningitis.

Objective To study the CT and MRI imaging features of central nervous system Rosai-Dorfman disease and to enhance knowledge and differential diagnostic ability for central nervous system Rosai-Dorfman disease.Methods The CT and MRI imaging appearances in 4 cases of pathologically proven Rosai-Dorfman disease were retrospectively evaluated and the literature of central nervous system Rosai-Dorfman disease were reviewed.Results Two cases had cranial CT scans,4 cases had cranial MRI scans,On CT scans,cerebral edema was demonstrated in one case and the other case was normaL MRI scans showed the lesions were solitary in saddle area in 3 cases,and multiple in anterior cranial fossa in 1 case.The lesions exhibited iso- to hypointensity on both T1WI and T2WI images.Following intravenous injection of contrast medium,ring-like enhancement was seen in 2 cases and homogeneous enhancement in 1 case.Nodular enhancement was seen in the case of multiple lesions in the anterior cranial fosse.All lesions were dural-based.Conclusions In patients with fever,headache,elevation of the erythrocyte sedimentation rate (ESR) and a polyclonal increase in .γ-globulins,the possibility of central nervous system Rosai-Dorfman disease should be considered when single or multiple dural-based mass lesions,especially in sellar region,were identified by CT and MRI.

Autoimmune encephalitis is a kind of inflammatory disease of central nervous system caused by abnormal immune response of body immune system to neuronal antigen,and is generally considered to be reversible encephalitis caused by noninfectious factors.Its characteristic manifestations include acute and subacute onset of cognitive dysfunction,epilepsy and mental disorder.With the discovery of related antibodies,summaries of clinical syndrome and application of new functional imaging instruments,the diagnosis of autoimmune encephalitis is increasingly standardized.The priority treatment of autoimmune encephalitis is immunomodulatory therapy,including glucocorticoid,immunoglobulin,plasma exchange and immunosuppressant.The other treatments could be the related tumor resection,electroshock therapy,etc.The symptoms in most patients can get substantial relief with active treatment.The present paper would focus on the research progress in treatment of autoimmune encephalitis.

Objective To analyze the sensitivity of auxiliary examinations in different periods of sporadic Creutzfeldt-Jakob disease (sCJD).Methods The clinical data of 53 sCJD patients were retrospectively analyzed including the different stages of skull diffusion-weighted magnetic resonance imaging (DWI),24-hour ambulatory electroencephalogram (EEG),18F-FDG PET/CT (PET-CT)and cerebrospinal fluid 14-3-3 protein.When calculating the sensitivity of an auxiliary examination,the diagnostic criteria were defined by combining the specific clinical manifestations with two or more positive results of other auxiliary examinations.Results There were 24,53 and 22 sCJD patients,respectively,met the criterion of early (E),middle (M) and later (L) stage of disease (some patients fit 2 or 3 stages).The sensitivity ofDWl (E:58.3％ M:85.4％,L:94.7％),EEG (E:45.8％,M:62.7％,L:77.8％),14-3-3 protein in cerebrospinal fluid (E:11.1％,M:52.9％) and PET-CT (E:80％,M:100％) increased gradually with disease progression,The sensitivity of PET-CT was higher than the other auxiliary examinations for E and M stages;no PET-CT was conducted in L stage.High signal regions mainly distributed in the cortex in E and M stages,but in L stage,no significant difference was found on the distribution of high signal regions between cortex and basal ganglia.Conclusions The sensitivities of the auxiliary examinations were different for sCJD patients in different stages.Reexaminations in different periods may improve the sensitivity for sCJD diagnosis.The sensitivity of PET-CT was high,and the combination of PET-CT and other auxiliary examinations may play a key role in the diagnosis of sCJD.

