IMAGES IN NEUROLOGY
|Year : 2006 | Volume
| Issue : 2 | Page : 124-125
S Raghavendra, T Chemmanam, C Kesavadas, C Sarada
Departments of Neurology and Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695011, India
Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum - 695011
|How to cite this article:|
Raghavendra S, Chemmanam T, Kesavadas C, Sarada C. Moyamoya disease. Ann Indian Acad Neurol 2006;9:124-5
| Case Report|| |
A 2-year-old boy, with normal birth and development, presented with recurrent neurological deficits. He currently presented with acute hemiparesis. He previously had left hemiparesis three months ago and right hemiparesis a month ago that had completely recovered over few weeks. There was no other significant past medical history. General physical and systemic examination was normal. Neurological examination revealed grade 3 left hemiparesis. Cranial nerves, sensory and cerebellar systems were normal.
Investigations showed a normal haemogram and peripheral smear. Sickling test was negative. Biochemical tests including fasting blood glucose, renal function tests and liver function tests were normal. Urine for homocysteine was negative. A cardiac work-up including echocardiography and ECG were normal. ELISA for HBsAg and HIV were negative. Carotid and renal doppler were normal. CSF did not reveal any abnormalities.
MRI brain, 4 weeks after the episode of right hemiparesis, showed an old left MCA territory infarct [Figure - 1]A. The vascular flow voids showed the supraclinoid carotids, M1 segment of the middle cerebral artery (MCA) and anterior cerebral artery (ACA) to be significantly narrowed on both sides [Figure - 1]B. In addition, there was proliferation of the fine collateral vessels in the circle of Willis [Figure - 1]C.
Digital subtraction angiogram (DSA) showed significant narrowing of supraclinoid ICA, M1 segments of the MCA on both sides and A1 segment of the right ACA. Left ACA segment was occluded. There was proliferation of the lenticulostriate branches of MCA and ethmoidal branches of the ophthalmic artery, giving a puff of smoke appearance at the basal region (basal moyamoya) and in the orbit (efthmoidal moyamoya) [Figure - 1] (C, D, E). ACA and MCA branches were filling partially from PCA on both sides [Figure - 1]F. External carotid artery, arch aortogram and renal angiogram were normal. Incidentally, a fetal PCA was also noted.
| Discussion|| |
The causes of recurrent strokes involving both the MCA territories in a 2-year old boy, includes cardiovascular and hematological disorders, homocysteinemia and large vessel vasculitis, apart from metabolic conditions like mitochondrial disorders, aminoacidurias and haemolytic uremic syndrome. MRI brain in our patient showed narrowing of the supraclinoid ICA, MCA and ACA bilaterally, in addition to the MCA infarct. The findings were further corroborated by the angiographic changes. The patient fulfilled the clinical and angiographic criteria for definitive diagnosis of moyamoya syndrome.
Moyamoya disease is a clinical entity with bilateral stenosis or occlusion of the large intracranial vessels at the base of brain, with proliferation of fine collaterals. The presentation is usually characterised by ischemic events in children and intracranial bleed in adults. Ethomidal moyamoya, as found in our patient, is more common in children and reflects directly to the severity of the disease, unlike in adults.
| References|| |
|1.||Gosalakkal JA. Moyamoya disease: A review. Neurol India 2002;50:6-10. |
|2.||Suzuki J, Kodama N. Moyamoya disease- A review. Stroke 1983;14:104-9. [PUBMED] |
[Figure - 1]