scholarly journals Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study

2021 ◽  
Vol 12 ◽  
Author(s):  
Shan Qiao ◽  
Huai-kuan Wu ◽  
Ling-ling Liu ◽  
Ran-ran Zhang ◽  
Mei-ling Wang ◽  
...  

Objective: This study aimed to investigate epidemiological characteristics, clinical manifestations, and long-term outcomes of patients with autoimmune encephalitis (AE) in the east of China.Methods: From January 2015 to December 2019, 226 potential AE patients were recruited from five clinical centers, and a total of 185 patients who met the diagnostic criteria were included in the study. We retrospectively reviewed clinical features, auxiliary examinations, details of treatments, and outcomes of AE, and identified risk factors of poor prognosis. Modified Rankin Scale scores were used to evaluate neurological function, and scores of 3–6 indicated a poor-prognosis.Results: Patients with five main subtypes of AE were enrolled in the study, as follows: anti-NMDAR (79), anti-LGI1 (55), anti-CASPR2 (30), anti-GABABR (16), and anti-AMPAR (5). Among 185 patients, 58.38% (108/185) were male and 41.62% (77/185) were female. The median age at disease onset was 41 years (interquartile range, 17–62). The most common clinical manifestations of AE were seizures (146, 78.92%) and memory deficit (123, 66.49%). A total of 95 (51.35%) patients had abnormal brain magnetic resonance imaging results. Electroencephalographic findings were abnormal in 131 (70.81%) patients, and 168 (90.81%) and 26 (14.05%) patients were treated with first- and second-line immunotherapies, respectively. All surviving patients were followed-up for at least 1 year (range 12–36 months). Good clinical outcomes were achieved in 117 (63.24%), while 68 (36.76%) patients had a poor prognosis. Further, 33 (17.84%) patients relapsed and 10 (5.41%) died within 1 year post-discharge. Older patients tended to have a poorer prognosis, and the occurrence of mental behavioral disorders, movement disorders, disturbance of consciousness, central hypoventilation, and tumors were overrepresented in the poor-prognosis group.Conclusions: AE is a treatable disease, and most patients have a good prognosis. There are differences in the clinical manifestations of patients with different AE subtypes. Some with AE will relapse, and long-term follow-up is of great significance for further research.

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhe Zhang ◽  
Siyuan Fan ◽  
Haitao Ren ◽  
Lixin Zhou ◽  
Hongzhi Guan

Abstract Background Encephalitis associated with antibodies against alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is an extremely rare type of antibody-mediated encephalitis. This research aims to investigate the clinical characteristics and prognosis of anti-AMPAR encephalitis. Methods This retrospective study enrolled nine patients with anti-AMPAR encephalitis. Demographic information, clinical manifestations, laboratory and radiological findings, treatment and response were collected and analyzed. These patients were followed up with an average period of 72 weeks to gather prognostic information. Results Nine patients (7 females and 2 males) were enrolled with a mean age at disease onset of 59 years old. Three clinical pictures, including limbic encephalitis (n = 7; 78%), pure amnesia (n = 1; 11%) and fulminant encephalitis (n = 1; 11%) were identified. New symptoms of dysphagia and deafness were identified in the clinical spectrum of anti-AMPAR encephalitis. All patients had positive blood AMPAR antibodies, and six of them (67%) had paired positive antibodies in cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) was abnormal in 75% of the patients with no specific patterns recognized. Six patients (67%) had tumors, including lung cancers and thymomas. After immunotherapy and oncotherapy, partial improvement of neurological symptoms was observed among all 6 patients with available records during their hospitalization. After a mean follow-up of 72 weeks, 3 patients had marked decrease of modified Rankin Scale (mRS) score, 1 patient had unchanged mRS score, 4 patients died and the other one was lost. Conclusions Anti-AMPAR encephalitis mainly presents as limbic encephalitis, and is paraneoplastic in 67% of cases. Thus, intensive screening for tumors is recommended for all anti-AMPAR patients. Although patients showed a good short-term therapeutic response, the overall prognosis was not satisfactory.


