Recurrent Anti-AMPA Receptor Limbic Encephalitis: A Case Report and Literature Review

Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor encephalitis is a relatively rare anti-neuronal surface antigen autoimmune encephalitis (LE). We described a case of a 47-year-old Chinese man having anti-AMPA receptor limbic encephalitis initially presented with cognitive decline, undetectable antibodies, and normal imaging findings in magnetic resonance image (MRI) and then developed into typical autoimmune limbic encephalitis a few months later with a course of multiple relapses. In addition, we found progressive brain atrophy in our case, which was a rare presentation of LE. This report also summarized the characteristics of nine reported cases of anti-AMPA receptor limbic encephalitis with relapse up to date. This case highlighted that autoimmune limbic encephalitis is an important differential diagnosis for patients with typical symptoms even when the MRI and antibodies are normal, and more attention should be paid to the relapse of anti-AMPA receptor encephalitis.

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Autoimmune Limbic Encephalitis: PET Imaging Findings of Two Distinct Metabolic Patterns (P03.152)

Neurology ◽

10.1212/wnl.78.1_meetingabstracts.p03.152 ◽

2012 ◽

Vol 78 (Meeting Abstracts 1) ◽

pp. P03.152-P03.152

Author(s):

E. Lai ◽

P. Schulz ◽

N. Patel ◽

R. Fisher

Keyword(s):

Pet Imaging ◽

Limbic Encephalitis ◽

Imaging Findings ◽

Autoimmune Limbic Encephalitis

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Autoimmune Limbic Encephalitis in a Patient with Acute Encephalopathy and Hyponatremia

Case Reports in Medicine ◽

10.1155/2019/9051738 ◽

2019 ◽

Vol 2019 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

K. A. Agarwal ◽

M. Albertolle ◽

M. Tiru

Keyword(s):

Hospital Admissions ◽

Limbic Encephalitis ◽

Autoimmune Encephalitis ◽

Developed Countries ◽

Acute Encephalopathy ◽

Neurological Outcomes ◽

Common Clinical Presentation ◽

Autoimmune Limbic Encephalitis ◽

The Developed Countries ◽

Mri Changes

Acute encephalopathy is a common clinical presentation for hospital admissions. Autoimmune encephalitis is a rare cause of encephalopathy which has increasingly been recognized over the last decade. The detection of various neuronal antibodies has helped diagnose these syndromes, but they have limited availability, mostly in the developed countries. We present a case of a middle-aged female presenting with memory impairment, gait disturbances, and hyponatremia. A clinical diagnosis of autoimmune limbic encephalitis was made based on faciobrachial dystonic seizures, SIADH, and MRI changes 10 days prior to autoantibody titer returned. Prompt treatment with steroids and intravenous immunoglobulin was started with improvement in her neurological symptoms. This case highlights the importance of considering autoimmune encephalitis syndromes in the differential diagnosis of patients with classical neurological presentations and prompt diagnosis and immunotherapy to improve neurological outcomes.

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Micronodular pattern organising pneumonia mimicking miliary lung disease: a rare radiological presentation

BMJ Case Reports ◽

10.1136/bcr-2020-239856 ◽

2021 ◽

Vol 14 (2) ◽

pp. e239856

Author(s):

David Ng ◽

Garun Hamilton ◽

Eric Hu ◽

Kenneth Lau

Keyword(s):

Differential Diagnosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Hypersensitivity Pneumonitis ◽

Imaging Findings ◽

Review Of The Literature ◽

Radiological Findings ◽

Rare Presentation

Organising pneumonia (OP) is an interstitial lung disease characterised by granulation tissues in alveoli and alveolar ducts. Typical imaging findings are migratory airspace opacities with peripheral or peribronchovascular distribution. Diffuse micronodular OP (MNOP) is a rare imaging manifestation, which has imaging differential diagnosis of endobronchial infection such as tuberculosis, hypersensitivity pneumonitis and respiratory bronchiolitis. Although clinical and ancillary radiological findings may aid in refining the differential diagnosis, histopathological assessment is frequently required for this rare presentation due to implications of treatment and prognosis. We report a case of MNOP and performed a review of the literature.

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Use of intravenous immunoglobulin for the treatment of autoimmune encephalitis: audit of the NHS experience

JRSM Open ◽

10.1177/2054270418793021 ◽

2018 ◽

Vol 9 (9) ◽

pp. 205427041879302 ◽

Cited By ~ 2

Author(s):

JA Kinsella ◽

SR Irani ◽

R Hollingsworth ◽

D O’Shaughnessy ◽

P Kane ◽

...

