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Neurological manifestations of thrombotic microangiopathy syndromes in adult patients

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Abstract

The objective of this study was to compare the frequency and severity of neurologic manifestations in adult patients diagnosed with thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS) and atypical HUS (aHUS). This is a retrospective cohort study of adult patients diagnosed with TTP, HUS and atypical HUS hospitalized at a tertiary center between January 2004 and October 2016. A total of 42 TTP, 16 HUS and 20 aHUS episodes were reviewed to collect clinical, laboratory and radiographic data, as well as information regarding long-term functional outcome. Neurologic symptoms are more common in patients with TTP and HUS as compared to aHUS (p < 0.001 and p = 0.002, respectively). Encephalopathy occurred in 29 TTP (69%) and 11 HUS (68%) episodes. Focal deficits were only observed in patients with TTP (n = 8 [19%]). Seizures were most commonly seen in HUS patients (n = 8 [50%]). Posterior reversible encephalopathy syndrome (PRES) was the most common neuroimaging finding in these syndromes; ischemic infarcts and hemorrhages occurred uncommonly. There was no correlation between presence of neurologic symptoms or neuroimaging abnormalities and poor outcome. Patients with TTP and HUS appear to have a similar spectrum of neurologic manifestations, whereas neurologic involvement is less common in aHUS. PRES is the most common imaging abnormality, and may present atypically. Despite presence of neurologic symptoms or neuroimaging abnormalities, patients with thrombotic microangiopathy (TMA) tend to have favorable long-term outcomes.

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Anonymized data can be made available for review upon reasonable request from qualified investigators.

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This research received no specific funding.

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Contributions

All authors contributed to the study conception and design. Data collection, data analysis and manuscript preparation were performed by EW. Interpretation of data and critical revisions of the manuscript were performed by AR. All authors have approved the final manuscript.

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Correspondence to Alejandro A. Rabinstein.

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The authors have no conflict of interest to disclose, financial or otherwise.

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This study has been performed in accordance with ethical standards laid down in the 1964 Declaration of Helsinki.

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Written informed consent was waived due to the retrospective nature of the study. All data was anonymized.

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Weil, E.L., Rabinstein, A.A. Neurological manifestations of thrombotic microangiopathy syndromes in adult patients. J Thromb Thrombolysis 51, 1163–1169 (2021). https://doi.org/10.1007/s11239-021-02431-5

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