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DOI: 10.1055/s-2003-43451
Georg Thieme Verlag Stuttgart · New York
Zerebrale Perfusionsstörung und reversibles posteriores Leukenzephalopathie-Syndrom bei Eklampsie
Cerebral Circulation and Reversible Posterior Leukoencephalopathy Syndrome in EclampsiaPublication History
Eingang Manuskript: 15. Januar 2002
Eingang revidiertes Manuskript: 18. Juni 2003
Akzeptiert: 6. Juni 2003
Publication Date:
06 November 2003 (online)
Zusammenfassung
Eklampsie und Präeklampsie stellen die häufigste maternale Todesursache im Zusammenhang mit Schwangerschaft und Geburt in den entwickelten Ländern dar. Der vorliegende Artikel soll anhand von drei Kasuistiken, unter Berücksichtigung der aktuellen Literatur, eine Übersicht über die Ätiologie und Pathophysiologie zerebraler Perfusionsstörungen bei Eklampsie geben. Darauf aufbauend werden Diagnostik und Therapie neurologischer Störungen bei Eklampsie diskutiert und ein Vorschlag zur pragmatischen Vorgehensweise skizziert.
Bei der Eklampsie handelt es sich um eine systemische Erkrankung mit schwangerschaftsinduziertem arteriellen Hypertonus, generalisierter Endotheldysfunktion, gestörter Hämostase sowie Dysregulation von vaskulären und Entzündungsmediatoren. In der Folge entwickelt sich eine Schädigung innerer Organe und des Gehirns. Die zerebrale Arteriopathie führt zu komplexen Durchblutungsstörungen mit regionaler oder generalisierter Hyper- und Hypoperfusion. Bildmorphologisch entspricht das Schädigungsmuster dem „reversiblen posterioren Leukenzephalopathie-Syndrom“, welches durch multifokale zerebrale Ödeme, vor allem in den posterioren Großhirnhemisphären, gekennzeichnet ist. Sie bilden sich zumeist vollständig zurück, komplizierend können aber auch Hirninfarkte und -blutungen auftreten. Dopplersonographisch findet sich bei der Eklampsie ein Anstieg der Blutflussgeschwindigkeiten in den Hirnbasisarterien, verursacht durch Hyperperfusion oder Vasokonstriktion.
Die eklamptische Enzephalopathie ist durch epileptische Anfälle, Vigilanzstörung und fokal-neurologische Defizite gekennzeichnet. Zur Differenzierung von anderen akut-neurologischen Erkrankungen sind Kernspintomographie und neurologische Zusatzuntersuchungen erforderlich. Eine Überwachung der zerebralen Perfusion durch transkranielle Doppler- und farbkodierte Duplexsonographie sowie Kernspinangiographie ist ratsam. Im Falle einer hochgradigen Vasokonstriktion mit zerebraler Ischämie sollte der arterielle Blutdruck auf leicht hypertensive Werte eingestellt werden, um eine ausreichende Perfusion von Stenose und Kollateralkreisläufen zu gewährleisten. Eklamptische Anfälle sollten durch frühzeitige Gabe von Magnesiumsulfat oder Antikonvulsiva behandelt werden.
Aufgrund der komplexen Pathophysiologie und klinischen Manifestation empfiehlt sich eine interdisziplinäre Zusammenarbeit in der Diagnostik und Therapie der Eklampsie.
Abstract
Eclampsia and preeclampsia are the leading causes of maternal death in developed countries. We present three case reports and review the literature on the pathophysiology of impaired cerebral circulation and the resulting neurologic problems in women with eclampsia.
