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.

A narrative review of pharmacologic approaches to symptom management of pediatric patients diagnosed with anti-NMDA receptor encephalitis

2021 ◽  
pp. 1-11
Author(s):  
Joshua A. Vova
Keyword(s):  
Nmda Receptor ◽  
Symptom Management ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Neuropsychiatric Symptoms ◽  
Disease Process ◽  
Autoimmune Encephalitis ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor ◽  
Autonomic Instability

Anti-N-Methyl-D-Aspartate Receptor Encephalitis (ANMDARE) is one of the most common autoimmune encephalitis in the pediatric population. Patients with ANMDARE initially present with a prodrome of neuropsychiatric symptoms followed by progressively worsening seizures, agitation, and movement disorders. Complications can include problems such as aggression, insomnia, catatonia, and autonomic instability. Due to the complexity of this disease process, symptom management can be complex and may lead to significant polypharmacy. The goal of this review is to educate clinicians about the challenges of managing this disorder and providing guidance in symptom management.

scholarly journals Clinical case of encephalitis with antibodies to NMDA receptors

2019 ◽  
Vol 18 (4) ◽  
pp. 67-69
Author(s):  
L. U. Ulukhanova ◽  
N. S. Karnayeva ◽  
M. M. Yaraliev ◽  
A. G. Gadzhimirzaevа ◽  
S. G. Agaevа
Keyword(s):  
Nmda Receptor ◽  
Clinical Observation ◽  
Neuropsychiatric Symptoms ◽  
Fatal Outcome ◽  
Autoimmune Encephalitis ◽  
Final Diagnosis ◽  
Nmda Receptor Encephalitis ◽  
General Weakness ◽  
The Central Nervous System ◽  
Nr2 Subunits

Anti-NMDA receptor encephalitis is an autoimmune disease of the central nervous system caused by autoantibodies to the NR1 and NR2 subunits of the glutamate NMDA receptor, with the possibility of both death and rapid remission. The binding of these antibodies blocks receptors and causes slowly developing psychiatric disorders, motor disorders and seizures.Presented clinical observation in a 9-year-old patient. The disease debuted with prodromal flu-like symptoms, fever, headache and general weakness, after which neuropsychiatric symptoms, impaired memory and speech developed, further progression of the disease, convulsive status, and coma with a fatal outcome were noted. The final diagnosis of autoimmune encephalitis was made after identification of antibodies to the N-methyl-D-aspartate (NMDA) receptor in the blood.

scholarly journals NATURAL PRODUCTS IN TREATMENT OF ENCEPHALITIS: A REVIEW

2021 ◽  
Vol 21 (no 1) ◽  
Author(s):  
Danishpreet Kaur Takhar ◽  
Mukta Gupta
Keyword(s):  
Natural Products ◽  
Nmda Receptor ◽  
Plasma Exchange ◽  
Intravenous Immunoglobulin ◽  
Psychotic Disorders ◽  
Treatment Options ◽  
Therapeutic Approaches ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor

Brain on fire, an unusual phrase used for the deadliest autoimmune ailment, called anti-NMDA (N-methyl-D-aspartate) receptor encephalitis, characterizing extreme psychiatric and neurotic signs. Though being the deadliest one, still it can be treated with the help of various therapeutic approaches such as Corticosteroids, Intravenous immunoglobulin (IVIG) and plasmapheresis or plasma exchange. Although the prevalence of encephalitis can be observed in both the sexes, however the majority of ailment (95%) is seen in women with teratoma ovaries or different neoplasms. Recognition of nti-NMDA receptor encephalitis could be very essential to avoid any misconception regarding incorrect interpretation of various psychotic disorders. However, various treatment options are available still further investigation should be required to carried out to find out other clinically beneficial drugs.

scholarly journals Early multidisciplinary intensive-care therapy can improve outcome of severe anti-NMDA-receptor encephalitis presenting with extreme delta brush

2019 ◽  
Vol 10 (1) ◽  
pp. 241-243 ◽  
Author(s):  
R. Schneider ◽  
M. Brüne ◽  
TG. Breuer ◽  
C. Börnke ◽  
R. Gold ◽  
...  
Keyword(s):  
Intensive Care ◽  
Autoimmune Encephalitis ◽  
Second Line ◽  
Early Administration ◽  
Course Of Disease ◽  
Intensive Care Therapy ◽  
Young Females ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor ◽  
Antibody Titers

