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Severe Reversible Cerebral Vasoconstriction Syndrome with Large Posterior Cerebral Infarction
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Severe Reversible Cerebral Vasoconstriction Syndrome with Large Posterior Cerebral Infarction Hiroki Yamada, MD, Ryogo Kikuchi, MD, PhD, Akiyoshi Nakamura, MD, PhD, and Hiromichi Miyazaki, MD, PhD
Reversible cerebral vasoconstriction syndrome is characterized by thunderclap headache and multifocal cerebral vasoconstriction. Cerebral vasoconstriction is reversible, and most cases have good prognosis. However, clinical outcome is possibly severe when it is complicated by stroke, yet detailed reports on such a case are few. We experienced a case of severe reversible cerebral vasoconstriction syndrome in a 32-year-old woman with medical history of preeclampsia 3 years prior. She presented with sudden sharp headache followed by altered mental status and vasoconstriction of the bilateral posterior cerebral arteries. She was treated with intravenous and oral calcium channel blockers, edaravone, and glycerol. However, the cerebral infarction in the posterior circulation subsequently remained, and her impaired consciousness did not recover. Furthermore, although imaging findings of vasoconstriction showed improvement a day after the occurrence of symptom, the same vessels showed poor visualization 7 weeks later, which indicated the recurrence of vasoconstriction, without additional symptom due to the fixed infarction. Although most cases of reversible cerebral vasoconstriction syndrome show good prognosis, neurologists must monitor the possibility of worse clinical course and permanent neurological deficit when associated with stroke, such as cerebral infarction. Strict management and treatment are needed in these cases. Key Words: Cerebral infarction—permanent neurological deficit—severe reversible cerebral vasoconstriction syndrome—stroke © 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved.
Case Report A 32-year-old woman, with medical history of preeclampsia 3 years prior, experienced sudden severe headache and subsequent coma. She had no significant family history or history of illicit drug use; she has allergy to ampicillin, but has not taken it recently. Upon arrival, her blood pressure, heart rate, oxygen saturation, and body temperature were 158/96 mmHg, 70 beats per minute, 100% (under From the Department of Neurosurgery, Hiratsuka City Hospital, Hiratsuka, Kanagawa 254-0065, Japan. Received May 21, 2018; revision received June 28, 2018; accepted June 30, 2018. Grant Support: None. Address correspondence to Hiroki Yamada, md, Department of Neurosurgery, Hiratsuka City Hospital, 1-19-1, Minamihara, Hiratsuka, Kanagawa 254-0065, Japan. E-mail: [email protected] 1052-3057/$ - see front matter © 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jstrokecerebrovasdis.2018.06.044
10 L of oxygen), and 36.1 °C, respectively. Head computed tomography, magnetic resonance imaging, and magnetic resonance angiography (MRA) showed restricted cortical subarachnoid hemorrhage (SAH); a wide, unclear, yet high-intensity area of the diffusion-weighted image of the posterior circulation; and vasoconstriction of the bilateral posterior cerebral arteries (PCAs), with no cerebral aneurysm detected (Fig 1A C, respectively). Computed tomography venography showed no abnormal findings. Blood test showed mild increase in white blood cells (10,500/mL), but no other abnormal findings including D-dimer, C-reactive protein, and autoantibodies; cerebrospinal fluid data were within normal limits. Reversible cerebral vasoconstriction syndrome (RCVS) was considered, and continuous intravenous nicardipine and oral verapamil, edaravone, and glycerol were administered. However, her impaired consciousness continued. On the following day, MRA showed improvement of the PCA vasoconstriction, which confirmed the RCVS diagnosis, but magnetic resonance imaging showed completion of a large infarction in
Journal of Stroke and Cerebrovascular Diseases, Vol. &&, No. && (&&), 2018: pp 1-3
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ARTICLE IN PRESS H. YAMADA ET AL.
