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Research ArticleEXTRACRANIAL VASCULAR

TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity

A. Lecler, M. Obadia, J. Savatovsky, H. Picard, F. Charbonneau, N. Menjot de Champfleur, O. Naggara, B. Carsin, M. Amor-Sahli, J.P. Cottier, J. Bensoussan, E. Auffray-Calvier, A. Varoquaux, S. De Gaalon, C. Calazel, N. Nasr, G. Volle, D.C. Jianu, O. Gout, F. Bonneville and J.C. Sadik
American Journal of Neuroradiology July 2017, 38 (7) 1391-1398; DOI: https://doi.org/10.3174/ajnr.A5214
A. Lecler
aFrom the Departments of Radiology (A.L., J.S., F.C., J.C.S.)
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M. Obadia
bNeurology (M.O., G.V., O.G.)
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  • ORCID record for M. Obadia
J. Savatovsky
aFrom the Departments of Radiology (A.L., J.S., F.C., J.C.S.)
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  • ORCID record for J. Savatovsky
H. Picard
cClinical Research Unit (H.P.), Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
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  • ORCID record for H. Picard
F. Charbonneau
aFrom the Departments of Radiology (A.L., J.S., F.C., J.C.S.)
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N. Menjot de Champfleur
dDepartment of Neuroradiology (N.M.d.C.), Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier, France
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O. Naggara
fDepartment of Neuroradiology (O.N.), Centre Hospitalier Sainte-Anne, Paris, France
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B. Carsin
eDepartment of Radiology (B.C.), Centre Hospitalier Régional Universitaire de Rennes, Rennes, France
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M. Amor-Sahli
gDepartment of Neuroradiology (M.A.-S.), Pitié-Salpêtrière Hospital, Paris, France
hCentre D'imagerie Médicale Tourville (M.A.-S.), Paris, France
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J.P. Cottier
iDepartment of Radiology (J.P.C.), Centre Hospitalier Régional Universitaire de Tours, Tours, France
jBrain and Imaging Laboratory Unite Mixte de Recherche U930 (J.P.C.), Institut National de la Santé et de la Recherche Médicale, François-Rabelais University, Tours, France
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J. Bensoussan
mDepartment of Radiology (J.B.), Hotel-Dieu Hospital, Paris, France
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E. Auffray-Calvier
kDiagnostic and Interventional Neuroradiology Department (E.A.-C.)
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A. Varoquaux
nDepartment of Radiology (A.V.), Conception Hospital, Aix-Marseille University, Marseille, France
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S. De Gaalon
lNeurology Department (S.D.G.), Hôpital René et Guillaume-Laënnec, Centre Hospitalier Universitaire de Nantes, Saint-Herblain, France
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C. Calazel
oDepartments of Neuroradiology (C.C., F.B.)
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N. Nasr
pNeurology (N.N.), Hôpital Pierre-Paul-Riquet, Centre Hospitalier Universitaire Purpan, Toulouse, France
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G. Volle
bNeurology (M.O., G.V., O.G.)
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D.C. Jianu
qDepartment of Neurology (D.C.J.), Victor Babes University of Medicine and Pharmacy, Timisoara, Romania.
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O. Gout
bNeurology (M.O., G.V., O.G.)
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F. Bonneville
oDepartments of Neuroradiology (C.C., F.B.)
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J.C. Sadik
aFrom the Departments of Radiology (A.L., J.S., F.C., J.C.S.)
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    Fig 1.

    Diagnostic ultrasonography (A–D) shows an eccentric perivascular infiltration (arrowhead) at the level of bifurcation, with a soft intimal plaque (arrow) and a mild lumen narrowing without a hemodynamic change in Doppler mode. Follow-up ultrasonography (E) shows a marked decrease in the perivascular infiltration (arrowhead) and complete disappearance of the soft intimal plaque.

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    Fig 2.

    CTA shows a left posterolateral eccentric perivascular infiltration (arrowhead) surrounding the carotid artery, with a distinct low-density soft intimal plaque (arrow).

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    Fig 3.

    Initial diagnostic ultrasonography (A) and follow-up ultrasonography at 14 days (B) and 6 months (C) show a perivascular infiltration (arrowheads) at the level of the internal carotid artery just at the level of bifurcation, with a quick decrease at 14 days and the persistence of a thin abnormality at 6 months.

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    Fig 4.

    Pre (A) and post (B) contrast fat-suppressed 3D T1 and 3D T2-weighted (C) MR imaging in an axial plane shows T1 hypointense and T2 hyperintense perivascular infiltration (arrowhead) at the level of the carotid artery bifurcation, enhanced after gadolinium injection. A distinct soft intimal plaque (arrow) is visible at the posterior part of the carotid artery. A sagittal curvilinear reconstruction of the right internal carotid artery on the postcontrast T1-weighted imaging (D) shows the PVI (arrowhead) centered at the level of the right carotid artery bifurcation and extended to both the distal common carotid artery and proximal internal carotid artery. Note that there is no vascular or perivascular abnormality involving other parts of the common or the internal carotid arteries.