2021 ◽  
Author(s):  
Zhe Zhang ◽  
Siyuan Fan ◽  
Haitao Ren ◽  
Lixin Zhou ◽  
Hongzhi Guan

Abstract Background Encephalitis associated with antibodies against alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is an extremely rare type of antibody-mediated encephalitis. This research aims to investigate the clinical characteristics and prognosis of anti-AMPAR encephalitis. Methods This retrospective study enrolled nine patients with anti-AMPAR encephalitis. Demographic information, clinical manifestations, laboratory and radiological findings, treatment and response were collected and analyzed. These patients were followed up with an average period of 72 weeks to gather prognostic information. Results Nine patients (7 females and 2 males) were enrolled with the mean age of disease onset as 59 years old. Three clinical syndromes, including limbic encephalitis (n=7; 78%), pure amnesia (n=1; 11%) and fulminant encephalitis (n=1; 11%) were identified. New symptoms of dysphagia and deafness were identified in the clinical spectrum of anti-AMPAR encephalitis. All patients had positive blood AMPAR antibodies, and six of them (67%) had paired positive antibodies in cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) were abnormal in 75% of the patients with no specific patterns recognized. Six patients (67%) had tumors, including lung cancers or thymomas. After immunotherapy and oncotherapy, partial improvement of neurological symptoms was observed among all 6 patients with available records during their hospitalization. During a mean follow-up of 72 weeks, 3 patients had marked improvement of modified Rankin Scale (mRS), one patient had unchanged mRS, 4 patients died and the other one was lost. Conclusions Anti-AMPAR encephalitis mainly presents as limbic encephalitis. Anti-AMPAR encephalitis is paraneoplastic in 67% cases and intensive screening for tumors is recommended for all anti-AMPAR patients. Although patients showed a good short-term therapeutic response, the overall prognosis was not satisfactory.


2021 ◽  
Vol 8 (6) ◽  
pp. e1088
Author(s):  
Franziska S. Thaler ◽  
Luise Zimmermann ◽  
Stefan Kammermeier ◽  
Christine Strippel ◽  
Marius Ringelstein ◽  
...  

Background and ObjectivesTo determine the real-world use of rituximab in autoimmune encephalitis (AE) and to correlate rituximab treatment with the long-term outcome.MethodsPatients with NMDA receptor (NMDAR)-AE, leucine-rich glioma-inactivated-1 (LGI1)- AE, contactin-associated protein-like-2 (CASPR2)-AE, or glutamic acid decarboxylase 65 (GAD65) disease from the GErman Network for Research on AuToimmune Encephalitis who had received at least 1 rituximab dose and a control cohort of non–rituximab-treated patients were analyzed retrospectively.ResultsOf the 358 patients, 163 (46%) received rituximab (NMDAR-AE: 57%, CASPR2-AE: 44%, LGI1-AE: 43%, and GAD65 disease: 37%). Rituximab treatment was initiated significantly earlier in NMDAR- and LGI1-AE (median: 54 and 155 days from disease onset) compared with CASPR2-AE or GAD65 disease (median: 632 and 1,209 days). Modified Rankin Scale (mRS) scores improved significantly in patients with NMDAR-AE, both with and without rituximab treatment. Although being more severely affected at baseline, rituximab-treated patients with NMDAR-AE more frequently reached independent living (mRS score ≤2) (94% vs 88%). In LGI1-AE, rituximab-treated and nontreated patients improved, whereas in CASPR2-AE, only rituximab-treated patients improved significantly. No improvement was observed in patients with GAD65 disease. A significant reduction of the relapse rate was observed in rituximab-treated patients (5% vs 13%). Detection of NMDAR antibodies was significantly associated with mRS score improvement. A favorable outcome was also observed with early treatment initiation.DiscussionWe provide real-world data on immunosuppressive treatments with a focus on rituximab treatment for patients with AE in Germany. We suggest that early and short-term rituximab therapy might be an effective and safe treatment option in most patients with NMDAR-, LGI1-, and CASPR2-AE.Class of EvidenceThis study provides Class IV evidence that rituximab is an effective treatment for some types of AE.


2022 ◽  
Vol 20 (4) ◽  
pp. 18-24
Author(s):  
I. A. Palchikova ◽  
O. A. Denisova ◽  
G. M. Chernyavskaya ◽  
I. L. Purlik ◽  
T. P. Kalacheva ◽  
...  

