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A case of Moyamoya disease with progressive involvement from unilateral to bilateral
Surg Neurol 1988;30:471-5
471
A Case of Moyamoya Disease With Progressive Involvement From Unilateral to Bilateral T o s h i o M a t s u s h i m a , M . D . , S a c h i k o T a k e , M . D . , K i y o t a k a Fujii, M . D . , M a s a s h i F u k u i , M . D . , Kanehiro Hasuo, M.D., Yasuo Kuwabara, M.D., and Katsutoshi Kitamura, M.D. Department of Neurosurgery, Neurological Institute, and the Department of Radiology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Matsushima T, Take S, Fujii K, Fukui M, Hasuo K, Kuwabara Y, Kitamura K. A case of Moyamoya disease with progressive involvement from unilateral to bilateral. Surg Neurol 1988;30:471-5.
A case of Moyamoya disease in a child starting with unilateral lesion and developing into bilateral involvement is reported. Angiographic findings at onset showed unilateral involvement, hence, it was filed as a "probable" case according to the diagnostic criteria of the Japanese Cooperative Research Committee. The carotid angiograms on the other side were totally normal. Three years later the occlusive lesion became bilateral, to meet the criteria as a "definite" case. Clinical manifestations, angiograms, electroencephalograms, and positron emission tomograms in this case are presented, and the relation between the probable and definite cases is discussed. KEY WORDS: Moyamoya disease; Unilateral moyamoya disease; Progression in moyamoya disease; Probable moyamoya disease; Akin moyamoya disease
Moyamoya disease is a pathologic condi.tion which, since first being reported by Takeuchi and Shimizu [20], is characterized by angiographic findings of stenosis or occlusion at the terminal portion of the internal carotid artery (ICA), together with a peculiar vascular network at the base of the brain on both sides. According to the diagnostic criteria of the Japanese Cooperative Research Committee on this disease, only the cases with bilateral lesion are diagnosed as "definite," and those with unilateral involvement should be classified as "probable," even if the angiographic findings on the affected side are typical and the cause is unknown [3,8]. It is because the unilateral arterial occlusive lesion may Address reprint requests to: Toshio Matsushima, M.D., Department of Neurosurgery, Neurological Institute, Faculty of Medicine, Kyushu University 60, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812, Japan. Received May 23, 1988; accepted July 18, 1988.
© 1988 by ElsevierSciencePublishing Co., Inc.
include various other diseases of known cause. The lesion in this disease very likely starts with only a mild arterial stenosis and, as observed on angiograms, progresses along with the course of the disease [1,4, 7,9,16,17,21,22,25]. There are cases with the typical findings on one side and only minimal pathologic change on the other side. It can also be assumed that the lesion appears to be purely unilateral at first and becomes bilateral later on. Notwithstanding, such a case has only rarely been reported [9]. We recently encountered a child with Moyamoya disease whose follow-up angiograms revealed bilateral progression of the unilateral lesion at the initial stage. The relation between the unilateral and bilateral lesions is discussed in the aspect of disease entity.
Case R e p o r t An 8-year-old girl started to complain of a headache when she was 6 years old. One year later she experienced her first transient ischemic attack (TIA) of paresthesia in the right fingers. She had had a few more similar TIAs before the first admission 1 year and 7 months later. Neurologic examination on admission revealed no abnormalities, and computed tomography (CT) scans demonstrated no low-density lesions either. On electroencephalograms (EEG), hyperventilation of 3 minutes produced a bilateral and symmetrical slowing of theta waves. Left hemispheric slowing reappeared in 50 seconds in a fashion of rebuild-up phenomena (Figure 1). On the left internal carotid (IC) angiograms, there were abnormal findings consistent with Moyamoya disease, such as severe stenosis at the supraclinoid portion of the left internal carotid artery (ICA), occlusion of the proximal portion of the left middle cerebral artery (MCA), and a peculiar network, commonly called Moyamoya vessels, around the carotid fork (Figure 2 A,B). Transdural anastomoses were also found on the left external carotid (EC) angiograms. On the right side, however, abnormal findings such as arterial stenosis or Moyamoya 0090-3019/88/$3.50
472
Surg Neurol 1988;30:471-5
Matsushima et al
o.
