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Neurological manifestations of thrombotic microangiopathy syndromes in adult patients.
J Thromb Thrombolysis. 2021 May; 51(4):1163-1169.JT

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.

Authors+Show Affiliations

Department of Neurology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA.Department of Neurology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA. Rabinstein.Alejandro@mayo.edu.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

33755882

Citation

Weil, Erika L., and Alejandro A. Rabinstein. "Neurological Manifestations of Thrombotic Microangiopathy Syndromes in Adult Patients." Journal of Thrombosis and Thrombolysis, vol. 51, no. 4, 2021, pp. 1163-1169.
Weil EL, Rabinstein AA. Neurological manifestations of thrombotic microangiopathy syndromes in adult patients. J Thromb Thrombolysis. 2021;51(4):1163-1169.
Weil, E. L., & Rabinstein, A. A. (2021). Neurological manifestations of thrombotic microangiopathy syndromes in adult patients. Journal of Thrombosis and Thrombolysis, 51(4), 1163-1169. https://doi.org/10.1007/s11239-021-02431-5
Weil EL, Rabinstein AA. Neurological Manifestations of Thrombotic Microangiopathy Syndromes in Adult Patients. J Thromb Thrombolysis. 2021;51(4):1163-1169. PubMed PMID: 33755882.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neurological manifestations of thrombotic microangiopathy syndromes in adult patients. AU - Weil,Erika L, AU - Rabinstein,Alejandro A, Y1 - 2021/03/23/ PY - 2021/03/13/accepted PY - 2021/3/24/pubmed PY - 2021/3/24/medline PY - 2021/3/23/entrez KW - Encephalopathy KW - Hemolytic uremic syndrome KW - Posterior reversible encephalopathy syndrome (PRES) KW - Seizures KW - Thrombotic thrombocytopenic purpura SP - 1163 EP - 1169 JF - Journal of thrombosis and thrombolysis JO - J Thromb Thrombolysis VL - 51 IS - 4 N2 - 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. SN - 1573-742X UR - https://www.unboundmedicine.com/medline/citation/33755882/Neurological_manifestations_of_thrombotic_microangiopathy_syndromes_in_adult_patients. L2 - https://doi.org/10.1007/s11239-021-02431-5 DB - PRIME DP - Unbound Medicine ER -
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