scholarly journals Severe tooth loss secondary to orofacial dyskinesias in anti-NMDA receptor encephalitis

2019 ◽  
Vol 12 (3) ◽  
pp. e228380
Author(s):  
Daniel Garbin Di Luca ◽  
Jason H Margolesky
Keyword(s):  
Emergency Department ◽  
Nmda Receptor ◽  
Tooth Loss ◽  
Altered Mental Status ◽  
Psychiatric History ◽  
Aggressive Treatment ◽  
Orofacial Dyskinesia ◽  
Speech Output ◽  
Nmda Receptor Encephalitis ◽  
Receptor Antibodies

A 24-year-old woman with no significant medical or psychiatric history was brought to the emergency department due to altered mental status and bizarre behaviour. Physical examination was remarkable for decreased speech output and orofacial dyskinesia. Upon further evaluation, electroencephalogram showed extreme delta brush waves and cerebrospinal fluid was positive for anti-NMDA receptor antibodies. Despite aggressive treatment with steroids and immunosuppressive therapy, her dyskinesia was severe enough to cause tooth loss, tongue and lip laceration.

scholarly journals Anti-NMDAR Encephalitis: Higher Suspicious Needed for Earlier Diagnosis (Case Report, Literature Review and Diagnostic Criteria)

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Chanaka Amugoda ◽  
Noushin Chini Foroush ◽  
Hamed Akhlaghi
Keyword(s):  
Mental Health ◽  
Emergency Department ◽  
Nmda Receptor ◽  
Psychiatric Symptoms ◽  
Health Assessment ◽  
Young Patients ◽  
Psychotic Episode ◽  
Acute Psychosis ◽  
Nmda Receptor Encephalitis ◽  
Immune Mediated

Background. Auto-immune mediated anti-NMDA receptor encephalitis is a very common delayed diagnosed encephalitis which predominately affecting young population. Objectives. This encephalitis is relatively unknown amongst emergency physicians and a majority of patients are admitted to psychiatric wards before their diagnosis is confirmed and appropriate treatments are commenced. We reported a case of a 22-year-old female presented to our emergency department with acute psychiatric symptoms. She was initially diagnosed with first presentation of acute psychosis and was hospitalised under mental health act. further assessment in the emergency department identified possible an organic cause for her acute psychosis and she was later admitted under medical team after her mental health assessment order was revoke. Several days later, her CSF result was positive with anti-NMDA receptor anti-bodies. Appropriate treatments were instituted leading to her full recovery. Conclusion. This case was the first confirmed anti-NMDA receptor encephalitis in our emergency department. It highlights the importance of thorough assessment of psychiatric presentations to emergency departments and consideration of auto-immune medicated encephalitis as one of the differential diagnosis in young patients presenting with first acute psychotic episode.

scholarly journals Anti-NMDA Receptor Encephalitis in a Patient with a History of Autism Spectrum Disorder

2020 ◽  
Vol 10 (3) ◽  
pp. 231-235
Author(s):  
Xavier Diao ◽  
Milana Mor
Keyword(s):  
Autism Spectrum Disorder ◽  
Nmda Receptor ◽  
Neuropsychiatric Symptoms ◽  
Antipsychotic Agents ◽  
Altered Mental Status ◽  
Autism Spectrum ◽  
Acute Onset ◽  
Spectrum Disorder ◽  
Nmda Receptor Encephalitis ◽  
History Of

Background: Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune syndrome characterized by a well-described constellation of neuropsychiatric symptoms. Its exact pathophysiology is poorly understood, but it is thought to be mediated by autoantibodies against NMDA (N-methyl-D-aspartate)-type glutamate receptors in the central nervous system. There is ongoing literature to suggest that patients with autism spectrum disorder (ASD) have evidence of neuroinflammation—or by definition, encephalitis. Objective: To investigate the link between autism spectrum disorder and autoimmune encephalitides. Methods: We present a case of anti-NMDA receptor encephalitis in a patient with autism spectrum disorder. “OP” is a 16-year-old male with a history of attention-deficit/ hyperactivity disorder (ADHD) and autism spectrum disorder (ASD) who presented with a 3-day history of acute-onset altered mental status, electroencephalogram (EEG)-corroborated seizures, and slurred speech. Laboratory studies were significant for serum- and cerebrospinal fluid (CSF)-positive NMDA antibodies. The child psychiatry consult-liaison service was consulted for significant agitation and behavioral dyscontrol. We recommended 1:1 observation for safety, as well as antipsychotic agents titrated to clinical effect. The patient had a protracted hospital course, but was eventually discharged to an acute rehabilitation facility for continued stabilization and therapy. Conclusion: It remains to be seen if the relation between encephalitis and ASD is uni- or bidirectional, that is: whether children with ASD have a genetic diathesis to developing encephalitides (such as those mediated by the NMDAR), or conversely, if deranged or inflamed neuroreceptor processes are implicated in the development of ASD.

