Postpartum posterior reversible encephalopathy syndrome (PRES) preceding severe postpartum cerebral angiopathy (PPA).
Abstract Number: 152
Abstract Type: Case Report/Case Series
A 37 yo G5P4 presented with severe non-postural headache on postpartum day 5. Her pregnancy and delivery were uneventful except for an intrathecal catheter for labor following wet tap. She had a mild post-dural puncture headache (PDPH) which resolved before discharge on day 2. Two hours after arrival, the patient had a seizure. MRI brain revealed changes consistent with PRES. Although her BP was normal and proteinuria was absent, she was treated for eclampsia. Magnesium (Mg) was initiated. Over the next 4 days, she had 2 episodes of elevated BP which responded to IV labetalol. She was discharged with lessened headache and planned follow-up in 2 weeks.
The patient returned 3 days later, with persistent headache and recurrent transient vision loss associated with right face and arm numbness. MRA brain reported, "worsening of postpartum cerebral angiopathy (PPA) and appearance suggestive of cerebral vasculitis or diffuse vasospasm". She was monitored for 4 days, receiving Mg and labetalol. Only a few episodes of elevated BP were noted. She continued with headaches, but was otherwise stable and discharged on hydralazine and ibuprofen.
One day after discharge, she returned with worsening headache, now postural, and episodic bilateral vision loss. An epidural blood patch was performed, with improvement. After 2 hrs, she requested to be discharged, against medical advice.
She returned the next day complaining of new right visual field changes. Physical exam revealed right homonymous hemianopsia. Repeat MRI/MRA reported, "worsening of postpartum cerebral vasculopathy, and acute infarct in the left PCA territory". She was re-admitted and treated with nimodipine and Mg. Her headache resolved after 4 days but she continued with right visual field deficits at discharge. The final MRI showed "stable infarct and significant improvement of abnormal FLAIR hyperintensity lesions".
In this case, it was important to initially consider PDPH, however her headache was not postural. After her seizure, a diagnosis of eclampsia was made, however there was doubt with no proteinuria or elevated BP. The MRI was consistent with PRES, which is associated with eclampsia and it has been suggested that peripartum patients with PRES should be managed as though they are eclamptic, despite the absence of hypertension and proteinuria.
In retrospect, we question whether intracranial hypotension due to CSF leak could have induced PRES/PPA. There have been multiple reports of PRES/PPA associated with intracranial hypotension. We also speculate whether the blood patch caused an exacerbation of the vasospasm.
PRES and PPA have a high degree of overlap, and outcomes can be devastating with delayed management. Their pathophysiology is uncertain. With PRES, BP maintenance within normal is important. With PPA, Mg and nimodipine are advocated. We question whether longer monitoring and continuous Mg and nimodipine may have prevented disease progression in this patient.