Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.
Shiori Yamakawa
Department of Neurology, Kumamoto University, Kumamoto, Japan

Main Body of the Abstract
Background:
ATTRv amyloidosis Val30Met have a low incidence of central nervous system complications. However, prognosis of these patients has improved with liver transplantation (LT) and long-lived patients often suffer from central nervous system complications.
Objective:
To analyze clinical and pathological findings of an autopsied case of ATTRv amyloidosis Val30Met after LT who developed cardiogenic cerebral embolism(CCE) and died from subarachnoid hemorrhage(SAH).
Method:
We investigated course of the patient from onset to death by referring to medical records. We evaluated histopathological changes by Congo red and immunohistochemical staining, mass spectrometry.
Results:
She developed lower extremity peripheral neuropathy at 36 years old and underwent LT at 40 years old. Tafamidis was started at 19 years after LT due to progressive cardiac amyloidosis. At 20 years after LT, she developed paroxysmal atrial fibrillation and CCE and started on warfarin. Her head MRA showed no cerebral aneurysm. She died from SAH at 21 years after LT.
Congo red and immunochemical staining of tissues revealed TTR-positive amyloid deposition in the brain, heart, and peripheral nerves. Mass spectrometry analysis of amyloid deposition showed that mutant TTR was predominant in leptomeningeal vessels, whereas wild-type TTR was predominant in the heart.
Summary & Discussion:
It is possible that progression of cardiac amyloidosis by wild-type TTR amyloid have caused paroxysmal atrial fibrillation and CCE.
The cause of subarachnoid hemorrhage is unknown. However, warfarin, which was administered to prevent recurrence of CCE, may have increased the risk of bleeding.

**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Shiori Yamakawa
Department of Neurology, Kumamoto University, Kumamoto, Japan

**Main Body of the Abstract**
**Background:**
ATTRv amyloidosis Val30Met have a low incidence of central nervous system complications. However, prognosis of these patients has improved with liver transplantation (LT) and long-lived patients often suffer from central nervous system complications.
**Objective:**
To analyze clinical and pathological findings of an autopsied case of ATTRv amyloidosis Val30Met after LT who developed cardiogenic cerebral embolism(CCE) and died from subarachnoid hemorrhage(SAH).
**Method:**
We investigated course of the patient from onset to death by referring to medical records. We evaluated histopathological changes by Congo red and immunohistochemical staining, mass spectrometry.
**Results:**
She developed lower extremity peripheral neuropathy at 36 years old and underwent LT at 40 years old. Tafamidis was started at 19 years after LT due to progressive cardiac amyloidosis. At 20 years after LT, she developed paroxysmal atrial fibrillation and CCE and started on warfarin. Her head MRA showed no cerebral aneurysm. She died from SAH at 21 years after LT.
Congo red and immunochemical staining of tissues revealed TTR-positive amyloid deposition in the brain, heart, and peripheral nerves. Mass spectrometry analysis of amyloid deposition showed that mutant TTR was predominant in leptomeningeal vessels, whereas wild-type TTR was predominant in the heart.
**Summary & Discussion:**
It is possible that progression of cardiac amyloidosis by wild-type TTR amyloid have caused paroxysmal atrial fibrillation and CCE.
The cause of subarachnoid hemorrhage is unknown. However, warfarin, which was administered to prevent recurrence of CCE, may have increased the risk of bleeding.

Clinicopathological analyses of ATTRv amyloidosis after liver transplantation with cardiogenic cerebral embolism and subarachnoid hemorrhage PC34 (#89)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.