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Delirium
Published in Henry J. Woodford, Essential Geriatrics, 2022
Non-infectious limbic encephalitis has an estimated incidence of around 5–10 per 100,000 per year.44 The average age of onset is around 45–62 years, but it can sometimes occur in people aged over 70.47–49 It has a neuropsychiatric presentation: confusion (memory loss), seizures (faciobrachial dystonic), altered sleep, changes in personality, anxiety, depression and/or hallucinations. Hyponatraemia may be present. The onset is subacute (typically over two to six weeks). Brain MRI is abnormal in around 80% of cases, e.g. medial temporal hyperintensities.49 EEG testing may show focal or diffuse slow waves, possibly with foci of epileptic activity.
Oncological effects on the central nervous system
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Magnetic resonance imaging (MRI) of the brain (and often spinal cord) and CSF analysis are required. The MRI findings are variable and changes may only be present in up to 58% of cases with limbic encephalitis (3). The typical features of a limbic encephalitis on MRI (both autoimmune and paraneoplastic limbic encephalitis) are those of increased T2/FLAIR signal abnormality and swelling in the hippocampal structures, best seen on coronal sequences (Figure 37.1). Enhancement may be absent or faint; a minority will show transient restricted diffusion, and a significant proportion may develop mesial temporal sclerosis as a long-term sequela (5).
Autoimmune disorders that can be mistaken for viral illness
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Maxwell Greene, Eric Lancaster
Autoimmune causes of encephalitis should enter into the differential of viral infections of the CNS. There are common presentations of autoimmune encephalitis, including limbic encephalitis, rhombencephalitis, cerebellitis, or seizures. Specific antibodies are related to different clinical phenotypes, and may carry with them different risks (depending on the particular antibody) of an occult malignancy. Autoimmune encephalitis is commonly paraneoplastic, and depending on the likelihood of cancer with the specific antibody, clinical syndrome, or patient demographics, an aggressive search for malignancy is usually necessary, and if one is not initially found, tumor surveillance should continue at intervals over time. There are particular subtleties in laboratory evaluations, and in particular low titers, should be interpreted in the overall context of the clinical scenario. Treatment for autoimmune encephalitis can involve first- and second-line therapy, from corticosteroids, IVIg, and plasmapheresis, to rituximab or cyclophosphamide, and different risks and benefits are associated with each. Relapses or worsening of symptoms should warrant further treatment and a search for tumor recurrence if appropriate. Symptomatic and behavioral treatment is often necessary in conjunction with the treatment of the autoimmune disease.
Clinical features of patients with anti-leucine-rich glioma inactivated-1 protein associated encephalitis: a Chinese case series
Published in International Journal of Neuroscience, 2019
Xue Yang, An-Ning Li, Xiu-He Zhao, Xue-Wu Liu, Sheng-Jun Wang
Leucine-rich glioma inactivated 1 (LGI1), which interacts with presynaptic disintegrin and metalloproteinase domain-containing protein 23 (ADAM23) and postsynaptic ADAM22, is a secreted protein associated with voltage-gated potassium channels (VGKC) [1]. Autoantibodies against LGI1 have been proved to be associated with limbic encephalitis (LE), faciobrachial dystonic seizures (FBDS) and epileptic seizures [2]. Limbic encephalitis is characterized by subacute onset of memory loss, consciousness disturbance, psychiatric symptoms and seizures [3]. Excluding infectious etiology, autoimmune mechanisms account for the majority of the pathogenesis of LE [2]. Antibodies against various antigens, such as VGKC complex, γ-aminobutyric acid-B receptor (GABABR) and α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptor (AMPAR), have been identified in autoimmune LE [4–9]. The autoimmune encephalitis associated with LGI1 antibody has been identified to be infrequent concurrent with tumors and often show a good response to immunotherapy.
Plasmablasts and neuroimmunological disorders
Published in Immunological Medicine, 2019
Norio Chihara, Riki Matsumoto, Takashi Yamamura
Another group of the disease is autoimmune limbic encephalitis. Autoantibodies against N-methyl-d-aspartate receptors (NMDARs) are reported to induce synaptic structural changes, which cause reversible loss of NMDARs, impair glutamate receptor signaling, and result in learning dysfunction, memory and other behavior observed in patients with anti-NMDAR encephalitis [8]. Recently, several other autoantibodies inhibitive to neurotransmitter receptors and related proteins in the central nervous system (CNS) have been reported to be associated with autoimmune limbic encephalitis, such as antibody against α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA), γ-aminobutyric acid (GABA), and leucine-rich glioma-inactivated 1 (LGI1) [9–11]. Within them, anti-GABAAR antibodies often cause extra-limbic cortical and subcortical lesions [9]. Of note, brain lesions show only mild nonspecific perivascular lymphocytes infiltration and reactive astrocytes [12]. The pathogenesis of these newly identified autoantibodies related to limbic encephalitis still needs to be investigated.
Retrospective study of paraneoplastic neurological syndromes in a Chinese Han population from Shandong, East China
Published in International Journal of Neuroscience, 2018
Shuai Miao, Shaohua Liao, Heng Li, Bing Niu, Huaiqiang Hu, Ying Qian, Hongwei Guo, Bingzhen Cao
Limbic encephalitis is a rare disorder characterized by short-term memory loss, seizures, cognitive decline and psychiatric symptoms [20]. The EEG characteristics of this disease are generalized/focal slow-wave abnormalities or epileptiform activity in the unilateral/bilateral temporal lobe [21,22]. The typical brain MRI associated with limbic encephalitis shows signal abnormalities in the limbic system, especially in the unilateral or bilateral mesial-temporal lobe, which can be found in approximately 57% of limbic encephalitis patients [20,21]. In our study, 5 patients exhibited generalized slow-wave EEG activity, but only two had abnormal MRI findings in T2/FLAIR images. All of the patients were negative for onconeural antibodies, while 3 were positive for anti-NMDAR antibodies. Patients with anti-NMDAR antibodies, which target cell surface antigens, develop more complex symptoms than patients with classical limbic encephalitis [23–25]. Recently, varieties of neural cell surface antibodies have been discovered, including antibodies against alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor [26], gamma-amino-butyric acid receptor [27] and leucine-rich glioma-inactivated-1 protein [28]. The relationships between these antibodies and paraneoplastic disorders are not yet clear and are the focus of ongoing research. The detection of these antibodies may be helpful in the diagnosis of limbic encephalitis.