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Year : 2018  |  Volume : 6  |  Issue : 3  |  Page : 108-110

Moyamoya disease masquerading as recurrent headaches in a 4-year-old child

Department of Paediatrics, G.B. Pant Hospital, Srinagar, Jammu and Kashmir, India

Date of Web Publication12-Sep-2018

Correspondence Address:
Dr. Ishtiyaq Qadri
G.B. Pant Hospital, Srinagar, Jammu and Kashmir - 190 004
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartindia.heartindia_4_17

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Moyamoya disease can have varied clinical manifestations and the diagnosis is often made only on acute presentation. We present a case of 4 year old male child diagnosed with moyamoya disease who had a history of recurrent headaches in past.

Keywords: Headaches, moyamoya disease, transient ischemic attack

How to cite this article:
Qadri I, Ashraf M, Kumar Parihar A. Moyamoya disease masquerading as recurrent headaches in a 4-year-old child. Heart India 2018;6:108-10

How to cite this URL:
Qadri I, Ashraf M, Kumar Parihar A. Moyamoya disease masquerading as recurrent headaches in a 4-year-old child. Heart India [serial online] 2018 [cited 2022 Jan 28];6:108-10. Available from: https://www.heartindia.net/text.asp?2018/6/3/108/241073

  Introduction Top

Moyamoya disease is a rare cerebrovascular problem often kept low in differential diagnoses of neurological ailments in children. It is a progressive disease of the cerebral vasculature with occlusive involvement of the circle of Willis and its feeding arteries.[1] Moyamoya disease occurs primarily in Asians, incidence being highest in Japan,[2] but cases in Whites, Blacks, and Hispanics have also been reported. The symptoms and clinical course are variable. Adults commonly present with intracranial hemorrhage while children usually present with hemiparesis, monoparesis, sensory impairment, involuntary movements, dizziness, or seizures. We report a case of a 4-year-old male child who had recurrent headaches and one episode of transient ischemic attack and on evaluation turned out to be a case of moyamoya disease.

  Case Report Top

A 4-year-old male child presented to hospital with complaint of sudden-onset weakness in his right arm and leg, associated with multiple episodes of projectile vomiting on the preceding day. History details revealed that the patient used to have headaches since last 5 months. The headaches were frequent, throbbing in nature, global in distribution, of moderate intensity not relieved by acetaminophen and with no associated signs or symptoms (auras). The patient was being managed as a case of migraine. The patient had no associated history of fever, night sweats, ataxia, photophobia, tingling sensation or numbness, difficulty swallowing, difficulty hearing, vision problems, convulsions, trauma head, altered sensorium, or drug intake. Family history did not reveal any history of recurrent headaches or acute neurological events. Child's development was normal. On examination, vitals were stable and within normal parameters. Higher mental functions were normal and no features of raised intracranial pressure were present. There were no neurocutaneous markers. Cranial nerves were intact and vision was 6/6 in both eyes. Gait was hemiplegic, tone was decreased over the right side, power was 2/5 in the right upper and lower limb, deep tendon reflexes were absent, and planter being extensor on the right side. There was slightly decreased vibratory sensation and proprioception in his toes more than fingers. The patient did not have neck stiffness. Fundoscopic examination was normal. Hematological parameters, coagulation profile, and cerebrospinal fluid (CSF) examination were normal. Electrocardiography and echocardiography were also normal. Non contrast computed tomography head noted a hypodense area involving left occipital area suggestive of infarct [Figure 1]. Magnetic resonance (MR) angiography was done as part of the diagnostic workup which showed a profuse collateral formation with attenuated supraclinoid internal carotid arteries and their main branches and bilateral posterior carotid arteries [Figure 2]. The patient was managed conservatively and showed marked improvement over 24 h with no residual weakness on discharge. The patient was then attached to the neurosurgery department for cerebral revascularization surgery and is also being followed up at pediatric medicine outpatient department at our hospital.
Figure 1: Non contrast CT of the patient

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Figure 2: The three images are MR Angio of the same patient

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  Discussion Top

The first case of moyamoya disease was reported from Japan in 1963. There were 821 registered cases of moyamoya disease in Japan up to 1994.[3] The disease has been now found in all races with a variable age distribution and presentation. The term means a “hazy puff of smoke” and was used to describe the abnormal vasculature at the base of the brain.[4] It involves a progressive occlusion of the circle of Willis arteries, leading to the development of characteristic collateral vessels. Overall etiology of the disease is unknown. Genetic factors might play a role as evidenced by higher incidence of disease in Japan and Korea and the familial occurrence. Cerebral vessel narrowing has been ascribed to be a reaction of brain vasculature to a variety of external stimuli modified by genetic defects. Sickle cell anemia, neurofibromatosis-1, Down's syndrome, congenital heart defects, antiphospholipid syndrome, renal artery stenosis, and thyroiditis have been found to be associated with moyamoya disease.[5] The process of blockage once it begins is progressive. There is some evidence to show that CSF brain fibroblast growth factor and transforming growth factor beta-1 may play a role in the pathogenesis of the disease.[6] Further, inconclusive evidence from some studies has suggested a relationship with Epstein–Barr virus infection.[7]

The symptoms of disease vary widely, with the disease ranging from being asymptomatic to manifesting as transient events to severe neurologic deficits. Adults present with hemorrhage more commonly while cerebral ischemic events are more common in children. Moyamoya disease presents mostly in the first decade of life in children.[2] The various presentations in children include hemiparesis,[8] monoparesis, sensory impairment, involuntary movements, dizziness, or psychomotor impairment.[9] There have been many case reports of adult moyamoya disease presenting as recurrent headaches. However, such a presentation is relatively rare in children and only one such case report was noted in pediatric age group on review of literature.

