© 1989 S. Kargcr AG, Basel
0014-3022/89/0295-026652.75/0

Eur Neurol 1989;29:266-268

Giant-Cell Arteritis Presenting with Ipsilateral Hemiplegia and
Lateral Medullary Syndrome
Antonio Colladoa , Joan Santamaría a , Teresa Ribaltab, M. Cinta Cid3, J.D. Cañete3, Eduardo Tolosa3
Departments of “Neurology and bPathology, Hospital Clínico y Provincial, Barcelona, Spain

Key Words. Giant-cell arteritis • Ipsilateral hemiplegia • Lateral medullary syndrome • Opalski’s syndrome •
Wallenberger’s syndrome
Abstract. We describe the clinical and anatomopathological findings in an 85-year-old male presenting with a
lateral medullary syndrome and ipsilateral hemiplegia (Opalski’s syndrome) due to giant-cell arteritis of the vertebral
artery. Other common associated symptoms of the disease were absent, and the erythrocyte sedimentation rate was
only of 30 mm/h. This case emphasizes that limited intracranial giant-cell arteritis may present rarely as a stroke.

Case Report
The patient was an 85-year-old male, heavy smoker (140
packs/year) and drinker (300 g alcohol/day), with several years his­
tory of bilateral deafness. Four days before his present admission to
the hospital, he began to experience lack of coordination in the right
hand, dizziness, nausea, vomiting, hiccoughs and difficult walk­
ing.
One year previously he had a transient episode of visual blurring
and unsteady gait, from which he had completely recovered. On
admission his blood pressure was 160/90 and his heart rate 60/min.
He was alert with bilateral deafness. The general examination was
normal. Temporal arteries were normally felt. He had no meningeal
signs. Hypoalgesia in the right half of the face and left half of the

body was found with severe dysmetria of the right arm, and moder­
ate right-side hemiparesia, which worsened in the subsequent days.
A right Babinski sign was present.
The blood count revealed a hemoglobin of 13.1 g/dl, hematocrit
of 38% with normal white blood cell, differential and platelet
counts. The erythrocyte sedimentation rate was 30 mm/h. Blood
chemistry was normal. A cerebral computed-tomographic scan
showed an old left temporo-occipital infarct with slight ventricular
dilatation and cortical atrophy.
The patient became progressively worse during the days follow­
ing his admission, with impairment of his consciousness level, fever
and respiratory distress as a consequence of pneumonia in the right
lower lobe resulting in his subsequent death. A partial necropsy of
the cerebrum was done.

Anatomopathological Description
The encephalon fixed in formol weighed 1,400 g. An
enlargement and flattening of the gyri could be seen, as
well as a moderate atheromatosis of the vessels of the
circle of Willis. The left vertebral artery was larger than
the right. The bore of the right vertebral artery was found
to be totally obstructed in the final 2 cm by a thrombus

Downloaded by:
Nagoya University
133.6.82.173 - 1/15/2019 8:36:23 AM

The prevalence of stroke due to giant-cell arteritis
(GCA) of the head or neck vessels is rare and has been
pathologically documented in less than 3% of the pa­
tients [1-4], very few cases having it as the initial mani­
festation of the disease. We report here a patient who
presented with a lateral medullary syndrome associated
with homolateral hemiplegia (Opalski’s syndrome) [5-7]
as the initial manifestation of a GCA of the right verte­
bral artery.

Giant Cell Arteritis Presenting with Opalski's Syndrome

267

which extended into the ostium of the posteroinferior
cerebellar artery (PICA). A recent ischemic infarct was
observed in the right lateral territory of the lower medul­
la, which extended from the upper medulla to the pyra­
midal decussation (fig. 1). In the cerebellum a recent
infarct of the right posteroinferior region was observed,
with tonsillar herniation. In the cerebrum there was a
moderate ventricular dilatation and a cystified infarct of
3 X 2 X 2 cm in the left calcarine area. Microscopic
study of the right vertebral artery showed a luminal
thrombosis with a transmural vasculitis with lymphoplasmocytic infiltration and multinucleated giant cells
closely associated with the internal elastic lamina (fig. 2).
No similar inflammatory changes were observed in other
basal arteries or in the leptomeningeal and intraparenchymatous vessels.

Discussion

Fig. I. Transversal section of the bulb, in the region of the
pyramidal decussation, where a recent infarct of the right lateral
territory may be observed.
Fig. 2. Right vertebral artery. The arterial wall shows a chronic
transversal inflammatory infiltration with multinucleate giant cells
aligned in the region of the internal elastic lamina, which is
destroyed. In the upper third of the image a part of the organizing
luminal thrombosis can be seen.

which the intracranial vessels are affected is directly
related to the prognosis of the disease [1],
Our case then is exceptional in as much as it shows
the clinical presentation of GCA as ipsilateral hemiple­
gia and Wallenberg’s syndrome (previously reported
with atheromatosis [7]), and without other accompa­
nying symptoms of the disease, making the diagnosis
very difficult.

