Alternating facial palsy as presenting symptom of intimal fibroplasia

Facial nerve palsy is a common symptom in children with a broad panel of differential diagnoses, however alternating facial palsy is rarely reported and should always be suspicious of a systemic disorder. We present the case of a 1 year old boy, who exhibited recurrent alternating facial nerve palsy and one brief and transient episode of unilateral weakness. Routine examinations during the first two episodes including infectiological work-up and MRA of the brain were normal. During the third episode extremely high blood pressure (MAP of 200mmHg) was noted associated with fundoscopic and cardiac changes. Ultrasound showed dysplasia of the left kidney with compensatory hypertrophy of the renal unit. Homozygous MTHFR-mutation was discovered as additional risk factor for thromboses. As blood pressure normalisation could not be achieved by antihypertensive drugs, left heminephrectomy was performed. Histology revealed intimal fibroplasia of large and small vessels with multiple thromboses of the renal parenchyma. Clinically blood pressure was stable on a high level, no crises were noted postoperatively, but facial palsy was still present. In the fourth postoperative week the patient developed macrohematuria secondary to left renal vein thrombosis, so total nephrectomy was performed. Although blood pressure was now completely normalized, facial palsy was almost unchanged. With starting anticoagulation we hope to improve existing and prevent further neurological sequelae. We propose that alternating facial palsy especially if aggravated by thrombophilia could be a presenting symptom of intimal fibroplasia.