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Amyloid-β-related angiitis: a rare cause of recurrent transient neurological symptoms

Nature Clinical Practice Neurology volume 4, pages 279–283 (2008)Cite this article

Abstract

Background A 79-year-old woman presented with transient right-sided weakness. She subsequently developed further transient neurological signs and symptoms across a range of vascular territories. Diagnostic workup revealed no clear etiology. The patient developed a sudden, fatal intracerebral hemorrhage, and the diagnosis was only revealed at autopsy.

Investigations Neurological examination, head CT, brain MRI, fluid-attenuated inversion recovery imaging, magnetic resonance angiography, lumbar puncture, CT angiography, electroencephalography, and brain autopsy.

Diagnosis Amyloid-β-related angiitis.

Management The patient expired before treatment could be initiated. Immunosuppressants are recommended for future cases.

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Figure 1: Images of the patient's brain demonstrating her clinical progression.
Figure 2: Postmortem brain autopsy revealed a diagnosis of amyloid-β-related angiitis.

References

  1. Aznar AO et al. (2007) Intravascular large B-cell lymphoma presenting with neurological syndromes: clinicopathologic study. Clin Neuropathol 26: 180–186

    Article  Google Scholar 

  2. U-King-Im JM et al. (2005) Current diagnostic approaches to subarachnoid haemorrhage. Eur Radiol 15: 1135–1147

    Article  Google Scholar 

  3. Alvarez-Sabín J et al. (1995) Delayed posttraumatic hemorrhage: “spät-apoplexie”. Stroke 26: 1531–1535

    Article  Google Scholar 

  4. Scolding NJ et al. (2005) Aβ-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 128: 500–515

    Article  Google Scholar 

  5. Gras P et al. (1993) Transient neurologic deficit preceding intracerebral hemorrhage: physiopathological hypotheses [French]. Rev Neurol (Paris) 149: 224–226

    CAS  Google Scholar 

  6. Fountain NB and Eberhard DA (1996) Primary angiitis of the central nervous system associated with cerebral amyloid angiopathy: report of two cases and review of the literature. Neurology 46: 190–197

    Article  CAS  Google Scholar 

  7. Kinnecom C et al. (2007) Course of cerebral amyloid angiopathy-related inflammation. Neurology 68: 1411–1416

    Article  CAS  Google Scholar 

  8. Eng JA et al. (2004) Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 55: 250–256

    Article  Google Scholar 

  9. Anders KH et al. (1997) Giant cell arteritis in association with cerebral amyloid angiopathy: immunohistochemical and molecular studies. Hum Pathol 28: 1237–1246

    Article  CAS  Google Scholar 

  10. Gray F et al. (1990) Cerebral amyloid angiopathy and granulomatous angiitis: immunohistochemical study using antibodies to the Alzheimer A4 peptide. Hum Pathol 21: 1290–1293

    Article  CAS  Google Scholar 

  11. Mandybur TI (1979) Cerebral amyloid angiopathy: possible relationship to rheumatoid vasculitis. Neurology 29: 1336–1340

    Article  CAS  Google Scholar 

  12. Mandybur TI and Balko G (1992) Cerebral amyloid angiopathy with granulomatous angiitis ameliorated by steroid-cytoxan treatment. Clin Neuropharmacol 15: 241–247

    Article  CAS  Google Scholar 

  13. Tamargo RJ et al. (2003) Clinicopathological review: primary angiitis of the central nervous system in association with cerebral amyloid angiopathy. Neurosurgery 53: 136–143

    Article  Google Scholar 

  14. Schwab P et al. (2003) Cerebral amyloid angiopathy associated with primary angiitis of the central nervous system: report of 2 cases and review of the literature. Arthritis Rheum 49: 421–427

    Article  Google Scholar 

  15. Riemer G et al. (1999) Isolated angiitis of the central nervous system: lack of inflammation after long-term treatment. Neurology 52: 196–199

    Article  CAS  Google Scholar 

Download references

Acknowledgements

The authors would like to acknowledge S Savitz, S Kumar and M Mehdiratta (Stroke Neurologists) for their care of the patient, and M Anderson (Neuropathologist) for additional brain tissue staining (all based at the Beth Israel Deaconess Medical Center, Boston, MA, USA).

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Authors and Affiliations

  1. A Amick is a Fellow in Neurophysiology, N Silvestri is a Neurology Resident, and M Selim is Co-Director of the Beth Israel Deaconess Medical Center Stroke Center, at the Beth Israel Deaconess Medical Center, Boston, MA, USA,

    Amy Amick, Nicholas Silvestri & Magdy Selim

  2. J Joseph is a Professor of Pathology at the University of Calgary and Section Head of Neuropathology for the Calgary Laboratory Services, Calgary, AL, Canada.,

    Jeff Joseph

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  1. Amy Amick
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  2. Jeff Joseph
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Correspondence to Amy Amick.

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The authors declare no competing financial interests.

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Amick, A., Joseph, J., Silvestri, N. et al. Amyloid-β-related angiitis: a rare cause of recurrent transient neurological symptoms. Nat Rev Neurol 4, 279–283 (2008). https://doi.org/10.1038/ncpneuro0769

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