Cranial nerve palsies during pregnancy require prompt attention. While a wide variety of causes may be implicated, it is of utmost importance to screen for preeclampsia. While often asymptomatic, preeclampsia can have dire consequence if untreated (1). Our report illustrates the need for thorough patient evaluation and careful follow-up in this clinical setting.
A previously healthy 32-year-old woman presented to the ophthalmology clinic with a history of recent onset horizontal binocular diplopia. Three days previously she delivered twins. The successful pregnancy required ovarian stimulation with letrozole and artificial insemination. The pregnancy, which was her first, had been uncomplicated until 36 weeks and 6 days of gestation, when she was diagnosed with asymptomatic preeclampsia, associated with hypertension up to 142/96 mm Hg, possible diabetes insipidus, increased liver enzymes [aspartate aminotransferase 122 U/L (normal, 13–39), alanine aminotransferase 89 U/L (normal, 8–31), gamma-glutamyl-transferase 63 U/L (normal, 7–33), alkaline phosphatase 247 U/L (normal, 36–110)], acute renal failure with proteinuria (urine albumin 379 mg/L (normal, <30), and increased creatinine [(150 μmol/L (normal, 42–89)]. She neither fulfilled the diagnostic criteria for HELLP (hemolysis, elevated liver enzymes, and low platelets syndrome) nor the syndrome of acute fatty liver of pregnancy, having a normal renal and liver ultrasound, normal platelet count, and fibrinogen levels.
The patient was hospitalized, and the next day, she underwent pregnancy induction with oxytocin and seizure prevention with magnesium sulfate. She did not need antihypertensive treatment, as her blood pressure remained between 130/70 and 140/100 mm Hg. During labor with uncomplicated epidural anesthesia, she reported numbness on the entire left side of her face. This was confirmed on neurologic examination along with decreased ipsilateral masseter strength. She successfully delivered twins vaginally, with 600 mL of blood loss and the only complication being uterine atony, resolving after uterine massage.
A few hours after delivery, the patient complained of facial asymmetry and was found to have a peripheral left seventh nerve palsy. The remainder of the neurological examination was normal, and review of systems was negative. Treatment was instituted of valacyclovir, 1 gm 3 times a day, and prednisone, 60 mg daily. Both medications were prescribed orally. Two days later, the patient complained of binocular horizontal diplopia, worse on left gaze, and she was found to have a left sixth nerve palsy, while the remainder of her ophthalmic examination was normal. By that time, her blood pressure was within normal range and stable.
A noncontrast brain magnetic resonance imaging study [including a constructive interference steady state sequence] and computed tomographic angiography of the head and neck failed to detect a cause for the cranial neuropathies. Additional testing with normal results included anti-DNA antibodies, extractable nuclear antigen, antinuclear antibodies, treponema pallidum enzyme immunoassay, angiotensin-converting enzyme, and serum glucose. Given the nonprogression of her symptoms, normalized blood pressure, renal and hepatic function, and negative investigations, it was decided not to do a lumbar puncture but rather to observe the patient. She continued valacyclovir and prednisone for 7 and 10 days, respectively, and her cranial nerve palsies completely resolved over 3 months.
A number of cranial neuropathies have been described in pregnancy, most frequently involving the seventh nerve followed by the sixth nerve. Specifically for pregnant patients, the diagnosis of preeclamptic neuropathy must be considered early and the appropriate investigations undertaken. Our case is unusual with simultaneous ipsilateral involvement of 3 cranial nerves.
Seventh Nerve Palsy
Seventh nerve palsy, in addition to being more common in women of reproductive age compared with men of the same age (2, 3), affects pregnant women more often than nonpregnant women (3, 4). It occurs most often in the third trimester or the puerperium (3–6) and seems to have a predilection for preeclamptic women (5, 7). The etiology of seventh palsy in pregnant women is unclear, but a number of etiologies have been proposed, including hypercoagulopathy (3, 5, 8), ovarian hormones (9), hypertension (5, 10), high extracellular fluid content (3, 5), viral infection (3, 11), and immunosuppression (3). Although the presentation is similar in pregnant and nonpregnant patients, the former seem to recover faster (12). According to some reports, corticosteroids can relieve the associated pain but do not improve recovery (4, 8, 11, 13), whereas other authors recommend early treatment with corticosteroids to achieve better outcomes (14). Use of antivirals, such as valacyclovir, may be warranted given the possibility of a viral etiology (3, 11) and, when used in combination with corticosteroids, may improve the clinical outcome (15).
