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CASE REPORTUDC 618.8-008.6

A REVERSIBLE POSTERIOR ENCEPHALOPATHY SYNDROME CAUSED BY ECLAMPSIA

PRES SINDROM KAO POSLJEDICA EKLAMPSIJE

Daliborka Tadić1, Siniša Miljković1, Vlado Đajić1, Vojo Buzadžija2, Biljana Đukić 3

Abstract: Th e clinical and radiological presentation described in patients with diff erent pathological condi-tions, such as hypertensive encephalopathy, eclampsia, uremia, use of immunosuppressives and porphyria, represents a syndrome called PRES, or a reversible posterior encephalopathy syndrome. We report two pa-tients with PRES due to eclampsia, aiming to draw attention of clinicians and radiologist to the existence of this clinical and radiological entity. When early recognized and adequately treated, PRES may be revers-ible, otherwise permanent neurological sequelae are likely.

Key words: posterior reversible encephalopathy syndrome, eclampsia, reversibility, neurological defi cit

Sažetak: Ista klinička slika i radiološka prezentacija opisana kod pacijenata sa više različitih patoloških stanja (hipertenzivna encefalopatija, eklampsija, uremija, primjena imunosupresivne terapije, porfi rija) predstavlja sindrom nazvan PRES (reversible posterior encephalopathy syndrome). Slučajevi prikazani u ovom radu su primjer PRES sindroma nastalog kao posljedica eklampsije. Željeli smo ih prikazati zbog potrebe da podsjetimo radiologe i kliničare na postojanje ovog kliničkog i radiološkog entiteta kod paci-jenata oboljelih od prethodno navedenih stanja, radi njegove potencijalne reverzibilnosti u slučaju pra-vovremene primjene adekvatne terapijske strategije, a u suprotnom, veoma izvjesne mogućnosti zaostajan-ja trajnog neurološkog defi cita.

Ključne riječi: PRES sindrom, eklampsija, reverzibilnost, neurološki defi cit

1 Clinic for Neurology, Clinical Centre of Banja Luka, Republika Srpska, Bosnia and Herzegovina2 Institute of Medical Rehabilitation ”Dr Miroslav Zotović”, Banja Luka, Republika Srpska, Bosnia and

Herzegovina3 Health Care Centre of Banja Luka, Republika Srpska, Bosnia and HerzegovinaCorrespondence to: Daliborka Tadić, MD, MSc, Address: Lovćenska 10, 78250 Laktaši, Republika Srpska, Bosnia and

Herzegovina, Tel. +38765659651, e-mail: tadic.daliborka@gmail.com* Received December 16, 2010; accepted March 22, 2011.

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INTRODUCTION

Since 1980, the same clinical picture and radiolog-ical fi ndings have been described in patients with diff erent pathological conditions, such as hyper-tensive encephalopathy, eclampsia, uremia, the use of immunosuppressive therapy, and porphyria (1). Hynchey and colleagues unifi ed all the conditions resulting from the same pathophysiological mech-anisms into a syndrome termed reversible posteri-or encephalopathy syndrome, or PRES (2). We re-port two patients with typical clinical and radio-logical presentation of eclamptic encephalopathy.

CASE PRESENTATION

CASE 1

A 19-year-old female patient developed high blood pressure accompanied by swelling of the legs and occasional headache during the seventh month of her fi rst pregnancy. Until then the pregnancy had been normal, and the patient had been healthy be-fore the pregnancy. Th e patient went into labor at the term and got to the gynecology ward of the lo-cal general hospital where the delivery was start-ed, which for its long duration ended in a cesare-an section. One day aft er the delivery the patient’s blood pressure increased and she complained of severe headache, followed by a generalized tonic-clonic seizure, aft er which the patient was somno-lent. Her condition persisted. Because of the com-plications, the patient was referred to the univer-sity hospital clinic for gynecology and obstetric in Banja Luka. She was examined by a neurolo-gist, and underwent brain CT, which revealed mi-crovascular ischemic lesions in the occipital sub-cortex bilaterally and the left frontal area. Th e pa-tient was referred to the university hospital neu-rology clinic in Banja Luka. On the neurologi-cal examination, all clinically signifi cant myotatic (deep tendon) refl exes were more brisk than usu-

al, Babinski’s sign was positive bilaterally, and the patient had displacement of the right bulbus oculi due to a previous injury. Th e high blood pressure persisted (210/140 mmHg). Somatic fi ndings of the body systems were normal. One day aft er the admission to the neurology clinic the patient was in a mild coma, responding to painful stimuli with inarticulate sounds. She was treated with antiede-ma drugs, sedatives, and antihypertensives. Sys-tolic blood pressure ranged 190-210 mmHg and diastolic 100-140 mmHg. On day 2 aft er admis-sion, the patient’s level of consciousness deterio-rated, she fell into a deep coma, not responding to painful stimuli. Lumbar punction was performed and the fi nding was a clear, cytologically and bio-chemically normal CSF. Magnetic resonance im-aging (MRI) of the brain performed on the same day showed a pathological signal on T2W/Flair sequences in both cerebral and cerebellar hemi-spheres, temporal and occipital lobes, and in the brain stem (Figure 1). Magnetic resonance venog-raphy (MRV) showed a reduced blood fl ow signal in the left transversal sinus to the sigmoid sinus and partly in the sigmoid sinus (venous throm-bus) (Figure 2). Since the fi ndings indicated brain tissue damage characteristic of PRES and cerebral venous sinus thrombosis, the patient was treated with anticoagulants and intensive antihyperten-sive and antibiotic therapy. On day 3, the patient’s level of consciousness improved; she was still som-nolent but responded with occasional adequate re-sponses. Within the following 10 days the patient’s condition gradually improved until a complete re-covery. Th e subsequent brain MRI-MRV exami-nation showed almost complete regression of pre-viously described changes in the white matter of the cerebral and cerebellar hemispheres, as well as recanalization of the thrombus in the transversal venous sinus (Figure 3). Aft er 23-day hospitaliza-tion at the neurology clinic, the patient was dis-charged, and the follow-up neurological examina-tion was completely normal.

