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Anti-NMDA Receptor Encephalitis
NMDA Receptor Antibody Encephalitis

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WIKIDATA, CC BY-SA 4.0

Presentation

Infectious disease work-up was negative and anti-NMDA receptor antibodies were present in the CSF and serum. [ncbi.nlm.nih.gov]

Unlike the presentation in adults, in children, it is less frequently associated with tumors but, movement disorders are more common. Here, we present two cases of children with anti-R NMDA encephalitis. [pesquisa.bvsalud.org]

Entire Body System

  • Fever

    She developed disorientation and hallucination after fever and vomiting. Hypothermia, hypoventilation, hypertension, paralytic ileus and hyponatremia were present. [ncbi.nlm.nih.gov]

  • Hyperthermia

    Keywords: NMDA, receptor, autoimmune, synapse, paraneoplastic, schizophrenia, paraneoplastic disorder, neurologic symptoms, hyperthermia, hypoventilation, hypersexuality, hyperphagia, hypersomnia, Amnesia, Corticosteriods Abstract: Anti-NMDA receptor [dx.doi.org]

    Tachycardia and bradycardia, blood pressure lability, hyperthermia, hypoventilation, and/or apnea were each reported in most patients with autonomic instability. [ncbi.nlm.nih.gov]

  • Fatigue

    Median duration since diagnosis was similar for children (2.42 years, interquartile range 2.12-3.32) and adults (3.55 years, interquartile range 2.08-5.50 years). 3/4 (75%) pediatric and 3/5 (60%) adult patients reported neuropsychiatric symptoms (fatigue [ncbi.nlm.nih.gov]

    […] receptor encephalitis have the presence of a tumour (most commonly teratoma) CLINICAL PRESENTATION Up to 90% of patients are female The disorder typically progresses over 1-2 weeks An initial non-specific flu-like prodrome (sub-febrile temperature, headache, fatigue [lifeinthefastlane.com]

    In the pro-dromal phase, affected individuals have a flu-like illness with fever, malaise, headache, and fatigue. [dx.doi.org]

  • Unconsciousness

    It is a life threatening condition that typically presents with viral illness, headaches, severe psychiatric symptoms, seizures, behavioural changes, decreasing levels of unconsciousness and progressive unresponsiveness, cognitive impairment, abnormal [ncbi.nlm.nih.gov]

    Their functions are critical for judgement, perception of reality, human interaction, the formation and retrieval of memory, and the control of unconscious activities (such as breathing, swallowing, etc), also known as autonomic functions. [antinmdafoundation.org]

  • Malaise

    In the pro-dromal phase, affected individuals have a flu-like illness with fever, malaise, headache, and fatigue. [dx.doi.org]

Respiratoric

  • Cough

    During this first round of IVIg treatment, the patient continued to have periods of vital sign instability and developed a new cough, with decreased ability to protect his airway. [dx.doi.org]

Gastrointestinal

  • Nausea

    She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness. [ncbi.nlm.nih.gov]

Cardiovascular

  • Tachycardia

    Tachycardia and bradycardia, blood pressure lability, hyperthermia, hypoventilation, and/or apnea were each reported in most patients with autonomic instability. [ncbi.nlm.nih.gov]

    Autonomic instability, evidenced by blood pressure and temperature fluctuations, tachycardia, bradycardia, and even cardiac pauses, is not uncommon. [dx.doi.org]

    These include: Mild neurologic symptoms such as twitches, catatonia, mutism, seizures Extrapyramidal symptoms when treated with antipsychotics Tachycardia, fever or elevation of the blood pressure, which are signs of autonomic disturbance It is also important [news-medical.net]

  • Hypertension

    Hypothermia, hypoventilation, hypertension, paralytic ileus and hyponatremia were present. Neurological examination showed mild consciousness disturbance and bilateral ophthalmoplegia on admission, flaccid paraplegia with leg areflexia on Day 4. [ncbi.nlm.nih.gov]

    Enalapril was prescribed to manage hypertension; the patient had consistent systolic readings in the 150–170 mm Hg range and diastolic readings in the 80–90 mm Hg range. [dx.doi.org]

Psychiatrical

  • Delusion

    Here, we present a case of a 15 year-old female adolescent with first-episode psychosis with anti-NMDA receptor encephalitis not related to tumor, which manifested with delusion, hallucinations, panic attacks, agitation, and neurological symptoms, and [ncbi.nlm.nih.gov]

  • Impulsivity

    Similarly, mood dysregulation disorders, disorders of impulse control, and sleep disorders may be identified. [dx.doi.org]

    NMDA receptors are proteins that control electrical impulses in the brain. [antinmdafoundation.org]

  • Aggressive Behavior

    A previously healthy 22-year-old Asian male demonstrated extremely aggressive behavior (eg, he had thrown someone through a glass door on the day of admission) and was initially admitted for psychosis. [doi.org]

    Warning signs in ‘new-onset psychosis’ In a few cases, psychiatric symptoms usually in the form of manic symptoms are the only features, these include delusions, aggressive behavior and mood disorders. [news-medical.net]

  • Disorganized Behavior

    Psychiatric manifestations include anxiety, mania, social withdrawal, and psychosis (i.e., delusions, hallucinations, disorganized behavior). [ncbi.nlm.nih.gov]

  • Paranoid Ideation

    There is a dearth of information regarding management of psychiatric and behavioral symptoms, including agitation, anxiety, mood dysregulation, depression, disorganized thinking, paranoid ideation, and hallucinations. [dx.doi.org]