T~--A2 "
~ R~tlng
~J~"V" End of 3~ifl h y p e r ~ t l ~ t ~
~ ]lhmc ~ t - h y p e ~ t l l a t l o n
lmdn pmJt-h~,W¥~tlletku~
Figure 1. Electroencephalograms. Rebuild-up phenomenon is seen on the left side after hyperventilation.
vessels were not present at all (Figure 3). The left posterior cerebral artery (PCA) was well developed. On positron emission tomography (PET), the regional cerebral blood flow (rCBF) did not decrease, but the regional
Figure 2. Left internal carotid angiograms, anteroposteriorview (A) and lateral view (B). There is a severestenosis of the supraclinoid portion of the left ICA, an occlusion of the proximal portion of the left MCA, and basal Moyamoya vessels at the carotid fork.
cerebral blood volume (rCBV) slightly increased in the left cerebral hemisphere (Figure 4). At this time the diagnosis as a probable case of Moyamoya disease was made. One month after admission encephalo-duro-arterio-synangiosis (EDAS) was performed on the left side using the posterior branch of the superficial temporal artery (STA). Postoperative angiography performed 6 months after the first operation revealed a new collateral formation through the left STA and middle meningeal artery (MMA) (Figure 5 A , B ) . Postoperatively the attacks almost completely subsided and she led her daily life without any major troubles. However, the patient had another attack of transient paresthesia in the left hand, the side opposite to the former disorder, 1 year and 10 months after surgery. After that she had repeated TIAs occurring on the left side, suggesting involvement of the right cerebral hemisphere. She was readmitted 2 years and 9 months after surgery. Left IC angiograms showed almost the same findings as the last ones did, and left EC angiograms demonstrated the collateral circulation being further developed to supply not only the MCA territory but also the anterior cerebral artery (ACA) territory. Right IC angiograms presented new advancing findings such as severe stenosis at the supraclinoid portion of the right IC and fine Moyamoya vessels in the adjacent area (Figure 6 A , B ) . These findings were not present on the previous angiograms. So-called ethmoidal Moyamoya
B
Moyamoya Progressing From Unilateral to Bilateral
Surg Neurol 1988;30:471-5
473
for the diagnosis of "definite" case of this disease as follows: (1) stenosis or occlusion at the terminal portion of the ICA and at the proximal portions of the ACA and MCA; (2) abnormal vascular network seen in the arterial phase of angiograms in the vicinity of the arterial occlusion; and (3) bilateral involvement. Absence of any known cause is also required [3]. Even with similar angiographic findings, those with only unilateral involvement should be classified as probable cases. This is because various other diseases of known cause may present unilateral carotid occlusion, and the differential diagnosis is often too difficult. The term "unilateral Moyamoya disease" has been used practically [2,5,6,9,11,12,23,24]. Nevertheless, in most of the cases it was easily used when angiography only presented stenosis or occlusion of the major intracranial artery in association with abnormal fine vessels on one side. True unilateral Moyamoya disease, if present, should be a unilateral involvement of the same pathologic process as in the definite case. Does it truly exist? As mentioned by Nishimoto [12], Watanabe and Suzuki [24], and Kitamura and Matsushima [6], cases reported as unilateral Moyamoya disease are of two types, i.e., one presenting with typical pictures only uniFigure 3. Right internal carotid angiograms, anteroposterior view. Neither stenosis nor abnormal vessel is seen.
vessels were also demonstrated in the frontobasal region through the enlarged ophthalmic artery. The right ACA and MCA groups were only poorly demonstrated. Vertebral angiography was not performed because of a TIA during the procedure. The diagnosis was changed to definite case. PET findings were also compatible with the involvement of the right cerebral hemisphere. The rCBF decreased in the right hemisphere and in the left occipital region, and the rCBV increased bilaterally (Figure 4). Three years after the first surgery, EDAS and encephalo-myo-synangiosis (EMS) were performed in the right parietal region.
