International Clinical Neuroscience Journal

Vol. 11 No. 1 (2024)

Diffuse Systemic Sclerosis with Brainstem Encephalitis: A Case Report An Illustrative case

International Clinical Neuroscience Journal, Vol. 11 No. 1 (2024), 4 Aban 2025 , Page e1
https://doi.org/10.22037/icnj.v11i1.39994

Abstract

Brainstem encephalitis (BE) is a rare disease that affects 1 in 10,000 individuals each year. However, quite little is known about the aetiologies causing brainstem encephalitis. Scleroderma is a rare connective tissue disorder with pathophysiology still under research. We hereby report a case of systemic sclerosis that primarily presented with brainstem encephalitis, a relatively uncommon presentation, and was retrogradely evaluated, assessed, and diagnosed as diffuse systemic sclerosis (Ds-SSc).

Systemic sclerosis is associated with multisystem involvement (multiple internal organs simultaneously or sequentially) and is associated with increased morbidity and mortality, with uncommon involvement of the brainstem. Hence, in any case of brainstem encephalitis in a young patient with no apparent infectious cause, an elaborate examination of all systems and autoimmune profiles must be performed to enable rapid initiation of treatment and, therefore, improve prognosis.

How to Cite

1.
Puppala S, Choudhury SS, Acharya A, Dash S. Diffuse Systemic Sclerosis with Brainstem Encephalitis: A Case Report: An Illustrative case. Int Clin Neurosci J [Internet]. 2025 Nov. 4 [cited 2026 Jul. 5];11(1):e1. Available from: https://journals.sbmu.ac.ir/neuroscience/article/view/39994

References

1. Glaser CA, Honarmand S, Anderson LJ, et al. Beyond viruses: clinical profiles and etiologies associated with encephalitis. Clin Infect Dis. 2006;43(12):1565–77. doi: 10.1086/509330

2. Tan IL, Mowry EM, Steele SU, Pardo CA, McArthur JC, Nath A, Venkatesan A. Brainstem encephalitis: etiologies, treatment, and predictors of outcome. J Neurol. 2013;260(9):2312-9. doi: 10.1007/s00415-013-6986-z

3. Moragas M, Martínez-Yélamos S, Majós C, Fernández-Viladrich P, Rubio F, Arbizu T. Rhombencephalitis: a series of 97 patients. Medicine (Baltimore) 2011;90(4):256–261. doi: 10.1097/MD.0b013e318225460c

4. Coentro JQ, Pugliese E, Hanley G, Raghunath M, Zeugolis DI. Current and upcoming therapies to modulate skin scarring and fibrosis. Adv Drug Deliv Rev. 2019;146:37-59. doi: 10.1016/j.addr.2018.08.007

5. Politikou O, Giesen T, Reissner L, Calcagni M. Hand and wrist joint procedures in patients with scleroderma: a systematic review. J Hand Surg Eur Vol. 2019;44(4):402-7. doi: 10.1177/1753193419830998

6. Hunzelmann N. [Current treatment of systemic scleroderma]. Hautarzt. 2018;69(11):901-7. doi: 10.1007/s00105-018-4267-3

7. Zoghaib R, Sreij A, Maalouf N, Freiha J, Kikano R, Riachi N, et al. Autoimmune Brainstem Encephalitis: An Illustrative Case and a Review of the Literature. J Clin Med. 2021 Jul 1;10(13):2970. doi: 10.3390/jcm10132970

  • Abstract Viewed: 112 times
  • PDF Downloaded: 49 times

Download Statastics