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 Autoimmune Encephalitis in Latin America: A Critical Review

2021 ◽  
Vol 11 ◽  
Author(s):  
Gabriel de Albuquerque Vasconcelos ◽  
Rodrigo Montenegro Barreira ◽  
Karmelita Emanuelle Nogueira Torres Antoniollo ◽  
Alina Maria Nuñez Pinheiro ◽  
Cíntia Fernandes Rodrigues Maia ◽  
...  
Keyword(s):  
Latin America ◽  
Latin American ◽  
World Literature ◽  
Case Reports ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Developed Countries ◽  
Future Research ◽  
Epidemiologic Factors ◽  
Nmda Receptor Encephalitis

Autoimmune encephalitis is an increasingly recognized cause of encephalitis. The majority of case series report patients residing in developed countries in the northern hemisphere. The epidemiologic features of autoimmune encephalitis in Latin America are still unclear. The aim of the study was to perform a review of the clinical presentation of autoimmune encephalitis in Latin America and compare to world literature. References were identified by an in-depth literature search and selected on the basis of relevance to the topic and authors' judgment. We selected clinical studies and case reports published from 2007 to July, 2020 including patients from Latin American countries. Of the 379 patients included, the majority were cases of anti-NMDA receptor encephalitis (93.14%), followed by anti-VGKC-complex encephalitis (N = 17; 4.48%), anti-GAD encephalitis (N = 9; 2.37%), anti-AMPA receptor encephalitis (N = 1; 0.26%), anti-GABA receptor encephalitis (N = 1; 0. 26%), anti-mGluR5 encephalitis (N = 1; 0. 26%), and anti-mGluR1 encephalitis (N = 1; 0. 26%). Reported cases of Anti-NMDA encephalitis in Latin-America had a very slight female predominance, lower prevalence of associated tumors and a lower incidence of extreme delta brush on electroencephalogram. Autoimmune encephalitis is possibly underdiagnosed in underdeveloped countries. Its outcome after treatment, however, appears to be similarly favorable in Latin American patients as has been reported in developed countries based on available case reports and case series. Regional specificities in the manifestation of autoimmune encephalitis could be related to epidemiologic factors, such as the presence of different triggers and different genetic and immunologic background, that need to be studied by future research.

scholarly journals Battey’s operation as a treatment for hysteria: a review of a series of cases in the nineteenth century

2019 ◽  
Vol 31 (1) ◽  
pp. 55-66
Author(s):  
Tomoko Komagamine ◽  
Norito Kokubun ◽  
Koichi Hirata
Keyword(s):  
Nineteenth Century ◽  
Differential Diagnosis ◽  
Nmda Receptor ◽  
Neuropsychiatric Symptoms ◽  
Acute Onset ◽  
Cystic Degeneration ◽  
Late Nineteenth Century ◽  
Paraneoplastic Encephalitis ◽  
Nmda Receptor Encephalitis ◽  
Late Nineteenth

Ovarian resection as a treatment for hysteria, called ‘Battey’s operation’ or ‘normal ovariotomy’, was performed in the nineteenth century. Battey later reported that the resected ovaries appeared to have ‘cystic degeneration’. Currently, patients with acute neuropsychiatric symptoms are screened for teratomas for the differential diagnosis of anti-NMDA receptor encephalitis. There is now a hypothesis that ovarian lesions resulting in paraneoplastic encephalitis were among the patients who underwent Battey’s operation. We identified 94 published cases of Battey’s operation for neuropsychiatric symptoms in the late nineteenth century. Among 36 cases with detailed descriptions, we found 3 patients who showed acute onset neuropsychiatric symptoms with macropathological ovarian findings that were compatible with teratoma. They showed favourable prognoses after surgery and might have motivated the surgeons to perform the operation.

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 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 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.

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 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 Case Report: Daratumumab in a Patient With Severe Refractory Anti-NMDA Receptor Encephalitis

2020 ◽  
Vol 11 ◽  
Author(s):  
Dominica Ratuszny ◽  
Thomas Skripuletz ◽  
Florian Wegner ◽  
Matthias Groß ◽  
Christine Falk ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Neuropsychiatric Symptoms ◽  
Clinical Status ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Intravenous Immunoglobulins ◽  
Nmda Receptor Encephalitis ◽  
Life Threatening ◽  
Therapy Strategies

Anti-NMDA receptor encephalitis is the most common type of antibody mediated autoimmune encephalitis (AIE). Patients often develop neuropsychiatric symptoms and seizures, women are affected about four times more than men, and in about 50% the disease is associated with a neoplasia, especially teratomas of the ovary. We describe the case of a 20-year-old woman suffering from a severe therapy refractory course of anti-NMDA receptor encephalitis. Treatment included glucocorticoids, plasma exchange, intravenous immunoglobulins, rituximab, and bortezomib without clinical improvement. Due to a therapy refractive course 28 weeks after disease onset, the patient received 10 cycles of daratumumab. Therapy escalation was performed with the anti-CD38 monoclonal antibody daratumumab as off label treatment, based on the therapy of refractory myeloma and led to an improvement of her clinical status. She spent about 200 days on the intensive care unit, followed by several weeks on the intermediate care unit with close follow ups every 4–6 weeks afterward. During follow-up, the patient was able to resume everyday and self-care activities, reflected by the modified Rankin scale (mRS) and Barthel index. Because this disease is potentially life threatening and can lead to irreversible brain atrophy, development of further therapy strategies are of great importance. Our case describes a successful treatment for therapy refractory anti-NMDA receptor encephalitis using the anti-CD38 antibody daratumumab.

