Search

This article reported the clinical features of a rare patient with anti-metabotropic glutamate receptor 5 （mGluR5） encephalitis with mental disorders as the initial symptom， so as to provide references for clinical diagnosis and treatment. The patient was a 38-year-old male， developed pharyngeal pain as prodromal symptoms， and the main clinical manifestations included rapidly progressive memory loss， anxiety and depression， and psychomotor excitement symptoms including irritability and impulsive behaviors. The disease had a progressive deterioration. In the most severe state， the patient became unconscious in a shallow coma， with further cognitive decline， hallucinations and delusions， and lack of self-awareness. Both cerebrospinal fluid and serum anti-mGluR5 antibody were strongly positive （1∶100）. After two sessions of hormone shock therapy， the patient showed significantly improvement in consciousness， cognitive， emotional and psychiatric dimensions.

Copyright of Sichuan Mental Health is the property of Sichuan Mental Health Center and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The clinical data of 7 patients with anti-α-amino-3-hydroxy-5-methyl-4- isoxazolepropionic acid receptor (AMPAR) encephalitis admitted to the First Affiliated Hospital of Zhengzhou University from January 2015 to January 2024 were retrospectively collected, and 87 cases with complete literature data were included for summary analysis. Seven casess exhibited limbic encephalitis, including 3 with lung cancer and 1 with thymoma. All cases were treated with glucocorticoids and/or human immunoglobulin. Four cases with concurrent tumors were followed up for 1 to 25 months and all died. Among the 94 patients (7 admitted cases and 87 documented cases), there were 76 cases with tumor (80.9%), including 33 cases of thymoma (43.4%), 23 cases of lung cancer (30.3%), 9 cases of breast cancer (11.8%), and 6 cases of ovarian tumor (7.9%). The tumor was diagnosed in 13 cases (18.8%) before encephalitis, 40 cases (58.0%) within 1 month after encephalitis, 13 cases (18.8%) within 1-6 months, 2 case (2.9%) within 6-12 months, and 1 case (1.4%) after 1 year. Compared to the 18 patients without tumors who were followed up for>1 year, the 76 patients with tumors showed an increase in age and incidence of mental disorders (both P <0.05), while follow-up time and proportion of good prognosis were decreased (both P <0.05). Closely follow-up should be conducted within 1 year after anti-AMPAR encephalitis, especially in the lungs, thymus, breast, and ovaries. The prognosis of patients with tumors is poor, and older age and mental disorders may indicate the occurrence of tumors.

Objective To investigate the clinical characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated encephalitis. Methods and Results From June 2016 to October 2017, a total of 7 patients with anti-LGI1 antibody-associated limbic encephalitis were diagnosed and treated, with an average age at onset (48.29 ± 15.09) years. Patients presented acute (4 cases) or subacute onset (3 cases), with seizures and memory dysfunction as the main manifestations. It may be accompanied by faciobrachial dystonic seizures (FBDS, 5 cases), mental and behavioral abnormalities or personality changes (4 cases), or even combined with intractable hyponatremia (2 cases) or thymoma (one case). Serum anti-LGI1 antibody tests showed positive results in 7 cases, and cerebrospinal fluid (CSF) anti-LGI1 antibody tests showed positive results in 6 cases. MRI showed unilateral or bilateral medial temporal lobe abnormal signals (6 cases), and EEG showed continuous spike-slow waves or slow waves (one case). High-dose glucocorticoid sequential therapy was effective in 6 cases. During the follow-up period, 2 cases were lost, and the other 5 cases presented long-term memory disorder (3 cases) or long-term and short-term memory disorders (2 cases). Among them, one case relapsed 6 months after discharge. Conclusions According to specific clinical manifestations of patients (such as onset of FBDS, memory disorders, etc.), combined with imaging and CSF examination results, this disease can be clearly diagnosed. Immunosuppressive agents can effectively improve the clinical symptoms and prognosis. DOI: 10.3969/j.issn.1672-6731.2019.04.010

