BACKGROUND Paraneoplastic limbic encephalitis(LE)is an inflammatory condition that affects the limbic system,cerebellum,and peripheral nervous system.It causes a range of symptoms including short-term memory loss,impa...BACKGROUND Paraneoplastic limbic encephalitis(LE)is an inflammatory condition that affects the limbic system,cerebellum,and peripheral nervous system.It causes a range of symptoms including short-term memory loss,impaired cognitive function,behavioral and psychological disorders,and seizures.Paraneoplastic LE can occur when an immune response is activated due to antibodies targeting gammaaminobutyric acid(GABA)B receptor(GABABR)interacting with antigens on tumor cells and the nervous system,resulting in tumors primarily as small cell lung carcinoma(SCLC).CASE SUMMARY We discuss two cases of GABABR antibody-related LE resulting from SCLC.The patients’symptoms were managed with immunotherapy but ended in premature death due to chemotherapy-related complications.CONCLUSION Paraneoplastic syndrome is a notable cause of LE.Early intravenous immunoglobulin therapy may lead to temporary remission.展开更多
Limbic encephalitis represents a cluster of autoimmune disorders, with inflammation in the medial temporal lobe characterised by the subacute onset of neuropsychiatric symptoms such as anxiety, affective symptoms, psy...Limbic encephalitis represents a cluster of autoimmune disorders, with inflammation in the medial temporal lobe characterised by the subacute onset of neuropsychiatric symptoms such as anxiety, affective symptoms, psychosis, short-term memory impairment, as well as faciobrachial and grand mal seizures. The limbic system is a complex anatomical structure which this paper seeks to explain in terms of its anatomy and physiology, before exploring what happens when it is impaired as is the case of autoimmune and limbic encephalitis. We will discuss the pathophysiology, clinical symptomatology and diagnosis of autoimmune encephalitis, a cluster of symptoms which can be easily overlooked or misdiagnosed within psychiatric settings. Characteristic indicators of autoimmune encephalitis include neurologic symptoms such as facial twitching, seizures, confusion, and cognitive decline;however, our experience realises that autoimmune encephalitis is not easy to identify as most patients initially present with psychiatric symptomatology rather than these neurological symptoms. Furthermore, immunological and laboratory testing take a long time to diagnose the condition. Importantly, few psychiatrists consider the autoimmune nature of the neuropsychiatric presentation. It is hence vital to consider autoimmune encephalitis in all patients with atypical presentations.展开更多
BACKGROUND： The biochemical hypothesis of dopamine hyperfunction in the brain can explain the pathological mechanisms of schizophrenia. Surgery is performed based on limbic system circuit theory correspondence to abo...BACKGROUND： The biochemical hypothesis of dopamine hyperfunction in the brain can explain the pathological mechanisms of schizophrenia. Surgery is performed based on limbic system circuit theory correspondence to above-mentioned hypothesis. Stereotactic surgery for the treatment of mental disorders is related to stereotactic surgery that influences the Papez circuit. OBJECTIVE： To observe the effects of stereotactic multi-target limbic leucotomy on the improvements in memory, intelligence and psychiatric symptoms in the treatment of intractable psychiatric disease. DESIGN： Self-control case analysis and follow-up of therapeutic effects. SETTING： Department of Neurosurgery, First Hospital, Hebei Medical University. PARTICIPANTS： Thirty patients with intractable psychiatric disease, who received stereotactic surgery in the Department of Neurosurgery, First Hospital, Hebei Medical University between July 2002 and August 2005, were included in this study. The patients, 21 males and 9 females, all met the diagnostic criteria of intractable psychiatric disease, determined by the national psychosurgery cooperation team in 1998. Informed consents for surgery and clinical follow-up exams were obtained from patients and/or patients＇ relatives （guardians）. METHODS： In 30 patients with intractable psychiatric disease, limbic leucotomy was performed by stereotactic technique. Multi-target radiofrequency hyperthermia was performed in the intracranial amygdaloid nucleus, anterior limb of internal capsule, callosal gyrus, among other regions. The therapeutic effects of patients were evaluated by Brief