Abstract Details

Title Hashimoto's Encephalopathy Associated with Anti-NAE Autoantibodies: Analysis of 101 Patients

Topic Neurotoxicology

Presentation(s) S29 - (-)

Poster/Presentation
Number 002

Objective

Background HE is a treatable autoimmune encephalopathy associated with autoimmune thyroiditis and has various neuropsychiatric symptoms, leading to under or miss diagnosis. We have therefore developed anti-NAE Abs, which demonstrated high specificity for HE and were a useful diagnostic marker for HE.

Design/Methods The clinical features, treatments, laboratory findings, brain imaging and serum anti-NAE Abs were investigated retrospectively in 101 patients who presented with neuropsychiatric symptoms, had anti-thyroid antibodies and anti-NAE Abs and received to immunotherapy.

Results The patients had various symptoms; disturbance in consciousness (60%), psychiatric symptoms (45%), cognitive impairment (38%), cerebellar ataxia (28%), involuntary movements (28%) and seizures (26%). Most of patients were euthyroid states (70%). Elevation of protein in CSF was observed in 44 patients (46%). Brain MRI demonstrated abnormal study in 46 patients (45%). 81 of 101 patients were improved by immunotherapy (the effective group), and the remaining 20 patients did not show any response to immunotherapy (the no-effective group). The frequencies of disturbance of consciousness (66%) and psychiatric symptoms (53%) were significantly higher in the effective group than those in the no-effective group (38%, 14%, respectively) (p = 0.01, p < 0.001, respectively). While the fractions of insidious onset (43%) and abnormality of brain MRI (80%) in the no-effective group were showed higher than those in the effective group (16%, 36%, respectively) (p = 0.02, p = 0.001, respectively).

Conclusions The present study suggested that acute or subacute onset and disturbance in consciousness and/or psychiatric symptoms were common in HE associated with anti-NAE Abs, and these patients tended to be improved by immunotherapy. Thus, anti-NAE Abs are not only a diagnostic marker for HE but also a predictive marker for responsiveness to immunotherapy.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Masamichi Ikawa (University of Fukui)	No disclosure on file
	No disclosure on file
Makoto Yoneda (Univ of Fukui)	No disclosure on file
	No disclosure on file

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