Objective To summarize clinical seizures and electroencephalography (EEG) characteristics of patients with anti ? leucine ? rich glioma ? inactivated 1 (LGI1) antibody ? associated encephalitis. Methods Clinical manifestations, imaging and EEG characteristics, treatment and prognosis of 35 patients with anti?LGI1 antibody?associated encephalitis, who were clearly diagnosed by laboratory examinations and followed up for 2 years, were analyzed. Results Thirty?five patients included 26 males and 9 females, median age 57.35 years. Of all 35 cases, 31 cases (88.57%) had epileptic seizures, mainly as complex partial seizure (CPS), and 13 cases (37.14%) had faciobrachial dystonic seizures (FBDS). Brain MRI showed T2WI and FLAIR high?intensity signals in bilateral (16 cases) or unilateral (12 cases) mesial temporal lobes. 18F ? fluoro ? 2 ? deoxy ? D ? glucose (18F ? FDG) PET examination showed hypermetabolism in temporal lobe and/or basal ganglia (22/24). Thirty?one patients performed video EEG (VEEG) examination, among whom 23 patients showed abnormal background activity, manifesting as diffuse slow waves or regional slow waves in frontotemporal region; 18 cases showed anterior temporal epileptic discharges. There were 22 cases grasping seizures in VEEG, among whom 18 cases obtained epileptic seizures and 13 of them were temporal lobe onset CPS (10 cases) or simple partial seizure (SPS, 7 cases) including pilo?erection seizure in 5 cases, myoclonic seizure in 4 cases and secondary generalized tonic?clonic seizure (GTCS) in one case. Two of 6 FBDS patients showed epileptic discharges followed by temporal lobe onset. Twenty?seven cases received oral antiepileptic drugs (AEDs), among whom 4 of 25 patients with epileptic seizures had reduced frequency ≥ 50%, while 11 FBDS patients had no effect, 8 cases (29.63%) had anaphylaxis. Thirty?five patients received immunomodulation treatment, including intravenous immunoglobulin (IVIg) in 33 cases, corticosteroids in 33 cases, mycophenolate in 4 cases and zathioprine in 2 cases, the seizure frequency of all patients reduced ≥ 50% after treatment. After hospital discharge, 17 patients stop taking AEDs within one year and 26 patients stop taking AEDs within 2 years. Of the patients who were followed up for 2 years, 29 cases achieved epileptic seizure free, only 2 patients developed to chronic epilepsy (anti?LGI1 antibody were negative while seizures were not free). Conclusions Anti?LGI1 antibody?associated encephalitis is a kind of autoimmune limbic encephalitis with specific clinical characteristics, often presenting with FBDS and temporal lobe seizures (typically with pilo?erection aura) combined with amnesia, psychiatric disorder/abnormal behavior and hyponatremia. Early diagnosis and timely immunotherapy can achieve good prognosis. DOI: 10.3969/j.issn.1672-6731.2018.05.006

DOI: 10.3969/j.issn.1672-6731.2018.05.013

Objective To investigate the clinical characteristics of anti - leucine - rich glioma - inactivated 1 (LGI1) antibody - associated encephalitis. Methods and Results From June 2016 to October 2017, a total of 7 patients with anti - LGI1 antibody - associated limbic encephalitis were diagnosed and treated, with an average age at onset (48.29 ± 15.09) years. Patients presented acute (4 cases) or subacute onset (3 cases), with seizures and memory dysfunction as the main manifestations. It may be accompanied by faciobrachial dystonic seizures (FBDS, 5 cases), mental and behavioral abnormalities or personality changes (4 cases), or even combined with intractable hyponatremia (2 cases) or thymoma (one case). Serum anti - LGI1 antibody tests showed positive results in 7 cases, and cerebrospinal fluid (CSF) anti - LGI1 antibody tests showed positive results in 6 cases. MRI showed unilateral or bilateral medial temporal lobe abnormal signals (6 cases), and EEG showed continuous spike - slow waves or slow waves (one case). High - dose glucocorticoid sequential therapy was effective in 6 cases. During the follow - up period, 2 cases were lost, and the other 5 cases presented long - term memory disorder (3 cases) or long - term and short - term memory disorders (2 cases). Among them, one case relapsed 6 months after discharge. Conclusions According to specific clinical manifestations of patients (such as onset of FBDS, memory disorders, etc.), combined with imaging and CSF examination results, this disease can be clearly diagnosed. Immunosuppressive agents can effectively improve the clinical symptoms and prognosis. [ABSTRACT FROM AUTHOR]