Downloaded by:
Nagoya University
133.6.82.173 - 1/15/2019 8:36:23 AM

The frequency with which the vertebral and internal
carotid arteries are affected in GCA is not known. Nev­
ertheless, there are studies [2, 8] which show that these
arteries are affected in GCA and may be asymptomatic,
probably due to collateral supply from the circle of Willis
[2]. The vessels affected in GCA are usually extradural,
without involvement of other intracranial vessels, except
for the first 5 mm of the intradural vertebral artery [8].
In spite of this it is unusual for GCA to present initially
as a stroke [9], and in most of the reported cases the
stroke appeared after the vasculitis had been diagnosed.
The more common neurological signs reported are: lat­
eral medullary syndrome [10], hemianopsia [11], cortical
blindness [12], hemiparesis and dementia [13]. In our
patient in arteritic thrombosis of the distal vertebral
artery, the lateral medullary branches and the ostium of
the PICA produced a lateral medullar and cerebellar
infarction, with caudal extension into the cervical cord
and involvement of ipsilateral corticospinal fibers
beyond the pyramidal decussation. Although we do not
know if the temporal arteries were affected, we think that
our patient presented a granulomatous arteritis of tem­
poral arteritis type, given that it did not fulfill the clinical
characteristics of the distribution of lesions which occur
in other granulomatous GCA such as Takayasu’s arteri­
tis [14], or granulomatous arteritis restricted to the cen­
tral nervous system [15-17],
The importance of early diagnosis in these cases is
evident because of the therapeutic possibilities of corticoids in GCA, and also as it seems that the extent to

Collado/Santamaria/Ribalta/Cid/Canete/Tolosa

References
1 Graham E, Holland A, Avery A, et al: Prognosis in giant-cell
arteritis. Br Med J 1981:282:269-271.
2 Meadows SP: Temporal or giant cell arteritis. Proc R Soc Med
1966;59:329-333.
3 Howard GF II, Ho SV, Kim KS, et al: Bilateral carotid artery
occlusion resulting from giant cell arteritis. Ann Neurol 1984; 15:
204-207.
4 Save-Soderbergh J, Malmvall BE, Andersson R, et al: Giant cell
arteritis as a cause of death: Report of nine cases. JAMA 1986;
255:493-496.
5 Opalski A: Syndrome partiel de l’artère vertébro-spinale posté­
rieure. Paris Méd 1946;1:214-220.
6 Osetowska E, Krasnickz Z: Sur le syndrome sous-bulbaire
d'Opalski avec une étude anatomique (Contributions aux as­
pects paradoxaux du syndrome de Wallenberg). Rev Neurol
1961;104:310-320.
7 Dhamoon SK. Ikbal J, Collins GH: Ipsilateral hemiplegia and
the Wallenberg syndrome. Arch Neurol 1984;41:179-180.
8 Wilkinson MS, Russell RWR: Arteries of the head and neck in
giant cell arteritis. Arch Neurol 1972;27:378-391.
9 Small P: Giant cell arteritis presenting as bilateral stroke. Arthri­
tis Rheum 1984;27:819-821.
10 Goodwing JA: Temporal arteritis; in Vinken PJ, Bruyn GW
(eds): Handbook of Clinical Neurology. New York, Elsevier/North-Holland, 1980, vol 39, pp 313-342.

11 Monteiro MLR, Coppeto JR, Greco P: Giant cell arteritis of
posterior cerebral circulation presenting with ataxia and oph­
thalmoplegia. Arch Ophthalmol 1984;102:407-409.
12 Chisholm IH: Cortical blindness in cranial arteritis. Br J Oph­
thalmol 1975;59:332-333.
13 Hollenshorst RW. Brown JR, Wagener HP. et al: Neurologic
aspects of temporal arteritis. Neurology 1959;14:490-498.
14 Leu MJ: Die unspezifische Aorto-Arteritis (Takayasu Erkran­
kung): Histologische Untersuchung an 15 Fällen. Virchows Arch
[A] 1976;370:239-250.
15 Jellinger K: Giant cell granulomatous angiitis of the central ner­
vous system. Neurology 1977;213:175-190.
16 Enzmann D, Scott WR: Intracranea! involvement of giant cell
arteritis. Neurology 1977;220:794-797.
17 Nurick S, Blackwood W, Mair WG: Giant cell granulomatous
angiitis of the central nervous system. Brain 1972;95:133-142.

Received: June 27, 1988
Accepted: November 22, 1988
Dr. Antonio Collado
Servicio de Reumatologia
Hospital Clinico y Provincial
C/ Villarroel 170
E-08036 Barcelona (Spain)

Downloaded by:
Nagoya University
133.6.82.173 - 1/15/2019 8:36:23 AM

268