Anti-NMDA-receptor encephalitis: aethology of psychiatric symptoms in young women

2011 ◽  
Vol 26 (S2) ◽  
pp. 876-876
Author(s):  
E. Marin Diaz-Guardamino ◽  
L. Larrañaga Rementeria ◽  
I. Hervella Garces ◽  
T. Aldasoro Carcedo ◽  
J. Fernandez Bilbao ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Young Women ◽  
Language Impairment ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Initial Assessment ◽  
Nmda Receptor Encephalitis ◽  
Women And Girls ◽  
Receptor Antibodies ◽  
Rule Out

IntroductionAnti-NMDA-receptor encephalitis is subacute disorder that has been recently described in young women and girls who often present neuropsychiatric symptoms as first clinical features; thus, it is common that they receive an initial assessment, diagnose or even treatment by a specialist in Psychiatry.ObjectivesPresenting a case of Anti-NMDA-receptor encephalitis that was observed at our hospital and the steps taken from its initial assessment by Psychiatry to the final diagnose and treatment by Neurology, our objective is to describe this disorder in order to make it known to the specialists in Psychiatry.MethodsThe case is that of a 14 year old girl who presents altered behavior, bizarre delusions and auditory hallucinations with subacute onset. In psychiatric assessment it is noted that the patient presents fever, slight and language impairment: Medical and Neurological assessment are requested. The steps followed to rule out other disorders are presented. SPECT and the detection of specific anti-NMDA-receptor antibodies in serum were necessary for an accurate diagnose.ResultsThe detection of specific antibodies is necessary for the diagnose of Anti-NMDA-receptor encephalitis. NMDA receptors are ligand-gated cation channels with crucial roles in synaptic transmission and plasticity. Their alteration could be a pathogenic mechanism in disorders such as epilepsy, dementia and schizophrenia.ConclusionsAnti-NMDA-receptor encephalitis is a recently described clinical entity that should be taken in consideration to rule out other causes of subacute onset psychiatric symptoms in young women and girls. Its well-defined set of clinical characteristics should become familiar to the specialists in Psychiatry.

scholarly journals Association of Autonomic Storming with Urinary Catheter Removal in NMDA Receptor Encephalitis: A Case Report

2020 ◽  
Vol 11 (1) ◽  
pp. 45-48
Author(s):  
Natalie Neale ◽  
Cody Nathan ◽  
Sok Lee ◽  
Atul Kalanuria
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Case Report ◽  
Nmda Receptor ◽  
Urinary Retention ◽  
Urinary Catheter ◽  
Altered Mental Status ◽  
Catheter Removal ◽  
Nmda Receptor Encephalitis ◽  
First Case

We present a case of a 47-year-old female who presented with altered mental status and was found to have severe anti-NMDA receptor encephalitis. Her intensive care unit course was complicated by paroxysmal sympathetic storming. She also had urinary retention for which a catheter was placed early in her admission, but attempts at removal were associated with worsening storming. Her average Clinical Features Scale score was 5.9 when the catheter was not in place compared to 3.6 with the catheter in place. This is the first case report to our knowledge demonstrating an association between urinary catheter removal and autonomic storming in anti-NMDA receptor encephalitis.

Bortezomib for treatment of anti–NMDA receptor encephalitis in a pediatric patient refractory to conventional therapy

2021 ◽  
Vol 78 (5) ◽  
pp. 395-400 ◽  
Author(s):  
Marroyln L Simmons ◽  
Kimberly A Perez
Keyword(s):  
Nmda Receptor ◽  
Clinical Improvement ◽  
Pediatric Patient ◽  
Mental Status ◽  
Clinical Status ◽  
Inpatient Rehabilitation ◽  
High Dose ◽  
Nmda Receptor Encephalitis ◽  
High Dose Methylprednisolone ◽  
Receptor Antibodies

