WED 148 Imaging findings in autoimmune encephalitis: a retrospective review

2018 ◽  
Vol 89 (10) ◽  
pp. A17.2-A17
Author(s):  
Fazal Jawad ◽  
Sadiq Nouman ◽  
Bowen Michael ◽  
Muhammad Wasim Wali ◽  
Sawlani Vijay ◽  
...  
Keyword(s):  
Retrospective Review ◽  
Autoimmune Encephalitis ◽  
Cerebral Atrophy ◽  
Mri Findings ◽  
Aspartate Receptor ◽  
High Clinical Suspicion ◽  
Improved Outcomes ◽  
Mesial Temporal Lobe ◽  
Intense Signal ◽  
Prompt Diagnosis

BackgroundNeuroimaging findings in autoimmune encephalitis may include normal MRI as well as limbic and extra-limbic involvement. Prompt recognition allows early immunosuppression and improved outcomes.MethodsRetrospective review of patients with Autoimmune encephalitis over the last 5 years.ResultsWe identified N-methyl-d-aspartate receptor (NMDAR) n=8, voltage gated potassium channel (VGKC) n=11, and leucine-rich glioma inactivated 1 (LGI1) n=11 encephalitis patients. 1/8 NMDAR encephalitis patients had abnormal MRI findings of T2 hyper intense signal in right anterior temporal and bilateral frontal and left insular lobe. DWI sequence showed restricted diffusion. 4/11 with VGKC encephalitis had positive MRI findings. Two patients had limbic involvement while one also had involvement of basal ganglia. Two patients had modest cerebral atrophy. Four patients had normal MRI findings. MRI scan was not available for three patients. 5/11 LGI1 encephalitis patients had bilateral mesial temporal lobe changes. In one patient, MRI could not be done due to permanent pacemaker. Five patients had normal MRI findings.ConclusionsIn our series, 33% patients had abnormal MRI findings consistent with a diagnosis of autoimmune encephalitis, which is lower than reported in literature. High clinical suspicion should lead to prompt diagnosis even in the absence of typical encephalitic changes on MRI.

Acute cerebral atrophy in autoimmune encephalitis complicated by haemophagocytic lymphohistiocytosis

2021 ◽  
Vol 14 (3) ◽  
pp. e240659
Author(s):  
Qian Wu ◽  
Shujuan Dai ◽  
Lin Zhu ◽  
Charlie Weige Zhao
Keyword(s):  
Case Report ◽  
Viral Encephalitis ◽  
Autoimmune Encephalitis ◽  
Cerebral Atrophy ◽  
Surface Antigens ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Specific Antibodies ◽  
Systemic Immune Activation ◽  
Aspartate Receptor ◽  
History Of

Autoimmune encephalitis is a disease characterised by neural-specific antibodies. This case report presents a 20-year-old young man with a recent history of suspected viral encephalitis who presented with recurrent fevers and episodes of confusion. He was found to have anti-N-methyl-D-aspartate receptor (NMDAR) and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 receptor (AMPAR1) positive autoantibodies and was diagnosed with autoimmune encephalitis. He subsequently developed global cerebral atrophy and was found to meet diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH). This patient’s presentation was consistent with existing literature showing that autoimmune encephalitis may develop after an initial viral meningoencephalitis. However, concurrent anti-NMDAR and anti-AMPAR1 positive autoimmune encephalitis has not been reported in literature to date, and this case report represents one instance of its presentation. We speculate that multiple antibodies against neural surface antigens may increase the risk for systemic immune activation leading to HLH and acute cerebral atrophy.

scholarly journals Catatonia in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Misdiagnosed as Schizophrenia

2020 ◽  
Vol 33 (3) ◽  
pp. 208
Author(s):  
André Ponte ◽  
Ana Brito ◽  
Camila Nóbrega ◽  
Sofia Pinheiro ◽  
João Gama Marques
Keyword(s):  
Psychotic Disorders ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Poor Response ◽  
Diagnostic Investigation ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor ◽  
Previous Diagnosis ◽  
The Central Nervous System ◽  
Prompt Diagnosis