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Figure 1. (A) Computed tomography during onset, showing bilateral parietal cortical subarachnoid hemorrhage. (B) Magnetic resonance image (MRI) diffusion-weighted image (DWI) during onset, showing an unclear yet high-intensity area at the bilateral occipital lobes and thalamus. (C) Magnetic resonance angiography (MRA) during onset, showing multiple vasoconstriction of the posterior cerebral artery (PCA) (white arrow heads). (D) MRI DWI performed on the following day, showing a clear high-intensity area. (E) MRA performed on the following day, showing improvement of vasoconstriction. (F) MRA performed 7 weeks after, showing poor visualization of the bilateral PCAs.
the bilateral posterior circulatory areas (Fig 1D, E) and bilateral thalamus infarction, which was considered as the cause of the impaired consciousness. The cortical SAH was unchanged. In contrast to the characteristic reversibility of RCVS, MRA performed at 7 weeks from admission showed poor visualization of the bilateral PCAs, and basilar artery (Fig 1F), but no new symptom appeared. She recovered slightly and could respond to a simple question, such as moving her fingertip, and was transferred to rehabilitation hospital 8 weeks from the onset.
Discussion RCVS can be severe when complicated by large cerebral infarction (CI). Most RCVS cases are benign,1,2 and detailed reports on severe cases remain few.3-5 Even when complicated with cerebral hemorrhage, good neurological recovery has been reported in some cases.6 Puerperal RCVS cases tend to be severe,7 but the present case was not postpartum. The patient was diagnosed to have RCVS from the beginning; thus, reversibility of vasoconstriction and symptom was expected. However, the large CI remained, and her impaired consciousness did not recover. Thus, neurologists must be aware that RCVS with CI can be serious, and strict monitoring and management of CI should be performed from the start. In this
case, managing with blood pressure or antithrombotic drugs during the initial stage could be considered, after confirming that the SAH was not increasing. In addition, intra-arterial calcium channel blockers or milrinone could be considered as a diagnostic and treatment option.8-10 We believe that the poorly visualized bilateral PCAs and basilar artery after the improvement of vasoconstriction can be attributed to the re-vasoconstriction. This contradicts the reversible characteristic of RCVS and is considered to be a recurrent type. In fact, RCVS recurrence is reported to occur in approximately 5% of all RCVS cases.11 Fortunately, this case did not have further harmful symptom, possibly due to the already fixed large CI. Therefore, in RCVS complicated by fixed CI, prognosis can be severe, and radiological findings in the cerebral vessels may show poor visualization after improvement of vasoconstriction. Stroke occurrence should be closely monitored in all RCVS cases.
References 1. Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012;11:906-917. 2. John S, Singhal AB, Calabrese L, et al. Long-term outcomes after reversible cerebral vasoconstriction syndrome. Cephalalgia 2016;36:387-394.
ARTICLE IN PRESS SEVERE RCVS WITH LARGE CEREBRAL INFARCTION 3. Fugate JE, Wijdicks EF, Parisi JE, et al. Fulminant postpartum cerebral vasoconstriction syndrome. Arch Neurol 2012;69:111-117. 4. John S, Donnelly M, Uchino K. Catastrophic reversible cerebral vasoconstriction syndrome associated with serotonin syndrome. Headache 2013;53:1482-1487. 5. Kunchok A, Castley HC, Aldous L, et al. Fatal reversible cerebral vasoconstriction syndrome. J Neurol Sci 2018;385: 146-150. 6. Stary JM, Wang BH, Moon SJ, et al. Dramatic intracerebral hemorrhagic presentations of reversible cerebral vasoconstriction syndrome: three cases and a literature review. Case Rep Neurol Med 2014;2014:782028. 7. Fugate JE, Ameriso SF, Ortiz G, et al. Variable presentations of postpartum angiopathy. Stroke 2012;43:670-676.
3 8. Kass-Hout T, Kass-Hout O, Sun CH, et al. A novel approach to diagnose reversible cerebral vasoconstriction syndrome: a case series. J Stroke Cerebrovasc Dis 2015;24:e31-e37. 9. Elstner M, Linn J, M€ uller-Schunk S, et al. Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. Cephalalgia 2009;29:677-682. 10. Bouchard M, Verreault S, Gariepy JL, et al. Intra-arterial milrinone for reversible cerebral vasoconstriction syndrome. Headache 2009;49:142-145. 11. Chen SP, Fuh JL, Lirng JF, et al. Recurrence of reversible cerebral vasoconstriction syndrome: a long-term followup study. Neurology 2015;84:1552-1558.
