Literature DB >> 31666467

Striatal Encephalitis in Neuropsychiatric Systemic Lupus Erythematosus.

Keigo Nakamura1, Atsuhiko Sugiyama1, Kazumoto Shibuya1, Satoshi Kuwabara1.   

Abstract

Entities:  

Keywords:  NPSLE; neuropsychiatric systemic lupus erythematosus; striatal encephalitis

Year:  2019        PMID: 31666467      PMCID: PMC7056380          DOI: 10.2169/internalmedicine.3693-19

Source DB:  PubMed          Journal:  Intern Med        ISSN: 0918-2918            Impact factor:   1.271


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A 53-year-old man with a 1-month history of arthralgia suffered acute conscious disturbance. He exhibited lethargy, bradykinesia, and rigidity in the four extremities on admission. Based on the presence of lymphocytopenia, hypocomplementemia, and antinuclear and anti-Sm antibodies positivity, he was diagnosed with neuropsychiatric systemic lupus erythematosus (SLE) (1). Fundoscopy revealed cotton-like white spots and retinal hemorrhage, which were consistent with the diagnosis of SLE. T2-weighted magnetic resonance imaging (MRI) of the brain demonstrated bilateral symmetric hyperintensity (Picture 1A). Fluid-attenuated inversion recovery (FLAIR) MRI of the brain (Picture 1B) revealed hyperintensity of the basal ganglia and thalami without postcontrast enhancement (Picture 1C) or restricted diffusion (Picture 1D, E). This characteristic imaging pattern suggested antibody-mediated autoimmune striatal encephalitis, including neuropsychiatric SLE and anti-N-methyl-D-aspartate receptor striatal encephalitis, as well as viral encephalitis [especially flaviviruses (e.g., Japanese, West Nile, etc.)] as a differential diagnosis (2). Treatment with high-dose pulsed intravenous methylprednisolone and intravenous cyclophosphamide resulted in the reduction of the high-intensity areas on T2-weighted MRI (Picture 2A) and FLAIR MRI (Picture 2B).
Picture 1.
Picture 2.

The authors state that they have no Conflict of Interest (COI).
  2 in total

1.  Neuropsychiatric Lupus with Antibody-Mediated Striatal Encephalitis.

Authors:  B P Kelley; J J Corrigan; S C Patel; B D Griffith
Journal:  AJNR Am J Neuroradiol       Date:  2018-11-22       Impact factor: 3.825

2.  Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.

Authors:  Michelle Petri; Ana-Maria Orbai; Graciela S Alarcón; Caroline Gordon; Joan T Merrill; Paul R Fortin; Ian N Bruce; David Isenberg; Daniel J Wallace; Ola Nived; Gunnar Sturfelt; Rosalind Ramsey-Goldman; Sang-Cheol Bae; John G Hanly; Jorge Sánchez-Guerrero; Ann Clarke; Cynthia Aranow; Susan Manzi; Murray Urowitz; Dafna Gladman; Kenneth Kalunian; Melissa Costner; Victoria P Werth; Asad Zoma; Sasha Bernatsky; Guillermo Ruiz-Irastorza; Munther A Khamashta; Soren Jacobsen; Jill P Buyon; Peter Maddison; Mary Anne Dooley; Ronald F van Vollenhoven; Ellen Ginzler; Thomas Stoll; Christine Peschken; Joseph L Jorizzo; Jeffrey P Callen; S Sam Lim; Barri J Fessler; Murat Inanc; Diane L Kamen; Anisur Rahman; Kristjan Steinsson; Andrew G Franks; Lisa Sigler; Suhail Hameed; Hong Fang; Ngoc Pham; Robin Brey; Michael H Weisman; Gerald McGwin; Laurence S Magder
Journal:  Arthritis Rheum       Date:  2012-08
  2 in total

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