Acta Neurologica Taiwanica Vol 16 No 3 September 2007 158 From the Section of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital, Taiwan. Received January 11, 2007. Revised March 2, 2007. Accepted April 23, 2007. Reprint requests and correspondence to: Yih-Hwa Chiou, MD. Section of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital, Taiwan. E-mail: [email protected]INTRODUCTION Reversible posterior encephalopathy syndrome (RPES), or as the previous name, parietal-occipital encephalopathy, or reversible posterior leukoen- cephalopathy (RPLS), refers to a clinical and radiologi- cal entity presented by headache, altered mental status such as confusion, lethargy, cortical visual disturbances, and seizures, with transient edematous changes of sub- cortical white matter on neuroimaging (1) . RPES will be the preferred term in this article, since lesions are not necessarily limited to the white matter (2) . Underlying clinical conditions predisposing to RPES include chron- ic renal insufficiency, toxemia of pregnancy, pediatric post-streptococcal glomerulonephritis, thrombotic thrombocytopenic purpura, autoimmune disorders alone or together with exposure to immunomodulating agents (cyclosporine, tacrolimus, interferon-alpha, cisplatin, cyclophosphamide, et al.) (1,3-10) . The well-known condi- tion of eclampsia related RPES can subside immediately after the delivery. Rare case of postpartum RPES was reported (11) . We report one case of postpartum RPES without preceding eclampsia here, and this case revealed the possibility of RPES in the purperium stage. Reversible Posterior Encephalopathy Syndrome as the Presentation of Late Postpartum Eclampsia: A Case Report Yih-Hwa Chiou and Ping-Hong Chen Abstract- We presented a case of previous healthy postpartum woman suffering from seizure of sudden onset with conscious change and anisocoric pupils. Head magnetic resonance imaging (MRI) revealed mul- tiple high signal-intensity lesions on fluid-attenuated inversion recovery (FLAIR) / T2 weighted image (T2WI) in bilateral subcortical white matters (especially the parieto-occipital areas), brain stem, and bilater- al cerebellum, and mild high signal-intensity lesions on diffusion weighted MRI (DWI). These neurological and radiological anomalies recovered completely later. The final diagnosis was the rare presentation of late postpartum eclampsia as reversible posterior encephalopathy syndrome (RPES). Key Words: Reversible posterior encephalopathy syndrome (RPES), Late postpartum eclampsia, Vasogenic edema Acta Neurol Taiwan 2007;16:158-162 Case Reports
Transcript
Acta Neurologica Taiwanica Vol 16 No 3 September 2007
158
From the Section of Neurology, Department of InternalMedicine, Far Eastern Memorial Hospital, Taiwan.Received January 11, 2007. Revised March 2, 2007. Accepted April 23, 2007.
Reprint requests and correspondence to: Yih-Hwa Chiou, MD.Section of Neurology, Department of Internal Medicine, FarEastern Memorial Hospital, Taiwan.E-mail: [email protected]
INTRODUCTION
Reversible posterior encephalopathy syndrome(RPES), or as the previous name, parietal-occipitalencephalopathy, or reversible posterior leukoen-cephalopathy (RPLS), refers to a clinical and radiologi-cal entity presented by headache, altered mental statussuch as confusion, lethargy, cortical visual disturbances,and seizures, with transient edematous changes of sub-cortical white matter on neuroimaging(1). RPES will bethe preferred term in this article, since lesions are notnecessarily limited to the white matter(2). Underlying
clinical conditions predisposing to RPES include chron-ic renal insufficiency, toxemia of pregnancy, pediatricpost-streptococcal glomerulonephritis, thromboticthrombocytopenic purpura, autoimmune disorders aloneor together with exposure to immunomodulating agents(cyclosporine, tacrolimus, interferon-alpha, cisplatin,cyclophosphamide, et al.)(1,3-10). The well-known condi-tion of eclampsia related RPES can subside immediatelyafter the delivery. Rare case of postpartum RPES wasreported(11). We report one case of postpartum RPESwithout preceding eclampsia here, and this caserevealed the possibility of RPES in the purperium stage.
