Atypical presentations and long-term sequelae of spontaneous intracranial hypotension: illustrative cases

BACKGROUND: Spontaneous intracranial hypotension (SIH) is typically recognized by orthostatic headache and characteristic neuroimaging findings such as brain sagging and dural enhancement, but atypical manifestations and long-term sequelae remain underreported. OBSERVATIONS: The authors present 5 cases that illustrate uncommon SIH presentations and complications, including reversible cerebral vasoconstriction syndrome, sinus vein thrombosis, ventral spinal cord herniation, thoracic meningocele, secondary Arnold-Chiari malformation type I, and superficial siderosis. LESSONS: SIH may present with diverse complications beyond orthostatic headache; awareness and follow-up imaging are important for timely diagnosis and management. https://thejns.org/doi/10.3171/CASE26236.

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Journal
Journal of Neurosurgery Case Lessons
Published
2026-08-03
DOI
https://doi.org/10.3171/case26236
Primary Topic
Neurosurgical Procedures and Complications
Type
article
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article

Atypical presentations and long-term sequelae of spontaneous intracranial hypotension: illustrative cases

Johannes Goldberg, Patrick Dorn, Werner Josef Z'Graggen, Christine Roten-Joss et al.
Journal of Neurosurgery Case Lessons
Neurosurgical Procedures and Complications
article

Atypical presentations and long-term sequelae of spontaneous intracranial hypotension: illustrative cases

Johannes Goldberg, Patrick Dorn, Werner Josef Z'Graggen, Christine Roten-Joss, Danial Nasiri, Aude Eckert, Alexis P. R. Terrapon, Eike I. Piechowiak, Tomas Dobrocky, Ralph T. Schär, Katharina Lutz, Levin Häni, Andreas Raabe
article en

Abstract

BACKGROUND: Spontaneous intracranial hypotension (SIH) is typically recognized by orthostatic headache and characteristic neuroimaging findings such as brain sagging and dural enhancement, but atypical manifestations and long-term sequelae remain underreported. OBSERVATIONS: The authors present 5 cases that illustrate uncommon SIH presentations and complications, including reversible cerebral vasoconstriction syndrome, sinus vein thrombosis, ventral spinal cord herniation, thoracic meningocele, secondary Arnold-Chiari malformation type I, and superficial siderosis. LESSONS: SIH may present with diverse complications beyond orthostatic headache; awareness and follow-up imaging are important for timely diagnosis and management. https://thejns.org/doi/10.3171/CASE26236.

Journal of Neurosurgery Case LessonsVol. 12(5)
University of Bern (CH), University Hospital of Bern (CH)
Good health and well-being
Openalex Percentile: Top 9%
Neurosurgical Procedures and Complications
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