Figure 4. PET studies on the first admission (upper row) and for followup (lower row). In the first study, rCBF did ~ e but rCBV increas~ left cerebral hemisphere. Follow-up study revealed folio ~, 'ing advancing findings: slight decrease in rCBF and increase in rCBV in the right hemisphere.
Lt
Rt
l
CBF
Discussion Moyamoya disease was first recognized by its characteristic angiographic pictures [13,18,20]. Although its pathogenesis has not yet been clarified, it is widely accepted as a special type of cerebrovascular occlusive disease and is commonly called "Moyamoya disease" because of its puffy (moyamoya in Japanese) outlook of the abnormal vascular network [16]. As mentioned earlier, it is emphasized that the lesion in this disease should be bilateral [12,14,19]. The Japanese Cooperative Study Committee proposed angiographic criteria
CBV
B
Figure 5. Postoperative external carotid angiograms, anteroposterior view (A) and lateral view (B). A new collateral circulation was formed through the left STA and MMA. Figure 6. Follow-up right internal carotid angiograms, anteroposterior view (A) and lateral view (B). There are new advancing findings of Moyamoya disease.
A
B
M o y a m o y a P r o g r e s s i n g F r o m U n i l a t e r a l to Bilateral
laterally, and the other with findings typical on one side and atypical contralaterally [6,12,24]. Several authors reported cases of the former type [ 2 , 5 , 9 - 1 1 , 1 5 , 18,21,23,24]. Most of them were adult cases with onset of bleeding. Because some adult patients with arteriosclerotic occlusive disease may present with Moyamoya p h e n o m e n o n around the occlusion, it is more doubtful than in children whether all those reported cases are of the same pathologic conditions as in the definite Moyamoya cases. Suzuki et al. [18] and Mizukawa [9] reported pediatric cases of unilateral type. The follow-up angiographic changes were not reported in the former paper. Mizukawa [9] stated that he found only 1 pediatric case of unilateral type progressing to bilateral type among 56 cases in which follow-up angiography was performed. To our knowledge, this is the only reported case of progression from unilateral to bilateral type. However, there is no detailed description on this case in the report. Sato et al [15] reported a case with interesting angiographic findings: the proximal portion of the PCA was occluded on the same side of the ICA occlusion, and was associated with Moyamoya vessels. Some of the pediatric cases reported as unilateral Moyamoya disease would have had the same pathologic condition as in the definite Moyamoya cases developing bilaterally in the long run. Some others, however, may be cases of akin Moyamoya, which is not, after all, the definite Moyamoya disease. The present case proved a possibility that some of the definite cases may present with a strictly unilateral lesion at the early stage. Features such as age of the patient, onset, symptoms including repeated TIAs, and response to hyperventilation, angiographic findings, rebuild-up p h e n o m e n o n in EEG, PET findings, and abundant new collateral formation after EDAS, were all compatible with those in definite cases except for the signs, symptoms, and findings being unilateral. It is not known yet whether the "true" unilateral Moyamoya disease stays unilateral throughout. Angiograms in probable cases should be carefully followed up to establish the disease entity of this lesion. The authors are grateful to Dr. T. Mitani, Chief of Neurosurgery, Kokura-Kinen Hospital, Kitakyushu, for kindness in providing the patient's clinical data.
Surg N e u r o l 1988;30:471-5
4. 5.
6.
7.
8. 9.
10. 11.
12.
13.
14.
15.
16. 17.
18.
19. 20. 21.
22. References 1. Carlson CB, Harvey FH, Loop J. Progressive alternating hemiplegia in early childhood with basal arterial stenosis and telangiectasia (moyamoya syndrome). Neurology 1973;23:734-44. 2. Gomez CR, Hogan PA. Unilateral moyamoya disease in an adult--a case history. Angiology 1987;38:342-6. 3. Gotoh F. Guideline to the diagnosis of occlusion of the circle of Willis. In: Gotoh F, ed. Annual report of 1978 on the cooperative
23.
