Autoimmune limbic encephalitis can arise both by paraneoplastic and nonparaneoplastic mechanisms. Ffdg pet imaging has been reported to typically reveal medial temporal lobe hypermetabolism even in mrinegative or inconclusive cases, suggesting that it could be more. Voltagegated potassium channel vgkc encephalitis is one of the most common group 2 subtypes of autoimmune encephalitis, which can demonstrate classic features of limbic encephalitis but is primarily defined by the early and prominent development of medically intractable epilepsy. Our aim was to investigate the brain mr imaging characteristics of anti n methyldaspartate receptor encephalitis and their associations with clinical outcome at a 2year followup.
Limbic encephalitis in children and adolescents archives. Common causes are autoimmune disorders or idiopathic disease, although it has been associated to tumors. Diagnostic tools for immune causes of encephalitis read. Role of 18 ffdgpet imaging in the diagnosis of autoimmune. In two patients with limbic encephalitis serial magnetic resonance mr imaging showed evolution of abnormal highsignal intensity in both hippocampal formations on t2weighted images. What is limbic encephalitiscausessymptomstreatment. Brain mri characteristics of patients with antinmethyld. The incidence of autoimmune encephalitis has risen globally. Objective limbic encephalitis is rare in people limbic encephalitis tested for specific neuronal autoantibodies abs over 3. Limbic encephalitis genetic and rare diseases information center. Mr imaging of human herpesvirus6 encephalopathy after hematopoietic stem cell transplantation in adults. Additional clinical findings may allow further differentiation between these different types 11, 12. Included patients underwent a brain fdgpetct study and had possible or definite ae, including definite limbic encephalitis, per consensus criteria. By sarosh irani, associate professor and consultant neurologist, university of oxford and john radcliffe hospital, oxford, uk.
Brain scans such as magnetic resonance imaging mri or computerized tomography ct for diagnosing limbic encephalitis. No differences were detected in magnetic resonance imaging mri and csf profile. In general, headache, photophobia and seizures can be caused by viralbacterial encephalitis meningitis and space occupying lesions. Limbic encephalitis represents a group of autoimmune conditions characterized by inflammation of the limbic system and other parts of the brain.
Patterns of imaging abnormalities are described in cohorts of more common encephalitis aetiologies, such as hsv encephalitis. Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis nicholas d. Initial diagnosis and treatment are based on the clinical presentation as well as antibody profiles and mri. This study investigated the diagnostic value of integrated 18ffdgpet mri in the diagnostic workup of patients with le for a cerebral and wholebody imaging. Diagnosis international autoimmune encephalitis society.
Limbic encephalitis is a descriptor in the national library of medicines controlled vocabulary thesaurus, mesh medical subject headings. Herein, we describe the incidence and clinical and magnetic resonance imaging mri findings of rsvrelated encephalitis, a major neurological complication of rsv infection. Paraneoplastic limbic encephalitis ple, a paraneoplastic neurological syndrome pns, is a rare nervous system disorder that results from the indirect effects of tumors and is commonly associated with smallcell lung cancer sclc. Autoimmune encephalitis radiology reference article. Limbic encephalitis associated with human herpes virus 6 after stem cell transplant.
Paraneoplastic limbic encephalitis associated with lung cancer. The cardinal sign of limbic encephalitis is a severe impairment of shortterm memory. As the older term limbic encephalitis implies, the most common location of involvement is the mesial temporal lobes and limbic systems, typically manifested by cortical thickening and increased t2flair signal intensity of these regions. Bilateral involvement is most common 60%, although often asymmetric 8. Introduction limbic encephalitis le is an immunerelated, sometimes paraneoplastic process of the central nervous system. An approach to the diagnosis and management of limbic encephalitis is presented. Mri, magnetic resonance imaging, is a technique that uses a strong magnet to identify areas of the brain that may be overly active. In nmdar encephalitis brain mri is normal in up to 66% of cases, while the remaining patients may have unspecific cortical or subcortical flairt2 abnormalities, sometimes involving the posterior fossa or medial temporal regions, often with small areas of demyelination, and more rarely with extensive demyelinating abnormalities. Mri imaging reveals t2 hyperintensity in the structures of the medial temporal lobes, and in some cases, other limbic structures. Many patients with a specific type of ae called limbic encephalitis who receive an fdg pet scan have hypermetabolism in a part of the brain called the medial temporal lobe 5,6. The clinical presentation often includes disorientation, disinhibition, memory loss, seizures, and behavioral anomalies. Common causes of le are autoimmune disorders, among which paraneoplastic findings are frequent 1, 2.
