Varied presentations of moyamoya disease in a tertiary care hospital of north-east India

Abstract

Introduction:Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India.Objectives:To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India.Material and Methods:Relevant investigations were done to rule out other causes of moyamoya syndrome.Results:We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs.Conclusion:Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.

Moyamoya disease presenting as alternating hemiparesis with relapsing remitting hemichorea: An unusual manifestation

“…Its low incidence always makes it prone to be missed during the presentation as in our case where the diagnosis was made during the third attack, similar to the findings suggested by Borah et al . [ 3 ] Epilepsy is another important presentation of MMD; however, it is reported in very few cases. [ 5 6 7 ] Movement disorder, an important clinical presentation especially among the pediatric patients, could be the initial manifestation or could be observed during the disease course with varied picture ranging from dystonia, chorea, or dyskinesia.…”

“…[ 1 ] Recurrent ischemic attack and seizure disorder are the frequent manifestations among the children, whereas hemorrhagic stroke is common among adults. [ 1 2 3 4 ] Movement disorder is an extremely rare presentation among the patients with MMD with 3%–6% of the cases with varied movement disorder. [ 1 2 4 ] Ischemic changes or manipulation in the inhibitory–excitatory circuits interconnecting the basal ganglia and cerebral cortex plays a crucial role for the movement disorder pathology among these patients.…”

“…There are majority of case reports with hemichorea, anterior circulation involvement, alternating hemiparesis, and hemiparesis with hemichorea. [ 3 ] Here we report a unique case with alternating hemiparesis with recurrent episodes of hemichorea.…”

Moyamoya Disease Associated With Morning Glory Disc Anomaly and Other Ophthalmic Findings: A Mini-Review

“…Diverse ocular manifestations are sometimes consequences of ischemia or hemorrhage in MMD and may differ from the regions involved. A total of 10 cases from eight articles referring to ischemia-or hemorrhage-related ocular manifestations are summarized and reviewed, five of which are from case series ( Table 2) (31)(32)(33)(34)(35)(36)(37)(38). Most patients had a significant ischemia lesion in the cerebral imaging, and only one patient was diagnosed as having a hemorrhage.…”

“…One patient in Case 23 did not have any suggestive change of ischemia or hemorrhage in imaging. She was found with recurrent amaurosis fugax, and cerebral angiography only showed bilateral narrowing of the ICA, ACA, and MCA (35).…”