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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Review ArticleAdult Brain
Open Access

Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum

G. Bathla, P. Watal, S. Gupta, P. Nagpal, S. Mohan and T. Moritani
American Journal of Neuroradiology July 2018, 39 (7) 1194-1200; DOI: https://doi.org/10.3174/ajnr.A5492
G. Bathla
aFrom the Division of Neuroradiology (G.B., P.W., P.N.,T.M.), Department of Radiology
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P. Watal
aFrom the Division of Neuroradiology (G.B., P.W., P.N.,T.M.), Department of Radiology
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S. Gupta
bDepartment of Pathology (S.G.), University of Iowa Hospitals and Clinics, Iowa City, Iowa
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P. Nagpal
aFrom the Division of Neuroradiology (G.B., P.W., P.N.,T.M.), Department of Radiology
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S. Mohan
cDivision of Neuroradiology (S.M.), Department of Radiology, University of Pennsylvania, Philadelphia, Pennsylvania.
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T. Moritani
aFrom the Division of Neuroradiology (G.B., P.W., P.N.,T.M.), Department of Radiology
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  • Fig 1.
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    Fig 1.

    Brain biopsies from different patients showing typical sarcoid granuloma (short arrows) with a Langerhans-type giant cell (long arrow) (H&E, original magnification ×20) (A), transmural granulomatous involvement of a meningeal vessel (short arrows, star represents the residual vessel lumen) (H&E, original magnification ×20) (B), and granulomatous vessel wall involvement (long arrows) with fibrinoid necrosis (short arrows) (H&E, original magnification ×10) (C).

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    Fig 2.

    Axial DWIs at the level of the corona radiata obtained at 2 different time points (A and B) show recurrent periventricular infarcts. Axial magnified pre- (C) and postcontrast (D) images reveal subtle perivascular enhancement involving the bilateral basal ganglia (arrowheads, D).

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    Fig 3.

    Axial DWIs reveal tiny subcortical infarcts (A and B) in a 22-year-old female patient. Axial postcontrast image (C) shows prominent involvement of the midline falx anteriorly. The infarcts were presumably secondary to involvement of the cortical vessels.

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    Fig 4.

    Axial DWI (A) in a 38-year-old male patient with NS reveals punctate infarcts involving the left cerebral peduncle and midbrain (arrows). A volume-rendered CT angiogram shows multifocal stenoses involving the P1 segments of the bilateral posterior cerebral arteries (B). The patient again presented after 2 months with severe headache. Noncontrast CT (C) image at this time shows acute subarachnoid hemorrhage over bilateral parietal convexities (arrows).

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    Fig 5.

    Axial SWI at the level of centrum semiovale (A) and basal ganglia (B) from 1 patient and at the level of basal ganglia in the other patient (C). There are multiple scattered foci of susceptibility involving bilateral subcortical white matter (arrows) with relative sparing of the basal ganglia in both patients.

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    Fig 6.

    A and B, Axial postcontrast images through the posterior fossa obtained over an interval of 10 years. The prominent left sigmoid sinus enhancement remains essentially unchanged. The patient also had superior sagittal sinus involvement (not shown).

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    Fig 7.

    Coronal pre- (A) and postcontrast (B) images in a patient with NS show nodular thickening and enhancement of the cavernous sinuses bilaterally. The patient had additional intracranial stigmata of NS (not shown), and the cavernous sinus involvement was presumed to be secondary to NS.

Tables

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    Table 1:

    Cases of NS with stroke in the reported English language literaturea

    AuthorYearAge (yr)/SexZajicek CriteriaCT/MRIDWITIAStrokeSystemic Disease
    Brown et al12198925/MProbableY/NSNSYLeft IC and corona radiataY
    Corse and Stern26199038/MProbableY/YNSYRight ICY
    Michotte et al4199129/MProbableY/YNSNLeft BG, right frontalY
    Zadra et al24199630/MProbableY/YNSNMultiple, anterior circulationY
    Das et al30199827/FProbableY/NSNSNLeft MCA infarctY
    Brisman et al3200641/MProbableNS/YYNMultiple, anterior circulationY
    Hodge et al2200736/FDefiniteNS/YYNLeft BGN
    Navi and DeAngelis31200935/MDefiniteNS/YNNPonsY
    Navi and DeAngelis31200946/FDefiniteY/YNSNPonsY
    González-Aramburu et al15201227/MProbableY/YYYRight IC and thalamusY
    Campbell et al32201526/MProbableY/YYYLeft centrum semiovaleY
    Raza and Schreck25201773/FProbableNS/YNSNRecurrent left MCA strokesY
    Macedo et al5201662/MPossibleY/YYYMultiple, anterior/posterior circulationY
    • Note:—BG indicates basal ganglia; IC, internal capsule; N, no; NS, not specified; Y, yes.

    • ↵a On the basis of the Zajicek criteria,1 cases may be definite (neural tissue biopsy), probable (presence of CNS inflammation with evidence of systemic disease), or possible (clinical presentation consistent with NS with exclusion of alternate diagnosis).

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    Table 2:

    Cases of NS with ICH in the reported English language literature

    AuthorYearAge (yr)/SexZajicek CriteriaCT/MRIMRA/DSASiteSystemic Disease
    Caplan et al28198321/MProbableY/NSNSLeft cerebellarY
    Oksanen461986NS/NSNSNS/NSNSNSNS
    Oksanen461986NS/NSNSNS/NSNSNSNS
    Berek et al40199335/MPossibleY/YNm DSASAH on LPN
    Libman et al42199748/FProbableY/Y+ DSAMultiple, left cerebral hemisphereY
    Eid et al43199831/FProbableY/NS+ DSARight frontalY
    Cipri et al47200031/MDefiniteY/YNm DSARight occipitalN
    Dakdouki et al45200525/MProbableNS/YNSPonsY
    Tsappidi et al38201143/MDefiniteY/YNm DSA/MRAMultiple, supratentorialN
    O'Dwyer et al36201341/MDefiniteY/YNm CTACerebellumN
    O'Dwyer et al36201341/MProbableY/YNm MRAPontomesecephalicY
    O'Dwyer et al36201336/MProbableY/YNm DSALeft frontalY
    Travers et al44201437/MProbableNS/YNmRight temporalY
    Pegat et al41201548/FProbableNS/YNSLeft frontoparietalY
    Vargas et al37201662/FProbableY/YNm MRAMultiple microhemorrhagesY
    Vukojević et al39201745/FProbableY/YNm CTAMultiple, supra- and infratentorialY
    • Note:—Nm indicates normal findings; LP, lumbar puncture; N, no; NS, not specified; Y, yes; +, positive.

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American Journal of Neuroradiology: 39 (7)
American Journal of Neuroradiology
Vol. 39, Issue 7
1 Jul 2018
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Cite this article
G. Bathla, P. Watal, S. Gupta, P. Nagpal, S. Mohan, T. Moritani
Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum
American Journal of Neuroradiology Jul 2018, 39 (7) 1194-1200; DOI: 10.3174/ajnr.A5492

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Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum
G. Bathla, P. Watal, S. Gupta, P. Nagpal, S. Mohan, T. Moritani
American Journal of Neuroradiology Jul 2018, 39 (7) 1194-1200; DOI: 10.3174/ajnr.A5492
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