Preferred Label : Autoimmune limbic encephalitis;
ICD-11 definition : 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 tumor 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
favorably 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).;
Origin ID : 1254443511;
Currated CISMeF NLP mapping
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 tumor 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
favorably 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).
