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Alejandro Rabinstein, MD

Mayo Clinic

� No relevant financial disclosures

� There are conditions that are more

common during pregnancy and

puerperium or exclusive of this period

� Pregnancy can cause exacerbation of

pre-existent neurological diseases

� Imaging limitations

� Treatment limitations

� High stakes

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� HeadacheHeadacheHeadacheHeadache

� SeizuresSeizuresSeizuresSeizures

� Focal deficitsFocal deficitsFocal deficitsFocal deficits

� Visual symptomsVisual symptomsVisual symptomsVisual symptoms

� PrePrePrePre----eclampsiaeclampsiaeclampsiaeclampsia ���� EclampsiaEclampsiaEclampsiaEclampsia� Postpartum Postpartum Postpartum Postpartum angioplathyangioplathyangioplathyangioplathy (RCVS)(RCVS)(RCVS)(RCVS)

� PRESPRESPRESPRES

� Cerebral Venous ThrombosisCerebral Venous ThrombosisCerebral Venous ThrombosisCerebral Venous Thrombosis

� ICHICHICHICH

� Ischemic strokeIschemic strokeIschemic strokeIschemic stroke

� Pituitary hemorrhage � apoplexy (�Sheehan Sx)� Choriocarcinoma

� Amniotic fluid embolism

� Air embolism

� TTP

� PrePrePrePre----eclampsiaeclampsiaeclampsiaeclampsia (2(2(2(2----8 % of all pregnancies)8 % of all pregnancies)8 % of all pregnancies)8 % of all pregnancies)

◦ BP > 140/90BP > 140/90BP > 140/90BP > 140/90

◦ Proteinuria > 300 mg in 24 Proteinuria > 300 mg in 24 Proteinuria > 300 mg in 24 Proteinuria > 300 mg in 24 hrhrhrhr

◦ Severe if: Severe if: Severe if: Severe if:

� BP > 160/110 x 2 (6 BP > 160/110 x 2 (6 BP > 160/110 x 2 (6 BP > 160/110 x 2 (6 hrhrhrhr apart) apart) apart) apart)

� Proteinuria > Proteinuria > Proteinuria > Proteinuria > 5 5 5 5 g in 24 g in 24 g in 24 g in 24 hrhrhrhr

� Other signs of endOther signs of endOther signs of endOther signs of end----organ damageorgan damageorgan damageorgan damage

� EclampsiaEclampsiaEclampsiaEclampsia◦ PrePrePrePre----eclampsiaeclampsiaeclampsiaeclampsia + Seizures+ Seizures+ Seizures+ Seizures

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� Smooth BP controlSmooth BP controlSmooth BP controlSmooth BP control

� Magnesium sulfate: 5g IV bolus Magnesium sulfate: 5g IV bolus Magnesium sulfate: 5g IV bolus Magnesium sulfate: 5g IV bolus ����2g/h infusion2g/h infusion2g/h infusion2g/h infusion

� Benzodiazepines can be used for Benzodiazepines can be used for Benzodiazepines can be used for Benzodiazepines can be used for persistent seizurespersistent seizurespersistent seizurespersistent seizures

� Yet, magnesium was superior to Yet, magnesium was superior to Yet, magnesium was superior to Yet, magnesium was superior to diazepam and phenytoin in head to diazepam and phenytoin in head to diazepam and phenytoin in head to diazepam and phenytoin in head to head RCTshead RCTshead RCTshead RCTs

� Commonly seen with Commonly seen with Commonly seen with Commonly seen with eclampsiaeclampsiaeclampsiaeclampsia

� VasogenicVasogenicVasogenicVasogenic edema characteristic but not edema characteristic but not edema characteristic but not edema characteristic but not indispensableindispensableindispensableindispensable

� Perhaps the underlying mechanism for Perhaps the underlying mechanism for Perhaps the underlying mechanism for Perhaps the underlying mechanism for most if not all cases of most if not all cases of most if not all cases of most if not all cases of eclampsiaeclampsiaeclampsiaeclampsia

� Can be associated with vasoconstriction Can be associated with vasoconstriction Can be associated with vasoconstriction Can be associated with vasoconstriction (RCVS spectrum)(RCVS spectrum)(RCVS spectrum)(RCVS spectrum)

� Can result in ischemia, hemorrhageCan result in ischemia, hemorrhageCan result in ischemia, hemorrhageCan result in ischemia, hemorrhage

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The atypical patterns:CerebellumThe atypical patterns:Cerebellum

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Lancet Lancet Lancet Lancet NeurolNeurolNeurolNeurol 2015;14:9142015;14:9142015;14:9142015;14:914----925925925925....

