Hashimoto's encephalopathy.
Hashimoto's encephalopathy (HE) is a controversial neurological disorder that comprises a heterogenous group of neurological symptoms that manifest in patients with high titers of antithyroid antibodies. Clinical manifestations of HE may include encephalopathic features such as seizures, behavioral and psychiatric manifestations, movement disorders, and coma. Although it has been linked to cases of Hashimoto's thyroiditis or thyroid dysfunction, the most common immunological feature of HE is the presence of high titers of antithyroglobulin or anti-TPO (antimicrosomal) antibodies. At present, it is unclear whether antithyroid antibodies represent an immune epiphenomenon in a subset of patients with encephalopathic processes or they are really associated with pathogenic mechanisms of the disorder. The significance of classifying encephalopathies under the term HE will be determined in the future once the relevance of the role of antithyroid antibodies is demonstrated or dismissed by more detailed experimental and immunopathological studies. The responsiveness of HE to steroids or other therapies such as plasmapheresis supports the hypothesis that this is a disorder that involves immune pathogenic mechanisms. Further controlled studies of the use of steroids, plasmapheresis, or immunosuppressant medications are needed in the future to prove the concept of the pathogenic role of antithyroid antibodies in HE.
Schiess
Nicoline
N
Department of Neurology, Division of Neuroimmunology and Neuroinfectious Disorders, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
Pardo
Carlos A
CA
D001927:Brain Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D003704:Dementia / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D004660:Encephalitis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D050031:Hashimoto Disease / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D012640:Seizures / Q000188:drug therapy / Q000276:immunology / Q000473:pathology