Patient Report

Yonago Acta medica 2016;59:248–254

Atypical Arteritis in Internal Carotid Arteries: A Novel Concept of Isolated Internal
Carotid Arteritis
Kazuki Fukuma,* Hisanori Kowa,* Hiroyuki Nakayasu† and Kenji Nakashima‡*Division of Neurology, Department of Brain and Neurosciences, Tottori University Faculty of Medicine, Yonago 683-8504, Japan, †Department of Neurology, Tottori Prefectural Central Hospital, Tottori 680-0901, Japan and ‡Department of Neurology, National Hospital
Organization Matsue Medical Center, Matsue 690-8556, Japan

ABSTRACT

riod, and contrast-enhanced magnetic resonance imaging (MRI) revealed wall enhancement in bilateral ICAs.
Subsequent administration of prednisolone and antithrombotic therapy successfully ameliorated the clinicalconditions.

We presented a 38-year-old woman suffering from acutecerebral infarction due to arteritis limited to bilateralinternal carotid arteries without a condition of giant cellarteritis or granulomatosis with polyangitis. Our caseis unprecedented and characterized by a young woman
with wall enhancement in the internal carotid arteries oncontrast-enhanced magnetic resonance imaging (MRI),
therapeutic effects of steroids, and positive status for human leucocyte antigen-B39, -B51 and -DR4. These disease characteristics were not in accordance with existing
diagnostic criteria of vasculitis, such as Takayasu’s arteritis, giant cell arteritis, granulomatosis with polyangiitis, and Behcet’s disease. We suggested consideration of
a novel “isolated internal carotid arteritis” disease concept.

PATIENT REPORT
A 38-year-old woman presented with pain in the extremities and fluctuating left-sided weakness in the beginningof August 2011. At the end of August, she presented withright-sided weakness and was admitted to our hospital.
She had no past medical history and was not taking any
prescription medications. Family history pertaining to
her mother revealed high blood pressure and pertaining
to her father revealed depression. Her blood pressure was
154/108mmHg and her body temperature was 37.2 °C.
Physical examinations revealed tenderness in both arms,
although no arterial bruits or prominent temporal arteries were evident. Neurological examinations showed impaired consciousness (Glasgow Coma Score, E3V4M6),
left hemispatial neglect, ocular deviation to the right, left
supranuclear facial palsy, dysarthria and breathy voice,left flaccid hemiplegia, right mild hemiparesis, left deep
and mild superficial hypoesthesia with dysesthesia of theleft side of the body, hyperactive tendon reflexes in theright upper and lower limbs, and a right Babinski’s sign.
Urine testing yielded normal results, whereas laboratory data revealed elevated leukocyte counts (9800 cells/μL), erythrocyte sedimentation rates (ESR; 45 mm/h),
C-reactive protein levels (0.32 mg/dl) and CH50 titers(50 U/mL). Tests of blood coagulation and fibrinolysis
yielded normal results. However, serotyping of humanleucocyte antigen (HLA) class I and II revealed that thepatient was positive for A2, A24, B39, B51, DR4 andDR11, and tests for serum autoantibodies, including cytoplasmic and perinuclear anti-neutrophil cytoplasmic
antibodies, were all negative. Levels of vitamins, aminoacids, alpha-galactosidase, tumor markers, and markers
of infection, including tuberculosis, were all normal. Cerebrospinal fluid testing also revealed normal results.
Onset at a young age and absence of risk factors
indicated the presence of an uncommon cause of stroke.
On admission, magnetic resonance angiography (MRA;

