Acute progression of cerebral amyloid angiopathy-related inflammation diagnosed by biopsy in an elderly patient: A case report
- Department of Comprehensive Strokology, Fujita Health University School of Medicine, Toyoake,
- Department of Neurosurgery, Nishichita General Hospital, Tokai,
- Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake,
- Department of Pathology, Nishichita General Hospital, Tokai, Japan.
Ichiro Nakahara, Department of Comprehensive Strokology, Fujita Health University School of Medicine, Toyoake, Japan.
DOI:10.25259/SNI_195_2022Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Kiyonori Kuwahara1,2, Shigeta Moriya2, Ichiro Nakahara1, Tadashi Kumai3, Shingo Maeda3, Yuya Nishiyama3, Midoriko Watanabe4, Yoshikazu Mizoguchi4, Yuichi Hirose3. Acute progression of cerebral amyloid angiopathy-related inflammation diagnosed by biopsy in an elderly patient: A case report. 23-Jun-2022;13:268
How to cite this URL: Kiyonori Kuwahara1,2, Shigeta Moriya2, Ichiro Nakahara1, Tadashi Kumai3, Shingo Maeda3, Yuya Nishiyama3, Midoriko Watanabe4, Yoshikazu Mizoguchi4, Yuichi Hirose3. Acute progression of cerebral amyloid angiopathy-related inflammation diagnosed by biopsy in an elderly patient: A case report. 23-Jun-2022;13:268. Available from: https://surgicalneurologyint.com/surgicalint-articles/11675/
Background: Cerebral amyloid angiopathy-related inflammation (CAA-I) presents with slowly progressive nonspecific neurological symptoms, such as headache, cognitive function disorder, and seizures. Pathologically, the deposition of amyloid-β proteins at the cortical vascular wall is a characteristic and definitive finding. Differential diagnoses include infectious encephalitis, neurosarcoidosis, primary central nervous system lymphoma, and glioma. Here, we report a case of CAA-I showing acute progression, suggesting a glioma without enhancement, in which a radiological diagnosis was difficult using standard magnetic resonance imaging.
Case Description: An 80-year-old woman was admitted due to transient abnormal behavior. Her initial imaging findings were similar to those of a glioma. She presented with rapid progression of the left hemiplegia and disturbance of consciousness for 6 days after admission and underwent emergent biopsy with a targeted small craniotomy under general anesthesia despite her old age. Intraoperative macroscopic findings followed by a pathological study revealed CAA-I as the definitive diagnosis. Steroid pulse therapy with methylprednisolone followed by oral prednisolone markedly improved both the clinical symptoms and imaging findings.
Conclusion: Differential diagnosis between CAA-I and nonenhancing gliomas may be difficult using standard imaging studies in cases presenting with acute progression. A pathological diagnosis under minimally invasive small craniotomy may be an option, even for elderly patients.
Keywords: Biopsy, Cerebral amyloid angiopathy-related inflammation, Glioma
Cerebral amyloid angiopathy (CAA) is pathologically characterized by the deposition of amyloid-β proteins in the walls of cortical arteries in elderly patients. Some studies have reported that patients with CAA and inflammation based on vasculitis or perivasculitis present with symptoms such as headache, dementia, and seizure and are recognized as having cerebral amyloid angiopathy-related inflammation (CAA-I).[
An 80-year-old woman presented with transient abnormal behavior, initially suggesting mild senile dementia. Computed tomography (CT) revealed a large low-density area in the right temporal, parietal, and occipital lobes [
Initial images of CT and MRI. (a) CT showing a low-density area in the right temporal lobe with a midline shift. (b) Fluid-attenuated inversion recovery image of MRI showing a high-intensity area (HIA) in the cortex of the right temporal lobe and occipital lobe combined with an HIA in the white matter indicating vasogenic edema. Periventricular HIAs were also shown in both the anterior and occipital horn of bilateral lateral ventricles. (c) T2 star-weighted MR image showing several low-intensity spots at the margin of the HIA in the right occipital lobe.
Intraoperative macroscopic view and pathological examination of biopsy specimen (a) Intraoperative macroscopic view of the occipital lobe showing swelling of the cortex. (b) A histopathological examination (Hematoxylin and eosin staining, ×40) showing the thickened cortical artery wall. (c) An immunohistochemical stain of the same section (Congo red staining, ×40) showing the deposition of amyloid-β proteins at the arterial wall. (d) Another immunohistochemical stain (Direct fast scarlet staining, ×40) also showing the deposition of amyloid-β proteins.
Steroid pulse therapy was started on the day after the surgery with intravenous methylprednisolone 1000 mg/day for 3 days, followed by oral prednisolone 60 mg/day through a nasal feeding tube. Her symptoms gradually improved a few days after the initiation of steroid therapy, and she recovered to an almost arousable state with the left hemiparesis as a manual muscle test (MMT) 3/5 on the 10th day. Meanwhile, a permanent pathological diagnosis with specific staining revealed leukoencephalopathy in the white matter without any neoplastic changes. In addition, Congo red staining [
The dose of oral prednisolone was decreased by 10 mg every week for 5 weeks from the 11th day and stopped on the 53rd day after the initiation of steroid therapy. The HIA on FLAIR decreased gradually in the following 2 months [
Follow-up images of the fluid-attenuated inversion recovery image of MRI. (a) Image on the 22nd day after admission. The high-intensity area (HIA) in the right temporal and occipital lobes was improved compared with the initial image [Figure 1b]. (b) Image on the 55th day after admission. The HIA in the right temporal and occipital lobes almost disappeared.
CAA-I is a rare disease that is pathologically characterized by the deposition of amyloid-β proteins in the cortical arteries, causing vasculitis or perivasculitis with widespread vasogenic edema. MRI is characterized by a wide HIA on FLAIR concomitant with MBs on T2*WI in the same lesion without enhancement by gadolinium.[
CAA-I is known to be relieved by steroid hormones and immunosuppressive agents, such as methotrexate and cyclophosphamide,[
It might be a choice to start medical therapy by assuming CAA-I without a pathological diagnosis, if we recognized it with emphasizing the findings of MBs on T2*WI. However, the differential diagnosis of CAA-I from a glioma is not always easy in cases, where the MBs are not limited to the predominant lesion, as in our case. MBs are not rare in elderly patients, even without CAA-I. The risk of a large dose of steroids and immunosuppressive agents due to incorrect diagnosis should not be ignored in elderly patients. Therefore, we deemed it appropriate that pathological confirmation of the diagnosis be undertaken by targeting biopsy with minimally invasive small craniotomy, as wide craniotomy may cause infection due to postoperative steroids and immunosuppressive drugs, particularly in the elderly. Endoscopic or stereotactic surgery, which may be a minimally invasive surgery, is considered an inappropriate option to avoid intraoperative hemorrhage, because vascular specimens should be obtained for the pathological diagnosis of CAA-I to observe findings of vasculitis or perivasculitis.
We report a case of CAA-I, in which an initial diagnosis was difficult between CAA-I and a nonenhancing glioma. A favorable result was obtained with steroid therapy following a pathological diagnosis by biopsy under minimally invasive small craniotomy. This approach may be the choice even for CAA-I in elderly patients, in whom differential diagnosis is difficult using standard imaging studies.
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The authors certify that they have obtained all appropriate patient consent.
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Conflicts of interest
There are no conflicts of interest.
The authors would like to thank Editage (https://www.editage.jp ) for the English language review.
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