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Delayed angiographic changes in postpartum cerebral angiopathy
Journal of Clinical Neuroscience 18 (2011) 435–436
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Short Communication
Delayed angiographic changes in postpartum cerebral angiopathy Darshan Ghia a,c, Ramesh Cuganesan b, Cecilia Cappelen-Smith a,c,⇑ a
Department of Neurology and Neurophysiology, Liverpool Hospital, Locked bag 7103, NSW 1871, Australia Department of Radiology, Liverpool Hospital, Sydney, NSW, Australia c South Western Sydney Clinical School, The University of New South Wales, Sydney, NSW, Australia b
a r t i c l e
i n f o
Article history: Received 27 November 2009 Accepted 24 July 2010
Keywords: Headache Vasoconstriction Vasospasm
a b s t r a c t Postpartum cerebral angiopathy is a well-recognised subgroup of the reversible vasoconstriction syndromes. Increasingly described is a delay between clinical onset and angiographic changes. We report a patient who presented 19 days postpartum with severe thunderclap headaches, vertigo and a seizure. The clinical syndrome preceded evidence of extensive vasoconstriction on MRA imaging by 15 days; the changes were reversible at 3 months. Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved.
1. Case report A 39-year-old female presented with abrupt onset of multiple, very severe, thunderclap headaches, vomiting and acute vertigo 19 days postpartum (G6P4). Other than gestational diabetes, the pregnancy and delivery were normal. She was normotensive. There was a history of migraine. She smoked 20 cigarettes daily. There was no use of vasoactive drugs, lactation suppressants, or other medications. Neurological examination revealed left beating nystagmus, but was otherwise normal. Her brain CT scan and venogram were normal. Lumbar puncture, routine blood tests, urinalysis, vasculitic screen, human immunodeficiency virus testing, lactate and pyruvate levels were unremarkable. Day 5 after admission/illness onset she had a 3-minute generalized tonic–clonic seizure. She became hypertensive at 160/ 100 mmHg. She had ongoing thunderclap headaches, nausea and vomiting. Examination now showed left beating nystagmus, hyperreflexia and bilateral extensor plantar responses. MRI brain showed multiple cortical T2 hyperintensities in the frontal, parietal and occipital lobes without restricted diffusion (Fig. 1). Her brain magnetic resonance angiography (MRA) and her magnetic resonance venography were normal. Symptomatic treatment with morphine and metoclopramide was given. A 24-hour magnesium-sulphate infusion, followed by intravenous methylprednisone 1 g/day for 5 days were given without improvement of headaches. Verapamil (SR 180 mg) was commenced with gradual improvement in headache. Further neurological ⇑ Corresponding author. Tel.: +61 2 98283646; fax: +61 2 98283648. E-mail address: [email protected] (C. Cappelen-Smith).
symptoms including blurred vision and right face and arm sensory disturbance developed on day 13, but resolved over several hours. Her headache had completely resolved by day 21. Serial MRI scans on days 9, 15 and 23 showed cortical T2 hyperintensities in the frontal, parietal and occipital lobes without restricted diffusion. MRI on day 15 also showed two small areas of infarction in the left parietal and right frontal lobes. MRA was normal until day 15, and then showed diffuse segmental arterial constriction of the circle of Willis (Fig. 2). Despite clinical improvement and resolution of headaches, MRA on day 23 showed persistent diffuse segmental arterial constriction of the circle of Willis. She was discharged home day 24. Repeat MRI/MRA imaging at 3 months showed complete resolution of arterial constriction. 2. Discussion Reversible cerebral vasoconstriction syndromes (RCVS) may occur spontaneously or in secondary clinical settings including vasoactive drug use, and/or the postpartum state.1,2 Postpartum cerebral angiopathy, a well-recognized subgroup of RCVS, usually occurs after a normal pregnancy, as in our patient, and two-thirds of patients present in the first postpartum week.2 The clinical features of postpartum angiopathy include recurrent acute severe headaches (thunderclap), focal neurological deficits and seizures, which may occur over days to 4 weeks.2,3 The pathophysiology remains unknown; but is believed to involve a transient disturbance in control of cerebrovascular tone causing segmental and multifocal arterial constriction, with complete or near complete resolution on repeat angiography at 3 months.1 Suggested treatment includes oral or intravenous calcium channel blockers, however steroids are no longer recommended.2
0967-5868/$ - see front matter Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2010.07.103
436
D. Ghia et al. / Journal of Clinical Neuroscience 18 (2011) 435–436
Fig. 2. Magnetic resonance angiography showing (A) normal scan day 5 after illness onset; and (B) diffuse narrowing in multiple vessels including the middle, anterior and posterior cerebral arteries day 15 after illness onset.