Psychiatric Rating Scale （BPRS） before surgery, and 6 months, 1, and 3 years after surgery. The Wechsler Adult Intelligence Scale （WAIS） and Clinical Memory Scale （CMS） were used to assess memory and intelligence before and after surgery. MAIN OUTCOME MEASURES： Memory, intelligence, and psychiatric symptoms of patients before and after operation. RESULTS： Thirty patients were included in the final analysis. （1） Memory and intelligence changes of patients： CMS and WAIS showed there were no significant differences in the changes of brain functions before and after operation （P 〉 0.05）. （2） Improved degree of symptoms： 5 patients were cured, 8 patients significantly improved, 8 patients improved, 3 patients had no changes, and 6 patients had recurrent symptoms. CONCLUSION： Stereotactic multi-target limbic leucotomy can improve the symptoms of patients with intractable psychiatric disease. Follow-up results showed that memory and intelligence were not noticeably improved after surgery.展开更多
BACKGROUND： Limbic encephalitis is a rare syndrome that specifically affects the limbic system. Magnetic resonance imaging （MRI） has been typically used to detect brain changes in this disease. However, the mechani...BACKGROUND： Limbic encephalitis is a rare syndrome that specifically affects the limbic system. Magnetic resonance imaging （MRI） has been typically used to detect brain changes in this disease. However, the mechanisms of limbic encephalitis-related white matter damage remain poorly understood. OBJECTIVE： To characterize white matter connectivity changes secondary to injuries of the limbic system in limbic encephalitis through combined application of diffusion tensor imaging （DTI） and voxel-based morphometry. DESIGN, TIME AND SETTING： A non-randomized, controlled, clinical, neuroimaging, DTI study was performed at the Department of Radiology, West China Hospital in December 2008. PARTICIPANTS： A male, 46-year-old, limbic encephalitis patient, as well as 11 age- and gender-matched healthy volunteers, were enrolled in the present study. METHODS： MRI was performed on the limbic encephalitis patient using a 3.0T MR scanner. Three-dimensional SPGR Tl-weighted images and DTI were acquired in the patient and controls. Data were analyzed using Matlab 7.0 and SPM2 software. MAIN OUTCOME MEASURES： Results from routine MRI scan with contrast enhancement of patient, as well as fractional anisotropy and mean diffusivity value map differences between patient and controls. RESULTS： Significant symmetric MRI signal intensity abnormalities were observed with routine MRI Affected bilateral hippocampi and amygdala exhibited hypointense signals in TIWI and hyperintense signals in T2 images. The DTI study revealed decreased fractional anisotropy values in the bilateral alveus and fimbria of the hippocampus, bilateral internal and external capsules, white matter of the right prefrontal area, and left corona radiate in the patient compared with normal controls （P 〈 0.001） Significantly increased fractional anisotropy, mean diffusivity, or decreased mean diffusivity were not observed in the patient, compared with controls. CONCLUSION： Secondary white matter damage to the hippocampal afveus and fimbria was apparent in the limbic encephalitis patient. In addition, other white matter fiber injuries surrounded the limbic structures, which were not attributed to secondary limbic system injuries.展开更多
This case study addresses episodic memory impairment or focal retrograde amnesia related to limbic encephalitis. The patient in question was a right-handed man in his 60s who developed focal retrograde amnesia due to ...This case study addresses episodic memory impairment or focal retrograde amnesia related to limbic encephalitis. The patient in question was a right-handed man in his 60s who developed focal retrograde amnesia due to limbic encephalitis. An abnormal MRI signal was observed in the right medial temporal lobe. Immediately after the encephalitis onset, mental symptoms—including impaired consciousness, disorientation, hallucinations, and delusions—appeared;however, his condition improved with medical treatment. After transfer to a convalescent and rehabilitation hospital, various neuropsychological tests were conducted but no apparent functional decline was observed in intellectual function, attention, memorization, or retention. However, the patient exhibited impairments related to autobiographical memory and memory of events 4?-?5 years before the onset of limbic encephalitis. Based on these observations, the patient was considered to exhibit focal retrograde amnesia due to limbic encephalitis. Despite exhibiting retrograde amnesia, he had no difficulty in his daily life in the ward. Moreover, after discharge from hospital, smooth work reinstatement was possible. This case demonstrates that, even after exhibiting retrograde amnesia, if its duration is relatively short and physical function and other higher-order brain functions are sustained, social rehabilitation—including work reinstatement—is feasible.展开更多
BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CAS...BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.展开更多
Limbic encephalitis (LE) is a clinical entity related to a mesial temporal lesion resulting in a combination of anterograde memory dysfunction, behavioral changes, and seizures. The most common causes of limbic enceph...Limbic encephalitis (LE) is a clinical entity related to a mesial temporal lesion resulting in a combination of anterograde memory dysfunction, behavioral changes, and seizures. The most common causes of limbic encephalitis are Herpes Simplex Virus (HSV) encephalitis, autoimmune encephalitis. Neurosyphilis is an exceptional aetiology. The early diagnosis and management of the disease determine the prognosis. This clinical course highlights the diagnostic challenge limbic encephalitis can cause and the importance of considering neurosyphilis in patients with specific or unspecific neurological symptoms. We report a case of a mesiotemporal form of neurosyphilis revealed by a LE.展开更多
To investigate the function of matrix metalloproteinase-2(MMP-2),matrix metalloproteinase-9(MMP-9) and tissue inhibitors of metalloproteinase-2(TIMP-2) in the invasion of gliomas of the limbic system.Methods The expre...To investigate the function of matrix metalloproteinase-2(MMP-2),matrix metalloproteinase-9(MMP-9) and tissue inhibitors of metalloproteinase-2(TIMP-2) in the invasion of gliomas of the limbic system.Methods The expression of MMP-2,MMP-9 and TIMP-2 in low and high-grade gliomas and meningiomas was detected by using immunohistochemical technique.Results (1) The expression levels of MMP-2 and MMP-9 were significantly higher in high grade gliomas than those in low grade gliomas (P<0.05 and P<0.01 respectively).They were also significantly higher in low grade gliomas than those in meninginomas (P<0.05 and P<0.01 respectively).(2) The TIMP-2 expression level was obviously lower in gliomas of grade Ⅲ and grade Ⅳthan that in gliomas of grade Ⅰ and grade Ⅱ.Also,the expression of TIMP-2 in gliomas of grade Ⅰ and grade Ⅱ was significantly lower than that in meningiomas. Conclusion (1) The expression of MMP-2 and MMP-9 in human brain gliomas is related to malignant progression of gliomas.They might be used as the indicators to evaluate the malignancy and prognosis of gliomas.(2) Imbalance between MMP-s and TIMP-s may be one of the improtant factors promoting glioma invasion.5 refs,3 figs.展开更多
Background： Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor （GABABR） in patients with limbic encephalitis （LE） was first described in 2010. We present a series of klan C...Background： Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor （GABABR） in patients with limbic encephalitis （LE） was first described in 2010. We present a series of klan Chinese patients tbr further clinical refinement. Methods： Serum and cerebrospinal fluid （CSF） samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed. Results： All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram （EEG） or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen （17/18） patients presented with new-onset refractory seizure or status epileptics. Twelve （12/18） patients had memory deficits, 11 （11/18） patients had personality change, 7 （7/18） patients had disturbance of consciousness, and 3 （3/18） patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 （6/18） patients. Ten （10/18） patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten （10/18） patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications. Conclusions： Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABABR antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.展开更多