Reversible Posterior Encephalopathy Syndrome as thePresentation of Late Postpartum Eclampsia: A Case Report
Yih-Hwa Chiou and Ping-Hong Chen
Abstract- We presented a case of previous healthy postpartum woman suffering from seizure of suddenonset with conscious change and anisocoric pupils. Head magnetic resonance imaging (MRI) revealed mul-tiple high signal-intensity lesions on fluid-attenuated inversion recovery (FLAIR) / T2 weighted image(T2WI) in bilateral subcortical white matters (especially the parieto-occipital areas), brain stem, and bilater-al cerebellum, and mild high signal-intensity lesions on diffusion weighted MRI (DWI). These neurologicaland radiological anomalies recovered completely later. The final diagnosis was the rare presentation of latepostpartum eclampsia as reversible posterior encephalopathy syndrome (RPES).
Acta Neurologica Taiwanica Vol 16 No 3 September 2007
CASE REPORT
The 25 year-old female, who denied previous majorsystemic disease, previous medicine use, or specialexposure/traveling history, was in her postpartum periodwith normal spontaneous delivery for her first baby(G1P1). No significant abnormal event was notedincluding eclampsia, during her pregnancy and the laborperiod. No Chinese herb was used after her delivery. Herbaby was also healthy. Headache has been noted sincetwo days after the delivery. No concurrent fever, nausea,vomiting, blurred vision, previous head injury, or otherdiscomfort was noted. However, her family found hersuddenly lying on the ground with unconsciousness, up-ward gaze deviation, and tongue biting one day after anepisode of sudden-onset headache at home. No convul-sion was observed initially. She was sent to emergencyservice immediately. At emergency service, the initialblood pressure was 109/61 mmHg with heart rate of76/min and respiratory rate of 20/min. No fever or legedema was noted. On neurological examination,Glasgow Coma Scale (GCS) was E4M5V3-4. Neutraleye position, isocoric pupils, bilateral intact light reflex,and mild left central facial palsy were noted with normalmuscle power bilaterally. Trace rigid neck was foundwith equivocal meningismus. The plantar reflexes werebilateral dorsiflexion in response. The blood testrevealed leukocytosis (WBC= 17140) with left-shifting(neutrophil= 92.2%). Normal serum creatinine level,sodium level, potassium level, glutamic oxaloacetictransaminase (SGOT) level, glucose level, lactic acidlevel, and arterial blood gas data were noted. Elevateduric acid (8.7 mg/dl) and C-reactive protein (CRP) (7.27mg/dl) level were also found. Urine routine analysisrevealed some proteinuria (++). Emergent head comput-er tomography (CT) revealed mild diffuse brain swellingonly; no obvious intracranial hemorrhage or mass lesionwas seen.