Fig. 1. Axial T2-weighted MRI showing gyriform areas of cortical T2 hyperintensity in the fronto-parietal regions.
In postpartum angiopathy, a lag between onset and resolution of the clinical and angiographic features has been increasingly described.2,4,5 The earliest symptom is usually a thunderclap headache. Cortical subarachnoid haemorrhages and posterior reversible encephalopathy syndrome are seen early (week 1) and ischaemic complications (transient ischaemic attacks and strokes) occur later in the illness (end of week 2, often after headache resolution).1–4 Vasoconstriction may not be imaged until headache resolution and may be absent on early angiography.2,5 In our patient the clinical syndrome preceded evidence of extensive vasoconstriction on MRA by up to 15 days. Clinical
improvement occurred despite ongoing extensive vasoconstriction. The temporal course of these findings, and those previously described in the literature, suggests a sequential involvement of arterial beds. Initial involvement of small distal arteries (not visible on angiography) progresses over days or weeks to involve medium and large sized arteries (visible by angiography).2 Formal angiography may have a higher early yield;2,5 however, this technique has been reported to cause transient neurological deficits in RCVS.1 Therefore, non-invasive methods are generally preferable, and if MRA is normal at onset, then progress imaging may be diagnostic.2,5 References 1. Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101. 2. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009;9:256–67. 3. Chen SP, Fuh JL, Lirng JF, et al. Recurrent primary thunderclap headache and benign CNS angiopathy. Spectra of the same disorder? Neurology 2006;67:2164–9. 4. Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007;146:34–44. 5. Chen SP, Fuh JL, Wang SJ, et al. Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol 2010;67:648–56.
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Short Communication
Delayed angiographic changes in postpartum cerebral angiopathy Darshan Ghia a,c, Ramesh Cuganesan b, Cecilia Cappelen-Smith a,c,⇑ a
Department of Neurology and Neurophysiology, Liverpool Hospital, Locked bag 7103, NSW 1871, Australia Department of Radiology, Liverpool Hospital, Sydney, NSW, Australia c South Western Sydney Clinical School, The University of New South Wales, Sydney, NSW, Australia b
a r t i c l e
i n f o
Article history: Received 27 November 2009 Accepted 24 July 2010
Keywords: Headache Vasoconstriction Vasospasm
a b s t r a c t Postpartum cerebral angiopathy is a well-recognised subgroup of the reversible vasoconstriction syndromes. Increasingly described is a delay between clinical onset and angiographic changes. We report a patient who presented 19 days postpartum with severe thunderclap headaches, vertigo and a seizure. The clinical syndrome preceded evidence of extensive vasoconstriction on MRA imaging by 15 days; the changes were reversible at 3 months. Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved.
1. Case report A 39-year-old female presented with abrupt onset of multiple, very severe, thunderclap headaches, vomiting and acute vertigo 19 days postpartum (G6P4). Other than gestational diabetes, the pregnancy and delivery were normal. She was normotensive. There was a history of migraine. She smoked 20 cigarettes daily. There was no use of vasoactive drugs, lactation suppressants, or other medications. Neurological examination revealed left beating nystagmus, but was otherwise normal. Her brain CT scan and venogram were normal. Lumbar puncture, routine blood tests, urinalysis, vasculitic screen, human immunodeficiency virus testing, lactate and pyruvate levels were unremarkable. Day 5 after admission/illness onset she had a 3-minute generalized tonic–clonic seizure. She became hypertensive at 160/ 100 mmHg. She had ongoing thunderclap headaches, nausea and vomiting. Examination now showed left beating nystagmus, hyperreflexia and bilateral extensor plantar responses. MRI brain showed multiple cortical T2 hyperintensities in the frontal, parietal and occipital lobes without restricted diffusion (Fig. 1). Her brain magnetic resonance angiography (MRA) and her magnetic resonance venography were normal. Symptomatic treatment with morphine and metoclopramide was given. A 24-hour magnesium-sulphate infusion, followed by intravenous methylprednisone 1 g/day for 5 days were given without improvement of headaches. Verapamil (SR 180 mg) was commenced with gradual improvement in headache. Further neurological ⇑ Corresponding author. Tel.: +61 2 98283646; fax: +61 2 98283648. E-mail address: [email protected] (C. Cappelen-Smith).
