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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1844–1846

Dural carotid-cavernous fistula presenting with confusion
and expressive aphasia
Sean Woolen b, Joseph J. Gemmete a,b,c,⇑, Aditya S. Pandey a,b, Neeraj Chaudhary a,b
a
b
c

Department of Neurosurgery, Room UH B1 D328, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA
Department of Radiology, University of Michigan Hospitals, Ann Arbor, MI, USA
Department of Otolaryngology, University of Michigan, Ann Arbor, MI, USA

a r t i c l e

i n f o

Article history:
Received 5 May 2015
Accepted 26 May 2015

Keywords:
Cortical venous reflux
Dural carotid cavernous fistula
Expressive aphasia

a b s t r a c t
We report a patient who presented with confusion and expressive aphasia at the onset of a cortical
venous reflux (CVR) related to a known dural carotid-cavernous fistula (DCCF). CVR and venous congestion are rare findings in a DCCF, however, it is important to recognize these findings early to reduce the
associated risks of venous hemorrhage and infarction. To our knowledge, no previous report has
described an association of expressive aphasia with CVR from a DCCF. This patient highlights the potential for venous congestion secondary to CVR from a DCCF presenting with a constellation of symptoms,
including confusion and expressive aphasia with corresponding MRI findings. Clinicians should consider
a DCCF in the differential diagnosis of this clinical presentation, with corroborative imaging findings.
Ó 2015 Elsevier Ltd. All rights reserved.

1. Introduction
Cortical venous reflux (CVR) and venous congestion are rare
findings in a dural carotid-cavernous fistula (DCCF), however, it
is important to recognize these findings early to reduce the associated risks of venous hemorrhage and infarction [1–4]. We report a
patient who presented with confusion and expressive aphasia at
the onset of CVR from a DCCF.
2. Case report
A 68-year-old woman was referred for evaluation and treatment of a DCCF. One year prior to treatment, she experienced double vision, primarily affecting her left eye, with no other reported
symptoms. She was diagnosed with a DCCF that was treated conservatively, with resolution of clinical symptoms at 3 months.
She then developed symptoms in her right eye 6 months after
the initial diagnosis, consisting of difficulty with eye movement,
blurred vision, diplopia, and proptosis. The diplopia was corrected
by refractory lens and timolol maleate ophthalmic solution eye
drops (Timoptic; Merck, Whitehouse Station, NJ, USA).
While traveling on an airplane 1 year after her initial diagnosis,
she developed acute mental status changes, confusion, and expressive aphasia with no other neurological deficits. Brain MRI was
obtained, and when compared to the prior MRI, it demonstrated
a new hyperintense fluid-attenuated inversion recovery (FLAIR)
signal abnormality within the left frontal operculum (Fig. 1A). An
ophthalmology examination confirmed persistent proptosis of
the right eye with an increase in intraocular pressure and
gaze palsy. A decision was made to perform embolization of the
DCCF.
A cerebral angiogram was performed to define the extent of the
fistula. Transarterial access was obtained in the right common
femoral artery, and a 4-French sheath was placed. A 4-French vertebral catheter was used to selectively catheterize the right internal carotid artery (ICA), right external carotid artery (ECA), right
vertebral artery, left ICA, left ECA, and left vertebral artery. An
angiogram of each of the selected vessels was performed in the
anteroposterior and lateral projections.
⇑ Corresponding author. Tel.: +1 734 936 4566; fax: +1 734 764 4230.
E-mail address: gemmete@med.umich.edu (J.J. Gemmete).