Key words

arteritis; carotid artery; internal; magnetic
resonance imaging; vasculitis

Here, we report a case of arteritis that was limited to theinternal carotid artery (ICA), presenting a challenge toexisting disease concepts. The first International Chapel
Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC) proposed names and definitions for the most common forms of vasculitis in 1994,
and these were then revised at the second International
CHCC in 2012.1 Although atypical cases exist, no casesof arteritis limited to the ICA have been reported. The
present case suffered repeated strokes within a short peCorresponding author: Kazuki Fukuma, MD
kazblues77@mac.com
Received 2016 June 20
Accepted 2016 July 27
Abbreviations: ACA, anterior cerebral artery; CCA, common carotid artery; CHCC, Chapel Hill Consensus Conference; CT, computed tomography; ESR, erythrocyte sedimentation rates; GCA, giant
cell arteritis; GPA, granulomatosis with polyangiitis; HLA, humanleucocyte antigen; ICA, internal carotid artery; IMP-SPECT, iodoamphetamine single photon emission computed tomography; LVGCA, large-vessel giant cell arteritis; MCA, middle cerebral artery;
MRA, magnetic resonance angiography; MRI, magnetic resonanceimaging; PR3-ANCA, proteinase 3-antineutrophil cytoplasmic
antibody; STA, superficial temporal artery; TA, temporal arteritis;
TAK, Takayasu’s arteritis

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Isolated internal carotid arteritis

Fig. 1. Magnetic resonance imaging, magnetic resonance angiography, and digital subtraction angiography.
A) MRA on admission showed multiple stenosis and distal occlusion (arrows) in the left ICA, severe stenosis (arrows) in the distal right
ICA, and bilateral signal loss in the ACA and MCA.
B) Diffusion-weighted MRI on admission showed acute ischemic infarcts in bilateral frontal lobes.
C) Diffusion-weighted MRI on day 17 of hospitalization showed recurrent infarcts in the right frontal and temporal lobes and left coronaradiata.
D) Right CCA angiography of the cervical portion on day 15 of hospitalization showed severe stenosis (arrow) of the supraclinoid segment
and multiple arterial wall irregularities in the right ICA, cross-flow into the left ACA and MCA via the anterior communicating artery,and no abnormality in the branches of the right external carotid artery.
E) Left CCA angiography of the cervical portion on day 15 of hospitalization showed a diffusely thin left ICA with multiple arterial wall
irregularities, and occlusion (arrow) just distal to the ophthalmic artery origin. No abnormalities indicated Moyamoya disease, fibromuscular dysplasia, dissection, or intracranial arteritis, and no abnormalities were evident in the branches of the left external carotid artery.
ACA, anterior cerebral artery; CCA, common carotid artery; ICA, internal carotid artery; MCA, middle cerebral artery; MRA, magnetic
resonance angiography; MRI, magnetic resonance imaging.

reducing end-diastolic velocity in the left CCA, thickening of the wall to the right ICA, and complete absence ofcolor-flow signals of the cervical portion in the left ICA.
The anti-coagulant argatroban and the neuroprotective
agent edaravone were administered to improve the levels

Fig. 1A) showed occlusion of the left ICA and severe stenosis of the right ICA. Moreover, MRI (Fig. 1B) demonstrated acute ischemic infarcts in bilateral frontal lobes,
and carotid ultrasonography showed the absence of macaroni signs in bilateral common carotid arteries (CCAs),
249

K. Fukuma et al.

Fig. 2. Contrast-enhanced T1-weighted MRI.
A1, A2), admission; B1, B2, B3), day 23 of hospitalization; C1, C2, C3), at the time of discharge after prednisolone treatment.
A1) Retrospective contrast-enhanced T1-weighted MRI showed wall enhancement as in B1. A2) Magnified view of A1.
B1) Contrast-enhanced T1-weighted MRI showed wall enhancement in bilateral ICAs and bilateral wall thickening in the ICA and distal
CCA. B2) Magnified view of B1. B3) Cervical MRA showed stenosis (arrows) in the left ICA.
C1) Contrast-enhanced T1-weighted MRI showed persistent wall enhancement in bilateral ICAs. C2) Magnified view of C1. C3) MRA
showed bilateral improvements of signal intensity in the ACA and MCA.
ACA, anterior cerebral artery; CCA, common carotid artery; ECA, external carotid artery; ICA, internal carotid artery; MCA, middle
cerebral artery; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging.

