Anti-NMDA receptor encephalitis: Difference between revisions
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==Background== | ==Background== | ||
Anti-NMDA receptor encephalitis is an under-recognized neurologic described disorder described in 2007 due to antibodies to the NMDA receptor and is often associated with | Anti-NMDA receptor encephalitis is an under-recognized neurologic described disorder described in 2007 due to antibodies to the NMDA receptor and is often associated with GYN tumors (most commonly ovarian teratoma)<ref>Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; Dec;7(12); 191-8. PMID: 18851928</ref> | ||
==Clinical Features== | ==Clinical Features== | ||
===History=== | ===History=== | ||
* Female predominance (up to 80-90%) | *Female predominance (up to 80-90%) | ||
* Predominantly in children and young adults, however can be found at any age<ref name="clinical">Dalmau J, Lancaster E, Martinez-Hernandez E, et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011 Jan;10(1):63-47. PMID: 21163445</ref><ref>Armangue T, Petit-Pedrol M, Dalmau J Autoimmune Encephalitis in Children. J Child Neurol. 2012 Nov;27(11):1460-9. PMID: 2293555</ref> | *Predominantly in children and young adults, however can be found at any age<ref name="clinical">Dalmau J, Lancaster E, Martinez-Hernandez E, et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011 Jan;10(1):63-47. PMID: 21163445</ref><ref>Armangue T, Petit-Pedrol M, Dalmau J Autoimmune Encephalitis in Children. J Child Neurol. 2012 Nov;27(11):1460-9. PMID: 2293555</ref> | ||
* Viral like prodrome ( | *[[viral syndrome|Viral like prodrome]] ([[headache]], low-grade [[fever]], malaise) | ||
* Psychiatric manifestations (anxiety, agitation, bizarre behavior, hallucinations, etc)and or decreased level of consciousness <ref name="clinical"></ref> | *Psychiatric manifestations ([[anxiety]], [[agitation]], bizarre behavior, [[hallucinations]], etc) and/or [[AMS|decreased level of consciousness]] <ref name="clinical"></ref> | ||
* Dyskinesia, movement disorders and increased rigidity | *Dyskinesia, movement disorders and increased rigidity | ||
* Autonomic instability: hyperthermia, | *Autonomic instability: [[hyperthermia]], [[tachycardia]]/[[bradycardia]], BP fluctuations, hypoventilation | ||
* Lethargy, seizures | *[[Lethargy]], [[seizures]] | ||
===Physical=== | ===Physical=== | ||
* Abnormality in vitals as above, rarely may find abdominal mass | *Abnormality in vitals as above, rarely may find abdominal mass | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Viral encephalitis]] | *[[Viral encephalitis]] | ||
| Line 17: | Line 19: | ||
*Primary psychiatric disorder | *Primary psychiatric disorder | ||
*[[Hyperthyroidism|Hyper]]/[[hypothyroidism]] | *[[Hyperthyroidism|Hyper]]/[[hypothyroidism]] | ||
*[[ | *[[Cushing syndrome]] | ||
*[[Addison's disease]]<ref name="Wadinger">Wandinger K, Saschenbrecker S, Stoecker W, Dalmau J Anti-NMDA-receptor encephalitis: A severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol. 2011 Feb;231(1-2):86-91. PMID: 20951441</ref> | *[[Addison's disease]]<ref name="Wadinger">Wandinger K, Saschenbrecker S, Stoecker W, Dalmau J Anti-NMDA-receptor encephalitis: A severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol. 2011 Feb;231(1-2):86-91. PMID: 20951441</ref> | ||
*[[Catatonia]]<ref name="Wadinger"></ref> | *[[Catatonia]]<ref name="Wadinger"></ref> | ||
*Cerebral space occupying lesions | *[[intracranial mass|Cerebral space occupying lesions]] | ||
