Difference between revisions of "Anti-NMDA receptor encephalitis"
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| − | + | * Generally, affects younger individuals (95% younger than 45, 37% younger than 18) | |
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* Typically, presentation begins with abnormal behavior (psychosis, delusions, hallucinations, agitation, aggression, or catatonia) with irritability and insomnia | * Typically, presentation begins with abnormal behavior (psychosis, delusions, hallucinations, agitation, aggression, or catatonia) with irritability and insomnia | ||
* Over weeks followed by speech dysfunction, dyskinesias, memory deficits, autonomic stability, and decreased level of consciousness. | * Over weeks followed by speech dysfunction, dyskinesias, memory deficits, autonomic stability, and decreased level of consciousness. | ||
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Patients with relapsing symptoms s/p herpes simplex encephalitis should also be tested for NMDA receptor antibodies, which is an autoimmune reaction affecting 20% of patients with HSE, manifesting as either new onset choreoathetosis or psychiatric symptoms a few weeks after viral infection. | Patients with relapsing symptoms s/p herpes simplex encephalitis should also be tested for NMDA receptor antibodies, which is an autoimmune reaction affecting 20% of patients with HSE, manifesting as either new onset choreoathetosis or psychiatric symptoms a few weeks after viral infection. | ||
| + | ==Diagnosis== | ||
| − | ''' | + | ''Diagnostic criteria for '''probable''' anti-NMDA receptor encephalitis'' |
| − | + | # Rapid onset (<3 months) of at least 4 of the 6 following major groups of symptoms: | |
| − | + | ## Abnormal (psychiatric) behavior or cognitive dysfunction | |
| − | + | ## Speech dysfunction (pressured, verbal reduction, mutism) | |
| − | + | ## Seizures | |
| − | + | ## Movement disorder, dyskinesias, or rigidity/abnormal postures | |
| − | + | ## Decreased level of consciousness | |
| − | + | ## Autonomic dysfunction or central hypoventilation | |
| − | + | # At least one of the following laboratory study results: | |
| − | + | ## Abnormal EEG (focal or diffuse slow or disorganized activity, epileptic activity, or extreme delta brush) | |
| − | + | ## CSF with pleocytosis or oligoclonal bands | |
| − | + | # Reasonable exclusion of other disorders | |
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| − | ''Diagnostic criteria for definite anti-NMDA receptor encephalitis'' | + | ''Diagnostic criteria for '''definite''' anti-NMDA receptor encephalitis'' |
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| + | # Diagnosis can be made in the presence of one or more of the six major groups of symptoms and IgG anti-GluN1 antibodies (CSF), after reasonable exclusion of other disorders | ||
== References == | == References == | ||
Graus, F. et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 15, 391–404 (2016). https://pubmed.ncbi.nlm.nih.gov/26906964/ | Graus, F. et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 15, 391–404 (2016). https://pubmed.ncbi.nlm.nih.gov/26906964/ | ||
Latest revision as of 13:52, 29 June 2021
- Generally, affects younger individuals (95% younger than 45, 37% younger than 18)
- Typically, presentation begins with abnormal behavior (psychosis, delusions, hallucinations, agitation, aggression, or catatonia) with irritability and insomnia
- Over weeks followed by speech dysfunction, dyskinesias, memory deficits, autonomic stability, and decreased level of consciousness.
Patients with relapsing symptoms s/p herpes simplex encephalitis should also be tested for NMDA receptor antibodies, which is an autoimmune reaction affecting 20% of patients with HSE, manifesting as either new onset choreoathetosis or psychiatric symptoms a few weeks after viral infection.
Diagnosis
Diagnostic criteria for probable anti-NMDA receptor encephalitis
- Rapid onset (<3 months) of at least 4 of the 6 following major groups of symptoms:
- Abnormal (psychiatric) behavior or cognitive dysfunction
- Speech dysfunction (pressured, verbal reduction, mutism)
- Seizures
- Movement disorder, dyskinesias, or rigidity/abnormal postures
- Decreased level of consciousness
- Autonomic dysfunction or central hypoventilation
- At least one of the following laboratory study results:
- Abnormal EEG (focal or diffuse slow or disorganized activity, epileptic activity, or extreme delta brush)
- CSF with pleocytosis or oligoclonal bands
- Reasonable exclusion of other disorders
Diagnosis can also be made in the presence of three of the above groups of symptoms accompanied by a systemic teratoma.
Diagnostic criteria for definite anti-NMDA receptor encephalitis
- Diagnosis can be made in the presence of one or more of the six major groups of symptoms and IgG anti-GluN1 antibodies (CSF), after reasonable exclusion of other disorders
References
Graus, F. et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 15, 391–404 (2016). https://pubmed.ncbi.nlm.nih.gov/26906964/
