Normal pressure hydrocephalus manifesting as transient prosopagnosia, topographical disorientation, and visual objective agnosia

Abstract Usually, dementia, gait disturbance and urinary incontinence are an integral part of the clinical presentation of normal pressure hydrocephalus (NPH). However, NPH with transient visual cognitive disorders has not been reported previously. We herein describe an extremely rare case of NPH that presented with transient visual cognitive disorders and long lasting visual memory disturbances that subsided after CSF shunting. A 38-years-old man developed transient prosopagnosia, topographical disorientation, color vision disturbance, and visual objective agnosia that progressed over 5 years. Magnetic resonance images showed ventriculomegaly with ischemic lesions in the paraventricular deep white matter. ECD-SPECT showed a reduction of the cerebral blood flow (CBF) in the periventricular deep white matter. Three months after CSF shunting, the radiological findings normalized with dramatic improvement of the extremely rare symptoms.