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1.
Eur J Neurol ; 27(11): 2381-2384, 2020 11.
Article in English | MEDLINE | ID: mdl-32248601

ABSTRACT

BACKGROUND AND PURPOSE: Glial fibrillary acidic protein (GFAP) is an intracellular protein of the astrocytic cytoskeleton. Recently, autoantibodies to GFAP detected by cell-based assay in cerebrospinal fluid (CSF) or serum have been implicated in cerebral astrocytopathy, presenting predominantly with autoimmune meningoencephalomyelitis. However, the phenotypic spectrum, prognosis and therapeutics of this new entity remain to be elucidated. METHODS: Herein, we report radiological, CSF and serological findings during disease exacerbation and remission, from a patient with autoimmune GFAP astrocytopathy, presenting as an immunotherapy responsive GFAP IgG-associated meningoencephalomyelitis. RESULTS: Brain and spine magnetic resonance imaging revealed meningeal enhancement, T2 hyperintensities, black holes, significant sulci widening and spinal atrophy. In addition, high levels of neurofilaments (NfL) and GFAP were also identified during disease exacerbation, consistent with the appearance of the black holes. CONCLUSIONS: To date, black holes and atrophy have never been reported before in autoimmune GFAP astrocytopathy. These findings, combined with the high levels of GFAP and NfL, suggest the existence of an underlying neurodegenerative mechanism in addition to the known inflammatory response. Further studies are needed to elucidate the pathomechanism of GFAP-astrocytopathies.


Subject(s)
Intermediate Filaments , Astrocytes , Autoantibodies , Autoimmune Diseases of the Nervous System , Glial Fibrillary Acidic Protein , Humans
2.
Biomed Mass Spectrom ; 6(11): 491-8, 1979 Nov.
Article in English | MEDLINE | ID: mdl-394768

ABSTRACT

Differentiation of normal human cells has been accomplished by pyrolysis gas chromatography mass spectrometry. Normal cells from human kidney, spleen, liver and brain tissues have been pyrolyzed and the products chromatographically separated and characterized by mass spectrometry. Molecular pyrolysis products giving rise to the characteristic pyro-mass chromatograms include, but are not limited to, alkenes, alkanes, nitriles and various ring compounds. Single ion mass chromatograms as well as multiple ion mass chromatograms have been used to explore the characteristic differences between various tissue materials. A dynamic computer methodology for comparing pyro-mass chromatograms has been developed for use in automatic identification and classification of the human cellular material.


Subject(s)
Cytological Techniques , Gas Chromatography-Mass Spectrometry/methods , Brain/cytology , Brain Chemistry , Computers , Humans , Kidney/analysis , Kidney/cytology , Liver/analysis , Liver/cytology , Spleen/analysis , Spleen/cytology
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