Can giant cell arteritis cause hemiplegia in an older adult?

Can Giant Cell Arteritis Cause Hemiplegia?

Yes, giant cell arteritis (GCA) can cause hemiplegia through ischemic stroke, though this is an uncommon presentation that requires immediate recognition and treatment to prevent devastating neurological outcomes.

Mechanism of Stroke in GCA

GCA causes hemiplegia through vasculitic involvement of cerebral vessels leading to ischemic stroke. The inflammatory process results in arterial wall thickening, intimal hyperplasia, and progressive luminal occlusion, which can affect both cranial and large vessels including the vertebral and carotid arteries 1, 2.

• Stroke occurs in 3-4% of GCA patients, typically from vertebral artery stenosis or occlusion 3
• The American Heart Association recognizes stroke as a less common but serious ischemic complication of GCA 2
• Posterior circulation strokes are particularly associated with GCA, often involving vertebral artery occlusion 4, 3

Clinical Recognition

Key Diagnostic Clues

When an older adult presents with hemiplegia, consider GCA if:

• Age >50 years (GCA does not occur below this age) 5
• Markedly elevated inflammatory markers: ESR >40 mm/h (typically >100 mm/h) and elevated CRP are present in >95% of cases 5, 4
• Associated cranial symptoms: New headache (present in 66-90% of cases), jaw claudication (LR+ 4.90), scalp tenderness (LR+ 3.14), or visual disturbances 5
• Systemic symptoms: Fever, weight loss, or polymyalgia rheumatica 6

Atypical Presentations

Stroke as the initial presentation of GCA is exceedingly rare but well-documented 4. A case report describes an 84-year-old presenting with dysarthria, dysphagia, facial droop, and posterior circulation stroke syndrome with ESR 112 mm/h and CRP 110 mg/L, ultimately diagnosed with GCA by temporal artery biopsy 4.

Immediate Management Algorithm

Step 1: Initiate Treatment Immediately

Do not delay treatment while awaiting diagnostic confirmation 1.

• For stroke/TIA with suspected GCA: Start high-dose oral glucocorticoids (prednisone 40-60 mg daily) immediately 1, 5
• For threatened vision loss or critical cranial ischemia: Use IV pulse methylprednisolone 1000 mg daily for 3 days, then transition to oral glucocorticoids 1, 5
• Add low-dose aspirin (75-150 mg daily) for all GCA patients to reduce cardiovascular and cerebrovascular events 5

Step 2: Confirm Diagnosis

While treatment is ongoing:

• Temporal artery biopsy (≥1 cm specimen) remains the gold standard 1, 5
• Vascular imaging (ultrasound with "halo sign," MR/CT angiography, or PET) can confirm diagnosis if biopsy is negative or unavailable 1, 5
• Baseline large vessel imaging (MR or CT angiography of neck/chest/abdomen/pelvis) to assess for large vessel involvement 1, 5

Step 3: Add Steroid-Sparing Agents

For patients with ischemic stroke or TIA from GCA, adjunctive therapy is reasonable to lower recurrent stroke risk 1:

• Tocilizumab is the preferred adjunctive agent, demonstrating efficacy in reducing glucocorticoid requirements and preventing relapses 1, 5
• Methotrexate is an alternative when tocilizumab is unavailable or contraindicated 1, 5
• Avoid infliximab: It is associated with increased disease activity and recurrent symptoms 1

Critical Pitfalls to Avoid

Common Diagnostic Errors

• Attributing stroke to atherosclerosis alone in older adults without checking inflammatory markers 4
• Waiting for temporal artery biopsy results before starting treatment—this can lead to irreversible vision loss or recurrent stroke 1, 5
• Dismissing GCA because of atypical presentation—stroke, especially posterior circulation, can be the initial manifestation 4, 3
• Assuming normal inflammatory markers exclude GCA—while rare, 5% of cases may have normal ESR/CRP 5

Treatment Complications

• Rapid glucocorticoid taper can cause disease exacerbation; taper slowly over months to years, targeting ≤5 mg/day after one year 1, 5
• Prolonged high-dose glucocorticoids without steroid-sparing agents increases risk of infections, diabetes, osteoporosis, and cataracts 1, 6
• Initiate bone protection (calcium, vitamin D, bisphosphonates) for all patients on glucocorticoids 5

Monitoring and Follow-up

• Clinical symptoms and inflammatory markers (ESR/CRP) should guide treatment decisions throughout the disease course 1, 5
• Periodic vascular imaging for patients with known large-vessel involvement to assess for aneurysms and stenoses 1, 5
• Up to 60% of patients experience relapses during glucocorticoid tapering, requiring dose escalation or addition of immunosuppressive agents 1

Endovascular Intervention

In rare cases of progressive vertebral artery occlusion despite medical therapy, endovascular angioplasty and stenting may be considered, though this is not yet established as standard treatment 3. One case report documented successful revascularization with significant neurological improvement within 3 days in a patient who developed complete bilateral vertebral artery occlusion with hemiplegia despite immunosuppression 3.