Autoimmune limbic encephalitis (Q84129)

Autoimmune limbic encephalitis is an immune mediated disease of the limbic system or medial temporal lobes. The basis of such autoimmunity remains unclear in many cases but may follow a variety of viral infections. Unlike paraneoplastic encephalitis, a tumour is not present. It typically manifests with rapidly progressive cognitive decline often with accompanying seizures although brainstem and basal ganglia involvement may occur. Patients may also complain of headache, irritability, sleep disturbance, delusions, hallucinations, agitation, and psychosis. Neurological symptoms may respond favourably to immunotherapy. Investigations may reveal mesial temporal lobe T2 signal abnormalities on MRI and an inflammatory spinal fluid. Associated neural autoantibodies include: neural antibodies targeting plasma membrane antigens they are effectors of injury (e.g. voltage gated potassium channel antibodies-LGI1 or CASPR2 subtypes).