Central nervous system involvement in amyloid A type amyloidosis

We report a 38-year-old male with recurrent cerebral infarction, who had a history of juvenile rheumatoid arthritis and suffered from ischemic necrosis of the jejunum at the age of 34 years. When the patient showed gastrointestinal symptoms, amyloid deposits were found with colon biopsy. The patient experienced recurrent episodes of headache and left homonymous lower quadrantanopsia at the age of 38 years. CT and MRI of the head revealed cerebral infarction in the right parieto-occipital region. At this time, amyloid deposits were detected in the mucosa of the stomach, duodenum, and rectum. Immunostaining revealed that amyloid fibrils were composed of amyloid A (AA) protein. It is possible that AA type amyloidosis may be of importance in the etiology of cerebral infarction in the young adult.