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Quantitative susceptibility mapping in superficial hemosiderosis of the central nervous system
Cyril Dargazanli, Jeremy Deverdun, Caroline Lionnet, Stéphanie Michau, Enes Ozluk, Astrid Corlobé, Xavier Ayrignac, Clarisse Carra-Dallière,
Emmanuelle Le Bars, Pierre Labauge, et al.
To cite this version:
Cyril Dargazanli, Jeremy Deverdun, Caroline Lionnet, Stéphanie Michau, Enes Ozluk, et al.. Quan- titative susceptibility mapping in superficial hemosiderosis of the central nervous system. Jour- nal de Neuroradiologie / Journal of Neuroradiology, Elsevier Masson, 2015, 42 (6), pp.370–372.
�10.1016/j.neurad.2015.04.007�. �hal-01987722�
Qua ntit tive susceptibility m pping in superfici l hemosiderosis of the centr l nervous system
Descriptionof the c se
A19-year-old manwith norelevant medical history except ahighvelocity head trauma afew years earlier wasadmit- ted inour institution for sudden onset binocular diplopia.
Head-CTperformed afteer the trauma wasdepicted asbeing normal. Hecomplained ofchronic headaches foraboutthree years, withrecent worsening andpainkillers resistance.
Clinical examination found binocular ophthalmoplegia withparesis of bothabducens nerves.
Non-contrast CT-scan and CT angiography of the circle ofWilliswere normal. Brain MRIacquisition wasperformed on a 3T magnet (Skyra, Siemens, Germany) with a 32- channel head coil and included a dual-echo Susceptibility Weighted Imaging (SWI), with the following parame- ters: TE1/TE2 =20/40 ms, TR=47ms, GRAPPA=2, voxel size=1× 1× 1.5 mm, 88 slices). Conventional sequences showed a subtle distension of the perioptic subarachnoid spaces oncoronal T2sequence (notshown), andalinear loss ofsignal onpial surface ofthe lefte lateral fissure (Fig. 1a).
Susceptibility-weighted imaging was performed inorder to quantify therelated susceptibility effeect, throughtheuseof quantitative susceptibility mapping. Markedpial signal loss on T2-GRE and susceptibility-weighted imaging (SWI) was observed atthe level ofthe brainstem, lefte lateral fissure, cerebellar folia (Fig. 1b—f). Phaseimage wasretrieved from SWIacquisition andunderwent laplacian unwrapping aswell as background field removal using regularization-enabled SHARPalgorithm. Finally, total variation usingsplitBregman [1]method enabled images conversion to quantitative sus- ceptibility maps(QSM).Reported susceptibility values were standardized according to the observed cerebrospinal fluid susceptibility. Innormal appearing graymatteer (Fig. 1g—h), thevalue was0.009 ± 0.1 ppm, whileincortico-pial affeected areas, measured susceptibility was0.24 ±0.1 ppm(Fig. 1f), suggesting a paramagnetic effeect. Discrete atrophy of the superior cerebellar vermis was also noted (Fig. 1k). T2- weighted images of the spinal cord showed a low signal lining on the spinal cord, suggesting hemosiderin deposit (Fig. 1i—j). Noacute subarachnoid bleeding was present.
Neither MRangiographyoftheintracranial vessels norspinal
MRIrevealed anyvascular malformation. Nointra-axial hem- orrhage wasfound.
Fundoscopic examination revealed bilateral papillary subedema that wasconfirmed by the fluorescein angiogra- physhowinglate papillary dye leakage.
Lumbar puncture was finally performed and showed severe intracranial hypertension (57 cmH2O, normal range 7—15 cmH20). Neither erythrocytes norxantochromia were present.
The diagnosis of superficial siderosis of the central ner- vous system was made based on the symptoms that were supported byradiological findings.
Discussion
Superficial siderosis ofthe central nervous system isarare disorder resulting fromchronic orintermitteent bleeding into the subarachnoid space with hemosiderin deposition (blood breakdown products) in the subpial layers of the brain- stem, cerebellum, cranial nerves, and spinal cord, leading to slowly progressive neurological dysfunction.
Thefollowing etiologies havebeen reported: 35%ofcases are caused by an idiopathic chronic subarachnoid hemor- rhage originating fromanoccult source andthe other cases are secondary toaknowncause ofsubarachnoid hemorrhage such asacurrent orprevious central nervous system tumor (21%), arteriovenous malformation or aneurysms (9%), or trauma (13%). Other less common causes of chronic sub- arachnoid hemorrhage leading to superficial siderosis are post-operative changes (7%), brachial plexus injury (6%), amyloid angiopathy (3%).
Clinical features are characterized bysensorineural deaf- ness, cerebellar ataxia, pyramidal signs, cognitive decline and bladder disturbance. Chronic increased intracranial pressure andhydrocephalus mayoccur asaresult ofobstruc- tion of the ventricular foramina and/or dysfunction of the Pachioni granules, due to hemosiderin deposits. Asymp- tomatic cases of superficial siderosis are of increasing frequency, because ofthe rising availability of MRI.