Role of 18ffdgpet imaging in the diagnosis of autoimmune. These pet findings were associated with clinical symptoms and active disease status more strongly than the. Eeg leading to the diagnosis of limbic encephalitis. Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity. Mri abnormalities in the medial temporal lobes are helpful in confirming a diagnosis of limbic encephalitis, but normal imaging does not exclude the diagnosis. Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis author links open overlay panel nicholas d. Volumetry of mesiotemporal structures reflects serostatus in. Limbic encephalitis is characterized as a syndrome of acute to subacute onset with neuropsychiatric symptoms and medial temporal lobe abnormalities on mri andor eeg. Anti n methyldaspertate receptor encephalitis is an autoimmunemediated disease without specific brain mri features. The antigad antibodies typically cause a form of autoimmune encephalitis with classic temporal lobe lesions on mr imaging with the expected clinical findings of limbic encephalitis, andor additional features of stiff person syndrome with early and prominent development of seizures 6. The antigad antibodies typically cause a form of autoimmune encephalitis with classic temporal lobe lesions on mr imaging with the expected clinical findings of limbic encephalitis, andor additional features of stiff person syndrome with early and prominent development of seizures. Target proteins associated with classical limbic encephalitis are ampa, gabab, lgi1, and gad. Interestingly, it is thought to be as common as its infectious disease counterpart and can share some clinical, radiological, and.
Antibodies to the voltage gated potassium vgkc channel interfere with peripheral neuromuscular and ganglionic transmission. Early and chronic gray matter volume changes in limbic encephalitis. Herpes simplex encephalitis, limbic encephalitis, computed tomography, magnetic resonance imaging, mr spectroscopy introduction herpes simplex encephalitis hse is one of the most serious viral diseases of the central nervous system cns. Limbic encephalitis investigated by 18fdgpet and 3d mri. Pdf cerebral fdgpet and mri findings in autoimmune limbic. Antibodyassociated limbic encephalitis le is an increasingly. Respiratory syncytial virus rsv is a common pathogen causing acute respiratory infection in children. Limbic encephalitis le is characterized by a subacute onset of impaired cognitive function, temporal lobe epilepsy, and psychiatric symptoms. A normal mri lessens suspicion for cns vasculitis, demyelinating diseases, infections, and malignancies. Differences and diversity of autoimmune encephalitis in 77 cases. First, in limbic encephalitis and in other autoimmune encephalitis subtypes 18 ffdgpet imaging also shows, in a relevant number of patients, extra limbic metabolic abnormalities mainly in the brainstem, cerebellum, or cerebral cortex. Epilepsy, magnetic resonance imaging, voxelbased morphometry.
Table 2 antibodies, csf analyses, eegs, and imaging results in le patients. Eeg and magnetic resonance imaging abnormalities in. Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain. Stata statistical software, release 14 statacorp, college station, texas. The term limbic encephalitis le describes the condition when limbic areas of the brain are inflamed swollen and. When an autoantibody is found it is usually to either vgkc or nmdar. Limbic encephalitis is a subacute onset syndrome characterized by shortterm memory impairment, confusion, temporal lobe epilepsy, hypothalamic dysfunction, and psychiatric symptoms. Abnormal brain metabolism on fdgpetct is a common early. Limbic encephalitis investigated by 18fdgpet and 3d mri article in journal of neuroimaging 111. Some cases are associated with cancer and some are not. Unfortunately, there is considerable heterogeneity in how the term limbic encephalitis is used. Limbic s awardwinning debut title, towermadness launched in 2009, with a sequel in 2014. The limbic system is a predilection in autoimmune encephalitis and is the most consistently affected structure.
Spss software version 17 was used to describe and compare. Paraneoplastic limbic encephalitis ple is a rare autoimmune neurological. These methods for diagnosing limbic encephalitis help in ruling out the chances of aneurysms a bulge in a blood vessel, brain tumors and strokes and determine the part of brain affected by inflammation. Extrahippocampal involvement in human herpesvirus 6. Autoimmune encephalitis ae refers to a central nervous system cns antibodymediated entity characterized by a rapid onset behavioural and cognitive decline that can be associated with movement disorders, epileptic and dysautonomic features. Setting assessment, diagnosis, treatment and followup at 12 neuropaediatric and. Introduction limbic encephalitis le is an immunerelated. Brain mri is usually unremarkable 39,40, although there are a few case reports of hiv primary infections presenting as acute limbic encephalitis 41,42 or. Of 1047 patients with neurological symptoms, whose sera or csf were examined for paraneoplastic antibodies, 79 had the presumptive diagnosis of limbic encephalitis, dementia, cognitive dysfunction. Cerebral fdgpet and mri findings in autoimmune limbic encephalitis. New onset psychosis and limbic encephalitis the journal. Here, we hypothesized that patients with limbic encephalitis would show. Limbic encephalitis le is an immunerelated, sometimes. Clinical approach to the diagnosis of autoimmune encephalitis in the.
The first step is to obtain mri of the brain and csf examination. The neurological symptoms usually precede the detection of the primary cancer. Aisheng dong, yong cui, yang wang, changjing zuo, yushu bai. All statistical analyses were performed using spss 22. Diagnostic imaging and related sciences published by advanced search. The term limbic encephalitis le describes the condition when limbic areas of the brain are inflamed swollen and consequently not functioning properly. Previous studies have reported that magnetic resonance imaging mri may be useful for diagnosing le.
Two patients with clinically probable or possible limbic encephalitis le are reported, both cases with typical findings in clinical symptoms severe neuropsychological deficits and complex partial seizures and in routine magnetic resonance imaging mri hyperintense mesiotemporal lesions. Fdgpet in paraneoplastic limbic encephalitis springerlink. The fourth patient had multifocal paraneoplastic encephalitis associated with small cell lung cancer. Viral encephalitis of the immunocompromised patient hiv primary infection may present as acute encephalitis.
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