Anti‐LGI1, anti‐GABABR, and Anti‐CASPR2 encephalitides in Asia: A systematic review

Ghimire, Prinska; Khanal, Ujjwal Prakash; Gajurel, Bikram Prasad; Karn, Ragesh; Rajbhandari, Reema; Paudel, Shishir; Gautam, Niraj; Ojha, Rajeev

doi:10.1002/brb3.1793

Cited by 35 publications

(32 citation statements)

References 79 publications

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“…In our study, male patients accounted for a larger proportion, and they were older than females when initial symptoms appeared (median at 44 vs 38 years old). This result is similar to those of other studies in Asia ( 21 ). As known, autoimmune encephalitis is characterized by acute or subacute onset.…”

Section: Discussionsupporting

confidence: 93%

“…Our patients had a median course of 17 days before admission, which might imply that CASPR2-associated encephalitis has a relatively short course of progression. However, we did notice that patients enrolled in our study went to the hospital sooner than those in other reports ( 21 , 22 ).…”

Section: Discussioncontrasting

confidence: 70%

“…Antiepileptic drugs help control seizures but allowed for relatively easy relapse in our study and others (31). Additionally, tumors usually present as a comorbid to autoimmune encephalitis, mostly in anti-GABAB encephalitis (21). In our study, only one patient (1/25) had a lung tumor, which was confirmed by imaging.…”

Section: Discussionsupporting

confidence: 68%

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Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

et al. 2021

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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.

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Section: Discussionsupporting

confidence: 93%

Section: Discussioncontrasting

confidence: 70%

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Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

et al. 2021

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“…The most common antibodies affecting the limbic system are antibodies against LGI1, GAD, GABA-BR, AMPAR, CASPR2, Ma2, and Hu [ 11 , 25 ]. A latest systemic review comprising 24 studies with 263 Asian patients with LE showed that 53% had LGI1, 43% had GABA-BR, and 4% had CASPR2 antibodies [ 26 ]. About 7% of patients with LE were seronegative [ 11 ].…”

Section: Antibodiesmentioning

confidence: 99%

“…The general mortality rate of LE was 9.7%, largely due to pulmonary infection or status epilepticus. GABA-BR antibody was attributed to the highest mortality rate (23.2%), largely due to tumor progression [ 26 ]. Cognitive dysfunction is a major complication in patients with long-term LE, and some patients are left disabled [ 56 ].…”

Section: Prognosismentioning

confidence: 99%

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

Kao

Lin²,

Weng

et al. 2020

IJMS

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Limbic encephalitis (LE) is a rare cause of encephalitis presenting as an acute and subacute onset of neuropsychiatric manifestations, particularly with memory deficits and confusion as core features, along with seizure occurrence, movement disorders, or autonomic dysfunctions. LE is caused by neuronal antibodies targeting the cellular surface, synaptic, and intracellular antigens, which alter the synaptic transmission, especially in the limbic area. Immunologic mechanisms involve antibodies, complements, or T-cell-mediated immune responses in different degree according to different autoantibodies. Sensitive cerebrospinal fluid markers of LE are unavailable, and radiographic findings may not reveal a typical mesiotemporal involvement at neurologic presentations; therefore, a high clinical index of suspicions is pivotal, and a neuronal antibody testing is necessary to make early diagnosis. Some patients have concomitant tumors, causing paraneoplastic LE; therefore, tumor survey and treatment are required in addition to immunotherapy. In this study, a review on the molecular and immunologic aspects of LE was conducted to gain awareness of its peculiarity, which we found quite different from our knowledge on traditional psychiatric illness.

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Autoimmune encephalitis: what the radiologist needs to know

Sanvito,

Pichiecchio,

Paoletti

et al. 2024

Neuroradiology

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Autoimmune encephalitis is a relatively novel nosological entity characterized by an immune-mediated damage of the central nervous system. While originally described as a paraneoplastic inflammatory phenomenon affecting limbic structures, numerous instances of non-paraneoplastic pathogenesis, as well as extra-limbic involvement, have been characterized. Given the wide spectrum of insidious clinical presentations ranging from cognitive impairment to psychiatric symptoms or seizures, it is crucial to raise awareness about this disease category. In fact, an early diagnosis can be dramatically beneficial for the prognosis both to achieve an early therapeutic intervention and to detect a potential underlying malignancy. In this scenario, the radiologist can be the first to pose the hypothesis of autoimmune encephalitis and refer the patient to a comprehensive diagnostic work-up – including clinical, serological, and neurophysiological assessments.In this article, we illustrate the main radiological characteristics of autoimmune encephalitis and its subtypes, including the typical limbic presentation, the features of extra-limbic involvement, and also peculiar imaging findings. In addition, we review the most relevant alternative diagnoses that should be considered, ranging from other encephalitides to neoplasms, vascular conditions, and post-seizure alterations. Finally, we discuss the most appropriate imaging diagnostic work-up, also proposing a suggested MRI protocol.

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Anti‐LGI1, anti‐GABABR, and Anti‐CASPR2 encephalitides in Asia: A systematic review

Abstract: This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Cited by 35 publications

References 79 publications

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

Autoimmune encephalitis: what the radiologist needs to know

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