A 43-year-old right-handed African American female with a history of migraines was followed clinically for SLE for 3 years prior to presentation. Her initial diagnosis was based on American College of Rheumatology criteria of: Antinuclear antibodies, dsDNA antibodies, arthritis, and serositis with pericarditis. Recent disease activity was moderate, with arthralgias, occasional fevers, oral ulcers, fatigue, a truncal maculopapular rash, and abnormal C4, dsDNA, and c-reactive protein. Medications included prednisone, hydroxychloroquine, and azathioprine.
The patient presented to the emergency room after a syncopal event with headache, dizziness, and nausea. She had no focal neurologic deficits, improved on migraine medications, and was discharged. Throughout the following week, headaches and fatigue persisted; however, her family reported that these symptoms were comparable to prior SLE flares.
Six days later, the patient was found unresponsive and with one episode of bowel incontinence. She was taken to the emergency department, where she was arousable and oriented. Neurologic examination was significant for moderate expressive aphasia, left-sided hemineglect, flattening of the left nasolabial fold, 4/5 strength in the right upper and lower extremities, and 0/5 strength in the left upper and lower extremities. Hoffman's and Babinski's signs were negative, no clonus was observed, and her remaining cranial nerves were intact. Musculoskeletal examination was significant for trace bilateral dorsal hand edema, mild proximal interpharyngeal joint tenderness without synovitis, and a papular, erythematous, scaling rash over her thighs bilaterally.
Computed tomography (CT) and CT angiography demonstrated multiple areas of acute infarct, most prominently in the anterior cerebral artery (ACA) distribution, with minimal interhemispheric SAH and a 8 x 5 mm bilobed saccular aneurysm located at the junction of the left ACA and ACoA [Figure 1a and b]. The patient was diagnosed with Hunt and Hess grade II SAH and admitted to the neurosurgical intensive care unit. Initial medical management included oral levetiracetam 500 mg, oral nimodipine 60 mg, and three doses of intravenous methylprednisolone 16 mg/kg. Azathioprine was held, as consideration of alternative immunosuppression with cyclophosphamide or mycophenolate mofetil was deliberated. Since the patient was postbleed day six from initial rupture on admission hospital, the decision was made to withhold endovascular intervention as long as she remained clinically stable and within the vasospasm window.
Five days after admission, the patient became acutely unresponsive, and repeat head CT demonstrated new subarachnoid blood. She was taken emergently for angiography and embolization of the aneurysm [Figure 2a and b]. Vasospasm involving the right internal carotid artery (ICA), bilateral ACA, ACoA, supraclinoid left ICA, and right posterior cerebral artery was observed. Of note, catheterization of the A1 segment was difficult, due to severe vasospasm and arterial stenosis.
Postembolization, the patient awoke with dense left hemiparesis, left hemineglect, and severe expressive aphasia. Over several days, the patient's neglect and aphasia resolved, and she was discharged to acute rehabilitation in stable condition. At follow-up one month after discharge, she demonstrated no signs of aphasia, and was able to converse and interact at her prerupture baseline. She had recovered antigravity strength in her left extremities, and her facial nerve deficit had resolved.
Review of imaging conducted as part of a headache workup 20 months prior to the current admission demonstrated a normal ACoA complex, as per multiple neuroradiologists at our institution [Figure 3]. A renal ultrasound was negative for polycystic kidney disease. Additional history did not reveal any other medical conditions or significant risk factors for aneurysm rupture, such as hypertension or smoking. Family history was negative for polycystic kidney disease, connective tissue disorders, SAH, intracranial aneurysms, or sudden deaths suspicious for undiagnosed aneurysm rupture.