Kainic acid(KA),a prospective neuroexcitable and neurotoxic analog of glumate,inducespersistent seizure activity in limbic structures which results in behavioral symptoms,electrographic and neuropathologic alternation...Kainic acid(KA),a prospective neuroexcitable and neurotoxic analog of glumate,inducespersistent seizure activity in limbic structures which results in behavioral symptoms,electrographic and neuropathologic alternations closely resembling human temporal lobe epi-lepsy.The hippocampal pyramidal cells possess abundant KA high affinity binding sites intheir membranes and are extremely vulnerable to KA neurotoxicity.NT-3 is a memberof the neurotrophic family(NTF)including nerve growth factor(NGF),brain derivedneurotrophic factor(BDNF),neurotrophin-4/5(NF-4/5).NT-3 mRNA was expressed展开更多
Paraneoplastic neurologic syndromes （PNSs） occur with increased frequency in patients with cancer and almost always antedate its diagnosis.These syndromes comprise a heterogeneous group of cancer-related neurologic ...Paraneoplastic neurologic syndromes （PNSs） occur with increased frequency in patients with cancer and almost always antedate its diagnosis.These syndromes comprise a heterogeneous group of cancer-related neurologic diseases,and they may affect any part of the nervous system.The simultaneous involvement of different areas of the nervous system by the paraneoplastic process is not unusual.Until date,this is the first report of concurrent development of paraneoplastic cerebellar degeneration （PCD） and paraneoplastic limbic encephalitis （PLE） associated with the advanced ovarian cancer and anti-Yo antibodies following hepatitis B （HB） vaccination.The cause of most PNS is believed to be an immune response against neuronal proteins expressed by the tumor.展开更多
Pain is a complex physiological and psychological activity,involving at least three dimensions,including pain sensation,pain emotion,and pain cognition.Acupuncture can clearly relieve the pain sensation of patients an...Pain is a complex physiological and psychological activity,involving at least three dimensions,including pain sensation,pain emotion,and pain cognition.Acupuncture can clearly relieve the pain sensation of patients and improve pain emotion and pain cognition induced by pain;acupuncture participates in the multi-dimensional regulation of pain through brain regions of the limbic system such as anterior cingulate cortex(ACC),amygdala(AMY),and hippocampus.By analyzing relevant literature,it has been found that the regulation of acupuncture on pain emotion is mainly related to the activation of pertinent opioid receptors in the ACC,the decrease of the expression of extracellular signal-regulated kinase(ERK),and the promotion of the expression of glutamic acid(Glu)A1,metabotropic glutamate receptor-1(mGluR1),andγ-aminobutyric acid aminobutyric acid(GABA)B2 protein in the AMY.The regulation of acupuncture on pain cognition is mainly related to the elevation of the expression of protein kinase A(PKA)and phospho-p38 mitogen-activated protein kinase(phospho-p38 MAPK)and the inhibition of cyclic adenosine monophosphate(cAMP)/PKA/cAMP response element-binding protein(CREB)signaling pathway in the ACC.展开更多
Paraneoplastic limbic encephalitis (PLE) is a rare immunopathological syndrome, reported in association with certain types of malignancies. Patients present with cognitive and memory impairments, disordered perception...Paraneoplastic limbic encephalitis (PLE) is a rare immunopathological syndrome, reported in association with certain types of malignancies. Patients present with cognitive and memory impairments, disordered perception, mood and behavioral changes, sleep disturbances, and seizures. Despite the growing number of cases being reported, it still poses a diagnostic challenge. We encountered a patient with a myriad of neuropsychiatric symptoms who exhibited a highly variable response to therapy. A 36-year-old male presented with memory impairment, excessive sleepiness, and slurred speech. Brain magnetic resonance imaging revealed hyperintensities in the temporal lobes and hypothalamus, all suggestive of limbic encephalitis. He was found to have a mixed germ cell testicular teratoma. Screening for commonly associated antibodies did not yield positive results, which emphasizes that sero-negative PLE can be missed in patients with malignancies. In reporting this case, we urge neurologists to consider PLE as part of the differential diagnosis in similar ambiguous clinical scenarios.展开更多
To emphasize the early diagnosis and treatment of anti-N-methyl-d-aspartate-receptor (NMDAR) autoimmune encephalitis, a rare clinical condition, teratoma-related, anti-NMDAR encephalitis should be suspected if young p...To emphasize the early diagnosis and treatment of anti-N-methyl-d-aspartate-receptor (NMDAR) autoimmune encephalitis, a rare clinical condition, teratoma-related, anti-NMDAR encephalitis should be suspected if young patients present with psychiatric, movement, and sensory symptoms. Early diagnosis and treatment can decrease the mortality and disability rate.展开更多