*[[Drugs]], [[toxins]], [[Alcohol withdrawal|withdrawal]]<ref>Punja M, Pomerleau JJ, Devlin MW, et al. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: an etiology worth considering in the differential diagnosis of delirium. Clin Toxicol 2013 Sep-Oct;51:794-7. PMID: 23962100</ref> | *[[Drugs]], [[toxins]], [[Alcohol withdrawal|withdrawal]]<ref>Punja M, Pomerleau JJ, Devlin MW, et al. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: an etiology worth considering in the differential diagnosis of delirium. Clin Toxicol 2013 Sep-Oct;51:794-7. PMID: 23962100</ref> | ||
== | {{Movement disorder DDX}} | ||
* Diagnosis confirmed by detection of antibodies to NR1 subunit of NMDAR in CSF or serum (typically send-out lab) | {{AMS DDX}} | ||
* LP: CSF lymphocytic pleocytosis or oligoclonal bands (can be normal initially) | |||
* EEG: to rule out seizure with movement disorders | ==Evaluation== | ||
* MRI brain: normal or transient FLAIR or contrast enhancing abnormalities in cortical or subcortical regions | *Diagnosis confirmed by detection of antibodies to NR1 subunit of NMDAR in CSF or serum (typically send-out lab) | ||
* Pelvic | *[[LP]]: CSF lymphocytic pleocytosis or oligoclonal bands (can be normal initially) | ||
*EEG: to rule out seizure with movement disorders | |||
*MRI brain: normal or transient FLAIR or contrast enhancing abnormalities in cortical or subcortical regions | |||
*Pelvic [[ultrasound]] or CT or MRI to evaluate for associated ovarian teratoma | |||
==Management== | ==Management== | ||
* Resection of tumor if there is an associated mass<ref name="treatment">Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013 Feb;12(2):157-65. PMID: 23290630 | *Resection of tumor if there is an associated mass<ref name="treatment">Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013 Feb;12(2):157-65. PMID: 23290630 | ||
</ref> | </ref> | ||
* | *[[Corticosteroids]]<ref name="treatment"></ref> | ||
*IVIG<ref name="treatment"></ref> | *[[IVIG]]<ref name="treatment"></ref> | ||
*Plasma exchange | *[[Plasma exchange]] | ||
*Second line rituximab and cyclophosphamide<ref name="treatment"></ref> | *Second line: [[rituximab]] and [[cyclophosphamide]]<ref name="treatment"></ref> | ||
==Disposition== | ==Disposition== | ||
* Admission with | *Admission with neurology consult | ||
==See Also== | ==See Also== | ||
*[[Altered mental status]] | *[[Altered mental status]] | ||
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*[http://www.antinmdafoundation.org/what-is-anti-nmda-receptor-encephalitis.html Anti-NMDA Foundation] | *[http://www.antinmdafoundation.org/what-is-anti-nmda-receptor-encephalitis.html Anti-NMDA Foundation] | ||
== | ==References== | ||
<references/> | <references/> | ||
[[Category: | [[Category:Neurology]] | ||
Latest revision as of 13:46, 14 November 2020
Background
Anti-NMDA receptor encephalitis is an under-recognized neurologic described disorder described in 2007 due to antibodies to the NMDA receptor and is often associated with GYN tumors (most commonly ovarian teratoma)[1]
Clinical Features
History
- Female predominance (up to 80-90%)
- Predominantly in children and young adults, however can be found at any age[2][3]
- Viral like prodrome (headache, low-grade fever, malaise)
- Psychiatric manifestations (anxiety, agitation, bizarre behavior, hallucinations, etc) and/or decreased level of consciousness [2]
- Dyskinesia, movement disorders and increased rigidity
- Autonomic instability: hyperthermia, tachycardia/bradycardia, BP fluctuations, hypoventilation
- Lethargy, seizures
Physical
- Abnormality in vitals as above, rarely may find abdominal mass
Differential Diagnosis
