Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment

Kloppenborg, Raoul P.; Richard, Edo; Sprengers, Marieke E.S.; Troost, Dirk; Eikelenboom, Piet; Nederkoorn, Paul J.

doi:10.1186/1742-2094-7-18

Cited by 56 publications

(32 citation statements)

References 19 publications

(14 reference statements)

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“…This inflammation appears to represent an autoimmune response to vascular β-amyloid deposits, and steroid therapy has a potential to relieve the CAA-related symptoms [2,10]. In this case, the inflammatory cells detected in the bronchial artery were not clearly determined as having a direct causal relationship with the vascular collapse; however, it may be said that the inflammation cells took part in the vascular collapse, at least in part.…”

Section: Discussionmentioning

confidence: 80%

Heart Failure Complicated by Alveolar Hemorrhage due to Vascular Collapse and Amyloid Deposits in Wild-Type Transthyretin Amyloidosis

Kido

Takahashi²,

Fukuma³

et al. 2016

Cardiology

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The main clinical manifestations of wild-type transthyretin (TTR)-related amyloidosis are progressive heart failure and neuropathy. There have been some reports on cerebral hemorrhage due to cerebral amyloid angiopathy in patients with TTR-related amyloidosis, but little is known about the vascular involvement in other organs. A 77-year-old woman experienced heart failure and was admitted for deteriorating heart failure status. Echocardiography showed diffuse hypokinesis of the left ventricle with biventricular wall thickness. On the 12th hospital day, the blood oxygen saturation level suddenly dropped and, despite oxygen supplementation and intensive care, the patient died. An autopsy revealed systemic deposition of amyloids which were immunolabeled by an anti-TTR antibody. Furthermore, gene-sequencing analysis showed no evidence of TTR gene mutations. The patient was diagnosed postmortem with wild-type TTR-related amyloidosis. Pathological findings revealed alveolar hemorrhage of the lung. Massive amyloid deposits were present in the vessels, and collapsed internal elastic laminae with lymphocyte infiltration were observed at the site of amyloid deposits in the bronchial artery, suggesting that deposits with inflammation might cause the collapse of the bronchial artery and lead to hemorrhage. In amyloidosis patients who suffer heart failure, there is the potential for vascular collapse caused by the accumulation of amyloid deposits with inflammation.

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Section: Discussionmentioning

confidence: 80%

Heart Failure Complicated by Alveolar Hemorrhage due to Vascular Collapse and Amyloid Deposits in Wild-Type Transthyretin Amyloidosis

Kido

Takahashi²,

Fukuma³

et al. 2016

Cardiology

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“…The presentation of CAA-related inflammation (also called amyloid β-related angiitis) includes rapid cognitive decline, seizures, headache, and diffuse white matter changes on MRI [122]. Recognition of CAA-related inflammation is important because it is potentially reversible with immunotherapy [123].…”

Section: Cerebral Amyloid Angiopathymentioning

confidence: 99%

Treatment of Vascular Cognitive Impairment

Ritter

Pillai

2015

Curr Treat Options Neurol

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Cerebrovascular disease (CVD) is an important cause of cognitive dysfunction and dementia. The term vascular cognitive impairment (VCI) is used to describe the entire spectrum of cognitive dysfunction-ranging from mild impairment to dementia-attributable to all forms of cerebrovascular disease. Accurate assessment and management of vascular risk factors are a top priority in the treatment of VCI, particularly early in the disease when prevention strategies may prove to be more effective. There are limited treatment options to improve cognition and function in VCI. Several acetylcholinesterase inhibitors and the NMDA receptor antagonist memantine have been studied in large, well-designed trials. These agents are safe and provide modest cognitive benefits in vascular dementia (VaD) but have demonstrated inconsistent efficacy on functional measures. Other therapies, such as aspirin, calcium channel blockers, and vitamin supplementation, have less evidence to support their use in improving cognition in VCI. Although primary prevention trials suggest that treatment of hypertension, adherence to a Mediterranean diet, physical activity, and smoking cessation may reduce the risk of cognitive decline, there is limited evidence regarding these interventions in helping improve cognition in VCI. The pathophysiology and treatment of cerebral autosomal dominant arteriopathy with subcortical infarcts (CADASIL), cerebral amyloid angiopathy (CAA), and subcortical white matter disease (SWMD) deserves special consideration.

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“…First line therapy is usually parenteral corticosteroids, and the response to corticosteroids is generally good [128,129,131,132]. In some cases, additional immunosuppressive agents, primarily cyclophosphamide, have been used [128].…”

Section: Cerebral Amyloid Angiopathy-related Inflammationmentioning

confidence: 99%

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

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Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment

Cited by 56 publications

References 19 publications

Heart Failure Complicated by Alveolar Hemorrhage due to Vascular Collapse and Amyloid Deposits in Wild-Type Transthyretin Amyloidosis

Heart Failure Complicated by Alveolar Hemorrhage due to Vascular Collapse and Amyloid Deposits in Wild-Type Transthyretin Amyloidosis

Treatment of Vascular Cognitive Impairment

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

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