Case Report

Malignant Middle Cerebral Artery Infarction Resulting
from Dissection of Middle Cerebral Artery
Kenichi Sakuta, MD,* Renpei Sengoku, MD,† Hidetaka Mitsumura, MD,*
Yu Kono, MD,* Issei Kan, MD,‡ Shogo Kaku, MD,‡ and Yasuyuki Iguchi, MD*

A 30-year-old man presented with headache, left hemiparesis, and uncal herniation.
Magnetic resonance imaging showed acute infarction in the right middle cerebral
artery (MCA). Lifesaving craniotomy proceeded because of the uncal herniation.
Assessment of brain tissue led to a diagnosis of MCA dissection. His skin biopsy
showed ultrastructural abnormalities of dermal connective tissue. This is the unique
case that dissection was diagnosed from both a biopsy specimen of the MCA and the
dermal connective tissue. Key Words: Middle cerebral artery—dissection—
malignant middle cerebral artery infarction—skin biopsy—acute ischemic stroke.
Ó 2015 by National Stroke Association

Arterial dissection is relatively rare in the anterior
compared with the posterior circulation, and middle cerebral artery dissection (MCAD) is exceptional, with only
a few cases having been reported,1-4 particularly in
Japanese.5 Herein, we describe a Japanese patient with
rapidly progressive ischemic stroke caused by right
MCAD who underwent craniotomy for decompression.
Neuropathologic findings revealed right MCAD.

Case Report
A 30-year-old man with moderate dyslipidemia was
admitted to our hospital with left-hand clumsiness. Two
days before admission, he developed a headache with
From the *Department of Neurology, The Jikei University School of
Medicine, Tokyo; †Department of Neurology, The Tokyo Metropolitan Geriatric Hospital, Tokyo; and ‡Department of Neurosurgery,
The Jikei University School of Medicine, Tokyo, Japan.
Received October 17, 2014; revision received February 26, 2015;
accepted April 3, 2015.
There are no disclosures.
Address correspondence to Kenichi Sakuta, MD, Department
of Neurology, The Jikei University School of Medicine, 3-25-8
Nishi-shimbashi, Minato-ku, Tokyo 105-8461, Japan. E-mail:
kenichisakuta@yahoo.co.jp.
1052-3057/$ - see front matter
Ó 2015 by National Stroke Association
http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2015.04.008

drug resistance. He did not have history of smoking,
problem alcohol drinking, or drug abuse. He had no
familial history especially in collagen diseases. Physical
examination did not reveal hyperextensible skin or moles.
Neurologic examination revealed drowsiness, right conjugate deviation of the eyes, left hemispatial neglect,
and left hemiparesis including the left side of the face.
The score on the National Institutes of Health Stroke Scale
was 11. Antinuclear antibody and autoantibodies were
negative, and alpha 1-antitrypsin and total homocysteine
levels were normal. He did not have mutation in COL3A1.
Brain magnetic resonance (MR) with diffusionweighted imaging immediately after admission showed
focal acute infarction in the right middle cerebral artery
(MCA) area (Fig 1, A,E), and MR angiography (MRA)
showed stenosis in the top of right internal carotid artery
and in the right MCA M1 and M2 segments. No embolic
sources were detected by transthoracic echocardiography
or conventional carotid ultrasonography.
His clumsiness gradually worsened after admission. On
hospital day 4, his left extremities became flaccid, and the
headache in his right temporal region continued. Brain
MR imaging and MRA showed enlargement of the infarction in diffusion-weighted imaging, and the right internal
carotid artery was abnormal compared with the left (Fig 1,
B,F). We initially considered a differential diagnosis of
reversible cerebral vasoconstriction syndrome, primary

Journal of Stroke and Cerebrovascular Diseases, Vol. 24, No. 10 (October), 2015: pp e287-e289

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K. SAKUTA ET AL.

Figure
1. Magnetic
resonance
(MR) with diffusion-weighted imaging
(DWI) and MR angiography (MRA)
findings from admission until hospital
day 9. On admission day, DWI shows
acute focal infarction in right middle
cerebral artery (MCA) area (A), and
MRA shows stenosis at top of right
internal carotid artery (ICA) and right
MCA M1 and M2 segments (arrow)
(E). Image on hospital day 4 shows
enlarged infarction in DWI (B), and
that of right compared with left ICA is
abnormal (F). DWI images on hospital
day 6 show enlarged infarction (C),
and right ICA image has worsened
(G). Images on hospital day 9 show
totally occluded right ICA–MCA (D)
and herniation (H).

angiitis of the central nervous system, and arterial dissection. However, intravenous methylprednisolone therapy
did not improve the symptoms, and the infarct region
expanded (Fig 1, C,G). He fell into a coma with anisocoria
and total occlusion of the right ICA–MCA (Fig 1, D,H) on
hospital day 9. We performed a lifesaving craniotomy for
decompression. Biopsy specimens of the right frontotemporal lobe showed dissection at the M2 and M3 segments
of the right MCA (Fig 2, A,B). Neuropathologic findings
revealed accumulated red blood cells above the inter-

nal elastic lamina without collapse of the lamina, and
increasing numbers of endothelial cells toward the lumen,
also indicating arterial dissection. We performed skin
biopsy to investigate the skin collagen abnormalities. Electron microscopy revealed collagen bundles within the
reticular dermis containing fibrils with highly variable,
small diameters compared with normal control (Fig 2,
C,D). Diseases causing arterial dissection were not
evident. He was transferred elsewhere for rehabilitation
on hospital day 65.

Figure 2. Elastica van Gieson (EVG)
stain of middle cerebral artery M3
segment and appearance of collagen
bundles. EVG staining of middle
cerebral artery and M3 segment (A).
Schema of artery (B) shows no
inflammatory-cell invasion. Inner membrane has moved to left (black arrow).
*Red blood cells have accumulated below
internal elastic lamina. Normal collagen
bundles within reticular dermis consist
of densely packed collagen fibrils with
uniform cross sections (C), and white
arrow shows irregular size of fibrils (D).

MALIGNANT MIDDLE CEREBRAL ARTERY INFARCTION

Discussion
The unique point about the situation with this patient is
that dissection was diagnosed from both a biopsy specimen of the MCA and from ultrastructural abnormalities
of dermal connective tissue, whereas most MCAD has
been diagnosed by angiography or at autopsy.1-4
To the best of our knowledge, this is the first description
of finding dermal connective abnormalities in a skin
biopsy of MCAD. Our findings, such as small diameter
fibrils, were in line with those of prior reports in cervicocerebral artery dissection cases having collagen bundles
within the reticular dermis containing several fibrils
with irregular contours and morphometry on skin
biopsies.6-10 Most patients with cervicocerebral artery
dissection accompanied by connective tissue disorders
have subtle clinical manifestations such as hyperextensible skin or capillary fragility. Thus, skin biopsies
might play an important role in confirming a diagnosis
of cervicocerebral artery dissection.9
Acknowledgment: The authors would like to acknowledge Junko Fujigasaki, who interpreted pathologic findings.

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