Some patients of moyamoya disease have pituitary and thyroid hypofunction and monitoring for hypothalamic–pituitary dysfunction is suggested.[10] MR angiography is used to confirm the diagnosis and to see the anatomy of the vessels involved. Angiographic criteria of the diagnosis of moyamoya disease include stenosis or occlusion of the distal parts of intracranial internal carotid arteries and proximal parts of anterior and middle arteries, as well as the presence of collateral vasculature in the regions of the brain base, without causal disease. In case of bilateral changes, the diagnosis is considered as sure. Unilateral changes are qualified as probable.[11] The role of radiological imaging in moyamoya disease is to screen the disease based on clinical findings, evaluation of the changes in vasculature and brain parenchyma, and clinical follow-up. Imaging findings can be classified as primary and secondary. The primary findings essentially consist of occlusion of the circle of Willis and collateral formation, including moyamoya vessel formation. The secondary findings include cerebral infarction, white matter lesions, atrophy, and hemorrhage. For the visualization of the primary and secondary findings as well as postoperative results, MR imaging and MR angiography are the most reliable methods and play important roles because of their excellent diagnostic yield and noninvasiveness.[12] Formation of moyamoya network by perforating branches of the posterior cerebral artery is known as posterior basal moyamoya as was the case in our patient. Acute management is mainly symptomatic and supportive. No effective medical therapy for moyamoya disease is available. Surgical revascularization is thought to improve cerebral perfusion and to reduce the risk of subsequent ischemic events. Surgical procedures include indirect revascularization procedures such as encephaloduroarteriosynangiosis, pial synangiosis, and indirect revascularization using omental flaps or direct revascularization procedures such as superficial temporal-middle cerebral artery bypass. Prognosis of patients is found to be related to age and the type of presentation.

  Conclusion Top

This is the first such case reported in pediatric age group from the state of Jammu and Kashmirr. The case highlights chronic headache as one of the important and often overlooked presentations of moyamoya disease in children and should be considered as one of the differentials in a child with chronic headaches in whom headache is not relieved on routine medical treatment.

Patient consent

Informed written consent for publication of clinical details was obtained from primary caretakers of the patient.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kuroda S, Houkin K. Moyamoya disease: Current concepts and future perspectives. Lancet Neurol 2008;7:1056-66.  Back to cited text no. 1
Kim JS. Moyamoya disease: Epidemiology, clinical features, and diagnosis. J Stroke 2016;18:2-11.  Back to cited text no. 2
Fukui M. Current state of study on moyamoya disease in Japan. Surg Neurol 1997;47:138-43.  Back to cited text no. 3
Burke GM, Burke AM, Sherma AK, Hurley MC, Batjer HH, Bendok BR, et al. Moyamoya disease: A summary. Neurosurg Focus 2009;26:E11.  Back to cited text no. 4
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. 5
Malek AM, Connors S, Robertson RL, Folkman J, Scott RM. Elevation of cerebrospinal fluid levels of basic fibroblast growth factor in moyamoya and central nervous system disorders. Pediatr Neurosurg 1997;27:182-9.  Back to cited text no. 6
Tanigawara T, Yamada H, Sakai N, Andoh T, Deguchi K, Iwamura M, et al. Studies on cytomegalovirus and Epstein–Barr virus infection in moyamoya disease. Clin Neurol Neurosurg 1997;99 Suppl 2:S225-8.  Back to cited text no. 7
Goyal JP, Rao SS, Trivedi S. Moya moya disease in a child: A case report. Case Rep Neurol Med 2011;2011:329738.  Back to cited text no. 8
Ashrafi MR, Alizadeh H, Yazdani SH, Mohseni M, Mohamadi M. Psychomotor delay, a possible rare presentation of moyamoya disease. Iran J Radiol 2011;8:75-8.  Back to cited text no. 9
Mootha SL, Riley WJ, Brosnan PG. Hypothalamic-pituitary dysfunction associated with moyamoya disease in children. J Pediatr Endocrinol Metab 1999;12:449-53.  Back to cited text no. 10
Tarasów E, Kułakowska A, Lukasiewicz A, Kapica-Topczewska K, Korneluk-Sadzyńska A, Brzozowska J, et al. Moyamoya disease: Diagnostic imaging. Pol J Radiol 2011;76:73-9.  Back to cited text no. 11
Hasuo K, Mihara F, Matsushima T. MRI and MR angiography in moyamoya disease. J Magn Reson Imaging 1998;8:762-6.  Back to cited text no. 12


  [Figure 1], [Figure 2]


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