After admission on the same day, sudden onset ofgeneralized convulsion with tonic posture over bilateralupper limbs occurred and the seizure was followed withloss of consciousness. Anisocoric pupils (3/4.5 mm,larger on the left side,) with impaired light reflex on the
left side were also noted. Lorazepam 1 mg intravenouspush was given immediately, and convulsion wasstopped after 30 seconds about. Phenytoin 750 mg intra-venous loading with maintaining dose (100 mg intra-venous per 8 hours) was also used. Vital sign was stablethen. Empirical ceftriaxone was started. Emergent headmagnetic resonance imaging (MRI) was arranged, and itrevealed multiple high signal lesions on fluid-attenuatedinversion recovery (FLAIR) / T2 weighted image(T2WI) in head of caudate nucleus, bilateral putamen &corona radiata, centrum semiovalis, bilateral parieto-occipital gyrus, midbrain-pontine area, bilateral cerebel-lum, and mild high signal on diffusion weighted MRI(DWI); vasogenic edema rather than cytotoxic edemawas considered (Fig. 1). No evidence of sinus thrombo-sis was noted under the magnetic resonance venography(MRV) study. Lumbar puncture study was also per-formed. The appearance of cerebrospinal fluid (CSF)was clear and colorless. Only slightly elevated CSFopening pressure (230 mm H2O) and mildly elevatedCSF protein level (96.5 mg/dl) were found on the CSFstudy in detail. Serial serological study, including VDRLand autoimmune profile (C3, C4, ANA, dsDNA, anti-phospholipid antibody), revealed negative finding. Theelectroencephalogram (EEG) study showed mild diffusecortical dysfunction without significant epileptiform dis-charge, considered as postictal changes. Her blood pres-sure was once elevated (up to 180/100 mmHg) with agi-tated temper under intensive care unit monitoring.Intermittent intravenous labetalol (12.5 mg) was used tocontrol blood pressure. Her consciousness improvedgradually under medical treatment. Her alertness andawareness completely total recovered 24 hours later.Follow-up neurological examinations revealed no signif-icant focal neurological deficits. The second head MRIstudy (9 days after first MRI) showed complete resolu-tion of brain lesion on previous head MRI study (Fig. 2).Due to mild elevated systolic blood pressure (up to 150-160 mmHg), regular low dose anti-hypertensive agent(Ramipril 2.5 mg oral per day) was given. Oral pheny-toin was kept with dosage of 300 mg per day. Thepatient was discharged under stable condition with out-patient clinic follow-up. Under consideration of the clin-
160
Acta Neurologica Taiwanica Vol 16 No 3 September 2007
ical course, specific reversible head MRI changes in ser-ial studies, reversible posterior encephalopathy syn-drome as the presentation of late postpartum eclampsiawas diagnosed.
DISCUSSION
Pre-eclampsia is one of the well-known obstetriccomplication. It consists of hypertension (blood pressure> 140/90 mmHg) and proteinuria. In the most severeform, it may be accompanied by seizures, and termedeclampsia. Generally, eclampsia occurs either before orwithin 48 hours of delivery. Eclampsia occurring morethan 48 hours but less than 4 weeks after delivery isknown as late postpartum eclampsia, and we present thisinstructive case here for two reasons: (1) late postpartumeclampsia accounts for less than 15% of eclampsia(12) and(2) the syndrome it is not so familiar to most obstetricdoctors, medical physician at emergent service, and neu-rologists. In our case with postpartum status, thesesymptoms of eclampsia were not so typical. The initialblood pressure was not markedly elevated, the leg edemawas not found, and only mild proteinuria (++) wasnoted. However, patients of late postpartum eclampsiamay not present with all the classical symptoms of intra-partum preeclampsia such as hypertension (blood pres-sure > 140/90 mmHg), proteinuria, and associated symp-toms such as headache, visual changes, abdominal pain,
Figure 1. The first head MRI revealed multi-ple high signal-intensity lesions onFLAIR/T2WI in the head of cau-date nucleus, bilateral putamen &corona radiata, centrum semio-valis, bilateral parieto-occipitalgyrus, midbrain-pontine area, bilat-eral cerebellum (white arrows inFigs. 1A-2A) and mild high signal-intensity lesions on DWI (whitearrows in Figs. 1B-2B). These pic-tures favor a process of vasogenicedema rather than cytotoxicedema is considered. No evidenceof sinus thrombosis was notedunder the MRV study (Figs. 1C-2C).
Figure 2. The follow-up head MR revealed complete resolutionof brain lesion on the previous head MRI study.
1A 1B 1C
2A 2B 2C
161
Acta Neurologica Taiwanica Vol 16 No 3 September 2007
or edema(13). Women with eclampsia after delivery usual-ly have lower blood pressures, minimal proteinuria, andsignificantly higher incidence of neurologic deficits thanthose with earlier-onset eclampsia(11). The pathophysiolo-gy of postpartum eclampsia may be different from thatof eclampsia in the antepartum period.
