Case Report

Transverse Sinus Thrombosis: An Unusual Cause of Headache

James Wasson, MD, James Redenbaugh, MD
From the Department of Medicine, Lehigh Valley Hospital, Allentown, Penn.
Address all correspondence to Dr. James Wasson, 300 North Dual Highway, Laurel, DE 19956.
Accepted for publication December 10, 1996.
A 24-year-old woman presented with headache of 6 days' duration, described as throbbing pain in the right
periorbital region radiating to her right ear and neck. The pain was unresponsive to oral analgesics. She had no
speech or hearing difficulties, no previous history of migraines, and no family history of neurologic disease. Her
medications included oral contraceptives for 8 months' duration. This report describes the salient features of dural
sinus thrombosis and recent advances in diagnostic and therapeutic procedures. The relationship between oral
contraceptives and protein S levels is also discussed. Cerebral dural sinus thrombosis should be considered in
the differential of new onset of headaches, seizures, or focal neurological deficits.
Key words: transverse sinus thrombosis, headache, oral contraceptives, protein S
(Headache 1997;37:457–459)
Headache is one of the most common presenting complaints seen in the outpatient setting. Although most headaches are
benign, a careful history and physical exam along with appropriate imaging studies may be necessary to differentiate less
serious causes from more serious causes of headache. Cerebral dural sinus thrombosis is an unusual cause for severe
headache. This case report describes the salient features of dural sinus thrombosis, as well as the most recent advances in
diagnostic and therapeutic procedures.
CASE HISTORY

A 24-year-old woman presented to the emergency department complaining of a headache. Her headache had begun 6
days previously and was described as throbbing pain located in the right periorbital region radiating to her right ear and down
her neck. The pain was unresponsive to oral analgesics. She denied fever, chills, sinus disease, and visual difficulty. She had
no speech or hearing difficulties and no previous history of migraines. Her medications included oral contraceptives for 8
months' duration. Her family history was negative for neurologic disease. She was employed as a nurse and did not smoke, or
drink alcohol.
On examination, she was afebrile with a blood pressure of 130/60, a pulse rate of 92 beats per minute, and respirations
were 12 per minute. Her right conjunctiva was slightly injected. There was no papilledema or sinus tenderness. Her neck was
supple, and no adenopathy was found. Her cardiopulmonary and abdominal examinations were benign. A complete
neurological exam revealed no abnormalities. The results of diagnostic tests revealed a normal blood count, chemistry profile,
and antinuclear antibody (ANA) titer. Her coagulation profile was within normal limits with the exception of a protein S level of
46% (normal 66% to 130%). An MRI with MR angiography revealed thrombosis of the right sigmoid and transverse sinuses
with extension into the right jugular vein (Figure).
[Fig]

