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Cardiogenic brain embolism and lupus anticoagulant.

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BRIEF COMMUNICATIONS
Cardiogenic Brain
EmboliYsm and Lupus
Anticoagulant
Seymour M. Young, MD,* Marc Fisher, MD,+
Ann Sigsbee, MD,t and Annmarie Errichetti, MDt
T h e lupus anticoagulant, a n acquired immunoglobulin
associated with an increased tendency for thrombosis,
has been linked to the occurrence of cerebral ischemia
presumably related to in situ thrombosis. Cardioembolic
cerebral ischemic events have rarely been reported. We
encountered 2 patients with focal cerebral ischemia,
substantial mitral valvular masses, and a circulating
lupus anticoagulant. I n each, diagnostic evaluation supported a cardioembolic etiology. These findings illustrate the need for evaluating patients with cerebral
ischemic events for a cardioembolic source when a circulating lupus anticoagulant is present.
Young SM, Fisher M, Sigsbee A, Errichetti A.
Cardiogenic brain embolism and lupus
anticoagulant. Ann Neurol 1989;26:390-392
Focal cerebral ischemia in young patients should
prompt an extensive investigation of its basis. Recently, the presence of a circulating coagulation inhibitor, the lupus anticoagulant (LA), has been increasingly recognized in these patients 111. LA, an
antiphospholipid antibody with implied anticoagulant
activity, paradoxically results in an increased risk for
thrombosis 121. I n situ arterial thrombosis has been
proposed as the etiological mechanism for focal cerebral ischemia in these patients, and cardioembolic
events have only rarely been reported 13-51. We encountered 2 young patients with cerebral ischemic
events who had a circulating LA, mitral valvular vegetations, and additional laboratory evidence supportive
of a cardioembolic mechanism.
Case Reports
Patient I
A 42-year-old woman with a history of systemic lupus
erythematosus (SLE) and hypertension was hospitalized for
transient focal neurological symptoms. She had initially been
diagnosed as having SLE 18 years earlier, but the disease had
been quiescent for 16 years when the occurrence of serositis
From the Departments of *Neurology and ?Medicine, Worcester
Memorial Hospital and the University of Massachusetts Medical
School, Worcester, MA.
Received Nov 30, 1988, and in revised form Jan 26, 1989. Accepted for publication Jan 28, 1989.
Address correspondence to Dr Fisher, Worcester Memorial Hospital, 119 Belmont Street, Worcester, MA 01605.
and peritonitis necessitated initiation of chronic oral prednisone therapy. The patient also began to complain of migraine
headaches with episodes of vertigo and diplopia. The history
included two n o d pregnancies, no use of oral contraceptives, and a daily habit of smoking one pack of cigarettes.
Three weeks prior to admission the frequency of headaches increased. On two occasions she experienced transient
periods of confusion and word-finding difficulty not associated with headache. Multiple episodes of paresthesias affecting the left upper extremity and lasting approximately 5 minutes were also noted.
On admission, the patient was afebrile and had a blood
pressure of 144/108. General physical examination demonstrated livedo reticularis but no malar rash. The cardiac
rhythm was regular withour murmur, rub, gallop, or additional heart sounds. Moderate splenomegaly was present.
Neurological findings on detailed testing were entirely normal.
Areas of decreased attenuation without enhancement or
mass effect were observed in the right frontal region and the
left occipital region on a contrast-enhanced computed tomographic scan of the head. Complete blood cell count;
serum electrolyte and complement levels; results of renal,
hepatic, and thyroid function tests; and the cerebrospinal
fluid were normal. Blood cultures were sterile. Anti-DNA
antibodies were present at a dilution of 1: 80. The prothrombin time (PT) was 17 seconds (control of 12 seconds) and the
partial thromboplastin time (PTT) was 79 seconds (control
of 31 seconds). The prolonged PTT was not corrected by the
addition of normal plasma in a 1: 1 dilution. Increasing dilution of the patient’s plasma with normal plasma corrected the
abnormal PTT. Assays for clotting factors VII, IX, XI,
and XI1 showed reduced activity in the absence of a specific coagulation factor deficiency. Anticardiolipin antibodies
were not detected. An electrocardiogram demonstrated sinus tachycardia. A chest radiograph was normal. Ten percent
stenosis of both proximal internal carotid arteries was observed on carotid noninvasive testing. An electroencephalogram (EEG) was abnormal due to the presence of centrotemporal theta rhythm over the left midtemporal region.
An echocardiogram demonstrated a large rounded echodensity attached to the posterior mitral valve leaflet on its atrial
aspect (Fig). A smaller, irregular echodensity was attached to
the anterior mitral valve leaflet along its atrial aspect and
moderate mitral regurgitation was also observed.
The patient was prescribed warfarin sodium and experienced no further cerebral ischemic episodes during 6 months
of follow-up.
