What is the emergent evaluation and management for a patient presenting with a unilateral headache, ipsilateral sudden vision loss, and contralateral hand numbness?

Emergent Evaluation and Management of Unilateral Headache with Vision Loss and Contralateral Hand Numbness

This presentation represents a neurological emergency requiring immediate stroke center evaluation, as the combination of unilateral headache, ipsilateral sudden vision loss ("blackening"), and contralateral hand numbness strongly suggests acute ischemic stroke affecting both the cerebral hemisphere and retinal circulation. 1, 2

Immediate Triage and Referral

• Transfer the patient immediately to a certified stroke center emergency department without performing extensive outpatient workup, as this presentation has a 10% risk of completed stroke within the first week, with highest risk in the first 48 hours. 1, 2

• The combination of contralateral numbness with unilateral symptoms carries a 72% probability of stroke when accompanied by other neurological deficits. 1

• If an ophthalmologist confirms retinal artery occlusion, send the patient directly to the stroke center ED with a note stating "Ocular Stroke" rather than delaying for additional ophthalmologic testing. 3, 2

Critical Initial Emergency Department Assessment

Rapid Stroke Evaluation

• Perform Cincinnati Prehospital Stroke Scale assessment immediately: evaluate facial droop, arm drift, and speech abnormalities, as any abnormality indicates 72% stroke probability. 1, 4

• Check bilateral radial pulses and blood pressure in both arms to differentiate stroke from acute arterial occlusion; a difference >20 mmHg between arms confirms significant arterial compromise. 1

• Assess the "6 P's" of acute limb ischemia if vascular occlusion is suspected: Pain, Pallor, Pulselessness, Paresthesias, Poikilothermia (cool limb), and Paralysis. 1

Vision-Specific Assessment

• Confirm whether vision loss is monocular (retinal) versus binocular (cortical) by having the patient cover each eye separately, as this fundamentally changes the differential diagnosis. 2

• Perform funduscopic examination to identify the "cherry-red spot" of central retinal artery occlusion or retinal whitening of branch retinal artery occlusion. 3

• Up to 25% of central retinal artery occlusion patients have concurrent silent brain infarction on MRI, making neuroimaging mandatory even if the vision loss appears purely ocular. 3, 2

Immediate Neuroimaging Protocol

Primary Imaging

• Brain MRI with diffusion-weighted imaging is the preferred initial imaging modality to detect acute ischemic changes and must be performed immediately. 1, 2, 4

• MRI differentiates ischemic from hemorrhagic stroke, which fundamentally changes treatment approach. 1

• If MRI is unavailable or contraindicated, perform non-contrast CT head to rule out hemorrhage, though CT is less sensitive for acute ischemia. 1, 2, 4

Vascular Imaging

• CT angiography or MR angiography of the head and neck must be included to evaluate for carotid and vertebral artery disease, as clinically significant carotid stenosis is present in up to 70% of symptomatic retinal vascular events. 3, 2, 4

• MR venography should be considered if conventional imaging is negative, as isolated cortical vein thrombosis can present with headache, focal neurological deficits, and visual symptoms. 3

• The "cord sign" (linear hyperdensity) on CT or signal loss on T2-susceptibility-weighted sequences on MRI may indicate cortical vein thrombosis. 3

Essential Laboratory Evaluation

• Obtain complete blood count with platelets, basic metabolic panel, coagulation studies (PT/PTT), hemoglobin A1c, and fasting lipid panel immediately. 2, 4

• In patients over 50 years old, measure ESR and CRP urgently to screen for giant cell arteritis, as ESR >60 mm/h has high likelihood ratio for this diagnosis. 2

• If giant cell arteritis is suspected based on age >50, jaw claudication, scalp tenderness, or elevated inflammatory markers, initiate empiric high-dose corticosteroids immediately without waiting for temporal artery biopsy to prevent vision loss in the contralateral eye. 2

Cardiac Assessment

• Obtain 12-lead electrocardiogram immediately on presentation. 2

• Transthoracic echocardiography is reasonable for all patients to identify cardiac sources of emboli. 2