- Viral encephalitis
- Neuroleptic malignant syndrome
- Primary psychiatric disorder
- Hyper/hypothyroidism
- Cushing syndrome
- Addison's disease[4]
- Catatonia[4]
- Cerebral space occupying lesions
- Drugs, toxins, withdrawal[5]
Movement Disorders and Other Abnormal Contractions
- Chorea
- Neuroleptic malignant syndrome
- Serotonin syndrome
- Hypocalcemia
- Strychnine toxicity
- Acute tetanus
- Parkinson's disease
- Mono amine oxidase inhibitor toxicity
- Phencyclidine toxicity
- Anti-NMDA receptor encephalitis
- Huntington disease
- Wilson's disease
- CVA
- Schizophrenia
- Psychotic agitation
- Dementia
- Lewy body dementia
- Vascular dementia
- Frontotemporal dementia
- Dystonic reaction
- Extrapyramidal reaction
- Torticollis
- Idiopathic movement disorder
Altered mental status
Diffuse brain dysfunction
- Hypoxic encephalopathy
- Acute toxic-metabolic encephalopathy (Delirium)
- Hypoglycemia
- Hyperosmolar state (e.g., hyperglycemia)
- Electrolyte Abnormalities (hypernatremia or hyponatremia, hypercalcemia)
- Organ system failure
- Hepatic Encephalopathy
- Uremia/Renal Failure
- Endocrine (Addison's disease, Cushing syndrome, hypothyroidism, myxedema coma, thyroid storm)
- Hypoxia
- CO2 narcosis
- Hypertensive Encephalopathy
- Toxins
- TTP / Thrombotic thrombocytopenic purpura
- Alcohol withdrawal
- Drug reactions (NMS, Serotonin Syndrome)
- Environmental causes
- Deficiency state
- Wernicke encephalopathy
- Subacute Combined Degeneration of Spinal Cord (B12 deficiency)
- Vitamin D Deficiency
- Zinc Deficiency
- Sepsis
- Osmotic demyelination syndrome (central pontine myelinolysis)
- Limbic encephalitis
Primary CNS disease or trauma
- Direct CNS trauma
- Diffuse axonal injury
- Subdural/epidural hematoma
- Vascular disease
- SAH
- Stroke
- Hemispheric, brainstem
- CNS infections
- Neoplasms
- Paraneoplastic Limbic encephalitis
- Malignant Meningitis
- Pancreatic Insulinoma
- Seizures
- Nonconvulsive status epilepticus
- Postictal state
- Dementia
Psychiatric
Evaluation
- Diagnosis confirmed by detection of antibodies to NR1 subunit of NMDAR in CSF or serum (typically send-out lab)
- LP: CSF lymphocytic pleocytosis or oligoclonal bands (can be normal initially)
- EEG: to rule out seizure with movement disorders
- MRI brain: normal or transient FLAIR or contrast enhancing abnormalities in cortical or subcortical regions
- Pelvic ultrasound or CT or MRI to evaluate for associated ovarian teratoma
Management
- Resection of tumor if there is an associated mass[6]
- Corticosteroids[6]
- IVIG[6]
- Plasma exchange
- Second line: rituximab and cyclophosphamide[6]
Disposition
- Admission with neurology consult
See Also
External Links
References
- ↑ Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; Dec;7(12); 191-8. PMID: 18851928
- ↑ 2.0 2.1 Dalmau J, Lancaster E, Martinez-Hernandez E, et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011 Jan;10(1):63-47. PMID: 21163445
- ↑ Armangue T, Petit-Pedrol M, Dalmau J Autoimmune Encephalitis in Children. J Child Neurol. 2012 Nov;27(11):1460-9. PMID: 2293555
- ↑ 4.0 4.1 Wandinger K, Saschenbrecker S, Stoecker W, Dalmau J Anti-NMDA-receptor encephalitis: A severe, multistage, treatable disorder presenting with psychosis. J Neuroimmunol. 2011 Feb;231(1-2):86-91. PMID: 20951441
- ↑ Punja M, Pomerleau JJ, Devlin MW, et al. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: an etiology worth considering in the differential diagnosis of delirium. Clin Toxicol 2013 Sep-Oct;51:794-7. PMID: 23962100
- ↑ 6.0 6.1 6.2 6.3 Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013 Feb;12(2):157-65. PMID: 23290630