The Anti-NMDA Receptor Encephalitis Foundation Newsletter




Human hippocampal cornu ammonis 3 damage impairs both recent and remote autobiographical episodic memory, and disrupts functional integration in medial temporal lobe subsystem regions of the default network.

 




The Anti-NMDA Receptor Encephalitis Prize in association with the Canadian Neurological Society

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In January 1973, Science (along with Nature, the most influential general science journal in the world) published an article that immediately captured……

 




PubMed comprises more than 30 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

 







If you’ve ever experienced brain fog, anxiety, or mood issues alongside your autoimmune symptoms, you’re not alone. Doctors know that mental health issues often increase with an autoimmune diagnosis, and a new scientific discovery might explain why.

 




“It was horrific. We didn’t know what was happening,” the mother, Christina Beck, said.

 




Abstract Objective To report the presenting syndromes and to determine whether pretreatment criteria of Hashimoto encephalopathy (HE) predict response to steroids. Methods We assessed symptoms and steroid responsiveness in 24 patients with pretreatment criteria of HE, including (1) subacute onset of cognitive impairment, psychiatric symptoms, or seizures; (2) euthyroid status or mild hypothyroidism; (3) serum thyroid peroxidase antibodies (TPOAb) >200 IU/mL; (4) absent neuronal antibodies in serum/CSF; and (5) no other etiologies. Additional studies included determination of TPOAb (>200 IU/mL) in 74 patients with criteria of possible autoimmune encephalitis (AE) without neuronal antibodies and 205 patients with different neuroimmunologic diseases, psychosis, or new-onset refractory status epilepticus (NORSE). Serum antibodies to the amino (ΝΗ2)-terminal of α-enolase (NH2-α-enolaseAb) were examined in the indicated 24 patients and 13 controls. Results The 24 patients (14 women) with suspected HE had a median age of 48 years (range 8–79 years). Four syndromes were identified: psychiatric (7, 29%), encephalopathy (7, 29%), NORSE-like (6, 25%), and limbic encephalitis (4, 17%). Only 6 of 19 (31.6%) patients completely responded to steroids. The frequency of TPOAb in the 74 patients with possible AE (6 of 74, 8.1%) was similar to that of the 205 controls (17 of 205, 8.2%; p = 0.84). NH2-α-enolaseAb were identified in 1 of 24 suspected HE cases and 1 of 13 controls. Conclusion Current pretreatment criteria of HE do not predict steroid responsiveness. The detection of TPOAb across all control groups reveals their poor disease-specificity. NH2-α-enolaseAb did not help in the diagnosis of HE. These findings imply a redefinition of HE that requires a systematic exclusion of antibody-mediated encephalitis. Glossary AE=autoimmune encephalitis; HE=Hashimoto encephalopathy; IVIG=IV immunoglobulin; LE=limbic encephalitis; NORSE=new-onset refractory status epilepticus; TPOAb=thyroid peroxidase antibodies Footnotes Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. Editorial, page 55 Podcast: NPub.org/w0glpg Received April 29, 2019. Accepted in final form August 2, 2019. © 2019 American Academy of Neurology

 




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