of consciousness and ameliorate the right hemiparesis.
Subsequent N-isopropyl-p-(123I) iodoamphetamine single photon emission computed tomography (123I-IMP
SPECT) showed hypoperfusion in the territory of thebilateral anterior and middle cerebral arteries, with left
dominance.
On the 5th day of hospitalization, progressive righthemiparesis occurred and brain computed tomography(CT) showed hemorrhagic infarction in the left anterior
lobe. Hence, argatroban was discontinued and the pa-

tient’s condition was stabilized. Cerebral angiography on
the 15th day of hospitalization (Figs. 1D and E) revealed
left ICA occlusion and no abnormalities in branches ofexternal carotid arteries. No indicators of Moyamoya
disease, fibromuscular dysplasia, dissection or intracranial arteritis were observed. Although cilostazol
therapy (200 mg/day, BID) was initiated to prevent recurrent stroke, the patient showed low fever, impaired
consciousness and progression of bilateral hemiparesis.
MRI showed recurrent infarcts in the right frontal and250

Isolated internal carotid arteritis

DISCUSSION
The present patient experience indicates that arteritis canbe limited to the ICA. In addition, the patient was: i) a
young woman; ii) had wall enhancements in contrast-enhanced MRI; iii) responded to steroid therapy and iv)
was positive for the serotypes HLA-B39, -B51 and -DR4.
In contrast with exiting disease concepts, arteritis
was caused by autoimmune disease in the present case,according to contrast-enhanced MRI findings and theefficacy of steroid therapy. Moreover, although we did
not observe symptoms or organ involvements, autoantibodies indicated autoimmune disease such as systemic
lupus erythematosus and medium- or small-vessel vasculitis, and the lesion was primarily located in bilateral
ICAs. Based on these findings, we suspected TAK or
GCA, both of which are types of large-vessel vasculitis
or Behçet’s disease, which induces lesions in large–
small arteries. We also considered tuberculosis because
a tuberculosis patient reportedly developed infectious
vasculitis with a primary lesion in the ICA.2 However,tuberculosis was not evident in the present case.
Cerebral infarction is an accepted diagnostic marker
of TAK.3 Although intracranial artery lesions are rarecomplications, the presence of such lesions has been observed previously using angiography, and in biopsies.4, 5However, to the best of our knowledge, no reports havedescribed TAK cases with primary lesions at the ICA,
and no reported TAK cases presented with aortic lesions
after follow-up surveillance of ICA lesions that developed during the early stages of disease. The present patient was a young woman and exhibited symptoms thatwere suggestive of TAK, including bilateral blood pressure differences and the presence of HLA-B39. However, the absence of lesions in the aorta or at the proximal
region of the first branch precluded diagnosis of TAK.
Numerous reports describe GCA primarily at the
ICA.6–14 Although onset at a young age is extremely rare,
pathologically demonstrated GCA has been reported inpatients of more than 50 years of age.15–17 However, STA
biopsy examinations can produce false-negative diagnoses, and even after identification of negative biopsies on
one side, only 3 to 10% of biopsies on the contralateralside reportedly result in positive findings.18
Atypical presentations of GCA have been reported
in previous case studies, and the present observations,
including histology of the primary lesion site, symptomatic headaches, augmented ESR and the presence of
HLA-DR4, are indicative of GCA. However, the present
case was not diagnosed with GCA according to diagnostic criteria, because onset occurred at a young age andno temporal artery anomalies or artery biopsy abnormalities were observed.

