Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy in Children: A Retrospective Study

2021 ◽  
Author(s):  
xiamei Zhuang ◽  
Ke Jin ◽  
Xiaoming Li ◽  
Junwei Li
Keyword(s):  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Pediatric Patients ◽  
Lactic Dehydrogenase ◽  
Subcortical White Matter ◽  
Acidic Protein ◽  
Cerebral White Matter ◽  
Periventricular White Matter ◽  
Cell Counts ◽  
Adenosine Deaminase Activity

Abstract Objective: To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.Method: Data from 11 pediatric patients with autoimmune GFAP astrocytopathywas retrospectively analyzed.Results: All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis, include fever (45.4%), headaches (27.3%), dizziness (18.2%), drowsiness (18.2%) and mental disorders (18.2%). Cerebrospinal fluid (CSF) were detected in all patients. The white cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions.Conclusion: The findings of pediatric patients with autoimmune GFAP astrocytopathy is different from the previous reports.

scholarly journals Glial fibrillary acidic protein as a biomarker for periventricular white matter injury

2013 ◽  
Vol 209 (1) ◽  
pp. 27.e1-27.e7 ◽  
Author(s):  
Amanda Stewart ◽  
Aylin Tekes ◽  
Thierry A.G.M. Huisman ◽  
Jacky M. Jennings ◽  
Marilee C. Allen ◽  
...  
Keyword(s):  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Acidic Protein ◽  
White Matter Injury ◽  
Periventricular White Matter

scholarly journals Changes in Plasma Glial Fibrillary Acidic Protein in Children Receiving Sevoflurane Anesthesia: A Preliminary Randomized Trial

2021 ◽  
Vol 10 (4) ◽  
pp. 662
Author(s):  
Eun-Hee Kim ◽  
Young-Eun Jang ◽  
Sang-Hwan Ji ◽  
Ji-Hyun Lee ◽  
Sung-Ae Cho ◽  
...  
Keyword(s):  
Glial Fibrillary Acidic Protein ◽  
Randomized Trial ◽  
Protein Concentration ◽  
Pediatric Patients ◽  
Neuronal Injury ◽  
Acidic Protein ◽  
Anesthesia Induction ◽  
Sevoflurane Anesthesia ◽  
Control Groups ◽  
Over Time

We investigated changes in plasma glial fibrillary acidic protein concentration during sevoflurane anesthesia induction in children < 3 years old and determined the effect of co-administering dexmedetomidine. This preliminary randomized trial included 60 pediatric patients who received sevoflurane anesthesia for >3 h. Patients were assigned to dexmedetomidine or control groups at a 1:1 ratio. The primary outcome was changes in plasma glial fibrillary acidic protein concentration of dexmedetomidine and control groups over time. Fifty-five patients were included in the final analysis. The median (interquartile range (IQR)) of the plasma glial fibrillary acidic protein level was 387.7 (298.9–510.8) pg·mL−1 immediately after anesthetic induction, 302.6 (250.9–412.5) pg·mL−1 at 30 min, and 321.9 (233.8–576.2) pg·mL−1 at 180 min after the first sample. These values did not change over time (p = 0.759). However, plasma glial fibrillary acidic protein increased after 180 min of infusion of dexmedetomidine compared with values at 30 min infusion (p = 0.04, mean difference and 95% confidence interval of 221.6 and 2.2 to 441.0 pg·mL−1). In conclusion, three hours of sevoflurane anesthesia in pediatric patients < 3 years old did not provoke neuronal injury assessed by the plasma biomarker. Further studies regarding the effect of prolonged dexmedetomidine infusion on anesthetic neuronal injury are required.

scholarly journals Glial Fibrillary Acidic Protein (GFAP) in Oligodendrogliomas: A Reflection of Transient GFAP Expression by Immature Oligodendroglia

