Silent cerebral infarct in sickle cell anemia patients of southern Turkey

Background/aim: Silent cerebral infarct (SCI) is an ischemic lesion seen before clinical signs of brain infarct and ischemic changes in brain tissue. This study aimed to detect SCI with noninvasive methods and to determine related risk factors in patients with sickle cell anemia (SCA). Materials and methods: Fifty-four SCA patients who had no history of cerebral infarct and whose neurological examinations were normal were included in this study. Brain magnetic resonance imaging (MRI) and diffusion MRI were taken and the acquired data was compared statistically. Results: SCI was detected in 11.1% (6/54) of the patients. No statistical differences in age, sex, physical examination findings, or treatments were detected between the 2 groups (with and without SCI). When examined in terms of HbS, the median (min­max) value in SCI-positive patients was 85.4 (80.5­92.1); the median value was 77.2 (49.0­96.7) in SCI-negative patients. The HbS values of the SCI group were statistically significantly higher than those of the group without SCI (P = 0.014). Patients with the HbSS or HbSß0 genotypes had a significantly higher prevalence of SCI when compared with other sickle cell syndromes (P = 0.038). Conclusion: SCI is not uncommon among SCA patients in Turkey. The presence of homozygote HbSS/Sß0 genotype, high MCV, and HbS are risk factors for SCI.

Are the Comments on HaNDL Syndrome in the ICHD-II Sufficient?

A 33-year-old man was admitted to our emergency department for severe frontal headache followed by a state of consciousness disturbance and right-sided hemiparesis. No previous febrile disease, head trauma, vascular risk factor, and medication for any systemic disease were defined in his history. He had experienced a similar disorder three years ago and had recovered completely. Cerebrospinal fluid (CSF) analysis revealed pleocytosis and electroencephalography (EEG) showed diffuse slow wave activity. Hyperintense foci on T2 and FLAIR sequences representing bilateral cortical ischemia, prominent on the right hemisphere, were seen on MRI. Contrast-enhanced T1 images showed marked leptomeningeal thickening with enhancement. The patient was considered as having CSF lymphocytosis (HaNDL syndrome) due to temporary headache with neurologic deficit and CSF pleocytosis. Diagnostic criteria have been identified for this syndrome according to the International Classification of Headache Disorders, 2nd edition (ICHD-II). According to these criteria, neuroimaging should be normal. Positive neuroimaging findings and impairment of consciousness have been reported in a limited number of HaNDL cases so far. Diversity of neurological signs, duration and distinctness from migraine headache have been described in comment section under the diagnostic criteria. Comments are inadequate in this regard.