Abstract Purpose The case of a pediatric patient with treatment refractory anti–N-methyl-d-aspartate (NMDA) receptor encephalitis treated with the plasma cell–depleting therapy bortezomib is reported. Summary A 5-year-old female presented to the hospital with a 1-week history of altered mental status, agitation, and possible seizure-like activity. She was admitted to the hospital for suspected meningitis or meningoencephalitis and an extensive workup was completed, including sending blood and cerebrospinal fluid (CSF) for testing for NMDA receptor antibodies. While test results were pending, the patient was treated initially with intravenous immunoglobulin (IVIG) for 4 days followed by high-dose methylprednisolone for 5 days. The patient’s serum and CSF studies were positive for NMDA receptor antibodies, confirming the diagnosis of anti–NMDA receptor encephalitis. She was then treated with plasmapheresis therapy every other day for 5 treatments, without any clinical improvement. The patient then received rituximab once weekly for 6 weeks. Three weeks after completion of rituximab therapy, the patient was started on her first cycle of bortezomib therapy. She received a total of 6 cycles, with improvement in her clinical status beginning with the third cycle. Upon completion of 6 cycles, the patient’s mental status and level of functioning had greatly improved. She was discharged to an inpatient rehabilitation facility and ultimately able to return home to her family. Conclusion A 5-year-old female with anti–NMDA receptor encephalitis was successfully treated with bortezomib after having shown no clinical improvement during treatment with IVIG, high-dose methylprednisolone, plasmapheresis, and rituximab.

scholarly journals Monoclonal Antibodies From Anti-NMDA Receptor Encephalitis Patient as a Tool to Study Autoimmune Seizures

2021 ◽  
Vol 15 ◽  
Author(s):  
Olga Taraschenko ◽  
Howard S. Fox ◽  
Ember Eldridge ◽  
Wenyi Wang ◽  
Samuel W. Dowd ◽  
...  
Keyword(s):  
Monoclonal Antibodies ◽  
Nmda Receptor ◽  
Clinical Manifestations ◽  
Continuous Administration ◽  
Nmda Receptor Encephalitis ◽  
Cortical Screw ◽  
Abnormal Movements ◽  
Continuous Eeg ◽  
Ccl2 Mrna ◽  
Receptor Antibodies

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis manifests with precipitous cognitive decline, abnormal movements, and severe seizures that can be challenging to control with conventional anti-seizure medications. We previously demonstrated that intracerebroventricular (i.c.v.) administration of cerebrospinal fluid from affected patients, or purified NMDA receptor antibodies from encephalitis patients to mice precipitated seizures, thereby confirming that antibodies are directly pathogenic for seizures. Although different repertoires of anti-NMDA receptor antibodies could contribute to the distinct clinical manifestations in encephalitis patients, the role of specific antibodies in the expression of seizure, motor, and cognitive phenotypes remains unclear. Using three different patient-derived monoclonal antibodies with distinct epitopes within the N-terminal domain (NTD) of the NMDA receptor, we characterized the seizure burden, motor activity and anxiety-related behavior in mice. We found that continuous administration of 5F5, 2G6 or 3C11 antibodies for 2 weeks precipitated seizures, as measured with continuous EEG using cortical screw electrodes. The seizure burden was comparable in all three antibody-treated groups. The seizures were accompanied by increased hippocampal C-C chemokine ligand 2 (CCL2) mRNA expression 3 days after antibody infusion had stopped. Antibodies did not affect the motor performance or anxiety scores in mice. These findings suggest that neuronal antibodies targeting different epitopes within the NMDA receptor may result in a similar seizure phenotype.

scholarly journals Anti-NMDA Receptor Encephalitis in a Patient with Previous Psychosis and Neurological Abnormalities: A Diagnostic Challenge

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
R. David Heekin ◽  
Maria C. Catalano ◽  
Alfred T. Frontera ◽  
Glenn Catalano
Keyword(s):  
Nmda Receptor ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Memory Loss ◽  
Autoimmune Disorder ◽  
Diagnostic Challenge ◽  
Manic Symptoms ◽  
Nmda Receptor Encephalitis ◽  
Autonomic Instability ◽  
Receptor Antibodies

