Presentation
Infectious disease work-up was negative and anti-NMDA receptor antibodies were present in the CSF and serum. [ncbi.nlm.nih.gov]
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
[…] 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]
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]
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]
- Hodgkin Lymphoma
Among the 13 males (age range 4–59, median 23 years) there was only one tumour, a recurrence of a previously-treated Hodgkin’s lymphoma at 49 years of age. [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]
MV, PEG Incomprehensible speech, choreoathetotic movements, hypersomnia, hyperthermia, autonomic instability 11 4, 7 F/28 1st episode: mature, right ovary, 14cm; 2nd episode: “benign,” left, 2cm; 3rd episode: “benign,” left, 1.7cm 1 month after tumor Cough [ncbi.nlm.nih.gov]
Gastrointestinal
- Nausea
She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness. [ncbi.nlm.nih.gov]
It follows a specific multistep clinical pattern starting with prodromal signs (such as headache, nausea, low-grade fever) and psychosis (hallucinations, delusions, suicidal thoughts) progressing to epileptic seizures, reduced levels of consciousness, [nature.com]
- Diarrhea
[…] tonic-clonic seizures, partial motor seizures, severe general encephalopathy, PEG Unable to speak and follow commands for 2 months; episodes of “facial twitches” 6 F/24 Mature, right ovary, 1.5cm 3 months (autopsy) Headache, hyperthermia, nausea, vomiting, diarrhea [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]
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]
Autonomic instability, evidenced by blood pressure and temperature fluctuations, tachycardia, bradycardia, and even cardiac pauses, is not uncommon. [dx.doi.org]
- 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]
- Hypotension
Five patients had signs of autonomic instability, including episodes of mydriasis, tachycardia, tachypnea, diaphoresis, or hypertension, which in one case alternated with hypotension. [ncbi.nlm.nih.gov]
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
In humans with this autoimmune disease, rogue antibodies bind to the NMDA receptors on neurons, impairing their ability to control electrical impulses in the brain. [smithsonianmag.com]
NMDA receptors are proteins that control electrical impulses in the brain. [antinmdafoundation.org]
Similarly, mood dysregulation disorders, disorders of impulse control, and sleep disorders may be identified. [dx.doi.org]
After discharge, 64 (85%) of the 75 patients who were left with mild deficits or eventually attained full recovery had signs of frontal-lobe dysfunction including poor attention and planning, impulsivity, and behavioral dysinhibition; 20 (27%) had prominent [ncbi.nlm.nih.gov]
- Aggressive Behavior
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]
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]
Disturbances of memory, behavior, cognition, and seizures can result from immune-mediated encephalitis. [ncbi.nlm.nih.gov]
- Withdrawn
During this time the patient also showed significant mood lability, laughing uncontrollably at times, and at others being irritable, withdrawn, and sullen. [dx.doi.org]
I became more withdrawn. I found it hard to walk with my father and mother around the hospital floor. Close friends were alarmed at my passive and incoherent state. Doctors secretly feared that they were losing me. [nypost.com]
- Disorganized Behavior
Psychiatric manifestations include anxiety, mania, social withdrawal, and psychosis (i.e., delusions, hallucinations, disorganized behavior). [ncbi.nlm.nih.gov]
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]
Urogenital
- Incontinence
[…] who had been in his usual state of good health until 4 days prior to admission presented with seizure-like episodes that included stiffening followed by rhythmic jerking of the entire body, right-sided facial twitching, and chewing without associated incontinence [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]
Visitors watched in confused horror as Knut drowned. In the wake of Knut’s death, hundreds of fans left stuffed animals and flowers near the deceased bear’s cage, and the mayor of Berlin even issued a public statement mourning his passing. [smithsonianmag.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]
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]
From Day 56, the patient recovered; and (e) normal EEG appearances recurred in sections in between abnormally high waves. [doi.org]
Treatment
The paper reviews the main clinical features and differential diagnosis, and provides a diagnostic algorithm as well as a flow chart for treatment in paediatric age. [moh-it.pure.elsevier.com]
TREATMENT IMPLICATIONS: Early diagnosis and aggressive treatment promote better outcomes. First-line treatment includes immunotherapy. © 2015 Wiley Periodicals, Inc. [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
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]
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]
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
The similarities identified between anti-NMDA-R encephalitis and schizophrenia-spectrum illnesses also raise questions about a common underlying pathophysiology particularly in regard to the NMDA-R. [ncbi.nlm.nih.gov]
Prevention
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]
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]
Immune suppressing medications may be taken for prolonged periods of time (even after recovery) to prevent a relapse. [antinmdafoundation.org]
To prevent this, an individual with a positive serum antibody test result should have it confirmed by an assay of a cerebrospinal fluid (CSF) sample, because this is 100% sensitive and specific. [news-medical.net]