Brain MRI screening showing evidences of early central nervous system involvement in patients with systemic sclerosis

Abstract

Systemic sclerosis is a multisystem autoimmune collagen disease where structural and functional abnormalities of small blood vessels prevail. Transient ischemic attacks, ischemic stroke, and hemorrhage have been reported as primary consequence of vascular central nervous system affection in systemic sclerosis. Magnetic resonance imaging is considered to be the most sensitive diagnostic technique for detecting symptomatic and asymptomatic lesions in the brain in cases of multifocal diseases. The objective of this study is to detect subclinical as well as clinically manifest cerebral vasculopathy in patients with systemic sclerosis using magnetic resonance imaging. As much as 30 female patients with systemic sclerosis aged 27-61 years old, with disease duration of 1-9 years and with no history of other systemic disease or cerebrovascular accidents, were enrolled. Age-matched female control group of 30 clinically normal subjects, underwent brain magnetic resonance examination. Central nervous system (CNS) involvement in the form of white matter hyperintense foci of variable sizes were found in significantly abundant forms in systemic sclerosis patients on magnetic resonance evaluation than in age-related control group, signifying a form of CNS vasculopathy. Such foci showed significant correlation to clinical features of organic CNS lesion including headaches, fainting attacks and organic depression as well as to the severity of peripheral vascular disease with insignificant correlation with disease duration. In conclusion, subclinical as well as clinically manifest CNS ischemic vasculopathy is not uncommon in systemic sclerosis patients and magnetic resonance imaging is considered a sensitive noninvasive screening tool for early detection of CNS involvement in patients with systemic sclerosis.

Study of MRI brain findings and carotid US features in systemic sclerosis patients, relationship with disease parameters

“…There was a statistically significant difference between the SSc patients and controls for multiple laboratory parameters such as the CRP, ESR, lipid profile, ECG abnormalities, right and left CCA intima-media thickness, and lesions detected by brain FLAIR-MRI, similar to previous studies [35–37]. These differences could be explained as a result of widespread vasculopathy and extracellular matrix deposition with fibrosis and autoimmune processes as part of the characteristic pathogenesis of SSc.…”

A case of anti‐N‐methyl‐D‐aspartate receptor encephalitis with systemic sclerosis

“…Not only stroke events such as transient ischemic attacks, ischemic stroke, and hemorrhage but neuropsychiatric manifestations such as loss of memory, disorientation, depression, hallucinations, and reduced mental acuity have also been described. Whether symptomatic or not, MRI revealed CNS involvement in the form of white matter hyperintensities mostly because of ischemic vasculopathy which was significantly more common in patients with SSc than in control groups [3]. In this case, abnormalities in basal ganglia and thalamus were detected by brain MRI, which corresponded well with the clinical observations.…”

“…There is no report that antibodies against NR1–NR2 heteromers of the NMDAR were present in patients with SSc. Recent reports support the idea that central nervous system (CNS) involvement in SSc is not uncommon [3,4]. Not only stroke events such as transient ischemic attacks, ischemic stroke, and hemorrhage but neuropsychiatric manifestations such as loss of memory, disorientation, depression, hallucinations, and reduced mental acuity have also been described.…”

X chromosome gene methylation in peripheral lymphocytes from monozygotic twins discordant for scleroderma

“…While we can only speculate on the putative mechanisms justifying this association, we are traced back to earlier reports of the co‐existence of phenylketonuria‐associated mental retardation and scleroderma [58]. Further, there are data suggesting that patients with SSc may have disturbances of the nervous system as represented by an impaired response to stress [59], the detection of central nervous system ischaemic vasculopathy at magnetic resonance imaging [60,61] and the changes in cerebrovascular reactivity [62].…”