COMMENTS

Cerebral dural sinus thrombosis was described as early as the 1800s. In 1825, Ribes described a case of
a 45-year-old man with 6 months of headache, epilepsy, and delirium. Examination at autopsy revealed
thrombosis of the superior sagittal sinus, left lateral sinus, and cortical veins.1 Cerebral dural sinus thrombosis
should be considered in the differential of new onset of headaches, seizures, or focal neurological deficits.
Presentation may be variable but, from a review of 38 cases, the earliest and most common neurologic
symptom found was headache (74%). Papilledema was seen in 45% of patients. Focal deficits, seizures, and
coma were seen in 25% to 35%.2
The true incidence of cerebral dural thrombosis is not known. In an autopsy series, Ehlers and Courville
found only 16 sinus thromboses in a series of 12500 autopsies.3 The etiology of cerebral dural thrombosis
includes infection, dehydration, rheumatologic diseases, local trauma, and hypercoagulable states.
Cavernous sinus thrombosis can be seen after infection of the facial skin, maxillary, sphenoid, or ethmoid
sinuses. Thrombosis secondary to severe dehydration is usually seen in the pediatric population.
Rheumatologic diseases that have been associated with thrombosis include systemic lupus erythematosus
and Behcet's disease. Other conditions that have been associated with both hypercoagulability and cerebral
dural thrombosis include nephrotic syndrome, anti-thrombin III deficiency, cancer, pregnancy (ie, postpartum
period),4 and oral contraceptive use.5–8 Protein S is a cofactor for anticoagulant and fibrinolytic properties of
activated protein C. Reduced protein S levels can be seen in nephrotic syndrome, pregnancy, and the use of
oral contraceptives.9–11
The diagnosis is based on history, physical examination, laboratory data, and imaging studies. An elevated
sedimentation rate and neutrophil level may be present but are nonspecific. In 84% of cases, abnormalities in
the cerebral spinal fluid included increased opening pressure, elevated protein, red cells, and pleocytosis.2
The most common EEG findings are generalized slowing, more marked on one side, with frequent epileptic
activity. A CT scan may be normal or have specific signs of cerebral dural sinus thrombosis such as "cord
sign" representing thrombosis of the cortical vein, "dense triangle sign" representing thrombosis of the
superior sagittal sinus, and the "empty delta sign" representing injection of the collateral veins in the superior
sagittal sinus wall contrasting with noninjection of the clot inside the sinus.5,12
Angiography had been the accepted standard for diagnosis; however, more recently, MRI with MR
angiography has been suggested as the imaging procedure of choice.13 Findings on MR may be variable and
are usually related to the age and evolution of the thrombosis. At times, there is an initial absence of a flow
void and, within a few days, a hyperintense thrombus may

Prothrombin time, sec
Prothrombin time (INR)
Partial thromboplastin time, sec
Platelets, x 109/L
Anticardiolipin IgG, GPL U/mL
Anticardiolipin IgM, MPL U/mL
Antithrombin III, %
Plasminogen, %
Protein C, % activity
Protein S, % activity
Thrombin time, sec
Lupus anticoagulant

Laboratory Results
Normal Range
At Admission
11.3–12..9
13.6
1.3
21.5–33.5
27.5
150–400
248
<24
7.3
<12
5.7
71.2–134.4
127.1
67.3–126.1
Not done
66.0–154
109.4
66.0–130
46.0
0–18
13.3
Negative

5 Months Postdischarge
11.4
1.0
26.5
Not done
10.4
5.1
90.8
106.8
102.0
95.0
13.3
Negative

15264610, 1997, 7, Downloaded from https://headachejournal.onlinelibrary.wiley.com/doi/10.1046/j.1526-4610.1997.3707457.x, Wiley Online Library on [11/04/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License

Hospital Course and Follow-up.—The patient was initially admitted to the hospital and treated with
intravenous heparin to keep the partial thromboplastin time at 1.5 times the control. After 7 days, she was
discharged on warfarin to maintain an international normalized ratio (INR) of 2 to 3. At discharge, her
neurologic examination was normal. She had a persistent headache for the first week which was treated with
oral narcotic analgesics. A repeat MRI 6 months later revealed recanalization of the sigmoid and transverse
sinuses. Her protein S level, without being on anticoagulation, was 95%. Laboratory test results at the time of
admission and 5 months postdischarge are shown in the Table

CONCLUSION

When a patient presents with a new or atypical headache, dural sinus thrombosis should be considered.
Oral contraceptives have been associated with a reduced protein S level and hypercoagulability. Magnetic
resonance imaging with MR angiography has been useful in the evaluation and follow-up of dural sinus
thrombosis patients. Anticoagulation and thrombolysis should be part of the treatment regimen.
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15264610, 1997, 7, Downloaded from https://headachejournal.onlinelibrary.wiley.com/doi/10.1046/j.1526-4610.1997.3707457.x, Wiley Online Library on [11/04/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License

be seen. Later, vascular recanalization may be demonstrated with resumption of a flow void.5 Magnetic
resonance angiography and venous transcranial Doppler ultrasound have been suggested for both
monitoring and follow-up of patients.14 Due to the low incidence and multiple etiologies of dural sinus
thrombosis, treatment has remained controversial. One randomized study15 and several case reports16,17
have shown the benefit of high-dose heparin therapy.5 More recently, however, thrombolytics using selective
catheterization and urokinase have been successful.18