Steroid-Responsive Recurrent Encephalopathy in a Patient with Cerebral Amyloid Angiopathy

McHugh, John; Ryan, Aisling; Lynch, Timothy; Dempsey, Elizabeth; Stack, John; Farrell, Michael; Kelly, Peter

doi:10.1159/000097030

Cited by 20 publications

(12 citation statements)

References 3 publications

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“…There have been 2 subtypes of CAA-ri described, including a non-vasculitic form (perivascular infiltration) and a vasculitic form (transmural granulomatous angiitis) [4]. These pathological forms can occur together in the same patient [12]. Neuropathological findings of the present study suggested that our patient had concurrent perivascular infiltration and transmural granulomatous angiitis.…”

Section: Discussionmentioning

confidence: 54%

Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammation

Kimura

Sakurai

Yagi

et al. 2013

J Neuroinflammation

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We studied longitudinal changes of the levels of anti-amyloid β (anti-Aβ) antibody, amyloid β (Aβ) protein, and interleukin 8 (IL-8) in cerebrospinal fluid (CSF) of a patient with cerebral amyloid angiopathy-related inflammation (CAA-ri) in whom steroid treatment resulted in clinical improvement. The diagnosis of CAA-ri was established with brain biopsy. Levels of anti-Aβ 42 antibody, Aβ 40, Aβ 42 and IL-8 in CSF were measured in the CAA-ri patient at 23 time points in the 8-month clinical course. These CSF samples were divided into 2 groups: those obtained before (n = 12) and those after (n = 11) oral corticosteroid therapy was started. We compared these levels between CSF samples obtained before and after therapy. The mean levels of anti-Aβ 42 antibody and IL-8 were significantly higher in CSF samples of the CAA-ri patient before oral corticosteroid therapy than those after therapy. A positive correlation was noted between levels of anti-Aβ 42 antibodies and IL-8 in CSF of this patient. There were no significant differences of mean levels of Aβ 40 and Aβ 42 between CSF samples obtained before and after oral corticosteroid therapy. It was possible that the autoinflammatory process with anti-Aβ 42 antibodies and IL-8 may have been involved in the pathogenesis of CAA-ri, and that corticosteroid therapy directly affected levels of anti-Aβ 42 antibody and IL-8. In summary, CAA-ri encephalopathy is a relapsing or progressive disorder and may be treatable by adequate immunosuppressive therapy. The anti-Aβ 42 antibody in CSF is a useful biological marker for therapeutic monitoring of CAA-ri.

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Section: Discussionmentioning

confidence: 54%

Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammation

Kimura

Sakurai

Yagi

et al. 2013

J Neuroinflammation

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“…Both pathologic forms can co-occur, suggesting at least a partial overlap [8]. The clinical and radiological findings of both variants are remarkably similar.…”

Section: Discussionmentioning

confidence: 99%

“…This is consistent with reactive edema in encephalopathy and suggests PVI. Although often called CAA-angiitis, the terms CAA-vasculopathy or CAA-encephalopathy are preferred, since these terms do not exclude the considerable numbers of cases with only perivascular inflammation [8]. …”

Section: Discussionmentioning

confidence: 99%

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

Kloppenborg

Richard

Sprengers

et al. 2010

J Neuroinflammation

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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.

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“…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].…”

Section: Discussionmentioning

confidence: 99%

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

Cano

Martínez-Yélamos

Majós

et al. 2010

Journal of the Neurological Sciences

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Steroid-Responsive Recurrent Encephalopathy in a Patient with Cerebral Amyloid Angiopathy

Cited by 20 publications

References 3 publications

Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammation

Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammation

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

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

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