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
. 2021 Jan 8;13(1):9-16.
doi: 10.1159/000510847. eCollection 2021 Jan-Apr.

Unusual Presentation in Infratentorial Superficial Siderosis: Acute Intracranial Hypertension

Maria Sheila Guimarães Rocha  1   2 , Hugo Salomão Grangeiro Mirô  1 , Gregori Manfroi  1 , Arthur de Medeiros Dias  1 , Raphaella Cardoso  1 , Sonia Maria Dozzi Brucki  1   3
Affiliations
Case Reports

Unusual Presentation in Infratentorial Superficial Siderosis: Acute Intracranial Hypertension

Maria Sheila Guimarães Rocha et al. Case Rep Neurol. .

Abstract

Superficial siderosis (SS) of the nervous system is a rare acquired condition related to hemosiderin deposits in subpial layers of the brain, brainstem, cerebellum, cranial nerves, and spinal cord, leading to brain iron-mediated neurodegeneration. The cardinal neurological features are slowly progressive hearing loss, ataxia, and pyramidal signs. Here we describe an atypical case of infratentorial SS evolving with acute intracranial hypertension in the absence of typical chronic signs.

Keywords: Ferritin; Intracranial hypertension; Neurodegeneration; Neurosensorial deafness; Superficial siderosis.

PubMed Disclaimer

Conflict of interest statement

No potential conflict of interest was reported by the authors.

Figures

Fig. 1
Fig. 1
Brain MRI depicting diffuse low signal at T2, T1, and GRE, on the infratentorial surfaces, at the level of brainstem and cerebellum (a, c, and f), on the supratentorial at the thalamic surface at lateral ventricles, corpus callosum, and quadrigeminal plate (b), and Sylvian fissure and paramedian surfaces at frontal and temporal lobes (d).
Fig. 2
Fig. 2
Sagittal T2 images depicting intense siderosis not only on spinal cord surfaces (a, b) but also in the sacrum (c).
See this image and copyright information in PMC

References

    1. Wilson D, Chatterjee F, Farmer SF, Rudge P, McCarron MO, Cowley P, et al. Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway. Ann Neurol. 2017 Mar;81((3)):333–43. - PubMed
    1. Hamill RC. Report of a Case of Melanosis of the Brain, Cord and Meninges. J Nerv Ment Dis. 1908;35((9)):594. Available from: https://journals.lww.com/jonmd/Fulltext/1908/09000/REPORT_OF_A_CASE_OF_M....
    1. Pichler M, Vemuri P, Rabinstein AA, Aakre J, Flemming KD, Brown RD, Jr, et al. Prevalence and Natural History of Superficial Siderosis: A Population-Based Study. Stroke. 2017 Dec;48((12)):3210–4. - PMC - PubMed
    1. Fearnley JM, Stevens JM, Rudge P. Superficial siderosis of the central nervous system. Brain. 1995 Aug;118(4):1051–66. - PubMed
    1. Charidimou A, Law R, Werring DJ. Amyloid “spells” trouble. Lancet. 2012 Nov;380((9853)):1620. - PubMed

Publication types

LinkOut - more resources