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Dural sinus thrombosis in beh┬зet's disease.

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Recent reviews (1) have divided the manifestations of Behqet’s disease into major and minor diagnostic criteria. Often the “minor criteria,” especially
central nervous system and vascular disease, are the
major causes of morbidity and mortality in this syndrome. Reports of the neurologic manifestations in
Behqet’s disease include patients with increased intracranial pressure. Recently (2-4) angiograms have demonstrated intracranial thrombophlebitis in several patients. In the following report, we describe a patient
with documented thrombosis of the dural sinuses and
intracranial hypertension. Venous thrombosis may
therefore be the mechanism of elevatcd intracranial
pressure in BehGet’s disease.
Case report. The patient, a 41-year-old man,
was seen in January 1983. He had previously been
healthy when he noted the onset of severe, bitemporal
headache, ill-defined visual disturbances, and a 15-lb
weight loss. Physical examination revealed a thin male
in no acute distress. Positive findings included shotty
cervical lymphadenopathy and bilateral papilledema
with hemorrhage and exudates on funduscopic examination. The neurologic examination results were normal, as were the chest radiograph and electrocardioFrom the Department of Medicine. College of Physicians
and Surgeons, Columbia University. New York, New York.
Supported by a grant from the Arthritis Foundation.
llan Bank, MD: Collegc of Physicians and Surgeons. Columbia University; Christine Weart. MD: College of Physicians and
Surgeons, Columbia University.
Address reprint requests to llan Bank, MD. Division of
Rheumatology, Department of Medicine. College of Physicians and
Surgeons, Columbia University. 630 West 168th Strcet, New York.
NY 10032.
Submitted for publication December 3, 1983; accepted in
revised form February 8, 1984.
Arthritis and Rheumatism, Vol. 27, No. 7 (July 1984)
gram. Laboratory test results were: erythrocyte
sedimentation rate I I 1 mm/hour; hemoglobin 10.9 gm/
dl; hematocrit 33.8%; platelets 701 ,OOO/pl; white blood
count 6,3OO/pl, normal differential; bilirubin 0.4 mg/dl;
alkaline phosphatase 256 unitsldl (normal 25-100);
aspartate transaminase 49 unitslml (normal 10-50);
alanine transaminase 94 units/ml (normal 1-35); amylase 71 units (normal 5-81). He had a normal coagulation profile. Serum protein electrophoresis revealed
diffusely elevated gamma globulin; urinalysis showed
0-2 white blood cells, 4-5 red blood cells, trace
protein. Antinuclear antibody, latex fixation, antiDNA antibodies, and complerncnt test values were
Computerized tomographic scan of the head
revealed a possible filling defect in the sagittal sinus,
with no shift or mass. Lumbar puncture revealed an
opening pressure of 500 mm HzO. The fluid was clear
and colorless. Tests for protein and glucose levels
showed 16 mg/dl, and 74 mg/dl (serum 121 mg/dl),
respectively. IgG in the cerebrospinal fluid was 3.1
mg/dl (18.2% of the total immunoglobulin), cell count
showed 6 mononuclear cells, 6 red blood cells. Gram
stain and cultures (bacterial, fungal, and acid-fast
bacilli) were negative as were the India ink stain,
VDRL, cytology, and oligoclonal band tests.
Several days after admission, punched out scrotal ulcerations were noted. Biopsy showed a patchy
infiltrate of mononuclear cells, polymorphonuclear
cells, and some eosinophils. Tzanck preparation and
cultures were negative.
Treatment with dexamethasonc was begun, and
lumbar punctures were performed daily. A cerebral
angiogram with injections into both internal carotid
arteries and the right vertebral artery showed a normal
Figure 1. Venous phase of cerebral angiogram. Arrow indicates the
point of occlusion of the superior sagittal sinus.
arterial phase. The distal superior sagittal sinus and
bilateral proximal lateral sinuses did not fill in the
venous phase (Figure 1). There was collateral filling of
the cortical veins going into the lateral sinuses.
Further exhaustive diagnostic evaluation in
search of an occult malignancy produced negative
results. As a test for Behqet's disease, saline was
injected intradermally, which resulted in the development of sterile pustules. The patient then developed
punched out oral ulcerations, an acneiform rash, and
basilar nodular densities in both lung fields. During the
third week of hospitalization, a transient right hemiparesis with an expressive aphasia developed. A further complication was the development of a right
lower extremity deep vein thrombosis. A short course
of heparin was administered with good response, but
in view of the risk of pulmonary hemorrhage, prolonged anticoagulation therapy was not given. Treatment was instituted with oral colchicine, 0.6 mg twice
daily and oral chlorambucil, 6 mg every other day.