Face, Head & Neck

  • Facial Grimacing

    Examination on the day of admission revealed an awake, alert, and interactive male with focal speech production difficulties, asymmetric facial grimace, hyperactive deep tendon reflexes, and normal sensation. [dx.doi.org]

Neurologic

  • Seizure

    A previously healthy 14-year-old girl presenting with generalized tonic clonic seizures and altered behavior. [ncbi.nlm.nih.gov]

  • Catatonia

    Malignant catatonia is a severe and life-threatening state associated with anti-NMDA receptor encephalitis. It is often inadequately assessed and may not respond to immunosuppressive treatment. [ncbi.nlm.nih.gov]

  • Headache

    It is an immune-mediated syndrome which presents with a variety of neurological symptoms including headache, fever, personality change and seizures. Most case reports to date are of young adults, it is much less frequently reported in older adults. [ncbi.nlm.nih.gov]

    […] anti-NMDA receptor encephalitis have the presence of a tumour (most commonly teratoma) CLINICAL PRESENTATION Up to 90% of patients are female The disorder typically progresses over 1-2 weeks An initial non-specific flu-like prodrome (sub-febrile temperature, headache [lifeinthefastlane.com]

  • Agitation

    Here, we present a case of a 15 year-old female adolescent with first-episode psychosis with anti-NMDA receptor encephalitis not related to tumor, which manifested with delusion, hallucinations, panic attacks, agitation, and neurological symptoms, and [ncbi.nlm.nih.gov]

    His affect fluctuated between flat, agitated, and appropriately calm. [dx.doi.org]

  • Confusion

    Recognition of the syndrome, especially in pediatric populations, is difficult and often undiagnosed and/or confused with neurological disorders with similar clinical features. [ncbi.nlm.nih.gov]

    PRESENTATION Up to 90% of patients are female The disorder typically progresses over 1-2 weeks An initial non-specific flu-like prodrome (sub-febrile temperature, headache, fatigue) followed by a psychotic stage with bizarre behaviour, disorientation, confusion [lifeinthefastlane.com]

    Flu-like symptoms Memory deficits, including loss of short-term memory; Sleep disorders; Speech dysfunction – the patient is no longer able to produce coherent language or may be completely unable to communicate Cognitive and behavioural disturbances – confused [antinmdafoundation.org]

    People are also often agitated or confused. [en.wikipedia.org]

Workup

Workup revealed antibodies against N-methyl-d-aspartate receptor (NMDAR). Immunotherapy treatment led to a very significant improvement with the patient presenting only slight frontal lobe dysfunction after one year of recovery. [ncbi.nlm.nih.gov]

Example of Workup Prior to Diagnosis of Anti-NMDAR Encephalitis If the diagnosis of anti-NMDAR encephalitis is not considered, the workup often includes an extensive study for infectious diseases. [doi.org]

EEG

  • Abnormal EEG

    A total of 93 instances of EEG monitoring were performed on 62 patients. At the peak stage, EEG presentations showed 61 cases (98.4%) were abnormal, cranial MRI showed 29 cases (46.8%) were abnormal among all 62 patients. [ncbi.nlm.nih.gov]

Treatment

Here, we report our experience with two pediatric patients, in whom no tumors were present during treatment for encephalitis, but in whom ovarian teratomas developed without encephalitis relapse after treatment was completed. [ncbi.nlm.nih.gov]

Prognosis

EEG could sensitively reflect the abnormal brain functions of patients and could assist with early clinical diagnosis and prognosis prediction. [ncbi.nlm.nih.gov]

Etiology

Introduction The causes of encephalitis are numerous, and most patients undergo extensive testing to identify infectious etiologies. However, other etiologies need to be considered. [doi.org]

Abstract The California Encephalitis Project (CEP), established in 1998 to explore encephalitic etiologies, has identified patients with N-methyl-D-aspartate receptor (NMDAR) antibodies, the likely etiology of their encephalitis. [ncbi.nlm.nih.gov]

Epidemiology

He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education. [lifeinthefastlane.com]

In 2007, the California Encephalitis Project (CEP), which was established to study the epidemiology of encephalitis, began identifying cases of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. [ncbi.nlm.nih.gov]

The California Encephalitis Project (CEP) was initiated in 1998 to identify the etiologic agents and define the clinical and epidemiologic characteristics associated with encephalitis. [doi.org]

We present a review of the literature on the disorder, including its clinical presentation, differential diagnosis, epidemiology, treatment and prognosis. [dx.doi.org]

Pathophysiology

These observations suggest that NMDA receptors may play a role in the pathophysiology of CNH. [ncbi.nlm.nih.gov]

[Anti-nMDA receptor encephalitis--clinical manifestations and pathophysiology]. T. IizukaF. [semanticscholar.org]

Prevention

No analysis of relapse risk has yet been reported in cases of tumor development after treatment, and at this point, whether or not resection is needed to prevent relapse remains unclear. [ncbi.nlm.nih.gov]

In fact, there are no studies to show that these drugs are effective in the acute stage or are useful to prevent relapses. We definitely need multicenter prospective studies to evaluate the efficacy of all these immunotherapies. [dx.doi.org]

Immune suppressing medications may be taken for prolonged periods of time (even after recovery) to prevent a relapse. [antinmdafoundation.org]

This cellular filter, separating the central nervous system from the circulatory system, normally prevents larger molecules from entering the brain. [en.wikipedia.org]

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