A Case of Cerebral Infarction Provoked by Carbon Monoxide Poisoning in Probable Unilateral Moyamoya Disease

CASE REPORT online © ML Comm J Neurocrit Care 2009;2:56-59 ISSN 2005-0348

Sang-Moo Lee, MD, PhD, Heui-Chul Choi, MD, PhD and Jong-Hee Sohn, MD Department of Neurology, Hallym University College of Medicine, Chuncheon Sacred Heart Hospital, Chuncheon, Korea

Background: Hemodynamic reserve is known to be reduced in moyamoya disease, which could account for increased susceptibility to cerebral ischemia. We report a case of acute cerebral infarction provoked by carbon monoxide (CO) poisoning in probable unilateral moyamoya disease. Case Report: A 26-year-old woman presented with mental changes followed by left hemiparesis after an exposure to CO. The initial serum carboxyhemoglobin level was 25.2%. She was alert but showed clinical signs of diffuse right hemispheric dysfunction. Brain MRI showed large territorial infarction in the right middle cerebral artery (MCA) and anterior cerebral artery (ACA). Brain MRA and conventional angiogram demonstrated complete occlusion of the proximal part of the right MCA and the ACA, which was suggestive of probable unilateral moyamoya disease. Conclusion: It was likely that cerebral hypoxia induced by CO poisoning produced acute cerebral infarction in a hemodynamically compromised state. J Neurocrit Care 2009;2:56-59

KEY WORDS: Moyamoya disease·Cerebral infarction·Carbon monoxide poisoning.

Introduction a shower with warm water in a temporary shower room. Alth- ough she became alert and oriented 3 hours later, she suffer- Moyamoya disease (MMD) is a progressive cerebrovascu- ed weakness on the left side limbs and thus visited our hospi- lar disease of unknown origin and characterized by progres- tal. But her friend recovered without neurologic symptoms. sive occlusion of the terminal portion of the internal carotid On initial physical examination, blood pressure was 140/ and proximal cerebral arteries with the development of typical 80 mmHg, body temperature 36.1℃ and pulse rate 103/min. extensive basal collateral network.1 Major clinical manifesta- There were no remarkable findings on physical examination. tions include repeated ischemic or hemorrhagic strokes and On neurologic examination, she was alert, but left side hemi- epileptic seizures. neglect was noted on higher cortical function test. On cranial It is known that hemodynamic reserve is reduced in MMD, nerves function test, forced conjugate deviation of both eyes to especially in ischemia related symptoms.2 This condition right side, left facial palsy of central type, hypoalgesia on the could account for increased potential for susceptibility to ce- left face and dysarthria were noted. Mild hemiparesis (Grade rebral ischemia-hypoxia. IV) and decreased senses of touch and pain were noted on the Recently, we experienced a case of acute cerebral infarction left side limbs. She had no known risk factors for stroke. Rou- in a patient with probable unilateral MMD that was provoked tine laboratory tests including all risk factors for young age by carbon monoxide (CO) poisoning. stroke were normal. But the initial serum carboxyhemoglobin (COHb) level was 25.2%. Brain CT revealed loss of the gray- Case white matter interfaces and diffuse low density in the right middle cerebral artery (MCA) and anterior cerebral artery (ACA) A 26-year-old woman was admitted with a symptom of men- territory, which was suggestive of acute cerebral infarction. tal changes followed by left side weakness. She and her friend Under the impression of carbon monoxide intoxication and ac- had suddenly experienced loss of consciousness while taking ute right MCA and ACA territorial infarction, pure oxygen was Address for correspondence: Sang-Moo Lee, MD, PhD supplied and intravenous rt-PA was started. Brain MRI (Fig. Department of Neurology, Hallym University College of Medicine, 1) showed findings consistent with the brain CT, and MRA Chuncheon Sacred Heart Hospital, 153 Gyo-dong, Chuncheon 200- showed occlusion of the right proximal MCA and the ACA. 704, Korea Tel: +82-33-240-5746, Fax: +82-33-255-1338 Conventional cerebral 4-vessel angiographic study demon- E-mail: [email protected] strated complete occlusion of the supraclinoid portion of the

