Abstract Details

Title A Case of Refractory Hashimoto's Encephalitis Associated with Anti-GAD Antibodies

Topic Behavioral Neurology

Presentation(s) P06 - (-)

Poster/Presentation
Number 064

Objective

Background BACKGROUND: Hashimoto Encephalitis is now a recognized steroid responsive encephalopathy. Progression of disease and importance of associated non-thyroid antibodies is not well described.

Design/Methods DESIGN/METHODS: Case report.

Results RESULTS: 70 year old gentleman developed confusion and non-threatening visual hallucinations suggesting diagnoses of anesthesia delirium after elective robotic prostatectomy. He was discharged home, but over the next 2 weeks he developed worsening visual hallucinations, paranoid delusions and agitation. Symptoms persisted despite treatment with anti-psychotics (haloperidol, risperidone, quetiapine) and benzodiazepines (lorazepam). Neurologic examination was unremarkable and neuro-psychometric analysis was consistent was encephalopathy. Routine serology and EEG were unremarkable. MRI Brain with Gadolinium showed non-specific white matter disease. CT of chest, abdomen and pelvis with contrast did not identify any underlying malignancy. FDG-PET Scan was consistent with Hashimoto Thyroiditis. CSF analysis was unremarkable including Cytology, IgG Index, Oligoclonal Bands and 14-3-3 analysis. B12, TSH and T4 levels were normal. RPR was nonreactive. Paraneoplastic panel identified elevated Anti-Thyroglobulin and Anti-Thyroid Peroxidase antibody titers. He was treated with IV solumedrol followed by oral steroids which resulted in significant improvement in symptoms confirming the diagnosis of Hashimoto encephalitis (Steroid-Responsive Encephalopathy associated with Autoimmune Thyroiditis). Subsequently, with tapering of steroid dose, symptoms recurred prompting treatment with IVIG which showed some benefit. Two months later symptoms worsened and did not respond to high doses of IV steroids. At this time, Anti-GAD antibodies were also identified. Patient underwent plasmapheresis without benefit. He continues to have disabling symptoms and has been under constant supervision in a nursing home.

Conclusions CONCLUSIONS: This case highlights the importance of recognizing that refractory psychiatric symptoms may be the initial presentation of auto-immune encephalopathies. Steroid responsive encephalopathies may evolve to an encephalopathy not responsive to immunosuppressive treatment, in particular when associated with non-thyroid antibodies.

Authors/Disclosures
Omar Ahmad, MD (Avita Health System) PRESENTER	No disclosure on file
Vivek Rai, MD	Dr. Rai has nothing to disclose.
Douglas L. Arnold, MD, FAAN (Montreal Neurological Institute, McGill Univ)	Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for BMS. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Frequency Therapeutics. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck. Dr. Arnold has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Arnold has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Shionogi. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Xfacto communications. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Find therapeutics. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for GSK. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Idorsia. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Kiniksa. Dr. Arnold has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Clario.
Daniel Miller, MD	Dr. Miller has nothing to disclose.

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