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March 08, 2016; 86 (10) Article

Pseudotumoral presentation of cerebral amyloid angiopathy–related inflammation

Solène Ronsin, Gianluca Deiana, Ana Filipa Geraldo, Françoise Durand-Dubief, Laure Thomas-Maisonneuve, Maïté Formaglio, Virginie Desestret, David Meyronet, Norbert Nighoghossian, Yves Berthezène, Jérôme Honnorat, François Ducray
First published February 5, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002444
Solène Ronsin
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Gianluca Deiana
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Ana Filipa Geraldo
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Françoise Durand-Dubief
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Laure Thomas-Maisonneuve
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Maïté Formaglio
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Virginie Desestret
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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David Meyronet
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Norbert Nighoghossian
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Yves Berthezène
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Jérôme Honnorat
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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François Ducray
From the Neuro-oncology Department (S.R., L.T.-M., J.H., F.D.), Neuro-radiology Department (G.D., A.F.G., Y.B.), Neurology Department A (F.D.-D.), Neurology Department D (M.F., V.D.), Neuropathology Department (D.M.), and Stroke Unit (N.N.), Hôpital Neurologique, Hospices Civils de Lyon; Université de Lyon–Université Claude Bernard Lyon 1 (S.R., G.D., A.F.G., F.D.-D., L.T.-M., M.F., V.D., D.M., N.N., Y.B., J.H., F.D.), France; Neurology Department and Stroke Unit (G.D.), Ospedale San Francesco, Nuoro, Italy; and Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292 (J.H., F.D.), France.
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Citation
Pseudotumoral presentation of cerebral amyloid angiopathy–related inflammation
Solène Ronsin, Gianluca Deiana, Ana Filipa Geraldo, Françoise Durand-Dubief, Laure Thomas-Maisonneuve, Maïté Formaglio, Virginie Desestret, David Meyronet, Norbert Nighoghossian, Yves Berthezène, Jérôme Honnorat, François Ducray
Neurology Mar 2016, 86 (10) 912-919; DOI: 10.1212/WNL.0000000000002444

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Abstract

Objective: To identify the clinical and radiologic features that should raise suspicion for the pseudotumoral presentation of cerebral amyloid angiopathy–related inflammation (CAA-I).

Methods: We retrospectively reviewed the characteristics of 5 newly diagnosed and 23 previously reported patients in whom the CAA-I imaging findings were initially interpreted as CNS neoplasms.

Results: Most cases (85%) occurred in patients >60 years old. The clinical characteristics at presentation included subacute cognitive decline (50%), confusion (32%), focal deficits (32%), seizures (25%), and headaches (21%). Brain MRI demonstrated infiltrative white matter lesions that exhibited a loco-regional mass effect without parenchymal enhancement (93%). In general, these findings were interpreted as low-grade glioma or lymphoma. Eighteen patients (64%) underwent a biopsy, which was nondiagnostic in 4 patients (14%), and 6 patients (21%) underwent a surgical resection. The primary reason for the misinterpretation of the imaging findings was the absence of T2*-weighted gradient recalled echo (T2*-GRE) sequences on initial imaging (89%). When subsequently performed (39%), the T2*-GRE sequences demonstrated multiple characteristic cortical and subcortical microhemorrhages in all cases. Perfusion MRI and magnetic resonance spectroscopy (MRS), which were performed on a subset of patients, indicated markedly reduced relative cerebral blood flow and a normal metabolic ratio.

Conclusion: The identification of one or several nonenhancing space-occupying lesions, especially in elderly patients presenting with cognitive impairment, should raise suspicion for the pseudotumoral presentation of CAA-I and lead to T2*-GRE sequences. Perfusion MRI and MRS appear to be useful techniques for the differential diagnosis of this entity.

GLOSSARY

Aβ=
β-amyloid;
CAA=
cerebral amyloid angiopathy;
CAA-I=
CAA-related inflammation;
DWI=
diffusion-weighted imaging;
FLAIR=
fluid-attenuated inversion recovery;
1H-MRS=
proton magnetic resonance spectroscopy;
MRA=
magnetic resonance angiography;
MTT=
mean transit time;
rCBF=
relative cerebral blood flow;
rCBV=
relative cerebral blood volume;
SWI=
susceptibility-weighted imaging;
T2*-GRE=
T2*-weighted gradient-recalled echo

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Supplemental data at Neurology.org

  • Received April 10, 2015.
  • Accepted in final form November 9, 2015.
  • © 2016 American Academy of Neurology
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