Iatrogenic cerebral amyloid angiopathy 30 years after childhood cadaveric dura mater transplantation: a case report.

BACKGROUND

Cerebral amyloid angiopathy (CAA) is rarely observed in young individuals. We herein report a case involving a 39-year-old man who underwent cadaveric dura mater (LYODURA) transplantation in childhood and subsequently developed repeated cerebral hemorrhages and infarctions.

CASE PRESENTATION: At 10 months of age, he had received a cadaveric dura mater graft following head trauma. Thirty-two years later, he began experiencing seizures, intracranial hemorrhages, and cerebral infarctions over several years. Brain MRI revealed multiple lobar microbleeds, cortical superficial siderosis, and bilateral infarctions, with new lesions on follow-up scans. A brain tissue sample obtained during evacuation of a left temporal hematoma showed arteriolar amyloid deposits predominantly composed of amyloid beta (Aβ) 40. Amyloid positron emission tomography (PET) demonstrated widespread cortical amyloid deposition, not limited to the area near the childhood surgical site.

CONCLUSIONS

These findings suggest that abnormal Aβ may have gradually propagated over decades from the transplanted cadaveric dura mater, resulting in CAA. Including this case, thirteen LYODURA-associated cases have been reported, with disease onset occurring more than 30 years after head surgery and dural transplantation. Until 1997, a large volume of LYODURA was imported into Japan, raising the possibility of additional future cases of CAA. This case highlights the importance of considering iatrogenic CAA in younger patients and demonstrates the potential value of non-invasive detection through amyloid PET.