Eclampsia is a systemic condition with pregnancy-induced hypertension, generalized endothelial dysfunction, coagulation disorders and dysregulation of vascular and inflammatory mediators. This causes lesions of multiple visceral organs and the brain. Cerebral arteriopathy induces complex disturbances of cerebral perfusion with regional or generalized hyperperfusion or hypoperfusion. Imaging techniques show a pattern of lesions resembling the reversible posterior leukoencephalopathy syndrome. This syndrome is characterized by multifocal cerebral edema, predominantly in the posterior regions of the cerebral hemispheres. It usually resolves completely but cerebral infarction or hemorrhage have been described. Doppler sonography in women with eclampsia shows increased cerebral blood flow velocities due to hyperperfusion or vasoconstriction.
The systemic pathophysiology and varied clinical presentation of eclampsia requires interdisciplinary collaboration for diagnosis and treatment. Eclamptic encephalopathy is characterized by seizures, impaired consciousness and focal neurologic deficits. Magnetic resonance (MR) imaging and neurologic studies are required to distinguish eclamptic encephalopathy from other acute neurological events. Monitoring cerebral perfusion with transcranial Doppler and color-coded duplex sonography and MR angiography is advisable. With severe vasoconstriction and cerebral ischemia blood pressure should be controlled in the range of mild hypertension to support sufficient transstenotic and collateral blood flow. Eclamptic seizures are treated with magnesium sulphate or anticonvulsants.
Schlüsselwörter
Eklampsie - reversibles posteriores Leukenzephalopathie-Syndrom - Hirnödem - transkranielle Doppler-Sonographie - Kernspintomographie
Key words
Eclampsia - reversible posterior leukencephalopathy syndrome - brain edema - transcranial Doppler-sonography - magnetic resonance tomography
Literatur
- 1 Mas J L, Lamy C. Stroke in pregnancy and the puerperium. J Neurol. 1998; 245 305-313
- 2 Douglas K, Redman C. Eclampsia in the United Kingdom. BMJ. 1994; 309 1395-1400
- 3 Kittner S J, Stern B J, Feeser B R. Pregnancy and the risk of stroke. N Engl J Med. 1996; 335 768-774
- 4 Sharshar T, Lamy C, Mas J L. Incidence and causes of strokes associated with pregnancy and the puerperium. A study in public hospitals of Ile de France. Stroke. 1995; 26 930-936
- 5 Dietl J. The pathogenesis of pre-eclampsia: new aspects. J Perinat Med. 2000; 28 464-471
- 6 Granger J P, Alexander B T, Llinas M T, Bennett W A, Khalil R A. Pathophysiology of hypertension during preeclampsia linking placental ischemia with endothelial dysfunction. Hypertension. 2001; 38 718-722
- 7 Roberts J M, Redman C WG. Pre-eclampsia: more than pregnancy-induced hypertension. Lancet. 1993; 341 1447-1451
- 8 Trommer B L, Horner D, Mikhael M A. Cerebral vasospasm and eclampsia. Stroke. 1988; 19 326-329
- 9 Will A D, Lewis K L, Hinshaw D B, Jordan K, Cousins L M, Hasso A N, Thompson J R. Cerebral vasoconstriction in toxemia. Neurology. 1987; 37 1555-1557
- 10 Schwartz R B, Jones K M, Kalina P, Bajakian R B, Mantello M T, Garada B, Holman B L. Hypertensive encephalopathy: findings on CT, MR imaging, and SPECT imaging in 14 cases. Am J Roentgenol. 1992; 159 379-383
- 11 Zunker P, Happe S, Georgiadis A L, Louwen F, Georgiadis D, Ringelstein E B, Holzgreve W. Maternal cerebral hemodynamics in pregnancy-related hypertension. A prospective transcranial Doppler study. Ultrasound Obstet Gynecol. 2000; 16 179-187
- 12 Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, Pessin M S, Lamy C, Mas J L, Caplan L R. A reversible posterior leukencephalopathy syndrome. N Engl J Med. 1996; 334 494-500
- 13 Digre K B, Varner M W, Osborn A G, Crawford S. Cranial magnetic resonance imaging in severe preeclampsia vs eclampsia. Arch Neurol. 1993; 50 399-406