The pathophysiology of reversible posteriorencephalopathy syndrome probably involves focalbreakdown of the blood brain barrier, with a loss of fluidfrom the intravascular compartment(14). In addition, manycases associated with immunosuppressive agents alsohave evidence of at least episodic hypertension, perhapsfacilitating the diffusion of cyclosporine into brainparenchyma, where it may induce neuronal apoptosis oroligodendrocyte death(15). In regards to the imaging find-ings of RPES, the head computer tomography (CT) usu-ally is negative in the early stage. However, in our case,the brain CT revealed diffuse brain swelling, which mayreflex the severity of her disease status. In the advancedstage, usually bilateral white matter edema in the poste-rior regions of the cerebral hemispheres is noted. In therecent decades, head magnetic resonance imaging (MRI)showed many characteristics of this syndrome(16). Theunderlying pathophysiology can be demonstrated by spe-cial MR spectrum. Under the head MRI, RPES usuallyshows high signal-intensity lesions on FLAIR (fluid-attenuated inversion recovery) images, predominantlylocalized in (but not limited to) the subcortical whitematter (mainly association fibers) of bilateral occipitaland parietal lobes. On DWI (diffusion weighted imag-ing), focal lesions of subtle bright signal-intensitylesions not enough to call acute infarct support thehypothesis of vasogenic edema of brain tissue(16). Theconcurrent increased appearant diffusion coefficient(ADC) value over the lesion site also proves this theo-ry(16).
In this case, the initial differential diagnoses werevery important for the further treatment. Under theimpression of sudden onset seizure with consciouschanges and anisocoric pupils, an acute cortical lesionwith concurrent brain stem involvement was suspected.Because of the acute clinical course, cerebral infarct,cerebral venous sinus thrombosis, subarachoid hemor-
rhage and central nervous system infection should betaken into consideration. In such case with postpartumseizure, cerebral venous sinus thrombosis and postpar-tum eclampsia must be excluded firstly. Emergent headMRI was indicated to clarify all these etiologies.However, because of the post-ictal agitated status of thepatient, the standard protocol of head MRI (including T1weighted image, T2 weighted image, FLAIR, DWI,apparent diffusion coefficient-ADC, and magnetic reso-nance arteriography-MRA) was not completed. OnlyFLAIR, DWI, and magnetic resonance venography(MRV), which are critical for identifying acute braindamage and for making differential diagnosis, were per-formed in the first head MRI study. Because cerebralvenous sinus thrombosis was an important differentialdiagnosis in postpartum woman with seizure, head MRV,which is not included in the routine head MRI protocol,was also performed emergently in this case.
Even the etiology of the syndrome may be heteroge-neous, the treatment of this syndrome can be achievedunder the same roles: blood pressure control, seizurecontrol, removing the disposing factors, and supportivecare. If these problems can be controlled early enough,the outcome of the most cases of RPES will be good,like our patient; the patient can usually have a completerecovery without detectable neurological sequelae andabnormal head MRI findings can be reversed as well.However, if the vasogenic edema of the damaged braintissue persisted for a long time or the severity of damageis strong and extensive, the evolution of vasogenic brainedema to cytotoxic brain edema may develop, and it maylead to irreversible damage to neurons(17). In our case,phenytoin was used for seizure control before the finaldiagnosis was achieved. However, if the diagnosis of latepostpartum eclampsia was established early enough,only intravenous magnesium sulfate (MgSO4) therapywas needed for seizure control in late postpartumeclampsia, instead of conventional anticonvulsants(13).
In conclusion, the rare presentation of late postpar-tum eclampsia as reversible posterior encephalopathysyndrome (RPES) should be taken into the list of differ-ential diagnoses of postpartum seizure with brain stemdysfunction. The emergent head MRI is important for
162
Acta Neurologica Taiwanica Vol 16 No 3 September 2007
identifying the etiology. Appropriate therapy for RPEScan result in satisfactory prognosis before the irre-versible brain damage develops.
REFERENCES
1. Kwon S, Koo J, Lee S. Clinical spectrum of reversible pos-