Autoimmune limbic encephalitis is most commonly associated with antibodies against the N‑methyl‑D‑aspartate receptor(NMDAR),among other neuronal cell surface receptors.Here,a case of a pregnant female with limbic ence...Autoimmune limbic encephalitis is most commonly associated with antibodies against the N‑methyl‑D‑aspartate receptor(NMDAR),among other neuronal cell surface receptors.Here,a case of a pregnant female with limbic encephalitis in the presence of multiple additional autoimmune antibodies is described.The patient was a 36‑year‑old female who presented with 4 days of confusion,hallucinations,hypersexuality,disinhibition,and pressured speech.The patient’s work‑up detected the presence of anti‑NMDAR antibodies,anti‑glutamic acid decarboxylase antibodies,and a yet uncharacterized neuronal autoantibody.The patient was also found to be pregnant.No evidence of ovarian or other pelvic malignancy was discovered.Symptomatic control was achieved with plasma exchange.展开更多
Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a c...Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a case of anti-GABA-B receptor encephalitis in a 57-year-old man who presented with seizures,memory loss,and abnormal behavior.He developed partially neurological responses to immunotherapy,but refused comprehensive tumor screening.The symptoms were aggravated again 4 months later.Workup showed antibodies to GABA-B receptors and tumor screening revealed SCLC.It highlights the importance of early screening of underlying tumor and anti-tumor treatment in paraneoplastic cases.展开更多
DDeeaarr EEddiittoorr,,The encoding and retrieval of emotional memories demands intricate interplay within the limbic network,where the network state is subject to significant reconfiguration by learning-induced plast...DDeeaarr EEddiittoorr,,The encoding and retrieval of emotional memories demands intricate interplay within the limbic network,where the network state is subject to significant reconfiguration by learning-induced plasticity,behavioral state,and contextual information[1].展开更多
文摘BACKGROUND Paraneoplastic limbic encephalitis(LE)is an inflammatory condition that affects the limbic system,cerebellum,and peripheral nervous system.It causes a range of symptoms including short-term memory loss,impaired cognitive function,behavioral and psychological disorders,and seizures.Paraneoplastic LE can occur when an immune response is activated due to antibodies targeting gammaaminobutyric acid(GABA)B receptor(GABABR)interacting with antigens on tumor cells and the nervous system,resulting in tumors primarily as small cell lung carcinoma(SCLC).CASE SUMMARY We discuss two cases of GABABR antibody-related LE resulting from SCLC.The patients’symptoms were managed with immunotherapy but ended in premature death due to chemotherapy-related complications.CONCLUSION Paraneoplastic syndrome is a notable cause of LE.Early intravenous immunoglobulin therapy may lead to temporary remission.
文摘Limbic encephalitis represents a cluster of autoimmune disorders, with inflammation in the medial temporal lobe characterised by the subacute onset of neuropsychiatric symptoms such as anxiety, affective symptoms, psychosis, short-term memory impairment, as well as faciobrachial and grand mal seizures. The limbic system is a complex anatomical structure which this paper seeks to explain in terms of its anatomy and physiology, before exploring what happens when it is impaired as is the case of autoimmune and limbic encephalitis. We will discuss the pathophysiology, clinical symptomatology and diagnosis of autoimmune encephalitis, a cluster of symptoms which can be easily overlooked or misdiagnosed within psychiatric settings. Characteristic indicators of autoimmune encephalitis include neurologic symptoms such as facial twitching, seizures, confusion, and cognitive decline;however, our experience realises that autoimmune encephalitis is not easy to identify as most patients initially present with psychiatric symptomatology rather than these neurological symptoms. Furthermore, immunological and laboratory testing take a long time to diagnose the condition. Importantly, few psychiatrists consider the autoimmune nature of the neuropsychiatric presentation. It is hence vital to consider autoimmune encephalitis in all patients with atypical presentations.
