Anti-NMDA receptor encephalitis: Difference between revisions
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==Background== | ==Background== | ||
Anti-NMDA receptor encephalitis is an under-recognized neurologic disorder due to antibodies to the NMDA receptor and is often associated with a | 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 a 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 | * 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 (HA, low-grade fever, malaise) | * Viral like prodrome (HA, low-grade fever, malaise) | ||
* Psychiatric manifestations (anxiety, agitation, bizarre behavior, hallucinations, etc)and or decreased level of consciousness< | * Psychiatric manifestations (anxiety, agitation, bizarre behavior, hallucinations, etc)and or decreased level of consciousness <ref name="clinical"></ref> | ||
* Dyskinesia, movement disorders and increased rigidity | * Dyskinesia, movement disorders and increased rigidity | ||
* Autonomic instability: hyperthermia, tachy/brady,BP fluctuations, hypoventilation | * Autonomic instability: hyperthermia, tachy/brady,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 | ||
* | *Neuroleptic malignant syndrome | ||
* | *Primary psychiatric disorder | ||
* | *Hyper/hypothyroidism | ||
* Cushings Syndrome | *Cushings Syndrome | ||
* Addisons disease | *Addisons 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> | ||
* cerebral space occupying lesions | * cerebral space occupying lesions | ||
* Drugs, toxins, withdrawl | * Drugs, toxins, withdrawl<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> | ||
==Workup== | ==Workup== | ||
* | * 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) | * LP: CSF lymphocytic pleocytosis or oligoclonal bands (can be normal initially) | ||
* EEG: to rule out seizure with movement disorders | * EEG: to rule out seizure with movement disorders | ||
| Line 30: | Line 30: | ||
==Management== | ==Management== | ||
* Resection of tumor | * 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 | ||
* Glucocorticoids | </ref> | ||
* IVIG | *Glucocorticoids<ref name="treatment"></ref> | ||
* | *IVIG<ref name="treatment"></ref> | ||
* Second line rituximab and cyclophosphamide | *Plasma exchange | ||
*Second line rituximab and cyclophosphamide<ref name="treatment"></ref> | |||
==Disposition== | ==Disposition== | ||
* Admission | * Admission with Neurology Consult | ||
==See Also== | ==See Also== | ||
[[Altered mental status]] | |||
[[Delirium]] | |||
==External Links== | |||
*[http://www.thepoisonreview.com/2013/08/28/must-read-anti-nmda-receptor-encephalitis/ Poison Review Anti-NMDA Encephalitis] | |||
*[http://www.antinmdafoundation.org/what-is-anti-nmda-receptor-encephalitis.html Anti-NMDA Foundation] | |||
==Sources== | ==Sources== | ||
<references/> | |||
[[Category:Tox]] | [[Category:Tox]] | ||
[[Category:Neuro]] | [[Category:Neuro]] | ||
Revision as of 15:21, 25 January 2015
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 a 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 (HA, 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, tachy/brady,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
- Cushings Syndrome
- Addisons disease[4]
- Catatonia[4]
- cerebral space occupying lesions
- Drugs, toxins, withdrawl[5]
Workup
- 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 US or CT or MRI to evaluate for associated ovarian tetratoma
Management
- Resection of tumor if there is an associated mass[6]
- Glucocorticoids[6]
- IVIG[6]
- Plasma exchange
- Second line rituximab and cyclophosphamide[6]
Disposition
- Admission with Neurology Consult
See Also
Altered mental status Delirium
External Links
Sources
- ↑ 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