temporal lobes and left corona radiata suggesting embolic infarction on the 17th day of hospitalization (Fig. 1C).
Impaired consciousness and right hemiparesis improved
after resuming argatroban and edaravone, although thepatient presented with gradually increasing fever, tenderness of bilateral carotid arteries, bilateral headache,odynophagia, arthralgia and an elevated erythrocyte
sedimentation rate (ESR) (97 mm/h) on the 22nd day of
hospitalization. Contrast-enhanced MRI and MRA (Figs.
2B1–B3) revealed wall enhancement in bilateral ICAs
and wall thickening in bilateral ICAs and distal CCAs,
warranting diagnosis of arteritis. Positron emission tomography–computed tomography, 3-dimensional CT angiography, and sonography showed no abnormal lesions
in arterial or venous systems. Moreover, a superficial
temporal artery (STA) biopsy showed only slight intimal
thickening without inflammation, with no evidence ofgiant cell arteritis (GCA). Ophthalmologic and dermatologic consultation detected no abnormalities such as
fundus abnormalities, uveitis, temporal artery distention,oral aphtha and skin lesions., implying particular immunological diseases including Behcet’s disease. Althoughsymptoms stabilized with argatroban therapy during theacute stage, stroke recurred following discontinuation ofthis treatment and initiation of cilostazol therapy. Hence,arteritis was the suspected cause of stroke recurrences,and oral prednisolone therapy (0.6 mg/kg body weight/day, 30 mg/day) was administered on the 36th day of
hospitalization according to the guidelines for GCA and
Takayasu’s arteritis (TAK). Heparin infusions (10,000 U/day) were then initiated and the dose was adjusted basedon activated partial thromboplastin times. Subsequently,
fever, body tenderness and inflammatory markers were
improved after three weeks, and the dose of prednisolone was gradually increased by 2.5-mg increments from
5 mg/week to 20 mg/day, and was then further tapered
by 2.5-mg increments every three weeks to 10 mg/day.
Follow-up MRA showed improved signal intensity in bilateral anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), whereas contrast-enhanced MRI
demonstrated persistent wall enhancement in bilateral
ICAs. The patient showed neurological symptoms, suchas moria, emotional incontinence, grasp reflex, righthemiparesis and left hemiplegia, and was transferred
to a rehabilitation center at 15 weeks after administration. Contrast-enhanced MRI at six months after onset
revealed persistent wall enhancement in bilateral ICAs.
However, no stroke recurrence has been identified with
continuing to take small doses of prednisolone (less than
10 mg/day) during four years of follow-up.

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K. Fukuma et al.
Table 1. Reported cases of non-infectious arteritis predominantly affecting the ICA, without involvement of
intracranial arteries (except ICA and vertebral artery), aortas or proximal aortic branches
Authors (ReferYear
ence number)

Age (years) Diagnosis Antibody Stenosis/ Modality/ Sex
Occlusion

Wilkinson 1
(8)

1972

74/ Male

GCA

ND

Bil. ICA,
Autopsy
Bil. VA

Biopsy STA
PSL(+), Autopsy

Died on the day ofadmission/ND

Wilkinson 2
(8)

1972

80/ Male

GCA

ND

Bil. ICA, Autopsy
Bil. VA

Biopsy STA PSL(+), Autopsy

Died 6 days after
admission/ ND

Wilkinson 3
(8)

1972

79/ Male

GCA

ND

Bil. ICA, Autopsy
Bil. VA

Biopsy STA PSL(+), Autopsy

Died/ND

Wilkinson 4
(8)

1972

75/ Male

GCA

ND

Bil. ICA,
Bil. VA Autopsy

Autopsy
STA (+)

Died 6 days after
admission/ND

Cull
(6)

1979

46/ Female GCA

Negative Rt. ICA

Angiography

Biopsy STA
PSL(+)

Alive/PSL

Howard(9)

1984

65/ Female GCA

Negative Bil. ICA

Autopsy

Autopsy
ICA (+)

Unknown

Died 33 days after
admission/ND

Bogousslavsky 1985
(10)60/ Male

GCA

ND

Bil. ICA

Autopsy

Autopsy
ICA (+)

Dexamethasone

Died 12 days after
admission/ND

Vincent(7)

1986

60/ Female GCA

ND

Bil. ICA

Biopsy STA
Angiography (+)
PSL, Aspirin Alive 3 Y later/PSL

Gonzalez
(11)

1998

65/ Female GCA

aCL Ab

Lt. ICASTA PSL, Aspirin Alive 5 Y later/AsAngiography Biopsy
pirin
(+)

Thielen(12)

1998

69/ Female GCA

Antinu- Bil. ICA, Angiography Biopsy STA Steroid
clear Ab Lt. VA(+)