文摘BACKGROUND： The biochemical hypothesis of dopamine hyperfunction in the brain can explain the pathological mechanisms of schizophrenia. Surgery is performed based on limbic system circuit theory correspondence to above-mentioned hypothesis. Stereotactic surgery for the treatment of mental disorders is related to stereotactic surgery that influences the Papez circuit. OBJECTIVE： To observe the effects of stereotactic multi-target limbic leucotomy on the improvements in memory, intelligence and psychiatric symptoms in the treatment of intractable psychiatric disease. DESIGN： Self-control case analysis and follow-up of therapeutic effects. SETTING： Department of Neurosurgery, First Hospital, Hebei Medical University. PARTICIPANTS： Thirty patients with intractable psychiatric disease, who received stereotactic surgery in the Department of Neurosurgery, First Hospital, Hebei Medical University between July 2002 and August 2005, were included in this study. The patients, 21 males and 9 females, all met the diagnostic criteria of intractable psychiatric disease, determined by the national psychosurgery cooperation team in 1998. Informed consents for surgery and clinical follow-up exams were obtained from patients and/or patients＇ relatives （guardians）. METHODS： In 30 patients with intractable psychiatric disease, limbic leucotomy was performed by stereotactic technique. Multi-target radiofrequency hyperthermia was performed in the intracranial amygdaloid nucleus, anterior limb of internal capsule, callosal gyrus, among other regions. The therapeutic effects of patients were evaluated by Brief Psychiatric Rating Scale （BPRS） before surgery, and 6 months, 1, and 3 years after surgery. The Wechsler Adult Intelligence Scale （WAIS） and Clinical Memory Scale （CMS） were used to assess memory and intelligence before and after surgery. MAIN OUTCOME MEASURES： Memory, intelligence, and psychiatric symptoms of patients before and after operation. RESULTS： Thirty patients were included in the final analysis. （1） Memory and intelligence changes of patients： CMS and WAIS showed there were no significant differences in the changes of brain functions before and after operation （P 〉 0.05）. （2） Improved degree of symptoms： 5 patients were cured, 8 patients significantly improved, 8 patients improved, 3 patients had no changes, and 6 patients had recurrent symptoms. CONCLUSION： Stereotactic multi-target limbic leucotomy can improve the symptoms of patients with intractable psychiatric disease. Follow-up results showed that memory and intelligence were not noticeably improved after surgery.
基金the National Natural Science Foundation of China,No.30800263
文摘BACKGROUND： Limbic encephalitis is a rare syndrome that specifically affects the limbic system. Magnetic resonance imaging （MRI） has been typically used to detect brain changes in this disease. However, the mechanisms of limbic encephalitis-related white matter damage remain poorly understood. OBJECTIVE： To characterize white matter connectivity changes secondary to injuries of the limbic system in limbic encephalitis through combined application of diffusion tensor imaging （DTI） and voxel-based morphometry. DESIGN, TIME AND SETTING： A non-randomized, controlled, clinical, neuroimaging, DTI study was performed at the Department of Radiology, West China Hospital in December 2008. PARTICIPANTS： A male, 46-year-old, limbic encephalitis patient, as well as 11 age- and gender-matched healthy volunteers, were enrolled in the present study. METHODS： MRI was performed on the limbic encephalitis patient using a 3.0T MR scanner. Three-dimensional SPGR Tl-weighted images and DTI were acquired in the patient and controls. Data were analyzed using Matlab 7.0 and SPM2 software. MAIN OUTCOME MEASURES： Results from routine MRI scan with contrast enhancement of patient, as well as fractional anisotropy and mean diffusivity value map differences between patient and controls. RESULTS： Significant symmetric MRI signal intensity abnormalities were observed with routine MRI Affected bilateral hippocampi and amygdala exhibited hypointense signals in TIWI and hyperintense signals in T2 images. The DTI study revealed decreased fractional anisotropy values in the bilateral alveus and fimbria of the hippocampus, bilateral internal and external capsules, white matter of the right prefrontal area, and left corona radiate in the patient compared with normal controls （P 〈 0.001） Significantly increased fractional anisotropy, mean diffusivity, or decreased mean diffusivity were not observed in the patient, compared with controls. CONCLUSION： Secondary white matter damage to the hippocampal afveus and fimbria was apparent in the limbic encephalitis patient. In addition, other white matter fiber injuries surrounded the limbic structures, which were not attributed to secondary limbic system injuries.
文摘This case study addresses episodic memory impairment or focal retrograde amnesia related to limbic encephalitis. The patient in question was a right-handed man in his 60s who developed focal retrograde amnesia due to limbic encephalitis. An abnormal MRI signal was observed in the right medial temporal lobe. Immediately after the encephalitis onset, mental symptoms—including impaired consciousness, disorientation, hallucinations, and delusions—appeared;however, his condition improved with medical treatment. After transfer to a convalescent and rehabilitation hospital, various neuropsychological tests were conducted but no apparent functional decline was observed in intellectual function, attention, memorization, or retention. However, the patient exhibited impairments related to autobiographical memory and memory of events 4?-?5 years before the onset of limbic encephalitis. Based on these observations, the patient was considered to exhibit focal retrograde amnesia due to limbic encephalitis. Despite exhibiting retrograde amnesia, he had no difficulty in his daily life in the ward. Moreover, after discharge from hospital, smooth work reinstatement was possible. This case demonstrates that, even after exhibiting retrograde amnesia, if its duration is relatively short and physical function and other higher-order brain functions are sustained, social rehabilitation—including work reinstatement—is feasible.