Keyword(s):

Intravenous Immunoglobulin ◽

Limbic Encephalitis ◽

Tertiary Care ◽

Case Series ◽

Autoimmune Encephalitis ◽

Symptomatic Treatment ◽

Outcome Data ◽

National Database ◽

Retrospective Audit ◽

Autoimmune Limbic Encephalitis

Objectives The treatments of limbic and other autoimmune encephalitis include immunosuppression, symptomatic treatment, and in the case of paraneoplastic syndromes, appropriate therapy for underlying neoplasms. When immunotherapy is considered, intravenous immunoglobulin is one option for treatment, either alone or in combination with corticosteroids. To date, however, evidence for the use of intravenous immunoglobulin in this context comes from case series/expert reviews as no controlled trials have been performed. We aimed to analyse the NHS England Database of intravenous immunoglobulin usage, which was designed to log use and guide procurement, to explore usage and therapeutic effect of intravenous immunoglobulin in autoimmune encephalitis in England. Design We conducted a retrospective audit and review of the NHS England Database on intravenous immunoglobulin use. Setting NHS England Database of intravenous immunoglobulin use which covers secondary and tertiary care prescribing and use of intravenous immunoglobulin for all patients in hospitals in England. Participants Hospital in-patients with confirmed or suspected autoimmune/limbic encephalitis between September 2010 and January 2017. Results A total of 625 patients who were 18 years of age or older were treated with intravenous immunoglobulin for autoimmune encephalitis, of whom 398 were determined as having 'highly likely' or 'definite' autoimmune/limbic encephalitis. Ninety-six percent were treated with a single course of intravenous immunoglobulin. The availability and accuracy of reporting of outcomes was very poor, with complete data only available in 27% of all cases. Conclusions This is the first review of data from this unique national database. Whilst there was evidence for clinical improvement in many cases of patients treated with intravenous immunoglobulin, the quality of outcome data was generally inadequate. Methods to improve quality, accuracy and completeness of reporting are crucial to maximise the potential value of this resource as an auditing tool.

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Case Report: Anti-LGI1 Limbic Encephalitis Associated With Anti-thyroid Autoantibodies

Frontiers in Neurology ◽

10.3389/fneur.2020.620483 ◽

2021 ◽

Vol 11 ◽

Author(s):

Ricardo Otiniano-Sifuentes ◽

Anali Cuba Antezana ◽

Walter F. De La Cruz Ramirez ◽

Kevin Pacheco-Barrios ◽

Darwin A. Segura Chavez

Keyword(s):

Limbic Encephalitis ◽

Brain Magnetic Resonance Imaging ◽

Autoimmune Encephalitis ◽

Epileptic Seizures ◽

Clonic Seizure ◽

Thyroglobulin Antibodies ◽

Temporal Lobes ◽

Important Differential Diagnosis ◽

The Right ◽

The Brain

Anti-LGI1 encephalitis is an autoimmune encephalitis with antibodies against leucine-rich glioma-inactivated 1 (LGI1), first described in 2010. It is a non-frequent and poorly understood entity that represents the second most frequent cause of autoimmune encephalitis. This entity is characterized by the presence of limbic encephalitis, hyponatremia, and faciobrachial dystonic seizures. Herein, we present the case of a male patient with an onset of epileptic seizures (generalized tonic-clonic seizure), and involuntary dystonic movements that affect the right side of his face and right upper limb associated with mental disorder, and affectation of higher functions. The electroencephalogram showed continuous generalized slowing of the background activity. The brain magnetic resonance imaging showed signal hyperintensity at the level of both mesial temporal lobes and hippocampi and in the head of the right caudate nucleus. Anti-thyroglobulin antibodies were positive, and he was initially diagnosed as Hashimoto's encephalopathy (HE). However, the response to corticosteroids was not completed as it is usually observed in HE. For that, antibodies for autoimmune encephalitis were tested, and the anti-LGI1 antibodies were positive in serum and cerebrospinal fluid. HE is an important differential diagnosis to consider. Furthermore, the presence of Anti-thyroglobulin antibodies should not be taken as the definitive diagnostic criteria, since these antibodies could be associated with other autoimmune encephalopathies, which include in addition to anti-LGI1, anti-NMDA and anti-Caspr2.

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True aneurysm of the digital artery: a case report and systematic literature review

Journal of Surgical Case Reports ◽

10.1093/jscr/rjz400 ◽

2020 ◽

Vol 2020 (2) ◽

Author(s):

Zara Sheikh ◽

Sadasivam Selvakumar ◽

Patrick Goon

Keyword(s):

Differential Diagnosis ◽

Case Report ◽

Artery Aneurysm ◽

The Body ◽

Digital Artery ◽

True Aneurysm ◽

Rare Presentation ◽

Important Differential Diagnosis ◽

The Common ◽

Successful Repair

Abstract Aneurysmal disease can occur in any vessel in the body and occur most commonly the aorta, cerebral and popliteal arteries; however, aneurysms of the digital artery remain a rare presentation. They form an important differential diagnosis in any patient presenting with a mass in the hand. This report presents the case of a 64-year-old man with a true aneurysm of the common palmar digital artery who underwent successful repair, following excision and end to end anastomosis. Only 21 cases of true digital artery aneurysm have been reported; we review the literature pertaining to the diagnosis and management of digital artery aneurysms since they were first described by Baruch et al in 1977.

