Anti-NMDA receptor encephalitis: Difference between revisions
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==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 (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 <ref name="clinical"></ref> | *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]] | *[[Viral encephalitis]] | ||
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==Diagnosis== | ==Diagnosis== | ||
* Diagnosis confirmed by detection of antibodies to NR1 subunit of NMDAR in CSF or serum (typically send-out lab) | *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 | ||
* MRI brain: normal or transient FLAIR or contrast enhancing abnormalities in cortical or subcortical regions | *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 teratoma | *Pelvic US 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> | ||
*Glucocorticoids<ref name="treatment"></ref> | *Glucocorticoids<ref name="treatment"></ref> | ||
| Line 41: | Line 41: | ||
*Second line rituximab and cyclophosphamide<ref name="treatment"></ref> | *Second line rituximab and cyclophosphamide<ref name="treatment"></ref> | ||
==Disposition== | ==Disposition== | ||
* Admission with Neurology Consult | *Admission with Neurology Consult | ||
==See Also== | ==See Also== | ||
*[[Altered mental status]] | *[[Altered mental status]] | ||
Revision as of 03:51, 6 July 2016
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 (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
- Cushing syndrome
- Addison's disease[4]
- Catatonia[4]
- Cerebral space occupying lesions
- Drugs, toxins, withdrawal[5]
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
Diagnosis
- 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 teratoma
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
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