Diagnostic and therapeutic aspects of Hashimoto's encephalopathy

Inan Olmez, Harold Moses, Subramaniam Sriram, Howard Kirshner, Andre H. Lagrange, Siddharama Pawate

Research output: Contribution to journalArticlepeer-review

75 Scopus citations

Abstract

Objective: To share our experience on clinical presentation and management of patients diagnosed with Hashimoto's Encephalopathy (HE) at Vanderbilt Medical Center between 1999 and 2012. Background: HE is a rare disorder characterized by encephalopathy and central nervous system (CNS) dysfunction, elevated antithyroid antibodies, the absence of infection or structural abnormalities in the CNS, and a response to treatment with steroids. The relationship between thyroid antibodies and encephalopathy has remained unresolved. Design/Methods: Retrospective chart review. Results: We identified 13 patients who met the criteria for the diagnosis of HE. The median age was 49 years (range, 2-66) and all except one were women. Encephalopathy in the form of altered mental status, stroke-like symptoms or seizures, with prompt resolution of symptoms upon receiving steroids, was the commonest presentation, seen in 7 patients. The second commonest presentation was subacute progressive decrease in cognitive function, which reversed within days to weeks after steroid therapy, seen in 4 patients. Electroencephalogram (EEG) was available in 12 patients and was abnormal in 8, showing nonspecific cerebral dysfunction in all 8 and epileptiform activity in 3. Treatment consisted of steroids in the acute phase for 12 of 13 patients with rapid improvement in symptoms. Maintenance therapy was rituximab in 7 patients, intravenous immunoglobulin (IVIg) in 7, azathioprine in 4, mycophenolate mofetil in 3, and methotrexate in 1 (some patients received sequential therapy with different agents). There was complete or near complete resolution of symptoms in 12 of the 13 patients. Conclusions: We present a cohort of patients in whom CNS dysfunction was associated with elevated antithyroid antibodies and reversal of disease followed immunomodulatory therapies.

Original languageEnglish (US)
Pages (from-to)67-71
Number of pages5
JournalJournal of the neurological sciences
Volume331
Issue number1-2
DOIs
StatePublished - Sep 15 2013

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

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