PMID- 23806220 OWN - NLM STAT- MEDLINE DCOM- 20130904 LR - 20211021 IS - 0722-5091 (Print) IS - 0722-5091 (Linking) VI - 32 IP - 4 DP - 2013 Jul-Aug TI - Clinical Neuropathology practice guide 4-2013: post-herpes simplex encephalitis: N-methyl-Daspartate receptor antibodies are part of the problem. PG - 251-4 AB - Classic herpes simplex virus encephalitis (HSVE) is an acute viral infection that usually follows a monophasic disease course; however some patients, mainly children, experience a relapse within weeks or months after the initial event. In a subset of these patients a viral reactivation is unlikely because the CSF PCR for HSV is negative, repeated MRI does not show new necrotic lesions, and the symptoms are refractory to antiviral therapy. These patients often develop choreoathetosis variably accompanied by behavioral changes and seizures, and a postinfectious immune-mechanism has been postulated. Recent studies demonstrated that 7% of patients with HSVE harbor NR1 N-methyl-D-aspartate receptor (NMDAR) IgG antibodies. Moreover, a child with post- HSVE choreoathetosis was found to have NMDAR antibodies; the patient did not improve with antiviral therapy but recovered after aggressive immunotherapy. Based on these findings, evidence is increasing that a subgroup of post-HSVE represents a separate disease entity, which in fact is anti-NMDAR encephalitis. Patients with relapsing HSVE or prolonged atypical symptoms, who have negative CSF PCR for HSV should routinely be tested for NMDAR IgG antibodies in CSF and serum. It is important to be aware of this differential diagnosis because patients respond to immunotherapy. FAU - Hoftberger, Romana AU - Hoftberger R AD - Institute of Neurology, Medical University of Vienna, Austria. romana.hoeftberger@meduniwien.ac.at FAU - Armangue, Thais AU - Armangue T FAU - Leypoldt, Frank AU - Leypoldt F FAU - Graus, Francesc AU - Graus F FAU - Dalmau, Josep AU - Dalmau J LA - eng GR - R01 NS077851/NS/NINDS NIH HHS/United States GR - R01CA089054/CA/NCI NIH HHS/United States GR - R01NS077851/NS/NINDS NIH HHS/United States GR - R01MH094741/MH/NIMH NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - Germany TA - Clin Neuropathol JT - Clinical neuropathology JID - 8214420 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Receptors, N-Methyl-D-Aspartate) SB - IM MH - Anti-N-Methyl-D-Aspartate Receptor Encephalitis/*immunology/virology MH - Autoantibodies/*immunology MH - Autoantigens/immunology MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Male MH - Neuralgia, Postherpetic/*immunology MH - *Practice Guidelines as Topic MH - Receptors, N-Methyl-D-Aspartate/*immunology PMC - PMC3749751 EDAT- 2013/06/29 06:00 MHDA- 2013/09/05 06:00 PMCR- 2013/07/01 CRDT- 2013/06/29 06:00 PHST- 2013/06/29 06:00 [entrez] PHST- 2013/06/29 06:00 [pubmed] PHST- 2013/09/05 06:00 [medline] PHST- 2013/07/01 00:00 [pmc-release] AID - 10720 [pii] AID - 10.5414/np300666 [doi] PST - ppublish SO - Clin Neuropathol. 2013 Jul-Aug;32(4):251-4. doi: 10.5414/np300666.