Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Feb 13;18(1):53.
doi: 10.1186/s13256-024-04359-9.

Corticosteroid treatment for acute hydrocephalus in neurosarcoidosis: a case report

Affiliations
Case Reports

Corticosteroid treatment for acute hydrocephalus in neurosarcoidosis: a case report

Edoardo Dalmato Schilke et al. J Med Case Rep. .

Abstract

Background: Neurosarcoidosis occurs symptomatically in 5-10% of patients with sarcoidosis, and hydrocephalus is a rare complication of neurosarcoidosis, with either acute or subacute onset and presenting symptoms related to increased intracranial pressure. It represents a potentially fatal manifestation with a mortality rate of 22% (increased to 75% in case of coexistence of seizures) that requires a prompt initiation of treatment. High-dose intravenous corticosteroid treatment and neurosurgical treatment must be considered in all cases of neurosarcoidosis hydrocephalus.

Case presentation: Here we present a case of hydrocephalus in neurosarcoidosis, complicated by generalized seizures, in a 29-year-old Caucasian male patient treated with medical treatment only, with optimal response.

Conclusion: Since neurosurgery treatment can lead to severe complications, this case report underlines the possibility to undergo only medical treatment in selected cases. Further studies are needed to stratify patients and better identify those eligible for only medical approach.

Keywords: Neuroimmunology; Neurosarcoidosis; Neurosurgery in sarcoidosis; Obstructive hydrocephalus.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Evidence of nodular contrast enhancement of the leptomeninges (a), optic nerves (b), white matter lesions (c), and cauda equina (d)
Fig. 2
Fig. 2
Main examinations performed after clinical worsening; brain MRI shows biventricular hydrocephalus and contrast enhancement of ependyma at the cerebral aqueduct (III and IV ventricles, a-c). Electroencephalogram shows intercritical epileptic discharges (d)

References

    1. Moller DR, Rybicki BA, Hamzeh NY, Montgomery CG, Chen ES, Drake W, Fontenot AP. Genetic, immunologic, and environmental basis of sarcoidosis. Ann Am Thorac Soc. 2017;14(Supplement_6):S429–S436. doi: 10.1513/AnnalsATS.201707-565OT. - DOI - PMC - PubMed
    1. Scott TF, Yandora K, Valeri A, Chieffe C, Schramke C. Aggressive therapy for neurosarcoidosis: long-term follow-up of 48 treated patients. Arch Neurol. 2007;64(5):691–696. doi: 10.1001/archneur.64.5.691. - DOI - PubMed
    1. Gelfand JM, Bradshaw MJ, Stern BJ, Clifford DB, Wang Y, Cho TA, Koth LL, Hauser SL, Dierkhising J, Vu N, Sriram S, Moses H, Bagnato F, Kaufmann JA, Ammah DJ, Yohannes TH, Hamblin MJ, Venna N, Green AJ, Pawate S. Infliximab for the treatment of CNS sarcoidosis: a multi-institutional series. Neurology. 2017;89(20):2092–2100. doi: 10.1212/WNL.0000000000004644. - DOI - PMC - PubMed
    1. Ramstein J, Broos CE, Simpson LJ, Ansel KM, Sun SA, Ho ME, Woodruff PG, Bhakta NR, Christian L, Nguyen CP, Antalek BJ, Benn BS, Hendriks RW, van den Blink B, Kool M, Koth LL. IFN-γ-Producing T-Helper 171 cells are increased in sarcoidosis and are more prevalent than T-helper type 1 cells. Am J Resp Critic Care Med. 2016;193(11):1281–1291. doi: 10.1164/rccm.201507-1499OC. - DOI - PMC - PubMed
    1. Stern BJ, Royal W, 3rd, Gelfand JM, Clifford DB, Tavee J, Pawate S, Berger JR, Aksamit AJ, Krumholz A, Pardo CA, Moller DR, Judson MA, Drent M, Baughman RP. 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. - DOI - PubMed

Publication types

MeSH terms

Substances

Supplementary concepts