- 14 Arora A, Chowdhury D, Daga M K, Arora N, Gaiha M. Reversible posterior leukencephalopathy syndrome: a report of 2 cases. Neurol India. 2001; 49 311-313
- 15 Ay H, Buonanno F S, Schaefer P W, Le D A, Wang B, Gonzalez R G, Koroshetz W J. Posterior leukencephalopathy without severe hypertension. Utility of diffusion-weighted MRI. Neurology. 1998; 51 1369-1376
- 16 Niehaus L, Meyer B U, Hoffmann K T. Transiente kortikale Blindheit bei EPH-Gestose infolge zerebraler Vasospasmen. Nervenarzt. 1999; 70 931-934
- 17 Sawhney I M, Subrahmanyan A V, Das C P, Khandelwal N, Sawhney H, Vashita K. Neurological complications of eclampsia. J Assoc Physicians India. 1999; 47 1068-1071
- 18 Sheehan H L, Lynch J B. Pathology of Toxemia of Pregnancy. New York; Churchill Livingstone 1973
- 19 Minagar A, DeToledo J C, Falcone S. Cortical-subcortical lesions in “reversible posterior leukoencephalopathy syndrome”. Encephalopathy or seizures?. J Neurol. 2001; 248 537-540
- 20 Mukherjee P, McKinstry R C. Reversible posterior leukoencephalopathy syndrome: Evaluation with diffusion-tensor MR imaging. Radiology. 2001; 219 756-765
- 21 Arnolds B J, Reutern G M. Transcranial Doppler sonography. Examination technique and normal reference values. Ultrasound Med Biol. 1986; 12 115-123
- 22 Widder B. Doppler- und Duplexsonographie der hirnversorgenden Arterien. 5. Aufl. Berlin; Springer 1999
- 23 Pourcelot L. Applications cliniques de l'examen Doppler transcutané. Les colloques de l'Institut national de la Santé et de la Recherche médicale. INSERM. 1974; 34 2451-2456
- 24 Royburt M, Seidman D S, Serr D M, Mashiach S. Neurologic involvement in hypertensive disease of pregnancy. Obstet Gynecol Surv. 1991; 46 656-664
- 25 Seidman D S, Serr D M, Ben-Rafael Z. Renal and ocular manifestations of hypertensive diseases of pregnancy. Obstet Gynecol Surv. 1991; 46 71-76
- 26 Cunningham F G, Fernandez C O, Hernandez C. Blindness associated with preeclampsia and eclampsia. Am J Obstet Gynecol. 1995; 172 1291-1298
- 27 Veltkamp R, Kupsch A, Polasek J, Yousry T A, Pfister H W. Late onset postpartum eclampsia without pre-eclamptic prodromi: clinical and neuroradiological presentation in two patients. J Neurol Neurosurg Psychiatry. 2000; 69 824-827
- 28 Manfredi M, Beltramello A, Bongiovanni L G, Polo A, Pistoia L, Rizzuto N. Eclamptic encephalopathy: imaging and pathogenetic considerations. Acta Neurol Scand. 1997; 96 277-282
- 29 MacKenzie E T, Strandgaard S, Graham D I, Jones J V, Harper A M, Farrar J K. Effects of acutely induced hypertension in cats on pial arteriolar caliber, local cerebral blood flow, and the blood brain barrier. Circ Res. 1976; 39 33-41
- 30 Beausang-Linder M, Bill A. Cerebral circulation in acute arterial hypertension - protective effects of sympathetic nervous activity. Acta Physiol Scand. 1981; 111 193-199
- 31 Johansson B B. The blood-brain barrier and cerebral blood flow in acute hypertension. Acta Med Scand. 1983; 678 (Suppl. ) 107-112
- 32 Paulson O B, Strandgaard S, Edvinsson L. Cerebral autoregulation. Cerebrovasc Brain Metabolism Rev. 1990; 2 161-192
- 33 Tamaki K, Sadoshima S, Baumbach G L, Iadecola C, Reis D J, Heistad D D. Evidence that disruption of the blood-brain barrier precedes reduction in cerebral blood flow in hypertensive encephalopathy. Hypertension. 1984; 6 (Suppl I) I-75-I-81
- 34 Redman C WG. Eclampsia still kills. Br Med J. 1988; 296 1209-1213
- 35 Qureshi A I, Frankel M R, Ottenlips J R, Stern B J. Cerebral hemodynamics in preeclampsia and eclampsia. Arch Neurol. 1996; 53 1226-1231
- 36 Duncan R, Hadley D, Bone I, Symonds E M, Worthington B S, Rubin P C. Blindness in eclampsia: CT and MR imaging. J Neurol Neurosurgery Psychiatry. 1989; 52 899-902