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an autoimmune disorder characterized by IgG autoantibodies directed against the NR1 subunit of the NMDA glutamate receptor. Psychiatric symptoms are common and include psychosis, mania, depressed mood, aggression, and speech abnormalities. Neurological symptoms such as seizures, decreased responsiveness, dyskinesias, and other movement abnormalities and/or autonomic instability are frequently seen as well. We present the case of a woman who was followed up at our facility for over 14 years for the treatment of multiple neuropsychiatric symptoms. Initially, she presented with paresthesias, memory loss, and manic symptoms. Nine years later, she presented to our facility again, this time with left sided numbness, left eyelid droop, and word finding difficulties. Finally, five years later, she presented with manic symptoms, hallucinations, and memory impairment. During her hospitalization, she subsequently developed catatonic symptoms and seizures. During her stay, it was discovered that she was positive for anti-NMDA receptor antibodies and her symptoms responded well to appropriate therapy. This case demonstrates that it may be useful for clinicians to consider screening for anti-NMDA receptor antibodies in long-term patients with neuropsychiatric symptoms that have not adequately responded to therapy.

scholarly journals Rate of Anti-NMDA Receptor Encephalitis in Ovarian Teratomas

2021 ◽  
Author(s):  
Jennifer H. Li ◽  
Sarah S. Milla ◽  
Grace Y. Gombolay
Keyword(s):  
Cohort Study ◽  
Nmda Receptor ◽  
Retrospective Cohort ◽  
Multiple Linear Regression Model ◽  
Ovarian Teratoma ◽  
Histopathological Diagnosis ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor ◽  
Receptor Antibodies ◽  
Ovarian Teratomas

Abstract Background The rate of anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) in ovarian teratomas is unknown. We aim to identify the prevalence of NMDARE as well as volumetric and histopathologic characteristics of ovarian teratomas in patients with versus without. Methods We performed a retrospective cohort study to identify patients with confirmed ovarian teratomas and the characteristics of teratomas in NMDARE compared with non-NMDARE patients. Patients aged between 0 and 21 years with confirmed histopathological diagnosis of ovarian teratoma after resection were included. The rate of NMDARE in ovarian teratomas was identified. Moreover, volumes of ovarian teratomas and the frequency of neuronal glial elements on histopathology in NMDARE versus non-NMDARE patients were assessed. Results Five out of one-hundred-and-sixty-three (3.07%) patients with histopathology confirmed ovarian teratomas were diagnosed with NMDARE. Age was not different between the NMDARE (mean: 13.8 years, standard deviation: 3.9) and non-NMDARE groups (median: 14, interquartile range [IQR]: 5). Teratoma volumes from NMDARE patients were smaller than those of non-NMDARE patients (median 28.3 cm3 with IQR of 431.2 and median 182.8 with IQR of 635.0, respectively). Both age and NMDARE diagnosis were statistically significant variables in the analysis of variance on a multiple linear regression model. Age (p = 0.013) had a positive correlation with teratoma size, whereas presence of NMDARE had a negative correlation (p = 0.008). Conclusion The rate of NMDARE in ovarian teratomas is low and NMDARE patients have smaller teratomas than non-NMDARE. Further studies are needed to understand the timing of anti-NMDA receptor antibodies in teratomas and the development of NMDARE.

scholarly journals Solitary juxtacortical lesion associated with anti-N-methyl-D-aspartate receptor encephalitis: a case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Rupan Gao ◽  
Xiang Zhang ◽  
Abhijeet Kumar Bhekharee ◽  
Yue Zhang
Keyword(s):  
Nmda Receptor ◽  
Frontal Lobe ◽  
Brain Mri ◽  
Brain Lesion ◽  
Autoimmune Encephalitis ◽  
Left Frontal Lobe ◽  
Solitary Lesion ◽  
Nmda Receptor Encephalitis ◽  
Epileptiform Discharges ◽  
Receptor Antibodies

Abstract Background Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe autoimmune encephalitis mediated by anti-NMDA receptor antibodies. Brain MRI manifestations vary and are non-specific. If there are any lesions, they tend to be diffusely or multifocally distributed. Solitary lesion is relatively rare. Case presentation We report a 16-year-old girl who initially presented with focal seizures but developed severe psychiatric and extrapyramidal symptoms later on. Brain MRI revealed a solitary juxtacortical demyelinating lesion in the left frontal lobe. No enhancement was noted. Electroencephalogram captured epileptiform discharges in the same region. NMDAR IgGs were tested positive in the serum and cerebrospinal fluid. Corticosteroid and intravenous IgG were administered and the patient completely recovered. Brain MRI revealed a fainter lesion in the left frontal lobe. Conclusion In very rare instances, anti-NMDA receptor encephalitis can present with a solitary brain lesion. A full panel of antibodies for autoimmune encephalitis is the key leading to the diagnosis.

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