Atypical clinical presentation in an anterior cerebral artery territory infarction - Case report

Abstract

Infarction of the anterior cerebral artery (ACA) territory accounts for only 0.3-4.4% of cerebral infarctions. We report an unusually prolonged progressing stroke of the ACA in a 58-year-old patient who had his diagnosis based on neuroimaging investigation and in anatomopathological exclusion of neoplastic disorder.

Full text

revisão
Rev Neurocienc 2008:in press
Atypical clinical presentation in an
anterior cerebral artery territory
infarction - case report
Apresentação clínica atípica em infarto no território de artéria cerebral anterior – relato de caso
Leopoldo Antônio Pires1, Marcelo Maroco Cruzeiro2, Thiago
Cardoso Vale3, Luciana de Souza Nogueira4, Gláucio Mendes
Franco5, Sérgio Franca de Souza6
RESUMO
Infarto no território da artéria cerebral anterior (ACA) ocorre em
apenas 0.3-4.4% dos infartos cerebrais. Relata-se um infarto pro-
gressivo, prolongado e incomum da ACA em paciente de 58 anos
que teve seu diagnóstico baseado em investigação neurorradio-
lógica e na exclusão anátomo-patológica de desordem neoplásica.
Unitermos. Infarto Cerebral, Artéria Cerebral Anterior, Trombose.
Citation. Pires LA, Cruzeiro MM, Vale TC, Nogueira LS,
Franco GM, Souza SF. Apresentação clínica atípica em infar-
to no território de artéria cerebral anterior – relato de caso.
SUMMARY
Infarction of the anterior cerebral artery (ACA) territory ac-
counts for only 0.3-4.4% of cerebral infarctions. We report an
unusually prolonged progressing stroke of the ACA in a 58-year-
old patient who had his diagnosis based on neuroimaging in-
vestigation and in anatomopathological exclusion of neoplastic
disorder.
Keywords. Cerebral Infarction, Anterior Cerebral Artery,
Thrombosis.
Citação. Pires LA, Cruzeiro MM, Vale TC, Nogueira LS, Franco
GM, Souza SF. Atypical clinical presentation in an anterior ce-
rebral artery territory infarction - case report.
Rev Neurocienc 2009;17(3): 279-82
relato de caso
Neurology and Neurosurgery, Department of Internal Medicine at
University Hospital of Federal University of Juiz de Fora, Juiz de
Fora-MG, Brazil.
1.Msc (Universidade Federal de São Paulo - UNIFESP), Neuro-
logist, MD. Head and Lecturer of Neurology in Department of
Internal Medicine at University Hospital of Federal University of
Juiz de Fora, Juiz de Fora-MG, Brazil.
2.PhD. (Fluminense Federal University - UFF), Neurologist and
Neurophysiologist, MD. Assistant Lecturer in Department of Inter-
nal Medicine at University Hospital of Federal University of Juiz de
Fora, Juiz de Fora-MG, Brazil.
3.Medical resident in Neurology at the Clinics Hospital, Federal
University of Minas Gerais (UFMG), Belo Horizonte-MG, Brazil.
4.Neurologist, MD. Former Medical Resident in Department of
Internal Medicine at University Hospital of Federal University of
Juiz de Fora, Juiz de For a-MG, Brazil.
5.Msc (Rio de Janeiro Federal University - UFRJ); PhD (Fluminen-
se Federal University - UFF). Neurologist and Neurophysiologist,
MD. Former Head and Senior Lecturer of Neurology in Depart-
ment of Internal Medicine at University Hospital of Federal Univer-
sity of Juiz de Fora, Juiz de For a-MG, Brazil.
6.Neurosurgeon, MD. Lecturer of Neurosurgery at University Hos-
pital of Federal University of Juiz de Fora, Juiz de For a-MG, Brazil.
Correspondence address:
Leopoldo A Pires
R. Dr José P Teixeira, 395
CEP 36021-540, Juiz de Fora-MG, Brazil
Email: leopoldo@artnet.com.br
Recebido em: 15/05/2007
Revisado em: 16/05/2007 a 31/07/2008
Aceito em: 01/08/2008
Conito de interesses: não
279