24. 25.
475
study of occlusion of the circle of Willis to the Ministry of Health and Welfare 1979:132. Handa J, Handa H. Progressive cerebral arterial occlusive disease: analysis of 27 cases. Neuroradiology 1972;3:119-33. Kasamo S, Asakura T, Yamamoto Y, Kobayashi E. Unilateral moyamoya disease associated with multiple aneurysms. A case report and review of the literature. Neurol Med Chir (Tokyo) 1984;24:30-4. Kitamura K, Matsushima T. Study on unilateral occlusion of the circle of Willis. In: H. Handa, ed. Annual report of 1985 on the cooperative study of occlusion of the circle of Willis to the Ministry of Health and Welfare 1986:12-8. Kodama N, Seki H. Angiograms of moyamoya disease. In: Suzuki J, ed. Moyamoya disease. Tokyo: Igaku-Shoin Ltd., 1983:13-46. Kudo T. Occlusion of the circle of Willis--akin cases. Clin Neurol 1966;6:336-47. Mizukawa N. Studies of Japanese cases with cerebral basal rete mirabile (moyamoya disease)--clinical, angiographical and pathological investigations. Okayama-Igaku-Zasshi 1976;88:853-88. Murphy MJ. Progressive vascular changes in moyamoya syndrome. Stroke 1980;11:656-8. Nijdam JR, Luijten JAFM, Gijn J. Cerebral haemorrhage associated with unilateral moyamoya syndrome. Clin Neurol Neurosurg 1986;88:49-51. Nishimoto A. Angiographic criteria of the cerebrovascular Moyamoya disease. Advances in Neurological Sciences 1976;20:75867. Nishimoto A, Sugiu R, Mannami T. Hemangiomatous malformation of bilateral internal carotid artery at the base of brain. Brain & Nerve (Tokyo) 1965;17:750-6. Pecker J, Simon J, Guy G, HerryJF. Nishimoto's disease. Significance of its angiographic appearance. Neuroradiology 1973;5:223-30. Sato S, Wada K, Yasumura S, Fujiwara H, Sasaki Y, Takeda T, Nakamura J, Suematsu K. A case of unilateral abnormal vascular network presenting a unique angiographical finding. Jpn J Clin Radiol 1983;28:248-50. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Arch Neurol 1969;20:288-99. Suzuki J, Takaku A. The disease showing the abnormal vascular network at the base of brain, particularly found in Japan. II. A follow-up study. Brain & Nerve (Tokyo) 1966;18:897-908. Suzuki J, Takaku A, Asahi M, Kowada M. Diseases showing the "Fibrille like vessels at the base of brain." Brain & Nerve (Tokyo) 1965;17:767-76. Takeuchi K. Moyamoya phenomenon and moyamoya disease. Brain & Nerve (Tokyo) 1978;30:1183-91. Takeuchi K, Shimizu K. Hypoplasia of the bilateral internal carotid arteries. Brain & Nerve (Tokyo) 1957;9:37-43. Takeuchi S. Studies on Japanese cases with abnormal vascular network at the base of the brain in cerebral angiography--Part 1. Clinical investigation. Okayama-Igaku-Zasshi 1967;79:311-36. Taveras JM. Multiple progressive intracranial arterial occlusions: a syndrome of children and young adults. Am J Roentgenol Radium Ther Nucl Med 1969;106:235-68. Watanabe S, Atsumi H, Okada S, Nishio T. Evaluation of a case presenting an abnormal vascular network unilaterally at the site of carotis-gable. Brain & Nerve (Tokyo) 1966;18:533-7. Watanabe T, Suzuki N. Akin moyamoya. In: Suzuki J, ed. Moyamoya disease. Tokyo: Igaku-Shoin Ltd., 1983:123-46. Yamashita M, Tanaka K, Kishikawa T, Yokota K. Moyamoya disease associated with renovascular hypertension. Hum Pathol 1984;15:191-3.
