AccScience Publishing / BH / Volume 2 / Issue 2 / DOI: 10.36922/bh.2133
Cite this article
Journal Browser
Volume | Year
News and Announcements
View All

Approaching an undetermined diagnosis in the aftermath of rhombencephalitis: A case report

Debabrata Chakraborty1*
Show Less
1 Department of Neurology, Apollo Multispeciality Hospital, Kolkata, West Bengal, India
Brain & Heart 2024, 2(2), 2133
Submitted: 27 October 2023 | Accepted: 26 January 2024 | Published: 23 April 2024
© 2024 by the Author (s). This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution 4.0 International License ( )

A 49-year-old male patient manifested acute onset increased imbalance, quadriparesis, and speech impairment 1 month after undergoing ventricular-peritoneal shunt for normal pressure hydrocephalus (although magnetic resonance imaging [MRI] of the brain with contrast was otherwise normal), which showed improvement afterward. He had hypercalcemia and elevated erythrocyte sedimentation rate. Repeat brain MRI revealed pachymeningitis coupled with rhombencephalitis, while cerebrospinal fluid study unveiled only increased protein level without evidence of active infection. Given these findings, neurosarcoidosis was preliminarily considered a plausible cause of the recent clinical manifestations, and the patient was prescribed methylprednisolone, which led to significant improvement. However, the steroid treatment was discontinued on the revelation that the patient grappled with severe sepsis. Despite an initial improvement following a post-management of sepsis, his condition deteriorated, and he became lost to follow-up after 4 months of initial presentation. An infective etiology was ruled out since his condition improved with steroid. Precluding vasculitis or demyelinating disorder left the physicians with primary central nervous system (CNS) lymphoma or sarcoidosis as a proper diagnosis based on the fact that the patient experienced deterioration when steroid was excluded from the treatment regimen. This case study portrays a need to conduct a more specific and elaborate investigation, driven by a strong perception to both primary CNS lymphoma and sarcoidosis, to optimize clinical diagnosis, which facilitates the formulation of an appropriate treatment regimen.

Primary central nervous system lymphoma
  1. Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: Diagnosis and treatment. Curr Neurol Neurosci Rep. 2008;8(5):371-376. doi: 10.1007/s11910-008- 0058-2


  1. Lee SC, Lueck CJ. Cerebrospinal fluid pressure in adults. J Neuroophthalmol. 2014;34(3):278-283. doi: 10.1097/WNO.0000000000000155


  1. Westhout FD, Linskey ME. Obstructive hydrocephalus and progressive psychosis: Rare presentations of neurosarcoidosis. Surg Neurol. 2008;69(3):288-292. doi: 10.1016/j.surneu.2007.01.068


  1. Tana C, Wegener S, Borys E, et al. Challenges in the diagnosis and treatment of neurosarcoidosis. Ann Med. 2015;47(7):576-591. doi: 10.3109/07853890.2015.1093164


  1. Stern BJ, Royal W 3rd, Gelfand JM, et al. Definition and consensus diagnostic criteria for neurosarcoidosis: From the neurosarcoidosis consortium consensus group. JAMA Neurol. 2018;75(12):1546-1553. doi: 10.1001/jamaneurol.2018.2295


  1. Boshrabadi AP, Naiem A, Ghazi Mirsaeid SS, et al. Hydrocephalus as the sole presentation of primary diffuse large B-cell lymphoma of the brain: Report of a case and review of literature. Surg Neurol Int. 2017;8:165. doi: 10.4103/sni.sni_446_16


  1. Scott BJ, Douglas VC, Tihan T, Rubenstein JL, Josephson SA. A systematic approach to the diagnosis of suspected central nervous system lymphoma. JAMA Neurol. 2013;70(3):311-319. doi: 10.1001/jamaneurol.2013.606
Conflict of interest
The author declares no competing interest.
Back to top
Brain & Heart, Electronic ISSN: 2972-4139 Published by AccScience Publishing