TY  - JOUR
A1  - Friedrich, Maximilian
A1  - Hartig, Johannes
A1  - Prüss, Harald
A1  - Ip, Wang Chi
A1  - Volkmann, Jens
T1  - Rapidly progressive dementia: Extending the spectrum of GFAP-astrocytopathies?
T2  - Annals of Clinical and Translational Neurology
N2  - 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.
KW  - GFAP-astrocytopathies
KW  - dementia
Y1  - 2022
UR  - https://opus.bibliothek.uni-wuerzburg.de/frontdoor/index/index/docId/31295
UR  - https://nbn-resolving.org/urn:nbn:de:bvb:20-opus-312957
VL  - 9
IS  - 3
ER  -