Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies?

Abstract

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. Case Reports: Methods and ResultsBoth patients presented around their seventh decade with abrupt onset and rapidly progressive neurocognitive deficits over 4-6 months accompanied by autonomic and psychiatric abnormalities. Focal neurologic signs (especially visual, motor, or sensory disturbances, movement 410