A B

FIGURE 1. T2-weighted (A), T1-weighted (B), diffusion-weighted (C), and apparent diffusion coefficient (D) axial MRI show acute cerebral infarction mainly involving diffuse cerebral cortex in right middle cerebral arterial and anterior cerebral arterial territory. C D right ICA with extensive collateral vessels, which was sugges- thin minutes of CO exposure. Blood flow remains elevated tive of probable unilateral moyamoya disease (Fig. 2). Oral an- until loss of consciousness, when transient cardiac compro- tiplatelet treatment was continued for 2 weeks after thromboly- mise causes blood pressure to decrease.6 Also hypoxia is only sis, and then she discharged with minimal higher cortical dy- seen in animals when COHb levels are very high (>70%) and sfunction. the cardiovascular and cerebrovascular homeostatic response is overwhelmed. These findings suggest that brain hypoxia is 3 Discussion probably not an initial feature of CO poisoning. There exists ample evidence that cerebral hemodynamics Carbon monoxide is a colorless, non-irritant, odorless, ubi- are disturbed locally in MMD.7,8 These hemodynamic distur- quitous environmental gas, and CO intoxication is a serious bances include reduced cerebral blood flow, reduced cerebral phenomenon that can potentially lead to acute hypoxia and de- perfusion pressure and prolonged circulation time.9,10 And hy- ath. CO is produced in fires, by generators, car exhaust systems, percapnic reactivity, defined as the percent change in cerebral heating system obstruction and endogenously as a neurotrans- blood flow per mmHg change in pCO2, and cerebral hemo- mitter.3 Clinical symptoms of CO intoxication are headache, na- dynamic reserve capacity to acetazolamide is also known to usea, vomiting, tachypnea, lethargy, fatigue, syncope, seizure, be reduced in MMD.2,11 Moreover the distribution of cerebral confusion, unconsciousness, and death. Formation of COHb ca- blood flow in MMD shows a dominant posterior distribution uses a decrease in oxyhemoglobin saturation leading to cellu- in contrast to dominant anterior distribution in normal control lar hypoxia and death, giving rise to many of the symptoms of subjects.1,9 These hemodynamic changes could account for in- CO poisoning.4 This patient seemed to be exposed to CO pro- creased potential for susceptibility to cerebral ischemia and duced by incomplete combustion of gas for water heating. more frequent damage in anterior circulation than posterior Acute brain injury by CO toxicity may largely arise from circulation. Likewise, acute tissue hypoxia induced by COHb hypoxia secondary to hypoxemia.4,5 Studies with mice, how- related hypoxemia could provoke acute cerebral infarction of ever, have shown that initial cerebral blood flow increases wi- anterior cerebral circulation in this patient. Because her friend

57 J Neurocrit Care ▌2009 ;2:56-59

A B

FIGURE 2. Right carotid artery anteroposterior (A) and lateral (B) angiograms show occlusion at supraclinoid portion with basal and lep- tomeningeal collateral vessels. Left carotid artery anteroposterior (C) and lateral (D) angiograms disclose C D normal finding. had normal cerebral hemodynamics, she could recover with- cephalopathy mainly involving the gray matter.16 out neurologic deficit. The involved region of infarction in this patient was distrib- Many ischemic symptoms of the MMD are usually provok- uted mainly in cerebral cortex. This pattern of involvement has ed by hyperventilation in relation to crying, exercise, eating been reported very rarely in acute CO poisoning.15 In acute hot food and in hot weather.12,13 Hyperventilation induced CO intoxication, there is a predilection for the temporal lobe hypocapnia leading to vasoconstriction of small cerebral ves- and hippocampus, which is different from this patient.15 Cere- sels is a possible explanation.13 But in spite of careful history bral infarction mainly involving cerebral cortex suggests that taking, she had suffered no previous ischemia-related symp- underlying mechanism is hypoxia-ischemia resulting from tom before this event. And common infarction patterns in poor hemodynamic reserve in this probable unilateral MMD MMD are large hemispheric infarction predominantly in wa- patient. tershed zone, and subcortical infarction in the centrum semi- 14 ovale and in the distal beds of the MCA and ACA. Consid- Conclusion ering predominant cortical infarction pattern and past medical history, cerebral infarction was likely to be caused by hypoxia This case is an acute cerebral infarction provoked by CO provoked by CO poisoning. poisoning in a patient with probable unilateral MMD. We sh- The most common site of involvement in CO poisoning is ould consider that any conditions that produce acute hypox- the globus pallidus. And the caudate nucleus, putamen, and emia-hypoxia could aggravate ischemic symptoms of MMD. thalamus are occasionally involved.15 Central nervous system Among these conditions, CO intoxication may be included. neurons are vulnerable to hypoxic-ischemic insult because they have the highest oxygen and glucose demands. Acute and in- REFERENCES tense CO poisoning can lead to diffuse hypoxic-ischemic en- 1. Yonekawa Y, Kahn N. Moyamoya disease. Adv Neurol 2003;92:113-8.

