TY - JOUR
T1 - Adenylate kinase 5 (AK5) autoimmune encephalitis
T2 - Clinical presentations and outcomes in three new patients
AU - McKeon-Makki, Iman
AU - McKeon, Andrew
AU - Yang, Binxia
AU - Pittock, Sean J.
AU - Lopez-Chiriboga, Sebastian
AU - Komorowski, Lars
AU - Miske, Ramona
AU - Zekeridou, Anastasia
N1 - Publisher Copyright:
© 2022 Elsevier B.V.
PY - 2022/6/15
Y1 - 2022/6/15
N2 - Adenylate kinase 5 (AK5) antibodies are biomarkers of a poorly responsive to immunotherapy, non-paraneoplastic, autoimmune limbic encephalitis. We detected 6 patients (all female, median age: 72 years [49–80]) with identical CSF antibody staining by indirect immunofluorescence on mouse tissues. We identified AK5 as the antigen and confirmed with standardized assays. Three patients with clinical information had limbic encephalitis, inflammatory CSF and mesiotemporal lobe T2 hyperintensities that evolved to atrophy on brain MRI. One patient had burning smell sensation with no evidence of seizures. Despite immunotherapy, minimal improvement was noticed in one patient; all had severe memory deficits remaining.
AB - Adenylate kinase 5 (AK5) antibodies are biomarkers of a poorly responsive to immunotherapy, non-paraneoplastic, autoimmune limbic encephalitis. We detected 6 patients (all female, median age: 72 years [49–80]) with identical CSF antibody staining by indirect immunofluorescence on mouse tissues. We identified AK5 as the antigen and confirmed with standardized assays. Three patients with clinical information had limbic encephalitis, inflammatory CSF and mesiotemporal lobe T2 hyperintensities that evolved to atrophy on brain MRI. One patient had burning smell sensation with no evidence of seizures. Despite immunotherapy, minimal improvement was noticed in one patient; all had severe memory deficits remaining.
KW - Anterograde amnesia
KW - Limbic encephalitis
KW - Paraneoplastic neurological syndrome
KW - Phantosmia
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U2 - 10.1016/j.jneuroim.2022.577861
DO - 10.1016/j.jneuroim.2022.577861
M3 - Article
C2 - 35405429
AN - SCOPUS:85127718424
SN - 0165-5728
VL - 367
JO - Journal of neuroimmunology
JF - Journal of neuroimmunology
M1 - 577861
ER -