Consecutive Pregnancy with Chorea Gravidarum Associated with Moyamoya Disease
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Journal of Perinatology (2009) 29, 317–319 r 2009 Nature Publishing Group All rights reserved. 0743-8346/09 $32 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION Consecutive pregnancy with chorea gravidarum associated with moyamoya disease A Kim1, CH Choi2, CH Han3 and JC Shin3,4 1Fertility Center of CHA General Hospital, Department of Obstetrics and Gynecology, College of Medicine, Pochon CHA University, Seoul, Korea; 2Department of Internal Medicine, College of Medicine, Chung-Ang University, Seoul, Korea and 3Department of Obstetrics and Gynecology, College of Medicine, Catholic University of Korea, Seoul, Korea neoangiogenesis.3 The disease mainly affects young women Chorea gravidarum is uncommon movement disorder of pregnancy, originated from Japan, China and Korea.4 We report the case of a characterized by involuntary, abrupt, non-rhythmic movements. It can be patient with chorea gravidarum during two consecutive idiopathic or secondary to the underlying pathology. A 28-year-old, pregnancies in which moyamoya disease acts as the possible primigravida woman who was 8 weeks and 6 days of gestation presented etiology. with a history of involuntary choreiform movements in the left side limbs and facial twitch for 2 weeks. The symptoms started just after onset of severe emesis gravidarum. There was no meaningful medical history or family Case history, and she was taking no regular medication. Magnetic resonance A 28-year-old, primigravida woman with 8 weeks and 6 days of imaging of the brain revealed moyamoya disease. The symptoms, as well as gestation was referred due to severe hyperemesis gravidarum with the hyperemesis gravidarum, improved with gestational age; however, they weight reduction of 4 kg during past 3 weeks. She was right-handed were sustained up to 30 weeks of gestation. She delivered 2.61 kg, healthy and complained of involuntary movements of the left arm, hand male neonate at 40 weeks’ pregnancy by vaginal delivery under epidural and left foot and twitch on left face for 2 weeks. These symptoms analgesia. After 3 years later, she got pregnant again, and delivered at 41 insidiously initiated after onset of severe emesis gravidarum. weeks of pregnancy without complication. She experienced similar but There was no significant past medical history or family history, and milder symptoms to that of the first pregnancy until the late second she was not taking any regular medication. On examination, trimester. After the delivery, full evaluation was lost due to her refusal. In there were choreiform movements of the left upper limb and left this report, we present the case of a patient with chorea gravidarum in foot and involuntary twitch on the face was aggravated just after which moyamoya disease acts as an etiologic factor and a review of severe vomiting. Her speech was normal and there was no other literature with management proposal. neurological abnormality. Under the impression of chorea Journal of Perinatology (2009) 29, 317–319; doi:10.1038/jp.2008.183 gravidarum, variable tests were carried out. Full blood counts, Keywords: chorea gravidarum; moyamoya disease; pregnancy; delivery blood chemistry, liver function tests, thyroid function tests, laboratory tests for antiphospholipid syndrome and rheumatic fever, and electrocardiography were normal. Obstetric ultrasound Introduction showed a viable fetus with appropriate for gestational age. Magnetic resonance imaging of the brain revealed moyamoya disease Chorea gravidarum is the rare disease during pregnancy of which 1 involving bilateral middle cerebral arteries, distal inferior cerebral the incidence has been reported from 1/3,500 to 1/140,000. The arteries and anterior cerebral arteries with narrowing of bilateral clinical features involve extrapyramidal symptoms such as carotid arteries (Figure 1). Small multiple cerebral infarcts were involuntary movements and slurred speech, suggesting that the 2 suspected. She was diagnosed asymptomatic incidental moyamoya chorea of the patient is related to some other cause. Moyamoya disease and the neurologists recommended cesarean section and disease is a vascular disorder with progressive narrowing and further work-up after that. Choreiform movements sustained for 4 occlusion of basal cranial vessels and secondary specific weeks after the diagnosis and then, improved spontaneously and Correspondence: Dr JC Shin, Department of Obstetrics and Gynecology, College of Medicine, gradually without special treatment. Emetic symptom was Catholic University of Korea, Seoul, Korea. controlled by antiemetics and subsided slowly. After the E-mail: [email protected] disappearance of emesis, choreiform movements occasionally 4Current address: