Atypical Stroke Linked To Half Of Acute Ischemic Neurological Events in SCD Children


Data show 12 of 25 strokes (48%) in a cohort of children did not met the definition of typical stroke related to sickle cell cerebral vasculopathy.

Although typical cerebral vasculopathy in sickle cell disease is a stenosis of ≥1 intracranial or cervical arteries, there have been previous data reporting on the occurrence of stroke in children with sickle cell disease in the absence of typical cerebral vasculopathy.

Accordingly, a team of investigators led by Sarah Liane Linguet, MD, Sickle Cell Disease Center, Hematology Unit, Robert Debré Hospital explored the prevalence of stroke without cerebral vasculopathy in children with sickle cell disease.

They found atypical strokes account for nearly 50% of all acute ischemic neurological events, related to hyperviscosity, RVCS, or inflammatory vasculitis in a cohort of children with sickle cell disease with efficient stroke prevention programs.

The study was presented at the 2021 American Society of Hematology (ASH) Annual Meeting & Exposition.


The study consisted of a large cohort of nearly 1500 children with sickle cell disease in Paris, France. Investigators recorded all strokes occurring between 2007 - 2020, with exclusions in venous thrombosis and PRESS Syndrome.

Children were then followed in a university hospital, with the study noting that all benefited from early screening for cerebral vasculopathy and an adapted stroke prevention program.

A typical stroke was considered as any new ischemic lesion of the cerebral parenchyma associated with an acute neurological syndrome occurring in the territory of a pre-existing and/or non-regressive stenosis.


During the study period, a total of 25 strokes occurred, with 12 (48%) not meeting the definition of a typical stroke related to sickle cell cerebral vasculopathy.

Children who had atypical strokes were shown to be generally older (9 years old ± 4.6 versus 6.5 years old ± 4.3 in the typical group, P = .0086) and less frequently of sickle cell genotype (33% non-sickle cell vs 8% non-sickle cell in the typical group).

Additionally, they had a lower leukocyte count (11.3 G/L ± 4.6 versus 15.7 G/L ± 2.3 in the typical group, P = .04) and higher hemoglobin level at the time of the stroke (11 g/dL ± 3 versus 7.4 g/dL ± 1.3 in the typical group, P = .027).

Data show 17% of atypical strokes had posterior ischemic lesions, 33% had anterior lesions, and 17% had multiple systematized lesions, in counting junctional lesions, while investigators also found 33% of ischemic lesions of the cerebellum.

Then, when considering a potential trigger of the stroke, 58% of atypical events were hospitalized in an anesthesia or intensive care unit at the time of stroke or less than 7 days before, compared to 8% of children with a classic stroke (P = .011).

Another point of note showed the etiologies adopted by clinicians and radiologists for the atypical stroke were reversible cerebral vasoconstriction syndrome (RCVS), cerebral fat embolism, hyperviscosity and vasculitis in systemic inflammatory context.

In the atypical group, the evolution was more favorable in terms of recurrence (0% within 2 years versus 42% in the typical group, P = .045). However, only 33% (n = 3) of atypical strokes were under an exchange transfusion program 24 months after the stroke versus 92% in the group of typical stroke.


In summary, the investigators highlighted the importance of physicians being aware of potential triggers and the context in which these events occur.

“Nevertheless, stroke without cerebral vasculopathy may not require long-term transfusion program contrary to stroke with cerebral vasculopathy, given the very low risk of recurrence we highlighted,” Linguet and colleagues wrote.

Stroke without Cerebral Vasculopathy in Sickle Cell Disease Children: Which Causes? Which Treatment?,” was presented online by ASH.

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