scholarly journals Case Report: Refractory Hypotension of GABA B Receptor Autoimmune Encephalitis

2020 ◽  
Vol 11 ◽  
Author(s):  
Kong Hanxin ◽  
Wang Xiaomin ◽  
Ying Hao ◽  
Zheng Shuihong
Keyword(s):  
Case Report ◽  
Psychiatric Symptoms ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Autonomic Nerve ◽  
Antibody Detection ◽  
High Dose ◽  
Gamma Aminobutyric Acid ◽  
Autonomic Nerve Function ◽  
Refractory Hypotension

Introduction: With the development of antibody detection technology, Gamma-Aminobutyric Acid (GABA) B receptor encephalitis is a known autoimmune disease. This paper describes a patient with refractory hypotension who suffered GABA B receptor autoimmune encephalitis.Case Report: We describe a 63-year-old man with GABA B receptor autoimmune encephalitis who had hypotension on day 17 of the disease onset. Despite two rounds of immunoglobulin administration, high-dose intravenous steroid injections and immunosuppressive therapy on day 35 of hospitalization, psychiatric symptoms and seizures were significantly improved; however, the patient's blood pressure remained low.Conclusion: This case study and literature review investigated the impairment of autonomic nerve function and its subsequent management in patients with GABA B receptor autoimmune encephalitis.

scholarly journals Vgkc Encephalitis With Low-Titer Antibodies: Case Report

2020 ◽  
pp. 1-3
Author(s):  
Sena Aksoy ◽  
◽  
Mina Uzülmez ◽  
Aysun Soysal ◽  
◽  
...  
Keyword(s):  
Case Report ◽  
Potassium Channel ◽  
Memory Impairment ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Voltage Gated ◽  
With Memory

Voltage-gated potassium channel (VGKC) encephalitis is a type of autoimmune encephalitis, that presents with memory impairment, headache, psychiatric symptoms and seizures. Although contactin-associated protein 2 (CASPR2) and leucine-rich glioma inactivated 1 (LGI1) are clearly identifed as components of the VGKC complex, additional subtypes are known to exist. In this report, we present a case of 64-year-old patient with VGKC antibodies, negative for CASPR2 and LGI1

scholarly journals Anti-NMDA Encephalitis: A Rare Encephalitis in a Child- A Case Report

2020 ◽  
Vol 19 (2) ◽  
pp. 108-111
Author(s):  
Saurav Khetan ◽  
Nikhil Agrawal ◽  
Prakash Rajoli
Keyword(s):  
Case Report ◽  
Japanese Encephalitis ◽  
Viral Encephalitis ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
High Index ◽  
First Case ◽  
High Index Of Suspicion

Anti-NMDA encephalitis is second commonest cause of autoimmune encephalitis among children; however, it is hardly diagnosed and often not considered as one of the differentials when a child presents with encephalitis-related symptoms. In children, it presents mostly with seizures or psychiatric symptoms without prodrome. Here we present a six years old girl who presented with seizures and inappropriate behaviour. We investigated her in the line of viral encephalitis such as Japanese Encephalitis, which is very common in our region. However, results were not suggestive of Japanese Encephalitis and further investigations subsequently lead to diagnosis of anti- NMDA encephalitis. This is probably one of the first case report of this disease in our country. We want to highlight the significance of high index of suspicion for looking out for an organic cause in any child who presents with psychiatric symptoms, along with seizures.

scholarly journals Autoimmune encephalitis: the clinical evolution as a key to the diagnosis

2019 ◽  
Vol 12 (9) ◽  
pp. e231094 ◽  
Author(s):  
Sara Teixeira ◽  
Joana Caldeira Santos ◽  
Marta Vila Real ◽  
Fátima Santos
Keyword(s):  
Neurological Examination ◽  
Psychiatric Symptoms ◽  
Focal Epilepsy ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Lower Limbs ◽  
Response To Treatment ◽  
Late Response ◽  
Nmdar Encephalitis ◽  
Age Appropriate

Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a devastating disease, that despite being increasingly diagnosed, there are no consensus guidelines for the optimal management. A previously healthy 3-year-old-boy brought to the emergency department due to seizures. Neurological examination was normal, and electroencephalogram (EEG) suggested focal epilepsy. Anticonvulsive medication was initiated. He progressively lost age-appropriate language skills, presented behavioural changes and psychiatric symptoms. Neurological examination at that time revealed symmetric gross motor weakness of the lower limbs. Brain and spinal cord MRI and cerebrospinal fluid were normal. Repeated EEG showed global lentification. Steroid therapy was initiated for the suspicion of autoimmune encephalitis, later confirmed as NMDAR encephalitis. He became clinically improved after 10 days of treatment but only returned to his baseline after 3 months of disease onset. The authors emphasised the variable course of the disease and possible late response to treatment.

scholarly journals NMDA-receptor encephalitis: An unsual case of refractory status epilepticus

