Non-inflammatory cerebral amyloid angiopathy as a cause of rapidly progressive dementia: A case study

Abstract A 77 year-old men developed a subacute-onset, rapidly progressive cognitive decline. After 6 months of evolution, he scored 6 on the Mini-Mental State Examination and had left hemiparesis and hemineglect. The patient died 11 months after the onset of cognitive symptoms. Brain MRI showed microhemorrhages on gradient-echo sequence and confluent areas of white matter hyperintensities on T2-weighted images. Brain biopsy revealed amyloid-b peptide deposition in vessel walls, some of them surrounded by micro-bleeds. In this case report, we discuss the role of cerebral amyloid angiopathy (CAA) in cognitive decline, due to structural lesions associated with hemorrhages and infarcts, white matter lesions and co-morbidity of Alzheimer's disease, as well as the most recently described amyloid angiopathy-related inflammation.

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A case of inflammatory cerebral amyloid angiopathy with white matter lesions appearing after brain biopsy

Rinsho Shinkeigaku ◽

10.5692/clinicalneurol.cn-001534 ◽

2021 ◽

Vol 61 (3) ◽

pp. 188-193

Author(s):

Yosuke Takeuchi ◽

Shuei Murahashi ◽

Yasuyuki Hara ◽

Makoto Nakajima ◽

Mitsuharu Ueda

Keyword(s):

White Matter ◽

Cerebral Amyloid Angiopathy ◽

White Matter Lesions ◽

Brain Biopsy ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

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Abstract 36: The Boston Criteria V2.0 for Cerebral Amyloid Angiopathy: Updated Criteria and Multicenter MRI-Neuropathology Validation

Stroke ◽

10.1161/str.52.suppl_1.36 ◽

2021 ◽

Vol 52 (Suppl_1) ◽

Author(s):

Andreas Charidimou ◽

Gregoire Boulouis ◽

Matthew Frosch ◽

Jean-Claude Baron ◽

Marco Pasi ◽

...

Keyword(s):

White Matter ◽

Intracerebral Hemorrhage ◽

Diagnostic Accuracy ◽

Cerebral Amyloid Angiopathy ◽

Brain Mri ◽

Amyloid Angiopathy ◽

Perivascular Spaces ◽

Validation Sample ◽

Temporal Validation ◽

Cerebral Amyloid

Introduction: The Boston criteria are used worldwide for in vivo diagnosis of cerebral amyloid angiopathy (CAA). Given substantial advances in CAA research, we aimed to update the Boston criteria and externally validate their diagnostic accuracy across the spectrum of CAA-related presentations and across international sites. Methods: As part of an International CAA Association multicenter study, we identified patients age 50 or older with potential CAA-related clinical presentations (spontaneous intracerebral hemorrhage, cognitive impairment, or transient focal neurological episodes), available brain MRI, and histopathologic assessment for the diagnosis of CAA. We derived Boston criteria v2.0 by selecting MRI features to optimize diagnostic specificity and sensitivity in a pre-specified derivation sample (Boston cases 1994 to 2012, n=159), then externally validated in pre-specified temporal (Boston cases 2012-2018, n=59) and geographical (non-Boston cases 2004-2018; n=123) validation samples and compared their diagnostic accuracy to the currently used modified Boston criteria. Results: Based on exploratory analyses in the derivation sample, we derived provisional criteria for probable CAA requiring presence of at least 2 strictly lobar hemorrhagic lesions (intracerebral hemorrhage, cerebral microbleed, or cortical superficial siderosis focus) or at least 1 strictly lobar hemorrhagic lesion and 1 white matter characteristic (severe degree of visible perivascular spaces in centrum semiovale or white matter hyperintensities multispot pattern). Sensitivity/specificity of the criteria were 74.8/84.6% in the derivation sample, 92.5/89.5% in the temporal validation sample, 80.2/81.5% in the geographic validation sample, and 74.5/95.0% in cases across all samples with autopsy as the diagnostic gold standard. The v2.0 criteria for probable CAA had superior accuracy to the currently modified Boston criteria (p<0.005) in the autopsied cases. Conclusion: The Boston criteria v.2.0 incorporate emerging MRI markers of CAA to enhance sensitivity without compromising their high specificity. Validation of the criteria across independent patient settings firmly supports their adoption into clinical practice and research.

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A case of cerebral amyloid angiopathy with reversible white matter lesions and multiple cerebral microbleeds

Rinsho Shinkeigaku ◽

10.5692/clinicalneurol.52.90 ◽

2012 ◽

Vol 52 (2) ◽

pp. 90-95 ◽

Cited By ~ 1

Author(s):

Yasushi Hosoi ◽

Tsuyoshi Uchiyama ◽

Mari Yoshida ◽

Daisuke Takechi ◽

Takako Shimizu ◽

...