Aim. To study clinical and morphological phenotypes in different variants of the course of intrathoracic sarcoidosis and isolate new phenotypes.Materials and methods. The study included 121 patients with intrathoracic sarcoidosis aged 21–66 years (50.4% were men, 49.6% were women, the average age at the time of the disease onset was 38 years) over the period 2007– 2019. During the examination, patients’ complaints were studied thoroughly, and the diagnosis was histologically verified in all cases. During an extended histological examination, the quantitative and qualitative composition of biopsy specimens was investigated. The number of granulomas in the field of vision and the content of giant cells, macrophages, lymphocytes, neutrophils, and eosinophils in them were studied. Qualitative parameters were assessed for the presence of hyalinosis, Schaumann bodies, necrosis, stamping, calcification, fibrosis, and vasculitis. All patients were retrospectively divided into two clinical groups depending on the outcomes of the disease: group 1 included patients with a favorable course of sarcoidosis, proceeding without relapses and signs of progression; group 2 encompassed patients with an unfavorable course of the disease with relapses and progression, requiring long-term administration of systemic glucocorticoids.Results. The analysis showed that among all general clinical manifestations, only the presence of dyspnea, skin manifestations, and weight loss occurred significantly more often in the patients with an unfavorable course of intrathoracic sarcoidosis (р = 0.04; 0.02; and 0.01, respectively). Among morphological parameters, a large number of macrophages was significantly more frequent in the biopsy specimens in this group of patients (р < 0.01). 


2021 ◽  
Vol 12 ◽  
Author(s):  
Xiaoxiao Qin ◽  
Huajun Yang ◽  
Fei Zhu ◽  
Qun Wang ◽  
Wei Shan

ObjectiveTo examine the clinical characteristics of autoimmune encephalitis associated with the contactin-associated protein-2 (CASPR2) antibody.Materials and MethodsMedical records of all patients diagnosed with CASPR2 antibody-associated encephalitis were retrospectively analysed. Data regarding demographic features, neurological symptoms and signs, laboratory tests, imaging results, treatments, and prognosis were collected.ResultsA total of 25 patients aged from 3 to 79 years old were enrolled in this study, with a median age of 43. Eight of 25 (32%) were female, and 17 of 25 (68%) were male. The median age of symptom onset was 42 years old with the course of disease from onset to hospital admission ranging from 2 days to 6 months (median was 17 days). Six patients (6/25) had fever as an onset symptom. During the course of disease, cognitive disturbance was the most common symptom, which was observed in 17 patients (17/25) in total. Eight patients (8/25) met the criteria for limbic encephalitis. Epileptic seizure occurred in six of these eight patients. Four patients (4/25) were diagnosed as Morvan syndrome. All patients were positive for anti-CASPR2 antibody in the serum (1:10–1:300). In six patients, antibodies were detected both in the blood and CSF (1:32–1:100). White blood cell (WBC) counts in the CSF were elevated in eight patients (8/25). The concentration of proteins in CSF increased in 10 patients (ranging from 480 to 1,337.6 mg/dl), decreased in seven patients (ranging from 23.2 to 130.5 mg/dl) and remained at a normal range in the other eight patients (ranging from 150 to 450 mg/dl). Abnormal electroencephalogram (EEG) activities included slow background activity and epileptic patterns. Abnormal signals in the bilateral hippocampus were detected by magnetic resonance imaging (MRI) in three patients presenting cognitive disturbance. In one patient who had limbic encephalitis, increased metabolism of bilateral basal ganglia and the mesial temporal lobe was revealed by PET-CT. Eleven of 15 patients receiving immunotherapy experienced varying degrees of improvement. Relapse occurred in four of 25 patients (4/25) after 2 months.ConclusionCASPR-antibody-mediated autoimmune encephalitis is characterized by diverse clinical manifestations. The most prominent conclusion revealed by this retrospective analysis is the involvement of both central and peripheral nerve systems, as well as a lower relapse rate, a good response to immunotherapy, and favorable short-term prognosis after treatment was also demonstrated. Besides, additional work is necessary to evaluate the long-term prognosis.