Copyright of Chinese Journal of Contemporary Neurology & Neurosurgery is the property of Chinese Journal of Contemporary Neurology & Neurosurgery and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A 78-year-old man was admitted to Peking Union Medical College Hospital with fever, weakness of lower extremities, less speech, loss of memory. Fever was relieved after antibiotic treatment, while cognitive impairment and disorder of consciousness progressed rapidly, followed by critical pulmonary infections, respiratory failure, and septic shock. Lab tests showed negative occult blood, normal serum CEA level and positive Anti-nuclear-antibody. PET-CT suggested that strong FDG uptake signals were seen at sigmoid, while bilateral frontal lobe, temporal lobe, parietal lobe, posterior cingulate gyrus showed lower metabolic activity. Colonoscopy biopsy revealed differentiated adenocarcinoma of sigmoid colon. Therefore, paraneoplastic syndrome of nervous system secondary to colon cancer was considered. Rapid and proper diagnosis and treatment were completed by multidisciplinary team including departments of neurology, gastroenterology, general surgery, ICU, rheumatology, clinical nutrition. The laparoscopic sigmoid colectomy was performed under general anesthesia. The patient finally presented with significant improvement of cognition and consciousness. Respiratory function was totally recovered.

Objective: To analyze the differences of clinical characteristics and outcomes between relapsing and monophasic patients with anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis. Methods: Medical records of confirmed anti-LGI1 encephalitic patients who underwent immunotherapy were retrospectively collected from January 2015 to January 2019 in the first affiliated hospital of Zhengzhou University. Clinical data, treatment methods, duration of treatment and outcomes were analyzed between the relapsing and monophasic groups. Results: Among the 33 anti-LGI1 encephalitic patients, there were 12 and 21 cases in the relapsing and monophasic groups, respectively. No difference was found in age, sex, precipitating factors, intensive care unit (ICU) admission, symptoms and modified Rankin Scale (mRS) score in the acute phase ( P >0.05). As to the lab test and image examination, no statistic difference was found in serum and cerebral spinal fluid (CSF) positive rate, hyponatremia, abnormal rate of electrocardiogram (ECG), electroencephalogram (EEG), CSF and magnetic resonance imaging (MRI) and lesion locations ( P >0.05). No difference was found in time to diagnose the disease between the 2 groups ( P >0.05). The median immunotherapy period was 102.5 days in relapsing group and 194.0 days in monophasic group, with a statistic difference ( P= 0.001). No patients had bad outcomes in the monophasic group at the last follow-up, while 6 patients had poor outcomes in the relapsing group (4 patients died). The patients in relapsing group had a worse prognosis compared to those in the monophasic group ( P= 0.007). Conclusions: Relapse is common in anti-LGI1 encephalitis. Patients in the relapsing group received a shorter term of immunotherapy and had worse outcomes than those in the monophasic group.

Leucine-rich glioma-inactivated protein 1(LGI1)antibody-associated encephalitis is an autoimmune brain disease mainly seen in mid-aged and elderly people.Its main clinical manifestations include abnormal mental behaviors,facial-arm dystonia,hyponatremia,and hypokalemia.Immunotherapy with gamma globulin and/or hormone is effective.Two patients with LGT1 antibody-associated encephalitis were diagnosed in our center between January 2018 and October 2018,with typical clinical findings.The disease was cursed after immunoglobulin and hormone treatments.

Objective: This study was to describe the clinical characteristics of Anti-leucine-rich glioma inactivated 1 protein(LGI1) antibody associated limbic encephalitis. Methods: Clinical data including clinical features, laboratory and radiological findings, treatment and prognosis of the 9 patients were analyzed. Results: In all 9 cases, 6 cases experienced epileptic seizure, 5 cases had psychosis, 7 cases presented with memory impairment, 4 cases showed faciobrachial dystonic seizure, 2 had refractory hyponatremia. One case presented with typically acute Guillain-Barre syndrome (GBS). Anti-LGI1 antibody was detected in 6 cases in cerebrospinal fluid (CSF) samples and 9 in serum samples. Seven cases out of 9 had brain imaging abnormalities. All 9 cases found no evidence of tumors. Eight cases were given immune therapy. During a 1-16 months follow-up, 1 case had complete recovery, 5cases had various degree of sequelae , among whom 4 had memory disturbance and 1 case had changed personality. 2cases were lost to follow-up. Conclusions: Limbic encephalitis is the most common manifestation of anti-LGI1 antibody associated encephalitis. Faciobrachial dystonic seizure (FBDS) is a specific symptom which favors an early diagnosis. Tumor is uncommon to see. The disease has a relatively favorable prognosis while impaired memory can be seen as a common sequelae.