Definition

Pathogenesis

Clinical manifestation

Diagnostic workup

Treatment

Anti-NMDA encephalitis

A rare encephalitis syndrome seen in patients with teratomas and small cell lung cancer; 40% cases not seen with tumor

Associated with antibodies against NR1 and NR2 heteromers of the NMDA receptor

Probably caused by immunemediated response due to cross-reactivity between native NMDA receptors and tumor cells expressing NMDA receptors

Commonly seen in young women; presents with psychiatric, cognitive, and autonomic symptoms followed by seizures, dyskinesias, and ataxia, and encephalopathy

May be preceded by viral like syndrome

Clinical history

Brain MRI may be normal or show nonspecific increase in T2 or FLAIR signal in the temporal lobes, brainstem, basal ganglia, and cerebral cortex

CSF may show pleocytosis, oligoclonal bands, and NR1 antibodies

Variable response to empirical treatment with corticosteroids, intravenous immunoglobulin (IVIG), plasma exchange (PE).

Recovery usually slow, persistent amnesia common

Rituximab (anti-CD 20 monoclonal antibody) may be considered

ITM

Severe focal inflammatory disorder of spinal cord resulting in devastating myelopathy syndrome

Believed to be postinfectious autoimmune process, as often follows an antecedent infection

Acute or subacute myelopathic syndrome with motor, sensory, and autonomic involvement; respiratory involvement may be seen with high cervical lesions

Rapidly progressive severe myelopathy

MRI—mono or multifocal cord lesions, may show enhancement; the length of lesions varies from one to many

CSF—pleocytosis, elevated protein, variable oligoclonal band positivity

Prognosis variable; most patients left with moderate-to-severe disability