Anti-N-Mmethyl-D-aspartate receptor encephalitis is an autoimmune disease of the central nervous system with prominent neurologic and psychiatric features. Symptoms appear progressively and sometimes with an exclusively psychiatric initial presentation. The patient’s evaluation should be meticulous, and we should use all the diagnostic tests required for the exclusion of entities that can mimic this disease. We report the diagnostic investigation of a case of anti-N-methyl-D-aspartate receptor encephalitis in a patient with a previous diagnosis of schizophrenia with poor response to antipsychotics. The aim of this case report is to highlight the importance of close surveillance for neuropsychiatric symptoms, especially catatonia, and to recognize autoimmune encephalitis in the differential diagnosis of psychotic disorders with neurological symptoms and resistance or intolerance to antipsychotics. A prompt diagnosis will contribute to a faster onset of therapy and an overall improvement in prognosis.

scholarly journals Rapidly progressive global cerebral atrophy in the setting of anti-LGI1 encephalitis

2019 ◽  
Vol 12 (5) ◽  
pp. e228428 ◽  
Author(s):  
Anudeep Yelam ◽  
Elanagan Nagarajan ◽  
Pradeep C Bollu
Keyword(s):  
Autoimmune Encephalitis ◽  
Cerebral Atrophy ◽  
Ivig Therapy ◽  
Cortical Atrophy ◽  
Hippocampal Atrophy ◽  
Serological Testing ◽  
Mri Findings ◽  
The Right ◽  
The Brain

A 47-year-old man presented with complaints of breakthrough seizures, psychiatric and behavioural changes and catatonic features. MRI of the brain showed mild cerebral and right hippocampal atrophy, while the electroencephalogram showed intermittent right temporal slowing. With a presumed diagnosis of autoimmune encephalitis, he was treated with intravenous immunoglobulin (IVIG) and methylprednisolone, which significantly improved the symptoms. Serological testing later was positive for antileucine-rich glioma inactivated 1 antibody. Two months after the initial presentation, patient had a relapse of the symptoms without any further episodes of seizures. Repeat MRI of the brain showed a significant rapidly progressive diffuse cortical atrophy and hippocampal atrophy, more prominent on the right side along with hydrocephalus ex vacuo when compared with the initial MRI. He is currently on monthly IVIG therapy. At 4 months follow-up from the second imagining study, the patient had persistent MRI findings.

scholarly journals 265. Rocky Mountain Spotted Fever Encephalopathy

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S238-S239
Author(s):  
Sangeetha Isaac ◽  
Mohammed Afraz Pasha ◽  
Jean H Vincent ◽  
Khushdeep Chahal
Keyword(s):  
Spotted Fever ◽  
Autoimmune Encephalitis ◽  
Cerebral Atrophy ◽  
Rocky Mountain ◽  
Natural Course ◽  
Progressive External Ophthalmoplegia ◽  
High Dose ◽  
Urine Drug Screen ◽  
Rocky Mountain Spotted Fever ◽  
Mri Findings

