PrP systemic amyloidosis

Symptoms

The initial presentation of PrP systemic amyloidosis is chronic diarrhea in the fourth decade of life, which may be misdiagnosed as Crohn's disease or irritable bowel syndrome. Sensory and/or autonomic neuropathy follows.

In one case, the disease caused optic nerve atrophy and visual disturbances in a Japanese woman.

Eventually, the disease will impact the central nervous symptom, leading to cognitive decline, seizures, and death.