scholarly journals Glial Fibrillary Acidic Protein Astrocytopathy in Pediatric Patients: A Retrospective Study

2021 ◽  
Vol 8 ◽  
Author(s):  
Haixia Huang ◽  
Ke Bai ◽  
Yueqiang Fu ◽  
Siwei Lu ◽  
Yunni Ran ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Glial Fibrillary Acidic Protein ◽  
Pediatric Patients ◽  
Clinical Symptoms ◽  
Acidic Protein ◽  
Gadolinium Enhancement ◽  
Urine Retention ◽  
Limb Weakness ◽  
Disturbance Of Consciousness ◽  
Intravenous Steroids

Autoimmune glial fibrillary acidic protein astrocytopathy is a novel form of autoimmune meningoencephalitis related to GFAP autoantibodies. This condition is still being characterized, and few pediatric patients have been identified. Here, we report three patients presenting with fever, nausea, and headache, following progressive disturbance of consciousness, limb weakness, dyspnea, or urine retention. MRI analysis revealed that T2-hyperintense lesions, or enhancement of the meninges and spinal cord. CSF and serum analyses revealed they were positive for GFAP antibody, confirming GFAP astrocytopathy diagnosis. Treating the patients with IVIG, with or without intravenous steroids, gradually improved their clinical symptoms. Our findings indicate that GFAP astrocytopathy should be considered in children who are clinically diagnosed with meningoencephalitis, whether or not myelitis is present, and if the MRI reveals enhancement of meninges or spinal cord, T2-hyperintense lesions, or a pattern of linear perivascular gadolinium enhancement. Suspected cases should be tested for GFAP antibody as soon as possible because these patients may benefit from immunotherapy.

scholarly journals Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord

2020 ◽  
Vol 13 ◽  
pp. 175628642090997
Author(s):  
Xue-Lin Li ◽  
Jinming Han ◽  
Hao-Tian Zhao ◽  
You-Ming Long ◽  
Bing-Wei Zhang ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cerebrospinal Fluid ◽  
Glial Fibrillary Acidic Protein ◽  
Clinical Symptoms ◽  
Brain Lesions ◽  
Acidic Protein ◽  
Resonance Imaging ◽  
Contrast Enhanced ◽  
Blurry Vision ◽  
Sensory Abnormality

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality. GFAP-IgG was detected in her cerebrospinal fluid. Magnetic resonance imaging using both T2-weighted and contrast-enhanced T1-weighted images revealed a rare finding of lesions distributed mainly in the entire spinal cord rather than typical brain lesions. After treating with corticosteroids, her clinical symptoms were alleviated, and the spinal cord lesion enhancement was reduced. Our observations extend the clinical spectrum of autoimmune GFAP astrocytopathy. We suggest that rare distributed lesions in the entire spinal cord in patients with autoimmune GFAP astrocytopathy cannot be ignored by neurologists. The identification of potential atypical lesions broadens the understanding of autoimmune GFAP astrocytopathy.

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.

A Mesenchymal-Like ZEB1+Niche Harbors Dorsal Radial Glial Fibrillary Acidic Protein-Positive Stem Cells in the Spinal Cord

Stem Cells ◽  
2009 ◽  
Vol 27 (11) ◽  
pp. 2722-2733 ◽  
Author(s):  
Jean-Charles Sabourin ◽  
Karin B. Ackema ◽  
David Ohayon ◽  
Pierre-Olivier Guichet ◽  
Florence E. Perrin ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Stem Cells ◽  
Glial Fibrillary Acidic Protein ◽  
Acidic Protein ◽  
Radial Glial

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.

Lentiviral-mediated silencing of glial fibrillary acidic protein and vimentin promotes anatomical plasticity and functional recovery after spinal cord injury

2014 ◽  
Vol 93 (1) ◽  
pp. 43-55 ◽  
Author(s):  
Mathieu Desclaux ◽  
Florence E. Perrin ◽  
Anh Do-Thi ◽  
Monica Prieto-Cappellini ◽  
Minerva Gimenez y Ribotta ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Glial Fibrillary Acidic Protein ◽  
Functional Recovery ◽  
Acidic Protein ◽  
Anatomical Plasticity ◽  
Cord Injury

scholarly journals Abnormal Reaction to Central Nervous System Injury in Mice Lacking Glial Fibrillary Acidic Protein and Vimentin

1999 ◽  
Vol 145 (3) ◽  
pp. 503-514 ◽  
Author(s):  
Milos Pekny ◽  
Clas B. Johansson ◽  
Camilla Eliasson ◽  
Josefina Stakeberg ◽  
Åsa Wallén ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Glial Fibrillary Acidic Protein ◽  
Intermediate Filament ◽  
Glial Scar ◽  
Scar Formation ◽  
Brain Lesions ◽  
Acidic Protein ◽  
The Central Nervous System

In response to injury of the central nervous system, astrocytes become reactive and express high levels of the intermediate filament (IF) proteins glial fibrillary acidic protein (GFAP), vimentin, and nestin. We have shown that astrocytes in mice deficient for both GFAP and vimentin (GFAP−/−vim−/−) cannot form IFs even when nestin is expressed and are thus devoid of IFs in their reactive state. Here, we have studied the reaction to injury in the central nervous system in GFAP−/−, vimentin−/−, or GFAP−/−vim−/− mice. Glial scar formation appeared normal after spinal cord or brain lesions in GFAP−/− or vimentin−/− mice, but was impaired in GFAP−/−vim−/− mice that developed less dense scars frequently accompanied by bleeding. These results show that GFAP and vimentin are required for proper glial scar formation in the injured central nervous system and that some degree of functional overlap exists between these IF proteins.

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