Alive 15M later/ND

Noguchi(23)

1999

76/ Female GCA

Bil. ICA,
Biopsy STA Steroid, AP,
Negative Bil. ECA Angiography (+)
AC

Alive 2 Y later/
PSL, AP

Takekawa
(24)

2008

56/ Male

GCA

ND

Bil. ICA

Angiography Unknown

Improvement of
MRA findings/ND

Solans-Laque
(13)

2008

90/ Male

GCA

ND

Lt. ICA

US/CTA/MRA

Lu-Emerson1
(14)

2010

64/ Female GCA

ND

Stepwise decline
Bil. ICA, Angiography Biopsy STA Steroid,
Aspirin,
CPA,
and died 2 Y later/
Bil. VA(+), Autopsy Rituximab
ND

Lu-Emerson 2 2010(14)

65/ Male

ND

Logar
(21)

1994

32/ Female GPA

PR3ANCA

Schmidt(22)

2001

37/ Male

PR3ANCA

Present case

GCA

GPA

Pathology

Treatment

PSL

PSL

Biopsy STA mPSL, PSL,(+)
AP, AC

Prognosis/Prevention

Alive 40 M later/
PSL, APAP, intraar- Stepwise decline
Bil. ICA, Angiography Biopsy STA terial stentdied 7 M later/
placement , andBil. VA(+)
ND
Steroid, CPA
BiopsymPSL, PSL, Improvement of
Bil. ICA Angiography Kidney (+) CPA
laboratory Findings/ND
Improvement of
MRA,
BiopsyPSL, CPA,
Lt. ICA US,
laboratory findings/
Angiography Kidney (+) Aspirin
ND

38/ Female Undeter- Negative Bil. ICA
mined

US, MRA,
Biopsy STA AP, AC, PSL Alive 3 Y later/
Angiography (−)
PSL, AP

Autopsy (+)” or “Biopsy (+)” indicates the presence of pathological findings which support the diagnosis: giant cell, granulomas or others.
Ab, antibody; AC, anti-coagulant drug; aCL, anticardiolipin; AP, anti-platelet drug; Bil, bilateral; CPA, cyclophosphamide; CTA, computed tomography angiography; ECA, external carotid artery; GCA, giant cell arteritis; GPA, granulomatosis with polyangiitis; ICA, internal
carotid artery; Lt, left; M, months; mPSL, methylprednisolone; MRA, magnetic resonance angiography; ND, no description; PR3-ANCA, proteinase 3-antineutrophil cytoplasmic antibody; PSL, prednisolone; Rt, right; STA, superficial temporal artery; US, ultrasonography; VA, vertebral artery; W, weeks; Y, years.

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Clinical hallmarks of GCA, including the presentation of vasculitis of the temporal artery and the intracranial artery (cranial GCA), and arteritis of the aorta
or first branch [large-vessel GCA (LV-GCA)], have been
specified as clinical presentations of classical temporalarteritis (TA).19 Moreover, LV-GCA is observed in 10 to
15% of all GCA cases, and reportedly presents as lesions
in the aorta, carotid arteries and subclavian arteries in
the absence of TA in biopsies. In addition, both GCA
and TAK are characterized by histopathological formations of granulomatous lesions, and TAK and LV-GCA
exhibit similarities in affected vessel morphology, histopathology and clinical symptoms, suggesting inclusion
within the same disease spectrum.20 However, althoughtemporal artery biopsy resulted in negative findings in
the present case, the clinical spectrum for the affectedvessel did not coincide with that of LV-GCA.
Lesions of any size can occur in Behçet’s disease
and predominantly affect veins rather than arteries. Typically, vasculo-Behçet’s disease occurs several years after the onset of Behçet’s disease. The present patient was
HLA-B51-positive and the affected vessel was somewhat
consistent with Behçet’s disease. However, because
whole-body symptoms of Behçet’s disease and veinlesions were not observed, Behçet’s disease was considered unlikely. The present patient was diagnosed with aform of non-infectious vasculitis with atypical clinicalpresentation, and after consideration of large-vessel vasculitis diseases TAK and GCA, Behçet’s disease criteria
were examined. However, the present patient conditions
were not sufficiently consistent with any of these diseases, and the patient was diagnosed as having a type ofvasculitis with primary lesions in the ICA that was on
the disease spectrum of TAK and GCA, but with different clinical presentation.
In comparison with the 17 previously reported casesof ICA arteritis,6–14, 21, 22 distinguishing characteristicsof the present female case included relative youth, wallenhancement on contrast-enhanced MRI, therapeutic
effects of steroids and the presence of HLA serotypes(HLA-B39, -B51 and -DR4) (Table 1). Fifteen of thesecases were diagnosed with GCA6–14 and the remaining
2 cases had granulomatosis with polyangiitis (GPA).21, 22
Although these two GPA cases had onset at a young age,
renal lesions and proteinase 3-antineutrophil cytoplasmic
antibody (PR3-ANCA) expression were not consistent
with the present case. Another previous case describeda patient with autoimmune disease who developed ICA
lesions25; however, the distributions of vascular lesions
were dissimilar to those in the present case. Thus, noneof the 17 cases presented the clinical characteristics thatwere consistent with the present case.