文摘BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.
文摘Limbic encephalitis (LE) is a clinical entity related to a mesial temporal lesion resulting in a combination of anterograde memory dysfunction, behavioral changes, and seizures. The most common causes of limbic encephalitis are Herpes Simplex Virus (HSV) encephalitis, autoimmune encephalitis. Neurosyphilis is an exceptional aetiology. The early diagnosis and management of the disease determine the prognosis. This clinical course highlights the diagnostic challenge limbic encephalitis can cause and the importance of considering neurosyphilis in patients with specific or unspecific neurological symptoms. We report a case of a mesiotemporal form of neurosyphilis revealed by a LE.
文摘To investigate the function of matrix metalloproteinase-2(MMP-2),matrix metalloproteinase-9(MMP-9) and tissue inhibitors of metalloproteinase-2(TIMP-2) in the invasion of gliomas of the limbic system.Methods The expression of MMP-2,MMP-9 and TIMP-2 in low and high-grade gliomas and meningiomas was detected by using immunohistochemical technique.Results (1) The expression levels of MMP-2 and MMP-9 were significantly higher in high grade gliomas than those in low grade gliomas (P<0.05 and P<0.01 respectively).They were also significantly higher in low grade gliomas than those in meninginomas (P<0.05 and P<0.01 respectively).(2) The TIMP-2 expression level was obviously lower in gliomas of grade Ⅲ and grade Ⅳthan that in gliomas of grade Ⅰ and grade Ⅱ.Also,the expression of TIMP-2 in gliomas of grade Ⅰ and grade Ⅱ was significantly lower than that in meningiomas. Conclusion (1) The expression of MMP-2 and MMP-9 in human brain gliomas is related to malignant progression of gliomas.They might be used as the indicators to evaluate the malignancy and prognosis of gliomas.(2) Imbalance between MMP-s and TIMP-s may be one of the improtant factors promoting glioma invasion.5 refs,3 figs.
文摘Background： Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor （GABABR） in patients with limbic encephalitis （LE） was first described in 2010. We present a series of klan Chinese patients tbr further clinical refinement. Methods： Serum and cerebrospinal fluid （CSF） samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed. Results： All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram （EEG） or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen （17/18） patients presented with new-onset refractory seizure or status epileptics. Twelve （12/18） patients had memory deficits, 11 （11/18） patients had personality change, 7 （7/18） patients had disturbance of consciousness, and 3 （3/18） patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 （6/18） patients. Ten （10/18） patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten （10/18） patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications. Conclusions： Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABABR antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.
基金Project supported by the National Natural Science Foundation of China.
文摘Kainic acid(KA),a prospective neuroexcitable and neurotoxic analog of glumate,inducespersistent seizure activity in limbic structures which results in behavioral symptoms,electrographic and neuropathologic alternations closely resembling human temporal lobe epi-lepsy.The hippocampal pyramidal cells possess abundant KA high affinity binding sites intheir membranes and are extremely vulnerable to KA neurotoxicity.NT-3 is a memberof the neurotrophic family(NTF)including nerve growth factor(NGF),brain derivedneurotrophic factor(BDNF),neurotrophin-4/5(NF-4/5).NT-3 mRNA was expressed
文摘Paraneoplastic neurologic syndromes （PNSs） occur with increased frequency in patients with cancer and almost always antedate its diagnosis.These syndromes comprise a heterogeneous group of cancer-related neurologic diseases,and they may affect any part of the nervous system.The simultaneous involvement of different areas of the nervous system by the paraneoplastic process is not unusual.Until date,this is the first report of concurrent development of paraneoplastic cerebellar degeneration （PCD） and paraneoplastic limbic encephalitis （PLE） associated with the advanced ovarian cancer and anti-Yo antibodies following hepatitis B （HB） vaccination.The cause of most PNS is believed to be an immune response against neuronal proteins expressed by the tumor.
基金This work was supported by 2021 Scientific Planning Project of Gansu Province(2021年甘肃省科技计划项目No.21JR1RA266)Project of National Natural Science Foundation of China(国家自然科学基金项目,No.81460744).
