OBSTETRICS

Posterior reversible encephalopathy syndrome (PRES) in pregnancy:

A diagnostic challenge to obstetricians
U. KRISHNAMOORTHY
1
, P. K. SARKAR
1
, Y. NAKHUDA
2
& P. D. MULLINS
3
Departments of
1
Obstetrics and Gynaecology,
2
Radiology and
3
Medicine, Acute Unit, Burnley General Hospital,
East Lancashire Hospitals NHS Trust, UK
Summary
Post-partum clinical presentation with seizures and focal neurological decit has a wide differential diagnosis. Two cases of
the rare condition Posterior Reversible Encephalopathy Syndrome (PRES) associated with pregnancy are presented with
complete recovery following multidisciplinary care. One of the cases was associated with Factor VII deciency in pregnancy
and the other with twin gestation and psoas abcess.
Keywords
Posterior reversible encephalopathy syndrome, PRES, eclampsia, factor VII deciency, psoas abcess
Case report 1
A 22-year-old primigravida with singleton gestation and
uneventful antenatal period presented with spontaneous
labour at 39 weeks gestation. Emergency caesarean
section was performed under epidural anaesthesia for
secondary arrest. A baby boy weighing 3,780 g was
delivered in good condition. She had an uneventful
recovery and went home on the third postoperative day.
Blood pressure (BP) was normal throughout this period.
Five days later, she was readmitted with headache,
epigastric pain, vomiting and BP of 171/97 mmHg.
Neurological assessment found her to be unresponsive
revealing a Glasgow coma score of 6/15. Intravenous
labetalol at a dose of 50 mg was administered for BP
control. Prolonged grand mal seizure lasting approxi-
mately 4 min occurred after admission. Diagnosis of
eclampsia was made and airway secured by endotracheal
intubation after rapid sequence induction. Intravenous
magnesium sulphate as 4 g bolus followed by main-
tenance infusion of 4 g/h was started.
Non-enhanced cranial computerised tomogram (CT)
was normal. After 36 h of stability, the decision was made
to extubate. Following extubation, BP abruptly increased
to 187/115 mmHg and she sustained an episode of grand
mal seizure lasting 2 min necessitating further 4 g bolus
magnesium sulphate and maintenance infusion was con-
tinued. Within an hour she sustained four further episodes
of grand mal convulsions 510 min apart. Repeat bolus of
4 g magnesium sulphate was administered besides 100 mg
labetalol. Although repeat intubation was not required,
magnesium sulphate infusion was continued for a further
48 h.
Neurological assessment revealed weakness of the right
lower limb and repeat cranial CT was normal. In view of
recurrent convulsive episodes despite magnesium sulphate,
differential diagnoses were considered. Phaeochromocyto-
ma was ruled out by normal levels of urinary metanephrine
and normetanephrine. Normal results of a lumbar punc-
ture that was performed following correction of coagulo-
pathy with transfusion of fresh frozen plasma helped
exclude iatrogenic meningitis secondary to epidural anaes-
thesia. Clear cerebrospinal uid under no tension ruled out
subarachnoid haemorrhage.
At this point, magnetic resonance imaging (MRI) of the
brain was performed (Figure 1). Axial T2 (Fig. 1a) and
uid attenuated inversion recovery MR (FLAIR) (Fig. 1b)
sequence demonstrated subcortical white matter hyperin-
tensity lesions in occipital and parietal lobes in keeping with
focal oedema. Diffusion-weighted imaging (DWI) axial
image (Fig. 1c) demonstrated isointensity, whereas corre-
sponding apparent diffusion coefcient (ADC) map image
(Fig. 1d) revealed hyperintensity (Fig. 1d) in the region of
interest, conrming diagnosis of posterior reversible
encephalopathy syndrome (PRES).
Anticoagulation with therapeutic doses of heparin was
recommended by physicians, but this was deferred as
coagulation studies showed prolonged international nor-
malised ratio (INR) of 1.8 and prothrombin time (PT) of
23. Mechanical thromboprophylaxis only was therefore
provided. There was no neurological decit when she was
allowed home 12 days later and she was maintained on oral
This article is not subject to United States copyright laws.
