Abnormal Neurologic Findings in Patients With Sickle Cell Disease Without a History of Major Neurologic Events

Background and Objectives Patients with sickle cell disease (SCD) are prone to symptomatic neurologic complications. Previous studies reported accrual of neural injury starting at early age, even without having symptomatic neurologic events. The aim of this study was to assess the prevalence and risk factors of abnormal neurologic findings in patients with SCD with no history of major symptomatic neurologic events.

Methods Our study extracted patients diagnosed with SCD from the Cooperative Study of Sickle Cell Disease. Patients who underwent a neurologic evaluation were included in our analysis. Patients with previous documented major symptomatic neurologic events were excluded. We compared patients with SCD with abnormal neurologic findings with those without in terms of clinical and laboratory parameters using multivariate binary logistic regression.

Results A total of 3,573 patients with SCD were included (median age = 11 [IQR = 19] years, male = 1719 [48.1%]). 519 (14.5%) patients had at least one abnormal neurologic finding. The most common findings in descending order were abnormal reflexes, gait abnormalities, cerebellar dysfunction, language deficits, nystagmus, abnormal muscle tone and strength, Romberg sign, Horner syndrome, and intellectual impairment. History of eye disease (odds ratio [OR] = 2.76, 95% confidence interval [CI] = 1.63–4.68) and history of osteomyelitis (OR = 2.55, 95% CI 1.34–4.84) were the strongest predictors of abnormal neurologic findings, followed by smoking (OR = 1.59, 95% CI 1.08–2.33), aseptic necrosis (OR = 1.57, 95% CI 1.06–2.33), hand-foot syndrome (OR = 1.48, 95% CI 1.04–2.12), and male sex (OR = 1.42, 95% CI 1.01–2.02).

Discussion Neurologic deficits are relatively common in patients with SCD, even without documented major neurologic insults. They range from peripheral and ophthalmic deficits to central and cognitive disabilities. Patients with SCD should have early regular neurologic evaluations and risk factor modification, particularly actively promoting smoking cessation.