Peripartum angiopathy with simultaneous sinus venous thrombosis, cervical artery dissection and cerebral arterial vasoconstriction

Abstract

A 38 year old gravida 2, para 1 was admitted to our hospital at 38 ? 4 weeks with a 2-week long history of intractable headache of undulating intensity, radiating from the neck to the back of her head. Magnetic resonance imaging (MRI) showed a complete thrombosis of the right sigmoid and adjacent transverse sinus, and a partial thrombosis of the superior sagittal sinus (Fig. 1a). Intravenous heparin was started. Screening for coagulopathies yielded elevated homocysteine levels and a positive factor V mutation. On the next day, a healthy girl (birth weight 2,785 g) was delivered via cesarean section (day 0). After 2 days of satisfactory pain control with intravenous acetaminophen, without any apparent trigger, the patient developed a maximum-intensity holocephalic headache of peracute, explosive onset, subsiding over several hours. Concomitantly, she developed a hypertensive emergency with systolic blood pressures up to 240 mmHg and a transient left-sided weakness. The emergently performed MRI showed an improved venous flow signal; the arterial time-of-flight angiogram was normal (day 3). Because of the need for analgesics, the patient was started on an 11-day course of bromocriptine to inhibit lactation (given on days 3–13). Further, but with time decreasingly intense headache episodes occurred within the following 9 days (days 4–12); afterwards, no further headache episodes were noted. Moreover, the persistent baseline headache of about three points on the visual analog scale gradually diminished. On day 6 after childbirth, color-coded duplex sonography was performed and a stenosis of the distal right internal carotid artery was noted. The contrast-enhanced MRA revealed a dissection of the arterial wall (Fig. 1b), and, moreover, multiple stenoses in bilateral posterior and branches of both middle cerebral arteries. There was no Horner’s sign, pain in the lateral region of the throat or involvement of cranial nerves. A therapy with nimodipine, magnesium sulfate and simvastatin was initiated (day 7). Tests for autoimmune vasculitis (ANA, ANCA, complement levels, anti-phospholipid antibodies) and cerebrospinal fluid analysis (day 8) were unremarkable. In the follow-up MRI, the lumina of the sinuses were completely restored, but worsened and more proximally located stenoses were seen in all large arteries of both the anterior and the posterior circulation (day 15, Fig. 1c). Prednisolone 100 mg daily was given additionally. Over the following 2 weeks, there was a gradual improvement of the stenoses as assessed by repeated ultrasound examinations and MRI. The patient was started on warfarin and discharged on day 22 postpartum without neurological deficits; steroids and nimodipine were tapered. Seven months after, the MRI including time-offlight arteriogram was normal (Fig. 1d). Cerebral sinus venous thrombosis (CSVT) is a well known cause for headache and stroke in the peripartum period [1]. Coagulation disorders such as the factor V Leiden mutation and hyperhomocysteinemia are further risk factors [2, 3]. Reversible cerebral vasoconstriction syndromes (RCVS) are rare, but also associated with the early postpartum period [4], and in this case are often referred to as M. Hoeren (&) S. Strumpell C. Weiller M. Reinhard Department of Neurology, University of Freiburg, Breisacher Str. 64, 79106 Freiburg, Germany e-mail: markus.hoeren@uniklinik-freiburg.de