Hypertensive Emergency with Medullary and Spinal Hemorrhage in Turner Syndrome

Excerpt

An 8-y-female with Turner syndrome (TS) presented with an episode of seizure 7 d back followed by weakness of trunk and lower limbs. At admission, the blood pressure was 184/111 mmHg with no difference in all 4 limbs. Neurological examination revealed normal sensorium; lower limb weakness (power of 2/5 Medical Research Council grading), exaggerated deep tendon reflexes, and extensor plantar reflex; saddle anesthesia; and intermittent urinary retention suggesting conus medullaris syndrome (CMS). Magnetic resonance imaging (MRI) showed medullary and spinal hemorrhage (Fig. 1). Hypertensive emergency was managed in pediatric intensive care unit (PICU) with intravenous infusion of sodium nitroprusside and labetalol; and oral labetalol, amlodipine, enalapril, and clonidine. Complete blood counts, renal, liver, and thyroid function tests; serum cortisol; and ultrasonography of kidney, and renal Doppler were normal. Echocardiography revealed left ventricular hypertrophy and fundus examination showed Grade 4 hypertensive retinopathy. Angiography ruled out vascular abnormalities in aorta, cerebral, spinal or renal vessels. Aldosterone/renin ratio was 4.8 (Normal < 20), aldosterone level 26.2 ng/dL (Normal: 3–16 ng/dL), and plasma renin activity was 5.41 ng/mL/h (Normal: 0.15–2.33 ng/mL/h in supine position). She was discharged after a hospital stay of 36 d with normal sensorium, lower limb power of 4/5, no urinary retention, and blood pressure at 90th centile.

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