□ CASE REPORT □

Global Aphasia without Hemiparesis Caused by a Dural
Arteriovenous Fistula
Jumpei Togawa 1, Takekazu Ohi 2 and Satoru Kawarazaki 3

Abstract
A 61-year-old Japanese woman with chronic renal failure suddenly became silent at the end of hemodialysis. On a neurological examination, she was unable to respond to one-step commands, state the names of objects, repeat single words, read words aloud or write her name. Because she exhibited no paralysis of the extremities, we diagnosed her as having global aphasia without hemiparesis (GAWH). Cerebral angiography
showed a dural arteriovenous fistula (AVF) in the left isolated transverse sinus with leptomeningeal venous
drainage (Borden type 3, Lalwani grade 4). This case highlights dural AVF as an etiology of GAWH.
Key words: global aphasia without hemiparesis, dural arteriovenous fistula, venous infarction
(Intern Med 53: 135-138, 2014)
(DOI: 10.2169/internalmedicine.53.1072)

Introduction

Case Report

Global aphasia is usually accompanied by weakness of
the right side of the face and right hemiplegia in righthanded patients (1). Global aphasia without hemiparesis
(GAWH) is a rare syndrome in which these symptoms are
not observed. The pathogenic mechanisms underlying the
development of GAWH are still not fully understood, and it
has been previously reported that cerebral infarction, subarachnoid hemorrhage and brain tumors can cause
GAWH (2-9).
Dural arteriovenous fistulas (AVFs) are abnormal vascular
connections located within the dura matter that can result in
life-threatening brain edema, hemorrhage and venous infarction (10-13). Dural AVFs with retrograde leptomeningeal venous drainage are regarded as high-risk lesions having the
potential to cause hemorrhage and serious neurological deficits (10-13).
We herein report a case of GAWH caused by a dural
AVF. To our knowledge, this is the first clinical report of
GAWH caused by a dural AVF.

A 61-year-old Japanese woman was transferred to our
hospital after suddenly becoming silent at the end of hemodialysis.
She had a medical history of hypertension, diabetes mellitus, hypothyroidism, chronic renal failure and early Alzheimer’s disease and exhibited only a mild disturbance of
memory with no problems engaging in ordinary conversation. She had no history of either drinking or smoking.
On a neurological examination, the patient’s spontaneous
speech was markedly impaired; she said only the Japanese
word for “ouch!” when her skin was pinched and spoke no
other words spontaneously. She was unable to repeat single
words, respond to one-step commands, read single words
aloud or write single words or her name. She exhibited nonfluent aphasia without comprehension. Because she had no
paralysis, we diagnosed her as having GAWH.
The patient’s complete blood cell count and blood chemistry parameters were within the normal ranges, except for
moderate anemia and renal failure. A lumbar puncture was
performed, and the white blood cell count, protein level and
glucose level were found to be normal. No Herpes simplex
virus DNA was detected in the cerebrospinal fluid (CSF).

１

Department of Neurology, Otsu Red Cross Hospital, Japan, ２Department of Neurology, Kyoto Hakuaikai Hospital, Japan and ３Department of
Neurosurgery, National Hospital Organization Kyoto Medical Center, Japan
Received for publication May 28, 2013; Accepted for publication August 22, 2013
Correspondence to Dr. Jumpei Togawa, togawa-kyt@umin.org

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DOI: 10.2169/internalmedicine.53.1072

Figure 1. Head CT (A), brain MRI (B, C) and MRA (D) performed on admission, IMP-SPECT of
the brain (E) performed two days after admission and brain MRI (F, G) performed three weeks after
admission. A: Plain head CT performed on admission revealed a hemorrhage in the left lateral cerebral ventricle (arrow). B: DWI showed HISA in the left temporal lobe (arrows). C: The left temporal
lobe exhibited iso or low intensity signals on ADC mapping (arrows). D: MRA showed intact cerebral
arteries. E: IMP-SPECT performed two days after admission demonstrated hyperperfusion in the
left temporal lobe (arrows). F: T1-weighted MRI performed three weeks after admission demonstrated laminar necrosis in the left temporal lobe (arrows). G: The precentral gyrus was spared.