1986 ◽  
Vol 13 (4) ◽  
pp. 307-311 ◽  
Author(s):  
V. Jagadha ◽  
W.C. Halliday ◽  
L.E. Becker
Keyword(s):  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Tumor Cells ◽  
Intermediate Filaments ◽  
Intermediate Filament ◽  
Immunohistochemical Staining ◽  
Neuron Specific Enolase ◽  
Acidic Protein ◽  
Gfap Expression ◽  
S 100

ABSTRACT:Fourteen pure oligodendrogliomas were studied by light- and electronmicroscopy and immunohistochemistry to examine glial fibrillary acidic protein (GFAP) positivity in the tumors. To compare the immunohistochemical staining patterns of neoplastic oligodendroglia and immature oligodendroglia, myelination glia in the white matter of eight normal brains from children under 6 months of age were studied. The tumors possessed light microscopic and ultrastructural features characteristic of oligodendrogliomas. Microtubules were found in the cytoplasm of nine tumors on electronmicroscopy. In one, intermediate filaments and microtubules were observed in occasional tumor cells with polygonal crystalline structures in the cytoplasm. Using the peroxidase-antiperoxidase technique, all specimens were stained for GFAP, vimentin, S-100 and neuron-specific enolase (NSE). In nine tumors, variable numbers of cells with an oligodendroglial morphology reacted positively for GFAP. All tumors were positive for S-100 and negative for vimentin and NSE. The myelination glia in the eight normal brains stained positively for GFAP but not for vimentin. Vimentin is expressed by developing, reactive and neoplastic astrocytes. Thus, GFAP positivity combined with vimentin negativity in both neoplastic and immature oligodendroglia suggests that GFAP positivity in oligodendrogliomas may reflect the transient expression of this intermediate filament by immature oligodendroglia.

scholarly journals The molecular cloning of glial fibrillary acidic protein in Gekko japonicus and its expression changes after spinal cord transection

2010 ◽  
Vol 15 (4) ◽  
Author(s):  
Dehong Gao ◽  
Yongjun Wang ◽  
Yan Liu ◽  
Fei Ding ◽  
Xiaosong Gu ◽  
...  
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Acidic Protein ◽  
Time Interval ◽  
Short Time Interval ◽  
Specific Marker ◽  
Class Iii ◽  
Intermediate Filament Proteins ◽  
Gekko Japonicus

AbstractThe glial fibrillary acidic protein (GFAP) is an astrocyte-specific member of the class III intermediate filament proteins. It is generally used as a specific marker of astrocytes in the central nervous system (CNS). We isolated a GFAP cDNA from the brain and spinal cord cDNA library of Gekko japonicus, and prepared polyclonal antibodies against gecko GFAP to provide useful tools for further immunochemistry studies. Both the real-time quantitative PCR and western blot results revealed that the expression of GFAP in the spinal cord after transection increased, reaching its maximum level after 3 days, and then gradually decreased over the rest of the 2 weeks of the experiment. Immunohistochemical analyses demonstrated that the increase in GFAP-positive labeling was restricted to the white matter rather than the gray matter. In particular, a slight increase in the number of GFAP positive star-shaped astrocytes was detected in the ventral and lateral regions of the white matter. Our results indicate that reactive astrogliosis in the gecko spinal cord took place primarily in the white matter during a short time interval, suggesting that the specific astrogliosis evaluated by GFAP expression might be advantageous in spinal cord regeneration.

scholarly journals 079 Glial fibrillary acidic protein (GFAP) astrocytopathy associated with cerebral micro-infarction and poor therapeutic response

2019 ◽  
Vol 90 (e7) ◽  
pp. A25.2-A25 ◽  
Author(s):  
Allycia MacDonald ◽  
James Triplett ◽  
Srimathy Vijayan ◽  
Michael Bynevelt ◽  
Rahul Lakshmanan ◽  
...  
Keyword(s):  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Early Recognition ◽  
Oral Intake ◽  
Brain Biopsy ◽  
Acidic Protein ◽  
Gait Disturbance ◽  
High Dose ◽  
Elevated Protein ◽  
Upper Thoracic

