CASE REPORT UDC 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: The clinical and radiological presentation described in patients with different pathological conditions, 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 patients 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 reversible, otherwise permanent neurological sequelae are likely. Key words: posterior reversible encephalopathy syndrome, eclampsia, reversibility, neurological deficit Sažetak: Ista klinička slika i radiološka prezentacija opisana kod pacijenata sa više različitih patoloških stanja (hipertenzivna encefalopatija, eklampsija, uremija, primjena imunosupresivne terapije, porfirija) 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 pacijenata oboljelih od prethodno navedenih stanja, radi njegove potencijalne reverzibilnosti u slučaju pravovremene primjene adekvatne terapijske strategije, a u suprotnom, veoma izvjesne mogućnosti zaostajanja trajnog neurološkog deficita. Ključne riječi: PRES sindrom, eklampsija, reverzibilnost, neurološki deficit 1 Clinic for Neurology, Clinical Centre of Banja Luka, Republika Srpska, Bosnia and Herzegovina 2 Institute of Medical Rehabilitation Dr Miroslav Zotović, Banja Luka, Republika Srpska, Bosnia and Herzegovina 3 Health Care Centre of Banja Luka, Republika Srpska, Bosnia and Herzegovina Correspondence 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. 36
. INTRODUCTION Since 1980, the same clinical picture and radiological findings have been described in patients with different pathological conditions, such as hypertensive encephalopathy, eclampsia, uremia, the use of immunosuppressive therapy, and porphyria (1). Hynchey and colleagues unified all the conditions resulting from the same pathophysiological mechanisms into a syndrome termed reversible posterior encephalopathy syndrome, or PRES (2). We report two patients with typical clinical and radiological 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 first pregnancy. Until then the pregnancy had been normal, and the patient had been healthy before the pregnancy. The patient went into labor at the term and got to the gynecology ward of the local general hospital where the delivery was started, which for its long duration ended in a cesarean section. One day after the delivery the patient s blood pressure increased and she complained of severe headache, followed by a generalized tonicclonic seizure, after which the patient was somnolent. Her condition persisted. Because of the complications, the patient was referred to the university hospital clinic for gynecology and obstetric in Banja Luka. She was examined by a neurologist, and underwent brain CT, which revealed microvascular ischemic lesions in the occipital subcortex bilaterally and the left frontal area. The patient was referred to the university hospital neurology clinic in Banja Luka. On the neurological examination, all clinically significant myotatic (deep tendon) reflexes were more brisk than usual, Babinski s sign was positive bilaterally, and the patient had displacement of the right bulbus oculi due to a previous injury. The high blood pressure persisted (210/140 mmhg). Somatic findings of the body systems were normal. One day after the admission to the neurology clinic the patient was in a mild coma, responding to painful stimuli with inarticulate sounds. She was treated with antiedema drugs, sedatives, and antihypertensives. Systolic blood pressure ranged 190-210 mmhg and diastolic 100-140 mmhg. On day 2 after admission, the patient s level of consciousness deteriorated, she fell into a deep coma, not responding to painful stimuli. Lumbar punction was performed and the finding was a clear, cytologically and biochemically normal CSF. Magnetic resonance imaging (MRI) of the brain performed on the same day showed a pathological signal on T2W/Flair sequences in both cerebral and cerebellar hemispheres, temporal and occipital lobes, and in the brain stem (Figure 1). Magnetic resonance venography (MRV) showed a reduced blood flow signal in the left transversal sinus to the sigmoid sinus and partly in the sigmoid sinus (venous thrombus) (Figure 2). Since the findings indicated brain tissue damage characteristic of PRES and cerebral venous sinus thrombosis, the patient was treated with anticoagulants and intensive antihypertensive and antibiotic therapy. On day 3, the patient s level of consciousness improved; she was still somnolent but responded with occasional adequate responses. Within the following 10 days the patient s condition gradually improved until a complete recovery. The subsequent brain MRI-MRV examination showed almost complete regression of previously 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). After 23-day hospitalization at the neurology clinic, the patient was discharged, and the follow-up neurological examination was completely normal. 37
Figure 1. Brain MRI on admission Figure 2. Brain MRV on admission Figure 3. Brain MRI and MRV performed after 10 days 38
