Rate of Leptomeningeal Enhancement in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody–Associated Encephalomyelitis

2021 ◽  
Vol 36 (11) ◽  
pp. 1042-1046
Author(s):  
Judith A. Gadde ◽  
David S. Wolf ◽  
Stephanie Keller ◽  
Grace Y. Gombolay

Introduction: Myelin oligodendrocyte glycoprotein antibodies (MOG-abs) are associated with demyelinating diseases. Leptomeningeal enhancement occurs in 6% of adult MOG-abs patients but rates in pediatric MOG-abs patients are unknown. Methods: Retrospective review of pediatric MOG-abs patients was performed. Results: Twenty-one patients (7 boys, 14 girls) were included with an average age of 8.6 years (range 2-15 years). Seven of 21 (33%) pediatric MOG-abs patients had leptomeningeal enhancement. Two patients’ relapses were manifested by leptomeningeal enhancement alone and another patient presented with seizures, encephalopathy, and aseptic meningitis without demyelinating lesions. Cerebrospinal fluid pleocytosis was seen in both leptomeningeal (4/7 patients) and nonleptomeningeal enhancement (10/14 patients). Interestingly, 3 patients with leptomeningeal enhancement had normal cerebrospinal fluid white blood cell count. Cortical edema was more likely in patients with leptomeningeal enhancement ( P = .0263). Conclusion: We expand the clinical spectrum of anti-MOG antibody–associated disorder. Patients with recurrent leptomeningeal enhancement without demyelinating lesions should be tested for MOG antibodies.

2020 ◽  
Vol 41 (9) ◽  
pp. 2635-2638 ◽  
Author(s):  
Eiichiro Amano ◽  
Akira Machida ◽  
Naomi Kanazawa ◽  
Takahiro Iizuka

2020 ◽  
Vol 65 (No. 7) ◽  
pp. 297-300 ◽  
Author(s):  
A Galan ◽  
A Seisdedos-Benzal ◽  
BE Carletti ◽  
S Quiros ◽  
EM Martin ◽  
...  

The analysis of cerebrospinal fluid biomarkers provides a wide range of information about the neurological health of a patient. Lactate is a metabolic precursor necessary for brain gluconeogenesis. When oxidative impairment or mitochondrial damage is present, lactate alteration occurs. The rostro-caudal dynamics of cerebrospinal biomarkers along the craniospinal axis in humans and horses was demonstrated in other studies. To the authors’ knowledge, no clinical study has, so far, investigated the cerebrospinal fluid lactate concentration in dogs in association with the puncture site. The purpose of this study was to compare the cerebellomedullary cistern and lumbar cistern cerebrospinal fluid lactate concentrations in healthy dogs. Cerebellomedullary and lumbar cerebrospinal fluids were collected for the cell count, total protein determination and lactate analysis from ten healthy Beagle dogs. The results revealed a significantly increased lumbar cerebrospinal fluid lactate concentration when compared with the cerebellomedullary cistern level. The results included: the total nucleated cell count < 5 cells/µl, the red blood cell count < 500 cell/µl, the total proteins < 0.3 g/l, as well as the cerebellomedullary lactate values (1.44 ± 0.06 mM/l) and the lumbar cistern lactate values (1.58 ± 0.1 mM/l). The results of this study highlight useful data that help to understand the physiological lactate variations depending on the cerebrospinal fluid puncture site.


2020 ◽  
pp. 135245852095104
Author(s):  
Amy Kunchok ◽  
Eoin P Flanagan ◽  
Karl N Krecke ◽  
John J Chen ◽  
J Alfredo Caceres ◽  
...  

Background: The presence of co-existent neuronal antibodies (neuronal-IgG) in patients with myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG1) is not yet well understood. Objectives: The aim of this study was to investigate the co-existence of a broad range of neuronal-IgG in MOG-IgG1+ patients. Methods: MOG-IgG1+ patients were tested for 17 neuronal-IgGs in cerebrospinal fluid (CSF) and serum including NMDA-R-IgG, AMPA-R-IgG, GABAB-R-IgG, LGI1-IgG, CASPR2-IgG, GABAA-R-IgG, GAD65-IgG, mGLUR1-IgG, DPPX-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1,2,Tr, and ANNA1,2,3. Clinical and radiological features of MOG-IgG1+ with NMDA-R-IgG in CSF were compared to a control cohort of MOG-IgG1+ patients without NMDA-R-IgG. Results: A total of 376 MOG-IgG1+ patients underwent testing for neuronal-IgGs. Serum testing for neuronal-IgGs (113 adults, 142 children) identified one child with NMDA-R-IgG (0.7%), one child with CASPR2-IgG (0.7%), one adult with LGI1-IgG (0.9%) and one adult with GABAA-R-IgG (0.9%). CSF testing for neuronal-IgGs (97 adults, 169 children) identified seven children (4%) and seven adults (7%) with NMDA-R-IgG, and one adult with GABAA-R-IgG (1%). The MOG-IgG1+/NMDA-R-IgG+ patients had a median age of 17 (range: 2–39) years. Features associated with MOG-IgG1+/NMDA-R-IgG+ included encephalopathy ( p = 0.001), seizures ( p = 0.045), and leptomeningeal enhancement ( p = 0.045). Conclusion: NMDA-R-IgG was the most frequently detected neuronal-IgG to co-exist with MOG-IgG1. MOG-IgG1+/NMDA-R-IgG+ patients most often presented with encephalopathy and seizures. Testing for MOG-IgG1 and NMDA-R-IgG may be warranted in patients with encephalopathy and inflammatory demyelinating syndromes.