Keyword(s):

White Matter ◽

Cerebral Amyloid Angiopathy ◽

White Matter Lesions ◽

Cerebral Microbleeds ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

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Cerebral amyloid angiopathy-related leukoencephalopathy: Successful steroid treatment for neurological deficits and subcortical white matter lesions partly involving the cortical gray matter

Neurology and Clinical Neuroscience ◽

10.1111/ncn3.101 ◽

2014 ◽

Vol 2 (4) ◽

pp. 119-121

Author(s):

Takenobu Murakami ◽

Julia Morimoto ◽

Akihiko Hoshi ◽

Setsu Nakatani-Enomoto ◽

Masahiro Ichikawa ◽

...

Keyword(s):

White Matter ◽

Cerebral Amyloid Angiopathy ◽

Gray Matter ◽

White Matter Lesions ◽

Subcortical White Matter ◽

Steroid Treatment ◽

Neurological Deficits ◽

Amyloid Angiopathy ◽

Cortical Gray Matter ◽

Cerebral Amyloid

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Cerebral Amyloid Angiopathy: Multiple neurolgic problems

Psychology and Mental Health Care ◽

10.31579/2637-8892/015 ◽

2017 ◽

Vol 1 (3) ◽

pp. 01-01

Author(s):

Paul Gilbert

Keyword(s):

White Matter ◽

Cerebral Amyloid Angiopathy ◽

Clinical Symptoms ◽

Brain Biopsy ◽

Acute Onset ◽

Amyloid Angiopathy ◽

Migraine Headaches ◽

Diffuse White Matter ◽

Cerebral Amyloid ◽

Intravenous Steroids

A 72-Year-old female with a history of migraine headaches presented with an acute onset of expressive aphasia, difficulty with memory and worsening of her headaches. An MRI of the brain was done which revealed diffuse white matter T2 hyperintensities (Figures 1). Due to worsening of the patient’s clinical symptoms a repeat MRI was performed four days later that revealed multiple micro-bleeds (Figure 2), as well as a lobar hemorrhage in left temporal lobe (Figure 2). An extensive workup including HIV testing, CSF examination for infectious etiology including protein 14-3-3 and demylineating disease was negative. Paraneoplastic and autoimmune workup was also non-diagnostic. A brain biopsy was performed due to the extensive white matter disease, which revealed Cerebral Amyloid Angiopathy (CAA) with focal granulomatous angiitis. The patient was treated with intravenous steroids with no significant improvement clinically. Two months after diagnosis, her disease course has remained static, without improvement or deterioration.

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083 Response to immunosuppressive therapy in cerebral amyloid angiopathy-related inflammation

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-anzan.82 ◽

2018 ◽

Vol 89 (6) ◽

pp. A33.3-A34

Author(s):

Jasmin Tilling ◽

Benjamin Trewin ◽

Stanley Levy

Keyword(s):

White Matter ◽

Cognitive Decline ◽

Cerebral Amyloid Angiopathy ◽

White Matter Hyperintensities ◽

Neurological Deficits ◽

Amyloid Angiopathy ◽

Dramatic Improvement ◽

Age Related ◽

Cerebral Amyloid ◽

The Brain

IntroductionCerebral amyloid angiopathy (CAA) is a common age-related condition characterised by amyloid beta-peptide deposition affecting the medium sized cortical and leptomeningeal arteries, arterioles and capillaries. CAA-related Inflammation (CAA-I) is an increasingly recognised variant of CAA, which is thought to be due to perivascular auto-inflammation in response to amyloid deposition. We describe the clinical course of two cases of probable CAA-I.CasesA 71 year old man presented with new-onset seizures, headaches and subacute cognitive decline. MRI of the brain demonstrated confluent subcortical T2 white matter hyperintensities and cerebral oedema, with predominantly superimposed widespread cortico-subcortical micro-haemorrhages, in keeping with the diagnosis of CAA-I. A course of immunosuppresive therapy was commenced with five days of intravenous methylprednisolone, resulting in marked radiological and clinical improvement within two weeks.A 76 year old female presented with subacute cognitive dysfunction and apraxia, and transient left-sided weakness. MRI scan of the brain initially demonstrated a right temporo-occipital infarct, leading to primary treatment for stroke, but subsequently evolved to reveal diffuse multi-lobar T2 white matter hyperintensities with leptomeningeal involvement. A provisional diagnosis of CAA-I was made and following a poor clinical response to a trial of corticosteroid therapy, treatment with intravenous cyclophosphamide was commenced.ConclusionThese cases emphasise the importance of CAA-I as part of the differential diagnosis in patients presenting with symptoms of subacute cognitive decline, seizures, headaches and focal neurological deficits, given the potential for dramatic improvement with readily accessible immunosuppressive therapies.

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