Patient 2
A 35-year-old woman was hospitalized for the sudden onset
of left hemiparesis and left visual field defect. She had been
evaluated 2 years earlier for complaints of menorrhagia and a
tendency to bruise easily. At that time, the PTT was 69
seconds (control of 38 seconds) and there were no other
coagulation abnormalities. A circulating LA was documented
by failure of the PTT to be corrected by 1: 1 dilution of the
patient’s plasma with normal plasma. Increasing dilution of
the patient’s plasma with normal plasma corrected the abnormal PTT. Assays for clotting factors VII, IX, XI, and XI1
showed reduced activity in the absence of a specific coagula-
390 Copyright 0 1989 by the American Neurological Association
serum complement concentration, and serum protein electrophoresis. The erythrocyte sedimentation rate was 48 m d
hour. Antinuclear antibodies were present at a dilution of
1: 64. Results of the Venereal Disease Research Laboratory
test were negative. The PT was 14 seconds (control of 12
seconds) and the PTT was 53 seconds (control of 31 seconds). An electrocardiogram, chest radiograph, and urinalysis results were normal. A parieto-occipital infarction in the
right hemisphere was observed on a noncontrast-enhanced
computed tomographic head scan. Echocardiography demonstrated echodensities on the undersurface of the posterior
leaflet of the mitral valve and associated prolapse. Cardiac
catheterization of both sides of the heart confirmed these
findings. Occlusion of a parietal branch of the right middle
cerebral artery and a proximal branch of the right posterior
cerebral artery was observed on cerebral angiography. There
was no angiographic evidence of vasculitis. The patient was
initially treated with heparin sodium and then switched to
warfarin sodium. There were no recurrent ischemic cerebral
vascular events during 8 years of follow-up.
An apicacal view of two chambers on the echocardiogram demonstrates an echogenic mass attached to the atridl surface of the
posterior mitral leajet (arrow). LV = left ventricle, LA =
left atrium.
tion factor deficiency. A serological evaluation was negative
for SLE.
Except for the menorrhagia the patient was well until 3
weeks prior to admission when she experienced a grand mal
seizure. Findings on neurological examination, computed tomographic scan of the head, EEG, and cerebrospinal fluid
studies were all unremarkable. Phenytoin sodium, 100 mgs
tid, was started. The medical history was unremarkable except that the patient had smoked one pack of cigarettes per
day for 20 years.
Three weeks after the seizure, she suddenly developed
weakness on the right and right visual field loss. The disturbance cleared in a few hours, but later that day she developed weakness and paresthesias on the left with nausea and
vomiting. On admission blood pressure was 150/88. A grade
216 systolic ejection murmur was heard at the cardiac apex,
and radiated to the left sternal border. Neurological examination demonstrated a homonymous hemianopia on the left;
central facial weakness o n the left; moderate left hemiparesis,
being worse for the arm than for the leg; decreased primary
sensory modalities over the left side; hyperreflexia in the left
arm and leg; and a left extensor plantar response.
Laboratory values revealed normal complete blood cell
count, serum electrolyte levels, renal and hepatic functions,
Discussion
We report the cases of 2 relatively young women, one
with and one without SLE, who had cerebral ischemic
events in association with a circulating LA. The presence of LA was documented by accepted hematological criteria [6]. Both patients had echocardiographic
evidence of mitral valvular masses when focal cerebral
ischemic events developed, suggesting a cardioembolic
pathogenesis. In Patient 1, computed tomographic
scans revealed several infarcts in multiple vascular distributions, supportive of cerebral ischemia on a cardioembolic basis [7]. The multiple intracranial arterial
branch occlusions observed in Patient 2 also support a
cardioembolic mechanism. Cerebral angiography and
carotid noninvasive studies excluded atherosclerotic
disease of a proximal carotid artery as a potential embolic source in these patients.
The LA is an acquired immunoglobulin that results
in an enhanced tendency for thrombosis. Paradoxically, the LA was originally described as a potential
cause for hemorrhagic tendencies. Originally observed
in patients with SLE, the LA has now been associated
with a broad spectrum of conditions [GI. The LA may
result in either arterial or venous thrombosis, and it
has been associated with transient ischemic attacks and
cerebral infarcts [1-5, 81. How the LA induces hypercoagulability remains speculative, but this tendency
may be secondary to reduced fibrinolytic activity, altered function of antithrombin 111, inhibition of prostacyclin, delay in protein C activation, or other as yet
undefined mechanisms [l, 61.
Previous reports have suggested a potential cardioembolic mechanism for ischemic stroke in patients
with circulating LA [Z, 4, 91. None of these reports
indicated evidence of large valvular mass lesions at the
time of stroke, as our report does. Ventricular thrombus associated with stroke and the presence of anticar-
Brief Communication: Young et al: Embolic Stroke and Lupus Anticoagulant 391
diolipin antibodies, another antiphospholipid antibody,
has been reported in a patient without circulating LA
W1.
Although some 5 to 10% of patients with SLE have
cerebral infarcts, cardiogenic emboli have rarely been
diagnosed El 13. The tendency for thrombosis associated with LA may significantly increase the risk of cardioembolic cerebral ischemic events in some patients
with SLE, as well as in those without SLE who have
laboratory evidence of its presence. The LA has been
observed to enhance the development of endocardial
scarring, a situation that may facilitate the deposition of
fibrin and platelets on cardiac valves [3-5}. These
vegetations, in turn, may serve as a nidus for embolization. Despite scarring and distortion of valves, these
masses may not cause hemodynamic compromise and
remain clinically silent until embolization occurs 15,
12).