Tables

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    Table 1:

    Characteristics of patients

    CharacteristicsPatients%
    No. of patients47
    No. of carotid arteries49
    Sex ratio (women/men)27/18; 1.5:1
    Median age (yr) (IQR)48 (39–56)
    At least 1 vascular risk factor2247%
        High blood pressure817%
        Dyslipidemia36%
        Mellitus diabetes00%
        Smoking1940%
    History of autoimmune disease817%
    History of vascular event24%
    Recent history of viral episode24%
    Recent history of cervical chiropractic manipulation00%
    Recent history of cervical trauma12%
    Acute neck tenderness or cervical pain47100%
    Affected side (left/right)28/21
    Bilateral involvement24%
    Median pain intensity (10-point scale) (IQR)5 (3–6)
    Cervical swelling or palpable abnormality over the carotid bifurcation613%
    Enlarged lymph nodes at palpation817%
    Fever24%
    Flu-like symptoms12%
    Neurologic-associated symptoms817%
    No abnormal biologic test findings4289%
        Biologic inflammatory syndrome (elevated ESR or CRP)36%
        Positive serum IgM2/1811%
    Treatments3779%
        Anti-inflammatory treatment3472%
        Steroids36%
    Follow-up47100%
        Median follow-up duration (days) (IQR)163 (88–354)
        Full clinical recovery (SD)47100%
        Mean recovery delay (days) (IQR)13 (10–15)
        Relapse919%
        Median delay before relapse (mo)6
        Persistence of abnormal laboratory test findings00%
    • Note:—ESR indicates erythrocyte sedimentation rate; CRP, C-reactive protein; IgM, immunoglobulin-M; IQR, interquartile range.

    • View popup
    Table 2:

    Diagnostic imaging data

    CharacteristicsUS%CT%MRI%
    No. of patients2349%1328%4391%
    No. of carotid arteries2449%1327%4592%
    Median delay between onset of symptoms and diagnostic exam (days) (IQR)5 (3–7.5)
    High confidence in diagnosis24100%969%45100%
    Preferential side of PVI: lateral/medial39/10: 3.9
    Preferential side of PVI: posterior/anterior38/11: 3.5
    Localization of PVI: carotid bifurcation2396%1292%4191%
    Circumferential00%00%24%
    Percentage of carotid circumference involved (%) (IQR)40 (30–40)30 (20–40)40 (30–57.5)
    PVI24100%13100%45100%
    Pericarotid fat stranding24100%13100%45100%
    Lumen caliber narrowing938%431%1227%
    Mean percentage of carotid stenosis (%) (IQR)30 (20–30)22.5 (18–26)20 (14–30)
    Mean PVI span (mm) (IQR)15 (10–22)15 (10–26)28 (17–34)
    Median PVI largest diameter (mm)4 (2.4–6)4 (3.6–4.8)5 (4–7)
    Soft intimal plaque1458%646%1227%
    Enlarged lymph nodes833%00%818%
    PVI enhancementNA1185%45100%
    Laryngeal or pharyngeal inflammation00%00%12%
    • Note:—IQR indicates interquartile range; NA, not applicable.

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    Table 3:

    Follow-up imaging data

    CharacteristicsUS%MRI%
    No. of patients1144%2351%
    Median delay between onset of symptoms and follow-up exam (days) (IQR)89 (71.5–171)
    PVI11100%1565%
    Complete PVI disappearance00%835%
        PVI decrease11100%1565%
        PVI stability00%00%
        PVI increase00%00%
    Lumen caliber narrowing218%14%
    Median PVI largest diameter (mm) (IQR)1.5 (0.9–2.4)2.9 (2.0–3.5)
    Median decrease of the PVI largest diameter61%55%
    Median PVI span (mm) (IQR)9 (5–10)13 (9.5–16)
    Median decrease of the PVI span62%50%
    Persistence of a soft intimal plaque450%333%
    Disappearance of a soft intimal plaque450%667%
    PVI enhancementNA1252%
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A. Lecler, M. Obadia, J. Savatovsky, H. Picard, F. Charbonneau, N. Menjot de Champfleur, O. Naggara, B. Carsin, M. Amor-Sahli, J.P. Cottier, J. Bensoussan, E. Auffray-Calvier, A. Varoquaux, S. De Gaalon, C. Calazel, N. Nasr, G. Volle, D.C. Jianu, O. Gout, F. Bonneville, J.C. Sadik
TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity
American Journal of Neuroradiology Jul 2017, 38 (7) 1391-1398; DOI: 10.3174/ajnr.A5214

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TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity
A. Lecler, M. Obadia, J. Savatovsky, H. Picard, F. Charbonneau, N. Menjot de Champfleur, O. Naggara, B. Carsin, M. Amor-Sahli, J.P. Cottier, J. Bensoussan, E. Auffray-Calvier, A. Varoquaux, S. De Gaalon, C. Calazel, N. Nasr, G. Volle, D.C. Jianu, O. Gout, F. Bonneville, J.C. Sadik
American Journal of Neuroradiology Jul 2017, 38 (7) 1391-1398; DOI: 10.3174/ajnr.A5214
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