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Quiz Page September 2011
QUIZ PAGE SEPTEMBER 2011 A Patient With Postpartum Hypertension and Seizure CLINICAL PRESENTATION Eight days after delivering a full-term healthy baby, a 27-year-old woman with a history of hypothyroidism presents to the emergency department with shortness of breath, systolic blood pressure of 180 mm Hg, and features of mild congestive heart failure. The pregnancy was uneventful, without hypertension or evidence of preeclampsia. On evaluation, complete blood cell count and basic metabolic panel results were in the reference ranges. Uric acid level was 7.3 mg/dL (434 mol/L), and D-dimer level was ⬎1,000 mg/L FEU (fibrinogen equivalent unit). A chest radiograph suggested pulmonary congestion, and echocardiogram and computed tomographic angiogram findings were unremarkable. She responded to diuretic therapy and was discharged to home. Soon after, the patient developed blurring of vision and headaches, and on postpartum day 12, she experienced a tonic-clonic seizure. After the seizure, she had no focal neurologic deficits. Blood pressure was 170/96 mm Hg, and laboratory investigation results were within the reference ranges, except for a uric acid level of 7.8 mg/dL (464 mol/L) and mild proteinuria using urine dipstick. Computed tomography (CT) of the head (Fig 1) and subsequent magnetic resonance imaging (MRI) of the brain were performed (Fig 2).
Figure 1. Computed tomographic scan of the patient’s head.
What causes hypertension in the peripartum period? What is the pathophysiology of this condition? What imaging abnormalities are seen in this case? What are the treatment and prognosis for this condition?
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Magnetic resonance image of the patient’s brain with T2, fluid-attenuated inversion recovery (FLAIR), and diffusionweighted imaging sequences.
Figure 2.
QUIZ PAGE SEPTEMBER 2011 ANSWERS DISCUSSION
Box 1. Causes of PRES ●
f What causes hypertension in the peripartum period?
QUIZ PAGE
The common causes of hypertension in this clinical scenario include the following: ●
Preeclampsia/eclampsia
●
Pre-existing hypertension
●
Preeclampsia superimposed on pre-existing hypertension
●
Gestational hypertension
●
Posterior reversible leukoencephalopathy syndrome (PRES)
Preeclampsia typically is marked by new-onset hypertension and proteinuria after 20 weeks of gestation, whereas gestational hypertension is newonset hypertension after 20 weeks of gestation without proteinuria. Eclampsia is defined as a seizure complicating either of these conditions, typically occurring late in the third trimester or in the early postpartum period. Occasionally, a patient with hypertension predating pregnancy develops proteinuria after 20 gestational weeks. PRES, a syndrome of reversible encephalopathy with radiologic evidence of posterior white matter involvement, can occur in several settings, including the peripartum period (Box 1). Clinical features of PRES are listed in Box 2. xxx
● ● ●
●
● ● ●
Hypertension 〫 Well controlled, uncontrolled, malignant Pregnancy 〫 Pregnancy-induced hypertension, eclampsia Vasculitides 〫 SLE, Wegener granulomatosis, PAN, cryoglobulinemia Drug induced 〫 Immunosuppressive agents, such as cyclosporine, tacrolimus, sirolimus 〫 Chemotherapeutic agents, such as methotrexate, cisplatin, cytarabine, vincristine, sunitinib, bevacizumab 〫 Immunomodulators, such as IVIG, interferon alfa, rituximab 〫 Contrast agents Hematologic disorders 〫 Thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, porphyria, lymphoproliferative malignancies, blood transfusion Acute & chronic kidney diseases Idiopathic Miscellaneous 〫 Sepsis, hypomagnesemia, hypocalcemia
Abbreviations: IVIG, intravenous immunoglobulin; PAN, polyarteritis nodosa; PRES, posterior reversible leukoencephalopathy syndrome; SLE, systemic lupus erythematosus.
