{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T03:15:05Z","timestamp":1787022905606,"version":"build-2736575974"},"reference-count":28,"publisher":"American Academy of Pediatrics (AAP)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,9,1]]},"abstract":"<jats:p>A 14-year-old boy presented to our institution with a 1-month history of neurocognitive decline and intermittent fevers. His history was significant for fevers, headaches, and a 10-lb weight loss. Previous examinations by multiple medical providers were significant only for bilateral cervical lymphadenopathy. Previous laboratory workup revealed leukopenia, neutropenia, and elevated inflammatory markers. Despite improvement in his laboratory values after his initial presentation, his fevers persisted, and he developed slowed and \u201cjerky\u201d movements, increased sleep, slurred speech, delusions, visual hallucinations, and deterioration in his school performance. A brain MRI performed at an outside hospital before admission at our institution was concerning for patchy, increased T2 and fluid-attenuated inversion recovery signal intensity in multiple areas, including the basal ganglia. After transfer to our institution and admission to the pediatric hospital medicine team, the patient had an acute decompensation. Our subspecialists will discuss the initial evaluation, workup, differential diagnosis, definitive diagnosis, and subsequent management of this patient.<\/jats:p>","DOI":"10.1542\/peds.2017-3258","type":"journal-article","created":{"date-parts":[[2018,8,2]],"date-time":"2018-08-02T03:25:24Z","timestamp":1533180324000},"source":"Crossref","is-referenced-by-count":1,"title":["A 14-Year-Old Boy With Fevers, Cytopenias, and Neurocognitive Decline"],"prefix":"10.1542","volume":"142","author":[{"given":"William B.","family":"Lapin","sequence":"first","affiliation":[{"name":"Sections of Allergy, Immunology, and Rheumatology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariel M.","family":"Lyons-Warren","sequence":"additional","affiliation":[{"name":"Neurology and Developmental Neuroscience, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah R.","family":"Risen","sequence":"additional","affiliation":[{"name":"Neurology and Developmental Neuroscience, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nisha","family":"Rathore","sequence":"additional","affiliation":[{"name":"Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine and Texas Children\u2019s Hospital, Houston, Texas; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy S.","family":"Slone","sequence":"additional","affiliation":[{"name":"Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine and Texas Children\u2019s Hospital, Houston, Texas; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Tarek","family":"Elghetany","sequence":"additional","affiliation":[{"name":"Departments of Pathology and Immunology, and"},{"name":"Pediatrics, Baylor College of Medicine, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"Marcus","sequence":"additional","affiliation":[{"name":"Sections of Allergy, Immunology, and Rheumatology,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2018,9,1]]},"reference":[{"issue":"2","key":"2021120916442056100_B1","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1017\/S0317167100053828","article-title":"Neurological manifestations of West Nile virus infection.","volume":"31","author":"Burton","year":"2004","journal-title":"Can J Neurol Sci"},{"issue":"1","key":"2021120916442056100_B2","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1186\/s12879-016-1985-1","article-title":"Mycoplasma pneumoniae-associated mild encephalitis\/encephalopathy with a reversible splenial lesion: report of two pediatric cases and a comprehensive literature review.","volume":"16","author":"Ueda","year":"2016","journal-title":"BMC Infect Dis"},{"issue":"10","key":"2021120916442056100_B3","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1007\/s00234-015-1568-7","article-title":"Bilateral symmetrical basal ganglia and thalamic lesions in children: an update (2015).","volume":"57","author":"Zuccoli","year":"2015","journal-title":"Neuroradiology"},{"issue":"5","key":"2021120916442056100_B4","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1097\/RMR.0b013e3182972c03","article-title":"Idiopathic inflammatory demyelinating disorders of the central nervous system in children.","volume":"22","author":"da Rocha","year":"2011","journal-title":"Top Magn Reson Imaging"},{"issue":"6","key":"2021120916442056100_B5","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1191\/096120300678828578","article-title":"Parkinsonism as unusual neurological complication in childhood systemic lupus erythematosus.","volume":"9","author":"Kwong","year":"2000","journal-title":"Lupus"},{"issue":"7","key":"2021120916442056100_B6","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1016\/j.bbadis.2008.08.001","article-title":"Viral parkinsonism.","volume":"1792","author":"Jang","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"2021120916442056100_B7","doi-asserted-by":"crossref","first-page":"bcr2016215859","DOI":"10.1136\/bcr-2016-215859","article-title":"Dopa-responsive acute disseminated encephalomyelitis with marked atrophy of the striate body.","volume":"2016","author":"Goto","year":"2016","journal-title":"BMJ Case Rep"},{"key":"2021120916442056100_B8","doi-asserted-by":"crossref","first-page":"S82","DOI":"10.1016\/j.bbmt.2009.11.014","article-title":"Histiocytic disorders: recent insights into pathophysiology and practical guidelines.","volume":"16","author":"Filipovich","year":"2010","journal-title":"Biol Blood Marrow Transplant"},{"issue":"15","key":"2021120916442056100_B9","doi-asserted-by":"crossref","first-page":"4041","DOI":"10.1182\/blood-2011-03-278127","article-title":"How I treat hemophagocytic lymphohistiocytosis.","volume":"118","author":"Jordan","year":"2011","journal-title":"Blood"},{"issue":"3","key":"2021120916442056100_B10","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/j.pediatrneurol.2013.10.014","article-title":"Central nervous system involvement in hemophagocytic lymphohistiocytosis: a single-center experience.","volume":"50","author":"Jovanovic","year":"2014","journal-title":"Pediatr Neurol"},{"issue":"13","key":"2021120916442056100_B11","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1182\/blood-2013-10-535393","article-title":"Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations.","volume":"123","author":"Price","year":"2014","journal-title":"Blood"},{"issue":"1","key":"2021120916442056100_B12","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/s13311-015-0410-6","article-title":"Antibody-mediated autoimmune encephalopathies and immunotherapies.","volume":"13","author":"Gastaldi","year":"2016","journal-title":"Neurotherapeutics"},{"key":"2021120916442056100_B13","volume-title":"Textbook of Pediatric Rheumatology","author":"Petty","year":"2016","edition":"7th ed."