After further clinical improvement, the patient
was discharged. As an outpatient, he required repeated lumbar punctures for persistently elevated intracranial pressure. A lumbar peritoneal shunt was performed with resolution of the papilledema and
headaches. On a continued therapy of colchicine and
chlorambucil the dermatologic, mucosal, and pulmonary manifestations resolved, and he regained his I5
Discussion. The diagnosis of Behqet's disease
depends, at present, on clinical criteria. A combination of 3 or more of the following findings is used by
most authors as grounds for the diagnosis (5): 1)
recurrent aphthous stomatitis; 2) recurrent genital
ulcers; 3) anterior or posterior uveitis; 4) vasculitis of
cutaneous or large vessels; 5) arthritis; 6) meningoencephalitis; and 7) cutaneous hyperreactivity to minor
trauma. Others have used a combination of minor and
major criteria (1). In any case, the manifestations may
be myriad (6),and include neurologic findings ( I ,3,79). Necropsy studies demonstrate meningeal lymphocytic infiltration, necrotic foci in the white matter,
demyelinization, and scarring throughout the nervous
This patient presented with signs and symptoms
of increased intracranial pressure, only later to develop both oral and scrota1 ulcerations. This serves to
underscore the fact that not all manifestations need
occur simultaneously and that Behqet's syndrome
should be kept in mind in any patient with compatible,
even if incomplete, symptomatology.
The immediate and pivotal problem in this
patient pertained to intracranial hypertension, secondary to dural sinus occlusion. In a recent review of 27
patients with Behqet's disease (lo), thrombosis of the
superior sagittal sinus was found in 2 of 5 patients with
neurologic disease. One patient went on to develop
bilateral iridocyclitis and blindness, and occlusion of
the inferior vena cava. Pamir et a1 (4) documented a
similar occlusion in 2 patients, 1 of whom developed a
right hemiparesis. In the other the papilledema resolved after 2 months. Three patients have been
described by Bousser (2) who claims a role for steroid
therapy and anticoagulation in the treatment of this
Other less clear-cut cases include a case report
(11) in which the diagnosis was presumed but not
proven. In addition, Bienenstock and Margulies (12)
described a 38-year-old pilot with focal neurologic
findings who had both arterial and venous thromboses.
The cerebrospinal fluid pressure was not noted. Also
of interest is a report of 3 patients with Behqet's
syndrome who were seen with signs of intracranial
hypertension (1 3). Intracranial venous thrombi were
postulated and their clinical courses were benign. If
indeed, the pathology in these patients was thrombosis, recanalization and/or collaterals may have developed. Our case and those of others serve to emphasize
that in some patients the process may not be benign.
To date, no angiographic evidence has been presented
proving recanalization of dural vein thrombosis in
Behqet’s disease.
The treatment of choice for this complication of
Behqet’s disease is unclear. Although steroids are
useful to ride out the acute episode and to decrease the
intracranial pressure, their long-term role in treatment
of Behqet’s disease is questionable (1). The use of
anticoagulants has been suggested (2), but lung involvement may constitute a relative contraindication
to long-term anticoagulation. Lung involvement is
well-described in Behqet’s disease (l4,15). Several
patients with pulmonary findings have developed massive hemoptysis (l5,16), and in some of these cases,
pulmonary arterial aneurysms have been demonstrated (16,17). In 1 report (18) intracranial hypertension
and hemoptysis occurred in the same patient. We
elected, therefore, to treat o u r patient first with colchicine and later with chlorambucil, both of which have
been used for various manifestations of Behqet’s disease (1,19,20). Although the systemic manifestations,
such as cachexia and mucosal, skin, and lung lesions
have abated, our patient’s major problem of intracrania1 hypertension did not resolve. It is hoped, however,
that the treatment will prevent new thromboses, possibly by decreasing the underlying disease activity ( I ) .
In summary, we have described a patient with
Behqet’s disease in whom ititracerebral dural sinus
thrombosis led t o intracranial hypertension. We suggest that patients with Behqet’s diseasc and intract-anial hypertension should be evaluated for thrombosis.
Once diagnosed, the long-term prognosis remains
guarded. Treatment is directed toward control of both
the underlying disease activity and the local intracercbra1 process, and must be individualized, taking into
account other organ involvement and the effect of the
treatment on the course of the systemic disease (21 ).
Acknowledgments. The authors wish to thank Dr. I.
Jaffe for his helpful comments in editing this paper. The
secretarial assistance of Ms L. Nichols and Ms C. Allen was
also greatly appreciated.
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thrombosis, duran, beh, disease, зet, sinus
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