Our findings suggest a relationship between cardiac
valvular masses, circulating LA, and cerebral ischemic
events. Both patients had an obvious cardiac valvular
mass as a source for emboli. We suggest that patients
with focal cerebral ischemia and circulating LA have a
careful cardiological evaluation. Echocardiographic examination for valvular masses appears indicated in patients with circulating LA, especially when the cerebral
events involve several vascular distributions. At the
present time optimal management of patients with circulating LA and cerebral ischemic events is uncertain,
but oral treatment with anticoagulants has been associated with a decrease in the risk of recurrent episodes
and our findings support this empirical recommendation 161.
References
1. Levine SR, Welch KMA. Cerebrovascular ischemia associated
with lupus anticoagulant. Stroke 1987;18:257-263
2. Hart RG, Miller VT, Coull BM, Bril V. Cerebral infarction associated with lupus anticoagulants-preliminary report.
Stroke 1984;15:114-1 18
3. Anderson D, Bell D, Lodge R, Grant E. Recurrent cerebral
ischemia and mitral valve vegetation in a patient with antiphospholipid antibodies. J Rheumatol 1987;14:839-841
4. DAlton JG, Preston DN, Bormanis J, et al. Multiple transient
ischemic attacks, lupus anticoagulant and verrucous endocarditis. Stroke 1985;16:512-514
5. Ford PM, Ford SE, Lillierap DP. Association of lupus anticoagulant with severe valvular heart disease in systemic lupus erythematosus. J Rheumatol 1988;15:597-600
6. Levine SR, Welch KMA. The spectrum of neurological disease
associated with antiphospholipid antibodies: lupus anticoagulants and anticardiolipin antibodies. Arch Neurol 1987;44:876883
7. Cerebral Embolism Task Force. Cardiogenic brain embolism.
Arch Neurol 1986;43:71-84
8. Landi G, Calloni M, Sabbadini M, et al. Recurrent ischemic
attacks in two young adults with lupus anticoagulant. Stroke
1983;14:377-379
9. Jacobson DM, Lewis JH, Bontempo FA, et al. Recurrent cerebral infarctions in two brothers with antiphospholipid antibodies
that block coagulation reactions. Stroke 1986;17:98-102
10. Levine SR, Kim S, Deegan MJ, Welch KMA. Ischemic stroke
associated with anticardiolipin antibodies. Stroke 1987,18:
1101-1106
11. Gorelick PB, Rusinowitz MS, Tiku M, et al. Embolic stroke
complicating systemic lupus erythematosus. Arch Neurol
1985;42:813-815
12. Asherson RA, Lubbe WF. Cerebral and valve lesions in SLE
association with antiphospholipid antibodies. J Rheumatol
1988;15:539-543
Hyperkalemic Periodic
P&lysis in Gordon's
Svndrome: A Possible
Defect in Atrial Natriuretic
Peptide Function
J. W. Pasman, MD,* F. J. M. Gabreels, MD, PhD,"
B. Semmekrot, MD,t W. 0. Renier, MD, PhD,L
and L. A. H. Monnens, MD, PhDt
We present the case of a 14-year-old boy who had secondary hyperkalemic periodic paralysis caused by Gordon's syndrome. This syndrome consists of hypertension,
tubular acidosis, and hyperkalemia with normal glomerular filtration rate. The pathophysiological mechanism
is still unknown. Pathophysiologicalstudies suggest that
in this disorder the kidney lacks sensitivity to atrial natriuretic peptide. After treatment with hydrochlorothiazide, serum potassium and plasma aldosterone values,
plasma renin activity, and blood pressure became normal and the attacks of periodic paralysis disappeared.
Pasman JW, Gabreels FJM, Semmekrot B, Renier WO,
Monnens LAH. Hyperkalemic periodic paralysis in
Gordon's syndrome: a possible defect
in atrial natriuretic peptide function.
Ann Neurol 1989;26:392-395
Periodic paralysis can be associated with hypokalemia,
normokalemia, and hyperkalemia. The hyperkalemic
periodic paralysis can be classified in a primary and a
secondary form. The primary form is characterized by
an autosomal dominant inheritance, although sporadic
cases have been reported 11-31. The secondary form
of hyperkalemic periodic paralysis is associated with
endocrine dysfunction (e.g., hypoaldosteronism) and
disorders affecting potassium metabolism (e.g., renal
From the Institutes of "Neurology and ?Pediatrics, University Hospital Nijmegen, The Netherlands.
Received Jul 7, 1988, and in revised form Dec 21. Accepted for
publication Feb 7, 1989.
Address correspondence to Dr Gabreels, Institute of Neurology,
University Hospital Nijmegen, PO Box 9101,6500 HE Nijmegen,
The Netherlands.
392 Copyright 0 1989 by the American Neurological A ssociation
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