Severe Reversible Cerebral Vasoconstriction Syndrome with Large Posterior Cerebral Infarction Hiroki Yamada, MD, Ryogo Kikuchi, MD, PhD, Akiyoshi Nakamura, MD, PhD, and Hiromichi Miyazaki, MD, PhD
Reversible cerebral vasoconstriction syndrome is characterized by thunderclap headache and multifocal cerebral vasoconstriction. Cerebral vasoconstriction is reversible, and most cases have good prognosis. However, clinical outcome is possibly severe when it is complicated by stroke, yet detailed reports on such a case are few. We experienced a case of severe reversible cerebral vasoconstriction syndrome in a 32-year-old woman with medical history of preeclampsia 3 years prior. She presented with sudden sharp headache followed by altered mental status and vasoconstriction of the bilateral posterior cerebral arteries. She was treated with intravenous and oral calcium channel blockers, edaravone, and glycerol. However, the cerebral infarction in the posterior circulation subsequently remained, and her impaired consciousness did not recover. Furthermore, although imaging findings of vasoconstriction showed improvement a day after the occurrence of symptom, the same vessels showed poor visualization 7 weeks later, which indicated the recurrence of vasoconstriction, without additional symptom due to the fixed infarction. Although most cases of reversible cerebral vasoconstriction syndrome show good prognosis, neurologists must monitor the possibility of worse clinical course and permanent neurological deficit when associated with stroke, such as cerebral infarction. Strict management and treatment are needed in these cases. Key Words: Cerebral infarction—permanent neurological deficit—severe reversible cerebral vasoconstriction syndrome—stroke © 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved.
Case Report A 32-year-old woman, with medical history of preeclampsia 3 years prior, experienced sudden severe headache and subsequent coma. She had no significant family history or history of illicit drug use; she has allergy to ampicillin, but has not taken it recently. Upon arrival, her blood pressure, heart rate, oxygen saturation, and body temperature were 158/96 mmHg, 70 beats per minute, 100% (under From the Department of Neurosurgery, Hiratsuka City Hospital, Hiratsuka, Kanagawa 254-0065, Japan. Received May 21, 2018; revision received June 28, 2018; accepted June 30, 2018. Grant Support: None. Address correspondence to Hiroki Yamada, md, Department of Neurosurgery, Hiratsuka City Hospital, 1-19-1, Minamihara, Hiratsuka, Kanagawa 254-0065, Japan. E-mail: [email protected] 1052-3057/$ - see front matter © 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jstrokecerebrovasdis.2018.06.044
10 L of oxygen), and 36.1 °C, respectively. Head computed tomography, magnetic resonance imaging, and magnetic resonance angiography (MRA) showed restricted cortical subarachnoid hemorrhage (SAH); a wide, unclear, yet high-intensity area of the diffusion-weighted image of the posterior circulation; and vasoconstriction of the bilateral posterior cerebral arteries (PCAs), with no cerebral aneurysm detected (Fig 1A C, respectively). Computed tomography venography showed no abnormal findings. Blood test showed mild increase in white blood cells (10,500/mL), but no other abnormal findings including D-dimer, C-reactive protein, and autoantibodies; cerebrospinal fluid data were within normal limits. Reversible cerebral vasoconstriction syndrome (RCVS) was considered, and continuous intravenous nicardipine and oral verapamil, edaravone, and glycerol were administered. However, her impaired consciousness continued. On the following day, MRA showed improvement of the PCA vasoconstriction, which confirmed the RCVS diagnosis, but magnetic resonance imaging showed completion of a large infarction in
Journal of Stroke and Cerebrovascular Diseases, Vol. &&, No. && (&&), 2018: pp 1-3
1
ARTICLE IN PRESS H. YAMADA ET AL.