Abstract Background Rocky mountain spotted fever (RMSF) is a rickettsial disease with incidence of 11 per million and is rarely associated with encephalopathy. Here we discuss a patient with RMSF encephalopathy, highlighting the natural course. Methods A 54 year old man with history of hypertension and chronic progressive external ophthalmoplegia, presented with waxing and waning confusion, headache, slurred speech, agitation and difficulty swallowing. He was afebrile and hemodynamically stable. Investigations showed leukocytosis of 15400 and mild transaminitis. Computed-tomography (CT) head was unremarkable. Lumbar puncture revealed normal pressure. Cerebrosopinal fluid (CSF) analysis was notable for WBC 7, glucose 76 and moderately elevated total protein 114. Urine drug screen was negative. Blood, fungal, and CSF cultures were sent and empiric therapy with vancomycin, ceftriaxone, ampicillin and acyclovir commenced, for suspected encephalitis. High dose solumedrol 1gm/day was given due to suspicion of autoimmune encephalitis. MRI brain showed cerebral atrophy. There was slight abnormal FLAIR/T2 signal within the medial aspect of the temporal lobes, right more than left. Results Occupational history revealed that he was a logger by profession, which steered our focus on tick borne diseases. Extensive serologic evaluation was requested and RMSF IgG titres came back positive at 1:512. Doxycycline was added, while ampicillin and ceftriaxone were discontinued. With doxycycline, patient made remarkable recovery and was discharged home well. However, he returned within 48 hours with recurring encephalopathy. His clinical presentation remained convincing for RMSF encephalitis, with the natural course of the illness spanning over weeks, with waxing and waning symptoms. Patient was managed with IV doxycycline for 72 hours following which he returned to his baseline mental status. Figure 1. MRI findings Figure 2. Serological investigations Figure 3. CSF studies Conclusion Patient’s occupation played a pivotal role in establishing diagnosis. In RMSF, IgM and IgG antibodies appear 7 to 10 days after the onset of the illness, and a fourfold rise in IgG is diagnostic of seroconversion and recent illness. Patient’s waxing and waning symptoms, persisting for weeks and remarkable response to doxycycline, are typical features of RMSF encephalitis. Disclosures All Authors: No reported disclosures

scholarly journals Ovarian Torsion in a 5-Year Old: A Case Report and Review

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Matthew F. Ryan ◽  
Bobby K. Desai
Keyword(s):  
Emergency Department ◽  
Abdominal Pain ◽  
Case Report ◽  
Urinary Tract ◽  
Clinical Suspicion ◽  
Ovarian Torsion ◽  
Surgical Emergencies ◽  
High Clinical Suspicion ◽  
Tract Infections ◽  
Prompt Diagnosis

Ovarian torsion represents a true surgical emergency. Prompt diagnosis is essential to ovarian salvage, and high clinical suspicion is important in this regard. Confounding the diagnosis in general are more commonly encountered abdominal complaints in the Emergency Department (ED) such as constipation, diarrhea, and urinary tract infections and more common surgical emergencies such as appendicitis. Prompt diagnosis can be further complicated in low-risk populations such as young children. Herein, we describe the case of a 5-year-old girl with a seemingly benign presentation of abdominal pain who was diagnosed in the ED and treated for acute ovarian torsion after two prior clinic visits. A brief discussion of evaluation, treatment, and management of ovarian torsion follows.

scholarly journals Somatosensory aura in mesial temporal lobe epilepsy: semiologic characteristics, MRI findings and differential diagnosis with parietal lobe epilepsy

2006 ◽  
Vol 12 (3) ◽  
pp. 155-160 ◽  
Author(s):  
Márcio Andriani Rahal ◽  
Gerardo Maria de Araújo Filho ◽  
Luís Otávio Sales Ferreira Caboclo ◽  
Vivianne Pellegrino Rosa ◽  
Ricardo da Silva Centeno ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Parietal Lobe ◽  
Mesial Temporal Lobe Epilepsy ◽  
Mesial Temporal Sclerosis ◽  
Presurgical Evaluation ◽  
Mri Findings ◽  
Tingling Sensation ◽  
Mesial Temporal Lobe ◽  
Prevalent Symptom