471
A Case of Moyamoya Disease With Progressive Involvement From Unilateral to Bilateral T o s h i o M a t s u s h i m a , M . D . , S a c h i k o T a k e , M . D . , K i y o t a k a Fujii, M . D . , M a s a s h i F u k u i , M . D . , Kanehiro Hasuo, M.D., Yasuo Kuwabara, M.D., and Katsutoshi Kitamura, M.D. Department of Neurosurgery, Neurological Institute, and the Department of Radiology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Matsushima T, Take S, Fujii K, Fukui M, Hasuo K, Kuwabara Y, Kitamura K. A case of Moyamoya disease with progressive involvement from unilateral to bilateral. Surg Neurol 1988;30:471-5.
A case of Moyamoya disease in a child starting with unilateral lesion and developing into bilateral involvement is reported. Angiographic findings at onset showed unilateral involvement, hence, it was filed as a "probable" case according to the diagnostic criteria of the Japanese Cooperative Research Committee. The carotid angiograms on the other side were totally normal. Three years later the occlusive lesion became bilateral, to meet the criteria as a "definite" case. Clinical manifestations, angiograms, electroencephalograms, and positron emission tomograms in this case are presented, and the relation between the probable and definite cases is discussed. KEY WORDS: Moyamoya disease; Unilateral moyamoya disease; Progression in moyamoya disease; Probable moyamoya disease; Akin moyamoya disease
Moyamoya disease is a pathologic condi.tion which, since first being reported by Takeuchi and Shimizu [20], is characterized by angiographic findings of stenosis or occlusion at the terminal portion of the internal carotid artery (ICA), together with a peculiar vascular network at the base of the brain on both sides. According to the diagnostic criteria of the Japanese Cooperative Research Committee on this disease, only the cases with bilateral lesion are diagnosed as "definite," and those with unilateral involvement should be classified as "probable," even if the angiographic findings on the affected side are typical and the cause is unknown [3,8]. It is because the unilateral arterial occlusive lesion may Address reprint requests to: Toshio Matsushima, M.D., Department of Neurosurgery, Neurological Institute, Faculty of Medicine, Kyushu University 60, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812, Japan. Received May 23, 1988; accepted July 18, 1988.
© 1988 by ElsevierSciencePublishing Co., Inc.
include various other diseases of known cause. The lesion in this disease very likely starts with only a mild arterial stenosis and, as observed on angiograms, progresses along with the course of the disease [1,4, 7,9,16,17,21,22,25]. There are cases with the typical findings on one side and only minimal pathologic change on the other side. It can also be assumed that the lesion appears to be purely unilateral at first and becomes bilateral later on. Notwithstanding, such a case has only rarely been reported [9]. We recently encountered a child with Moyamoya disease whose follow-up angiograms revealed bilateral progression of the unilateral lesion at the initial stage. The relation between the unilateral and bilateral lesions is discussed in the aspect of disease entity.
Case R e p o r t An 8-year-old girl started to complain of a headache when she was 6 years old. One year later she experienced her first transient ischemic attack (TIA) of paresthesia in the right fingers. She had had a few more similar TIAs before the first admission 1 year and 7 months later. Neurologic examination on admission revealed no abnormalities, and computed tomography (CT) scans demonstrated no low-density lesions either. On electroencephalograms (EEG), hyperventilation of 3 minutes produced a bilateral and symmetrical slowing of theta waves. Left hemispheric slowing reappeared in 50 seconds in a fashion of rebuild-up phenomena (Figure 1). On the left internal carotid (IC) angiograms, there were abnormal findings consistent with Moyamoya disease, such as severe stenosis at the supraclinoid portion of the left internal carotid artery (ICA), occlusion of the proximal portion of the left middle cerebral artery (MCA), and a peculiar network, commonly called Moyamoya vessels, around the carotid fork (Figure 2 A,B). Transdural anastomoses were also found on the left external carotid (EC) angiograms. On the right side, however, abnormal findings such as arterial stenosis or Moyamoya 0090-3019/88/$3.50
472
Surg Neurol 1988;30:471-5
Matsushima et al
o.