},{"issue":"4","key":"2021120916442056100_B14","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1038\/gene.2012.3","article-title":"Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment.","volume":"13","author":"Ravelli","year":"2012","journal-title":"Genes Immun"},{"issue":"5","key":"2021120916442056100_B15","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/nrrheum.2015.179","article-title":"Macrophage activation syndrome in the era of biologic therapy.","volume":"12","author":"Grom","year":"2016","journal-title":"Nat Rev Rheumatol"},{"issue":"7","key":"2021120916442056100_B16","first-page":"1536","article-title":"The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus.","volume":"29","author":"Sibbitt","year":"2002","journal-title":"J Rheumatol"},{"issue":"10","key":"2021120916442056100_B17","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1177\/0961203306070990","article-title":"Neuropsychiatric manifestations in pediatric systemic lupus erythematosus: a 20-year study.","volume":"15","author":"Yu","year":"2006","journal-title":"Lupus"},{"issue":"9","key":"2021120916442056100_B18","doi-asserted-by":"crossref","first-page":"2989","DOI":"10.1002\/art.22048","article-title":"A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics\/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus.","volume":"54","author":"Guti\u00e9rrez-Su\u00e1rez","year":"2006","journal-title":"Arthritis Rheum"},{"issue":"6","key":"2021120916442056100_B19","doi-asserted-by":"crossref","first-page":"e153","DOI":"10.3928\/00904481-20150611-11","article-title":"Neuropsychiatric systemic lupus erythematosus in children.","volume":"44","author":"Soybilgic","year":"2015","journal-title":"Pediatr Ann"},{"issue":"8","key":"2021120916442056100_B20","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1177\/0961203307078971","article-title":"Neuropsychiatric involvement in pediatric systemic lupus erythematosus.","volume":"16","author":"Benseler","year":"2007","journal-title":"Lupus"},{"issue":"3","key":"2021120916442056100_B21","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1111\/1756-185X.12235","article-title":"Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases.","volume":"17","author":"Sato","year":"2014","journal-title":"Int J Rheum Dis"},{"issue":"10","key":"2021120916442056100_B22","doi-asserted-by":"crossref","first-page":"2449","DOI":"10.1007\/s10067-016-3376-9","article-title":"Evaluation of magnetic resonance imaging abnormalities in juvenile onset neuropsychiatric systemic lupus erythematosus.","volume":"35","author":"Al-Obaidi","year":"2016","journal-title":"Clin Rheumatol"},{"issue":"3","key":"2021120916442056100_B23","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1177\/0961203309352092","article-title":"Neurocognitive deficits and neuroimaging abnormalities are prevalent in children with lupus: clinical and research experiences at a US pediatric institution.","volume":"19","author":"Muscal","year":"2010","journal-title":"Lupus"},{"issue":"1","key":"2021120916442056100_B24","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1111\/nyas.12553","article-title":"Autoimmune encephalopathies.","volume":"1338","author":"Leypoldt","year":"2015","journal-title":"Ann N Y Acad Sci"},{"issue":"5, pt 1","key":"2021120916442056100_B25","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1111\/j.1600-6143.2006.01288.x","article-title":"Rituximab, an anti-cd20 monoclonal antibody: history and mechanism of action.","volume":"6","author":"Pescovitz","year":"2006","journal-title":"Am J Transplant"},{"issue":"2","key":"2021120916442056100_B26","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S1474-4422(12)70310-1","article-title":"Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study.","volume":"12","author":"Titulaer","year":"2013","journal-title":"Lancet Neurol"},{"issue":"11","key":"2021120916442056100_B27","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1002\/art.1780241109","article-title":"Central nervous system involvement in childhood systemic lupus erythematosus.","volume":"24","author":"Yancey","year":"1981","journal-title":"Arthritis Rheum"},{"issue":"3","key":"2021120916442056100_B28","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/0887-8994(95)00110-2","article-title":"Neurologic manifestations of pediatric systemic lupus erythematosus.","volume":"13","author":"Steinlin","year":"1995","journal-title":"Pediatr Neurol"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2017-3258\/1064981\/peds_20173258.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2017-3258\/1064981\/peds_20173258.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,9]],"date-time":"2021-12-09T23:52:09Z","timestamp":1639093929000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/142\/3\/e20173258\/38663\/A-14-Year-Old-Boy-With-Fevers-Cytopenias-and"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,1]]},"references-count":28,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2018,9,1]]},"published-print":{"date-parts":[[2018,9,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2017-3258","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,1]]},"article-number":"e20173258"}}