Clinical Reasoning Series: A 70-Year-Old Man With Systemic Illness Related Strokes Refractory to Medical Treatment Managed With Intracranial Stent
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A 70-year-old man with atrial fibrillation and a prior stroke presented with acute neurological deficits and a high NIHSS score, raising suspicion for an acute large vessel occlusion. However, imaging revealed severe, rapidly progressive stenosis of the bilateral supraclinoid internal carotid arteries—changes not seen on prior imaging—alongside watershed infarcts and subarachnoid hemorrhage. This atypical pattern prompted a broad differential, including vasculitis and reversible cerebral vasoconstriction syndrome (RCVS). The absence of thunderclap headache, lack of typical RCVS angiographic features (like 'sausage-string' appearance), and the presence of temporal headache, jaw claudication, and systemic inflammation shifted the focus toward vasculitis. Despite negative ANCA and rheumatoid factor, biopsy showed neutrophilic inflammation without giant cells, leading to a diagnosis of polyarteritis nodosa. Aggressive medical therapy with steroids and cyclophosphamide failed to halt progression, and the patient suffered recurrent strokes. In the face of refractory symptoms and life-threatening stenosis, the team performed sequential balloon angioplasty and ultimately placed an intracranial stent in the right ICA. The patient recovered fully, with complete resolution of stenosis on follow-up angiography and full functional independence at one year.
Watershed infarcts with bilateral intracranial stenosis in a patient with systemic symptoms should raise suspicion for vasculitis, not just atherosclerosis.
Giant cell arteritis is not the only cause of intracranial vasculitis—polyarteritis nodosa must be considered, especially with negative ANCA and neutrophilic inflammation on biopsy.
A negative temporal artery biopsy does not rule out giant cell arteritis, and the absence of giant cells is common in routine samples.
Rapidly progressive intracranial stenosis over three years is highly atypical for atherosclerosis and should prompt investigation for vasculitis.
In refractory CNS vasculitis with life-threatening stenosis, intracranial stenting may be a safe and effective bridge to recovery when medical therapy is too slow.
…and 3 more takeaways available in PodZeus
Introduction to the Clinical Reasoning Podcast
Host Zohib Siddiqui introduces the Neurology® Podcast's Clinical Reasoning series and welcomes Laurence Poirier, a fifth-year neurology resident and author of a case study on a complex stroke case involving systemic vasculitis.
Patient Presentation and Initial Localization
The 70-year-old male presents with acute right-sided hemianopia, weakness, aphasia, and sensory loss, with an NIHSS of 12. Localization points to a large left MCA territory stroke, but the absence of a clear large vessel occlusion on imaging raises concerns about atypical causes.
Imaging Findings and Diagnostic Dilemma
CT and CTA reveal no acute ischemia or large vessel occlusion but show severe bilateral supraclinoid ICA stenosis—worsening over three years. MRI confirms watershed infarcts and subarachnoid hemorrhage, prompting a search for non-atherosclerotic causes like vasculitis.
Differential Diagnosis: RCVS vs. Vasculitis
The team rules out RCVS due to lack of thunderclap headache and absence of 'sausage-string' angiographic pattern. The presence of temporal headache, jaw claudication, and systemic inflammation shifts focus to vasculitis, including giant cell arteritis and polyarteritis nodosa.
Workup and Final Diagnosis of Polyarteritis Nodosa
Extensive autoimmune workup is negative for ANCA, ANA, and rheumatoid factors. Biopsy shows neutrophilic inflammation without giant cells. Despite negative serologies, the clinical and pathological picture supports polyarteritis nodosa as the most likely diagnosis.
“If we didn't perform any intervention, he could have had a really big stroke.”
“Even patients with typical vascular risk factors can still present with atypical causes of stroke.”
“The most common stroke type in polyarteritis nodosa is actually lacunar, unlike the cortical strokes that we saw in that patient.”
Hosts
Guest
Laurence Poirier
person
Zohib Siddiqui
person
Neurology® Podcast
organization
Neurology
other
Stacey Clardy
person
prednisone
product
temporal artery biopsy
other
cyclophosphamide
product
University of Ottawa
organization
aspirin
product
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