Correspondence: U. Krishnamoorthy, 7 Hawthorn Road, Bamford, Rochdale, Lancashire OL11 5JG, UK. E-mail: ukrishnamoorthy@hotmail.com
Journal of Obstetrics and Gynaecology, April 2009; 29(3): 192194
ISSN 0144-3615 print/ISSN 1364-6893 online
DOI: 10.1080/01443610802716026
labetalol at a dose of 200 mg twice daily. Further
coagulation prole and thrombophilia screen performed
in view of deranged coagulation prole revealed reduced
factor VII levels that stayed consistently low when repeated
after 3 months, conrming deciency of factor VII. She
remained well at 6 months follow-up, when all drug
therapy was eventually withdrawn.
Case report 2
A 35-year-old woman with no signicant background
medical history was booked for antenatal care in her third
pregnancy. The rst pregnancy was a spontaneous rst
trimester miscarriage and the second was an uneventful
term vaginal delivery. Booking and serial ultrasound scans
conrmed a dichorionic, diamniotic twin pregnancy with
progressive growth along the 50th percentile. At 34 weeks
gestation, pre-term labour with spontaneous rupture of
membranes proceeded to normal vaginal delivery of live
twins in good condition. She had epidural analgesia and
her BP throughout was normal. She was discharged home,
after an uneventful recovery, on the third day.
Ten days later she was re-admitted with complaints of
right iliac fossa pain, epigastric pain, headache, vomiting
and diplopia followed by grand mal seizures at presenta-
tion. Eclampsia was diagnosed as BP was elevated at 160/
90 mmHg. Intravenous magnesium sulphate was started as
bolus followed by maintenance infusion and intravenous
labetalol as in Case report 1. Three further episodes of
recurrent seizures necessitated a further bolus of magne-
sium sulphate. Neurological assessment revealed disorien-
tation, bilateral lower limb weakness and exaggerated
reexes. She was pyrexial with temperature of 38.58C,
3 g of proteinuria was noted and blood tests revealed
leucocytosis and elevated C-reactive protein levels.
Cranial CT and lumbar puncture were reported normal.
MRI of the brain (Figure 2) showed bilateral symmetrical
vasogenic oedema with predominant involvement of sub-
cortical white matter in the posterior parietal and occipital
lobes. MR venography ruled out suspicion of venous sinus
thrombosis. DWI MRI, to exclude ischaemia as the cause of
oedema, showed no infarction, and a diagnosis of posterior
reversible encephalopathy syndrome (PRES) was made.
Gradual improvement was noted on labetalol, magnesium
sulphate and therapeutic doses of heparin. Incidentally, an
abdominal CT scan performed due to persistent right iliac
fossa pain revealed a small right-sided psoas abscess
(4 63 cm) which resolved with intravenous antibiotics.
Within 2 weeks, there was complete resolution of clinical
and radiological features and oral labetalol only at a dose of
200 mg twice daily was continued. She was well at 6 months
review and the labetalol was stopped.
Discussion
PRES was rst described in 1996 as a usually reversible
neurological syndrome with a variety of presenting
symptoms ranging from headache, altered mental status,
seizures and vision loss, to loss of consciousness (Hinchey
et al. 1996). The term describes a potentially reversible
imaging appearance and symptomatology that is shared by
a diverse array of associated conditions (McKinney et al.
2007) (Table I).
Figure 1. Axial MRI images of the brain. Axial T2 (a) and FLAIR
(b) images demonstrate bilateral symmetrical high signal intensity
area with predominant involvement of subcortical white matter in
the right parieto-occipital region (arrowed). Diffusion weighted
(DWI) MRI axial image (c) demonstrates signal iso-intensity in the
same area. Apparent diffusion coefcient (ADC) image (d)
demonstrates increased signal intensity due to increased water
mobility signifying vasogenic oedema.
Figure 2. (a) Axial T2 and (b) axial FLAIR (uid-attenuated inversion recovery) T2 MRI images demonstrating bilateral symmetrical high
signal intensity areas (arrows), with predominant involvement of sub-cortical white matter in the posterior parietal and occipital lobes.
PRES in pregnancy 193
Classical MRI brain appearance in PRES is as follows:
reversible parieto-occipital white matter oedema on FLAIR
MR; areas of abnormal T2 signal; normal DWI and
increased ADC values (McKinney et al. 2007; Finocchi
et al. 2005). Reversible, vasogenic oedema of PRES has
different appearances compared with those of irreversible,
cytotoxic oedema present in brain infarction (Fujiwara
et al. 2005). Hypothesis suggests that endothelial dysfunc-
tion and a state of hyperperfusion leads to breakdown in the
blood-brain barrier and extravasation of uid resulting in
cortical and/or sub-cortical oedema (McKinney et al.
