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OJHAS Vol. 11, Issue 1:
(Jan-Mar 2012) |
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A Rare Case of Reversible Encephalopathy Syndrome Accompanying Late Postpartum Eclampsia or Hypertensive Encephalopathy-A Clinical Dilemma |
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Shakuntala PN, Department of Obstetrics and Gynaecology,
Padmini Isaac, Department of Obstetrics and Gynaecology,
Rashmi, Department of Obstetrics and Gynaecology,
Neeta Appaiah, Department of General Medicine,
Thomas M, Department
of Neurology,
St. Martha’s Hospital, Bengaluru |
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Address for Correspondence |
Department of Obstetrics and Gynaecology,
St. Martha’s Hospital,
Bengaluru, India.
E-mail:
shakuntala_pn@yahoo.com |
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Shakuntala PN, Isaac P, Rashmi, Appaiah N, Thomas M. A Rare Case of Reversible Encephalopathy Syndrome Accompanying Late Postpartum
Eclampsia or Hypertensive Encephalopathy-A Clinical Dilemma. Online J Health Allied Scs.
2012;11(1):20 |
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Submitted: Feb 8,
2012;
Accepted: Mar 21, 2012; Published: Apr 15, 2012 |
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| Abstract: |
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Posterior Reversible Encephalopathy Syndrome (PRES) refers to a
clinic-radiologic diagnosis. Clinically it is characterized by non specific symptoms such as headache,
confusion, visual disturbances and seizures. The radiological findings
in PRES are thought to be due to vasogenic oedema, predominantly in
the posterior cerebral hemispheres, and are reversible with appropriate
management. We report a case of reversible encephalopathy diagnosed
by MRI scan occurring in atypical areas like the caudate and lentiform
nuclei of the brain following an uneventful lower segment caesarean
section in a normotensive patient, who was successfully
treated with antihypertensives, anticonvulsants and supportive treatment. The differential diagnosis of convulsions
in the post-partum period is discussed.
Key Words:
PRES; Vasogenic edema; Postpartum; Normotensive.
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Posterior
reversible encephalopathy syndrome (PRES) is a well-recognized, clinical
and neuro-radiological entity first described in 1996 by Hinchey et al.(1) This is characterized by headache, vomiting, confusion, seizures, visual abnormalities and motor signs. These transitory neurological disturbances are thought to be due to cerebral vasospasm causing
ischemia of the involved territory. The ensuing cerebral ischemia has a characteristic imaging pattern on MRI scan. It is associated with a variety of underlying conditions including hypertensive encephalopathy, preeclampsia, systemic lupus erythematosus,
thrombotic thrombocytopenia purpura, treatment with immunosuppressants,
renal failure and central nervous system infections. Importantly, these
changes appear to be completely reversible if the underlying cause is
treated or the precipitating drug withdrawn early in the clinical course.(1) We report a case of reversible encephalopathy syndrome, occurring
in the post partum period managed successfully.
A 24 years old
multi-parous lady underwent an elective lower segment caesarean section
under spinal anaesthesia. She was normotensive at presentation and had
no other contributing risk factors. Her blood pressure was within normal
limits throughout the surgery and in the post-operative recovery period.
On the fourth postoperative day she complained of bifrontal headache
which was constricting in type especially on standing, suggestive of
post spinal headache. Her blood pressure was in the normotensive range
and she responded to paracetamol with domperidone combination and good
hydration.
On the fifth postoperative day she developed severe headache along
with visual disturbances followed by generalised tonic and clonic convulsions
and postictal confusion. Her blood pressure was 220/120 mm Hg. A provisional
diagnosis of postpartum eclampsia was thought off. Blood pressure could
not be controlled even after starting her on magnesium sulphate and
calcium channel blockers. She was shifted to medical intensive care
unit by the physicians who suspected post partum cerebral venous thrombo-embolism.
Her investigations including serum electrolytes, serum calcium, serum
magnesium, liver function tests and coagulation profile were within
normal limits. There was no evidence of urinary proteinuria. Her Serum
uric acid was raised, which measured 9.8 mg/dl with changes in serum
creatinine-3.4 mg/dl and blood urea- 77mg/dl. Antinuclear antibody
evaluation was negative. Fundoscopy did not reveal any papilloedema.
CT brain and MRI venogram (Figure-1) were normal.
On brain MRI scanning the T2 weighted axial image showed hyperintense
areas within the cerebellar hemispheres, gray and subcortical white
matter of bilateral frontal, parietal, occipital and temporal regions,
left lentiform nucleus and right caudate nucleus suggesting the possibility
of posterior reversible encephalopathy syndrome(Figure-2 and 3).
She was started on anti-hypertensives, anticonvulsants, anti-edema
measures, proton pump inhibitors and antibiotics. The patient stabilized
by the fourth day. Her neurological symptoms completely resolved by
7 days. She was discharged on anti-hypertensive and anti-convulsive
medications. She completed her treatment and followed up for almost
15 months and was free of neurological symptoms.
