Abstract Details

Title Treatment of Refractory Hashimoto's Encephalitis by Thyroidectomy

Topic Neurotoxicology

Presentation(s) P01 - (-)

Poster/Presentation
Number 053

Objective

Background Hashimoto's encephalitis (HE) is an autoimmune, neurological disorder with multifaceted clinical presentations associated with high titers of anti-tpo antibodies (TPOa). While the diagnostic aspects of HE remain controversial, several variants of HE have been described. Clinical manifestations of HE include encephalopathy, seizures, behavioral symptoms, involuntary movements, and coma. Although immune-modulating therapies such as steroids, plasma exchange (PE), IVIG and immunosuppressive drugs are routinely used, many patients are refractory to treatment. We report two patients with HE refractory to immune-modulating therapies and their successful treatment with total thyroidectomy.

Design/Methods A 60-year old woman (P1) was admitted with status epilepticus. Laboratory evaluations revealed a normal TSH, high TPOa (51 IU/ml) and anti-thyroglobulin antibody (111 IU/ml). The seizures remained uncontrolled with 5 anti-epileptic medications. Repeated treatments with steroids, plasmapheresis, IVIG and Rituximab failed to reduce the seizure frequency or the TPOa level. Another 71-year old woman (P2) presented with subacute onset of dementia and agitation; TSH, B12 levels, CSF (including 14-3-3) were normal. TPOa levels were high (77 IU/ml). Treatment with steroids, PE and IVIG failed to improve her symptoms or reduce the TPOa level.

Results Following total thyroidectomy both patients had remarkable sustained improvements in their symptoms and TPOa levels. P1 became seizure-free and P2 improved in agitation and cognition.

Conclusions We describe the clinical profile and successful treatment of HE by thyroidectomy in two patients resistant to immune-modulating treatments. We propose that total thyroidectomy should be considered as a treatment option in HE, when refractory to medical treatment. The long-term benefits of this treatment need to be evaluated further.

Authors/Disclosures
Hesham A. Allam, MD (Saint Louis University Hospital) PRESENTER	Dr. Allam has nothing to disclose.
Darine Kassar, MD	Dr. Kassar has nothing to disclose.
Pratap R. Chand, MD, FRCP, FAAN (St Louis University School of Medicine)	Dr. Chand has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Boston Scientific. An immediate family member of Dr. Chand has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acorda. Dr. Chand has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva Neuroscience.
Stanley J. Iyadurai, MD, PhD, FAAN (Johns Hopkins All Children's Hospital/Catalyst)	Dr. Iyadurai has received personal compensation for serving as an employee of Catalyst Pharmaceuticals. Dr. Iyadurai has received stock or an ownership interest from Catalyst Pharmaceuticals.
	No disclosure on file
Leonid Gorelik, PhD	No disclosure on file

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