2
Figure 1. (A) Computed tomography during onset, showing bilateral parietal cortical subarachnoid hemorrhage. (B) Magnetic resonance image (MRI) diffusion-weighted image (DWI) during onset, showing an unclear yet high-intensity area at the bilateral occipital lobes and thalamus. (C) Magnetic resonance angiography (MRA) during onset, showing multiple vasoconstriction of the posterior cerebral artery (PCA) (white arrow heads). (D) MRI DWI performed on the following day, showing a clear high-intensity area. (E) MRA performed on the following day, showing improvement of vasoconstriction. (F) MRA performed 7 weeks after, showing poor visualization of the bilateral PCAs.
the bilateral posterior circulatory areas (Fig 1D, E) and bilateral thalamus infarction, which was considered as the cause of the impaired consciousness. The cortical SAH was unchanged. In contrast to the characteristic reversibility of RCVS, MRA performed at 7 weeks from admission showed poor visualization of the bilateral PCAs, and basilar artery (Fig 1F), but no new symptom appeared. She recovered slightly and could respond to a simple question, such as moving her fingertip, and was transferred to rehabilitation hospital 8 weeks from the onset.
Discussion RCVS can be severe when complicated by large cerebral infarction (CI). Most RCVS cases are benign,1,2 and detailed reports on severe cases remain few.3-5 Even when complicated with cerebral hemorrhage, good neurological recovery has been reported in some cases.6 Puerperal RCVS cases tend to be severe,7 but the present case was not postpartum. The patient was diagnosed to have RCVS from the beginning; thus, reversibility of vasoconstriction and symptom was expected. However, the large CI remained, and her impaired consciousness did not recover. Thus, neurologists must be aware that RCVS with CI can be serious, and strict monitoring and management of CI should be performed from the start. In this
case, managing with blood pressure or antithrombotic drugs during the initial stage could be considered, after confirming that the SAH was not increasing. In addition, intra-arterial calcium channel blockers or milrinone could be considered as a diagnostic and treatment option.8-10 We believe that the poorly visualized bilateral PCAs and basilar artery after the improvement of vasoconstriction can be attributed to the re-vasoconstriction. This contradicts the reversible characteristic of RCVS and is considered to be a recurrent type. In fact, RCVS recurrence is reported to occur in approximately 5% of all RCVS cases.11 Fortunately, this case did not have further harmful symptom, possibly due to the already fixed large CI. Therefore, in RCVS complicated by fixed CI, prognosis can be severe, and radiological findings in the cerebral vessels may show poor visualization after improvement of vasoconstriction. Stroke occurrence should be closely monitored in all RCVS cases.
References 1. Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012;11:906-917. 2. John S, Singhal AB, Calabrese L, et al. Long-term outcomes after reversible cerebral vasoconstriction syndrome. Cephalalgia 2016;36:387-394.
ARTICLE IN PRESS SEVERE RCVS WITH LARGE CEREBRAL INFARCTION 3. Fugate JE, Wijdicks EF, Parisi JE, et al. Fulminant postpartum cerebral vasoconstriction syndrome. Arch Neurol 2012;69:111-117. 4. John S, Donnelly M, Uchino K. Catastrophic reversible cerebral vasoconstriction syndrome associated with serotonin syndrome. Headache 2013;53:1482-1487. 5. Kunchok A, Castley HC, Aldous L, et al. Fatal reversible cerebral vasoconstriction syndrome. J Neurol Sci 2018;385: 146-150. 6. Stary JM, Wang BH, Moon SJ, et al. Dramatic intracerebral hemorrhagic presentations of reversible cerebral vasoconstriction syndrome: three cases and a literature review. Case Rep Neurol Med 2014;2014:782028. 7. Fugate JE, Ameriso SF, Ortiz G, et al. Variable presentations of postpartum angiopathy. Stroke 2012;43:670-676.
3 8. Kass-Hout T, Kass-Hout O, Sun CH, et al. A novel approach to diagnose reversible cerebral vasoconstriction syndrome: a case series. J Stroke Cerebrovasc Dis 2015;24:e31-e37. 9. Elstner M, Linn J, M€ uller-Schunk S, et al. Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. Cephalalgia 2009;29:677-682. 10. Bouchard M, Verreault S, Gariepy JL, et al. Intra-arterial milrinone for reversible cerebral vasoconstriction syndrome. Headache 2009;49:142-145. 11. Chen SP, Fuh JL, Lirng JF, et al. Recurrence of reversible cerebral vasoconstriction syndrome: a long-term followup study. Neurology 2015;84:1552-1558.