scholarly journals Autoimmune glial fibrillary acidic protein astrocytopathy presented as ataxia, myoclonus and bulbar syndrome: a case report and review of the literature

2021 ◽  
Vol 3 (2) ◽  
pp. e000142
Author(s):  
Azael C Novo ◽  
Begoña Venegas Pérez
Keyword(s):  
Glial Fibrillary Acidic Protein ◽  
Acidic Protein ◽  
Review Of The Literature ◽  
Atypical Case ◽  
Neurological Improvement ◽  
Csf Analysis ◽  
Elevated Protein ◽  
Progressive Ataxia ◽  
Atypical Manifestations ◽  
Elevated Protein Level

ObjectiveTo describe an atypical case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.ResultsA patient in his 60s presented with 6 months of progressive ataxia, proximal myoclonus and bulbar symptomatology. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level and elevated adenosine deaminase (ADA) level. CSF microbiological studies were negative and brain and cervical MRI showed no significant findings. We tested for nuclear, cytoplasmatic and synaptic neural autoantibodies as well as anti-GFAP antibodies. While awaiting these results, the patient was commenced on methylprednisolone boluses (1 g/day for 5 days), noting rapid neurological improvement. Eventually, CSF tests were positive for anti-GFAP antibodies.ConclusionWe report atypical manifestations of GFAP astrocytopathy. Further research is needed to fully understand the spectrum of neurological manifestations of this autoimmune disease and facilitate timely diagnosis.

scholarly journals Autoimmune glial fibrillary acidic protein astrocytopathy manifesting as subacute meningoencephalitis with descending myelitis: a case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Han Wang ◽  
Jerome H. Chin ◽  
Bo-yan Fang ◽  
Xi Chen ◽  
Ai-lin Zhao ◽  
...  
Keyword(s):  
Glial Fibrillary Acidic Protein ◽  
Brain Magnetic Resonance Imaging ◽  
Repeated Administration ◽  
Acidic Protein ◽  
Csf Analysis ◽  
Elevated Protein ◽  
Low Glucose ◽  
Csf Pleocytosis ◽  
Magnetic Resonance Imaging Mri ◽  
Tuberculosis Therapy

Abstract Background Glial fibrillary acidic protein (GFAP) autoimmune astrocytopathy is characterized by GFAP autoantibody positive encephalitis, meningoencephalitis or meningoencephalomyelitis. The initial clinical presentation may be similar to central nervous system infections making early diagnosis challenging. Case presentation A Chinese female patient presented with subacute meningitis with symptoms of headache, vomiting, and fever. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level, low glucose level, and negative basic microbiological studies including Xpert MTB/RIF. Brain magnetic resonance imaging (MRI) showed bilateral cerebral cortical and white matter hyperintensities on FLAIR sequences. The patient was diagnosed with possible tuberculous meningitis and started on anti-tuberculosis therapy (ATT). Three months later, the patient developed cervical myelopathy and encephalopathy with persistent CSF pleocytosis. Five months later, tissue-based and cell-based assays demonstrated GFAP antibodies in blood and CSF. Her symptoms improved with repeated administration of intravenous immunoglobulin (IVIG) and corticosteroids. One-and-a-half -year follow-up showed neither clinical progression nor relapses. Conclusions Anti-GFAP astrocytopathy should be included in the differential diagnosis of patients who present with subacute meningitis with negative microbiological studies and a progressive clinical course including encephalitis and/or myelitis.

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 Primary Diffuse Leptomeningeal Gliomatosis

1985 ◽  
Vol 12 (3) ◽  
pp. 278-281 ◽  
Author(s):  
Peter Bailey ◽  
Yves Robitaille
Keyword(s):  
Cerebrospinal Fluid ◽  
Glial Fibrillary Acidic Protein ◽  
Review Of The Literature ◽  
Chronic Meningitis ◽  
Primary Diffuse Leptomeningeal Gliomatosis ◽  
Fungal Meningitis ◽  
Csf Cells ◽  
Elevated Protein ◽  
Cranial Nerve Palsies ◽  
Low Glucose

ABSTRACT:A review of the literature on primary diffuse meningeal gliomatosis (DMG) yielded three cases and we report a fourth. DMG is a syndrome characterized by extensive basal and spinal chronic meningitis with mental confusion, headaches, diplopia, papilledema and cranial nerve palsies. The cerebrospinal fluid (CSF) has a markedly elevated protein content, moderate mononuclear pleocytosis and a normal or low glucose. This picture invariably leads to the diagnosis and treatment of tuberculous or fungal meningitis despite persistently negative cerebrospinal fluid (CSF) cultures. Reaction of exfoliated CSF cells with glial fibrillary acidic protein (GFAP) immunoperoxidase labelled antibody is suggested as a diagnostic tool. A basal meningeal biopsy appears to be the only alternative diagnostic approach.

scholarly journals THE POSSIBILITY OF USING GLIAL FIBRILLARY ACIDIC PROTEIN AS A BIOMARKER IN THE ACUTE PERIOD OF STROKE

2019 ◽  
pp. 4-15
Author(s):  
M. P. Topuzova ◽  
T. M. Alekseeva ◽  
E. B. Panina ◽  
T. V. Vavilova ◽  
O. A. Portik ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Glial Fibrillary Acidic Protein ◽  
Disease Control ◽  
Brain Tissue ◽  
High Mortality ◽  
Acidic Protein ◽  
Review Of The Literature ◽  
Therapeutic Approaches ◽  
Acute Period ◽  
The World

High mortality and disability resulting from a stroke throughout the world is the cause of fi nding new opportunities to improve early diagnosis and optimize therapeutic approaches. The article presents a review of the literature devoted to the study of glial fi brillary acidic protein (GFAP) as a biomarker in the acute period of stroke, which showed that its use is possible not only to diff erentiate the ischemic and hemorrhagic process, but also to determine the degree of destruction of brain tissue, as in the fi rst hours of a stroke, and in the dynamics. In addition, the GFAP analysis can be useful for monitoring the course of the disease, control the eff ectiveness of therapy, as well as to predict outcomes.

Expression of glial fibrillary acidic protein, actin, fibronectin and factor VIII antigen in human astrocytomas

Pathology ◽  
1983 ◽  
Vol 15 (4) ◽  
pp. 373-378 ◽  
Author(s):  
Stephen J. Lolait ◽  
J.H. Harmer ◽  
G. Auteri ◽  
J.S. Pedersen ◽  
B.H. Toh
Keyword(s):  
Glial Fibrillary Acidic Protein ◽  
Factor Viii ◽  
Acidic Protein ◽  
Factor Viii Antigen ◽  
Human Astrocytomas
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