Abstract Anti-N-methyl-D-aspartate receptor encephalitis (Anti-NMDARE) is a synaptic autoimmune encephalitis syndrome mainly affecting young females. An underlying tumor, most commonly ovarian teratomas in young females, may indicate a paraneoplastic syndrome. Prognostic factors of the clinical course of disease and outcome play a central role in view of early administration of second-line immunotherapy and intensive-care therapy. We report a case of severe Anti-NMDARE associated with unfavorable predictors including an extreme delta brush (EDB) electroencephalographic-pattern and high anti-NMDAR-antibody titers in the cerebral spinal fluid (CSF), which necessitated the admission to an intensive care unit. In spite of the poor prognosis, the patient completely recovered; we attribute this to an early escalation to second-line immunotherapy with rituximab and multidisciplinary intensive-care therapy. The present case underlines the relevance of multidisciplinary management for individuals with Anti-NMDARE.

scholarly journals Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Bissene Douma ◽  
Thouraya Ben Younes ◽  
Hanene Benrhouma ◽  
Zouhour Miladi ◽  
Imen Zamali ◽  
...  
Keyword(s):  
Brain Mri ◽  
Autoimmune Encephalitis ◽  
Therapeutic Interventions ◽  
High Dose ◽  
First Line ◽  
Nmda Receptor Encephalitis ◽  
Progressive Encephalopathy ◽  
Laboratory Features ◽  
Fluid Attenuated Inversion Recovery ◽  
The Central Nervous System

Background. Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy caused by antibodies targeting neurons in the central nervous system generating specific immune responses. It is increasingly recognized in children. Objective. To describe clinical, neuroimaging, and laboratory features, treatment, and outcome in a cohort of Tunisian children with AE. Methods. We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with autoimmune encephalitis between 2004 and 2020. Clinical, neuroimaging, laboratory features, therapeutic data, and outcome were analyzed. Results. Nineteen children were included in the study (12 girls and 7 boys). The median age at diagnosis was 7.68 years (range: 10 months-13 years). The most frequent manifestations were seizures and behavioral disorders. Eleven cases were diagnosed with anti-NMDA receptor encephalitis, 4 cases with anti-Ma2 encephalitis, 3 cases with anti-GAD encephalitis, and 1 case with anti-SOX1 encephalitis. Brain MRI showed increased T2 and fluid-attenuated inversion recovery (FLAIR) signal of the temporal lobe in 5 patients. Eighteen patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin). One patient with anti-GAD encephalitis died despite escalating immunotherapy. Conclusion. Diagnosis of autoimmune encephalitis is challenging in children, because of misleading presentations. An early and accurate diagnosis is important to enable proper therapeutic interventions.

scholarly journals Headache, Delirium or Encephalitis? A Case of Residual Mutism Secondary to Anti-NMDA Receptor Encephalitis

2019 ◽  
Vol 11 (3) ◽  
pp. 330-343
Author(s):  
Omar Jiménez-Zarazúa ◽  
María Andrea Martínez-Rivera ◽  
Pedro Luis González-Carrillo ◽  
Lourdes N. Vélez-Ramírez ◽  
María Alcocer-León ◽  
...  
Keyword(s):  
Neuropsychiatric Symptoms ◽  
Hospital Setting ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Systemic Steroid ◽  
Paraneoplastic Encephalitis ◽  
Nmda Receptor Encephalitis ◽  
Management Procedure ◽  
Specialized Hospital ◽  
Systemic Steroid Therapy