CASE 2 A 35-year-old female was admitted to the intensive care unit in Banja Luka in the last trimester of her first pregnancy for occasional variations in the blood pressure and peripheral edemas. Prior to the hospitalization, the patient had had a generalized tonic-clonic seizure and suffered a head injury resulting from her having fallen onto a hard surface. The patient was transferred to the gynecology clinic where she underwent an emergency cesarean section. On the neurological evaluation after the delivery, right-sided hemiparesis with a positive Babinski response and more expressed myotatic reflexes were determined, so brain CT was performed, which revealed a hypodensity zone along the posterior horn of the left lateral ventricle (Figure 4). Subsequently, the patient had a series of generalized tonic-clonic seizures resistant to standard antiepileptic treatment and severe hypertension and was therefore hospitalized in the intensive care unit of the gynecology clinic, where she was put into a barbiturate-induced coma. After awakening from the three-day barbiturate coma the patient did not have new seizures, but her high blood pressure persisted, up to 160/100mmHg. The patient was rehospitalized at the gynecology clinic, where a follow-up neurological examination was performed. Since the neurological deficits persisted, brain MRI was performed and showed T2W/Flair hyperintense areas deep in the occipital and temporal white matter bilaterally, more pronounced on the right, corresponding to the changes characteristic of PRES and aplasia of the right transversal sinus with collateral drainage (Figure 5). On the neurological examination five days after delivery, the patient had small-amplitude horizontal nystagmus on lateral gaze, and the abnormal myotatic reflexes persisted. Her blood pressure was above normal. After her gynecological condition stabilized, the patient was transferred to the neurology clinic. With antihypertensive and antiedema drugs, antibiotics and sedatives, the patient s general condition stabilized and blood pressure normalized. After ten days of treatment at the neurology clinic, the patient was discharged, without neurological deficits. Figure 4. Brain CT performed on admission 39
Figure 5. Brain NMR performed after 5 days DISCUSSION A reversible posterior encephalopathy syndrome (PRES) is a term denoting several pathological conditions that have the same radiological presentation and result in the development of neurological deficit. The syndrome was first described by Hynchey et al. in 1996 (2). The pathophysiological mechanism underlying PRES is basically the same, however it may be triggered by different factors involving impaired autoregulation of the cerebral blood flow due to hypertensive encephalopathy, eclampsia, iatrogenic factors (cytostatics), toxic and metabolic disorders (uremia, porphyria) (3). The diagnosis of PRES is established on the basis of the presence of high blood pressure and neurological manifestations such as headache, nausea, vomiting, confusion, behavioral disorders, impaired level of consciousness (from somnolence to coma), visual disturbances (blurred vision, hemianopsia, cortical blindness), mental status changes (confusion, amnesia, impaired concentration, lethargy), and seizures. The radiological findings include lesions in the white matter involving posterior circulation, i.e. mostly in the parieto-occipital lobes, although cases with involvements of the anterior circulation, thalamus and brainstem have also been described (4). Despite the dramatic neurological and radiological picture, the syndrome may be reversible, as suggested by its name. However, if not adequately and timely treated, there may remain permanent neurological deficits, most frequently in the form of epilepsy (5). The therapeutic approach depends on the underlying cause and includes antihypertensives, antiedema drugs, sedatives, and antiepileptics. Furthermore, 40 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 discontinuation of immunosuppressant drugs causing the syndrome (6). CONCLUSION The 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 existence of this clinical and radiological entity, because the syndrome may be reversible if an adequate and timely treatment is initiated, otherwise neurological sequelae are likely to persist. REFERENCES 1. Narborne MC, Musolino R, Granata F. PRES: posterior or potentially reversible encephalopathy syndrome? Neurol Sci. 2006; 27: 187-189. 2. Hynchey I, Chaves C, Appignani B. A reversible posterior encephalopathy syndrome. N Engl J Med. 1996; 334: 499-500. 3. Servillo G, Striano P, Striano S. PRES (reversible posterior encephalopathy syndrome) in critical obstetric patients. Intensiv Care Med. 2006; 29: 2323-2326. 4. Bertynski WS, Boardman JE. Distinct imaging patterns and lesion distribution of PRES. AINR 2007; 28:1320-7. 5. Striano P. PRES : a dramatic but potentially reversible syndrome needing a prompt diagnosis. Neurol Sci 2006; 27:154. 6. Petrović B, Kostić V, Šternić N, Kolar J, Tasić N. Posterior reversible encephalopathy syndrome. Srp Arh Celok Lek 2003; 131: 461-6.