f What is the pathophysiology of this condition? PRES results from a combination of deranged cerebral autoregulation and endothelial dysfunction.1 Increasing blood pressure, especially that which occurs suddenly, overwhelms cerebral autoregulation, leading to arteriolar dilatation and an unregulated increase in cerebral blood flow. This hyperperfusion causes extravasation of fluid and protein, resulting in vasogenic edema.1,2 Endothelial dysfunction from the release of inflammatory cytokines causes protein and fluid leakage into the interstitium. Endothelial dysfunction has been implicated in patients with preeclampsia, sepsis, autoimmune disorders, and those on cytotoxic therapy.1,3 Cerebral ischemia caused by reactive focal vasospasm after failure of
cerebral autoregulation has been noted in some severe cases,4 but is not the usual mechanism of injury in PRES. f What imaging abnormalities are seen in this case? Typical neuroradiologic changes of PRES are in the white matter and posterior brain. White matter is more vulnerable to edema than the cortex because of the density of the tissue. The posterior brain, with its relative lack of sympathetic innervations, is more prone to blood pressure fluctuations. Patients with sepsis have more cortical involvement. Diffuse subcortical edema over the bilateral frontal and posterior parietal lobes with obliteration of cortical sulci usually is evident on CT. MRI will show more extensive changes inAm J Kidney Dis. 2011;58(3):xxix-xxxi
Box 2. Clinical Presentation of PRES ● ● ● ● ● ●
Headache: insidious, nonfocal, unresponsive to analgesics Altered mental state: ranging from mild confusion to coma Visual disturbances: blurring of vision, field defects, even cortical blindness Seizures: usually tonic-clonic and rarely status epilepticus Hypertension: most patients are hypertensive, but some might be normotensive Neurologic examination: brisk reflexes, Babinski positive, incoordination and rarely weakness, depressed Glasgow Coma Scale score (may be normal) Abbreviation: PRES, posterior reversible leukoencephalopathy syndrome.
volving the occipital lobes and bilateral cerebellar white matter. f What are the treatment and prognosis for this condition? PRES has no specific treatment, but prompt recognition and reversal/removal of the underlying trigger when possible usually leads to resolution of the clinical presentation with good clinical outcomes. Increased blood pressure, seen in most patients, should be decreased cautiously. Seizures can be treated using benzodiazepines and/or phenytoin. Magnesium sulfate is used safely in patients with eclampsia. In antenatal presentation, delivery of the fetus may facilitate resolution. Patients with PRES do not show increased susceptibility to recurrence of seizures after the clinical condition has resolved, and they do not need long-term treatment with anticonvulsants. Although PRES usually is benign and fully reversible within
FINAL DIAGNOSIS Posterior reversible leukoencephalopathy syndrome (PRES).
REFERENCES 1. Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334(8):494-500. 2. Primavera A, Audenino D, Mavilio N, et al. Reversible posterior
CASE PROVIDED AND AUTHORED BY Ashish Verma, MD, Paul Jodka, MD, and Shaji P. Daniel, MD, Baystate Medical Center/Tufts University School of Medicine, Springfield, MA. Address correspondence to Shaji P. Daniel, MD, Pioneer Valley Nephrology, 300 Stafford St, Springfield, MA 01104. E-mail: [email protected] © 2011 by the National Kidney Foundation, Inc. doi:10.1053/j.ajkd.2011.04.022 SUPPORT: None. FINANCIAL DISCLOSURE: The authors declare that they have no relevant financial interests.
xxxi
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Am J Kidney Dis. 2011;58(3):xxix-xxxi
days to weeks, excellent outcomes are not universal.5 An increased creatinine level, MRI findings of hyperintense signals on diffusion-weighted imaging, and more extensive brain involvement, particularly in the brain stem, are poor prognostic markers. The patient’s clinical and radiologic presentation is consistent with PRES. She was treated successfully using labetalol and magnesium sulfate. Follow-up MRI confirmed resolution of the imaging abnormalities seen at presentation.