2019 ◽  
Author(s):  
Juan Du ◽  
Shixiao Dong ◽  
Shengnan Jia ◽  
Qiaoru Zhang ◽  
Mingyan Hei

Abstract Background Congenital tuberculosis (TB) is a rare disease with a high mortality. In this study, we reviewed patients with congenital TB that were promptly treated with an anti-TB regimen, including linezolid, based on new clinical diagnosis criteria. We described the clinical manifestations, treatment, and long-term prognosis of this disease in these patients. Methods This was a retrospective observational study that enrolled patients with congenital TB that were admitted to Beijing Children’s Hospital between 2009 and 2018. Results A total of 10 congenital TB patients were enrolled. Ninety-percentage of the mothers were diagnosed at postpartum; however, none of the mothers received anti-TB treatment during pregnancy. The onset age of congenital TB ranged from 1 to 53 days. Chest computed tomography scans demonstrated pulmonary nodules in 100% of the patients and mediastinal adenopathy in 40% of the patients. Abdominal ultrasonography showed hypoechoic nodules in the liver and spleen in 40% of the patients. The positive rate of GeneXpert and T-spot were relatively high—80% and 78%, respectively. Anti-TB treatment was started when congenital TB was confirmed for 1 patient based on TB-specific tests or clinically diagnosed for the other 9 patients based on confirmed maternal TB history and typical radiological findings. Beside isoniazid, rifampicin, and pyrazinamide, linezolid was also administrated in 70% of the patients for a period of 2–6 months. One patient was reported to present with spontaneously resolved thrombocytopenia. The survival rate was 100%, and 33% of the patients have thrived and reached normal developmental milestones since the last documented follow-up. Conclusion Congenital TB is a potentially fatal disease, but early diagnosis based on maternal history, typical imaging results, and timely treatment can improve the outcome. Linezolid is safe and might be effective in reducing the mortality of congenital TB.


2011 ◽  
Vol 6 (3) ◽  
pp. 181 ◽  
Author(s):  
Laura Gioia ◽  
Alexandre Y Poppe ◽  
Sylvain Lanthier ◽  
◽  
◽  
...  

Primary angiitis of the central nervous system (PACNS) is a rare and life-threatening form of vasculitis confined to the CNS. A timely diagnosis is a real challenge because clinical manifestations of PACNS are diverse and nonspecific. Headaches, cerebrospinal fluid inflammation and abnormal brain magnetic resonance imaging are prevalent. When PACNS is suspected, a thorough investigation is mandatory to rule out several potential simulators and confirm the diagnosis. Treatment of PACNS is also a challenge involving competing forces, which include the threat of serious adverse effects of potent immunosuppressive agents and the risk of neurological deteriorations due to insufficient immunosuppressant therapy. Efforts are ongoing to delineate subtypes requiring different therapeutic approaches and having distinct prognoses. Despite recent progress, PACNS is still fatal in as much as one-sixth of cases. Long-term follow-up is mandatory in patients with PACNS.


2019 ◽  
Vol 47 (3-4) ◽  
pp. 105-111 ◽  
Author(s):  
Shunsuke Nomura ◽  
Koji Yamaguchi ◽  
Hiroyuki Akagawa ◽  
Akitsugu Kawashima ◽  
Yosuke Moteki ◽  
...  

Background: Homozygosity of this p.R4810K founder variant of RNF213moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear. Objectives: The principal goal of this study was to investigate whether and how the p.R4810K variant of RNF213influences the long-term phenotype in Japanese patients with MMD. Method: This retrospective cohort study included 94 Japanese patients with MMD who underwent direct or combined bypass for revascularization with the p.R4810K genotype determined in our hospital. The following phenotypic parameters were analyzed at disease onset and over a long-term period: age and initial presentation at onset, recurrent stroke after initial revascularization, and final modified Rankin Scale. Results: The p.R4810K genotype was significantly associated with the phenotype at onset, especially in younger patients. Over a median follow-up period of 100 months, recurrent stroke occurred in 6 out of 94 patients: none out of 5 patients with the homozygous variant, 5 out of 64 with the heterozygous variant, and 1 out of 25 in the wild-type group. There were no significant differences among the genotypes. In particular, recurrent cerebral hemorrhage occurred in 5 patients, all possessing the heterozygous variant. The log-rank test showed no difference between the genotypes in the stroke-free survival rate. Furthermore, the p.R4810K genotype was not associated with a poor functional condition. Conclusions: The p.R4810K founder variant of RNF213 affects the phenotype at disease onset. However, the optimal revascularization may be effective, regardless of the genotype, even for the homozygous variant, which has been thought to be the most pathogenic. This genotype may not strongly influence the long-term clinical manifestations or poor prognosis in MMD.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1204.2-1204
Author(s):  
I. Pontikaki ◽  
S. Carbogno ◽  
F. Corona ◽  
A. Petaccia ◽  
R. Cimaz