-
37 Roberts J M.
Pregnancy related hypertension. Creasy RK, Resnik R Maternal-Fetal Medicine: Principles and Practice. 2. Aufl. Philadelphia; Saunders 1989: 277-823 - 38 Zammit V C, Whitworth J A, Brown M A. Preclampsia: the effect of serum in endothelial cell prostacyclin, endothelin, and cell membrane integrity. Am J Obstet Gynecol. 1996; 174 737-743
- 39 Schobel H P, Fischer T, Heuszer K, Geiger H, Schmieder R E. Preeclampsia - a state of sympathetic overactivity. N Engl J Med. 1996; 335 1480-1485
- 40 Conrad K P. Animal models of pre-eclampsia: do they exist?. Fetal Med Rev. 1990; 2 67-88
-
41 August P, Lindheimer M D.
Pathophysiology of preeclampsia. Laragh JL, Brenner BM Hypertension. 2. Aufl. New York; Raven Press 1995: 2407-2426 - 42 Roberts J M, Taylor R N, Goldfien A. Clinical and biochemical evidence of endothelial cell dysfunction in the pregnancy syndrome preeclampsia. Am J Hypertens. 1991; 4 700-708
- 43 Schaefer P W, Buonanno F S, Gonzalez G, Schwamm L H. Diffusion-weighted imaging discriminates between cytotoxic and vasogenic edema in a patient with eclampsia. Stroke. 1997; 28 1082-1085
- 44 Johansson B, Strandgaard S, Lassen N A. On the pathogenesis of hypertensive encephalopathy. Circ Res. 1974; 2 167-171
- 45 Johansson B B. The blood-brain barrier in acute and chronic hypertension. Adv Exp Med Biol. 1980; 131 211-226
- 46 Beausang-Linder M. Effects of sympathetic stimulation on cerebral and ocular blood flow. Acta Physiol Scand. 1982; 114 217-224
- 47 Nag S, Robertson D M, Dinsdale H B. Cerebral cortical changes in acute hypertension: an ultrastructural study. Lab Invest. 1977; 39 150-161
- 48 Giese J. Acute hypertensive vascular disease. 2: Studies on vascular reaction patterns and permeability changes by means of vital microscopy and colloidal tracer technique. Acta Pathol Microbiol Scand. 1964; 62 497-515
- 49 Johansson B B. The blood-brain barrier and cerebral blood flow in acute hypertension. Acta Med Scand. 1983; 678 (Suppl) 107-112
- 50 Strandgaard S. Autoregulation of cerebral circulation in hypertension. Acta Neurol Scand. 1978; 57 (Suppl) 1-82
- 51 Zunker P, Happe S, Louwen F, Evers S, Ringelstein E B. Peripartal temporal course of endothelin-1 (ET-1), angiotensin II (ANG II) and atrial natriuretic peptide (ANP) in preeclampsia and normotensive pregnancy. Fetal Diagn Ther. 1998; 13 308-313
- 52 Demarin V, Rundek T, Hodek B. Maternal cerebral circulation in normal and abnormal pregnancies. Acta Obstet Gynecol Scand. 1997; 76 619-624
- 53 Belfort M A, Giannina G, Herd J A. Transcranial and orbital Doppler ultrasound in normal pregnancy and preeclampsia. Clin Obstet Gynecol. 1999; 42 479-506
- 54 Williams K P, Wilson S. Persistence of cerebral hemodynamic changes in patients with eclampsia: a report of three cases. Am J Obstet Gynecol. 1999; 181 1162-1165