T~--A2 "
~ R~tlng
~J~"V" End of 3~ifl h y p e r ~ t l ~ t ~
~ ]lhmc ~ t - h y p e ~ t l l a t l o n
lmdn pmJt-h~,W¥~tlletku~
Figure 1. Electroencephalograms. Rebuild-up phenomenon is seen on the left side after hyperventilation.
vessels were not present at all (Figure 3). The left posterior cerebral artery (PCA) was well developed. On positron emission tomography (PET), the regional cerebral blood flow (rCBF) did not decrease, but the regional
Figure 2. Left internal carotid angiograms, anteroposteriorview (A) and lateral view (B). There is a severestenosis of the supraclinoid portion of the left ICA, an occlusion of the proximal portion of the left MCA, and basal Moyamoya vessels at the carotid fork.
cerebral blood volume (rCBV) slightly increased in the left cerebral hemisphere (Figure 4). At this time the diagnosis as a probable case of Moyamoya disease was made. One month after admission encephalo-duro-arterio-synangiosis (EDAS) was performed on the left side using the posterior branch of the superficial temporal artery (STA). Postoperative angiography performed 6 months after the first operation revealed a new collateral formation through the left STA and middle meningeal artery (MMA) (Figure 5 A , B ) . Postoperatively the attacks almost completely subsided and she led her daily life without any major troubles. However, the patient had another attack of transient paresthesia in the left hand, the side opposite to the former disorder, 1 year and 10 months after surgery. After that she had repeated TIAs occurring on the left side, suggesting involvement of the right cerebral hemisphere. She was readmitted 2 years and 9 months after surgery. Left IC angiograms showed almost the same findings as the last ones did, and left EC angiograms demonstrated the collateral circulation being further developed to supply not only the MCA territory but also the anterior cerebral artery (ACA) territory. Right IC angiograms presented new advancing findings such as severe stenosis at the supraclinoid portion of the right IC and fine Moyamoya vessels in the adjacent area (Figure 6 A , B ) . These findings were not present on the previous angiograms. So-called ethmoidal Moyamoya
B
Moyamoya Progressing From Unilateral to Bilateral
Surg Neurol 1988;30:471-5
473
for the diagnosis of "definite" case of this disease as follows: (1) stenosis or occlusion at the terminal portion of the ICA and at the proximal portions of the ACA and MCA; (2) abnormal vascular network seen in the arterial phase of angiograms in the vicinity of the arterial occlusion; and (3) bilateral involvement. Absence of any known cause is also required [3]. Even with similar angiographic findings, those with only unilateral involvement should be classified as probable cases. This is because various other diseases of known cause may present unilateral carotid occlusion, and the differential diagnosis is often too difficult. The term "unilateral Moyamoya disease" has been used practically [2,5,6,9,11,12,23,24]. Nevertheless, in most of the cases it was easily used when angiography only presented stenosis or occlusion of the major intracranial artery in association with abnormal fine vessels on one side. True unilateral Moyamoya disease, if present, should be a unilateral involvement of the same pathologic process as in the definite case. Does it truly exist? As mentioned by Nishimoto [12], Watanabe and Suzuki [24], and Kitamura and Matsushima [6], cases reported as unilateral Moyamoya disease are of two types, i.e., one presenting with typical pictures only uniFigure 3. Right internal carotid angiograms, anteroposterior view. Neither stenosis nor abnormal vessel is seen.
vessels were also demonstrated in the frontobasal region through the enlarged ophthalmic artery. The right ACA and MCA groups were only poorly demonstrated. Vertebral angiography was not performed because of a TIA during the procedure. The diagnosis was changed to definite case. PET findings were also compatible with the involvement of the right cerebral hemisphere. The rCBF decreased in the right hemisphere and in the left occipital region, and the rCBV increased bilaterally (Figure 4). Three years after the first surgery, EDAS and encephalo-myo-synangiosis (EMS) were performed in the right parietal region.
