PMID- 28272206
OWN - NLM
STAT- MEDLINE
DCOM- 20170328
LR  - 20210109
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 10
DP  - 2017 Mar
TI  - Limbic encephalitis associated with anti-NH2-terminal of alpha-enolase antibodies: A 
      clinical subtype of Hashimoto encephalopathy.
PG  - e6181
LID - 10.1097/MD.0000000000006181 [doi]
LID - e6181
AB  - Several types of autoantibodies have been reported in autoimmune limbic 
      encephalitis (LE), such as antibodies against the voltage-gated potassium channel 
      (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently 
      reported a patient with autoimmune LE and serum anti-NH2-terminal of alpha-enolase 
      (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), 
      who was diagnosed with HE based on the presence of antithyroid antibodies and 
      responsiveness to immunotherapy. This case suggests that LE patients with 
      antibodies to both the thyroid and NAE could be diagnosed with HE and respond to 
      immunotherapy. The aim of this study was to clarify the clinicoimmunological 
      features and efficacy of immunotherapy in LE associated with anti-NAE antibodies 
      to determine whether the LE is a clinical subtype of HE.We examined serum 
      anti-NAE antibodies in 78 LE patients with limbic abnormality on magnetic 
      resonance imaging and suspected HE based on positivity for antithyroid 
      antibodies. Nineteen of the 78 patients had anti-NAE antibodies; however, 5 were 
      excluded because they were double positive for antibodies to the VGKC complex 
      including LGI1. No antibodies against the N-methyl-D-aspartate receptor (NMDAR), 
      contactin-associated protein 2 (Caspr2), gamma-aminobutyric acid-B receptor (GABABR), 
      or alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptor (AMPAR) were 
      detected in the 19 patients. Among the remaining 14 who were positive only for 
      anti-NAE antibodies, the median age was 62.5 (20-83) years, 9 (64%) were women, 
      and 8 (57%) showed acute onset, with less than 2 weeks between onset and 
      admission. Consciousness disturbance (71%) and memory disturbance (64%) were 
      frequently observed, followed by psychiatric symptoms (50%) and seizures (43%). 
      The frequency of these symptoms significantly differed between the acute- and 
      subacute-onset groups. Abnormalities in cerebrospinal fluid and 
      electroencephalogram were commonly observed (92% for both). Tumors were not 
      identified in any cases. All patients responded to immunotherapy or spontaneously 
      remitted, thereby fulfilling the criteria of HE.This study demonstrated that LE 
      associated with anti-NAE antibodies is a nonparaneoplastic LE and various limbic 
      symptoms that depend on the onset type. Favorable therapeutic efficacy suggests 
      that this LE can be considered a clinical subtype of HE and that anti-NAE 
      antibodies may be a promising indicator of the need for immunotherapy.
FAU - Kishitani, Toru
AU  - Kishitani T
AD  - The Second Department of Internal Medicine, Faculty of Medical Sciences, 
      University of Fukui, Fukui Department of Neurology and Geriatrics, Kagoshima 
      University Graduate School of Medical and Dental Sciences, Kagoshima Department 
      of Neurology, Kanazawa Medical University, Ishikawa Faculty of Nursing and Social 
      Welfare Sciences, Fukui Prefectural University, Fukui, Japan.
FAU - Matsunaga, Akiko
AU  - Matsunaga A
FAU - Ikawa, Masamichi
AU  - Ikawa M
FAU - Hayashi, Kouji
AU  - Hayashi K
FAU - Yamamura, Osamu
AU  - Yamamura O
FAU - Hamano, Tadanori
AU  - Hamano T
FAU - Watanabe, Osamu
AU  - Watanabe O
FAU - Tanaka, Keiko
AU  - Tanaka K
FAU - Nakamoto, Yasunari
AU  - Nakamoto Y
FAU - Yoneda, Makoto
AU  - Yoneda M
LA  - eng
PT  - Journal Article
PT  - Observational Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Autoantibodies)
RN  - EC 4.2.1.11 (Phosphopyruvate Hydratase)
RN  - Hashimoto's encephalitis
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - *Autoantibodies
MH  - Encephalitis/*immunology
MH  - Female
MH  - Hashimoto Disease/*immunology
MH  - Humans
MH  - Limbic Encephalitis/*immunology
MH  - Male
MH  - Middle Aged
MH  - Phosphopyruvate Hydratase/*immunology
MH  - Young Adult
PMC - PMC5348154
COIS- The authors have no conflicts of interest to disclose.
EDAT- 2017/03/09 06:00
MHDA- 2017/03/30 06:00
PMCR- 2017/03/10
CRDT- 2017/03/09 06:00
PHST- 2017/03/09 06:00 [entrez]
PHST- 2017/03/09 06:00 [pubmed]
PHST- 2017/03/30 06:00 [medline]
PHST- 2017/03/10 00:00 [pmc-release]
AID - 00005792-201703100-00014 [pii]
AID - MD-D-16-06803 [pii]
AID - 10.1097/MD.0000000000006181 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Mar;96(10):e6181. doi: 10.1097/MD.0000000000006181.