Moyamoya syndrome in a splenectomized patient with β-thalassemia intermedia

Masafumi Sanefuji, Shouichi Ohga, Ryutaro Kira, Takashi Yoshiura, Hiroyuki Torisu, Toshiro Hara

Research output: Contribution to journalArticle

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Abstract

We describe a 14-year-old Japanese girl with β-thalassemia intermedia who developed moyamoya syndrome after splenectomy. This patient had compound heterozygous mutations of the β-globin gene and received occasional transfusions. After splenectomy at 12 years of age, she transiently required partial exchange transfusions for leukoerythroblastosis but attained transfusion independence. Two years after the splenectomy, transient ischemic attacks occurred repeatedly with right hemiparesis or left paresthesia. Magnetic resonance imaging revealed bilateral stenosis of the internal carotid arteries and dilatation of the perforating branches with the formation of moyamoya vessels but not infarctions. The strict adherence to aspirin and dipyridamole has led to no stroke or progression of the vasculopathy for 8 years. Moyamoya disease has been reported in a patient with β-thalassemia major. Cerebral vasculopathy can be a rare but grave consequence of the thromboembolic complications in β-thalassemia major/intermedia.

Original languageEnglish
Pages (from-to)75-77
Number of pages3
JournalJournal of Child Neurology
Volume21
Issue number1
DOIs
Publication statusPublished - Jan 1 2006

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Moyamoya Disease
beta-Thalassemia
Splenectomy
Globins
Dipyridamole
Paresthesia
Carotid Stenosis
Transient Ischemic Attack
Paresis
Infarction
Aspirin
Dilatation
Stroke
Magnetic Resonance Imaging
Mutation
Genes

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Moyamoya syndrome in a splenectomized patient with β-thalassemia intermedia. / Sanefuji, Masafumi; Ohga, Shouichi; Kira, Ryutaro; Yoshiura, Takashi; Torisu, Hiroyuki; Hara, Toshiro.

In: Journal of Child Neurology, Vol. 21, No. 1, 01.01.2006, p. 75-77.

Research output: Contribution to journalArticle

Sanefuji, Masafumi ; Ohga, Shouichi ; Kira, Ryutaro ; Yoshiura, Takashi ; Torisu, Hiroyuki ; Hara, Toshiro. / Moyamoya syndrome in a splenectomized patient with β-thalassemia intermedia. In: Journal of Child Neurology. 2006 ; Vol. 21, No. 1. pp. 75-77.
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