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Endothelial Dysfunction

Sudden hypertension

Vasoconstriction

Hyperperfusion

T cell activation

HypoperfusionCytokine release

� Abrupt onset postAbrupt onset postAbrupt onset postAbrupt onset post----partumpartumpartumpartum

� Thunderclap headache, recurrentThunderclap headache, recurrentThunderclap headache, recurrentThunderclap headache, recurrent

� Ischemic stroke, ICH, SAH (Ischemic stroke, ICH, SAH (Ischemic stroke, ICH, SAH (Ischemic stroke, ICH, SAH (convexalconvexalconvexalconvexal))))

� Can be associated with PRES (seizures, Can be associated with PRES (seizures, Can be associated with PRES (seizures, Can be associated with PRES (seizures, visual changes, transient focal deficits)visual changes, transient focal deficits)visual changes, transient focal deficits)visual changes, transient focal deficits)

� CSF: slight CSF: slight CSF: slight CSF: slight pleocytosispleocytosispleocytosispleocytosis, mildly high , mildly high , mildly high , mildly high proteinproteinproteinprotein

� Vasoconstriction may not be initially Vasoconstriction may not be initially Vasoconstriction may not be initially Vasoconstriction may not be initially visiblevisiblevisiblevisible

EclampsiaEclampsiaEclampsiaEclampsia

PRESPRESPRESPRES RCVSRCVSRCVSRCVS

All endothelial disorders?All endothelial disorders?All endothelial disorders?All endothelial disorders?

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18 18 18 18 ptsptsptspts3 centers3 centers3 centers3 centersMedian age: 38 Median age: 38 Median age: 38 Median age: 38 yryryryr38 weeks gestation38 weeks gestation38 weeks gestation38 weeks gestationSymptoms startedSymptoms startedSymptoms startedSymptoms started5 days after delivery5 days after delivery5 days after delivery5 days after delivery70% had 70% had 70% had 70% had abnlabnlabnlabnl imagingimagingimagingimaging(ICH, ischemia, edema)(ICH, ischemia, edema)(ICH, ischemia, edema)(ICH, ischemia, edema)

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� More variable than previously reportedMore variable than previously reportedMore variable than previously reportedMore variable than previously reported

� Full recovery = 50%Full recovery = 50%Full recovery = 50%Full recovery = 50%

� Residual deficits = 28%Residual deficits = 28%Residual deficits = 28%Residual deficits = 28%

� DeathsDeathsDeathsDeaths = 22% (n=4)= 22% (n=4)= 22% (n=4)= 22% (n=4)

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� Mostly postpartum (75%)

� Risk factors: dehydration, anemia, intracranial

hypotension (epidural)

� Headache, seizures, focal signs

� Generally progressive over several days

� LP with high OP and CSF can have moderately

high protein and few cells

� Imaging: edema, venous infarctions

� Venogram is diagnostic (non-invasive

venograms are very accurate)

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� Heparin or LMWH during pregnancyHeparin or LMWH during pregnancyHeparin or LMWH during pregnancyHeparin or LMWH during pregnancy

� WarfarinWarfarinWarfarinWarfarin: crosses placenta, teratogenic = : crosses placenta, teratogenic = : crosses placenta, teratogenic = : crosses placenta, teratogenic = AVOIDAVOIDAVOIDAVOIDduring pregnancy during pregnancy during pregnancy during pregnancy

� Warfarin can be used safely during Warfarin can be used safely during Warfarin can be used safely during Warfarin can be used safely during puerperiumpuerperiumpuerperiumpuerperium(not contraindicated in lactating mothers) (not contraindicated in lactating mothers) (not contraindicated in lactating mothers) (not contraindicated in lactating mothers)