Background:Juvenile Idiopathic Arthritis (JIA) is a chronic pediatric inflammatory disease that shows many differences compared to adult-onset arthritis. The different clinical manifestations, the assessment and the management of JIA is the reason that the transition from childhood to adulthood is an important multidimensional process that emphasizes a lot of aspects.Objectives:To describe the long-term outcome of JIA.Methods:Five-hundred and twenty patients affected by JIA and referred to a transition care rheumatology tertiary centre were considered between 1999 and 2019. The outcome assessment included remission, disease duration, medications, number of prosthesis implantation, pregnancies, mortality and social integration (employment status and educational level).Results:A hundred and thirty-eight (26%) males and 382 (73%) females were included; 157 (30%) patients were lost to follow up. The mean age of the patients was 27 (18-57) years, with a mean age at onset of 8 years and an average disease duration of 19 years. Subtypes of JIA at disease onset included 252 (48%) oligoarthritis, 134 (26%) polyarthritis, 64 (12%) systemic arthritis, 22 (4%) psoriatic arthritis, 43 (8%) enthesitis related arthritis and 1 (0.1%) undifferentiated arthritis. Ninty-three (18%) patients suffered of uveitis. Ninty-five implant prosthesis and 16 arthrodesis were recorded. At follow up 198 (38%) patients were on remission of which 107 (20%) off medication. Among the 322 patients still on medication, 84 (16%) were under treatment with oral steroids, 226 (43%) with sDMARDs and 249 (40%) with bDMARDs. Five deaths (1%) occurred in this cohort. Two hundred and thirty-five subjects had a higher educational level, 327 had an employment. We have data of twenty-nine pregnancies. The transition age was considered after the age of 16 years old. The key word for the management of this cohort was the multidisciplinary approach towards each patient, with the collaboration of other specialists (ophthalmologist, orthopedic, dermatologist, gastroenterologist, obstetric and psychologist).Conclusion:In the era of biologic therapy the long-term outcome of JIA underwent an outstanding improvement regarding a lot of variables. Two hundred and thirty-two patients were still followed, not only because of the continuation of the biological therapy, but also for a multidisciplinary care even during remission. JIA often persists over the adulthood, therefore the long term follow-up and care of these patients needs to be conducted by a rheumatologist expertized in JIA in collaboration with other specialists.Disclosure of Interests:None declared


2021 ◽  
pp. jim-2020-001757
Author(s):  
Xiurong Li ◽  
Jianan Dong ◽  
Yaping Sun ◽  
Qing Li ◽  
Li Shi

Our study was conducted to explore the relationship between cerebral venous sinus thrombosis (CVST) and intracranial arteritis during the time of pregnancy as well as puerperium. The current retrospective case study involved a total of 153 patients with pregnancy-related CVST. CVST was diagnosed mainly based on clinical manifestations and imaging results. Detailed information on demographics, risk factors (excluding intracranial arteritis), and pregnancy outcomes was systematically recorded. The average age of patients diagnosed with CVST was 28.67±3.54 years old. Among these patients, there were 62 cases of puerperal CVST and 91 cases of pregnancy-related CVST. The clinical manifestations of patients with CVST included headache in 55 cases (35.95%), symptomatic seizures in 26 cases (16.99%), symptomatic limb weakness in 22 cases (14.38%), symptomatic nausea/vomiting in 18 cases (11.76%), symptomatic disturbance of consciousness in 15 cases (9.80%), symptomatic blurred vision in 10 cases (6.54%), and symptomatic fever in 7 cases (4.58%). A proportion of patients reported higher abnormal cerebrospinal fluid pressure (98.28%), white cell count (79.31%), total cholesterol (71.55%) and low-density lipoprotein (62.93%) (p<0.01). The proportion of intracranial arteritis lesions and CVST lesions was high, including 51 cases (43.97%) on the left side and 31 cases (26.72%) on the right side. Bilateral CVST lesions occurred in 13 cases (11.20%) of unilateral intracranial arteritis lesions, and bilateral intracranial arteritis lesions occurred in 16 cases (13.79%) of unilateral CVST lesions. In summary, CVST indicates a potential positive link to intracranial arteritis at the time of pregnancy and puerperium.


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