Moyamoya syndrome with spherocytosis: Effect of splenectomy on strokes

Yoichi Tokunaga, Shouichi Ohga, Sachiyo Suita, Toshio Matsushima, Toshiro Hara

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

A 3-year-old male patient with hereditary spherocytosis who developed moyamoya syndrome, presenting hemiplegia, and slurred speech is reported. Transient ischemic attacks occurred repeatedly with hemolytic crises. Magnetic resonance imaging and angiography revealed bilateral occlusion of the internal carotid and middle cerebral arteries with the formation of moyamoya vessels and multiple infarctions in the basal ganglia. Although splenectomy can increase the risk of stroke, no stroke occurred after splenectomy. On aspirin and dipyridamole therapy the patient has been free of neurologic deficits and progression of the vasculopathy for 5 years. This rare observation suggests that anemic hypoxia more greatly contributes to the progression of moyamoya syndrome than postsplenectomy thrombocytosis or reduced deformability of spherocytes.

Original languageEnglish
Pages (from-to)75-77
Number of pages3
JournalPediatric Neurology
Volume25
Issue number1
DOIs
Publication statusPublished - Aug 20 2001

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Moyamoya Disease
Splenectomy
Spherocytes
Stroke
Hereditary Spherocytosis
Thrombocytosis
Hemiplegia
Dipyridamole
Magnetic Resonance Angiography
Transient Ischemic Attack
Middle Cerebral Artery
Neurologic Manifestations
Basal Ganglia
Infarction
Aspirin
Magnetic Resonance Imaging
Observation
Therapeutics
Hypoxia

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology

Cite this

Moyamoya syndrome with spherocytosis : Effect of splenectomy on strokes. / Tokunaga, Yoichi; Ohga, Shouichi; Suita, Sachiyo; Matsushima, Toshio; Hara, Toshiro.

In: Pediatric Neurology, Vol. 25, No. 1, 20.08.2001, p. 75-77.

Research output: Contribution to journalArticle

Tokunaga, Yoichi ; Ohga, Shouichi ; Suita, Sachiyo ; Matsushima, Toshio ; Hara, Toshiro. / Moyamoya syndrome with spherocytosis : Effect of splenectomy on strokes. In: Pediatric Neurology. 2001 ; Vol. 25, No. 1. pp. 75-77.
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