The cerebral angiogram identified bilateral DCCF. The right ICA
angiogram demonstrated early filling of the right cavernous sinus
from the meningohypophyseal trunk, with drainage anteriorly into
the ophthalmic vein and contralateral filling of the left cavernous
sinus, with CVR in the left cerebral hemisphere (Fig. 1B). The left
ECA angiogram demonstrated early filling of the left cavernous
sinus with CVR into the left superficial middle cerebral vein
(SMCV) and left superior petrosal sinus (SPS), with partial filling
of the right cavernous sinus (Fig. 1C). The left ICA angiogram
demonstrated early filling of the left cavernous sinus from
branches of the meningohypophyseal trunk.
The bilateral DCCFs were treated via a transvenous approach
through the inferior petrosal sinus by placing multiple microcoils
into the left and right cavernous sinuses. Selective venograms were
performed by injecting contrast into the left cavernous sinus,
which demonstrated CVR with filling of the left SMCV and left
SPS (Fig. 2A, B). A cone beam CT scan showed the engorged venous
channels within the left frontal operculum corresponding to the
hyperintense FLAIR signal abnormality within the left frontal operculum (Fig. 2C). The final cerebral angiogram showed complete
occlusion of the fistula (Fig. 3A, B).
The clinical follow-up visits at 1 month and 1 year showed resolution of the clinical signs and symptoms of confusion, expressive
aphasia, right eye proptosis, restriction of gaze movements, and
diplopia. The intraocular pressure normalized in both eyes. The
MRI showed resolution of the hyperintense FLAIR signal abnormality in the left frontal operculum due to normalization of the venous
congestion (Fig. 3C). The follow-up cerebral angiogram, 6 months
after the embolization, demonstrated continued occlusion of the
fistula.
3. Discussion
DCCF have a slow, progressive course, with the majority of
patients presenting with progressive glaucoma, proptosis, ophthalmoparesis, or conjunctival injection [2,5]. The natural history of a
DCCF is variable, and the literature reports that spontaneous resolution without treatment occurs in 10–60% of patients, possibly
due to spontaneous thrombosis of the involved segment of the cavernous sinus [6].
CVR can occur in 27–63% of carotid-cavernous fistula (CCF)
patients with a majority of these from direct CCF [3,4]. CCF with

Case Reports / Journal of Clinical Neuroscience 22 (2015) 1844–1846

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Fig. 1. (A) Axial, fluid-attenuated inversion recovery (FLAIR) MRI shows abnormal high signal within the left frontal operculum (black arrow). (B) The frontal right internal
carotid angiogram shows earlier filling of both cavernous sinuses and the left superficial middle cerebral vein (SMCV; black arrows). (C) The frontal left external carotid
angiogram shows early filling of both cavernous sinuses and left SMCV (black arrows).

Fig. 2. Selective venograms of the left cavernous sinus in (A) anteroposterior projection shows filling of the cavernous sinus and left SMCV with venous congestion in the left
frontal operculum (black arrows), and (B) lateral projection shows retrograde filling of the left SMCV (black arrows) and superior petrosal sinus (small black arrow). There is
engorgement of cortical venous structures within the left frontal operculum. (C) The axial cone beam CT scan from contrast injected into the left cavernous sinus shows
engorgement of the cortical venous structures (white arrow) within the left frontal operculum corresponding to the abnormal high signal within the left frontal operculum,
seen in Figure 1A.

CVR are considered to be at increased risk for venous hemorrhage
or infarction (30–40%) compared to those without CVR [1,2].
Therefore, CVR on digital subtraction angiography is an indication
to perform urgent endovascular treatment [2,7]. However, to our
knowledge, no previous report discusses the change in clinical
symptoms that can occur as a DCCF starts to develop CVR.
Our patient presented with the typical slow progression of
symptoms with diplopia, conjunctival injection, and proptosis.
Her conservative treatment resulted in symptom progression,
including confusion and expressive aphasia associated with the
development of CVR. This correlated with a hyperintense FLAIR signal abnormality in the frontal operculum.
Fig. 3. (A) The frontal right common carotid angiogram after coil embolization of
both cavernous sinuses shows no early filling of the cavernous sinuses and left
superficial middle cerebral vein (SMCV). (B) The frontal left external carotid
angiogram after coil embolization of both cavernous sinuses shows no early filling
of both cavernous sinuses and venous structures in the left cerebral hemisphere. (C)
Axial, fluid-attenuated inversion recovery MRI at 1 month after embolization shows
interval resolution of the abnormal high signal within the left frontal operculum.

Conflicts of Interest/Disclosures
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1846–1847

References
[1] Cognard C, Gobin YP, Pierot L, et al. Cerebral dural arteriovenous fistulas:
clinical and angiographic correlation with a revised classification of venous
drainage. Radiology 1995;194:671–80.
[2] Meyers PM, Halbach VV, Dowd CF, et al. Dural carotid cavernous fistula:
definitive endovascular management and long-term follow-up. Am J
Ophthalmol 2002;134:85–92.
[3] Takahashi S, Sakuma I, Otani T, et al. Carotid-cavernous fistula associated with
an intracranial lesion caused by cortical venous reflux. Interv Neuroradiol
2006;12:167–73.