2007). PRES triggered by transient hypercoagulable state
and successfully treated with anticoagulant therapy using
heparin has been reported (Yano et al. 2005).
Factor VII deciency is a rare hereditary coagulation
disorder with an incidence estimated at 1 in 500,000 (Rizk
et al. 1999). Case report 1 is the rst report of PRES in
pregnancy associated with factor VII deciency. Hae-
mostatic dysfunction consequent upon clotting factor
deciency may be the pathogenesis besides endothelial dys-
function caused by eclampsia. PRES associated with sepsis,
dural puncture and regional anaesthesia have been reported
(Bartynski et al. 2006; Servillo et al. 2008). Case report 2 is
the rst report of PRES in association with twin gestation
and psoas abscess. Hypercoagulable twin pregnant state
besides underlying sepsis could be precipitating factors.
An opportunistic recent survey of 120 obstetricians in
the North West Region in March 2008 revealed paucity of
awareness of condition. PRES although rare is presently a
well acknowledged association of pregnancy, pre-eclampsia
and eclampsia. Obstetricians and other healthcare profes-
sionals involved in the care of pregnant women need to be
aware of this rare but increasingly reported syndrome of
PRES. Advances in the eld of radiology and increasing
expertise in the eld accounts for the rising diagnoses of
PRES. Prompt diagnosis facilitating appropriate treatment
is associated with good outcome, reversibility of clinico-
radiological variabilities and prevents progression to
irreversible brain damage (Finocchi et al. 2005). Appro-
priate MR imaging with DWI and ADC on all cases of
maternal convulsions when CT scan reveals normal
ndings is recommended by the authors, so that pregnancy
associated PRES is diagnosed and treated in a timely
manner.
Declaration of interest: The authors report no conicts
of interest. The authors alone are responsible for the
content and writing of the paper.
References
Bartynski WS, Boardman JF, Zeigler ZR et al. 2006.
Posterior reversible encephalopathy syndrome in infection,
sepsis, and shock. American Journal of Neuroradiology 27:
21792190.
Finocchi V, Bozzao A, Bonamini M et al. 2005.
Magnetic resonance imaging in posterior reversible
encephalopathy syndrome: Report of three cases and
review .of literature. Archives of Gynaecology and Obstetrics
271:7985.
Fujiwara Y, Higaki H, Yamada T et al. 2005. Two cases of
reversible posterior leukoencephalopathy syndrome, one with
and the other without pre-eclampsia. Journal of Obstetric and
Gynaecological Research 31:520526.
Hinchey J, Chaves C, Appignani B et al. 1996. A reversible
posterior leukoencephalopathy syndrome. New England Journal
of Medicine 334:494500.
Mckinney AM, Short J, Truwit LC et al. 2007. Posterior reversible
encephalopathy syndrome: Incidence of atypical regions of
involvement and imaging ndings. American Journal of Radi-
ology 189:904912.
Rizk DE, Castella A, Shaheen H et al. 1999. Factor VII deciency
detected in pregnancy: a case report. American Journal of
Perinatology 16:223226.
Servillo G, Apicella E, Striano P. 2008. Posterior reversible
encephalopathy syndrome (PRES) in the parturient with
preeclampsia after inadvertent dural puncture. International
Journal of Obstetric Anesthesia 17:8889.
Yano Y, Kario K, Fukunaga T et al. 2005. A case of reversible
posterior leukoencephalopathy syndrome caused by transient
hypercoagulable state induced by infection. Hypertension
Research 28:619623.
Table I. Recognised conditions associated with PRES.
Hypertension
Pre-eclampsia
Eclampsia
Systemic lupus erythematosus
Immunosuppressive medications (e.g. cyclosporine)
Anti-neoplastic agents
Severe hypercalcemia
Thrombocytopenic syndromes
Henoch Scho nlein purpura
Haemolytic uraemic syndrome
Amyloid angiopathy
Various causes of renal failure
Regional anaesthesia
Infection/sepsis
Drugs: cocaine, methamphetamine
Sources: McKinney et al. (2007); Servillo et al. (2008).
194 U. Krishnamoorthy et al.