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Moyamoya disease: the clue from computed tomography
The Journal of Emergency Medicine, Vol. 26, No. 3, pp. 339⫺342, 2004 Copyright © 2004 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/04 $–see front matter
doi:10.1016/j.jemermed.2003.09.010
Visual Diagnosis in Emergency Medicine
MOYAMOYA DISEASE: THE CLUE FROM COMPUTED TOMOGRAPHY Chiung-Yuan Hsu,
MD,
Zui-Shen Yen, MD, MPh, Wen-Jone Chen, and Shry-Chyr Chen, MD
MD, PhD,
Wen-Chu Chiang,
MD,
Department of Emergency Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan Reprint Address: Chiung-Yuan Hsu, MD, Department of Emergency Medicine, National Taiwan University Hospital, No.7, Chung-Shan South. Rd, Taipei, Taiwan 100, ROC
diagnosed and serial work-up for pan-hypopituitarism after admission disclosed a small pituitary gland on a magnetic resonance imaging (MRI) study that may have resulted from repeated vascular events. Unfortunately, the patient experienced ischemic stroke and intracranial hemorrhage during the admission. Despite a long-term rehabilitation training program, he has a persistent unsatisfactory neurological status. Moyamoya disease is characterized as bilateral internal carotid artery stenosis and it involves the middle and posterior cerebral arteries. Because of its rarity, most Emergency Physicians have little or no experience with the disease. It is an important diagnosis to consider in young patients with stroke, especially when they are from East Asia. Delayed diagnosis may miss a chance for appropriate and effective vascular bypass surgery and may result in repeat strokes. The optimal imaging study to diagnose moyamoya disease is angiography, and even with great improvement in magnetic resonance angiography, we consider head computed tomography with contrast medium enhancement to remain useful and to suggest the correct diagnosis (1– 4).
A 48-year-old man with a past history of pituitary insufficiency was brought to the Emergency Department (ED) for a change in the level of consciousness, headache, and nausea for 1 day. He was diagnosed with encephalitis from a normal non-contrast enhanced head computed tomography (CT) scan, and a mildly increased intracranial pressure with pleocytosis in the cerebrospinal fluid (CSF) by laboratory studies. (CSF: open pressure 280 mm H2O, WBC 5 ⫻ 11/9/uL, total protein: 50 mg/dL, sugar: 50mg/dL, serum: 70 mg/dL). The patient’s consciousness improved soon after supportive treatment, and he was discharged 5 days later from the medical ward but the virologic and serologic tests for viral infection were all negative. One week later, the patient returned to the ED for near syncope, headache, vomiting, and neck stiffness. Head CT scan this time revealed unusual bilateral intraventricular hemorrhage (Figure 1a and b). The radiologist also found many bright, curved linear densities over the brain base (Figure 2a and b). Typical bilateral internal carotid artery stenosis over M1 segments with much collateral vascular formation was clearly demonstrated on the carotid arteriogram (Figure 3). Moyamoya disease was
Visual Diagnosis in Emergency Medicine is coordinated by Stephen R. Hayden, MD, of the University of California San Diego Medical Center, San Diego, California
RECEIVED: 31 January 2003; FINAL ACCEPTED: 16 September 2003
SUBMISSION RECEIVED:
7 August 2003; 339
340
C.-Y. Hsu et al.
Figure 1. Head CT scan showing bilateral intraventricular hemorrhage before contrast medium injection. (a) Brain base; (b) Supratentorium.
Moyamoya Disease Diagnosed by CT
341
Figure 2. Head CT scan after contrast medium injection demonstrates many bright curved liner densities over the brain base and occipital lobe. (a) Brain base; (b) Supratentorium.
342
C.-Y. Hsu et al.
Figure 3. Angiogram revealing bilateral internal carotid artery stenosis in M1 segments (Arrow: M1 of right internal carotid artery) with the smoky appearance of collateral vascularization.
REFERENCES 1. Ueki K, Meyer FB, Mellinger JF. Moyamoya disease: the disorder and surgical treatment. Mayo Clin Proc 1994;69:749 –57. 2. Hung CC, Tu YK, Su CF, Lin LS, Shih CJ. Epidemiological study of moyamoya disease in Taiwan. Clin Neurol Neurosurg 1997; 99(Suppl 2):S23–5.