leucoencephalopathy syndrome in systemic lupus and vasculitis. Ann Rheum Dis. 2001;60(5):534-537. 3. Dekker GA, Sibai BM. Etiology and pathogenesis of preeclampsia: current concepts. Am J Obstet Gynecol. 1998;179(5):1359-1375. 4. Koch S, Rabinstein A, Falcone S, Forteza A. Diffusionweighted imaging shows cytotoxic and vasogenic edema in eclampsia. AJNR Am J Neuroradiol. 2001; 22(6):1068-1070. 5. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusionweighted MR images. AJNR Am J Neuroradiol. 2002;23(6):10381048.
Figure 1. Computed tomographic scan of the patient’s head.
What causes hypertension in the peripartum period? What is the pathophysiology of this condition? What imaging abnormalities are seen in this case? What are the treatment and prognosis for this condition?
xxix
QUIZ PAGE
Am J Kidney Dis. 2011;58(3):xxix-xxxi
Magnetic resonance image of the patient’s brain with T2, fluid-attenuated inversion recovery (FLAIR), and diffusionweighted imaging sequences.
Figure 2.
QUIZ PAGE SEPTEMBER 2011 ANSWERS DISCUSSION
Box 1. Causes of PRES ●
f What causes hypertension in the peripartum period?
QUIZ PAGE
The common causes of hypertension in this clinical scenario include the following: ●
Preeclampsia/eclampsia
●
Pre-existing hypertension
●
Preeclampsia superimposed on pre-existing hypertension
●
Gestational hypertension
●
Posterior reversible leukoencephalopathy syndrome (PRES)
Preeclampsia typically is marked by new-onset hypertension and proteinuria after 20 weeks of gestation, whereas gestational hypertension is newonset hypertension after 20 weeks of gestation without proteinuria. Eclampsia is defined as a seizure complicating either of these conditions, typically occurring late in the third trimester or in the early postpartum period. Occasionally, a patient with hypertension predating pregnancy develops proteinuria after 20 gestational weeks. PRES, a syndrome of reversible encephalopathy with radiologic evidence of posterior white matter involvement, can occur in several settings, including the peripartum period (Box 1). Clinical features of PRES are listed in Box 2. xxx
● ● ●
●
● ● ●
Hypertension 〫 Well controlled, uncontrolled, malignant Pregnancy 〫 Pregnancy-induced hypertension, eclampsia Vasculitides 〫 SLE, Wegener granulomatosis, PAN, cryoglobulinemia Drug induced 〫 Immunosuppressive agents, such as cyclosporine, tacrolimus, sirolimus 〫 Chemotherapeutic agents, such as methotrexate, cisplatin, cytarabine, vincristine, sunitinib, bevacizumab 〫 Immunomodulators, such as IVIG, interferon alfa, rituximab 〫 Contrast agents Hematologic disorders 〫 Thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, porphyria, lymphoproliferative malignancies, blood transfusion Acute & chronic kidney diseases Idiopathic Miscellaneous 〫 Sepsis, hypomagnesemia, hypocalcemia
Abbreviations: IVIG, intravenous immunoglobulin; PAN, polyarteritis nodosa; PRES, posterior reversible leukoencephalopathy syndrome; SLE, systemic lupus erythematosus.