2015 ◽  
Vol 42 (S1) ◽  
pp. S33-S33
Author(s):  
S Lapointe ◽  
C Legault ◽  
P Giacomini ◽  
R La Piana ◽  
J Teitelbaum ◽  
...  
Keyword(s):  
Case Report ◽  
Status Epilepticus ◽  
Nmda Receptor ◽  
Refractory Status Epilepticus ◽  
Antibody Detection ◽  
High Dose ◽  
Temporal Lobes ◽  
Nmda Receptor Encephalitis ◽  
Refractory Status ◽  
Cerebellar Involvement

Objective: Case report of NMDA receptor encephalitis in a young man with early refractory status epilepticus and atypical radiological findings. Background: Anti-NMDA receptor encephalitis is an autoimmune disorder due to antibodies to the NR1-NR2B heterodimer of NMDA receptor. On imaging, it typically presents with T2 hyperintensities in mesio-temporal lobes, cerebral cortex and basal ganglia. We present a case with a dramatic clinical evolution and novel imaging findings. Design/Methods: Case report and review of imaging. Results: 29-year-old male presented with mood disturbance followed by partial-complex seizures, facial dyskinesia and choreo-athetotic movements. Initial MRI showed subtle T2-hyperintensities in mesio-temporal lobes. Diagnosis of NMDA-receptor encephalitis was confirmed after CSF antibody detection. Prior to diagnostic confirmation, he developed refractory status epilepticus, and concomitant signs of herniation. A repeated MRI showed increased T2-hyperintensities of thalami and mesencephalon, with cerebellar involvement and transtentorial/foraminal herniation. Restricted diffusion was documented in the cerebellar cortex/thalami/putamina and caudate. IV corticosteroids and hypertonic fluid reversed herniation, and halted the seizures. Conclusions: To our knoweldge, we report the first case report of uncal and tonsillar herniation in NMDA-r encephalitis secondary to atypical, predominant cerebellar involvement. This case highlights life-threatening manifestation that physicians might encounter, and a possible role for high dose IV corticosteroids as an adjunct treatment for brain edema and seizures.

scholarly journals Rituximab for Autoimmune Encephalitis with Epilepsy

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Mohankumar Kurukumbi ◽  
Rahul H. Dave ◽  
Jose Castillo ◽  
Tulsi Shah ◽  
Joanne Lau
Keyword(s):  
Intractable Epilepsy ◽  
Autoimmune Encephalitis ◽  
Aminobutyric Acid ◽  
High Dose ◽  
Gamma Aminobutyric Acid ◽  
Acid Receptor ◽  
Voltage Gated ◽  
Aspartate Receptor ◽  
A Cell

Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.

scholarly journals Case Report: Anti-LGI1 Encephalitis Following COVID-19 Vaccination

2022 ◽  
Vol 12 ◽  
Author(s):  
Yair Zlotnik ◽  
Avi Gadoth ◽  
Ibrahim Abu-Salameh ◽  
Anat Horev ◽  
Rosa Novoa ◽  
...  
Keyword(s):  
Adverse Event ◽  
Cognitive Impairment ◽  
Case Report ◽  
Autoimmune Encephalitis ◽  
Neurological Complications ◽  
Severe Adverse Event ◽  
High Dose ◽  
Progressive Dementia ◽  
Vaccination Response ◽  
Muscle Spasms

Anti-leucine rich glioma inactivated 1 (LGI1) autoimmune encephalitis (AE) is characterized by cognitive impairment or rapid progressive dementia, psychiatric disorders, faciobrachial dystonic seizures (FBDS) and refractory hyponatremia. Since December 2020, millions of people worldwide have been vaccinated against COVID-19. Several soft neurological symptoms like pain, headache, dizziness, or muscle spasms are common and self-limited adverse effects after receiving the COVID-19 vaccine. However, several major neurological complications, despite the unproven causality, have been reported since the introduction of the COVID-19 vaccine. Herein, we describe a 48 years old man presenting with rapidly progressive cognitive decline and hyponatremia diagnosed with anti LGI1 AE, occurring shortly after the second dose of mRNA COVID -19 vaccine and possibly representing a severe adverse event related to the vaccination. Response to high dose steroid therapy was favorable. As millions of people worldwide are currently receiving COVID-19 vaccinations, this case should serve to increase the awareness for possible rare autoimmune reactions following this novel vaccination in general, and particularly of anti-LGI1 AE.

Anti-GAD positive autoimmune encephalitis presenting with dementia, psychiatric symptoms and MS findings on the MRI: A case report

2017 ◽  
Vol 381 ◽  
pp. 536
Author(s):  
J.M.D. Alencar ◽  
T.A.G.J. Ribeiro ◽  
D.S. Diniz ◽  
G.D.S. Melo ◽  
I.M.L. Bezerra ◽  
...  
Keyword(s):  
Case Report ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis

Confabulation, amnesia and motor memory loss as a presentation of apparent ITPR1 antibody autoimmune encephalitis

2021 ◽  
Vol 14 (9) ◽  
pp. e244316
Author(s):  
Julia R Schiff ◽  
Benjamin P Fiorillo ◽  
Raha Sadjadi ◽  
Tracey L Henry ◽  
Judah K Gruen ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Altered Mental Status ◽  
Motor Memory ◽  
Inositol Triphosphate ◽  
High Dose ◽  
Wernicke Encephalopathy ◽  
Intravenous Steroids ◽  
Primary Presentation