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Tentorial Dural Arteriovenous Fistulas as a Cause of Thalamic Edema: 2 Cases of an Important Differential Diagnosis to Consider

The Neurohospitalist ◽

10.1177/1941874420944333 ◽

2020 ◽

Vol 11 (1) ◽

pp. 33-39

Author(s):

Mougnyan Cox ◽

Pavel Rodriguez ◽

Suyash Mohan ◽

Neda I. Sedora-Roman ◽

Bryan Pukenas ◽

...

Keyword(s):

Differential Diagnosis ◽

Delayed Diagnosis ◽

Imaging Findings ◽

Imaging Diagnosis ◽

Cross Sectional ◽

Arteriovenous Fistulas ◽

Important Differential Diagnosis ◽

Dural Arteriovenous Fistulas ◽

Cross Sectional Imaging ◽

Sectional Imaging

The differential diagnosis for bilateral thalamic edema is extensive and includes vascular, neoplastic, metabolic, and infectious causes. Of the vascular causes of thalamic edema, arterial and venous infarctions are well-documented, but dural arteriovenous fistulas (dAVFs) are a relatively uncommon and widely underrecognized cause of thalamic edema. Dural AVFs are notoriously difficult to diagnose clinically, especially in the absence of hemorrhage, and cross-sectional imaging findings can be subtle. This can result in a delayed diagnosis, and occasionally, an invasive biopsy for further clarification of a purely vascular disease. In this review, we detail our experience with the imaging diagnosis of dAVF as a cause of thalamic edema and present a short differential of other vascular causes.

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Autoimmune Encephalitis is Recognised as an Important Differential Diagnosis in a Kenyan Tertiary Referral Centre

BMJ Military Health ◽

10.1136/jramc-2019-001338 ◽

2020 ◽

Vol 166 (5) ◽

pp. 358-358

Author(s):

Dilraj Singh Sokhi ◽

Onkar Singh Bhogal

Keyword(s):

Differential Diagnosis ◽

Autoimmune Encephalitis ◽

Referral Centre ◽

Tertiary Referral ◽

Tertiary Referral Centre ◽

Important Differential Diagnosis

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Atypical Association of Autoimmune Limbic Encephalitis with Anti-NMDA Receptor Antibodies in a Young Male Patient: Clinical, Imaging, and Neuropsychological Characteristics

Case Reports in Neurology ◽

10.1159/000518195 ◽

2021 ◽

pp. 541-548

Author(s):

José Alberto Choreño-Parra ◽

Tania de la Rosa-Arredondo ◽

André Garibay-Gracián ◽

Ingrid Montes de Oca-Vargas ◽

Deyanira Capi-Casillas ◽

...

Keyword(s):

Nmda Receptor ◽

Limbic Encephalitis ◽

Psychiatric Symptoms ◽

Brain Mri ◽

Clinical Manifestations ◽

Memory Loss ◽

Autoimmune Encephalitis ◽

Young Male ◽

Autoimmune Limbic Encephalitis ◽

Receptor Antibodies

The spectrum of autoimmune encephalitis (AE) encompasses several entities characterized by a variable frequency of psychiatric symptoms, cognitive dysfunction, focal deficits, and seizures. Although patients with AE can be categorized in specific syndromes, overlapping manifestations are also common. Furthermore, atypical correlations between clinical phenotypes and autoantibody profiles could occur in rare cases. Here, we report the rare case of a young adult man attending due to new-onset seizures and a history of memory loss, autonomic disturbances, headache, behavioral changes, and visual and olfactory hallucinations. The patient was subjected to a complete diagnostic approach that included a comprehensive laboratory workup, neuropsychological testing, electroencephalogram, cerebrospinal fluid (CSF) analysis, brain MRI, and positron emission tomography/computed tomography scan that revealed a functional and structural compromise of the bilateral medial temporal lobes. Together with the clinical manifestations of the patient, these findings were compatible with the diagnosis of autoimmune limbic encephalitis (ALE). Strikingly, further analysis of the CSF showed autoantibodies against the N-methyl-D-aspartate (NMDA) receptor. We found very few cases of the co-occurrence of anti-NMDA receptor antibodies and nonparaneoplastic ALE in the literature, especially in male patients. Our report exemplifies the complicated differential diagnosis of ALE and adds clinical information of the association with anti-NMDA receptor antibodies.

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