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Figure 1. Brain MRI on admission

Figure 2. Brain MRV on admission

Figure 3. Brain MRI and MRV performed aft er 10 days

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CASE 2

A 35-year-old female was admitted to the inten-sive care unit in Banja Luka in the last trimester of her fi rst pregnancy for occasional variations in the blood pressure and peripheral edemas. Prior to the hospitalization, the patient had had a gener-alized tonic-clonic seizure and suff ered a head in-jury resulting from her having fallen onto a hard surface. Th e patient was transferred to the gyne-cology clinic where she underwent an emergen-cy cesarean section. On the neurological evalu-ation aft er the delivery, right-sided hemiparesis with a positive Babinski response and more ex-pressed myotatic refl exes were determined, so brain CT was performed, which revealed a hy-podensity zone along the posterior horn of the left lateral ventricle (Figure 4). Subsequently, the pa-tient had a series of generalized tonic-clonic sei-zures resistant to standard antiepileptic treatment and severe hypertension and was therefore hospi-talized in the intensive care unit of the gynecolo-gy clinic, where she was put into a barbiturate-in-duced coma. Aft er awakening from the three-day

barbiturate coma the patient did not have new sei-zures, but her high blood pressure persisted, up to 160/100mmHg. Th e patient was rehospitalized at the gynecology clinic, where a follow-up neu-rological examination was performed. Since the neurological defi cits persisted, brain MRI was per-formed and showed T2W/Flair hyperintense ar-eas deep in the occipital and temporal white mat-ter bilaterally, more pronounced on the right, cor-responding to the changes characteristic of PRES and aplasia of the right transversal sinus with col-lateral drainage (Figure 5). On the neurological examination fi ve days aft er delivery, the patient had small-amplitude horizontal nystagmus on lat-eral gaze, and the abnormal myotatic refl exes per-sisted. Her blood pressure was above normal. Af-ter her gynecological condition stabilized, the pa-tient was transferred to the neurology clinic. With antihypertensive and antiedema drugs, antibiotics and sedatives, the patient’s general condition sta-bilized and blood pressure normalized. Aft er ten days of treatment at the neurology clinic, the pa-tient was discharged, without neurological defi -cits.

Figure 4. Brain CT performed on admission

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Figure 5. Brain NMR performed aft er 5 days

DISCUSSIONA reversible posterior encephalopathy syndrome (PRES) is a term denoting several pathological conditions that have the same radiological presen-tation and result in the development of neurolog-ical defi cit. Th e syndrome was fi rst described by Hynchey et al. in 1996 (2).

Th e pathophysiological mechanism underlying PRES is basically the same, however it may be trig-gered by diff erent factors involving impaired auto-regulation of the cerebral blood fl ow due to hyper-tensive encephalopathy, eclampsia, iatrogenic fac-tors (cytostatics), toxic and metabolic disorders (uremia, porphyria) (3).

Th e diagnosis of PRES is established on the basis of the presence of high blood pressure and neu-rological manifestations such as headache, nausea, vomiting, confusion, behavioral disorders, im-paired level of consciousness (from somnolence to coma), visual disturbances (blurred vision, hemi-anopsia, cortical blindness), mental status chang-es (confusion, amnesia, impaired concentration, lethargy), and seizures. Th e radiological fi ndings include lesions in the white matter involving pos-terior circulation, i.e. mostly in the parieto-occip-ital lobes, although cases with involvements of the anterior circulation, thalamus and brainstem have also been described (4). Despite the dramatic neu-rological and radiological picture, the syndrome may be reversible, as suggested by its name. How-ever, if not adequately and timely treated, there may remain permanent neurological defi cits, most frequently in the form of epilepsy (5). Th e therapeutic approach depends on the underlying cause and includes antihypertensives, antiedema drugs, sedatives, and antiepileptics. Furthermore,

the presence of the syndrome requires induction of labor in the case of eclampsia, elimination of toxic blood products in the case of uremia, or dis-continuation of immunosuppressant drugs caus-ing the syndrome (6).

CONCLUSION

Th e two patients reported represent the typical clinical and radiological presentation of eclamptic encephalopathy. It is of the utmost importance for radiologists and clinicians to be aware of the ex-istence of this clinical and radiological entity, be-cause the syndrome may be reversible if an ade-quate and timely treatment is initiated, otherwise neurological sequelae are likely to persist.REFERENCES

Narborne MC, Musolino R, Granata F. PRES: pos-1. terior or potentially reversible encephalopathy syndrome? Neurol Sci. 2006; 27: 187-189.Hynchey I, Chaves C, Appignani B. A reversible 2. posterior encephalopathy syndrome. N Engl J Med. 1996; 334: 499-500. Servillo G, Striano P, Striano S. PRES (reversible 3. posterior encephalopathy syndrome) in critical obstetric patients. Intensiv Care Med. 2006; 29: 2323-2326.Bertynski WS, Boardman JE. Distinct imaging 4. patterns and lesion distribution of PRES . AINR 2007; 28:1320-7.Striano P. PRES : a dramatic but potentially revers-5. ible syndrome needing a prompt diagnosis. Neu-rol Sci 2006; 27:154.Petrović B, Kostić V, Šternić N, Kolar J, Tasić N. 6. Posterior reversible encephalopathy syndrome. Srp Arh Celok Lek 2003; 131: 461-6.