Carotid artery ultrasonography and an echocardiogram
and electrocardiogram revealed no abnormal findings. Electroencephalography showed no paroxysmal activity.
Head computed tomography (CT) disclosed a hemorrhage
in the left lateral cerebral ventricle (Fig. 1A), and brain
magnetic resonance imaging (MRI) demonstrated a highintensity signal area (HISA) in the left temporal lobe on a
diffusion-weighted image (DWI) (Fig. 1B). On apparent diffusion coefficient (ADC) mapping, the left temporal lobe
exhibited iso or low signal intensity (Fig. 1C). MR angiography (MRA) showed intact cerebral arteries (Fig. 1D).
N-isopropyl-p-[123I]iodoamphetamine (IMP)-single photon emission computed tomography (SPECT) performed two
days after admission demonstrated hyperperfusion in the left
temporal lobe (Fig. 1E).
Cerebral angiography showed a dural AVF involving the
left isolated transverse sinus fed by the left meningeal artery, posterior auricular artery and occipital artery with leptomeningeal venous drainage (Borden type 3, Lalwani grade
4) (Fig. 2). The internal carotid and vertebral arteries were
intact bilaterally.
After admission, 10% glycerol (200 mL/day) was administered to prevent brain edema in addition to nicardipine (5
mg/h).
Three weeks after admission, the patient’s symptoms were
slightly improved. She was able to follow one-step com-

Figure 2. Cerebral angiography of the left external carotid
artery. Lateral angiography of the left external carotid artery
(a) showed a dural AVF involving the left isolated transverse
sinus (b) fed by the left meningeal artery (c), posterior auricular artery (d) and occipital artery (e) with leptomeningeal venous drainage (f) (Borden type 3, Lalwani grade 4).

mands. Her spontaneous speech was still impaired; however,
she was able to say to a nurse that she did not want any
more food when she finished her dinner. She could some-

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DOI: 10.2169/internalmedicine.53.1072

times repeat single words, such as “handkerchief,” but not
“tangerine.” She could write simple words, but not her
name. She read words incorrectly; for example, she said
“apple” when she saw “tangerine” written on a paper. A
follow-up MRI showed laminar necrosis in the left temporal
lobe with sparing of the precentral gyrus (Fig. 1F, G).
She received intravenous embolization therapy six weeks
after admission; however, the global aphasia persisted.

Discussion
Van Horn and Hawes reported three patients who developed GAWH in 1982, all of whom were diagnosed with an
embolic cerebral infarction. The authors suggested that
GAWH is a sign of embolic encephalopathy (3). In 1987,
Legatt et al. reported six patients with GAWH, two of
whom had an intracranial hematoma and subarachnoid hemorrhage. The authors commented that GAWH has multiple
etiologies (4). Pai et al. reported four patients with GAWH
in 2011, two of which had a cardioembolic source: one case
was due to internal carotid artery occlusion and one case
was of an undetermined cause (5). We herein report a patient with GAWH diagnosed as having a dural AVF on cerebral angiography. We speculate that arterial blood passed directly through to the venous side through the dural AVF,
causing venous pressure elevation and finally resulting in
venous infarction. The dural AVF exhibited retrograde leptomeningeal venous drainage and was classified as Borden
type 3 and Lalwani grade 4; such lesions are at a high risk
of causing intracranial hemorrhage or nonhemorrhagic neurological deficits (10-13). Brain IMP-SPECT showed hyperperfusion of the left temporal lobe, which is of interest because that area had exhibited hypoperfusion in cases of
GAWH due to arterial infarction in a previous report (6). A
SPECT study of patients with dural AVFs presenting with
venous ischemia showed hypoperfusion in the lesions (14).
Hyperperfusion in dural AVFs is rare. Kuroda et al. reported
a case of a dural AVF presenting with postoperative hyperperfusion (15), and Yamada et al. noted that IMP uptake is
increased in a rat model of venous hypertension (16). We
speculate that IMP uptake was increased in our patient possibly because the first-pass extraction of IMP was decreased
due to slow circulation resulting from venous congestion.
A diagnosis of arterial infarction was ruled out due to the
absence of cerebral artery occlusion on MRA and cerebral
angiography. Encephalitis was also ruled out because the
CSF was normal.
Although early studies suggested that the development of
GAWH requires two discrete lesions involving the anterior
and posterior language areas (3, 7), later studies reported
that a single lesion can cause GAWH (2, 5, 8). In our case,
a single lesion located in the temporal lobe caused GAWH;
this location resembled that observed in cluster 1 in Hanlon’s study and case 8 in Bang’s study (2, 5). Posterior lesions located in the temporal lobe may disrupt fluency and
cause global aphasia in the absence of anterior involve-

ment (17). In addition, our patient had a history of early Alzheimer’s disease; therefore, her verbal fluency may have
been subclinically impaired before the event (18). Shindo et
al. examined the underlying pathogenic mechanism of
GAWH using transcortical magnetic stimulation and concluded that GAWH is caused by sparing of the decussated
pyramidal tract (9). We speculate that a dural AVF involving
an isolated transverse sinus caused laminar necrosis sparing
the deep white matter including the pyramidal tract in our
patient, so that the motor function was not involved.
Dural AVFs can cause GAWH, especially when they result in venous infarction in the temporal area of the dominant hemisphere. Physicians should consider dural AVFs as
a potential cause of GAWH.
The authors state that they have no Conflict of Interest (COI).

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