- 55 Hansen W F, Burnham S J, Svendsen T O, Katz V L, Thorp J M, Hansen A R. Transcranial Doppler findings of cerebral vasospasm in preeclampsia. J Matern Fetal Med. 1996; 5 194-200
- 56 Riskin-Mashia S, Belfort M A, Saade G R, Herd J A. Cerebrovascular reactivity in normal pregnancy and preeclampsia. Obstet Gynecol. 2001; 98 827-832
- 57 Zunker P, Ley-Pozo J, Louwen F, Schuierer G, Holzgreve W, Ringelstein E B. Cerebral hemodynamics in pre-eclampsia/eclampsia syndrome. Ultrasound Obstet Gynecol. 1995; 6 411-415
- 58 Serra-Serra V, Kyle P M, Chandran R, Redman C WG. Maternal middle cerebral artery velocimetry in normal pregnancy and postpartum. Br J Obstet Gynaecol. 1997; 104 904-909
- 59 Romner B, Bellner J, Kongstad P, Sjoholm H. Elevated transcranial Doppler flow velocities after severe head injury: cerebral vasospasm or hyperemia?. J Neurosurg. 1996; 85 90-97
- 60 Rajendran J G, Lewis D H, Newell D W, Winn H R. Brain SPECT used to evaluate vasospasm after subarachnoid hemorrhage: correlation with angiography and transcranial Doppler. Clin Nucl Med. 2001; 26 125-130
- 61 Lindegaard K F. The role of transcranial Doppler in the management of patients with subarachnoid haemorrhage - a review. Acta Neurochir Suppl. 1999; 72 59-71
- 62 The Magpie Trial Collaborative Group . Do women with pre-eclampsia, and their babies, benefit from magnesium sulphate? The Magpie Trial: a randomised placebo-controlled trial. Lancet. 2002; 359 1877-1890
- 63 Lucas M J, Leveno K J, Cunningham F G. A comparison of magnesium sulfate with phenytoin for the prevention of eclampsia. N Engl J Med. 1995; 333 201-205
- 64 Eclampsia Trial Collaborative Group . Which anticonvulsant for women with eclampsia? Evidence from the Collaborative Eclampsia Trial. Lancet. 1995; 345 1455-1461
- 65 Sadeh M. Action of magnesium sulfate in the treatment of preeclampsia-eclampsia. Stroke. 1989; 20 1273-1275
- 66 Goldman R, Finkbeiner S M. Therapeutic use of magnesium sulfate in selected cases of cerebral ischemia and seizure. N Engl Med J. 1988; 319 1224-1225
- 67 Victor M, Ropper (Hrsg) A H. Adams and Victor's Principles of Neurology. 7th ed. New York; McGraw-Hill 2001: 355-356
- 68 Belfort M A, Moise K J. Effect of magnesium sulfate on maternal brain blood flow in preeclampsia: a randomized, placebo-controlled study. Am J Obstet Gynecol. 1992; 167 661-666
- 69 Naidu S, Payne A J, Moodley J, Hoffmann M, Gouws E. Randomised study assessing the effect of phenytoin and magnesium sulphate on maternal cerebral circulation in eclampsia using transcranial Doppler ultrasound. Br J Obstet Gynaecol. 1996; 103 111-116
- 70 Easton J D. Severe preeclampsia/eclampsia: hypertensive encephalopathy of pregnancy?. Cerebrovasc Dis. 1998; 8 53-58
Dr. med. Eckard Oehm
Neurologische Universitätsklinik
Universität Freiburg
Breisacher Straße 64
79106 Freiburg
Email: oehm@nz.ukl.uni-freiburg.de