INTRODUCTION: Somatosensory auras (SSAs) are more usually described in patients with parietal lobe epilepsy (PLE), producing more commonly a localized cutaneous tingling sensation, involving hands and fingers followed by tonic or clonic focal seizures. These usually originate in the contralateral hemisphere. Etiology includes dysplasias, tumours, ischemic or postencephalitic gliosis. However, other focal epilepsies, such as frontal and temporal, may also originate SSAs. Although this type of aura is reported as rare in patients with mesial temporal lobe epilepsy (MTLE), this association has not been systematically studied. OBJECTIVES: The aim of this article was to describe the cases of four patients with refractory MTLE and SSAs, reporting their clinical characteristics and MRI findings. We discuss the localizing and lateralizing value of SSAs, particularly in the context of MTLE. METHODS AND RESULTS: Four patients with refractory MTLE and SSAs followed-up in the outpatient's clinic at the Epilepsy Section, Universidade Federal de São Paulo, were submitted to presurgical evaluation and corticoamygdalohippocampectomy. MRI in all cases showed unilateral mesial temporal sclerosis (MTS). Regarding seizure semiology, tingling sensation involving the upper extremity was the most prevalent symptom. Three of the four patients had SSAs contralateral to the MTS. Following the SSAs all patients most of the time presented other symptoms such as autonomic or psychic auras evolving to psychomotor seizures. After surgical treatment, two of the patients presented infrequent auras, and two were rendered seizure-free. CONCLUSION: Although rare, SSAs can be present in MTLE. The characteristics of autonomic or psychic auras, psychomotor seizures, neuropsychological deficits, and typical neurophysiologic and MRI findings may help differentiate patients with MTLE from those with PLE.

Anti-N-methyl-D-aspartate receptor encephalitis and ovarian teratomas: is there any association?

2020 ◽  
Vol 19 (5) ◽  
pp. 124-131
Author(s):  
N.M. Podzolkova ◽  
◽  
V.V. Korennaya ◽  
O.S. Levin ◽  
E.E. Vasenina ◽  
...  
Keyword(s):  
Pathological Condition ◽  
Rare Complication ◽  
Autoimmune Encephalitis ◽  
Neurological Deficits ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Life Threatening ◽  
Causal Treatment ◽  
Ovarian Teratomas

This article aims to explore the problem of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and also describes our own experience in treating anti-NMDAR encephalitis in patients with ovarian teratomas. This pathological condition is a rare complication of cancer. Practicing gynecologists are often unfamiliar with it, although its consequences can be life-threatening. Early diagnosis and causal treatment, particularly teratoma removal, are critical for the outcome and can prevent death or the development of neurological deficits in patients. Key words: anti-NMDAR, autoimmune encephalitis, ovarian cysts, complications, ovarian teratoma, encephalitis, encephalitis in women

scholarly journals The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis

2019 ◽  
Author(s):  
Sudarshini Ramanathan ◽  
Adam Al-Diwani ◽  
Patrick Waters ◽  
Sarosh R. Irani
Keyword(s):  
Cerebrospinal Fluid ◽  
Close Association ◽  
Autoimmune Encephalitis ◽  
Clinical Syndrome ◽  
Clinical Observations ◽  
Improved Outcomes ◽  
Clinical Syndromes ◽  
Specific Autoantibody ◽  
Autoantibody Detection

Abstract The autoimmune encephalitis (AE) syndromes have been characterised by the detection of autoantibodies in serum and/or cerebrospinal fluid which target the extracellular domains of specific neuroglial antigens. The clinical syndromes have phenotypes which are often highly characteristic of their associated antigen-specific autoantibody. For example, the constellation of psychiatric features and the multi-faceted movement disorder observed in patients with NMDAR antibodies are highly distinctive, as are the faciobrachial dystonic seizures observed in close association with LGI1 antibodies. These typically tight correlations may be conferred by the presence of autoantibodies which can directly access and modulate their antigens in vivo. AE remains an under-recognised clinical syndrome but one where early and accurate detection is critical as prompt initiation of immunotherapy is closely associated with improved outcomes. In this review of a rapidly emerging field, we outline molecular observations with translational value. We focus on contemporary methodologies of autoantibody detection, the evolution and distinctive nature of the clinical phenotypes, generalisable therapeutic paradigms, and finally discuss the likely mechanisms of autoimmunity in these patients which may inform future precision therapies.

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