孕妇产后神经疾病的诊断

1、ReviewDiagnosis of acute neurological emergencies in pregnant and post-partum womenJonathan A Edlow, Louis R Caplan, Karen OBrien, Carrie D TibblesAcute neurological symptoms in pregnant and post-partum women could be caused by exacerbation of a pre-existing neurological condition, the initial prese

2、ntation of a non-pregnancy-related problem, or a new acute-onset neurological problem that is either unique to or occurs with increased frequency during or just after pregnancy. Pregnant and postpartum patients with headache and neurological symptoms are often diagnosed with pre-eclampsia; however,

3、a range of other causes must also be considered, such as cerebral venous sinus thrombosis and reversible cerebral vasoconstriction syndrome. Precise diagnosis is essential to guide subsequent management. Our ability to di erentiate between the speci c causes of acute neurological symptoms in pregnan

4、t and post-partum patients is likely to improve as we learn more about the pathogenesis of these disorders. Lancet Neurol 2013; 12: 17585Department of Emergency Medicine (Prof J A Edlow MD, C D Tibbles MD, Department of Neurology (Prof L R Caplan MD, and Department of Obstetrics and Gynecology, Divi

5、sion of Maternal and Fetal Medicine, (K OBrien MD Beth IsraelDeaconess Medical Center,Harvard Medical School,Boston, MA, USAaccount for the seizure. Up to 0·6% of mildly pre-Acute neurological symptoms in pregnant and eclamptic women and 23% of severely pre-eclamptic Correspondence to:postpartu

6、m women could be caused by exacerbation of a women have eclamptic seizures.4 Maternal mortality Prof Jonathan A Edlow, pre-existing neurological condition (eg, multiple sclerosis rates for eclamptic women have been reported to be Department of EmergencyMedicine, Beth Israel Deaconess or a seizure di

7、sorder or by initial presentation of a 014% during the past few decades, and are higher in Medical Center, West Clinical non-pregnancy-related problem (eg, brain neo p lasm. poor countries than in high-income countries.5 The most Center, Boston, MA 02215, USAPre-eclampsia occurs in 28% of all pregna

8、ncies.3 H ere we review clinical presentations and diagnostic Eclampsia is de ned as pre-eclampsia and a grand mal evaluation of common and serious neurological seizure in the absence of other conditions that could emergencies that present in pregnant and post-partumReviewFigure 1: Diagnostic algori

9、thm for pregnant and postpartum patients with acute neurological symptoms(A Diagnostic approach for pregnant and post-partum women with acute neurological symptoms. (B Diagnostic approach for pregnant and post-partum patientswith isolated headache. (C Diagnostic approach for patients with other neur

10、ological symptoms or signs, or eclamptic patients not responding to treatment. Ifpatients are being monitored and are not improving, the clinician should loop back to more testing. SAH=subarachnoid haemorrhage. CVT=cerebral venous sinusthrombosis. RCVS=reversible cerebral vasoconstriction syndrome.

11、PRES=posterior reversible encephalopathy syndrome. MRA/CTA=magnetic resonance angiographyand CT angiography.(eg, blackness or loss of vision.12 Positive phenomenaThe neurological symptoms develop and usuallybrightness or sparkling in vision, tingling, or prickling disappear in 2030 min. Because visu

12、al symptoms arefeelings in the limbs or bodyspread gradually and often common with pre-eclampsia, diagnosis should not belead to loss of function, such as scotoma or numbness. made without a consideration of other disorders thatSymptoms often clear in one modality, for example a ect the visual pathw

13、ays, such as PRES, pituitaryvision, and then begin and spread in another modality. apoplexy, and strokes. Another consideration is orbital Reviewhaemorrhage, which presents as acute diplopia, proptosis, Valsalva or neck hyperextension during anaesthesia;and eye pain, and can arise during the rst tri

14、mester however, no convincing epidemiological evidence for this(from hyperemesis and during labour (from pushing.32 exists. In the largest reported series of eight postpartumStroke in pregnant and post-partum women is rare; cases, the only di erences between post-partum and non-however, risk is incr

15、eased compared with non-pregnant post-partum cases were coincidental PRES, RCVS, andage-matched controls, especially in late pregnancy and SAH , which further complicated the occurrence ofearly puerperium.21,33 Recent evidence suggests that the overlapping clinical syndromes in these patients.43 Mos

16、t ofrate of strokes occurring during pregnancy and these dissections occur during the post-partum period,postpartum is increasing, substantially for ICH and but they have also been reported during pregnancy.46CVT. 34 The ranges of event rates per 100 000 deliveries are In patients with ICH and SAH,

