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J Neurol Sci. 2013 Aug 15;331(1-2):67-71. doi: 10.1016/j.jns.2013.05.009. Epub 2013 Jun 5.

Diagnostic and therapeutic aspects of Hashimoto's encephalopathy.

Author information

1
Vanderbilt University, Department of Neurology, Nashville, TN 37232, USA.

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.

KEYWORDS:

Altered mental status; Anti-thyroid antibodies; Mycophenolate mofetil; Nonvasculitic autoimmune meningoencephalitis (NAIM); Rituximab; Steroid responsive encephalopathy with antithyroid antibodies (SREAT)

PMID:
23759502
DOI:
10.1016/j.jns.2013.05.009
[Indexed for MEDLINE]

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