[4] Willinsky R, Goyal M, terBrugge K, et al. Tortuous, engorged pial veins in
intracranial dural arteriovenous fistulas: correlations with presentation,
location, and MR findings in 122 patients. AJNR Am J Neuroradiol
1999;20:1031–6.
[5] Gemmete JJ, Ansari SA, Gandhi DM. Endovascular techniques for treatment of
carotid-cavernous fistula. J Neuroophthalmol 2009;29:62–71.
[6] Barrow DL, Spector RH, Braun IF, et al. Classification and treatment of
spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985;62:248–56.
[7] Halbach VV, Hieshima GB, Higashida RT, et al. Carotid cavernous fistulae:
indications for urgent treatment. AJR Am J Roentgenol 1987;149:587–93.

http://dx.doi.org/10.1016/j.jocn.2015.05.022

Episodic weakness and vacuolar myopathy in hypokalemic periodic
paralysis
Diana Basali b, Richard A. Prayson a,⇑
a
b

Department of Anatomic Pathology, Cleveland Clinic, Level 25, 9500 Euclid Avenue, Cleveland, OH 44195, USA
Stritch School of Medicine, Loyola University Chicago, Maywood, IL, USA

a r t i c l e

i n f o

Article history:
Received 12 June 2015
Accepted 20 June 2015

Keywords:
Autosomal dominant mutation
CACN1AS gene
Hypokalemic periodic paralysis
Periodic paralyses
SCN4A gene
Vacuolar myopathy

a b s t r a c t
We report a 50-year-old woman who presented with a 20 year history of gradually progressive lower
extremity weakness, characterized by knee buckling with occasional falls and foot dragging. She also
experienced difficulty in lifting her arms above her shoulders. The primary periodic paralyses are rare
disorders caused by dysfunctional ion channels in skeletal muscle. The hypokalemic type is generally
an autosomal dominant condition, due to missense mutations in the alpha subunits of the skeletal muscle
L-type calcium channel genes, CACN1AS, or the skeletal muscle sodium channel gene, SCN4A. The
affected patients typically present with episodic weakness. For our patient, the consumption of foods
high in carbohydrates seemed to precipitate the episodes of weakness. Her family history was significant
for six blood relatives, including three sons and three relatives on the paternal side, who had experienced
similar symptoms. A biopsy of the left rectus femoralis muscle showed vacuolar myopathic changes in
the scattered muscle fibers, accompanied by occasional degenerating and regenerating muscle fibers.
There was no evidence of inflammation on the biopsy. The vacuoles were often associated with increased
acid phosphatase staining. An electron microscopic examination showed that the vacuolar changes were
due to T-tubule dilation, a characteristic of hypokalemic periodic paralysis. Other metabolic etiologies of
vacuolar myopathy, such as acid phosphatase (lysosomal) associated acid maltase deficiency (a glycogen
storage disease), need to be considered in the differential diagnosis.
Ó 2015 Elsevier Ltd. All rights reserved.

1. Introduction
The primary periodic paralyses are disorders of dysfunctional
ion channels and are classified as either hyperkalemic or hypokalemic, based on serum potassium levels and the response to potassium administration. Hypokalemic periodic paralysis (HPP) is a
rare disorder, with a prevalence of 1:100,000, and consists of
attacks of flaccid paralysis that often occur at night or early in
the morning [1]. The attacks can either occur spontaneously, or
be provoked by exercise or a high carbohydrate meal. We report
a 50-year-old woman with symptoms, family history, and muscle
biopsy findings consistent with HPP.

2. Case report
A 50-year-old woman who presented with a 20 year history of
gradually progressive proximal lower extremity weakness,
described episodes of knee buckling with occasional falls, foot

⇑ Corresponding author. Tel.: +1 216 444 8805; fax: +1 216 445 6967.
E-mail address: praysor@ccf.org (R.A. Prayson).

dragging and difficulty in lifting her arms above her shoulders.
One year prior, she fractured her left hip and subsequently
required a walker for ambulation. She also described episodes of
weakness which seemed to be precipitated by eating certain
salads, pasta or pizza dinners. Her family history was remarkable
for three adult sons, a paternal aunt and two paternal cousins
who had experienced similar episodes of weakness, and a father
who had similar symptoms and who died of pneumonia at 39 years
of age from suspected respiratory weakness. Her general physical
examination was unremarkable. The electromyography studies
showed a generalized myopathy, predominantly involving the
lower extremities, with associated fibrillation potentials and
occasional myotonic discharges. Her electrolytes were normal
except for low serum potassium. The sedimentation rate, aldolase,
vitamin B12 level and thyroid stimulating levels were normal.
A biopsy of the left rectus femoralis muscle was performed and
showed vacuolar myopathic changes in scattered muscle fibers
(Fig. 1). The occasional vacuole showed acid phosphatase associated staining. Increased periodic acid-Schiff staining, indicative of
glycogen accumulation, was not noted, but occasional degenerating and regenerating muscle fibers were observed. A rare focus of
chronic inflammation that was comprised primarily of benign