Open AccessRapidly progressive dementia: Extending the spectrum of GFAP ‐astrocytopathies?
Author(s) -
Friedrich Maximilian,
Hartig Johannes,
Prüss Harald,
Ip Chi Wang,
Volkmann Jens
Publication year - 2022
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.51513
Subject(s) - medicine , glial fibrillary acidic protein , context (archaeology) , dementia , serology , pathology , immunotherapy , immunohistochemistry , neurocognitive , antibody , immunology , psychiatry , immune system , disease , cognition , paleontology , biology
Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A) is a steroid‐responsive meningoencephalomyelitis, sometimes presenting with atypical clinical signs such as movement disorders or psychiatric and autonomic features. Beyond clinical presentation and imaging, diagnosis relies on detection of GFAP‐antibodies (AB) in CSF. Using quantitative behavioral, serologic, and immunohistochemical analyses, we characterize two patients longitudinally over 18–24 months who presented with rapidly progressive neurocognitive deterioration in the context of GFAP‐AB in CSF and unremarkable cranial MRI studies. Intensified immunotherapy was associated with clinical stabilization. The value of GFAP‐AB screening in selected cases of rapidly progressive dementias is discussed.