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Year : 2015  |  Volume : 4  |  Issue : 3  |  Page : 200-202

Moyamoya disease - A case report from North Western part of India

Department of General Medicine, Jawaharlal Nehru Medical College, Ajmer, Rajasthan, India

Date of Web Publication16-Jul-2015

Correspondence Address:
Maniram Kumhar
Jeevan Jadav, Gaurav Enclav, Civil Lines, Ajmer - 305 001, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2278-344X.160901

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Moyamoya disease is a chronic, progressive occlusion of the circle of Willis arteries that leads to the development of characteristic collateral vessels seen on imaging, particularly cerebral angiography. The disease may develop in children and adults, but the clinical features differ. Moyamoya disease occurs predominantly in Japanese individuals but has been found in all races with varying age distributions and clinical manifestations. As a result, moyamoya disease has been under-recognized as a cause of ischemic and hemorrhagic strokes in the Indian subcontinent. At this time, there is no known cure, and existing treatment options are controversial. We here describe the case of a 27-year-old Indian presenting as right-sided hemiparesis with accelerated hypertension.

Keywords: Hemiparesis, ivy sign, moyamoya

How to cite this article:
Sivaraman A, Kumhar M, Sahu UK, Mali MK. Moyamoya disease - A case report from North Western part of India. Int J Health Allied Sci 2015;4:200-2

How to cite this URL:
Sivaraman A, Kumhar M, Sahu UK, Mali MK. Moyamoya disease - A case report from North Western part of India. Int J Health Allied Sci [serial online] 2015 [cited 2023 Mar 28];4:200-2. Available from: https://www.ijhas.in/text.asp?2015/4/3/200/160901

  Introduction Top

Moyamoya disease was first described in Japan by Takeuchi and Shimizu in 1957. [1] Although the disease is most common in Japan, many subsequent cases have been reported elsewhere, including North America, Europe, and India. [2],[3],[4] Moyamoya disease deemed a progressive steno-occlusive disease at terminal portions of the bilateral internal carotid arteries with the development of "moyamoya vessels" as collateral channels of circulation. The appearance of these small, multiple vessels at the base of the brain on catheter angiography was originally described by the Japanese term moyamoya, which translates to "puff of smoke." [5] Although moyamoya syndrome has the same angiographic appearance as moyamoya disease, it is associated with other medical conditions such as arteriosclerosis, autoimmune disease, down syndrome, head trauma, meningitis, neurofibromatosis Type 1, and previous radiation therapy. Very few cases of moyamoya disease presenting in adulthood in non-Japanese individuals have been reported. [6]

  Case report Top

A 27-year-old young man presented to the hospital with generalized weakness involving right upper and lower limbs which was insidious in onset after the patient got up from the bed and was getting ready for the day-to-day activities. The patient was disoriented and weak after the episode. He did not have associated bowel or bladder incontinence.

On evaluation, the patient stated that he did not have similar episodes in the past. The patient denied any family history of stroke, seizure, or cancer. Socially, the patient was employed in a clerical job in his hometown. He did not smoke cigarettes, consume alcohol, or use illicit drugs.

On physical examination, the patient was conscious and oriented. His blood pressure was 200/106 mmHg in the left arm. On neurologic examination, the cranial nerves were normal. Power was decreased (2/5) and the tone was decreased, superficial reflexes were absent and deep tendon reflexes were exaggerated in both right upper and lower limbs. The left side neurological examination was normal, and there were no signs of sensory or cerebellar dysfunction. Results of a complete metabolic panel on admission remained within normal limits for the duration of the patient's hospital visit. The complete blood cell count showed normocytic anemia with normal leukocyte and platelet counts. Coagulation studies were within normal limits, and hypercoagulable workup was also normal. Specific tests for hypercoagulability disorders included activated protein C resistance, anticardiolipin antibody, antithrombin III, basal homocysteine, complete blood count (with examination of the peripheral smear), D-dimer, Factor V Leiden, fibrinogen, lupus anticoagulant, partial thromboplastin time, protein S, prothrombin time, thrombin time, and antinuclear antibody were normal. Other investigations including a chest radiograph, which was unremarkable. A magnetic resonance image (MRI) of the brain with and without gadolinium contrast revealed early subacute infarcts in left basal ganglia, periventricular white matter, centrum semiovale, and just inferior to left globus pallidus showing restricted diffusion on diffusion-weighted scans. Chronic infarcts with gliotic changes were seen in right basal ganglia, periventricular white matter. Late subacute-chronic infarct was also seen in the head of left caudate nucleus and left periventricular white matter with ex-vacuo prominence of the frontal horn of left lateral ventricle. There was hyperintensity over the sulci of right and left hemisphere with collaterals showing "ivy" sign [Figure 1].
Figure 1: Arrows showing hyperintensity over the sulci of right and left hemisphere with collaterals showing "ivy" sign