revisão
Rev Neurocienc 2008:in press
INTRODUCTION
Infarction of the anterior cerebral artery (ACA)
territory accounts for only 0.3-4.4% of cerebral infarc-
tions reported1-4. In Kumral et al´s large stroke regis-
try5, patients with ACA infarction represented 1.3%
of 3705 patients with ischemic stroke. According to
Bogousslavsky et al6, 63% of ACA territory infarctions
result from cardiogenic embolism or artery-to-artery
embolism. Gacs et al7
reported other possible causes:
contralateral or ipsilateral occlusions of the internal
carotid artery (ICA), distal extensions of ICA throm-
bosis, and local thrombosis caused by vasculitis.
In recent years, single case reports and case
series have focused on specic symptoms and syn-
dromes resulting from the involvement of dierent
territories of the frontal lobe, thus broadening our
knowledge of the clinical manifestations of ACA
infarctions5. In a prospective magnetic resonance
imaging (MRI) study conducted by Kumral et al5,
the rst MRI-based study on the clinical ndings
of ACA infarction, the main risk factors for ACA
infarctions were hypertension in 58% of patients,
diabetes mellitus in 29%, hypercholesterolemia in
25%, cigarette smoking in 19%, atrial brillation in
19%, and myocardial infarction in 6%. According
to their clinico-radiologic analysis, there were three
main clinical patterns depending on the lesion’s
side: left-sided infarction consisting of mutism,
transcortical motor aphasia, and hemiparesis with
lower limb predominance; right-sided infarction
accompanied by acute confusional state, motor he-
mineglect and hemiparesis; bilateral infarction pre-
senting with akinetic mutism, severe sphincter dys-
function and progressing to functional dependence5.
We report an unusually prolonged progressing
stroke of the ACA in a 58-year-old patient who had his
diagnosis based on neuroimaging investigation and in
anatomopathological exclusion of neoplastic disorder.
.
CASE REPORT
JMS, a 58-year-old right-handed man (HU-
SAME-297918) with hypertension, depression,
lower limb chronic arterial insuciency, peptic
ulcer complicated with perforation, erosive oe-
sophagitis, gastroesophageal reux disease, and
various episodes of pneumonia was brought to
the Emergency Room with a history of progres-
sively worsening weakness of the limbs, beha-
vioural disturbance, labile aect and apathy.
The rst episode of weakness occurred three
years before and was exclusively due to right lo-
wer limb paresis. A second similar episode occur-
red three months before admission and led to an
ascending paresis over the following months with
progressive involvement of the left lower limb.
Mild behavioural and cognitive disturbances as
well as impairment of the upper limbs were noti-
ced in the following weeks before actual hospita-
lization. This impairment was characterized by an
ideomotor apraxia, which enabled the patient to
perform in-phase alternating hand movements
well, but he had moderate diculties perfor-
ming out-of-phase alternating hand movements,
particularly if vision of the hands was occluded.
Brain MRI investigation of the second epi-
sode showed a left frontal hypointense lesion on
T1-weighted images. The lesion was hyperintense
with contrast enhancement on T2-weighted and
on uid-attenuated in version recovery (FLAIR)
images. The subsequent workup included caro-
tid and vertebral artery Doppler ultrasonogra-
phy, electrocardiography, and transthoracic and
transesophageal echocardiography with bubble
study, none of which showed any source of em-
bolism. Laboratory tests included normal levels of
antithrombin III activity, protein C and S antigen,
plasma homocysteine, antiphospholipid antibody
panel, anti-hepatitis C virus antibodies, antinuclear
antibody, anticardiolipin antibody IgG, anticardio-
lipin antibody IgM, vitamin B12, lupus anticoagu-
lant, Factor V Leiden, protrombin gene mutation,
activated protein C resistance and partial throm-
boplastin time, as well as a normal complete blood
count and routine blood chemistry. Tests for HIV I
+ II antibodies were negative. There was no fami-
ly history of haematologic or neurologic disease.
A new brain MRI investigation was perfor-
med three months after the second episode of
weakness and it showed progression of the pre-
vious lesions with involvement of the parietal lobe
(Figure 1). Cerebral arteriography revealed partial
occlusion of the left ACA (Figure 2). Conservative
treatment was proposed by the neurosurgery sta
after in-loco biopsy ruled out a neoplastic disorder.
At the time of admission, his physical exami-
nation was normal, except for Raynaud´s phenome-
non in his right lower limb. Neurologic examination
revealed upper motor neuron disease signs aec-
ting predominantly the right limbs. Neurophsyco-
logical standard instruments revealed moderate
relato de caso
Rev Neurocienc 2009;17(3): 279-82
280

