June 4, 2015: Steroid-responsive encephalopathy
June 4, 2015
Steroid-responsive encephalopathy, also known as Hashimoto’s encephalopathy, is felt to be an immune-mediated disorder, which manifests as altered consciousness, psychosis, myoclonus, and seizures. Elevated levels of anti-thyroid peroxidase antibody and antithyroglobulin antibody support an autoimmune etiology, although specific titers do not correlate with disease severity. Levels of functioning thyroid hormone are not felt to be supportive in diagnosing Hashiomoto’s encephalopathy, as both patients with hypo and hyperthyroidism can present with this entity. About one quarter of patients with Hashimoto’s encephalopathy may present with stroke-like episodes including recurrent episodes of focal neurological deficits, ultimately prompting a vascular evaluation. Imaging is often normal, but may demonstrate multifocal, T2 subcortical white matter changes. Although a specific regimen has not been identified some patients warrant high-dose intravenous methylprednisolone in comparison to oral prednisone, with a prolonged taper often required. Non-steroidal agents, IVIG and plasmaphoresis have also been used in treatment. Prognosis is often good, although recurrence of disease can occur.
- Brain L, Jellinek EH, Ball K. Hashiomoto’s disease and encephalopathy. Lancet 1966; 2: 512-514.
- Flanagan EP and Caselli RJ. Autoimmune encephalopathy. Seminars in Neurology. 2011; 31: 144-157.
Submitted by James Addington, M.D. Resident Physician, Department of Neurology, University of Virginia.
Disclosures: Dr. Addington is a member of the Residents & Fellows Section of Neurology.