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Cerebral Microbleeds in Lupus Anticoagulant-Hypoprothrombinemia Syndrome

Ashutosh P. Jadhav, MD, PhD1; Nima Aghaebrahim, MD1; Brian T. Jankowitz, MD2; Tudor G. Jovin, MD1
[+] Author Affiliations
1Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
2Department of Neurosurgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
JAMA Neurol. 2013;70(11):1452-1453. doi:10.1001/2013.jamaneurol.505.
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A 37-year-old woman with poorly controlled hypertension and antiphospholipid antibody syndrome developed a sudden-onset headache followed by unresponsiveness requiring intubation. On examination, the patient was able to follow commands but had near quadriplegia. Cranial nerve examination findings were notable for pinpoint pupils with limited horizontal movements and ocular bobbing. Magnetic resonance imaging of the head revealed a large pontine hemorrhage with intraventricular extension as well as multiple microbleeds in the bilateral cerebellar and cerebral hemispheres (Figure, A and B). A catheter-based cerebral angiogram demonstrated no evidence of vessel irregularities, and a brain biopsy revealed hemorrhagic microinfarcts and arteriole thickening (Figure, C). Laboratory examination revealed the presence of lupus anticoagulants and low prothrombin levels, suggestive of lupus anticoagulant-hypoprothrombinemia syndrome. The patient began treatment with high doses of steroids and electrophoresis, with follow-up studies revealing normalization of her prothrombin levels. The patient required tracheostomy and percutaneous gastrotomy. On discharge, she was able to follow commands and communicate by moving her head. She regained some strength in her arms and legs but remained dependent (modified Rankin Scale score of 4).

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Susceptibility-Weighted Angiography and Biopsy

Susceptibility-weighted angiography revealed a large hemorrhage in the pons as well as multiple microbleeds in the bilateral cerebelli (A) and cerebral hemispheres (B), and biopsy of the right frontal cortex demonstrated thickened arterioles, with morphological features being consistent with lupus-related arteriosclerosis (hematoxylin-eosin, original magnification ×10) (C).

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