Figure 4. PET studies on the first admission (upper row) and for followup (lower row). In the first study, rCBF did ~ e but rCBV increas~ left cerebral hemisphere. Follow-up study revealed folio ~, 'ing advancing findings: slight decrease in rCBF and increase in rCBV in the right hemisphere.
Lt
Rt
l
CBF
Discussion Moyamoya disease was first recognized by its characteristic angiographic pictures [13,18,20]. Although its pathogenesis has not yet been clarified, it is widely accepted as a special type of cerebrovascular occlusive disease and is commonly called "Moyamoya disease" because of its puffy (moyamoya in Japanese) outlook of the abnormal vascular network [16]. As mentioned earlier, it is emphasized that the lesion in this disease should be bilateral [12,14,19]. The Japanese Cooperative Study Committee proposed angiographic criteria
CBV
B
Figure 5. Postoperative external carotid angiograms, anteroposterior view (A) and lateral view (B). A new collateral circulation was formed through the left STA and MMA. Figure 6. Follow-up right internal carotid angiograms, anteroposterior view (A) and lateral view (B). There are new advancing findings of Moyamoya disease.
A
B
M o y a m o y a P r o g r e s s i n g F r o m U n i l a t e r a l to Bilateral
laterally, and the other with findings typical on one side and atypical contralaterally [6,12,24]. Several authors reported cases of the former type [ 2 , 5 , 9 - 1 1 , 1 5 , 18,21,23,24]. Most of them were adult cases with onset of bleeding. Because some adult patients with arteriosclerotic occlusive disease may present with Moyamoya p h e n o m e n o n around the occlusion, it is more doubtful than in children whether all those reported cases are of the same pathologic conditions as in the definite Moyamoya cases. Suzuki et al. [18] and Mizukawa [9] reported pediatric cases of unilateral type. The follow-up angiographic changes were not reported in the former paper. Mizukawa [9] stated that he found only 1 pediatric case of unilateral type progressing to bilateral type among 56 cases in which follow-up angiography was performed. To our knowledge, this is the only reported case of progression from unilateral to bilateral type. However, there is no detailed description on this case in the report. Sato et al [15] reported a case with interesting angiographic findings: the proximal portion of the PCA was occluded on the same side of the ICA occlusion, and was associated with Moyamoya vessels. Some of the pediatric cases reported as unilateral Moyamoya disease would have had the same pathologic condition as in the definite Moyamoya cases developing bilaterally in the long run. Some others, however, may be cases of akin Moyamoya, which is not, after all, the definite Moyamoya disease. The present case proved a possibility that some of the definite cases may present with a strictly unilateral lesion at the early stage. Features such as age of the patient, onset, symptoms including repeated TIAs, and response to hyperventilation, angiographic findings, rebuild-up p h e n o m e n o n in EEG, PET findings, and abundant new collateral formation after EDAS, were all compatible with those in definite cases except for the signs, symptoms, and findings being unilateral. It is not known yet whether the "true" unilateral Moyamoya disease stays unilateral throughout. Angiograms in probable cases should be carefully followed up to establish the disease entity of this lesion. The authors are grateful to Dr. T. Mitani, Chief of Neurosurgery, Kokura-Kinen Hospital, Kitakyushu, for kindness in providing the patient's clinical data.
Surg N e u r o l 1988;30:471-5
4. 5.
6.
7.
8. 9.
10. 11.
12.
13.
14.
15.
16. 17.
18.
19. 20. 21.
22. References 1. Carlson CB, Harvey FH, Loop J. Progressive alternating hemiplegia in early childhood with basal arterial stenosis and telangiectasia (moyamoya syndrome). Neurology 1973;23:734-44. 2. Gomez CR, Hogan PA. Unilateral moyamoya disease in an adult--a case history. Angiology 1987;38:342-6. 3. Gotoh F. Guideline to the diagnosis of occlusion of the circle of Willis. In: Gotoh F, ed. Annual report of 1978 on the cooperative
23.
24. 25.
475
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