Cerebral Amyloid Angiopathy in Pregnancy: A Case Report

Abstract

INTRODUCTION: Cerebral Amyloid Angiopathy (CAA) is amyloid deposition within cerebral vessel walls which increases risk for spontaneous intracerebral hemorrhage (ICH) and occurs almost exclusively in patients over age 65. We report a young woman patient who presented for ICH with both imaging and tissue pathology consistent with CAA.CASE PRESENTATION: A 36-year-old G1P0 woman at 34 weeks gestation presented to the hospital after one day of severe headache. She denied recent trauma. Upon arrival the fetus was in distress prompting emergent delivered via cesarean section. The patient subsequently underwent CT head revealing an acute 6.3cm x 5.4cm x 3.1cm right parieto-occipital parenchymal hemorrhage and significant vasgoenic edema. Also noted, a concomitant subdural component to the hemorrhage, extending extra-axially up to 1 cm in diameter in the same right parieto-occipital region. There was resulting right-to-left midline shift, with the body of the right lateral ventricle nearly completely effaced. She was intubated due to developing encephalopathy and neurosurgery completed an urgent craniotomy. She initially improved and extubated three days later; however, four days after the procedure she developed recurrent right subdural hemorrhage with CT pertinent for further right-to-left midline shift with right lateral ventricular narrowing and new uncal deviation. She underwent middle meningeal artery embolization via interventional radiology to remove the source of the bleed. She recovered in the following days without complications and transferred out of the ICU. During the initial craniotomy, a tissue sample sent for pathology evaluation noted congophilic tissue consistent with CAA. DISCUSSION: While CAA can be presumed with CT or MRI imaging, it is optimally confirmed via tissue sample with Congo Red stain under polarized light microscopy. Epidemiologically, CAA strongly correlates with age thus rarely considered in women of childbearing age. There are few reports of CAA diagnosis in the pregnant population, likely due to misdiagnosis given other common pathologies such as gestational hypertension and preeclampsia which predispose patients to ICH. Additionally, invasive nature of confirming this pathology may play a role in misdiagnosis due to the risk of procedures in pregnancy. Mortality of CAA with ICH is 10-30% depending on location and size of the bleeding. Risk of recurrent bleeding is much higher than other causes of ICH. There is no currently agreed upon management of CAA. However, there is some evidence for the use of steroids and immunosuppressants to decrease CAA related vasogenic edema. It is also reasonable to suggest prevention of further hemorrhagic events with the avoidance of blood thinners.CONCLUSIONS: CAA should be considered in patients who present with ICH regardless of age or pregnancy. Confirmation with tissue biopsy is preferred for proper diagnosis and treatment.