58 Cerebral Infarction by Carbon Monoxide in Moyamoya ▌SM Lee, et al.

2. Tatemichi TK, Prohovnik I, Mohr JP, Correll JW, Quest DO, Jarvis L. 10. Taki W, Yonekawa Y, Kobayashi A, Ishikawa M, Kikuchi H, Nishiza- Reduced hypercapnic vasoreactivity in moyamoya disease. Neurology wa S, et al. Cerebral circulation and metabolism in adults’ moyamoya 1998;38:1575-81. disease--PET study. Acta Neurochir (Wien) 1989;100:150-4. 3. Gorman D, Drewry A, Huang YL, Sames C. The clinical toxicity of car- 11. Joo SP, Kim TS, Lee JH, Lee JK, Kim JH, Kim SH, et al. Moyamoya bon monoxide. Toxicology 2003;187:25-38. disease associated with Behcet’s disease. J Clin Neurosci 2006;13: 4. Berent AC, Todd J, Sergeeff J, Powell LL. Carbon monoxide toxicity: 364-7. a case series. J Vet Emerg Crit Care 2005;15:128-35. 12. Kim HY, Chung CS, Lee J, Han DH, Lee KH. Hyperventilation-indu- 5. Piantadosi CA. Perspective: carbon monoxide poisoning. N Engl J Med ced limb shaking TIA in Moyamoya diaseae. Neurology 2003;60:137-9. 2002;347:1054-5. 13. Spengos K, Tsivgoulis G, Toulas P, Vemmos K, Vassilopoulos D, Spen- 6. Thom SR. Carbon monoxide-mediated brain lipid peroxidation in the gos M. Hyperventilation-enhanced chorea as a transient ischaemic rat. J Appl Physiol 1990;68:997-1003. phenomenon in a patient with moyamoya disease. Eur Neurol 2004; 7. Nariai T, Senda M, Ishii K, Wakabayashi S, Yokota T, Toyama H, et 51:172-5. al. Posthyperventilatory steal response in chronic cerebral hemodyna- 14. Bruno A, Yuh WT, Biller J, Adams HP Jr, Cornell SH. Magnetic reso- mic stress: a positron emission tomography study. Stroke 1998;29: nance imaging in young adult with cerebral infarction due to moyam- 1281-92. oya. Arch Neurol 1988;45:303-6. 8. Kuwabara Y, Ichiya Y, Sasaki M, Yoshida T, Masuda K, Ikezaki K, et 15. O’Donnel P, Buxton PJ, Pitkin A, Jarvis LJ. The magnetic resonance al. Cerebral hemodynamics and metabolism in moyamoya disease-a imaging appearances of the brain in acute carbon monoxide poison- positron emission tomography. Clin Neurol Neurosurg 1997;99 suppl ing. Clin Radiol 2000;55:273-80. 2:S74-8. 16. Lo CP, Chen SY, Lee KW, Chen WL, Chen CY, Hsueh CJ, et al. Brain 9. Okawa A, Yoshimoto T, Suzuki J, Sakurai Y. Cerebral blood flow in injury after acute carbon monoxide poisoning: early and late compli- moyamoya disease. Part 1: Correlation with age and regional distri- cations. AJR Am J Roentgenol 2007;189:W205-11. bution. Acta Neurochir (Wien) 1990;105:30-4.