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Figure 1: Norman MRI Venogram |
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Figure 2: Hyperintense areas within
bilateral cerebellar hemispheres, gray and sub-cortical white
matter of bilateral frontal (F) and occipital (O) regions. |
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Figure 3: Hyperintense areas within
left lentiform
nucleus (L) and right caudate nucleus (C) |
The differential diagnosis for seizures in the late post-partum period
includes eclampsia, subarachnoid haemorrhage, intracerebral haemorrhage,
thrombotic phenomena, intracranial neoplasm, head trauma, idiopathic
epilepsy, infection (meningo-encephalitis), amniotic fluid embolism,
postpartum angiopathy.(1,2)
There was no past history of epilepsy or head injury. Infection due
to dural puncture was a possibility, but the total count was 11,500
cells per cu mm which was within normal limits. Post dural puncture
spinal headache usually presents within 24 to 48 hours. Brain MRI with
venogram ruled out intracranial bleed, ischaemia secondary to thromboembolism,
vasospasm or space occupying lesion. Amniotic fluid embolism rarely
occurs after 48 hours post partum and generally presents with cardiopulmonary
collapse and coagulopathy which was not seen in our patient. Possibility
of systemic lupus erythematosis with renal involvement was considered,
but the Anti-nuclear antibody evaluation was negative.
An alternative explanation is the possibility of post-partum angiopathy.
This diagnosis should be considered in a post-partum patient with hypertension
and headache but no proteinuria as it was in this case. Post-partum
angiopathy is a form of reversible cerebral segmental vasoconstriction
characterised by severe “thunderclap” headaches, seizures, focal
neurological deficits and segmental narrowing and dilatation of large
and medium sized arteries. Typically, scanning reveals ischaemic lesions
but MRI findings consistent with reversible posterior leucoencephalopathy
syndrome have been reported.(1)
Posterior reversible encephalopathy syndrome mostly secondary to late
post partum eclampsia was suggested , as she presented with headache,
followed by seizures ,with raised serum uric acid levels 9.8 mg/dl (normal3.8-7.0
mg/dl), hypertension without protienuria , in uremia mostly secondary
to hypertensive encephalopathy itself . The clinical dilemma we faced
was: is it eclampsia or hypertensive encephalopathy due to other reasons?
What is the ideal treatment? With multi-disciplinary approach we were
able to scale down the differential diagnosis. MRI was typically consistent
with vasogenic oedema, but which was involving the atypical areas like
cerebellum, frontal, left lentiform nucleus and the right caudate nucleus
along with typical areas, bilateral occipital, parietal and temporal.
Usually it involves the posterior occipito-parietal lobes and hence
the nomenclature posterior reversible encephalopathy syndrome.(3)
PRES is still an under recognised and untreated condition. The incidence
in the peripartum setting is not known. After reviewing the literature
we could get access to 18 cases reported out of which 3 cases manifested
as late post partum eclampsia and only one among them was normotensive
with no abnormality in haemogram or biochemical findings before the
onset of convulsions.(4,5) So this is the second case reported
in the English literature, in a normotensive multiparous lady following
uneventful caesarean section where post dural puncture headache was
not contributary, presenting as reversible encephalopathy syndrome involving
atypical areas like caudate nucleus and lentiform nucleus ,accompanied
with late postpartum eclampsia or hypertensive encephalopathy. Due to
prompt intervention and supportive therapy, this woman recovered within
a week and did not have any neurologic deficits at the time of discharge.
She continued with antihypertensives and anticonvulsants and stopped
these medications after consultation and counselling by
neurologist and was clinically stable for the last 15 months.
Postpartum atypical reversible encephalopathy syndrome (PRES) following
an uneventful pregnancy is a very rare clinical entity. Recognition
at the earliest and prompt initiation of the supportive measures can
prevent permanent neurologic damage and thereby the associated morbidity.
Multidisciplinary care forms the corner stone to achieve a safe motherhood
in these women.
- Prout RE, Tuckey JP, Giffen NJ. Reversible posterior leucoencephalopathy
syndrome in a peripartum patient. International Journal of Obstetric
Anesthesia. 2007;16:74-76
- Krishnamoorthy U, Sarkar PK, Nakhuda Y, Mullins PD. Posterior reversible encephalopathy
syndrome (PRES) in pregnancy: A diagnostic challenge to obstetricians. Journal of Obstetrics and Gynaecology. April 2009;29(3):192-194.
- McKinney AM, Short J, Truwit CL, McKinney ZJ, Kozak OS, SantaCruz KS, Teksam M. Posterior Reversible Encephalopathy Syndrome:
Incidence of Atypical Regions of I nvolvement and Imaging Findings. AJR. 2007;189:904-912.
- Servillo G, Striano P, Striano S, Tortora F, Boccella P, De Robertis
E, Rossano F, Briganti F, Tufano R. Posterior reversible encephalopathy syndrome (PRES) in critically ill obstetric patients.
Intensive Care Med. Dec 2003;29(12):2323-2326.
- Jamie MN, Shervin E. Late Postpartum Eclampsia with Posterior Reversible Encephalopathy Syndrome.
Hospital Physician. June 2007;45–49.
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