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Case Reports
. 2020 Dec 16;55(4):449-452.
doi: 10.14744/TurkPediatriArs.2020.26576. eCollection 2020.

Autoimmune rhomboencephalitis: A pediatric case report

Achille Marino  1   2 , Francesco Canonico  3 , Raffaella Maria Pinzani  4 , Massimo Andreotti  1 , Tiziana Varisco  1 , Alberto Maria Cappellari  5
Affiliations
Case Reports

Autoimmune rhomboencephalitis: A pediatric case report

Achille Marino et al. Turk Pediatri Ars. .

Abstract
in English, Turkish

Rhomboencephalitis is a potentially life-threatening condition due to inflammation of the hindbrain with an unpredictable outcome depending on the wide spectrum of etiologies and the promptness of diagnosis and treatment. A 23-month-old Caucasian male presented with fever, clouding of consciousness, and trunk ataxia. Three weeks earlier he received active immunization against varicella-zoster virus. Routine laboratory tests were unremarkable. Cerebrospinal fluid showed might elevation of leukocytes. The infection panel was negative. Brain magnetic resonance showed signal hyperintensity in the dorsal portion of the pons, which was consistent with a rhomboencephalitis. Autoimmune pathogenesis was supposed and a high dose of steroids was started. The patient's neurologic status progressively improved till full recovery and complete regression of previous magnetic resonance lesions after 1 year. Nevertheless, longer follow-up is needed in order not to miss any possible signs of an underlying autoimmune neurologic disorder.

Rombensefalit arka beyinin enflamasyonuna bağlı olarak gelişen ve yaşamı tehdit etme potansiyeli olan bir durum olup, geniş spektrumda bulunan etiyolojiler ve tanı ve tedavinin hızı ile bağlantılı olarak, sonuçları öngörülemez. Yirmi üç aylık beyaz bir erkek hasta ateş, bilinç bulanıklığı ve gövde ataksisi ile başvurdu. Üç hafta önce varisella zoster virüsüne karşı aşılanmıştı. Rutin laboratuvar testlerinde özellik saptanmadı. Beyin omurilik sıvısında lökositlerde hafif artış gözlendi. Enfeksiyon paneli negatif saptandı. Beyin manyetik rezonans incelemesi ponsun dorsal bölümünde sinyal hiperintensitesi gösterdi ve bu durum rombensefalit ile uyumlu idi. Otoimmun patogenez varsayıldı ve yüksek doz steroid başlandı. Hastanın nörolojik durumu ilerleyici bir şekilde düzeldi ve tam iyileşme gözlendi. Bir yıl sonra, daha önce manyetik rezonans incelemesinde gözlenen lezyonlar tamamen geriledi. Bununla birlikte, altta yatan otoimmün nörolojik bir bozukluğun olası bulgularını atlamamak için daha uzun süreli izlem gerekmektedir.

Keywords: Autoimmune; brainstem encephalitis; rhomboencephalitis; steroid.

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Conflict of interest statement

Conflict of Interest: No conflict of interest was declared by the authors.

Figures

Figure 1
Figure 1
Signal FLAIR hyperintensity in the dorsal portion of the pons in axial (a) and T2 sagittal (b) view
See this image and copyright information in PMC

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