□ CASE REPORT □

Postpartum Angiopathy Associated with Reversible
Borderzone Ischemia
Yoko Okamoto and Toru Yamamoto

Abstract
A 32-year-old woman developed a headache, seizures, and stupor on postpartum day 8. An initial diagnosis of possible encephalitis was made considering the presence of fever, neck stiffness, and abnormal CSF
findings. MRI demonstrated hyperintense signals consistent with bilateral borderzone areas. MRA showed severe proximal narrowing of anterior, middle, and posterior cerebral arteries bilaterally. The patient recovered
completely over 2 weeks, and repeated MRI and MRA scans were normal. Reversible vasoconstrictions have
been known to occur during puerperium, and the clinical symptoms of our patient resembled such cases.
Prior reports attributed these cases of postpartum angiopathy to capillary leakage and edema resulting in leukoencephalopathy. Our case suggests reversible borderzone ischemia as an additional pathological process.
Key words: leukoencephalopathy, eclampsia, vasoconstriction
(DOI: 10.2169/internalmedicine.47.0404)

Introduction
Reversible vasoconstriction syndrome has been associated
with migraines, vasoactive drug use, hypertensive encephalopathy, eclampsia, immunosuppressive drug therapy, or organ
transplantation (1). Several cases have been reported in
which the patient developed postpartum reversible vasoconstriction, which is now known as “postpartum angiopathy” (2).
Postpartum angiopathy is characterized by sudden onset
headache, confusion, seizures, and focal neurological deficits
after delivery. Patients generally display edematous lesions
mainly distributed in the bilateral parieto-occipital white
matter. However, abnormal cerebrospinal fluid (CSF) findings have not been reported before. Here, we report a patient with postpartum angiopathy associated with severe
proximal narrowing of the major cerebral arteries, and reversible borderzone ischemia.

Case Report
A 32 year-old woman had an uncomplicated pregnancy
and delivered her first baby at 40 weeks of gestation. She
had no history of migraines. Furthermore, no signs of

edema, proteinuria, hypertension, or neurological complaints
had been observed during the pregnancy. Her antepartum
and intrapartum blood pressure ranged from 120/70 to 130/
80 mmHg, and she was normotensive at least to postpartum
day 6.
On postpartum day 4, she developed a severe throbbing
headache, which continued but improved with analgesics.
On postpartum day 8, her headache worsened upon awaking. Two hours later, she found herself unable to write, wash
clothes and subsequently panicked at her inability to remember how to do simple activities of daily living. In fact, her
husband found her at home crying next to the door, unable
to unlock it from the inside to let him in. That night, the patient experienced a generalized tonic clonic seizure, and was
transferred to our hospital immediately.
On arrival, she was confused, disorientated and somnolent. Her speech was slow and difficult to understand; however, she was able to express complaints of visual hallucinations consisting of red ants or green balls. Her blood pressure was 176/122 mmHg, and body temperature was 37.9℃.
A general physical examination revealed normal results, and
she did not have any peripheral edema or changes in the
retinal arterioles and optic discs. A general physical examination was significant only for mild nuchal rigidity. There
were no signs of peripheral edema or funduscopic changes.

Department of Neurology, Osaka Saiseikai Nakatsu Hospital, Nakatsu
Received for publication June 25, 2007; Accepted for publication November 5, 2007
Correspondence to Dr. Toru Yamamoto, 21001@nakatsu.saiseikai.or.jp

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DOI: 10.2169/internalmedicine.47.0404

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Strength, sensation and deep tendon reflexes were all intact.
The patient had two more generalized tonic clonic seizures
in our emergency room.
Laboratory results revealed leukocytosis of 17,100/mm3,
and a normal platelet count. Serum chemistry showed an
elevation of lactic dehydrogenase (LDH, 374 IU/L), creatinine kinase (CK, 1,488 IU/L), and C-reactive protein
(CRP, 2.71 mg/dl). Her urinalysis revealed proteinuria
(>3+). Electrolytes, liver and renal function test, rheumatoid
factor, antinuclear antibodies were all normal. Echocardiogram and electroencephalography were both unremarkable.
Lumbar puncture showed clear colorless cerebrospinal fluid
(CSF) with an opening pressure of 16 cm H2O. CSF contents showed protein elevated to 128 mg/dl, glucose normal
at 77 mg/dl, and a leukocyte count of 1/μL. Antiviral antibodies or herpes simplex virus DNA in the CSF were negative.
A head CT scan revealed hypodense lesions in both occipital lobes. An MRI demonstrated hyperintense lesions in
the bilateral parieto-occipital lobes and the frontal cortices
and subcortices (Fig. 1). Diffusion-weighted images showed
hyperintensities in the cortices consistent with borderzone
arterial territories (Fig. 1). The apparent diffusion coefficient