Sixth Nerve Palsy
Sixth nerve palsy in pregnancy seems to be primarily linked to preeclampsia, developing either before (15) or after delivery. It may occur the same day as delivery or up to 11 days later, even with blood pressure under control with antihypertensive medication, similar to our patient (17, 18). There also are reports of sixth nerve palsy associated with pregnancy occurring with transient hypertension (19), without hypertension or preeclampsia (20), and after a febrile illness (21, 22).
During the perinatal period, both unilateral and bilateral sixth nerve palsies have been described within a few days of dural puncture secondary to epidural anesthesia followed by intracranial hypotension (23–27). These patients tend to be symptomatic, usually reporting postural headache (24, 26). Sixth nerve palsy with intracranial hypotension may be due to traction on the nerve by displacement of the brain or compression of the nerve by dura, the petrous apex or branches of the basilar artery (28).
Fifth Nerve Palsy
Trigeminal nerve involvement has been documented primarily in the setting of spinal anesthesia (29–31). It may occur in combination with Horner syndrome or with hypoglossal or facial nerve palsy. Some of the cases had an identifiable dural leak with probable intracranial hypotension. Some of the other etiological hypotheses raised include spread of the anesthetic effect in the subarachnoid space, toxic reaction to the anesthetic, and compromised blood flow to the cranial nerves secondary to sudden change in intracranial pressure (31).
We are unaware of other reported cases of symptomatic multiple cranial neuropathies in a pregnant patient with preeclampsia without a dural leak. Multiple mechanisms, alone or in combination, could explain our patient's clinical findings: hypercoagulopathy, ovarian hormones, microvascular ischemia, increased extracellular fluid content and direct or indirect effects of spinal anesthesia.
1. Brewer J, Owens MY, Wallace K, Reeves AA, Morris R, Khan M, LaMarca B, Martin JN Jr. Posterior reversible encephalopathy syndrome in 46 of 47 patients with eclampsia. Am J Obstet Gynecol. 2013;208:468.e1–e6.
2. Cunningham FG, MacDonald PC, Gant N, Leveno K, Gilstrap L. Medical and surgical complications in pregnancy. In: Williams Obstetrics, 19th edition. Norwalk, CT: Appleton & Lange, 1993.
3. Hilsinger RL Jr, Adour KK, Doty HE. Idiopathic facial paralysis, pregnancy, and the menstrual cycle. Ann Otol Rhinol Laryngol. 1975;84:433–442.
4. Robinson JR, Pou JW. Bell's palsy. A predisposition of pregnant women. Arch Otolaryngol. 1972;95:125–129.
5. Falco NA, Eriksson E. Idiopathic facial palsy in pregnancy and the puerperium. Surg Gynecol Obstet. 1989;169:337–340.
6. Shapiro JL, Yudin MH, Ray JG. Bell's palsy and tinnitus during pregnancy: predictors of pre-eclampsia? Three cases and a detailed review of the literature. Acta Otolaryngol. 1999;119:647–651.
7. Shmorgun D, Chan WS, Ray JG. Association between Bell's palsy in pregnancy and pre-eclampsia. QJM. 2002;95:359–362.
8. Mair IW, Elverland HH, Johannessen TA. Idiopathic facial palsy and pregnancy. Ann Otol Rhinol Laryngol. 1973;82:235–239.
9. Ben David Y, Tal J, Podoshin L, Fradis M, Sharf M, Pratt H, Faraggi D. Brain stem auditory evoked potentials: effects of ovarian steroids correlated with increased incidence of Bell's palsy in pregnancy. Otolaryngol Head Neck Surg. 1995;113:32–35.
