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Abstract Details

Moyamoya Angiopathy related to Thalassemia- experience from a tertiary care in Eastern India
Cerebrovascular Disease and Interventional Neurology
Cerebrovascular Disease and Interventional Neurology Posters (7:00 AM-5:00 PM)
262
To observe the clinical and radiological characteristics of Moyamoya angiopathy(MMA) with thalassemia and scrutinize the literature for possible pathophysiological basis and prognostic indicators.
MMA is an intracranial angiopathy is characterized by bilateral progressive steno-occlusive changes of the intracranial portion of the internal carotid arteries and proximal portions of the anterior cerebral artery and/or middle cerebral artery, if associated with a well recognized condition, it is called Moyamoya syndrome(MMS).
Of the 98 cases of MMA collected in our Indian center in last 4 years, 4 new patients with the rare co-occurence of thalassemia and MMS were found. Thalassemia cases were confirmed by hemoglobin electrophoresis and MMA was diagnosed on the basis of MR angiography. Other known secondary causes of MMA were ruled out by relevant investigations. Thirteen previously reported cases of thalassemia and MMA were retrieved by literature search in PubMed and Google Scholar using the keywords “Moyamoya” AND “thalassemia”. Subsequently all data were analyzed and compared by using descriptive statistics.
Analysis of our 4 cases and those 13 found in the literature showed early childhood diagnosis of thalassemia and in most cases later manifestation of MMS in the age of 14.5+10.72 years(mean+SD) in our cases and with 10.97+6.47 years in previous cases. While 9 out of the former 13 and 3 of our 4 cases showed obvious infarcts in brain imaging, 1 case with HbE-β-thalassemia presented with intracerebral hemorrhage. Hemiplegia/hemiparesis was present among all of our 4 cases, while it was present in 69.23% cases of the previous 13 reports. Neither transfusion dependence nor the history of splenectomy was found to be associated with MMA development
These four new cases of MMS in thalassemia enlarged our knowledge about MMS in patients with thalassemia. MMS is a relevant complication in patients with thalassemia and early detection is essential to avoid disability.
Authors/Disclosures
Shambaditya Das, Jr., MD, MBBS (Bangur Institute of Neuroscience)
PRESENTER 		Dr. Das has nothing to disclose.
Souvik Dubey Souvik Dubey has nothing to disclose.
Biman K. Ray, MD Dr. Ray has nothing to disclose.