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Neurology. 2016 Mar 8;86(10):912-9. doi: 10.1212/WNL.0000000000002444. Epub 2016 Feb 5.

Pseudotumoral presentation of cerebral amyloid angiopathy-related inflammation.

Author information

1
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.
2
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. jérôme.honnorat@chu-lyon.fr.

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.

PMID:
26850981
PMCID:
PMC4782113
DOI:
10.1212/WNL.0000000000002444
[Indexed for MEDLINE]
Free PMC Article

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