Cortical petechial hemorrhage, subarachnoid hemorrhage and corticosteroid-responsive leukoencephalopathy in a patient with cerebral amyloid angiopathy

Abstract

We describe a 69-year-old woman who developed subacute onset cognitive decline after hitting the left side of her head. Cerebral spinal fluid showed yellowish discoloration with highly elevated protein content. FLAIR MRI revealed diffuse high signal intensity in all cortical sulci, and leptomeningeal enhancement in the left cerebral hemisphere was seen in the T1 image after contrast administration. She was treated with a corticosteroid. Consciousness disturbance was temporarily relieved but again worsened, resulting in an apathetic state due to communicating hydrocephalus. A shunt tube was placed in her right lateral ventricle. A brain biopsy disclosed multiple cortical microbleeds and heavy deposition of Abeta-immuoreactive amyloid on vascular walls. Inflammatory mononuclear cells surrounded a few leptomeningeal vessels. After the operation her condition further deteriorated and she fell into a coma. MRI showed diffuse swelling of the right cerebral white matter. She again received high-dose corticosteroid and gradually recovered during the following 2 months. On MRI the vast majority of abnormal signals in the right cerebral white matter disappeared. An initial manifestation of this patient was possibly caused by multiple microhemorrhages from fragile cortical and subarachnoid vessels with Abeta-amyloid deposition, which was triggered by head trauma. CAA-related inflammation possibly worsened this condition. Additionally, surgical intervention for communicating hydrocephalus might have induced cerebral amyloid angiopathy (CAA)-related leukoencephalopathy in her right cerebral hemisphere. These CAA-derived manifestations are unusual and high-dose corticosteroids seems to be useful for vascular events in CAA patients.

Cerebral Microbleeds: Detection, Mechanisms and Clinical Challenges

“…In this study, the majority of CMBs were found in cortical-subcortical locations, and the adjacent arterioles were affected by advanced CAA (vessel wall thickening with amyloid-b (Ab) deposition and lacking a muscularis layer) [42]. Taken together, the findings of neuroimaging-pathological correlation studies, as well as other histopathological analyses of CMBs [38,[44][45][46][47], show that CMBs are commonly associated with two different small-vessel pathologies: hypertensive vasculopathy and CAA. Moreover, these two microangiopathic disorders seem to influence CMB topography.…”

Hypoperfusion and Ischemia in Cerebral Amyloid Angiopathy Documented by 99mTc-ECD Brain Perfusion SPECT

“…Inflammation of the vascular wall, angitis, and hypoperfusion producing high signal intensity on T2-weighted images of white matter could also be possible causes of the signal intensity abnormality (18,20). In some subsets of patients with CAA, a specific form of perivascular inflammation known as the immune response to Ab deposition in the vascular wall has been reported.…”

“…The presence of microaneurysm can induce massive intraparenchymal hemorrhages in patients with CAA, and the presence of luminal stenosis can reflect hypoxic-ischemic changes. Although the most common presentation of CAA is spontaneous cerebral hemorrhage, a few reports of cases of nonhemorrhagic leukoencephalopathy as a result of hypoxic-ischemic changes in vessel stenosis have been published (6,(16)(17)(18)(19)(20).…”

Reversible acute leukoencephalopathy as a form of presentation in cerebral amyloid angiopathy

“…The majority of these lesions are also associated with inflammation and respond, to a greater or lesser extent, to anti-inflammatory treatment [6]. This response is also present in inflammation-related periventricular lesions [7,8].…”