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Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 58-60

A case report of moyamoya disease from nonendemic region of upper part of Brahmaputra Valley of North Eastern India

Department of Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India

Date of Web Publication1-Mar-2018

Correspondence Address:
Dr. Dhriti Sundar Das
Department of Medicine, Assam Medical College and Hospital, Dibrugarh - 786 002, Assam
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijhas.IJHAS_28_17

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Moyamoya disease is a chronic nonatherosclerotic, noninflammatory cerebrovascular disease characterized by slow progressive stenosis of the intracranial vessels. The clinical presentations of moyamoya disease differ considerably for both children and adults. Interestingly, moyamoya disease continues to remain as an “unsolved mystery” till date, and its occurrence in the nonendemic region of the upper part of Brahmaputra Valley is rare. In this context, the author wishes to report the clinical study of an 18-year-old young adolescent boy exhibiting sudden-onset dizziness, severe excruciating headache, vomiting, abnormal behavior, and urinary and fecal incontinence.

Keywords: Moyamoya, nonendemic occurrence, Suzuki-grade III

How to cite this article:
Das DS. A case report of moyamoya disease from nonendemic region of upper part of Brahmaputra Valley of North Eastern India. Int J Health Allied Sci 2018;7:58-60

How to cite this URL:
Das DS. A case report of moyamoya disease from nonendemic region of upper part of Brahmaputra Valley of North Eastern India. Int J Health Allied Sci [serial online] 2018 [cited 2022 Jun 28];7:58-60. Available from: https://www.ijhas.in/text.asp?2018/7/1/58/226256

  Introduction Top

Moyamoya disease is a stenotic cerebrovascular disorder wherein distal branches of internal carotid arteries are involved with collateral formation. The disease has a history of common occurrence in East Asia particularly in Japan,[1] with a few reports from other parts of the world including India.[2] The disease still remains to be an “enigma” and its definitive treatment is controversial.[3] However, the occurrence of such cases in nonendemic area of the northeastern part of India is rare. In this milieu, the present case study of moyamoya disease in an 18-year-old adolescent boy is worth reporting.

  Case Report Top

An 18-year-old adolescent boy was presented in the emergency department with the chief complaints of sudden-onset dizziness and vomiting. It was soon followed by altered behavior and involuntary passage of urine and stool. Briefly, the patient had an episode of altered sensorium along with bladder and bowel incontinence. The same was preceded by dizziness and vomiting along with the signs of focal neurological deficit, i.e., extensor plantar response on the right side, raising the suspicion of raised intracranial pressure which was further confirmed by neuroimaging.

On evaluation, his mother denied any significant past history of similar episode. His personal history does not reveal addiction to any substance apart from occasional chewing of betel nut and tobacco. In addition, he had no family history of cerebrovascular accident or seizure disorder or any history of early-onset vascular disease, neurofibromatosis, central nervous system infection, vasculitis, or radiation therapy.

After admission in the hospital, the patient developed severe excruciating headache which increased his restlessness. On general examination, he looked apparently healthy, vitals being blood pressure of 140/90 mmHg and pulse rate of 84/min, body temperature of 98.8°F, and oxygen saturation of 98%. Examination of the abdomen, skin, and musculoskeletal system was normal. Nervous system examination revealed normal cranial nerve examination. Normal power and tone were noted in both upper and lower limbs, and reflexes were normal except for the plantar response, which was extensor on the right side while withdrawal response on the left. Gait was normal. Gradually, headache increased which was not responding to analgesics and other supportive measures. Complete blood cell count showed leukocytosis with normal platelet counts. Coagulation studies were within normal limits. Diagnostic studies such as echocardiography were found to be normal. Computed tomography scan of the brain of the patient reported intraparenchymal hemorrhage in the left gangliothalamic region involving the thalamus and posterior limb of internal capsule with extension of bleed to all ventricles along with left high parietal lobe edema [Figure 1].
Figure 1: Computed tomography scan of brain, with an arrow showing intraparenchymal hemorrhage with ventricular extension

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The total leukocyte count of the patient was high, amounting to 17,300/cm 3 which may be attributed to infection or reactive leukocytosis. Magnetic resonance imaging of brain with magnetic resonance angiography reveals the following:

  1. Subacute bleed in the left thalamus with ventricular extension
  2. Chronic ischemic insult with encephalomalacic changes in the left frontal and in high parietal lobes
  3. Chronic lacunar infarcts in the white matter of left frontal and right occipital lobes
  4. Abrupt narrowing of the bilateral supraclinoid internal carotid artery with nonvisualization of distal branches with extensive basal ganglia and thalamoperforating collaterals bilaterally and no involvement of the posterior circulation [Figure 2].
Figure 2: Magnetic resonance imaging of brain with magnetic resonance angiography, with an arrow showing abrupt narrowing of internal carotid artery with collaterals

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These inferences are suggestive of moyamoya disease (Suzuki-grade III).

Conservative management was given which included osmotherapy, i.e., mannitol, analgesics for headache, intravenous antibiotics, proton pump inhibitors, and intravenous fluids as necessary along with others. Neurology and neurosurgery consultation were also taken.

