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

Abstract

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...

Cerebral amyloid angiopathy associated with inflammation: A systematic review of clinical and imaging features and outcome

“…The search (Figure 2) identified 186 abstracts, and a further 44 from reference lists, giving a total of 230. After inclusion and exclusion criteria were applied, 104 publications were included which described 213 distinct pathologically proven cases of CAARI/ABRA, 4,7…”

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature

“…In a more recent review of CAA-RI, Danve et al [ 9 ] reported that up to 26% of CAA-RI cases had mass-like lesions that were usually asymmetrical and either non-enhancing or minimally enhancing. The most frequent brain malignancies suspected in cases of pseudotumoral presentation of CAA-RI were low grade gliomas [ 12 - 20 ], lymphomas [ 21 , 22 ], multifocal glioma [ 23 , 24 ], oligodendroglioma [ 25 ], gliomatosis cerebrii [ 26 ] and metastases [ 27 ]. The pseudotumoral form of CAA-RI can be a challenging diagnosis, as its clinical and radiological presentation is not specific [ 10 ].…”

“…7,9 Many patients remain symptom-free after several years of follow up 10,13,14,25 but relapses can occur in 26% of cases, 9 generally after reduction or cessation of immunosuppression 20,27,39 therefore, clinical surveillance is necessary, and cyclophosphamide or azathioprine can be added in relapsing disease. 20,39 In the present pseudotumoral CAA-RI series, 27/42 patients received steroids as therapy, and in 15 of these, steroids were the only pharmacological treatment; 7/27 were also treated with cyclophosphamide or methotrexate as adjuvant therapy. While a review of the literature shows that only approximately 5% of cases of CAA-RI were surgically treated, 8 in this series of pseudotumoral form cases of CAA-RI 26% (11/42) of patients underwent surgical resection of the lesion due to suspicion of underlying malignancy.…”

“…Treatment of CAA-RI with steroids is usually effective, and clinical and radiological remission can be achieved in the first 3 weeks in approximately 70% of cases [ 7 , 9 ]. Many patients remain symptom-free after several years of follow up [ 10 , 13 , 14 , 25 ] but relapses can occur in 26% of cases [ 9 ], generally after reduction or cessation of immunosuppression [ 20 , 27 , 39 ] therefore, clinical surveillance is necessary, and cyclophosphamide or azathioprine can be added in relapsing disease [ 20 , 39 ]. In the present pseudotumoral CAA-RI series, 27/42 patients received steroids as therapy, and in 15 of these, steroids were the only pharmacological treatment; 7/27 were also treated with cyclophosphamide or methotrexate as adjuvant therapy.…”