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MR angiography done revealed narrowing in a cervical segment of left internal carotid artery (ICA). Moderate to severe stenosis (80%) of cavernous and supraclinoid segments of left ICA with distal flow limitation was noted. Moderate narrowing of a proximal A1 segment of left anterior cerebral artery (ACA) also seen with nonvisualization of the left middle cerebral artery (MCA) and its branches. Severe stenosis was also seen in right MCA and supraclinoid segments of right ICA with paucity of peripheral branches of right MCA. Right ACA and A2, A3 segments of left ACA are normal. Moderate to severe stenosis seen in P1, P2 segments of left posterior cerebral artery (PCA) with nonvisualization of distal branches of left PCA. Vertebral, basilar, and right PCAs are normal [Figure 2].
Figure 2: Moderate narrowing of a proximal A1 segment of left anterior cerebral artery also seen with nonvisualization of the left middle cerebral artery and its branches. Severe stenosis also seen in right middle cerebral artery and supraclinoid segments of right internal carotid artery with paucity of peripheral branches of right middle cerebral artery

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  Discussion Top

Moyamoya disease though first reported in Japan, [1] since then been reported worldwide. The disease has a particularly high incidence in Eastern Asia, especially in Japan. The overall prevalence rate of moyamoya disease in Japan in 1995 was 3.16 per lac with an incidence rate of 0.35 per lac. The male to female ratio was 1.8:1, and a family history of moyamoya was noted in 10% of cases. [7] Unfortunately, to the best of our knowledge, more recent data are unavailable.

The process of narrowing of cerebral vessels seems to be a reaction of brain blood vessels to a wide variety of external stimuli, injuries, or genetic defects. Conditions such as sickle cell anemia, neurofibromatosis-1, Down syndrome, congenital heart defects, antiphospholipid syndrome, renal artery stenosis, and thyroiditis have been found to be associated with moyamoya disease in the literature. But more than half of the adults seen with this disease have no cause for their moyamoya syndrome. The process of blockage, once it begins, tends to continue despite any known medical management unless treated with surgery. MRI not only reveals areas of infarctions but also allows direct visualization of these collateral vessels as multiple small flow voids at the base of brain and basal ganglia. MR angiography is used to confirm the diagnosis and to see the anatomy of the vessels involved. It typically reveals the narrowing and occlusion of proximal cerebral vessels and extensive collateral flow through the perforating vessels demonstrating the classic puff of smoke appearance. [8]

Acute management is mainly symptomatic and directed toward reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures. Revascularization procedures are currently performed to increase the perfusion to the hypoxic brain tissue. The literature supports these procedures, and the long-term favorable outcome has been reported in terms of improvement in symptoms and positive angiographic follow-ups in all age groups. [9] Hence patient was referred to the higher center for further management. On follow-up, it was found that the patient underwent direct revascularization by superficial temporal artery-MCA anastomosis [10] and had no complications in the postoperative period and was discharged.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Takeuchi K, Shimizu K. Hypogenesis of bilateral internal carotid arteries. No To Shinkei 1957;9:37-43.  Back to cited text no. 1
Goyal JP, Rao SS, Trivedi S. Moyamoya disease in a child: A case report. Case Rep Neurol Med 2011;2011:329738.  Back to cited text no. 2
Singhi P, Choudhary A, Khandelwal N. Pediatric moyamoya disease: Clinical profile, literature review and sixteen year experience from a tertiary care teaching institute. Indian J Pediatr 2013;80:1015-20.  Back to cited text no. 3
Chinchure SD, Pendharkar HS, Gupta AK, Bodhey N, Harsha KJ. Adult onset moyamoya disease: Institutional experience. Neurol India 2011;59:733-8.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969;20:288-99.  Back to cited text no. 5
Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y. Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 2005;36:2148-53.  Back to cited text no. 6
Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, et al. Epidemiological features of moyamoya disease in Japan: Findings from a nationwide survey. Clin Neurol Neurosurg 1997;99 Suppl 2:S1-5.  Back to cited text no. 7
Atlas SW. MR angiography: Techniques and clinical applications MRI of the brain and spine. 3 rd ed. Philadelphia, Pa, USA: Lippincott Williams and Wilkins; 2002.  Back to cited text no. 8
Han DH, Kwon OK, Byun BJ, Choi BY, Choi CW, Choi JU, et al. A co-operative study: Clinical characteristics of 334 Korean patients with moyamoya disease treated at neurosurgical institutes (1976-1994). The Korean Society for Cerebrovascular Disease. Acta Neurochir (Wien) 2000;142:1263-73.  Back to cited text no. 9
Smith ER, Scott RM. Surgical management of moyamoya syndrome. Skull Base 2005;15:15-26.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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