17、underlying structural411 (AIS, 3·79·0 (ICH, 2·47·0 (SAH, and 0·724·0 lesions such as vascular malformations and aneurysms(CVT.21,3338 These studies varied in their methods are common.33,35,38,40 SAH that occurs around the Circle of(table 1. The extreme range for CVT pro

18、bably results Willis is suggestive of an aneurysm, whereas highfrom di erent case- nding de nitions and technological convexal SAH suggests RCVS or CVT. Brain infarctionevaluations used in various studies. Additionally, the very and haemorrhage can result from many of thelow stroke rate in the most

19、recent study (table 1 is very vasculopathies, including RCVS and pre-eclampsia.likely attributable to the exclusion of post-partum Finally, thrombotic thrombo c ytopenic purpura, pituitary39patients (ie, the period of highest risk. apoplexy, amniotic uid embolism, choriocarcinoma, airPre-eclampsia a

20、nd eclampsia have causal roles in embolism, and cardioembolism from post-partum2550% of patients with stroke (table 1.21,33,35,38,40 Other cardiomyopathy are rare causes of stroke in thisstroke risk factors in these women include older age, population. 47 Extensive diagnostic testing includingAfrica

21、n-American race, concurrent hypertension and vascular imaging must be done in these patients toheart disease, caesarean delivery, migraine, thrombo-identify speci c treatable causes.philia, systemic lupus erythematosus, sickle cell disease,and thrombocytopenia.34,36,37 Thrombocytopenia also Seizures

22、suggests the HELLP syndrome (ie, haemolysis, elevated Pregnant or post-partum women with seizures can beliver enzymes, low platelets and thrombotic thrombo-grouped into three categories: rst, and most common,cytopenic purpura, the incidence of which is increased in are patients with an established s

23、eizure disorder beforepregnancy and which can present with stroke-like pregnancy; 48 second are patients with a new non-symptoms. 41 Another unusual cause of stroke in pregnant pregnancy-related seizure disorder, such as a new seizureand post-partum women is cervicocranial arterial from an undiagnos

24、ed brain tumour or hypoglycaemia;dissection. Although some reports have shown an third are patients with new seizures that are pregnancyincreased frequency in pregnant and post-partum women, related (caused by eclampsia, ICH, CVT, RCVS, PRES, orc ytopenic purpura. Whereas in no strong epidemiologica

25、l evidence that the incidence of thrombotic thrombocervicocranial arterial dissection is heightened in this patients with PRES, seizures are common and usuallypopulation exists.42 Patients with cervico-arterial occur at presentation in the absence of prodromaldissections often present with isolated

26、headache without symptoms, in CVT seizures usually occur later andneurological de cit,43 but they can also have brain nearly always after headache; brain CT can be normal ininfarctions. 44,45 Possible causal factors are labour-related both conditions. Seizures are much less common in ReviewRCVS. 49

27、Other than to emphasise that pregnant and post-partum patients need the same systematic diagnosticapproach to a new seizure as do all seizure patients, wewill not focus on the rst two groups.No quality data inform the ideal initial work-up inpatients in the third category. However, because of widedi

28、 erential diagnosis and the poor sensitivity of CT, webelieve that pregnant and post-partum patients with new-onset seizureseven those who have returned to normaland are neurologically intactshould undergo thoroughinvestigation, usually including MRI sequences, toestablish the cause of the seizure.

29、The one exception toroutine imaging is the patient whose seizures areconsistent with typical prenatal eclampsia ( gure 1.underlying thrombophilia,40 but more than 75% of cases of CVT are post partum.54 Risk factors include caesarean section, dehydration, traumatic delivery, anaemia, raised homocyste

30、ine concentrations, and low CSF pressure due to dural puncture from a neuraxial anaesthetic.28,37 CVT is believed to be more common in poor countries than in high-income countries because of the higher frequency of poor nutrition, infections, and dehydration.55,56 Patients with CVT caused by pregnan

31、cy or the use of oral contraceptive generally have better long-term outcomes than do men or women with CVT that is unrelated to pregnancy. 54Most patients present with a progressively severe, di use, constant headache, although 10% have thunderclapheadache. 57,58 Other ndings include dizziness, naus

32、ea,seizures, papilloedema, lateralising signs, lethargy, and Individual conditions that cause acutecoma. The speci c presentation depends on the extent and neurological symptomslocation of the dural sinuses and draining veins involved, Clinical featuresects on intracranial pressure, and The clinical

33、 presentations of acute neurological collateral circulation, esymptoms in pregnant and post-partum women have the presence of associated haemorrhage.59 Symptoms varysubstantial overlap between them, and several disorders and can uctuate over time.28,50,56 Non-contrast CT scans arecan even coexist. H