Objective To assess the diagnosis value of diffusion- weighted imaging (DWI) in Creutzfeldt-Jakob disease (CJD).Methods 8 cases of sporadic CJD who underwent MRI were reported. 4 cases were definite,3 cases were probable and 1 case was possible. The sensitivity of DWI and conventional MRI were compared. Results T_1WI and T_2WI revealed no abnormal signals except nonspecific diffuse brain atrophy in 4 cases, whereas DWI detected hyperintense abnormalities in all cases. 2 cases showed linear lesions only in the cerebral cortex, and 6 cases showed lesions in both the cerebral cortex and the striatum. The lesions were symmetric in 5 cases, but were asymmetric in the other 3 cases. Although fluid-attenuated inversion recovery (FLAIR) imaging also showed cortical hyperintensity in 1 case, the high signal changes were more evident and extensive on DWI. Conclusions The hyperintense changes in the cerebral cortices and/or striata on DWI are considered characteristic of CJD. DWI is more sensitive than conventional MRI in depicting lesions of CJD and may be an essential tool for the early diagnosis of this disease.

Sporadic Creutzfeldt-Jakob disease is a rare condition with a rapid disease course and a mortality rate of 100%. In clinical practice, it is difficult to diagnose, even if consistent conventional laboratory methodologies are used. This article will give a summary on the epidemiology, pathogenesis, clinical manifestations, auxiliary examination, diagnosis and differential diagnosis, management, and prognosis of sporadic Creutzfeldt-Jakob disease.

Objective:To summarize the clinical characteristics and therapeutic efficacy of central nervous system (CNS) aspergillosis.Methods:The clinical manifestations, laboratory examination, neuroimaging features, treatment and prognosis of 37 cases of CNS aspergillosis diagnosed and treated in the First Medical Center of People′s Liberation Army General Hospital from January 2000 to January 2021 were retrospectively analyzed. According to the correlation between intracranial lesions and paranasal sinus lesions, they were divided into two groups: rhino-cerebral aspergillosis (RA, n=21) group and cerebral aspergillosis (CA, n=16) group. Results:Only 16.2% (6/37) of CNS aspergillosis patients had a clear background of immunosuppression, but 35.1% (13/37) were complicated with diabetes. The most common clinical manifestations were headache (73.0%, 27/37), cranial nerve involvement (59.5%, 22/37) and fever (37.8%, 14/37). Cerebrospinal fluid characteristics included increased pressure (53.8%, 14/26), increased white blood cell count (46.7%, 14/30), decreased glucose (30.0%, 9/30), increased protein (70.0%, 21/30), and high positive results of the metagenomic next-generation sequencing (mNGS) of pathogenic microorganism (7/10). Cranial magnetic resonance imaging showed that commonly involved sites were sinus, orbital apex, posterior orbit, cavernous sinus (43.2%, 16/37) and cerebral lobes (27.0%, 10/37). Treatment options included antifungal drugs alone (64.9%, 24/37), combination of drugs and surgery (27.0%, 10/37) and surgery alone (8.1%, 3/37). Compared with the CA group, RA group had fewer males [47.6% (10/21) vs 14/16, χ2=6.34, P=0.012] and older age [(54.2±19.4) years vs (38.4±18.4) years, t=2.50, P=0.017], and was more prone to headache [85.7% (18/21) vs 9/16, χ2=4.00, P=0.046) and cranial nerve involvement [81.0% (17/21) vs 5/16, χ 2=9.31, P=0.006]. The misdiagnosis rate of these patients in the early stage was 73.0% (27/37). A total of 29 patients (85.3%, 29/34) were treated with voriconazole successively, and the course of treatment was 3.0 (0.5, 10.4) months. Compared with salvage therapy, the mortality of primary therapy was lower (4/17 vs 9/12, χ2=7.54, P=0.006). All patients were followed up to December 2021, and 17 patients died, with a mortality rate of 45.9% (17/37). Conclusions:CNS aspergillosis may have no definite immunosuppressive background. Some of CNS aspergillosis patients are complicated with diabetes, and the clinical manifestations of the disease lack specificity, with high misdiagnosis rate in the early stage, no inflammatory changes in cerebrospinal fluid, and high positive rate of mNGS for pathogenic microorganism. Early and long-term application of voriconazole can significantly reduce the mortality rate.