Page 88 - BH-2-3

Brain & Heart Thrombosis, essential thrombocythemia, and recurrent stroke

National Institutes of Health Stroke Scale (NIHSS) score since he was capable of walking independently despite the
was 18 during the symptom presentation. presentations of mild dysarthria and ataxia.
The magnetic resonance imaging (MRI) of the brain The patient was diagnosed with essential
(the time interval between the onset of symptoms and the thrombocythemia (CALR positive in genetic test) along
MRI was 5 h) revealed a left-sided acute evolving infarct with beta-thalassemia trait and was found to be positive
in the central pons, with restriction in diffusion-weighted for MTHFR gene mutation. In the beginning, hematologist
imaging (DWI) without the corresponding changes in prescribed him hydroxyurea (1 g once daily). The
fluid-attenuated inversion recovery (FLAIR), accompanied coagulation parameters, markers of hypercoagulability,
by old infarcts at the right centrum semiovale and putamen. and vasculitis markers of the patient were within limits and
There was an abrupt cut-off of the proximal part of the he showed no angiographic evidence of arterial dissection.
basilar trunk with blooming thrombus in the proximal and Transesophageal echocardiography was conducted to
mid-basilar trunk with the reformation of the distal basilar look specifically for any left atrial appendage thrombus,
trunk, bilateral superior cerebellar, and posterior cerebellar but the test ended up with negative finding. Repeated
arteries (Figure 1). Based on the DWI-FLAIR mismatch prolonged cardiac monitoring was also performed to
results, he was given thrombolytic agent tenecteplase, and detect occurrence of atrial fibrillation (AF), but the
afterward, his condition improved dramatically, with the results were not remarkable. It is worthy to mention that
NIHSS score improving from 18 to 2. hypertrophic cardiomyopathy, with or without AF, is an
A computed tomography angiogram (CTA) which was important cause of stroke. In a study on 32,206 patients
done after thrombolysis revealed the same result as in the with isolated hypertrophic cardiomyopathy, 38.8% of
1
magnetic resonance angiography, which was done just them had AF and 7.7% had ischemic stroke. To further
before the thrombolysis during this presentation with acute unravel in this direction, we performed MR vessel wall
stroke. The CTA further unveiled eccentrically calcified imaging on the patient (Figure 2), but the procedure did
plaques in both carotid bulbs, cavernous, supra-clinoid not yield significant or meaningful findings that would be
segments of both internal carotid arteries, and the V4 of benefit to the diagnosis. Thus, in this case, we regarded
segment of the right vertebral artery with no hemodynamic stroke mechanism as the possible pathogenic pathway
stenosis. Considering the pros and cons of the current contributing to intracranial atherosclerosis (ICAD).
NIHSS, we did not proceed with administering endovascular The patient was discharged home with aspirin 75 mg,
therapy in the patient as we reasoned that the occlusion clopidogrel 75 mg, and 80 mg of atorvastatin per day; he
could be chronic in view of the dramatically improved was reminded that he had heightened the risk for vascular
NIHSS even with such a big thrombotic occlusion, which pathology and multiple atherosclerotic plaques.
implied sufficient collaterals engaging in compensatory Sixteen days later, he suffered from another episode
mechanisms. With a low NIHSS, we were worried that an of acute dysarthria, right-sided hemiplegia, and transient
attempt for endovascular therapy might dislodge the clot hearing impairment (NIHSS of 17 at our emergency room,
(with the intact proximal and distal flow) and cause an 2 h after the ictus). MRI revealed evolving acute infarct at
unnecessary deterioration. Thus, we kept the patient under the left paramedian pons and right lateral aspect of pons
close scrutiny and provided immediate interventions in next to the cerebellar hemisphere (no corresponding
case of any new deficit. When his NIHSS improved to 1 change in FLAIR) (Figure 3). We injected the patient
(MRS of two), the patient was discharged from the hospital with tenecteplase and his NIHSS score improved to 2.

A B C

Figure 1. Magnetic resonance imaging of the brain revealed acute evolving infarct in the central pons. (A and B) (more inclined to left-sidedness) with an
abrupt cut-off of the proximal part of the basilar trunk with non-opacification of proximal and mid-basilar trunk due to the thrombus (marked with blue
arrow). (C) Reformation of the distal basilar trunk, bilateral superior cerebellar and posterior cerebellar arteries.

Volume 2 Issue 3 (2024) 2 doi: 10.36922/bh.3741