Clinical manifestations and treatment response of steroid in pediatric Hashimoto encephalopathy.
Hashimoto encephalopathy is a steroid-responsive encephalopathy associated with elevated titers of antithyroid antibodies. Clinical symptoms are characterized by behavioral and cognitive changes, speech disturbance, seizures, myoclonus, psychosis, hallucination, involuntary movements, cerebellar signs, and coma. The standard treatment is the use of corticosteroids along with the treatment of any concurrent dysthyroidism. Other options are immunoglobulins and plasmapheresis. We described symptoms and outcomes on 3 teenage girls with Hashimoto encephalopathy. Presenting symptoms were seizure or altered mental status. One patient took levothyroxine due to hypothyroidism before presentation of Hashimoto encephalopathy. After confirmation of elevated antithyroid antibodies, all patients were treated with steroids. One patient needed plasmapheresis because of the lack of response to steroids and immunoglobulins. Hashimoto encephalopathy should be considered in any patient presenting with acute or subacute unexplained encephalopathy and seizures. Even though the use of steroids is the first line of treatment, plasmapheresis can rescue steroid-resistant patients.
Department of Pediatrics Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Hee Joon
HJ
Yu
Department of Pediatrics Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Jeehun
J
Lee
Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Dae Won
DW
Seo
Department of Pediatrics Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea mhlee091@skku.edu.
Munhyang
M
Lee
D000293:Adolescent; D000906:Antibodies / Q000097:blood; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000175:diagnosis* / Q000188:drug therapy*; D002648:Child; D004569:Electroencephalography; D004660:Encephalitis; D005260:Female; D050031:Hashimoto Disease / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D013256:Steroids / Q000627:therapeutic use*; D013954:Thyroglobulin / Q000276:immunology
D000906:Antibodies; D013256:Steroids; D013954:Thyroglobulin
Hashimoto encephalopathy; plasmapheresis; steroid; steroid-resistant Hashimoto encephalopathy