Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

Abstract

Background and purpose Thrombotic microangiopathy (TMA) is a group of microvascular occlusive disorders that presents with neurological involvement in up to 87% of the cases. Although the central nervous system (CNS) is an important target organ in TMA, the role of neurological manifestations in the disease clinical course is not well established. In this study, we described the neurological manifestations and CNS radiological aspects in patients with a first, acute TMA event. We also examined the association between severe neurological involvement and adverse clinical outcomes in TMA. Methods A cohort of patients diagnosed with a first TMA event between 1995 and 2016 was included, their medical charts and imaging tests were retrospectively evaluated. Results A total of 49 patients were included, 85.7% were women and the mean age was 36.5 years-old (SD 13.0). Neurological manifestations were described in 85.7% of the patients, most of them (88%) were considered severe and consisted of confusion, compromised sensorimotor function, stupor, seizures, and personality change. Imaging tests were performed in 62% of the patients with neurological manifestations and detected acute CNS lesions, such as posterior reversible encephalopathy syndrome, hemorrhagic and ischemic stroke were observed, in 7 (27%) of them. While the need for intensive care unit admission was greater and longer among patients with severe neurological manifestations, the number of plasma exchange sessions, the total duration of hospitalization and in-hospital death were similar between groups. Conclusions Severe neurological manifestations are common in first TMA events and are responsible for a worse disease presentation at admission. While the effect of neurological manifestations on acute TMA clinical course seems to be modest, these manifestations may have an important impact on the development of chronic cognitive impairment, which highlights the need for proper diagnosis and treatment.

Predictors of acute ischemic cerebral lesions in immune-mediated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

“…The single PRES case in this study for both iTTP and HUS also contrasts with the high frequency of PRES repeatedly observed previous studies [16][17] . The frequency of confusion, seizures, stupor or coma was also lower than that observed in the largest series of TMA 18 . Recruitment bias, differences in the management and treatment, the wider inclusion criteria as well as the of measures of ADAMTS13 activity for categorizing patients in some studies, might explain this discrepancy.…”

Serum Level of Brain-Derived Neurotrophic Factor and Thrombotic Type Are Predictive of Cognitive Impairment in the Acute Period of Ischemic Strokes Patients

“…It is also known about the prognostic importance of subtypes of ischemic stroke in terms of patient recovery [ 28 ]. Atherothrombotic, thrombotic subtype, or large artery atherosclerosis is associated with cerebral angiopathy and recurrent transient ischemic attacks, which can lead to cognitive decline even before the development of a stroke and thus contribute to the risk of cognitive deterioration in the poststroke period [ 29 , 30 ].…”

Neuroimaging of COVID-19

“…43,57 On brain imaging, multifocal smallvessel cortical and subcortical infarcts and hemorrhages as well as posterior reversible encephalopathy syndrome (PRES) are the most common findings, but rarely large-vessel infarcts or large intraparenchymal hematomas can occur. [59][60][61] COVIDÀ19 patients suffering small-vessel infarcts, microhemorrhages, and subarachnoid hemorrhages have also been found to have evidence of vasculitis. 54,62 Vesselwall imaging findings of medium-and large-artery vessel wall thickening and concentric enhancement in the middle cerebral, basilar, and posterior cerebral arteries were noted in 11/69 (16%) patients in one series and 3 patients in another report, consistent with reported findings of vasculitis.…”