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Table of Contents
Year : 2020  |  Volume : 69  |  Issue : 3  |  Page : 182-184

Moyamoya disease in a 29-year-old female of Indian Origin

1 Department of Anatomy, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Date of Submission23-Aug-2020
Date of Acceptance22-May-2020
Date of Web Publication30-Sep-2020

Correspondence Address:
Dr. Shilpi Gupta Dixit
Department of Anatomy, All India Institute of Medical Sciences, Jodhpur - 342 005, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JASI.JASI_111_19

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The case presented here is a 29-year-old female patient with complaints of weakness over the left upper and lower limbs for 2 weeks. Digital subtraction angiography of the brain was suggestive of cerebral arteries, showing proliferation of cortical as well as collateral arteries arising from the internal carotid arteries (ICAs). Moyamoya is a progressive steno-occlusive disease at the terminal portions of the bilateral ICAs, with the development of “moyamoya vessels” giving a “Puff of Smoke” appearance on catheter angiography. Cerebral angiography is gold standard for diagnosis and helpful for detecting aneurysm and for surgical planning. There is no standardized surgical strategy for the treatment of this disease. Innumerable revascularization procedures have been employed with the aim of decelerating the progression of disease. Recognition of various symptoms of moyamoya would enable early diagnosis so that it could be treated surgically, as soon as possible, leading to a better neurological outcome.

Keywords: Digital subtraction angiography, moyamoya disease, moyamoya syndrome, neurosurgical interventions, revascularization, stroke

How to cite this article:
Sharma S, Dixit SG, Khera PS, Garg PK, Nayyar AK, Ghatak S. Moyamoya disease in a 29-year-old female of Indian Origin. J Anat Soc India 2020;69:182-4

How to cite this URL:
Sharma S, Dixit SG, Khera PS, Garg PK, Nayyar AK, Ghatak S. Moyamoya disease in a 29-year-old female of Indian Origin. J Anat Soc India [serial online] 2020 [cited 2023 Jan 27];69:182-4. Available from: https://www.jasi.org.in/text.asp?2020/69/3/182/296904

  Introduction Top

Moyamoya disease was first narrated in 1957 by Takeuchi and Shimizu in Japan. Cases have been reported worldwide, but this disease is most common in Japan.[1] Many cases have been reported in India too.[2] It is a rare cerebrovascular disease with an incidence of 0.086/100,000 population.[3] Exact etiology of this disease is not known. It is a progressive steno-occlusive disease. The site of its occurrence is at the terminal portions of the bilateral internal carotid arteries (ICAs). Collateral channels of circulation develop. These small, multiple vessels at the base of the brain are known as “moyamoya vessels.” This appearance was observed on catheter angiography and was given the name moyamoya, which in Japanese means “puff of smoke.”[4],[5] Cerebral revascularization surgery leads to favorable outcome of disease progression. Patients are likely to suffer stroke–ischemic or hemorrhagic.[6] Moyamoya disease generally occurs either in the pediatric age group between 5 and 10 years or in the fourth decade of life.[7] Generally, ischemic stroke is there in the pediatric group while hemorrhagic stroke in the adults.[8] Demographically, the ratio of females to males is 2:1.[9] Although moyamoya syndrome is linked with other medical conditions such as arteriosclerosis, Down syndrome, head trauma, autoimmune disease neurofibromatosis type 1, meningitis, or previous radiation therapy, it has the same angiographic appearance as moyamoya disease.[6]

  Case Report Top

This study was conducted at AIIMS, Jodhpur. Clearance of the institutional ethics committee was taken before commencement of the study. Institutional ethics committee stated “after through consideration accorded its approval on the above project.”

A 29-year-old female was admitted to our hospital with complaints of weakness of the left upper and lower limbs for 2 weeks. There was a history of occasional headache and sudden fall thrice in the past 5 months. These sudden falls indicate transient ischemic attacks. There was no history of fever, vomiting, convulsion, and ear discharge. There was no past history of head injury, central nervous system infections, and facial palsy. There was no history of consumption of drugs or noxious substances. She was a full-term vaginal delivery with no complications. There was no history of delayed mile stones. On neurological examination, gait was hemiplegic, and tone as well as power was decreased over the left side. Blood, urine, and cerebrospinal fluid investigations were normal. Family history was negative for this disease or any other central nervous system disorder. Hence, after magnetic resonance imaging (MRI) brain, digital subtraction angiography (DSA) of the brain was done. In MRI, many small round and twisted portions of the artery were observed at the base of the brain. DSA was suggestive of significant stenosis of the bilateral ICAs, with the presence of multiple collateral arteries at the base of the brain supplied by vertebral and external carotid arteries, forming “puff of smoke” appearance [Figure 1] and [Figure 2].
Figure 1: Digital subtraction angiography of the left common carotid artery lateral (a) and anteroposterior (b) view showing significant attenuation of the supraclinoid internal carotid artery (black arrows) and terminating as ophthalmic artery. Extensive collaterals seen arising from the branches of the external carotid artery (white arrows)

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Figure 2: Digital subtraction angiography of the right vertebral artery anteroposterior (a) and lateral (b) view showing extensive collaterals at base of the skull forming puff of smoke appearance (asterisk). Majority of anterior circulation flow is maintained by vertebral artery

