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Cited by 216Hemoglobinopathies and Related DisordersMoyamoya disease diagnosis and treatmentAcute Ischemic Stroke Management

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

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Merkel, K H, Ginsberg, P L, Parker, J C, Post, M J·1978·Stroke
DOI 10.1161/01.str.9.1.45
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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 prliferation in basal ganglia in patients with SCA.

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  • Autopsy review of patients with Moyamoya syndrome revealed that all three layers of the large intracerebral arteries were affected, with intimal hyperplasia, medial atrophy with fibrosis, and adventitial fibrosis.

    “Autopsy review of patients with Moyamoya syndrome revealed that all three layers of the large intracerebral arteries were affected, with intimal hyperplasia, medial atrophy with fibrosis, and adventitial fibrosis (135).”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [135]

    The Discussion (page not provided) reviews postmortem reports of Moyamoya disease and lists the claimed changes in all three arterial layers. It says those layers “may be involved,” however, rather than establishing that all three were affected in every patient reviewed. (LLM, conf=0.84)

    Experimental — accuracy not yet measured

  • Pediatric sickle cell disease stroke lesions include intimal hyperplasia that obliterates the lumen.

    “Lesions include intimal hyperplasia leading to obliteration of the lumen”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [135]

  • Pediatric sickle cell disease stroke lesions include intraluminal thrombosis.

    “and intraluminal thrombosis (134, 135).”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [135]

  • In pediatric sickle cell disease stroke, the large and medium-sized branches of the internal carotid artery are affected.

    “These studies have shown that the large and medium-sized branches of the internal carotid artery are affected.”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [135]

  • Pediatric sickle cell disease stroke lesions include degeneration of the internal elastic lamina.

    “degeneration of the internal elastic lamina”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [135]

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Authors
Merkel, K. H., Ginsberg, P. L., Parker, J. C., & Post, M. J.
ORCID
Post, M J
Venue
Stroke
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
1978-01
Volume
9
Issue
1
Pages
45-52
ISSN
  • 0039-2499 (ISSN-L)
  • 1524-4628
DOI
10.1161/01.str.9.1.45
OpenAlex
W2142915976
Cited by
216
References
48
Citations by year
2026: 2 · 2025: 5 · 2024: 5 · 2023: 3 · 2022: 7 · 2021: 6 · 2020: 5 · 2019: 2 · 2018: 6 · 2017: 6 · 2016: 3 · 2015: 11 · 2014: 7 · 2013: 11 · 2012: 6
FWCI
2.03
Type
journal article
Language
en
Open access
Locations
  • Stroke (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
  • Health Sciences › Medicine › Rheumatology › Moyamoya disease diagnosis and treatment
  • Health Sciences › Medicine › Epidemiology › Acute Ischemic Stroke Management
Keywords
sickle cell disease, cerebrovascular disease, intimal hyperplasia, cerebral infarction, basal ganglia, moyamoya disease, circle of Willis, internal carotid artery occlusion, collateral circulation, cerebral angiography, cerebral perfusion, thrombus recanalization
MeSH terms
Anemia, Sickle Cell, Atrophy, Carotid Arteries, Carotid Artery Thrombosis, Carotid Artery, Internal, Cerebral Arteries, Cerebral Cortex, Intracranial Embolism and Thrombosis, Cerebrovascular Disorders, Child, Fibromuscular Dysplasia, Humans, Male, Moyamoya Disease, Radiography

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