scholarly journals Decreased inflammatory cytokine production of antigen-specific CD4+ T cells in NMDA receptor encephalitis

2021 ◽  
Author(s):  
Le-Minh Dao ◽  
Marie-Luise Machule ◽  
Petra Bacher ◽  
Julius Hoffmann ◽  
Lam-Thanh Ly ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
Ex Vivo ◽  
Autoimmune Encephalitis ◽  
Active Immunization ◽  
Inflammatory Cytokine Production ◽  
T Cell Infiltration ◽  
Nmda Receptor Encephalitis ◽  
Nmdar Encephalitis ◽  
Disease Specific

AbstractAnti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is the most common autoimmune encephalitis with psychosis, amnesia, seizures and dyskinesias. The disease is mediated by pathogenic autoantibodies against the NR1 subunit that disrupt NMDAR function. Antibody infusion into mouse brains can recapitulate encephalitis symptoms, while active immunization resulted also in strong T cell infiltration into the hippocampus. However, whether T cells react against NMDAR and their specific contribution to disease development are poorly understood. Here we characterized the ex vivo frequency and phenotype of circulating CD4+ T helper (TH) cells reactive to NR1 protein using antigen-reactive T cell enrichment (ARTE) in 24 patients with NMDAR encephalitis, 13 patients with LGI1 encephalitis and 51 matched controls. Unexpectedly, patients with NMDAR encephalitis had lower frequencies of CD154-expressing NR1-reactive TH cells than healthy controls and produced significantly less inflammatory cytokines. No difference was seen in T cells reactive to the synaptic target LGI1 (Leucine-rich glioma-inactivated 1), ubiquitous Candida antigens or neoantigens, suggesting that the findings are disease-specific and not related to therapeutic immunosuppression. Also, patients with LGI1 encephalitis showed unaltered numbers of LGI1 antigen-reactive T cells. The data reveal disease-specific functional alterations of circulating NMDAR-reactive TH cells in patients with NMDAR encephalitis and challenge the idea that increased pro-inflammatory NMDAR-reactive T cells contribute to disease pathogenesis.

Living With Both a Total Ankle Replacement and an Ankle Fusion: A Qualitative Study From the Patients’ Perspective

2021 ◽  
pp. 107110072110044
Author(s):  
Catherine Conlin ◽  
Ryan M. Khan ◽  
Ian Wilson ◽  
Timothy R. Daniels ◽  
Mansur Halai ◽  
...  
Keyword(s):  
Hospital Setting ◽  
Case Series ◽  
Total Ankle Replacement ◽  
Qualitative Description ◽  
Level Of Evidence ◽  
Foot Placement ◽  
Advantages And Disadvantages ◽  
Ankle Fusion ◽  
Ankle Replacement ◽  
Patient Reported

Background: Total ankle replacement (TAR) and ankle fusion are effective treatments for end-stage ankle arthritis. Comparative studies elucidate differences in treatment outcomes; however, the literature lacks evidence demonstrating what outcomes are important to patients. The purpose of this study was to investigate patients’ experiences of living with both a TAR and ankle fusion. Methods: This research study used qualitative description. Individuals were selected from a cohort of patients with TAR and/or ankle fusion (n = 1254). Eligible patients were English speaking with a TAR and contralateral ankle fusion, and a minimum of 1 year since their most recent ankle reconstruction. Surgeries were performed by a single experienced surgeon, and semistructured interviews were conducted by a single researcher in a private hospital setting or by telephone. Ankle Osteoarthritis Scale (AOS) scores, radiographs, and ancillary surgical procedures were collected to characterize patients. Themes were derived through qualitative data analysis. Results: Ten adults (8 men, 2 women), ages 59 to 90 years, were included. Average AOS pain and disability scores were similar for both surgeries for most patients. Participants discussed perceptions of each reconstructed ankle. Ankle fusions were considered stable and strong, but also stiff and compromising balance. TARs were considered flexible and more like a “normal ankle,” though patients expressed concerns about their TAR “turning” on uneven ground. Individuals applied this knowledge to facilitate movement, particularly during a first step and transitioning between positions. They described the need for careful foot placement and attention to the environment to avoid potential challenges. Conclusion: This study provides insight into the experiences of individuals living with a TAR and ankle fusion. In this unusual but limited group of patients, we found that each ankle reconstruction was generally perceived to have different characteristics, advantages, and disadvantages. Most participants articulated a preference for their TAR. These findings can help clinicians better counsel patients on expectations after TAR and ankle fusion, and improve patient-reported outcome measures by better capturing meaningful outcomes for patients. Level of Evidence: Level IV, case series.

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