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Posterior Reversible Encephalopathy Syndrome with a Distinct Radiological Pattern Related to Systemic Lupus Erythematosus—a Case Report and Short Review of Literature

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Abstract

Posterior reversible encephalopathy syndrome (PRES) represents an acute neurological complication, characterized by diverse symptoms (headache, visual disturbances, altered mental state, seizures, or other focal deficits) that usually relate to uncontrolled arterial hypertension (HT), eclampsia, sepsis, renal disease, autoimmune disorders, or cytotoxic medication. The accurate diagnosis relies on radiological features, commonly displaying parieto-occipital vasogenic edema. We report a particular case of PRES involving the brainstem and capsular-thalamic regions in a patient with systemic lupus erythematosus (SLE). A 25-year-old female, with a 5-year history of SLE with several manifestations (HT, seizures, lupus nephritis, pericarditis), was admitted for sudden onset of impaired consciousness and double vision. She was on maintenance immunosuppression, hydroxychloroquine, and antihypertensive and antiepileptic medication, denying any illicit drug intake or recent infections. The clinical exam revealed elevated blood pressure, altered mental state, left hemiparesis, bilateral pyramidal signs, ataxia, strabismus, and left facial palsy, with no meningeal signs or fever. The initial brain CT scan showed diffuse hypodense aspect of the pons, mesencephalon, and capsular-thalamic regions, confirmed by the MRI exam, displaying extensive hyper-T2-weighted lesions in the same areas, associating petechial hemorrhages and restricted diffusion. The laboratory work-up excluded infectious encephalitis, vasculitis, or other metabolic complications, and the favorable clinical-radiological remission confirmed the diagnosis of PRES. We conducted a literature review regarding PRES in SLE, with special focus on imagistic presentation and major complications. PRES may complicate the evolution of SLE associated with HT, renal dysfunction, or immunosuppression. Neurologists and radiologists should be aware of atypical imagistic patterns and hemorrhagic complications in PRES, as timely recognition is essential.

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Data Availability

Patient’s clinical information and medical history were obtained from the Nephrology Department - “Fundeni Clinical Institute,” Bucharest, Romania. The scientific information and recent data were gathered from articles and publications after a thorough search in the main online databases (e.g., PubMed).

Abbreviations

ADC:

apparent diffusion coefficient

BBB:

blood-brain barrier

BP:

blood pressure

CBF:

cerebral blood flow

CMB:

cerebral microbleeds

CNS:

central nervous system

CSF:

cerebrospinal fluid

CT-scan:

computed tomography scan

HT:

arterial hypertension

IL:

interleukin

MRA:

magnetic resonance angiography

MRI:

magnetic resonance imaging

MRS:

magnetic resonance spectroscopy

PRES:

posterior reversible encephalopathy syndrome

RCVS:

reversible cerebral vasoconstriction syndrome

SLE:

systemic lupus erythematous

SLEDAI:

systemic lupus erythematous disease activity index

SWI:

susceptibility weighted imaging

TNF:

tumor necrosis factor

VEGF:

vascular endothelial growth factor

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Author information

Authors and Affiliations

  1. Neurology Clinic, Fundeni Clinical Institute, Sos. Fundeni 258, 022328, Bucharest, Romania

    Adriana O. Dulămea, Oana Obrișcă & Ioan C. Lupescu

  2. University of Medicine and Pharmacy “Carol Davila”, Str. Dionisie Lupu 37, 020021, Bucharest, Romania

    Adriana O. Dulămea, Ioana G. Lupescu, Ileana Constantinescu & Gener Ismail

  3. Radiology Clinic, Fundeni Clinical Institute, Sos Fundeni 258, 022328, Bucharest, Romania

    Ioana G. Lupescu

  4. Center of Immunogenetics and Virology Fundeni, Sos Fundeni 258, 022328, Bucharest, Romania

    Ileana Constantinescu

  5. Nephrology Clinic, Fundeni Clinical Institute, Sos Fundeni 258, 022328, Bucharest, Romania

    Gener Ismail

Authors
  1. Adriana O. Dulămea
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  2. Oana Obrișcă
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  3. Ioana G. Lupescu
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  4. Ileana Constantinescu
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  5. Ioan C. Lupescu
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  6. Gener Ismail
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Contributions

Adriana O. Dulamea together with Gener Ismail have successfully managed the patient during her entire disease history. Ioana G. Lupescu was responsible for the entire radiological exams. Ileana Constantinescu contributed with the extensive laboratory work-up. Oana Obrisca collected the clinical data and was in charge with the detailed literature research and with the first draft of the article. All the authors revised the materials and contributed to the final manuscript.

Corresponding author

Correspondence to Oana Obrișcă.

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Written informed consent was obtained from the patient, so that all her medical data could be used for scientific purposes, including publication.

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The authors declare no competing interests.

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Dulămea, A.O., Obrișcă, O., Lupescu, I.G. et al. Posterior Reversible Encephalopathy Syndrome with a Distinct Radiological Pattern Related to Systemic Lupus Erythematosus—a Case Report and Short Review of Literature. SN Compr. Clin. Med. 3, 2269–2277 (2021). https://doi.org/10.1007/s42399-021-01027-7

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