Author(s):  
Kiem Hao Tran

Meningoencephalitis is not a rare disease in children. However, eosinophilic meningitis due to Angiostrongylus cantonensis is unusual in pediatric population. We describe the case of a 12-year-old girl from central zone of Vietnam with eosinophilic meningitis. The patient lived in a rural area, where farming is widespread, and presented with fever and headache. Laboratory results showed peripheral eosinophilia, cerebrospinal fluid white blood cell count 730/mm3 with many of eosinophils, cerebrospinal fluid ELISA positive for Angiostrongylus cantonensis, and blood ELISA positive for A. cantonensis. The presentation was consistent with a diagnosis of A. cantonensis eosinophilic meningitis. The patient recovered fully after administering albendazole (800mg/day for 2 weeks), and intravenous dexamethasone (0.6 mg/kg/day every 8 hours) and mannitol (1.5 g/kg/day every 8 hours) for the first 3 days, followed by 5 days of oral prednisolone (2 mg/kg/day).


PEDIATRICS ◽  
1995 ◽  
Vol 96 (1) ◽  
pp. 48-51
Author(s):  
Carol Carraccio ◽  
Krystyna Blotny ◽  
Margaret C. Fisher

Objective. Experience led us to question the applicability of standards for normal cerebrospinal fluid (CSF), originally developed in healthy children, to children with systemic illness but without central nervous system (CNS) infection. The purpose of this study was to test our hypothesis that systemically ill children, in the absence of CNS infection, have an elevated CSF white blood cell count and a greater percentage of neutrophils than accepted norms. Methods. We enrolled 345 patients in the following diagnostic categories: infants 1 month of age or younger with possible sepsis (n = 95), patients older than 1 month of age with possible sepsis (n = 155), patients with a focus of infection in close proximity to the CNS (n = 51), and patients presenting with seizures and fevers (n = 45). Sociodemographic data and results of CSF examination were abstracted from the medical records. Statistical analysis systems were used for data processing. Results. The CSF white blood cell count did not significantly differ from standards except for a lower mean count in the group presenting with seizures. The percent of CSF neutrophils was significantly greater than standards, however, for those patients older than 1 month of age with possible sepsis, those with a focus of infection in close proximity to the CNS, and those presenting with seizures. Data analysis by quantiles shows only 25% to 50% of patients, in each of the diagnostic categories, meeting the current definition of normal CSF neutrophil count. Conclusions. Our results show that a mean of at least 5% neutrophils may be present in the CSF with a diagnosis of fever without a source, a focus of infection in close proximity to the CNS, or a seizure with fever in the absence of CNS infection. These data support tailoring treatment based on clinical assessment rather than what is considered an abnormal CSF neutrophil count by current standards.


2010 ◽  
Vol 17 (3) ◽  
pp. 297-302 ◽  
Author(s):  
PH Lalive ◽  
MG Häusler ◽  
H Maurey ◽  
Y Mikaeloff ◽  
M Tardieu ◽  
...  

Background: Myelin oligodendrocyte glycoprotein (MOG) may be implicated in the immunopathogenesis of multiple sclerosis (MS) inducing demyelination in the animal model of MS. In adults reported anti-MOG antibody frequencies have been variable across a number of studies and can also be detected in controls. Objective: To measure antibodies against MOG in paediatric patients with demyelinating disorders of the central nervous system and in controls. Methods: Serum antibodies against MOG and myelin basic protein were measured by ELISA, flow cytometry (FACS) and in the liquid phase in 11 children with acute disseminated encephalomyelitis (ADEM), 22 children with MS, seven children with acute viral encephalitis and 13 healthy controls. The serostatus of Epstein–Barr virus (EBV) infections were assessed. Results: Anti-MOG antibodies, measured either by ELISA or FACS were exclusively detected in children with demyelination. In ADEM these antibodies were highly reactive. Anti-MBP reactivity was detectable equally in all groups. The presence of either autoantibodies did not associate with EBV serostatus, age, gender or disease course. Conclusions: This study independently corroborates recently published results of seroprevalence and specificity of the assay. Due to their low sensitivity anti-MOG antibodies will not serve as disease-specific biomarkers, but could help to support the diagnosis of ADEM in difficult cases.


Sign in / Sign up

Export Citation Format

Share Document