A 65-year-old Caucasian woman presented to her local hospital with sudden-onset, bifrontal, pounding headache described as "getting hit in the head with an axe." The headache was the worst of her life and did not improve after she took acetaminophen, caffeine, and butalbital. There was hyperacusis, photophobia and nausea. Noncontrast head computed tomography (CT) and brain magnetic resonance imaging (MRI) at the time of admission were normal and she was treated with prednisone for presumed intractable migraine. Aside from a similar but milder headache one week prior to her current presentation, she reported only a sparse past history of migraines that ceased after her hysterectomy and no family history of migraines or strokes. She had hyperlipidemia treated with simvastatin 40 mg daily, lumbar spinal compression fractures, multiple miscarriages and depression that had been treated for several years with citalopram 20 mg daily. On further questioning, our patient reported taking the weight-loss supplement Hydroxycut beginning two weeks prior to her thunderclap headache. On admission, her body mass index was 22.3, and she was normotensive on lisinopril 10 mg daily. She had not previously been on lisinopril, which was presumably initiated at the outside hospital for prednisone-induced hypertension. We held the lisinopril for the duration of her hospitalization given her normal to low blood pressures. Her fasting lipid panel revealed cholesterol 223 mg/dL, triglycerides 141 mg/dL, high density lipoprotein 61 mg/dL, low density lipoprotein 134 mg/dL, very low density lipoprotein 28 mg/dL and lipoprotein(a) 6 mg/dL.
Two days after admission, she developed bilateral leg weakness and left-sided visual disturbances that she described as "blank lines." A repeat MRI revealed areas of restricted diffusion consistent with acute infarcts in the bilateral anterior cerebral artery territories and in her right occipital lobe (Figure ). The following investigations were unrevealing: hypercoagulability studies, rheumatic and vasculitic screening labs, magnetic resonance venography, transthoracic echocardiogram with bubble contrast, and Holter monitoring. LA lumbar puncture, performed while our patient was being treated with prednisone, revealed 0 white blood cells (WBC), 48 red blood cells (RBC), cerebrospinal fluid (CSF) protein 27 mg/dL, glucose 81 mg/dL and no xanthochromia. CT angiography (CTA) was obtained, which revealed multifocal segmental cerebral artery vasoconstriction, most prominent in the bilateral anterior and posterior cerebral arteries (Figures and ).
RVCS-related ischemic strokes. Diffusion-weighted MRI (A, B, C) and apparent diffusion coefficient maps (D, E, F) revealed lesions in the right occipital lobe and bilateral anterior cerebral artery territories consistent with ischemic strokes.
Figure 2 RCVS on computed tomography angiography. CTA obtained during hospitalization showed multifocal segmental vasoconstrictions most prominent in the bilateral anterior (A) and posterior (B) cerebral arteries. Follow-up CTA six weeks after discharge revealed (more ...)
We made the diagnosis of RCVS and began treatment with nimodipine 30 mg three times daily. Over the subsequent days, her headache resolved and her vision and leg weakness improved. Our patient's blood pressures at admission and prior to starting nimodipine were 92-116/54-58 mmHg on no antihypertensive medications. After beginning nimodipine for RCVS, her systolic blood pressures ranged from the high 80s to low 100s (mmHg). We administered intravenous fluid bolus as needed to keep her systolic blood pressure above 90 mmHg, in an effort to balance maintaining adequate cerebral perfusion while continuing nimodipine treatment for RCVS. Our patient tolerated this well without any clinical decline or symptomatic hypotension.
She was discharged on nimodipine and advised not to take Hydroxycut and citalopram, which had been discontinued when a diagnosis of RCVS was first suspected. At the time of discharge, her systolic blood pressures remained in the 90s to low 100s mmHg. Therefore, she was advised to measure her blood pressure at home and take nimodipine only if systolic blood pressure was over 100 mmHg. Following discharge, our patient experienced no headaches and no recurrence of her presenting symptoms. At a follow-up appointment, she had no residual leg weakness and significant improvement of her left visual field deficit, although she reported that her vision had not returned to her baseline. CTA performed six weeks after discharge showed marked resolution of cerebral vasoconstriction, confirming the diagnosis of RCVS (Figures and ).