文摘Pain is a complex physiological and psychological activity,involving at least three dimensions,including pain sensation,pain emotion,and pain cognition.Acupuncture can clearly relieve the pain sensation of patients and improve pain emotion and pain cognition induced by pain;acupuncture participates in the multi-dimensional regulation of pain through brain regions of the limbic system such as anterior cingulate cortex(ACC),amygdala(AMY),and hippocampus.By analyzing relevant literature,it has been found that the regulation of acupuncture on pain emotion is mainly related to the activation of pertinent opioid receptors in the ACC,the decrease of the expression of extracellular signal-regulated kinase(ERK),and the promotion of the expression of glutamic acid(Glu)A1,metabotropic glutamate receptor-1(mGluR1),andγ-aminobutyric acid aminobutyric acid(GABA)B2 protein in the AMY.The regulation of acupuncture on pain cognition is mainly related to the elevation of the expression of protein kinase A(PKA)and phospho-p38 mitogen-activated protein kinase(phospho-p38 MAPK)and the inhibition of cyclic adenosine monophosphate(cAMP)/PKA/cAMP response element-binding protein(CREB)signaling pathway in the ACC.
文摘Paraneoplastic limbic encephalitis (PLE) is a rare immunopathological syndrome, reported in association with certain types of malignancies. Patients present with cognitive and memory impairments, disordered perception, mood and behavioral changes, sleep disturbances, and seizures. Despite the growing number of cases being reported, it still poses a diagnostic challenge. We encountered a patient with a myriad of neuropsychiatric symptoms who exhibited a highly variable response to therapy. A 36-year-old male presented with memory impairment, excessive sleepiness, and slurred speech. Brain magnetic resonance imaging revealed hyperintensities in the temporal lobes and hypothalamus, all suggestive of limbic encephalitis. He was found to have a mixed germ cell testicular teratoma. Screening for commonly associated antibodies did not yield positive results, which emphasizes that sero-negative PLE can be missed in patients with malignancies. In reporting this case, we urge neurologists to consider PLE as part of the differential diagnosis in similar ambiguous clinical scenarios.
文摘To emphasize the early diagnosis and treatment of anti-N-methyl-d-aspartate-receptor (NMDAR) autoimmune encephalitis, a rare clinical condition, teratoma-related, anti-NMDAR encephalitis should be suspected if young patients present with psychiatric, movement, and sensory symptoms. Early diagnosis and treatment can decrease the mortality and disability rate.
文摘Autoimmune limbic encephalitis is most commonly associated with antibodies against the N‑methyl‑D‑aspartate receptor(NMDAR),among other neuronal cell surface receptors.Here,a case of a pregnant female with limbic encephalitis in the presence of multiple additional autoimmune antibodies is described.The patient was a 36‑year‑old female who presented with 4 days of confusion,hallucinations,hypersexuality,disinhibition,and pressured speech.The patient’s work‑up detected the presence of anti‑NMDAR antibodies,anti‑glutamic acid decarboxylase antibodies,and a yet uncharacterized neuronal autoantibody.The patient was also found to be pregnant.No evidence of ovarian or other pelvic malignancy was discovered.Symptomatic control was achieved with plasma exchange.
文摘Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a case of anti-GABA-B receptor encephalitis in a 57-year-old man who presented with seizures,memory loss,and abnormal behavior.He developed partially neurological responses to immunotherapy,but refused comprehensive tumor screening.The symptoms were aggravated again 4 months later.Workup showed antibodies to GABA-B receptors and tumor screening revealed SCLC.It highlights the importance of early screening of underlying tumor and anti-tumor treatment in paraneoplastic cases.
基金supported by the National Natural Science Foundation of China(T2394531)the National Key R&D Program of China(2024YFF1206500)+1 种基金the Shanghai Municipal Science and Technology Major Project(2018SHZDZX01)ZJ Lab,and the Shanghai Center for Brain Science and Brain-Inspired Technology,China.
文摘DDeeaarr EEddiittoorr,,The encoding and retrieval of emotional memories demands intricate interplay within the limbic network,where the network state is subject to significant reconfiguration by learning-induced plasticity,behavioral state,and contextual information[1].