3. Hasuo K, Mihara F, Matsushima T. MRI and MR angiography in moyamoya disease. J Magn Reson Imaging 1998;8:762– 6. 4. Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘moyamoya’ disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 1997;99(Suppl 2):S238 – 40.
doi:10.1016/j.jemermed.2003.09.010
Visual Diagnosis in Emergency Medicine
MOYAMOYA DISEASE: THE CLUE FROM COMPUTED TOMOGRAPHY Chiung-Yuan Hsu,
MD,
Zui-Shen Yen, MD, MPh, Wen-Jone Chen, and Shry-Chyr Chen, MD
MD, PhD,
Wen-Chu Chiang,
MD,
Department of Emergency Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan Reprint Address: Chiung-Yuan Hsu, MD, Department of Emergency Medicine, National Taiwan University Hospital, No.7, Chung-Shan South. Rd, Taipei, Taiwan 100, ROC
diagnosed and serial work-up for pan-hypopituitarism after admission disclosed a small pituitary gland on a magnetic resonance imaging (MRI) study that may have resulted from repeated vascular events. Unfortunately, the patient experienced ischemic stroke and intracranial hemorrhage during the admission. Despite a long-term rehabilitation training program, he has a persistent unsatisfactory neurological status. Moyamoya disease is characterized as bilateral internal carotid artery stenosis and it involves the middle and posterior cerebral arteries. Because of its rarity, most Emergency Physicians have little or no experience with the disease. It is an important diagnosis to consider in young patients with stroke, especially when they are from East Asia. Delayed diagnosis may miss a chance for appropriate and effective vascular bypass surgery and may result in repeat strokes. The optimal imaging study to diagnose moyamoya disease is angiography, and even with great improvement in magnetic resonance angiography, we consider head computed tomography with contrast medium enhancement to remain useful and to suggest the correct diagnosis (1– 4).
A 48-year-old man with a past history of pituitary insufficiency was brought to the Emergency Department (ED) for a change in the level of consciousness, headache, and nausea for 1 day. He was diagnosed with encephalitis from a normal non-contrast enhanced head computed tomography (CT) scan, and a mildly increased intracranial pressure with pleocytosis in the cerebrospinal fluid (CSF) by laboratory studies. (CSF: open pressure 280 mm H2O, WBC 5 ⫻ 11/9/uL, total protein: 50 mg/dL, sugar: 50mg/dL, serum: 70 mg/dL). The patient’s consciousness improved soon after supportive treatment, and he was discharged 5 days later from the medical ward but the virologic and serologic tests for viral infection were all negative. One week later, the patient returned to the ED for near syncope, headache, vomiting, and neck stiffness. Head CT scan this time revealed unusual bilateral intraventricular hemorrhage (Figure 1a and b). The radiologist also found many bright, curved linear densities over the brain base (Figure 2a and b). Typical bilateral internal carotid artery stenosis over M1 segments with much collateral vascular formation was clearly demonstrated on the carotid arteriogram (Figure 3). Moyamoya disease was
Visual Diagnosis in Emergency Medicine is coordinated by Stephen R. Hayden, MD, of the University of California San Diego Medical Center, San Diego, California
RECEIVED: 31 January 2003; FINAL ACCEPTED: 16 September 2003
SUBMISSION RECEIVED:
7 August 2003; 339
340
C.-Y. Hsu et al.
Figure 1. Head CT scan showing bilateral intraventricular hemorrhage before contrast medium injection. (a) Brain base; (b) Supratentorium.
Moyamoya Disease Diagnosed by CT
341
Figure 2. Head CT scan after contrast medium injection demonstrates many bright curved liner densities over the brain base and occipital lobe. (a) Brain base; (b) Supratentorium.
342
C.-Y. Hsu et al.
Figure 3. Angiogram revealing bilateral internal carotid artery stenosis in M1 segments (Arrow: M1 of right internal carotid artery) with the smoky appearance of collateral vascularization.
REFERENCES 1. Ueki K, Meyer FB, Mellinger JF. Moyamoya disease: the disorder and surgical treatment. Mayo Clin Proc 1994;69:749 –57. 2. Hung CC, Tu YK, Su CF, Lin LS, Shih CJ. Epidemiological study of moyamoya disease in Taiwan. Clin Neurol Neurosurg 1997; 99(Suppl 2):S23–5.
3. Hasuo K, Mihara F, Matsushima T. MRI and MR angiography in moyamoya disease. J Magn Reson Imaging 1998;8:762– 6. 4. Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘moyamoya’ disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 1997;99(Suppl 2):S238 – 40.