IntroductionGlial fibrillary acidic protein (GFAP) astrocytopathy is a lesser recognised immune-mediated meningo-encephalomyelitis, which is steroid responsive in the majority of cases. Neuroimaging is unique with a distinctive symmetric white matter perivascular linear and punctate enhancement pattern. We present a case with classical phenotype but delayed clinical response, and highlight the importance of early recognition and treatment.CaseA 59-year-old Caucasian female presented with a two month history of headache, gait disturbance, insomnia, agitation, disorientation and reduced oral intake. Examination revealed a high frequency upper limb tremor, hypertonicity and pathologically brisk reflexes with impaired cognitive function. MRI brain and spinal cord demonstrated high T2 signal and striking perivascular and punctate enhancement in supratentorial white matter, cervical and upper thoracic cord. CSF examination revealed lymphocytic pleocytosis and elevated protein. Brain biopsy demonstrated reduced GFAP expression, perivascular T-lymphocytic infiltrate, and recent white matter microinfarction. CSF and serum GFAP antibodies were positive.Motor deterioration accompanied progression to a stuporous state. High dose corticosteroids were commenced, followed by intravenous immunoglobulin and mycophenolate. While there was marked improvement of perivascular contrast enhancement on imaging, the patient continued to demonstrate prominent tremor, gait disturbance and behavioural issues 9 months following symptom onset.ConclusionsThe persistence of disability in this case is likely the result of axonal loss from the initial insult, reflected by the biopsy evidence of microinfarction. Awareness of the unique pattern on MRI and the clinical phenotype will aid in early recognition and prompt treatment of this condition, thus preventing the potential long term morbidity.

scholarly journals Cerebral Blood Flow Heterogeneity in Preterm Sheep: Lack of Physiologic Support for Vascular Boundary Zones in Fetal Cerebral White Matter

2007 ◽  
Vol 28 (5) ◽  
pp. 995-1008 ◽  
Author(s):  
Melissa M McClure ◽  
Art Riddle ◽  
Mario Manese ◽  
Ning Ling Luo ◽  
Dawn A Rorvik ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
White Matter ◽  
Ischemia Reperfusion ◽  
Three Dimensional ◽  
Cerebral White Matter ◽  
Periventricular White Matter ◽  
Fetal Sheep ◽  
Border Zones ◽  
Neurologic Disability

Periventricular white matter (PVWM) injury is the leading cause of neurologic disability in survivors of prematurity. To address the role of ischemia in PVWM and cerebral cortical injury, we hypothesized that immaturity of spatially distal vascular ‘end zones’ or ‘border zones’ predisposes PVWM to greater decreases in cerebral blood flow (CBF) than more proximal structures. We quantified regional CBF with fluorescently labeled microspheres in 0.65 gestation fetal sheep in histopathologically defined three-dimensional regions by post hoc digital dissection and coregistration algorithms. Basal flow in PVWM was significantly lower than in gyral white matter and cortex, but was equivalent in superficial, middle, and deep PVWM. Absolute and relative CBF (expressed as percentage of basal) did not differ significantly during ischemia or reperfusion between PVWM, gyral white matter, or cortex. Moreover, CBF during ischemia-reperfusion was equivalent in three adjacent PVWM levels and was not consistent with the magnitude of severity of PVWM injury, defined by TUNEL (terminal deoxynucleotidyltransferase-mediated dUPT nick end labeling) staining. However, the magnitude of ischemia was predicted by the severity of discrete cortical lesions. Hence, unlike cerebral cortex, unique CBF disturbances did not account for the distribution of PVWM injury. Previously defined cellular maturational factors, thus, appear to have a greater influence on PVWM vulnerability to ischemic injury than the presence of immature vascular boundary zones.

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