Steroid-responsive Hashimoto encephalopathy mimicking Creutzfeldt-Jakob disease

Neurol Sci. 2011 Aug;32(4):719-22. doi: 10.1007/s10072-011-0610-8. Epub 2011 May 10.

Abstract

Hashimoto's encephalopathy (HE) is a rare neurological disorder with a heterogeneous group of neurological symptoms associated with high titres of anti-thyroid antibodies. Clinical manifestations may include encephalopathic features such as seizures, behavioural and psychiatric manifestations, movement disorders and coma. The objective of this presentation is to describe a patient with this rare and controversial clinical syndrome mimicking Creutzfeldt-Jakob disease, associated with a Hashimoto euthyroid thyroiditis and with a significant response to high dose intravenous prednisone. The responsiveness of this syndrome to steroids suggests that this disorder involves immune pathogenic mechanisms, as previous reviews reported.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Aggression
  • Anti-Inflammatory Agents / therapeutic use
  • Arousal / physiology
  • Autoantibodies / analysis
  • Brain / pathology
  • Brain Diseases / diagnosis*
  • Brain Diseases / drug therapy*
  • Brain Diseases / psychology
  • Cerebral Cortex / pathology
  • Confusion / etiology
  • Confusion / psychology
  • Creutzfeldt-Jakob Syndrome / diagnosis*
  • Diagnosis, Differential
  • Electroencephalography
  • Encephalitis
  • Hallucinations / etiology
  • Hallucinations / psychology
  • Hashimoto Disease / diagnosis*
  • Hashimoto Disease / drug therapy*
  • Hashimoto Disease / psychology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use
  • Neurologic Examination
  • Psychomotor Agitation / etiology
  • Thyroglobulin / immunology
  • Tomography, X-Ray Computed

Substances

  • Anti-Inflammatory Agents
  • Autoantibodies
  • Thyroglobulin
  • Methylprednisolone

Supplementary concepts

  • Hashimoto's encephalitis