PT  - JOURNAL ARTICLE
AU  - Kishitani, Toru
AU  - Matsunaga, Akiko
AU  - Ikawa, Masamichi
AU  - Kame, Tomomi
AU  - Yamamura, Osamu
AU  - Hamano, Tadanori
AU  - Watanabe, Osamu
AU  - Tanaka, Keiko
AU  - Nakamoto, Yasunari
AU  - Yoneda, Makoto
TI  - Clinical and immunological features of limbic form of Hashimoto’s encephalopathy (P4.045)
DP  - 2015 Apr 06
TA  - Neurology
PG  - P4.045
VI  - 84
IP  - 14 Supplement
4099  - http://n.neurology.org/content/84/14_Supplement/P4.045.short
4100  - http://n.neurology.org/content/84/14_Supplement/P4.045.full
SO  - Neurology2015 Apr 06; 84
AB  - OBJECTIVE: To evaluate the clinical and immunological features of Hashimoto’s encephalopathy (HE) presenting with limbic encephalitis (LE). BACKGROUND: LE is caused by viral infections and autoimmunity associated with specific autoantibodies (Abs) (anti-NMDAR, anti-VGKC etc.). HE is a steroid-responsible autoimmune disorder, associated with serum Abs against the NH2-terminal of α-enolase (anti-NAE), a diagnostic marker, and occasionally presents with LE. METHODS: The clinical features, laboratory findings, brain imaging and treatments were investigated in 19 HE-LE patients who had limbic lesions on the brain MRI with serum anti-NAE Abs. Anti-NMDAR and anti-VGKC Abs were also investigated. Immunofluorescence histochemistry of rat hippocampus was performed with patients’ sera. RESULTS: The patients consisted of 6 men and 13 women (mean age, 62 years old; range, 20-83). The intervals between the symptom onset and the start of treatment ranged from 1 day to 4 months. Patients were categorized into two groups; acute (≤ 2 weeks) and subacute onset (蠅 2w). Disturbance in consciousness was presented in 12 of 19 patients (63[percnt]; 100[percnt] in acute, 30[percnt] in subacute). Epilepsy occurred in 10 of 19 patients (50[percnt]; 77[percnt] in acute, 30[percnt] in subacute). Psychosis occurred in 12 of 19 patients (63[percnt]; 56[percnt] in acute, 70[percnt] in subacute). Memory disturbance occurred in 12 of 19 patients (63[percnt]; none in acute, 90[percnt] in acute). Anti-VGKC Abs were detected in 5 of 19 patients (26[percnt]) while anti-NMDAR Abs were not detected. Elevation of protein in CSF was observed in 9 of 18 (50[percnt]). The EEGs showed diffuse slow wave activity in 13 of 16 patients (81[percnt]). All patients responded to immunotherapies, mainly steroid, and showed good outcomes. The patients’ sera immunologically reacted with hippocampal neurons and merged with α-enolase. CONCLUSIONS: HE is an important treatable disorder on the differential diagnosis of LE.Disclosure: Dr. Kishitani has nothing to disclose. Dr. MATSUNAGA has nothing to disclose. Dr. Ikawa has nothing to disclose. Dr. Kame has nothing to disclose. Dr. Yamamura has nothing to disclose. Dr. Hamano has nothing to disclose. Dr. Watanabe has nothing to disclose. Dr. Tanaka has received personal compensation for activities with Biogen Idec, Nihon Seiyaku, Bayer Shering Pharma, and Tanabe Mitsubishi Pharma as a speaker. Dr. Nakamoto has nothing to disclose. Dr. Yoneda has nothing to disclose.Wednesday, April 22 2015, 7:30 am-12:00 pm