MRIusually shows hemosiderin deposition in the crests of cerebellar folia, superior vermis, eighth cranial nerve, quadrigeminal plate, andbasal cerebral surface, butitmay also involve interhemispheric fissure, lateral fissures, cor- tical sulci and pial surface of the spinal cord. Cerebellar atrophy iscommonly reported.
Figure1 CoronalT2-WIshows amarked loss ofsignal onpial surface of lefte insula, frontal andtemporal operculum andfrontal inferior gyrus(a), observed onaxial T2-GRE sequence (b). Magnetic susceptibility artifact outlining mesencephalon, folia of the culmen andcerebellar hemispheres isbetteer seen onSWI(cande) thanonT2-GREsequence (d). QSMmapreveals hypersignal atthe same level, duetoaparamagnetic effeect (f). BothSWIat thelevel ofcentrum semiovale anditscorresponding QSMmapshowthe absence ofpathologic hemosiderin deposit orsusceptibility artifact (g andh). Sagitteal T2-WIandaxial SWIshow thinhemosiderin deposits onthepial surface ofthemedulla andcervical spinal cord (iandj). Atrophyofthe superior cerebellar vermis isobserved onsagitteal T1-WI(k).
Inthecaseseries byKumaretal.[2], 16patients outof30 hadafluid-filled collection orpseudomeningocele, mostlyin the spinal canal. MRIofthe spine istherefore mandatory in case of superficial hemosiderosis.
Cerebrospinal fluidanalysisusuallyshowsxanthochromia andelevated erythrocyte count.
Therapy aimsatpreventing progression ofthedisease by ablating the source of subarachnoid hemorrhage. Unfortu- nately, extensive investigation for a source of hemorrhage israrely fruitful.
SWIsequence isarelatively newsequence. Itisbecoming aroutine practice duetoitsusefulness indiagnosingdiseases accompanied byirondeposition orintracranial calcifications [3].
SWIis more sensitive than T2-GRE for the detection of intra-axial hemorrhage and there is some evidence that SWIis also efficient in detecting extra-axial hemorrhage [4,5]. However, it remains a qualitative technique. Quaan- titative susceptibility maps provide a quantitative analysis of the susceptibility effeect and, to ourknowledge, there is no published data using QSMin the context of superficial hemosiderosis.
Conclusion
QSMuniquely allows extraction of numeric data and might beconsidered asanew biomarker forpatients’ follow-up in case of evolutive diseases (e.g. superficial hemosiderosis).
Disclosureof interest
The authors declare that they have noconflicts ofinterest concerning thisarticle.
References
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[2] Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE. Superficial siderosis. Neurology 2006;66(8):
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[3] Hodel J, Rodallec M,Gerber S, Blanc R, Maraval A, Caron S, et al. [Susceptibility weighted magnetic resonance sequences
‘‘SWAN, SWIand VenoBOLD’’: technical aspects and clinical applications]. J Neuroradiol 2012;39(2):71—86.
[4] Verma RK, Kotteke R, Andereggen L, Weisstanner C, Zubler C, Gralla J, et al. Detecting subarachnoid hemorrhage: com- parison of combined FLAIR/SWI versus CT. Eur J Radiol 2013;82(9):1539—45.
[5] DeChampfleurNM,LangloisC,Ankenbrandt WJ,LeBarsE,Leroy MA,DuffeauH, et al. Magnetic resonance imagingevaluation of cerebral cavernous malformations with susceptibility-weighted imaging. Neurosurgery 2011;68(3):641—7 [Discussion647—8].
CyrilDargazanlia Jérémy Deverduna,b Caroline Lionnetc Stéphanie Michaud EnesOzluke AstridCorlobéc Xavier Ayrignacc Clarisse Carra-Dallière c Emmanuelle LeBarsa Pierre Labaugec AlainBonaféa Nicolas Menjot deChampfleura,∗
aDepartment of Neuroradiology, Guide Chauliac Hospital, Montpellier University Hospital Center, 340 91 Montpellier cedex 5, France
bCNRSUMR5221, Montpellier IIUniversity, 340 Montpellier, France
cDepartment ofNeurology, Guide Chauliac Hospital, Montpellier University Hospital Center, 340 91 Montpellier cedex 5, France
dDepartment of Ophtalmology, Guide Chauliac Hospital, Montpellier University Hospital Center, 340 91 Montpellier cedex 5, France
eRadiology Department, Cerrahpas¸aFaculty ofMedicine, Istanbul, Turkey
∗Corresponding author.
E-mail addresses: [email protected] (C. Dargazanli), [email protected] (J. Deverdun), [email protected] (C. Lionnet), [email protected] (S. Michau), [email protected] (E. Ozluk), [email protected] (A. Corlobé), [email protected] (X. Ayrignac), [email protected] (C. Carra-Dallière),
e-le [email protected] (E. LeBars), [email protected] (P.Labauge), [email protected] (A. Bonafé), nicolasdechampfl[email protected] (N. Menjotde Champfleur)