34、owever, the detailseg, headache often negative, but 30% of cases might show a clot or signscharacteristics, evolution of symptoms over time, and of infarction ( gure 2.60 Ischaemic infarcts often undergofrequency of some symptoms such as seizures or visual haemor r hagic transformation. CT venograph

35、y often showsproblemscan often help to distinguish between them the clot, but magnetic resonance venographyespecially(table 2.with gradient spin-echo sequencesis diagnostic andgenerally the imaging study of choice.60Cerebral venous sinus thrombosisA rare cause of stroke overall, CVT is an importantc

36、onsideration in pregnant and post-partum women.5053A spike in incidence in the rst trimester might beattributable to women who become pregnant with an Reversible cerebral vasoconstriction syndromeRCVS is characterised by abrupt onset of thunderclap headaches and multifocal, reversible cerebral vaso-

37、constriction. 61 Two-thirds of patients with RCVS develop ReviewReviewconsistent with infarction or focal haemorrhage.85 Thus, components of PRES, areas of ischaemia or haemorrhage, and even RCVS can contribute to eclamptic seizures.Roughly 90% of eclampsia cases occur at or after 28 weeks of gestat

38、ion.5 Just over a third of eclamptic seizures occur at term, and develop intrapartum or within 48 h of delivery.5 So-called late post-partum eclampsiaie, eclampsia that arises more than 48 h after deliveryis being increasingly reported. In one large study of post-partum eclampsia,87 discharged and r

39、e-admitted because of late post-partum pre-eclamptic symptoms, most commonly headache. The proportion of pre-eclampsia and eclampsia diagnosed post partum is 1155% and the gures might increase with improved antepartum recognition.8891 Post-partum headache or abdominal painand only seek medical care

40、later, after a seizure.88,92Patients with post-partum eclampsia, especially those with late post-partum eclampsia, have a higher incidence of CVT, ICH, and AIS than do eclamptic patients diagnosed pre partum.5,93 Although most women with typical eclampsia do not need brain imaging,5 post-partum ecla

41、mptic patients, those with focal neurological de cits, persistent visual disturbances, and symptoms refractory to magnesium and antihypertensive treatment should undergo thorough diagnostic testing, preferably including MRI. Imaging might also reveal areas of vasoconstriction consistent with RCVS an

42、d, rarely, pregnant patients, especially those with RCVS, develop craniocervical arterial dissections.4345,94 Thus, the range of neurological imaging ndings in pre-eclamptic and eclamptic patients includes infarction, haemorrhage, vasoconstriction, dissection, and both vasogenic and cytotoxic oedema

43、.Rare conditions that cause acute neurological symptomsAmniotic uid embolism and metastatic choriocarcinoma are two pregnancy speci c conditions that can present with neurological symptoms. Amniotic uid embolism causes agitation, confusion, seizures, and encephalopathy in the context of cardiovascul

44、ar and respiratory collapse during or immediately after labour.95,96 Choriocarcinoma, a rare cancer of trophoblastic tissue, metastasises to the brain in 20% of patients.97,98 Because the tumour can cause mass e ect, bleed, and invade cerebral vessels, its clinical and imaging manifestations are var

45、iable.98,99Air embolism occurs when air that enters the myometrium during delivery enters the venous circulation and right ventricle, reducing cardiac output and resulting in seizures and abnormal cognition during or just after delivery.100 Nearly any focal or generalised neurological symptom can al

46、so occur because of right-to-left intracardiac shunting of air via a patent foramen ovale. 100 Presence of air in the retinal veins and a so-called mill-wheel cardiac murmur suggest the diagnosis.ReviewSearch strategy and selection criteriaWe searched for literature published from Jan 1, 1980 to Sep

47、 20, 2012. Because we were focusing on more than one condition, we searched PubMed for English language articles containing the terms “pregnancy” or “postpartum” in the abstract in conjunction with other key terms, including“pre-eclampsia”, “eclampsia”, “headache”, “seizures”, “stroke”, “visual symp

48、toms”, “PRES”, “reversible cerebralvasoconstriction”, and “cerebral vein thrombosis”, in the title. We manually searched the article bibliographies and our personal les for other sources. We gave priority to papers published in the past 10 years that included new data on diagnosis in large numbers o

49、f patients and those addressing serious neurological conditions. We also searched theNational Guideline Clearinghouse and modi ed our reference list on the basis of reviewers comments. Most of the articles that we identi ed in our literature search were case series (often in referral populations and review articles withconsensus-based recommendations. There were fewsystematic analyses of the diagnosis of consecutive patients in routine practice. We eliminated articles about minor(non-life-threatening neurological conditions (eg, carpal tunnel syndrome or peripheral seventh