Cerebrovascular disease in sickle cell anemia: A clinical, pathological and radiological correlation

K. H H Merkel, P. L. Ginsberg, J. C. Parker, M. Judith Post

Research output: Contribution to journalArticle

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Abstract

An opportunity to study cerebrovascular changes in sickle cell anemia (SCA) presented itself when a black child with this disorder died of bihemispheric strokes. Angiography demonstrated severe occlusive vascular disease involving primarily the circle of Willis and major bifurcations of both internal carotid arteries. Collateral circulation to the distal branches of the internal carotid arteries occurred through transdural anastomoses from the external carotid system and via the leptomeningeal route. Perfusion of the basal ganglia was accomplished by vessels arising from the proximal internal carotid arteries. These changes resembled those of Moyamoya disease. Autopsy showed old and recent cerebral infarcts. Two vascular processes were responsible for the arterial occlusions: (1) exuberant intimal hyperplasia, and (2) old and recent thrombi with partial recanalization. The former has been described only once before in SCA. Small vessels in the basal ganglia were exceptionally numerous and dilated. We conclude that intimal hyperplasia within large cerebral arteries may be responsible for infarction and small vessel proliferation in basal ganglia in patients with SCA.

Original languageEnglish
Pages (from-to)45-52
Number of pages8
JournalStroke
Volume9
Issue number1
StatePublished - Jan 1 1978

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Cerebrovascular Disorders
Sickle Cell Anemia
Internal Carotid Artery
Basal Ganglia
Tunica Intima
Hyperplasia
Circle of Willis
Moyamoya Disease
Collateral Circulation
Cerebral Arteries
Vascular Diseases
Infarction
Blood Vessels
Autopsy
Angiography
Thrombosis
Perfusion
Stroke

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Neuroscience(all)

Cite this

Merkel, K. H. H., Ginsberg, P. L., Parker, J. C., & Judith Post, M. (1978). Cerebrovascular disease in sickle cell anemia: A clinical, pathological and radiological correlation. Stroke, 9(1), 45-52.

Cerebrovascular disease in sickle cell anemia : A clinical, pathological and radiological correlation. / Merkel, K. H H; Ginsberg, P. L.; Parker, J. C.; Judith Post, M.

In: Stroke, Vol. 9, No. 1, 01.01.1978, p. 45-52.

Research output: Contribution to journalArticle

Merkel, KHH, Ginsberg, PL, Parker, JC & Judith Post, M 1978, 'Cerebrovascular disease in sickle cell anemia: A clinical, pathological and radiological correlation', Stroke, vol. 9, no. 1, pp. 45-52.
Merkel KHH, Ginsberg PL, Parker JC, Judith Post M. Cerebrovascular disease in sickle cell anemia: A clinical, pathological and radiological correlation. Stroke. 1978 Jan 1;9(1):45-52.
Merkel, K. H H ; Ginsberg, P. L. ; Parker, J. C. ; Judith Post, M. / Cerebrovascular disease in sickle cell anemia : A clinical, pathological and radiological correlation. In: Stroke. 1978 ; Vol. 9, No. 1. pp. 45-52.
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