� DOACs: No information on safety during DOACs: No information on safety during DOACs: No information on safety during DOACs: No information on safety during pregnancypregnancypregnancypregnancy

� Avoid OCPs afterwardsAvoid OCPs afterwardsAvoid OCPs afterwardsAvoid OCPs afterwards

� When to check for thrombophilia?When to check for thrombophilia?When to check for thrombophilia?When to check for thrombophilia?◦ RecurrentRecurrentRecurrentRecurrent

◦ Family historyFamily historyFamily historyFamily history

� Epidemiological observation in Maryland and Epidemiological observation in Maryland and Epidemiological observation in Maryland and Epidemiological observation in Maryland and Washington DCWashington DCWashington DCWashington DC

� Risk increased during Risk increased during Risk increased during Risk increased during puerperiumpuerperiumpuerperiumpuerperium but not but not but not but not during pregnancyduring pregnancyduring pregnancyduring pregnancy

� ARR 8.7 for ischemia and 28.3 for ICHARR 8.7 for ischemia and 28.3 for ICHARR 8.7 for ischemia and 28.3 for ICHARR 8.7 for ischemia and 28.3 for ICH

� Attributable excess risk: 8.1strokes per Attributable excess risk: 8.1strokes per Attributable excess risk: 8.1strokes per Attributable excess risk: 8.1strokes per 100,000 pregnancies100,000 pregnancies100,000 pregnancies100,000 pregnancies

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� PeripartumPeripartumPeripartumPeripartum cardiomyopathycardiomyopathycardiomyopathycardiomyopathy

� DissectionsDissectionsDissectionsDissections

� VasoconstrictionVasoconstrictionVasoconstrictionVasoconstriction

� Amniotic fluid embolismAmniotic fluid embolismAmniotic fluid embolismAmniotic fluid embolism

� Air embolismAir embolismAir embolismAir embolism

� The risk of rupture from previously The risk of rupture from previously The risk of rupture from previously The risk of rupture from previously unrupturedunrupturedunrupturedunruptured AVMs does AVMs does AVMs does AVMs does notnotnotnot appear to be appear to be appear to be appear to be increased during pregnancy or increased during pregnancy or increased during pregnancy or increased during pregnancy or puerperiumpuerperiumpuerperiumpuerperium

Neurology 2014;82:1798-1803

Neurosurgery 1990;27:867-871

� The risk of bleeding from a cavernous The risk of bleeding from a cavernous The risk of bleeding from a cavernous The risk of bleeding from a cavernous malformation does malformation does malformation does malformation does notnotnotnot appear to be appear to be appear to be appear to be increased during pregnancy or increased during pregnancy or increased during pregnancy or increased during pregnancy or puerperiumpuerperiumpuerperiumpuerperium

Neurosurgery 2012;71:626-630

� Ergo, pregnancy should Ergo, pregnancy should Ergo, pregnancy should Ergo, pregnancy should notnotnotnot be discouraged in be discouraged in be discouraged in be discouraged in patients with these vascular anomaliespatients with these vascular anomaliespatients with these vascular anomaliespatients with these vascular anomalies

� Particular set of diagnoses and challengesParticular set of diagnoses and challengesParticular set of diagnoses and challengesParticular set of diagnoses and challenges

� EclampsiaEclampsiaEclampsiaEclampsia: magnesium : magnesium : magnesium : magnesium –––– think of PRESthink of PRESthink of PRESthink of PRES

� Thunderclap headache Thunderclap headache Thunderclap headache Thunderclap headache ���� think RCVS think RCVS think RCVS think RCVS

(postpartum (postpartum (postpartum (postpartum angiopathyangiopathyangiopathyangiopathy) and be very watchful) and be very watchful) and be very watchful) and be very watchful

� Postpartum Postpartum Postpartum Postpartum angiopathyangiopathyangiopathyangiopathy usually has a good usually has a good usually has a good usually has a good

prognosis, but it can be fulminantprognosis, but it can be fulminantprognosis, but it can be fulminantprognosis, but it can be fulminant

� Think of CVT if progressive postThink of CVT if progressive postThink of CVT if progressive postThink of CVT if progressive post----partum partum partum partum

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