(ADC) in the lesions showed no obvious changes.
An initial diagnosis of possible encephalitis was made
considering the presence of fever, neck stiffness, and abnormal CSF findings. She was treated with acyclovir, glycerol,
phenytoin and analgesics. On the following day, the patient’s blood pressure fell to 110/72 mmHg without antihypertensive drugs, and she had no more seizures but still suffered from confusion and headaches. MR angiography
(MRA) performed 4 days later showed severe proximal narrowing of anterior, middle, and posterior cerebral arteries bilaterally (Fig. 2).
The remainder of her hospital course was uneventful, and
the proteinuria noted to be 3+ on admission decreased to 1+
upon discharge. A repeat CSF study performed 1 week after
admission was normal. Her headache and the clinical deficits completely disappeared over the following 2 weeks.
One month later, a follow up MRI (Fig. 3) and MRA
(Fig. 4) were normal.

Discussion
Reversible vasoconstriction syndrome was first angiographically demonstrated in 1988 by Call and Fleming (2).

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DOI: 10.2169/internalmedicine.47.0404

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the circle of Willis in four patients with severe headaches
and motor and sensory deficits. Reversible vasoconstriction
syndrome has subsequently been found to be associated with
conditions like migraine, vasoactive drug use, pregnancy and
the puerperium (1).
The symptoms of reversible vasoconstriction syndrome
are quite similar to that of eclampsia, a disorder defined by
the development of convulsion and/or coma during pregnancy and the postpartum period in severely hypertensive
women (3). The presence of generalized edema, hypertenFi
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signs of pending eclampsia. However, women who develop
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eclampsia may exhibit a much wider spectrum of signs with
no proteinuria, no edema, and only minimal hypertenTheir paper described transient, fully reversible vasoconstric- sion (3). The angiographic changes during eclampsia are yet
tions and dilatations predominantly involving arteries around to be fully characterized.
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Patients with postpartum angiopathy (PPA) are reported to
develop symptoms similar to reversible vasoconstriction syndrome, such as severe headaches with an abrupt onset, seizures, or focal neurological deficits. While eclampsia is
often thought to occur within 48 hours of delivery, the time
frame for PPA is much longer, with onset up to two weeks
after delivery (1). The neurological symptoms of PPA similarly remit within days to weeks. The symptoms of the present patient resembled such cases.
Prior reports attributed the case of postpartum angiopathy
to capillary leakage and edema resulting in leukoencephalopathy (4). However, in the present case, MRI demonstrated
hyperintense lesions in the cortices and the subcortices,
which were consistent with borderzone ischemia. Consider-

ing these findings, we speculate that proximal artery vasospasm and subsequent endothelial injury to small vessels of
the cortices due to borderzone ischemia caused leakage of
fluid across a transiently abnormal blood-brain barrier, and
thus resulted in elevated protein levels in the CSF. We are
unable to explain the precise mechanism why our case is
different from usual cases of PPA (leukoencephalopathy and
capillary leak).
It is possible that all of these disorders represent different
manifestations of similar underlying processes. Our patient
had aspects of reversible vasoconstriction syndrome, eclampsia, and PPA. The proximal artery vasospasm may cause
borderzone ischemia by a homodynamic mechanism.

References
1. Singhal AB. Postpartum angiopathy with reversible posterior leukoencephalopathy. Arch Neurol 61: 411-416, 2004.
2. Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher
CM. Reversible cerebral segmental vasoconstriction. Stroke 19:
1159-1170, 1988.
3. Sibai BM. Diagnosis, prevention, and management of eclampsia.

Obstet Gynecol 105: 402-410, 2005.
4. Shimada J, Takeda N, Yamauchi S, Urushibara T, Kawaguchi H.
Reversible posterior leukoencephalopathy syndrome: Experience
in 3 cases. No To Shinkei 56: 1036-1041, 2004 (in Japanese).

Ⓒ 2008 The Japanese Society of Internal Medicine
http://www.naika.or.jp/imindex.html

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