Department of Obstetrics and Gynecology, Kangnam St. Mary’s Hospital, Banpodong 505, Seochogu, Seoul, 137-040, Korea occurred until approximately 30 weeks of gestation. She refused Received 22 July 2008; revised 8 September 2008; accepted 21 September 2008 cesarean section and gave a birth to a female neonate by Chorea gravidarum with moyamoya disease A Kim et al 318 Figure 1 T2-weighted magnetic resonance images of brain: (a, b) Signal void of bilateral middle cerebral arteries and anterior cerebral arteries is not definite with replaced multiple collateral vessels. Scanty signal void of vessels is noted at the bilateral sylvian fissure. Bilateral carotid arteries were also narrowed. (c) Supraclinoid segments of the bilateral inferior cerebral arteries are also narrowed. spontaneous vaginal delivery with epidural analgesia at 40 weeks of We closely observed her and she had the successful vaginal delivery pregnancy. A baby was healthy but small for gestational age of a healthy male baby (3.2 kg) at 41 weeks of gestation without (2.61 kg, <5 percentile). During labor, no neurologic symptom any neurologic symptoms. Since this second pregnancy, she has emerged. After the delivery, the patient refused all evaluation not felt discomfort due to moyamoya disease. because of her financial situation, and was lost to follow-up. After 3 years, she presented at 7 weeks of gestation with hyperemesis gravidarum and the same choreiform symptoms in Discussion her left limbs but less severe than the previous pregnancy. These Chorea gravidarum with involuntary, abrupt and non-rhythmic choreiform movements lasted until the late second trimester. movements is an uncommon disorder of pregnancy and usually Journal of Perinatology Chorea gravidarum with moyamoya disease A Kim et al 319 presents in the first trimester of pregnancy.1,5,6 It is idiopathic in epidural or spinal anesthesia is possible when the birth canal is soft approximately half of the cases. In idiopathic chorea gravidarum, and delivery proceeds rapidly even in complicated cases.13 the suggested basic pathophysiology is enhanced dopaminergic Komiyama12 reported that in well-managed pregnant patients with sensitivity at the basal ganglion level due to estrogens.7 But it is moyamoya disease, delivery can be performed safely either by important to consider underlying pathology such as rheumatic cesarean section or vaginal delivery, and any anesthetic can be fever, antiphospholipid syndrome, other autoimmune disease, used whenever hypocapnia, hypotension and hypertension are Wilson’s disease, thyrotoxicosis, Huntington’s disease, subcortical avoidable. It seems unnecessary to insist patients without cerebral infarction, thrombophilic disease or drug-induced choreas.1,6,7 The hemorrhage or severe ischemia to undergo cesarean delivery. most common underlying factor of chorea gravidarum is Chorea gravidarum combined with moyamoya disease is really antiphospholipid syndrome. In some instances, pregnancy may rare. There is a case report of chorea gravidarum-associated unmask the underlying pathologic problems resulting in various moyamoya disease that ended in abortion and dramatic symptom neurologic symptoms, and positive family history could be relief.14 In this case, the patient had asymptomatic moyamoya observed.8 Various evaluations involving blood tests, image, and disease, and was diagnosed due to chorea during pregnancy. neurological tests are advisable to pregnant women with chorea Because choreiform movements started with emesis gravidarum due to diverse underlying disease. and were aggravated just after severe vomiting, it may have been Moyamoya disease can produce various neurologic symptoms. hyperventilation related to emesis in addition to basal ischemia Among other miscellaneous symptoms, involuntary movements of acting as a triggering factor for the chorea. Her symptoms chorea occur in 3–6% of patients with moyamoya disease.9 disappeared before pregnancy termination, and normal Ischemic dysfunction of the basal ganglia, cortex, subcortical white spontaneous vaginal delivery with epidural analgesia was possible matter, thalamus and subthalamic nucleus, has been suggested as without any neurologic symptoms in her consecutive pregnancy. the cause of chorea.10 Transient ischemic attacks are typically Though the prevalence is rare, moyamoya disease should be induced by hyperventilation, epileptic seizures and migraine-like considered as an etiologic factor of chorea gravidarum. It must headaches in moyamoya disease.9 Choreiform movements in also be emphasized that pregnancy and vaginal delivery are not pregnant moyamoya patients might be caused not only by contraindicated in moyamoya patients without cerebral enhanced dopaminergic sensitivity mediated by elevated female sex hemorrhage and severe ischemia. hormones during pregnancy, but also