symptoms including blurred vision and right face and arm sensory disturbance developed on day 13, but resolved over several hours. Her headache had completely resolved by day 21. Serial MRI scans on days 9, 15 and 23 showed cortical T2 hyperintensities in the frontal, parietal and occipital lobes without restricted diffusion. MRI on day 15 also showed two small areas of infarction in the left parietal and right frontal lobes. MRA was normal until day 15, and then showed diffuse segmental arterial constriction of the circle of Willis (Fig. 2). Despite clinical improvement and resolution of headaches, MRA on day 23 showed persistent diffuse segmental arterial constriction of the circle of Willis. She was discharged home day 24. Repeat MRI/MRA imaging at 3 months showed complete resolution of arterial constriction. 2. Discussion Reversible cerebral vasoconstriction syndromes (RCVS) may occur spontaneously or in secondary clinical settings including vasoactive drug use, and/or the postpartum state.1,2 Postpartum cerebral angiopathy, a well-recognized subgroup of RCVS, usually occurs after a normal pregnancy, as in our patient, and two-thirds of patients present in the first postpartum week.2 The clinical features of postpartum angiopathy include recurrent acute severe headaches (thunderclap), focal neurological deficits and seizures, which may occur over days to 4 weeks.2,3 The pathophysiology remains unknown; but is believed to involve a transient disturbance in control of cerebrovascular tone causing segmental and multifocal arterial constriction, with complete or near complete resolution on repeat angiography at 3 months.1 Suggested treatment includes oral or intravenous calcium channel blockers, however steroids are no longer recommended.2
0967-5868/$ - see front matter Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2010.07.103
436
D. Ghia et al. / Journal of Clinical Neuroscience 18 (2011) 435–436
Fig. 2. Magnetic resonance angiography showing (A) normal scan day 5 after illness onset; and (B) diffuse narrowing in multiple vessels including the middle, anterior and posterior cerebral arteries day 15 after illness onset.
Fig. 1. Axial T2-weighted MRI showing gyriform areas of cortical T2 hyperintensity in the fronto-parietal regions.
In postpartum angiopathy, a lag between onset and resolution of the clinical and angiographic features has been increasingly described.2,4,5 The earliest symptom is usually a thunderclap headache. Cortical subarachnoid haemorrhages and posterior reversible encephalopathy syndrome are seen early (week 1) and ischaemic complications (transient ischaemic attacks and strokes) occur later in the illness (end of week 2, often after headache resolution).1–4 Vasoconstriction may not be imaged until headache resolution and may be absent on early angiography.2,5 In our patient the clinical syndrome preceded evidence of extensive vasoconstriction on MRA by up to 15 days. Clinical
improvement occurred despite ongoing extensive vasoconstriction. The temporal course of these findings, and those previously described in the literature, suggests a sequential involvement of arterial beds. Initial involvement of small distal arteries (not visible on angiography) progresses over days or weeks to involve medium and large sized arteries (visible by angiography).2 Formal angiography may have a higher early yield;2,5 however, this technique has been reported to cause transient neurological deficits in RCVS.1 Therefore, non-invasive methods are generally preferable, and if MRA is normal at onset, then progress imaging may be diagnostic.2,5 References 1. Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101. 2. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009;9:256–67. 3. Chen SP, Fuh JL, Lirng JF, et al. Recurrent primary thunderclap headache and benign CNS angiopathy. Spectra of the same disorder? Neurology 2006;67:2164–9. 4. Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007;146:34–44. 5. Chen SP, Fuh JL, Wang SJ, et al. Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol 2010;67:648–56.