6 year old with headache &drowsiness



Doctor's Information

Affiliation :SRM Medical College Hospital & Research Centre
Academic Degree : MBBS (MD) fial yr PG
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Case Section



Patient's Information

Gender : Female
Age : 6


Clinical Summary

This article is a case report of a 6 YR old child who was admitted with c/o headache on & off for past 3 yrs assosciated with h/o drowsiness & vomitting .h/o drop attacks present .no h/o trauma.


Imaging Findings

IMAGE 1--MRI revealed presence of infarction in the bilateral frontal lobes as classic for a paediatric moya moya. (while haemorrhage from collaterals is common in adults) . IMAGE 2&3 -Leptomeningeal enhancement the “ivy sign,” represents the fine vascular network over the pial surface is demonstrated in FLAIR images . IMAGE 4&5--MRA was able to detect abnormal vessels in the basal ganglia ,basal cisterns & periventricular white matter which are known as “MOYA MOYA VESSELS”. IMAGE 5&6- CONVENTIONAL ANGIO (DSA) Occlusion of supra clinoid portion of internal carotid arteries on both sides (rt>lt) with complete stenosis of M1 segment of rt MCA & A1 SEGMENT OF RT ACA corresponding to stage IV in rt side & stage III in left side according to suzuki’s classification IMAGE 7,8&9-Small abnormal netlike vessels proliferate giving the characteristic "puff of smoke" appearance on direct angiography. 


Differential Diagnosis

Moyamoya syndrome (also termed the moyamoya pattern or phenomenon) is due to numerous conditions that can cause arterial occlusion of the circle of Willis, with resultant collaterals, and appearances reminiscent of moyamoya disease. These conditions include
• cranial radiotherapy
• neurofibromatosis type 1(NF1)
• tuberous sclerosis (TS)
• infection
o tuberculous meningitis
o bacterial meningitis
o post-varicella vasculitis
• connective tissue disorders
o systemic lupus erythematosus (SLE)
o sickle cell disease
• fibromuscular dysplasia
• Fanconi anaemia
• Graves disease 5
• anti phospholipid syndrome
• Down syndrome
• ulcerative colitis (UC)
• aplastc anaemia
• Apert syndrome
• leptospirosis
• oral contraceptive use
• atherosclerosis


Final Diagnosis

Moya Moya disease


Discussion (Related Text)

INCIDENCE The disease is common in japan with an incidence of 3 per 1,00,00 which is a common cause for paediatric stroke but definitely it is very rare in INDIA. Bi-modal age distribution early childhood : peak ~ 4 years of age middle age : 30 - 40 years of age M:F 1:2 Symptoms at presentation Common Ischemic stroke 50–75 Transient ischemic attack (including drop attacks) 50–75 Hemorrhage (in adults) 10–40 Less common Seizures Headache Suzuki Grading System
I Narrowing of ICA apex
II Initiation of moyamoya collaterals
III Progressive ICA stenosis with intensification of moyamoya-associated collaterals
IV Development of ECA collaterals
V Intensification of ECA collaterals and reduction of moyamoya-associated vessels
VI Total occlusion of ICA and disappearance of moyamoya-associated Collaterals
JINX about MR angiography --- It is safer and easier to perform than conventional angiography. Tends to overestimate the degree of stenosis in the internal carotid artery and other arteries. SURGICAL RX Direct superficial temporal artery–middle cerebral artery anastomosis is not easy to perform in children because both the donor scalp artery and the recipient cortical artery are small. Indirect surgical procedures for moyamoya disease include encephalomyosynangiosis and encephaloduroarteriosynangiosis. At present, encephaloduroarteriosynangiosis is generally accepted as the treatment of choice in childr en with moyamoya disease Conclusion Moyamoya is a rare disease but should be suspected in unexplained etiology of stroke in both children and adults. Patients with certain conditions such as Down’s syndrome and sickle cell disease may be particularly at risk for moyamoya. Characteristic radiographic findings confirm the diagnosis, and recognition of the disease early in its course, with prompt institution of therapy, is critical in order to achieve the best outcome in patients. Revascularization surgery appears to be effective in preventing stroke in patients with moyamoya. often fails to show fine collateral vessels and, as a result, tends to underestimate moyamoya vessels. Although it has a limitation in accurate preoperative staging, it may have a promising role for evaluating postoperative outcome



O. Togao,F. Mihara From the Department of Clinical Radiology (O.T., F.M., T.Y., A.T., T.N., Y.K., K.K., H.H.), Graduate School of Medical Sciences, Kyushu University, and the Department of Neurosurgery (T.M.), Hamanomachi Hospital, Fukuoka, Japan Hye-Kyung Yoon Department of Radiology, Samsung Medial Center, Sungkyunkwan University School of Medicine, 50 Irwon-Dong, Kangnam-Gu, Seoul, 135-230, Korea. R. Michael Scott, M.D., and Edward R. Smith, M.D. From the Department of Neurosurgery, Children’s Hospital Boston, and Harvard Medical School, Boston. Suzuki J, Kodama N. Moyamoya disease: a review. Stroke 1983;14:104–114


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