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Case Reports
. 2018 May 22:83:e229-e233.
doi: 10.5114/pjr.2018.76380. eCollection 2018.

Spontaneous intracranial hypotension: two steroid-responsive cases

Camilla Russo  1 , Vincenzo Buono  2 , Giacomo Fenza  3 , Alexis Zandolino  1 , Antonietta Serino  3 , Andrea Manto  3
Affiliations
Case Reports

Spontaneous intracranial hypotension: two steroid-responsive cases

Camilla Russo et al. Pol J Radiol. .

Abstract

Purpose: Spontaneous intracranial hypotension (SIH) is characterised by orthostatic headache, low cerebrospinal fluid pressure and diffuse pachymeningeal enhancement after intravenous gadolinium contrast administration. Magnetic resonance imaging (MRI) often plays a crucial role for correct diagnosis.

Case description: We described two similar cases of SIH, whose clinical and imaging features are typical for this pathology. At MRI brain scan, both patients showed diffuse and intense pachymeningeal enhancement and moderate venous distension and epidural vein engorgement. The two patients were treated with bed rest and oral steroid therapy, with complete and long-lasting symptomatic relief.

Conclusions: Orthostatic nature of headache is the most indicative clinical feature suggesting SIH; contrast-enhanced MRI provides definite imaging diagnostic findings. Conservative treatment coupled to steroid therapy is often sufficient to obtain complete disappearance of symptoms.

Keywords: cerebrospinal fluid (CSF) pressure; myelopathy; spontaneous intracranial hypotension (SIH).

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Figures

Figure 1
Figure 1
A) Normal axial un-enhanced computed tomography brain scan, showing as occasional finding a capillary telangiectasia in right putamen. B) Sagittal TSE-T2w magnetic resonance imaging (MRI) shows reduced mamillopontine distance and cerebellar tonsils ectopia, with normal callosal and pontomesencephalic angles; C) venous distention sign is positive (white arrow). D) Axial, E) coronal, and F) sagittal SE-T1w MRI after intravenous gadolinium administration shows diffuse pachymeningeal enhancement; right putaminal capillary telangiectasia is clearly visible
Figure 2
Figure 2
After 2 months, follow-up SE-T1w magnetic resonance imaging on the three orthogonal plans after intravenous gadolinium administration shows almost complete resolution of the pathological diffuse pachymeningeal enhancement
Figure 3
Figure 3
A) Cervical spine and B-D) brain SE-T1w magnetic resonance imaging after intravenous gadolinium administration shows diffuse and intense pachymeningeal enhancement, involving also spinal dura. E) Axial FE T2* shows moderate venous distension and epidural vein engorgement
Figure 4
Figure 4
A-C) After 3 months, follow-up SE-T1w magnetic resonance imaging on the three orthogonal plans after intravenous gadolinium administration shows a partial reduction of the diffuse pachymeningeal enhancement
See this image and copyright information in PMC

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