The relative youth and gender of the present casewas nearly in accordance with one GCA case6 and one
GPA case.21 However, wall enhancement on contrast-enhanced MRI was not described for any of the previously
reported 17 cases, despite its correlation with serum
markers of disease activity, and its utility for assessments of mural inflammation in GCA and TAK.26–28
Although enhanced MRI facilitates primary diagnosis
of TA, a recent study suggested that assessments of wallenhancement are limited indicators of disease activity inlong-term follow-up of TA.29 In agreement, contrast-enhanced T1-weighted MRI assessments of the ICA wereof diagnostic value in the present case, with wall enhancement on admission not only from a retrospective
viewpoint (Figs. 2A1 and A2) but also after symptoms
and inflammatory markers were improved by prednisolone therapy (Figs. 2C1 and C2). Hence, persistent vessel
wall enhancement may not always indicate increased
disease activity.
Steroids, immunosuppressants or antithrombotic
agents are common as acute and preventive therapy forclinical vasculitis,6–14, 21, 22 and are used in combination
for cases with favorable prognoses. Combined prednisolone and antithrombotic treatments were successful asacute therapy in the present case, and subsequent lowdose prednisolone and antiplatelet therapy prevented
further strokes. Hence, combination immune and antithrombotic therapy should be considered for arteritis
involving cervical and cerebral arteries.
Finally, onset of vasculitis has been associated withenvironmental and genetic factors and various correlations between vasculitis and HLA alleles have been
elucidated. Specifically, TAK is reportedly associatedwith HLA-B52 (B*5201) and -B39 (B*3902),30 whereas
Behçet’s disease is associated with HLA-B51 (B*5101),31
GCA is associated with HLA-DR4 (DRB1*04), cranial
GCA is associated with DRB1*0401, and large-vessel
GCA is associated with DRB1*0404.19 In the present
case, the HLA serotypes HLA-B39, -B51 and -DR4
were identified without HLA allele typing, unprecedentedly suggesting Behçet’s disease, TAK and GCA, respectively. In contrast, no HLA serotypes or alleles werereported in the aforementioned 17 cases.6–14, 21, 22 Thus,
further accumulations of HLA typing in similar cases
are required to evaluate these associations.
In summary, the present case was distinguishedfrom previous cases by relative youth and contrast-enhanced MRI and HLA characteristics.
Here we report a rare evidence of arteritis limited to
ICA in an unprecedented case of a young woman withwall enhancement on contrast-enhanced MRI, therapeutic effects of steroids, and HLA serotypes HLA-B39,
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K. Fukuma et al.

-B51 and -DR4. These disease characteristics were not inaccordance with existing diagnostic criteria, warranting
consideration of a novel “isolated internal carotid arteritis” disease concept.

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The authors declare no conflict of interest.

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