f What is the pathophysiology of this condition? PRES results from a combination of deranged cerebral autoregulation and endothelial dysfunction.1 Increasing blood pressure, especially that which occurs suddenly, overwhelms cerebral autoregulation, leading to arteriolar dilatation and an unregulated increase in cerebral blood flow. This hyperperfusion causes extravasation of fluid and protein, resulting in vasogenic edema.1,2 Endothelial dysfunction from the release of inflammatory cytokines causes protein and fluid leakage into the interstitium. Endothelial dysfunction has been implicated in patients with preeclampsia, sepsis, autoimmune disorders, and those on cytotoxic therapy.1,3 Cerebral ischemia caused by reactive focal vasospasm after failure of
cerebral autoregulation has been noted in some severe cases,4 but is not the usual mechanism of injury in PRES. f What imaging abnormalities are seen in this case? Typical neuroradiologic changes of PRES are in the white matter and posterior brain. White matter is more vulnerable to edema than the cortex because of the density of the tissue. The posterior brain, with its relative lack of sympathetic innervations, is more prone to blood pressure fluctuations. Patients with sepsis have more cortical involvement. Diffuse subcortical edema over the bilateral frontal and posterior parietal lobes with obliteration of cortical sulci usually is evident on CT. MRI will show more extensive changes inAm J Kidney Dis. 2011;58(3):xxix-xxxi
Box 2. Clinical Presentation of PRES ● ● ● ● ● ●
Headache: insidious, nonfocal, unresponsive to analgesics Altered mental state: ranging from mild confusion to coma Visual disturbances: blurring of vision, field defects, even cortical blindness Seizures: usually tonic-clonic and rarely status epilepticus Hypertension: most patients are hypertensive, but some might be normotensive Neurologic examination: brisk reflexes, Babinski positive, incoordination and rarely weakness, depressed Glasgow Coma Scale score (may be normal) Abbreviation: PRES, posterior reversible leukoencephalopathy syndrome.
volving the occipital lobes and bilateral cerebellar white matter. f What are the treatment and prognosis for this condition? PRES has no specific treatment, but prompt recognition and reversal/removal of the underlying trigger when possible usually leads to resolution of the clinical presentation with good clinical outcomes. Increased blood pressure, seen in most patients, should be decreased cautiously. Seizures can be treated using benzodiazepines and/or phenytoin. Magnesium sulfate is used safely in patients with eclampsia. In antenatal presentation, delivery of the fetus may facilitate resolution. Patients with PRES do not show increased susceptibility to recurrence of seizures after the clinical condition has resolved, and they do not need long-term treatment with anticonvulsants. Although PRES usually is benign and fully reversible within
FINAL DIAGNOSIS Posterior reversible leukoencephalopathy syndrome (PRES).
REFERENCES 1. Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334(8):494-500. 2. Primavera A, Audenino D, Mavilio N, et al. Reversible posterior
CASE PROVIDED AND AUTHORED BY Ashish Verma, MD, Paul Jodka, MD, and Shaji P. Daniel, MD, Baystate Medical Center/Tufts University School of Medicine, Springfield, MA. Address correspondence to Shaji P. Daniel, MD, Pioneer Valley Nephrology, 300 Stafford St, Springfield, MA 01104. E-mail: [email protected] © 2011 by the National Kidney Foundation, Inc. doi:10.1053/j.ajkd.2011.04.022 SUPPORT: None. FINANCIAL DISCLOSURE: The authors declare that they have no relevant financial interests.
xxxi
QUIZ PAGE
Am J Kidney Dis. 2011;58(3):xxix-xxxi
days to weeks, excellent outcomes are not universal.5 An increased creatinine level, MRI findings of hyperintense signals on diffusion-weighted imaging, and more extensive brain involvement, particularly in the brain stem, are poor prognostic markers. The patient’s clinical and radiologic presentation is consistent with PRES. She was treated successfully using labetalol and magnesium sulfate. Follow-up MRI confirmed resolution of the imaging abnormalities seen at presentation.
leucoencephalopathy syndrome in systemic lupus and vasculitis. Ann Rheum Dis. 2001;60(5):534-537. 3. Dekker GA, Sibai BM. Etiology and pathogenesis of preeclampsia: current concepts. Am J Obstet Gynecol. 1998;179(5):1359-1375. 4. Koch S, Rabinstein A, Falcone S, Forteza A. Diffusionweighted imaging shows cytotoxic and vasogenic edema in eclampsia. AJNR Am J Neuroradiol. 2001; 22(6):1068-1070. 5. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusionweighted MR images. AJNR Am J Neuroradiol. 2002;23(6):10381048.