Cerebral amyloid angiopathy associated with giant cell arteritis: a case report.

Murphy, Maurice; Sima, A. A. F.

doi:10.1161/01.str.16.3.514

Cited by 40 publications

(19 citation statements)

References 24 publications

(12 reference statements)

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“…Nineteen patients were men, 19 women. Of 45 reported cases of amyloid-β related angiitis (ABRA), 13 (29%) presented with ML;6 7 13 14 20 – 23 28 30 of these eight were men and five were women. The patients with ABRA were older with a median age of 66 years (range 49–74), as compared with a median age of 38 years (range 6–69) among the non-amyloid group.…”

Section: Resultsmentioning

confidence: 99%

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Molloy

Singhal

Calabrese

2008

Annals of the Rheumatic Diseases

108

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Although rare, PACNS should be considered in the differential diagnosis of ML; greater awareness of this manifestation may facilitate more prompt diagnosis and treatment. Biopsy evidence of angiitis is required for diagnosis; specimens should routinely be stained for amyloid. While excision of the lesion may be curative, aggressive immunosuppressive therapy is associated with favourable outcomes and may obviate the need for surgery.

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Section: Resultsmentioning

confidence: 99%

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Molloy

Singhal

Calabrese

2008

Annals of the Rheumatic Diseases

108

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“…Among 36 prior patient reports of ABRA [1,4–24], only three describe vasculitic changes within white matter, subcortical structures or the cerebellum. Probst and Ulrich [18] describe vascular changes in deep white matter veins of a 65‐year‐old man with leg pain and confusion.…”

Section: Cerebral Amyloid Angiitis Progressing To Generalized Angiitimentioning

confidence: 99%

Scientific correspondence

Wk³

et al. 2007

Neuropathology Appl Neurobio

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“…Oral prednisolone with or without initial methylprednisolone or cyclophosphamide is the most commonly used immunosuppressive therapy in individual series, and recovery from neurologic deficits has been reported for up to 9 months. 2,3 In contrast, 1 patient with granulomatous angiitis and a CAA-related mass lesion improved and remained stable for 13 years after surgical excision of the lesion. 4 After we introduced cyclophosphamide, our patient remained stable for 15 months.…”

mentioning

confidence: 96%

Good Outcome in a Patient Treated for Cerebral Amyloid Angiopathy Presenting as an Expansive Process with Inflammation and Contrast Enhancement: Fig 1.

Bekkelund¹,

Midtbu²,

Arvola³

et al. 2011

AJNR Am J Neuroradiol

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Cerebral amyloid angiopathy (CAA) is an important cause of cerebrovascular diseases because it can be treated. CAA may present as a cerebral tumor, but knowledge about the pathophysiology and clinical outcome in such cases is limited. A patient who developed rapid progressive gait disturbances due to CAA with giant cell vasculitis presented with a contrast-enhancing mass lesion on MR imaging. The gait difficulties and the mass lesion normalized after immunosuppressive treatment, while the contrast enhancement persisted.We illustrate this case of a 57-year-old man who gradually developed weakness in the left leg after a fall on an icy road. After a few weeks, he could not walk without assistance and needed hospitalization at a nursing home. On hospitalization, T1-weighted MR imaging showed an expansive process in the right hemisphere with meningeal contrast enhancement and an old infarction in the left occipital area (Fig 1A). A T2-weighted gradient-echo series revealed numerous small punctuate lesions representing microbleeding, indicating amyloid angiopathy, but this was first acknowledged after the diagnosis was proved histologically (Fig 1B). Suspecting a malignant cerebral tumor (glioma) with secondary edema, we treated him with intravenous methylprednisolone with temporary improvement of the weakness in the left leg. Cognitive functions and findings of blood samples and CSF analysis were normal.An open brain biopsy from the right frontal cortex revealed CAA with giant cell activation around the amyloid deposition ( Fig 1C). The patient was treated with intravenous methylprednisolone, 1 g daily for 3 days, followed by corticosteroid treatment with a maximum dose of 60 mg, tapering off within 3 months. During treatment, he improved, and at the 1-month follow-up consultation, he walked without support and was able to care for himself at home. On examination, he rose from the chair and walked across the room without difficulty. T1-weighted MR imaging showed regression of the mass lesion and almost normalization of the ventricle asymmetry, but meningeal contrast enhancement persisted ( Fig 1D). Four months later, he relapsed with recurrent weakness of the left leg and unsteady gait. Consequently, we administered intravenous cyclophosphamide, 1200 mg at 3-week intervals for 1 year, which resolved the neurologic deficits. During the treatment period and at 15-month follow-up, he remained stable.Outcome in patients with cerebral mass effect due to CAA-related inflammation is infrequently reported in the literature.1 The dramatic regression of the mass lesion demonstrated on MR imaging corresponded well with clinical improvement. Oral prednisolone with or without initial methylprednisolone or cyclophosphamide is the most commonly used immunosuppressive therapy in individual series, and recovery from neurologic deficits has been reported for up to 9 months. 2,3 In contrast, 1 patient with granulomatous angiitis and a CAA-related mass lesion improved and remained stable for 13 years after surgical excision of the les...

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Cerebral amyloid angiopathy associated with giant cell arteritis: a case report.

Cited by 40 publications

References 24 publications

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Scientific correspondence

Good Outcome in a Patient Treated for Cerebral Amyloid Angiopathy Presenting as an Expansive Process with Inflammation and Contrast Enhancement: Fig 1.

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