PMID- 24166348
OWN - NLM
STAT- MEDLINE
DCOM- 20140220
LR  - 20211021
IS  - 2168-6157 (Electronic)
IS  - 2168-6149 (Print)
IS  - 2168-6149 (Linking)
VI  - 70
IP  - 12
DP  - 2013 Dec
TI  - Anti-N-methyl-D-aspartate receptor encephalitis: a patient with refractory 
      illness after 25 months of intensive immunotherapy.
PG  - 1566-8
LID - 10.1001/jamaneurol.2013.3205 [doi]
AB  - IMPORTANCE: N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is an 
      autoimmune encephalitis that can be paraneoplastic and usually responds to 
      treatment. It is quickly becoming the most common paraneoplastic encephalitis. 
      OBSERVATIONS: We present a case of a woman in her late 30s who developed 
      psychiatric symptoms that progressed to encephalopathy, seizures, autonomic 
      instability, and hyperkinetic movements. The patient was found to have an ovarian 
      teratoma and serum and cerebrospinal fluid NMDAR antibodies. Despite resection of 
      the teratoma and treatment with immunosuppressive therapy, the patient progressed 
      to a minimally conscious state. She was supported medically in our institution 
      for 25 months. During her hospitalization, she was treated with multiple 
      immunosuppressive agents. With each treatment, we analyzed the serum and 
      cerebrospinal fluid for NMDAR antibodies. While there was some initial reduction 
      in the serum antibodies, the spinal fluid antibodies remained persistently 
      elevated. The patient did not have any clinical improvement and eventually died 
      after the family decided to withdraw care. CONCLUSIONS AND RELEVANCE: As far as 
      we know, this case represents the longest active treatment without improvement of 
      a patient with anti-NMDAR encephalitis. The patient had persistently high 
      cerebrospinal fluid and serum antibody titers, which may be of prognostic 
      significance.
FAU - Thomas, Alissa
AU  - Thomas A
AD  - Department of Neurology, Dartmouth Hitchcock Medical Center, Lebanon, New 
      Hampshire.
FAU - Rauschkolb, Paula
AU  - Rauschkolb P
AD  - Neurociences Institute, Intermountain Medical Center, Murray, Utah.
FAU - Gresa-Arribas, Nuria
AU  - Gresa-Arribas N
AD  - IDIBAPS, Hospital Clinic, Barcelona, Spain.
FAU - Schned, Alan
AU  - Schned A
AD  - Department of Neurology, Dartmouth Hitchcock Medical Center, Lebanon, New 
      Hampshire.
FAU - Dalmau, Josep O
AU  - Dalmau JO
AD  - Service of Neurology, Hospital Clinic, Barcelona, Spain.
FAU - Fadul, Camilo E
AU  - Fadul CE
AD  - Department of Neurology, Dartmouth Hitchcock Medical Center, Lebanon, New 
      Hampshire5Department of Medicine, Dartmouth Hitchcock Medical Center, Lebanon, 
      New Hampshire.
LA  - eng
GR  - R01 NS077851/NS/NINDS NIH HHS/United States
GR  - R01NS077851/NS/NINDS NIH HHS/United States
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - JAMA Neurol
JT  - JAMA neurology
JID - 101589536
RN  - 0 (Autoantibodies)
RN  - 0 (Receptors, N-Methyl-D-Aspartate)
SB  - IM
MH  - Adult
MH  - Anti-N-Methyl-D-Aspartate Receptor Encephalitis/blood/cerebrospinal 
      fluid/*etiology/immunology
MH  - Autoantibodies/blood/cerebrospinal fluid
MH  - Female
MH  - Humans
MH  - Immunotherapy/*adverse effects
MH  - Ovarian Neoplasms/immunology/therapy
MH  - Receptors, N-Methyl-D-Aspartate/immunology
MH  - Teratoma/immunology/therapy
PMC - PMC4065571
MID - NIHMS590215
COIS- Conflict of Interest Disclosures: Dr Dalmau has received a research grant from 
      EUROIMMUN and receives royalties from patents for the use of Ma2 and NMDAR as an 
      autoantibody test. No other disclosures were reported.
EDAT- 2013/10/30 06:00
MHDA- 2014/02/22 06:00
PMCR- 2014/06/21
CRDT- 2013/10/30 06:00
PHST- 2013/10/30 06:00 [entrez]
PHST- 2013/10/30 06:00 [pubmed]
PHST- 2014/02/22 06:00 [medline]
PHST- 2014/06/21 00:00 [pmc-release]
AID - 1762532 [pii]
AID - 10.1001/jamaneurol.2013.3205 [doi]
PST - ppublish
SO  - JAMA Neurol. 2013 Dec;70(12):1566-8. doi: 10.1001/jamaneurol.2013.3205.