scholarly journals Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Associated With Area Postrema Syndrome: A Case Report

2021 ◽  
Vol 12 ◽  
Author(s):  
Xin Gao ◽  
Ying Tang ◽  
Guo-Dong Yang ◽  
Wu Wei
Keyword(s):  
Cerebrospinal Fluid ◽  
Glial Fibrillary Acidic Protein ◽  
Area Postrema ◽  
Clinical Manifestations ◽  
Acidic Protein ◽  
High Signal ◽  
Mri Imaging ◽  
Predominant Symptom ◽  
The Central Nervous System ◽  
Dorsal Medulla

Glial fibrillary acidic protein astrocytopathy is an immunotherapy-responsive autoimmune disease of the central nervous system with various clinical manifestations; among these, there are few reports about area postrema syndrome (APS). The authors present the case of a female patient admitted to the hospital with intractable nausea and vomiting as the predominant symptom. The patient's cerebrospinal fluid was tested by cell-based assays (CBA) and found positive for the presence of anti-glial fibrillary acidic protein (GFAP) antibody, in addition, serological testing showed elevated levels of thyroglobulin and thyroperoxidase-specific antibodies. Brain and cervical MRI showed abnormally high signal on the T2 sequence in the dorsal medulla oblongata and right pontine arm. Therefore, the patient was diagnosed with autoimmune GFAP astrocytopathy. The symptoms improved rapidly after treatment with corticosteroids, and no recurrence has been observed thus far. APS may be a relatively rare clinical manifestation of GFAP astrocytopathy. Importantly, such presentation is challenging to correctly diagnose without typical MRI imaging findings. However, the detection of antibodies in the cerebrospinal fluid or serum may be valuable. Systemic and neurological autoimmunity often coexist, comprehensive antibody screening should be conducted.

Cerebrospinal fluid neurofilament and glial fibrillary acidic protein in patients with cerebral vasculitis

2002 ◽  
Vol 67 (6) ◽  
pp. 844-851 ◽  
Author(s):  
K. Nylén ◽  
J-E. Karlsson ◽  
C. Blomstrand ◽  
A. Tarkowski ◽  
E. Trysberg ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Glial Fibrillary Acidic Protein ◽  
Cerebral Vasculitis ◽  
Acidic Protein

scholarly journals Refining the concept of GFAP toxicity in Alexander disease

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Albee Messing
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Glial Fibrillary Acidic Protein ◽  
Intermediate Filament ◽  
Alexander Disease ◽  
Acidic Protein ◽  
Sequence Variants ◽  
Main Body ◽  
Gain Of Function ◽  
The Central Nervous System

Abstract Background Alexander disease is caused by dominantly acting mutations in glial fibrillary acidic protein (GFAP), the major intermediate filament of astrocytes in the central nervous system. Main body In addition to the sequence variants that represent the origin of disease, GFAP accumulation also takes place, together leading to a gain-of-function that has sometimes been referred to as “GFAP toxicity.” Whether the nature of GFAP toxicity in patients, who have mixtures of both mutant and normal protein, is the same as that produced by simple GFAP excess, is not yet clear. Conclusion The implications of these questions for the design of effective treatments are discussed.

The area postrema plays no role in the pressor action of angiotensin in the rat

1980 ◽  
Vol 239 (1) ◽  
pp. H108-H113 ◽  
Author(s):  
J. R. Haywood ◽  
G. D. Fink ◽  
J. Buggy ◽  
M. I. Phillips ◽  
M. J. Brody
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Carotid Arteries ◽  
Area Postrema ◽  
Renal Hypertension ◽  
Conscious Rat ◽  
The Central Nervous System ◽  
Pressor Activity ◽  
The Brain

The area postrema has been shown to have a major role in mediating the pressor effects of peripheral angiotensin in the dog, cat, and rabbit. The purpose of this study was to ascertain the function of the medullary circumventricular structure in the conscious rat. The pressor potency of angiotensin administered into the vertebral and carotid arteries was compared with intra-aortic infusions of angiotensin. Although no difference in pressor activity of angiotensin could be detected between intraaortic and intravertebral administration, greater sensitivity was observed during intracarotid infusion. No difference in the course of one-kidney renal hypertension was observed between sham-lesioned rats and animals with an area postrema lesion. In addition, lesioned and sham-lesioned animals showed equivalent responses to graded doses of angiotensin administered either intravenously or into the lateral ventricle. It was concluded that in the rat the area postrema plays no role in mediating the central nervous system actions of angiotensin whether the peptide reaches the brain via the blood or the cerebrospinal fluid.

Area postrema syndrome: Another feature of anti-GFAP encephalomyelitis

2019 ◽  
Vol 26 (2) ◽  
pp. 253-255 ◽  
Author(s):  
Jonathan Ciron ◽  
Fanny Sourdrille ◽  
Damien Biotti ◽  
Thierry Tchoumi ◽  
Anne Ruiz ◽  
...  
Keyword(s):  
Glial Fibrillary Acidic Protein ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Acidic Protein ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Spectrum Disorders

Anti–Glial fibrillary acidic protein (GFAP) encephalomyelitis is a recently described entity and while the spectrum of this disease has been explored, further research is needed to fully describe its phenotype. Area postrema syndrome (APS) is usually associated with neuromyelitis optica spectrum disorders (NMOSDs), whereas no case of APS has been previously reported with anti-GFAP encephalomyelitis. In this article, we report a case of APS in a 41-year-old woman in the context of anti-GFAP encephalomyelitis. This case was not associated with additional anti-AQP4 IgG and therefore extends the clinico-radiological spectrum of anti-GFAP encephalomyelitis.

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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