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Anti-NMDA receptor encephalitis
From WikEM
Contents
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, 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
- Drug reactions (NMS, Serotonin Syndrome)
- Environmental causes
- Deficiency state
- Wernicke encephalopathy
- Subacture Combined Degeneragion (B12 deficiency)
- Vitamin D Deficiency
- Zinc Deficiency
- Sepsis
Primary CNS disease or trauma
- Direct CNS trauma
- Diffuse axonal injury
- Subdural/epidural hematoma
- Vascular disease
- Intraparenchymal hemorrhage
- SAH
- Stroke
- Hemispheric, brainstem
- CNS infections
- Encephalitis
- Anti-NMDA receptor encephalitis
- Meningitis
- Neoplasms
- Paraneoplastic Limbic Encephalitis]
- Malignant Meningitis
- Pancreatic Insulinoma
- Seizures
- Nonconvulsive status epilepticus
- Postictal state
- Dementia
Psychiatric
- Acute psychosis
- Excited delirium
- Malingering
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