A 59-year-old woman presented to the hospital with acute, hypoactive altered mental status. Her symptoms had begun 3 days prior when she developed hallucinations, urinary and faecal incontinence, and somnolence. She also exhibited confabulations, amnesia, motor memory loss and a wide-based gait. Medical, psychiatric and neurological evaluations including imaging and laboratory workup were unrevealing. Treatment for possible Wernicke encephalopathy and psychosis with high-dose intravenous thiamine and antipsychotic medications did not lead to improvement. After discharge, a send-out cerebrospinal fluid autoimmune encephalitis panel resulted positive for the newly identified neuronal inositol triphosphate receptor one (ITPR1) antibody. This prompted readmission for intravenous steroids, plasmapheresis and intravenous immunoglobulin, which yielded mild clinical improvement. Here, we describe confabulations and psychiatric symptoms as novel manifestations of the primary presentation of anti-ITPR1 encephalitis in an effort to promote faster recognition of this disease and early initiation of treatment in suspected cases.

scholarly journals Molecular mimicry of NMDA receptors may contribute to neuropsychiatric symptoms in severe COVID-19 cases

2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Veronika Vasilevska ◽  
Paul C. Guest ◽  
Hans-Gert Bernstein ◽  
Matthias L. Schroeter ◽  
Christian Geis ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Molecular Mimicry ◽  
Psychiatric Symptoms ◽  
Auditory Hallucination ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Altered Mental Status ◽  
Warning Signs ◽  
High Dose ◽  
Nmda Receptor Encephalitis

AbstractApproximately 30% of individuals with severe SARS-CoV-2 infections also develop neurological and psychiatric complaints. In rare cases, the occurrence of autoimmune encephalitis has been reported after SARS-CoV-2 infection. In this systematic review, we have identified eight SARS-CoV-2-associated cases of anti-NMDA receptor encephalitis. All had cerebrospinal fluid antibodies against the NMDA receptor and a recent onset of working memory deficits, altered mental status, or psychiatric symptoms, such as confusion, agitation, auditory hallucination, catatonia and speech dysfunction. All patients received high-dose steroid and immunoglobulin therapeutics and conditions improved in each case. These findings suggest that clinical attention should be paid to warning signs of autoimmune encephalitis in severe COVID-19 cases. If characteristic features of autoimmune encephalitis are present, autoantibody diagnostics should be performed and confirmed cases should be treated with immunotherapy to minimize neurological impairments.

scholarly journals Clinical Characteristics and Outcome of Neuronal Surface Antibody-Mediated Autoimmune Encephalitis Patients in a National Cohort

2021 ◽  
Vol 12 ◽  
Author(s):  
Zsófia Hayden ◽  
Beáta Bóné ◽  
Gergely Orsi ◽  
Monika Szots ◽  
Ferenc Nagy ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Disease Onset ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Test Results ◽  
Nmdar Encephalitis

Background: In our previous single-center study of autoimmune encephalitis (AE) related autoantibody test results we found positivity in 60 patients out of 1,034 with suspected AE from 2012 through 2018 as part of a Hungarian nationwide program. In our current multicenter retrospective study, we analyzed the clinical characteristics and outcome of AE patients with positive neuronal cell surface autoantibody test results.Methods: A standard online questionnaire was used to collect demographic and clinical characteristics, laboratory and imaging data, therapy and prognosis of 30 definitive AE patients in four major clinical centers of the region.Results: In our study, 19 patients were positive for anti-NMDAR (63%), 6 patients (20%) for anti-LGI1, 3 patients for anti-GABABR (10%) and 3 patients for anti-Caspr2 (10%) autoantibodies. Most common prodromal symptoms were fever or flu-like symptoms (10/30, 33%). Main clinical features included psychiatric symptoms (83%), epileptic seizures (73%) and memory loss (50%). 19 patients (63%) presented with signs of central nervous system (CNS) inflammation, which occurred more frequently in elder individuals (p = 0.024), although no significant differences were observed in sex, tumor association, time to diagnosis, prognosis and immunotherapy compared to AE patients without CNS inflammatory markers. Anti-NMDAR encephalitis patients were in more severe condition at the disease onset (p = 0.028), although no significant correlation between mRS score, age, sex and immunotherapy was found. 27% of patients (n = 8) with associated tumors had worse outcome (p = 0.045) than patients without tumor. In most cases, immunotherapy led to clinical improvement of AE patients (80%) who achieved a good outcome (mRS ≤ 2; median follow-up 33 months).Conclusion: Our study confirms previous publications describing characteristics of AE patients, however, differences were observed in anti-NMDAR encephalitis that showed no association with ovarian teratoma and occurred more frequently among young males. One-third of AE patients lacked signs of inflammation in both CSF and brain MRI, which emphasizes the importance of clinical symptoms and autoantibody testing in diagnostic workflow for early introduction of immunotherapy, which can lead to favorable outcome in AE patients.

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