Shermyn Neo1, Tianrong Yeo2, Zhiyong Chen2, Nerice Heng Wen Ngiam3, Ethanyn Tyen-Xin Lim4, Kevin Tan5, Tchoyoson Choie Cheio Lim6. 1. Department of Neurology, National Neuroscience Institute, Singapore. Electronic address: shermyn.neo.x.m@singhealth.com.sg. 2. Department of Neurology, National Neuroscience Institute, Singapore. 3. Department of Anaesthesiology, Singapore General Hospital, Singapore. 4. Department of Diagnostic Radiology, National University Health System, Singapore. 5. Department of Neurology, National Neuroscience Institute, Singapore; Duke-NUS Medical School, Singapore. 6. Duke-NUS Medical School, Singapore; Department of Neuroradiology, National Neuroscience Institute, Singapore.
Abstract
BACKGROUND: Anti-N-methyl-d-aspartate receptor (NMDAR) and anti-voltage-gated potassium channel (VGKC) encephalitis are the commonest antibody-associated autoimmune encephalitides (AIE). Acute clinical features have been well-described, but data on the role of radiological findings in diagnosis and prognosis of AIE are limited. METHODS: Anti-NMDAR and anti-VGKC encephalitis patients from the National Neuroscience Institute were identified. We compared clinical and paraclinical features, at acute presentation and on follow-up between and within groups. RESULTS: Twenty-six anti-NMDAR and 11 anti-VGKC encephalitis patients were reviewed. At acute presentation, dysautonomia (57.7%) and impairment of consciousness (84.6%) occurred exclusively in anti-NMDAR encephalitis. Cerebrospinal fluid pleocytosis was more common in anti-NMDAR encephalitis (88.5% vs 20.0%, p = 0.003), while ictal electroencephalography abnormalities were more frequent in anti-VGKC encephalitis (11.5% vs 45.5%, p = 0.022). On acute imaging, leptomeningeal enhancement was seen only in anti-NMDAR encephalitis (37.5%), while hippocampal T2 hyperintensities supported the diagnosis of anti-VGKC encephalitis (63.6% vs 12.5%, p = 0.002). At follow-up (median 53.0 months, range 13.0-119.0), anti-NMDAR encephalitis patients had better modified Rankin scale scores (median 0.0 vs 3.0, p = 0.023). Relapses occurred equally in both groups. Anti-VGKC encephalitis patients with abnormal acute MRI were more likely to have poor outcomes compared to those with normal imaging (100% vs 25%, p = 0.008), whereas acute imaging features in anti-NMDAR encephalitis did not predict long-term outcomes. CONCLUSIONS: Acute MRI findings can aid in early diagnosis and prognostication in suspected AIE. Leptomeningeal enhancement in anti-NMDAR encephalitis and hippocampal lesions in anti-VGKC encephalitis, together with typical clinical features, may allow distinction between these antibody subtypes, and specific abnormal imaging features in anti-VGKC encephalitis may be used as a prognostic marker.
BACKGROUND: Anti-N-methyl-d-aspartate receptor (NMDAR) and anti-voltage-gated potassium channel (VGKC) encephalitis are the commonest antibody-associated autoimmune encephalitides (AIE). Acute clinical features have been well-described, but data on the role of radiological findings in diagnosis and prognosis of AIE are limited. METHODS: Anti-NMDAR and anti-VGKC encephalitispatients from the National Neuroscience Institute were identified. We compared clinical and paraclinical features, at acute presentation and on follow-up between and within groups. RESULTS: Twenty-six anti-NMDAR and 11 anti-VGKC encephalitispatients were reviewed. At acute presentation, dysautonomia (57.7%) and impairment of consciousness (84.6%) occurred exclusively in anti-NMDAR encephalitis. Cerebrospinal fluid pleocytosis was more common in anti-NMDAR encephalitis (88.5% vs 20.0%, p = 0.003), while ictal electroencephalography abnormalities were more frequent in anti-VGKC encephalitis (11.5% vs 45.5%, p = 0.022). On acute imaging, leptomeningeal enhancement was seen only in anti-NMDAR encephalitis (37.5%), while hippocampal T2 hyperintensities supported the diagnosis of anti-VGKC encephalitis (63.6% vs 12.5%, p = 0.002). At follow-up (median 53.0 months, range 13.0-119.0), anti-NMDAR encephalitispatients had better modified Rankin scale scores (median 0.0 vs 3.0, p = 0.023). Relapses occurred equally in both groups. Anti-VGKC encephalitispatients with abnormal acute MRI were more likely to have poor outcomes compared to those with normal imaging (100% vs 25%, p = 0.008), whereas acute imaging features in anti-NMDAR encephalitis did not predict long-term outcomes. CONCLUSIONS: Acute MRI findings can aid in early diagnosis and prognostication in suspected AIE. Leptomeningeal enhancement in anti-NMDAR encephalitis and hippocampal lesions in anti-VGKC encephalitis, together with typical clinical features, may allow distinction between these antibody subtypes, and specific abnormal imaging features in anti-VGKC encephalitis may be used as a prognostic marker.