revisão
Rev Neurocienc 2008:in press
Figure 1. Brain MRI showing a left fronto-parietal lesion with progres-
sion over a two-year period.
cognitive impairment. The patient developed a dy-
sexecutive syndrome caracterized by impairments
in planning and memory as well as a tendency to
confabulate. Poor insights into his problems and
an inability to adopt systematic strategies when
attempting to solve problems were also observed.
Anticoagulant therapy was established and
the patient remained cognitively impaired and
with a right lower limb sequela. This case report
was performed with the approval of the Ethics
Committee of the University Hospital of the Fede-
ral University of Juiz de Fora (Minas Gerais), Brasil.
DISCUSSION
According to Ay et al4, clinical progression
in patients with ischemic stroke may occur up to
seven days after onset. It can be territorial, a gra-
dual progression of symptoms attributable to
the involvement of a single arterial territory, or
non-territorial, a deterioration in consciousness
or the appearance of new signs following the in-
volvement of dierent vascular regions5. Territo-
rial progression spanning a period of months is
quite uncommon, particularly at the ACA territory.
The patient presented progressive right
hemiparesis with restrictions of daily activities as
well as bilateral lesions of frontal supplementa-
ry motor areas and adjacent medial frontal lobe
structures such as the anterior cingulate gyrus.
This case vignette is interesting because its cli-
nical presentation is similar to tumoral lesion
progression. Oddly enough, the patient did not
developed urinary incontinence, despite having
such a progressive and extensive territorial stroke.
Unusually prolonged infarction in
the ACA territory, unrelated to subara-
Figure 2: Cerebral conventional angiography yielding lack of perfusion
in the left ACA territory (top and bottom left) and a normal perfusion in
the right ACA territory (top and bottom right). The long and short arro-
ws point out the pericallosal and cingulate sulcus arteries, respectively.
chnoid hemorrhage, surgery, or trauma, has
been reported rarely. Ay et al4 have a very si-
milar case of unusually prolonged progressing
stroke, but without contralateral lobe impairment.
The current report shows that, in occasio-
nal patients, clinical worsening is not limited to
a matter of hours or days, but may last weeks,
which prompt a clinical suspicion of tumoral le-
sion. Reasons behind progressive strokes are not
available in literature to date. Above and beyond
all other consideration, timely recognition of pa-
tients under risk of territorial progression is es-
sential. The use of markers of tissue injury and
tissue perfusion might optimize future therapeu-
tic eorts to halt progression in these patients.
CONCLUSION
This case vignette describes a patient who-
se symptoms gradually progressed over two ye-
ars, highlighting this unusual manifestation of
vascular thrombosis in the ACA territory. What
makes it more intriguing is the absence of aneu-
rysm, which would be responsible for the throm-
bosis. Medical treatment and regular follow-up
were carried out with relative favorable outcome.
ACKNOWLEDGMENT
We are indebted to Ricardo Rocha Bastos,
lecturer in Department of Internal Medicine in
University Hospital of Federal University of Juiz de
Fora, Juiz de Fora (MG), Brazil, for his critical review
of the manuscript and many helpful suggestions.
relato de caso
Rev Neurocienc 2009;17(3): 279-82
281

revisão
Rev Neurocienc 2008:in press
REFERENCES
1.Orlandi G, Moretti P, Fioretti C, Puglioli M, Collavoli P, Murri L.
Bilateral medial frontal infarction in a case of azygous anterior
cerebral artery stenosis. Ital J Neurol Sci 1998;19:106-8.
2.Yamaguchi K, Uchino A, Sawada A, Takase Y, Kuroda Y, Kudo
S. Bilateral Anterior Cerebral Artery Territory Infarction Asso-
ciated with Unilateral Hypoplasia of the A1 Segment: Report
of Two Cases. Radiat Med 2004;22:422-5.
3 .Klatka LA, Depper MH, Marini AM. Infarction in the territory
of the anterior cerebral artery. Neurology 1999;52:1519.
4.Ay H, Uluc K, Arsava EM, Saatci I, Saribas O. Unusually prolon-
ged progressing stroke: an expanding anterior cerebral artery
infarction. Cerebrovasc Dis 2002;13:64-6.
282
relato de caso
Rev Neurocienc 2009;17(3): 279-82
5.Kumral E, Bayulkem G, Evyapan D, Yunten N. Spectrum of
anterior cerebral artery territory infarction: clinical and MRI
ndings. Eur J Neurol 2002;9:615–24.
6.Bogousslavsky J, Regli F. Anterior cerebral artery territory in-
farction in the Lausanne Stroke Registry. Clinical and etiologic
patterns. Arch Neurol 1990;47:144-50.
7.Gacs G, Fox AJ, Barnett HJ, Vinuela F. Occurrence and me-
chanisms of occlusion of the anterior cerebral artery. Stroke
1983;14:952-9.