Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppres
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CASE REPORT
JOURNAL OF NEUROINFLAMMATION
Open Access
Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment Raoul P Kloppenborg1, Edo Richard1, Marieke ES Sprengers2, Dirk Troost3, Piet Eikelenboom1, Paul J Nederkoorn1*
Abstract Cerebral amyloid angiopathy (CAA) is a common but often asymptomatic disease, characterized by deposition of amyloid in cerebral blood vessels. We describe the successful treatment of CAA encephalopathy with dexamethasone in a patient with CAA-related inflammation causing subacute progressive encephalopathy and seizures, which is an increasingly recognized subtype of CAA. The two pathological subtypes of CAA-related inflammation are described and a review of the literature is performed concerning immunosuppressive treatment of CAA-related inflammation with special attention to its pathological subtypes. Immunosuppressive therapy appears to be an appropriate treatment for CAA encephalopathy. Background Sporadic cerebral amyloid angiopathy (CAA) is a common but often asymptomatic neuropathological finding, characterized by the deposition of amyloid-b (Ab) in small and medium-sized cerebral arteries, arterioles and sometimes capillaries of the meninges and brain parenchyma. Its prevalence is strongly associated with increasing age and has been reported to be as high as 57% percent in case series of asymptomatic patients over 60 years of age [1]. CAA is a common finding in patients with Alzheimer’s disease (AD); but many patients with CAA do not develop AD. CAA can lead to lobar haemorrhage in non-hypertensive patients [2]. Other, less often reported clinical manifestations are seizures, transient neurological deficits and dementia other than AD [3]. In addition, more rare presentations have been reported, including space occupying lesions and leukoencephalopathy on magnetic resonance imaging (MRI) [4-6]. The latter is an increasingly recognized syndrome encompassing subacute encephalopathy, headache, seizures or focal neurological symptoms. Upon brain biopsy, an inflammatory process is found in relation to the vascular deposits of Ab. In contrast to other Ab-depositing disorders such as AD, immunosuppressive treatment has been reported to * Correspondence: [email protected] 1 Department of Neurology, Academic Medical Center, Amsterdam, The Netherlands
ameliorate both clinical and radiological symptoms of CAA encephalopathy, although with variable success [7]. This variability could be explained by the existence of different underlying pathological subtypes. We describe a patient with CAA-leukoencephalopathy, who was treated successfully with dexamethasone. We also performed a literature review concerning the use of immunosuppressive treatment for CAA-related inflammation with special attention to its pathological subtypes.
Case presentation A 74-year-old man with an unremarkable medical history noted a progressive gait disorder in the months prior to admission. His wife recalled increased sleepiness a
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