Encephalitis is a heterogeneous syndrome that is diagnosed through clinical assessment and the assistance of laboratory, neuroimaging and electroencephalographic workup. Over the past 10 years, autoimmune encephalitis has been more frequently recognized; however, most reports come from highly specialized hospital settings. Anti-N-methyl-D-aspartate receptor (NDMAR) encephalitis has been associated with paraneoplastic encephalitis syndromes and was first recognized in 2005. We present the case of a 34-year-old male patient who debuted clinically with a headache associated with neuropsychiatric symptoms (i.e., visual and auditory hallucinations, anxiety, aggressiveness) and memory deficits, progressing to autonomic dysfunction (i.e., tachycardia and hypertension), seizures, and stupor with catatonic features. Initially, infectious, metabolic, and toxicological etiologies were excluded; followed by the assessment of immunological and paraneoplastic etiologies, yielding positive IgG levels for anti-NMDAR antibodies. The patient was treated successfully with systemic steroid therapy and therapeutic plasmapheresis, while mutism was the only sequela. Although large case series reporting on paraneoplastic and autoimmune anti-NMDAR encephalitis have been reported in the literature in recent years, this case is of particular importance due to the stepwise differential diagnosis and treatment management procedure that was used in a regional but not highly specialized hospital setting.

scholarly journals Anti NMDA receptor encephalitis: a potentially treatable encephalitis

2020 ◽  
Vol 7 (7) ◽  
pp. 1624
Author(s):  
Archana K. M. ◽  
Sushma Save ◽  
Koppikar Rahul Gautam
Keyword(s):  
Nmda Receptor ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Ivig Therapy ◽  
Receptor Subunit ◽  
Nmda Receptor Subunit ◽  
Nmda Receptor Encephalitis ◽  
Autonomic Instability ◽  
High Index Of Suspicion ◽  
Age Range

Anti NMDA receptor encephalitis is autoimmune encephalitis where antibodies are directed against NMDA receptor subunit. It represents a new category of immune-mediated disorder that is often paraneoplastic, presenting with neuropsychiatric symptoms, which is treatable and can be diagnosed serologically affecting both children and adults. Patient can have variable clinical presentation ranging from prodromal illness, neuropsychiatric symptoms, seizures, autonomic instability, hyperkinesias, catatonia, hypoventilation and with or without an associated teratoma. A positive serum or CSF sample screening for antibodies to the NMDA receptor subunit is confirmative. Supportive findings include CSF study, EEG and MRI Brain. The first-line therapies includes IVIG, corticosteroids or plasma exchange. Second line immunotherapy is rituximab or cyclophosphamide or both. Given the high mortality rate (up to 25%), the likelihood of presentation across the age range and the potential for treatment, a high index of suspicion is warranted by clinicians. Authors report a case of a 5 year old child with anti NMDA receptor encephalitis who responded well to IVIG therapy.

scholarly journals Anti-N-methyl-D-aspartate receptor encephalitis presenting as atypical psychosis in multiple sclerosis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Khushminder Chahal ◽  
Tara Endeman ◽  
Sarah Scapinello ◽  
Michal Sapieha
Keyword(s):  
Multiple Sclerosis ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Psychiatric Inpatient ◽  
Treatment Protocol ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Atypical Symptoms ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

Abstract Background Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder which often presents with neuropsychiatric symptoms. A large proportion of cases are associated with an identifiable tumor, most commonly ovarian teratoma. However, recent literature has also described an overlap of anti-NMDAR encephalitis and demyelinating syndromes. Cases have been reported of anti-NMDAR encephalitis in patients with ADEM, optic neuritis, myelitis and multiple sclerosis. This link is considered rare, however has important clinical implications as treatments and prognosis may differ. Case presentation A 33-year-old female with a history of multiple sclerosis presented with new-onset neuropsychiatric symptoms. After substance-induced psychosis was ruled out, she was admitted to the medical ward for work up of psychosis secondary to multiple sclerosis. However, the consultation-liaison psychiatry service noted atypical symptoms which were concerning for autoimmune encephalitis. Admission to a psychiatric inpatient ward was deferred. Anti-NMDAR encephalitis was diagnosed with CSF analysis demonstrating lymphocytic pleocytosis and anti-NMDAR antibodies. In addition to first-line treatment of encephalitis with steroids, second-line immunotherapies were also implemented given the patient’s underlining demyelinating syndrome. The patient’s neurologic and psychiatric symptoms began to improve. Conclusions There is literature to demonstrate a possible connection between anti-NMDAR encephalitis and demyelinating syndromes. As such, autoimmune encephalitis should be considered in patients with multiple sclerosis presenting with atypical symptoms. Determining the correct diagnosis is crucial to inform the appropriate treatment protocol, and to improve prognosis.

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.

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.

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