Nazan Şimşek Erdem1, Ramazan Erdem2, Erdal Kurtoglu2, Gönül Oktay3

1Akdeniz University Hospital, Clinic of Neurology, Antalya, Turkey
2Antalya Training and Research Hospital, Clinic of Hematology, Antalya, Turkey
3Hatay State Hospital, Clinic of Thalassemia Center, Hatay, Turkey

Keywords: Neurocognitive dysfunction, adults with sickle cell anemia, Montreal Cognitive Assessment test, neurologic complications, Turkish population

Abstract

Objective: To screen cognitive functions using the Montreal Cognitive Assessment (MoCA) test and to determine the most common central nervous system complications in adults with sickle cell anemia (SCA).

Materials and Methods: One hundred adult patients with SCA and 82 healthy controls participated in this study. Controls were matched for age, sex, and education level. We reviewed the demographic information and laboratory values of all patients. The patients were questioned about common CNS complications including headache, ischemic or hemorrhagic stroke, epilepsy, and cerebral venous sinus thrombosis. The MoCA test was used to assess neurocognitive function in all participants.

Results: Of the 100 patients with SCA, 38 patients had chronic or recurrent headaches, 10 had a history of depression, and four patients had a history of ischemic stroke. None of the patients had a history of epilepsy, hemorrhagic stroke or cerebral venous sinus thrombosis. The median MoCA score of the patients was significantly decreased compared with that of the control group (p<0.001). MoCA scores below 21 points were observed in 50% of the patients. The MoCA scores were negatively correlated with age but positively correlated with education level (r=-0.181 p=0.015, r=0.483, p<0.001 respectively). There was a significant correlation between a history of chronic or recurrent headaches and lower MoCA (p=0.003).

Conclusion: Cognitive impairment was the most prevalent neurologic symptom in Turkish adult patients with SCA. The MoCA test may be a useful and easy screening test to evaluate and follow cognitive impairment. A history of first ischemic stroke during adulthood was observed in one patient. Two patients had severe neurologic sequela findings due to ischemic stroke.

Ethics Committee Approval

This retrospective crosssectional study was allowed by the University of Health Sciences Turkey, Antalya Training and Research Hospital Ethics Committee (approval number: 2019-12/16).

Peer Review

Externally peer-reviewed.

Author Contributions

Concept: N.Ş.E., R.E., G.O., E.K., Design: N.Ş.E., R.E., G.O., E.K., Data Collection or Processing: N.Ş.E., R.E., G.O., E.K., Analysis or Interpretation: N.Ş.E., R.E., G.O., E.K., Literature Search: N.Ş.E., R.E., G.O., E.K., Writing: N.Ş.E., R.E., G.O., E.K.

Conflict of Interest

No conflict of interest was declared by the authors.

Financial Disclosure

The authors declared that this study received no financial support.