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  Discussion Top

Moyamoya disease is a progressive occlusion of Circle of Willis arteries. This leads to the development of collateral arteries around blocked vessels. This helps in compensating for the blockage. However, the collateral arteries are small and weak. Hence, they are vulnerable to bleeding, thrombosis, and aneurysm. These collateral arteries appear as “puff of smoke” on angiography.[4] Histopathology of these vessels suggested of thickened intima due to smooth muscle proliferation, as well as tortuous duplicated internal elastic lamina. Abnormalities of the vascular growth factors and inflammatory mediators also have role in the etiology of moyamoya disease.[10]

Clinical features of moyamoya disease are different in children and adults. In adults, there is history of subarachnoid or intra-parenchymal hemorrhage. However, in children, there is a history of recurrent transient ischemic attacks or infarction of the cerebral artery. Classical symptoms of moyamoya disease are monoparesis, hemiparesis, dysarthria, aphasia, headache, convulsions, and involuntary movements. If diagnosis is done late, then there can be mental impairment too. Two factors on which prognosis of moyamoya disease patients depend are age and type of presentation of disease. Patients with a history of transient ischemic attacks or epilepsy have better prognosis compared to infarctions.[11]

DSA shows the arteries and collaterals supplying the brain. It is used to visualize the anatomy of the vessels and the presence of aneurysm and hence helps in the confirmation of diagnosis.[11] In the present case also, DSA showed immense collateral flow through the perforating vessels. Attenuation of the bilateral supraclinoid ICAs with multiple collaterals forming puff of smoke appearance was visualized [Figure 1] and [Figure 2].

Antiplatelet agents (including aspirin) and calcium channel blockers are given. Antiplatelet agents are given to prevent clots. Calcium channel blockers provide supportive management of moyamoya disease. Surgery is the best management of moyamoya disease.[12],[13] Moyamoya usually affects the ICA and adjacent anterior and middle cerebral arteries. Therefore, surgeons can use external carotid artery or superficial temporal artery to replace its circulation. The arteries are either sewn directly into brain circulation or placed on the surface of the brain to re-establish new circulation within a few weeks. Revascularization procedures are done at present to increase the perfusion of the hypoxic brain cells. This will ultimately prevent ischemic injury by increasing collateral blood flow.[14],[15] Revascularization procedures can be divided into three main groups: indirect (nonanastomotic) bypass techniques, direct (anastomotic) bypass techniques, and combined techniques. Results of direct techniques have been better than indirect and combined techniques.[16] Angiographic follow-ups have resulted in favorable consequence.

This case emphasizes the significance of considering moyamoya disease as one of the differential diagnoses in patients with a history of recurrent headaches, which cannot be relieved by routine medical treatment.

  Conclusion Top

Early diagnosis and management will lead to favorable results in moyamoya disease patients.

DSA helps a lot in diagnosis and henceforth in the management of this disease. This knowledge will be crucial in planning neurosurgical interventions and hence will influence prognosis of moyamoya disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Takeuchi K, Shimizu K. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve 1957;9:37-43.  Back to cited text no. 1
Menon G, Hegde A. Moyamoya disease. Arch Med Health Sci 2019;7:224-32.  Back to cited text no. 2
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Uchino K, Johnston SC, Becker KJ, Tirschwell DL. Moyamoya disease in Washington State and California. Neurology 2005;65:956-8.  Back to cited text no. 3
Suzuki J, Kodama N. Moyamoya disease-a review. Stroke 1983;14:104-9.  Back to cited text no. 4
Atlas SW. MR angiography: Techniques and Clinical Applications MRI of the Brain and Spine. 3rd ed. Philadelphia, PA, USA: Lippincott Williams and Wilkins; 2002.  Back to cited text no. 5
Lutterman J, Scott M, Nass R, Geva T. Moyamoya syndrome associated with congenital heart disease. Pediatrics 1998;101:57-60.  Back to cited text no. 6
Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008;79:900-4.  Back to cited text no. 7
Soriani S, Scarpa P, Voghenzi A, De Carlo L, Cilio R. Moyamoya disease in childhood: A familial case report. Childs Nerv Syst 1993;9:215-9.  Back to cited text no. 8
Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg 2004;100:142-9.  Back to cited text no. 9
Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoya disease. Neuropathology 2000;20 Suppl: S61-4.  Back to cited text no. 10
Joarder MA, Chandy M. Moyamoya disease in a child: A case report. Pulse 2013;6:62-5.  Back to cited text no. 11
Ibrahimi DM, Tamargo RJ, Ahn ES. Moyamoya disease in children. Childs Nerv Syst 2010;26:1297-308.  Back to cited text no. 12
Scott RM. Moyamoya syndrome: A surgically treatable cause of stroke in the pediatric patient. Clin Neurosurg 2000;47:378-84.  Back to cited text no. 13
Oya S, Tsutsumi K, Ueki K. Adult-onset moyamoya disease with repetitive ischemic attacks successfully treated by superficial temporal-middle cerebral artery bypass-case report. Neurol Med Chir (Tokyo) 2003;43:138-41.  Back to cited text no. 14
Golby AJ, Marks MP, Thompson RC, Steinberg GK. Direct and combined revascularization in pediatric moyamoya disease. Neurosurgery 1999;45:50-8.  Back to cited text no. 15
Fung LW, Thompson D, Ganesan V. Revascularisation surgery for paediatric moyamoya: A review of the literature. Childs Nerv Syst 2005;21:358-64.  Back to cited text no. 16


  [Figure 1], [Figure 2]


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