Abstract Details

Title Steroid therapy in probable cerebellar-type Hashimoto's encephalopathy

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-040

Objective To investigate clinical course and reactivity to steroid therapy in probable cerebellar-type Hashimoto's encephalopathy (HE) with negative anti-NH2-terminal of α-enolase (NAE) autoantibodies.

Background Chronic thyroiditis (Hashimoto's disease) is relatively common in Japan. Patients with HE exhibit various neurological presentation, however, diagnosis of is difficult especially when autoantibodies cannot be evaluated or detected. Stabilography is a non-invasive technique to analyze the equilibrium function.

Design/Methods A 63-year-old female, who developed seizures and depression in her 40s, progressive cerebellar ataxia in her 50s, was investigated. At age 61 she developed sever hypothyroidism with low free T4 (0.05 mg/mL, normal range 0.97-1.72 mg/mL) and high TSH (>100 µIU/mL, normal range 0.3-4.0µIU/mL), and treated with thyroid hormone replacement. She showed increase level of thyroid peroxidase (TPO) (241.2 IU/mL, normal range 0.0-15.9 IU/mL) and thyroglobulin (TGAb) (246.0 IU/mL, normal range 0.0-27.9 IU/mL) antibodies, but normal level of NAE autoantibodies. Genetic tests for spinocerebellar ataxia (SCA1, 2, 3, 6, 10, 12, 17, and 31) were negative. Based on the probable diagnosis of cerebellar-type HE, steroid therapy was commenced. Postural balance was measured with stabilography.

Results After 2 courses of steroid pulse therapy (daily intravenous steroids with 1 g of methylprednisolone for three days), she showed clinical improvement in dysarthria and imbalance. The stabilography measurements with closed eyes showed improvement in envelope area (from 6.20 to 4.45 cm²) and length of excursion per second (from 3.70 to 2.82 cm/s).

Conclusions Patients with HE can show adult-onset progressive cerebellar ataxia resembling to spinocerebellar degeneration. Immunotherapies should be considered in patients with high level of TPO and TGAb antibodies, even with normal level of NAE autoantibodies. The stabilography measurements are useful in evaluating postural instability precisely.

Authors/Disclosures
Masako Kinoshita, MD, PhD (8 Ondoyamacho) PRESENTER	Dr. Kinoshita has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Otsuka Pharmaceutical Co., Ltd.. Dr. Kinoshita has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Eisai Co., Ltd.. Dr. Kinoshita has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB Japan Co. Ltd. . Dr. Kinoshita has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Daiichi Sankyo Company, Limited.. The institution of Dr. Kinoshita has received research support from Japan Society for the Promotion of Science. The institution of Dr. Kinoshita has received research support from Nakatani Foundation for Advancement of Measuring Technologies in Biomedical Engineering .
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Makoto Yoneda	The institution of Makoto Yoneda has received research support from Cosmic Corporation.

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