10. Steiner I, Cohen O. Peripartum Bell's palsy. Lancet. 1996;347:1121–1122.
11. Saunders WH. Viral infections and cranial nerve paralysis. Arch Otolaryngol. 1963;78:85–90.
12. Cohen Y. Bell palsy complicating pregnancy: a review. Obstet Gynecol Surv. 2000;55:184–188.
13. McGregor JA, Guberman A, Amer J, Goodlin R. Idiopathic facial nerve paralysis (Bell's palsy) in late pregnancy and the early puerperium. Obstet Gynecol. 1987 ;69:435–438.
14. Kunze M, Arndt S, Zimmer A, Földi M, Hanjalic-Beck A, Echternach M, Birkenhäger R, Aschendorff A. Idiopathic facial palsy during pregnancy. HNO. 2012;60:98–101.
15. Lee HY, Byun JY, Park MS, Yeo SG. Steroid-antiviral treatment improves the recovery rate in patients with severe Bell's palsy. Am J Med. 2013;126:336–341.
16. Barry-Kinsella C, Milner M, McCarthy N, Walshe J. Sixth nerve palsy: an unusual manifestation of preeclampsia. Obstet Gynecol. 1994;83:849–851.
17. Thurtell MJ, Sharp KL, Spies JM, Halmagyi GM. Isolated sixth cranial nerve palsy in preeclampsia. J Neuroophthalmol. 2006;26:296–298.
18. Park CM, Kim SY. Abducens nerve palsy in pre-eclampsia after delivery: an unusual case report. J Obstet Gynaecol Res. 2007;33:543–545.
19. Fung TY, Chung TK. Abducens nerve palsy complicating pregnancy: a case report. Eur J Obstet Gynecol Reprod Biol. 1999;83:223–224.
20. Thamban S, Nama V, Sharma R, Kollipara PJ. Abducens nerve palsy complicating pregnancy. J Obstet Gynaecol. 2006;26:811–812.
21. Bladé J, Peborde J, Darleguy P. Paralysie du VI au cours d’un eclampsia. Bull Soc Ophtalmol Fr. 1968;68:284–287.
22. Sternberg I, Ronen S, Arnon N. Recurrent, isolated, post-febrile abducens nerve palsy. J Pediatr Ophthalmol Strabismus. 1980;17:323–324.
23. Fiala A, Furgler G, Baumgartner E, Paal P. Delayed subdural haematoma complicated by abducens nerve palsy and cortical vein thrombosis after obstetric epidural anaesthesia. Br J Anaesth. 2012;108:705–706.
24. Vial F, Bouaziz H, Adam A, Buisset L, Laxenaire MC, Battaglia A. Paralysie oculomotrice et rachianesthésie. Ann Fr Anesth Reanim. 2001;20:32–35.
25. Nishio I, Williams BA, Williams JP. Diplopia: a complication of dural puncture. Anesthesiology. 2004;100:158–164.
26. Lafuente N, Mateo A, Izquierdo B, Gutiérrez N. Paralisis del sexto par cranial derecho como complicación del bloqeo epidural. Rev Esp Anestesiol Reanim. 2008;55:251–253.
27. Arcand G, Girard F, McCormack M, Chouinard P, Boudreault D, Williams S. Bilateral sixth cranial nerve palsy after unintentional dural puncture. Can J Anaesth. 2004;51:821–823.
28. Yatziv Y, Stolowitch C, Segev Y, Kesler A. Acute esotropia after epidural anesthesia. Obstet Gynecol. 2008;111:540–541.
29. Lee JL, Roberts RB. Paresis of the fifth cranial nerve following spinal anesthesia. Anesthesiology. 1978; 49:217–218.
30. Carrero EJ, Augusti M, Fabregas N, Vallderoriola F, Fernandez C. Unilateral trigeminal and facial nerve palsies associated with epidural anesthesia in labour. Can J Anaesth. 1998; 45:893–897.
31. Fang JY, Lin JW, Li Q, Jiang N, Gao Y. Trigeminal nerve and facial nerve palsy after combined spinal-epidural anesthesia for cesarean section. J Clin Anesth. 2010;22:56–58.