Preferred Label : Autoimmune encephalopathy;
ICD-11 definition : Autoimmune encephalopathy is similar to autoimmune encephalitis [see autoimmune encephalitis
definition] but reflects a more subacute to insidious onset which tends to fluctuate
and often mimics neurodegenerative dementias (Creutzfeldt-Jakob disease or Lewy Body
disease). A personal or family history of autoimmunity is often found. Investigations
typically show an inflammatory spinal fluid, an epileptiform electroencephalogram
and MRI abnormalities in the mesial temporal lobes. Accompanying neural autoantibodies
(e.g. voltage gated potassium channel complex autoantibodies) and non-organ specific
(thyroid peroxidase [TPO] antibodies) may be found. Exclusion of alternative etiologies
(e.g. infections) is important. Response to immunotherapy may be used to confirm the
diagnosis. Associated autoantibodies: DPPX (dipeptidyl-peptidase–like protein-6);
GABAAR (Gamma-aminobutyric-acid type-A autoantibodies); gAChR (ganglionic acetylcholine
receptor autoantibodies (alpha-3 subunit)); GAD65 (glutamic acid decarboxylase-65
antibodies); VGKC (LGI1, CASPR2) (voltage gated potassium channel complex antibodies
).;
ICD-11 synonym : autoimmune dementia; immunotherapy responsive encephalopathy; SREAT - [steroid-responsive-encephalopathy-associated with autoimmune thyroiditis]; steroid-responsive-encephalopathy-associated with autoimmune thyroiditis; Hashimoto's encephalopathy;
ICD-11 acronym : SREAT;
Origin ID : 1445382314;
Automatic exact mappings (from CISMeF team)
Autoimmune encephalopathy is similar to autoimmune encephalitis [see autoimmune encephalitis
definition] but reflects a more subacute to insidious onset which tends to fluctuate
and often mimics neurodegenerative dementias (Creutzfeldt-Jakob disease or Lewy Body
disease). A personal or family history of autoimmunity is often found. Investigations
typically show an inflammatory spinal fluid, an epileptiform electroencephalogram
and MRI abnormalities in the mesial temporal lobes. Accompanying neural autoantibodies
(e.g. voltage gated potassium channel complex autoantibodies) and non-organ specific
(thyroid peroxidase [TPO] antibodies) may be found. Exclusion of alternative etiologies
(e.g. infections) is important. Response to immunotherapy may be used to confirm the
diagnosis. Associated autoantibodies: DPPX (dipeptidyl-peptidase–like protein-6);
GABAAR (Gamma-aminobutyric-acid type-A autoantibodies); gAChR (ganglionic acetylcholine
receptor autoantibodies (alpha-3 subunit)); GAD65 (glutamic acid decarboxylase-65
antibodies); VGKC (LGI1, CASPR2) (voltage gated potassium channel complex antibodies
).
