PMID- 23538571
OWN - NLM
STAT- MEDLINE
DCOM- 20130919
LR  - 20211021
IS  - 1543-3641 (Electronic)
IS  - 1543-3633 (Linking)
VI  - 26
IP  - 1
DP  - 2013 Mar
TI  - Neuropsychiatric autoimmune encephalitis without VGKC-complex, NMDAR, and GAD 
      autoantibodies: case report and literature review.
PG  - 36-49
LID - 10.1097/WNN.0b013e31828b6531 [doi]
AB  - We report a patient with a seronegative autoimmune panencephalitis, adding a 
      subtype to the emerging spectrum of seronegative autoimmune encephalitis, and we 
      review the sparse literature on isolated psychiatric presentations of autoimmune 
      encephalitis. (A PubMed search for "seronegative autoimmune encephalitis," 
      "nonvasculitic autoimmune inflammatory meningoencephalitis," and related terms 
      revealed <25 cases.) A 15-year-old girl developed an acute-onset isolated 
      psychosis with prominent negative symptoms and intermittent encephalopathy. 
      Despite clinical worsening, her brain magnetic resonance imaging (MRI) scans 
      remained normal for 7 years. Serology was negative for voltage-gated potassium 
      channel (VGKC)-complex, N-methyl-D-aspartate receptor (NMDAR), and glutamic acid 
      decarboxylase (GAD) autoantibodies. We excluded genetic, metabolic, 
      paraneoplastic, degenerative, and infectious etiologies. The patient's symptoms 
      remitted fully with immune therapy, but recurred in association with widespread 
      bihemispheric brain lesions. Brain biopsy revealed mild nonvasculitic 
      inflammation and prominent vascular hyalinization. Immune therapy with plasma 
      exchanges cleared the MRI abnormalities but, 10 years after onset, the patient 
      still suffers neuropsychiatric sequelae. We conclude that autoimmune 
      panencephalitis seronegative for VGKC-complex, NMDAR, and GAD autoantibodies is a 
      subtype of autoimmune encephalitis that can present with pure neuropsychiatric 
      features and a normal brain MRI. Immunologic mechanisms may account for 
      psychiatric symptoms in a subset of patients now diagnosed with classical 
      psychotic disorders. Delay in starting immune therapy can lead to permanent 
      neuropsychiatric sequelae. We propose a standardized classification system for 
      the autoimmune encephalitides, integrating earlier pathology-oriented terms with 
      more recently defined serologic and clinical phenotypes.
FAU - Najjar, Souhel
AU  - Najjar S
AD  - New York University Comprehensive Epilepsy Center, New York, NY 10016, USA. 
      mna1024231@aol.com
FAU - Pearlman, Daniel
AU  - Pearlman D
FAU - Devinsky, Orrin
AU  - Devinsky O
FAU - Najjar, Amanda
AU  - Najjar A
FAU - Nadkarni, Siddhartha
AU  - Nadkarni S
FAU - Butler, Tracy
AU  - Butler T
FAU - Zagzag, David
AU  - Zagzag D
LA  - eng
GR  - K23 NS057579/NS/NINDS NIH HHS/United States
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Cogn Behav Neurol
JT  - Cognitive and behavioral neurology : official journal of the Society for 
      Behavioral and Cognitive Neurology
JID - 101167278
RN  - 0 (Autoantibodies)
RN  - 0 (Potassium Channels, Voltage-Gated)
RN  - 0 (Receptors, N-Methyl-D-Aspartate)
RN  - EC 4.1.1.15 (Glutamate Decarboxylase)
RN  - Hashimoto's encephalitis
SB  - IM
MH  - Autoantibodies/*blood
MH  - Brain/*pathology
MH  - Brain Diseases/*diagnosis/immunology/pathology
MH  - Encephalitis
MH  - Female
MH  - Glutamate Decarboxylase/*immunology
MH  - Hashimoto Disease/*diagnosis/immunology/pathology
MH  - Humans
MH  - Potassium Channels, Voltage-Gated/*immunology
MH  - Receptors, N-Methyl-D-Aspartate/*immunology
MH  - Young Adult
EDAT- 2013/03/30 06:00
MHDA- 2013/09/21 06:00
CRDT- 2013/03/30 06:00
PHST- 2013/03/30 06:00 [entrez]
PHST- 2013/03/30 06:00 [pubmed]
PHST- 2013/09/21 06:00 [medline]
AID - 00146965-201303000-00007 [pii]
AID - 10.1097/WNN.0b013e31828b6531 [doi]
PST - ppublish
SO  - Cogn Behav Neurol. 2013 Mar;26(1):36-49. doi: 10.1097/WNN.0b013e31828b6531.