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Superior sagittal sinus thrombosis and systemic lupus erythematosus.

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Superior Sagittal Sinus
Thrombosis and Systemic
Lupus Erythematosus
Zenji Shiozawa, MD,* Motoaki Yoshida, MD,?
Kazuo Kobayashi, MD,? Shinichi Tsunoda, MD,"
and Tadaaki Mano, MDS
Superior sagittal sinus thrombosis (SSST) very rarely occurs
in association with systemic lupus erythematosus (SLE). W e
know of only one prior case of SSST with SLE [2}. Gibson
and Myers 121 briefly recorded the association in a list of 6
autopsied patients with SLE, but gave no further details. We
describe a patient with angiographically proved SSST, which
developed prior to SLE.
A 4 3-year-old woman had been hospitalized nine years
previously for evaluation of headaches. Examination revealed
only bilateral papilledema. Lupus erythematosus (LE)preparations were negative. Lumbar puncture showed an opening
pressure of 390 mm HzO with protein level of 40 mg/dl.
Bilateral carotid angiography showed the posterior portion
of the superior sagittal sinus to be markedly narrowed, with
marked collateral venous filling via parietal emissary veins
and anterior and posterior superficial temporal veins of the
scalp (Figure). Following large doses of antifibrinolytic agents
and adrenocorticosteroids, headache and papilledema subsided.
Two years later, she was readmitted for recurrent headaches. Physical examination showed a butterfly rash on both
cheeks and erythema nodosum at various other sites.
Neurologically she exhibited bilateral papilledema and abducens palsies. Pertinent laboratory data included an erythrocyte sedimentation rate of 60 m d h r . Urinalysis showed proteinuria and several granular casts. Blood urea nitrogen was
37 mg/dl. An LE test was positive, antinuclear antibody
(shaggy and homogeneous pattern) was 3 2 0 X , and antiD N A antibody was 64 Uiml. H ypocomplementemia was
observed. Lumbar puncture again showed elevated intracranial pressure (350 mm H20).Computed tomography of the
brain was normal. Carotid angiography showed almost the
same SSST abnormalities as before. Treatment with adrenocorticosteroids and antifibrinolytic agents was begun, and
two months later her condition had improved. She continues
to be observed as an outpatient, and still receives small doses
of adrenocorticosteroids.
Right carotid angiogram, venous phase, lateral view. The
superior sagittal sinus is extremely nawow around the junction
of the middle and posterior part (arrow). Collateral circulation
into the parietal emissaly veins and superficialanterior and posterior temporal veins of the scalp is seen.
However, the diagnosis of pseudotumor cerebri has been
based on the radiological exclusion of other disorders.
Therefore, it is possible that other disorders have masqueraded as pseudotumor cerebri. For example, in some patients with pseudotumor cerebri, dural sinus thrombosis has
been demonstrated 131. The cause, outcome, and treatment
of pseudotumor cerebri need to be clarified. W e believe
cerebral angiography is necessary for accurate diagnosis and
for exclusion of an underlying disorder, such as major venous sinus thrombosis [4].
*Department of Medicine
Yamanashi Medical University
Yamanashi, 409-38, Japan
?Department of Neurology
Tohoku University School of Medicine
Sendai, 480, Japan
$First Department of Internal Medicine
Nagoya University School of Medicine
Nagoya, 466, Japan
Our patient was initially diagnosed as having pseudotumor
cerebri (benign intracranial hypertension), but SSST was
proved by cerebral angiography. Treatment with large doses
of antifibrinolytic agents and adrenocorticosteroids seemed
to be effective. Concerning the association of SLE with
pseudotumor cerebri, Silberberg and Laties [ 5 } and Carlow
and Glaser 111 stress that pseudotumor cerebri is occasianally a manifestation of SLE and that awareness of this syndrome as a complication of SLE might make it possible to
avoid potentially hazardous radiographic contrast studies.
The use of cerebral angiography has been avoided in
SLE patients with clinically suspected pseudotumor cerebri.
1. Carlow TJ, Glaser JS: Pseudotumor cerebri syndrome in systemic
lupus erythematosus. JAMA 228:197-200, 1974
2. Gibson T, Myers AR: Nervous system involvement in systemic
lupus erythematosus. Ann Rheum Dis 35:398-406, 1976
3 . Ray BS, Dunbar HS: Thrombosis of the superior sagittal sinus as
a cause of pseudotumor cerebri; methods of diagnosis and treatment. Trans Am Neurol Assoc 75:12-17, 1950
4. Shiozawa 2, Yamada H, Mabuchi C, et al: Superior sagittal sinus
thrombosis associated with androgen therapy for hypoplastic
anemia. Ann Neurol 12:578-580, 1982
5 . Silberberg DH, Laties AM: Increased intracranial pressure in
disseminated lupus erythematosus. Arch Neurol 29:88-90,
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lupus, sagittal, thrombosis, systemic, erythematosus, superior, sinus
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