To our distress, they could not afford further investigation and follow-up owing to the financial condition of the patient. After discussing the prognosis with the attendant, he was discharged and advised to attend higher center if possible. The patient did not come up for follow-up subsequently.

However, due to its low prevalence in this part of India, it is frequently ignored and so the disease may not be considered in the differential diagnosis.

  Discussion Top

As stated earlier, moyamoya disease is common in Japan as compared to rest of the world, so the same is frequently overlooked in other populations. It is pertinent to bring this disease in the differential diagnosis of the patient, especially in the young of Asian origin presented with stroke. However, this disease is found in North-East India as well, due to genetic ties with the population of Japan. The high prevalence among Japanese and other Asian populations (including North-East India) – associated with occasional family history – implies the presence of a genetic predilection.[4],[5] The age distribution revealed two peaks: one between 45 and 49 years and second between 5 and 9 years.[6] The female-to-male ratio has been documented to be 1.8:2.2 (showing female predominance).[7],[8] The patient described here was diagnosed at 18 years, which falls in between the two peak age groups. About 33% pediatric patients encounter initially with a headache.[9] Severe excruciating headache frequently occurs in the frontotemporal area in the morning hours but resolves spontaneously.[10] The ischemic stroke is common in children and hemorrhagic stroke is common in adults of Japan.[11] Contrastingly, the report by Chiu et al.[12] reveals that ischemic stroke is common in both children and adults (cases studied at The University of Texas, Houston).

Presentations of moyamoya disease are varied and wide. Clinically, these include seizures, transient ischemic attacks, ischemic, and hemorrhagic strokes.[7],[13] In this case report, the patient presented with a severe headache coupled with altered behavior.

Literature reports that the common bleeding sites in patients with intracranial bleeds are in the lateral ventricle, basal ganglia, and thalamus.[7] This is due to the rupture of collaterals (moyamoya vessels) or aneurysms.[14] Owing to the poor prognosis, this disease needs to be diagnosed early and offered treatment.[15]

  Conclusion Top

Moyamoya disease should be brought into the differential diagnosis in children, young adults, and individuals of Asian origin presenting with stroke, particularly in this part of the country, i.e., North-East India due to the genetic links. This case report may be “a kid in the block” of the prevalence of moyamoya disease among the northeastern populations of India, which may further initiate similar studies in this vein.


The author is thankful to the team of Unit III, Department of Medicine, Assam Medical College and Hospital, Dibrugarh, India, for their cooperation and assistance. Dr. S. Pramanik is acknowledged for her help and suggestions in the preparation of the manuscript.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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. 1
Kuriyama S, Kusaka Y, Fujimura M, Wakai K, Tamakoshi A, Hashimoto S, et al. Prevalence and clinicoepidemiological features of moyamoya disease in Japan: Findings from a nationwide epidemiological survey. Stroke 2008;39:42-7.  Back to cited text no. 2
Borah P, Sharma V, Basumatary LJ, Das M, Goswami M, Kayal AK, et al. Varied presentations of moyamoya disease in a tertiary care hospital of North-East India. Ann Indian Acad Neurol 2014;17:317-20.  Back to cited text no. 3
[PUBMED]  [Full text]  
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. 4
Yamauchi T, Houkin K, Tada M, Abe H. Familial occurrence of moyamoya disease. Clin Neurol Neurosurg 1997;99:S162-7.  Back to cited text no. 5
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. 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
Takanashi JI. Moyamoya disease in children. Brain Dev 2011;33:229-34.  Back to cited text no. 8
Matsushima Y, Aoyagi M, Nariai T, Nojiri T, Ohno K. Headache in pediatric moyamoya patients: Pre-and postoperative changes. Nerv Syst Child 2000;25:442-7.  Back to cited text no. 9
Kawabori M, Kuroda S, Nakayama N, Hirata K, Shiga T, Houkin K, et al. Effective surgical revascularization improves cerebral hemodynamics and resolves headache in pediatric moyamoya disease. World Neurosurg 2013;80:612-9.  Back to cited text no. 10
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.  Back to cited text no. 11
Chiu D, Shedden P, Bratina P, Grotta JC. Clinical features of moyamoya disease in the United States. Stroke 1998;29:1347-51.  Back to cited text no. 12
Bertora P, Lovati C, Gambaro P, Vicenzi A, Rosa S, Osio M, et al. Moyamoya disease in a member of the roma gypsy community. Eur Neurol 2008;59:274-5.  Back to cited text no. 13
Ishimori ML, Cohen SN, Hallegua DS, Moser FG, Weisman MH. Ischemic stroke in a postpartum patient: Understanding the epidemiology, pathogenesis, and outcome of moyamoya disease. Semin Arthritis Rheum 2006;35:250-9.  Back to cited text no. 14
Janda PH, Bellew JG, Veerappan V. Moyamoya disease: Case report and literature review. J Am Osteopath Assoc 2009;109:547-53.  Back to cited text no. 15


  [Figure 1], [Figure 2]


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