Bahadir Konuskan1, Mirac Yildirim2, Haluk Topaloglu3, Ilknur Erol4, Ulkuhan Oztoprak5, Huseyin Tan6, Rahsan Gocmen7, Banu Anlar8. 1. Department of Pediatric Neurology, Hacettepe University Hospital, Ankara, Turkey. Electronic address: bahadirkonuskan@gmail.com. 2. Department of Pediatric Neurology, Hacettepe University Hospital, Ankara, Turkey. Electronic address: miracyildirim@hacettepe.edu.tr. 3. Department of Pediatric Neurology, Hacettepe University Hospital, Ankara, Turkey. Electronic address: htopalog@hacettepe.edu.tr. 4. Department of Pediatric Neurology, Baskent University Hospital, Adana, Turkey. Electronic address: ilknur_erol@yahoo.com. 5. Department of Pediatric Neurology, Dr. Sami Ulus Childrens Hospital, Ankara, Turkey. Electronic address: ulkuhantoprak@yahoo.com. 6. Department of Pediatric Neurology, Ataturk University Hospital, Erzurum, Turkey. Electronic address: htan@atauni.edu.tr. 7. Department of Radiology, Hacettepe University Hospital, Ankara, Turkey. Electronic address: gocmentr@yahoo.com. 8. Department of Pediatric Neurology, Hacettepe University Hospital, Ankara, Turkey. Electronic address: banlar@hacettepe.edu.tr.
Abstract
OBJECTIVE: The symptomatology and paraclinical findings of antibody-mediated encephalitis, a relatively novel disorder, are still being characterized in adults and children. A high index of suspicion is needed in order to identify these cases among children presenting with various neurological symptoms. The aim of this study is to examine the clinical, demographic and laboratory findings and outcome of children with anti-NMDAR and anti-VGKC encephalitis for any typical or distinctive features. METHODS: Cases diagnosed with anti-N-Methyl d-aspartate receptor (NMDAR) and anti-voltage gated potassium channel (VGKC) antibody-mediated encephalopathy in four major child neurology centers are described. RESULTS: In four years, 16 children with NMDAR and 8 children with VGKC antibody-associated disease were identified in the participating centers. The most frequent initial manifestation consisted of generalized seizures and cognitive symptoms in both groups. Movement abnormalities were frequent in anti-NMDAR patients and autonomic symptoms, in anti-VGKC patients. Cerebrospinal fluid (CSF) protein, cell count and IgG index were normal in 9/15 anti-NMDAR and 5/8 anti-VGKC patients tested. EEG and MRI findings were usually nonspecific and non-contributory. The rate and time of recovery was not related to age, sex, acute or subacute onset, antibody type, MRI, EEG or CSF results. Treatment within 3 months of onset was associated with normal neurological outcome. CONCLUSIONS: Our results suggest anti-NMDAR and VGKC encephalopathies mostly present with non-focal neurological symptoms longer than 3 weeks. In contrast with adult cases, routine CSF testing, MRI and EEG did not contribute to the diagnosis in this series.
OBJECTIVE: The symptomatology and paraclinical findings of antibody-mediated encephalitis, a relatively novel disorder, are still being characterized in adults and children. A high index of suspicion is needed in order to identify these cases among children presenting with various neurological symptoms. The aim of this study is to examine the clinical, demographic and laboratory findings and outcome of children with anti-NMDAR and anti-VGKC encephalitis for any typical or distinctive features. METHODS: Cases diagnosed with anti-N-Methyl d-aspartate receptor (NMDAR) and anti-voltage gated potassium channel (VGKC) antibody-mediated encephalopathy in four major child neurology centers are described. RESULTS: In four years, 16 children with NMDAR and 8 children with VGKC antibody-associated disease were identified in the participating centers. The most frequent initial manifestation consisted of generalized seizures and cognitive symptoms in both groups. Movement abnormalities were frequent in anti-NMDARpatients and autonomic symptoms, in anti-VGKC patients. Cerebrospinal fluid (CSF) protein, cell count and IgG index were normal in 9/15 anti-NMDAR and 5/8 anti-VGKC patients tested. EEG and MRI findings were usually nonspecific and non-contributory. The rate and time of recovery was not related to age, sex, acute or subacute onset, antibody type, MRI, EEG or CSF results. Treatment within 3 months of onset was associated with normal neurological outcome. CONCLUSIONS: Our results suggest anti-NMDAR and VGKC encephalopathies mostly present with non-focal neurological symptoms longer than 3 weeks. In contrast with adult cases, routine CSF testing, MRI and EEG did not contribute to the diagnosis in this series.