• Consider prolonged cardiac monitoring (inpatient telemetry or outpatient Holter) if the underlying cause remains unclear after initial imaging. 2

Critical Differential Diagnoses to Consider

Stroke Syndromes

• Posterior circulation stroke affecting the occipital cortex (causing cortical blindness) and contralateral sensory pathways is a key consideration. 4

• Hemispheric stroke with concurrent retinal artery occlusion from carotid disease represents "ocular stroke" requiring identical stroke protocol management. 3

Isolated Cortical Vein Thrombosis

• ICVT presents with headache (most common symptom, 90%), focal neurological deficits (62%), and can cause intracranial hemorrhage. 3

• Ipsilateral dural thickening and enhancement on MRI may indicate ICVT and warrants venographic study (CT venography or MR venography). 3

• If venographic studies are inconclusive with high clinical suspicion, catheter cerebral angiography is indicated as the gold standard. 3

Migraine with Aura (Lower Priority)

• Migraine with aura can cause unilateral headache with visual and sensory symptoms, but symptoms typically develop gradually over 5-60 minutes and resolve within 60 minutes. 3

• The persistence of "blackening" vision (rather than transient visual disturbances) and the acute onset make stroke far more likely than migraine. 3

• Never assume migraine in the acute setting without first ruling out stroke, as misdiagnosis can be catastrophic. 5

Treatment Decisions Based on Imaging Results

If Ischemic Stroke Confirmed

• Intravenous alteplase (0.9 mg/kg with 10% bolus over 1 minute, remainder over 59 minutes) should be administered if patient presents within 4.5 hours of symptom onset with no contraindications. 3

• Observational data shows 50% clinical recovery rate when thrombolysis is administered within 4.5 hours for retinal artery occlusion. 3

• Dual antiplatelet therapy (aspirin + clopidogrel 75 mg) should be initiated for the first 21 days in patients with symptomatic carotid stenosis not undergoing revascularization, followed by long-term single antiplatelet therapy. 1

• Carotid endarterectomy must be considered for extracranial carotid stenosis >70%. 1

If Cortical Vein Thrombosis Confirmed

• Systemic anticoagulation is the standard treatment, though hemorrhagic transformation may complicate management. 3

• If intracranial hemorrhage develops or expands despite anticoagulation, craniotomy and hematoma evacuation may be necessary. 3

If Giant Cell Arteritis Suspected

• Initiate high-dose corticosteroids (typically prednisone 60-80 mg daily) immediately without waiting for temporal artery biopsy results. 2

• Temporal artery biopsy should be performed within 1-2 weeks of steroid initiation but should never delay treatment. 2

Admission Criteria and Observation

• Hospitalize patients presenting within 72 hours if any of the following are present: abnormal brain DWI-MRI showing acute cerebral infarction, large artery atherosclerosis on vascular imaging, abnormal cardiac evaluation, or recurrent episodes. 3

• Patients should receive cardiac monitoring during a 23-hour observation period as part of standard stroke protocol. 3

• If no embolic cause is identified after comprehensive workup, discharge with optimal secondary stroke prevention (antithrombotic agent, statin, blood pressure control) and arrange outpatient neurology follow-up within 2 weeks. 3

Common Pitfalls to Avoid

• Never assume vision improvement eliminates stroke risk, as stroke risk remains highest in the first 7 days even if vision recovers. 2

• Never perform extensive ophthalmologic workup before stroke center referral if vascular etiology is suspected, as time to treatment is critical. 3, 2

• Never underestimate cardiovascular risk, as new significant cardiovascular conditions are discovered in up to 70% of central retinal artery occlusion patients. 2

• Never delay giant cell arteritis treatment for biopsy in patients over 50 with appropriate clinical features. 2

• Never diagnose migraine in the emergency setting without first excluding stroke with appropriate neuroimaging, as sudden severe headache with focal deficits requires urgent evaluation. 6, 5, 7