CASE 15541 Published on 18.02.2018

Spontaneous intracranial hypotension and spinal CSF leak

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Pablo Naval Baudin1; Christopher Tomingas2; Mauricio Castillo2

1. Deptartment of Radiology ICS-IDI. Hospital Universitari de Bellvitge. Barcelona, Spain
2. Department of Radiology. University of North Carolina. Chapel Hill, NC, USA.
Patient

67 years, male

Categories
Area of Interest Neuroradiology brain ; Imaging Technique MR
Clinical History
A 67-year-old female patient presenting with headache, neck pain, double vision and nausea, worsening over several months.
Imaging Findings
MRI with sagittal midline T1WI (a), axial T2 (b), and T1 post-contrast in axial (c) and sagittal planes (d) show decreased pontomesencephalic angle (42º) (red lines), subdural fluid collections (arrow heads), diffuse pachymeningeal enhancement (dashed arrows), as well as engorged pituitary, and engorged torcula and venous sinuses in general.

Workup CT myelogram at two consecutive levels (e) shows contrast leakage through the right intervertebral T1-T2 foramen (arrows).
Discussion
The present case depicts the typical clinical history and imaging findings of spontaneous intracranial hypotension syndrome. This patient was initially empirically treated with epidural blood patching. Due to lack of clinical improvement, she was subsequently sent to another center to receive directed second-line treatment.

A.-BACKGROUND

Intracranial hypotension syndrome can be: spontaneous, secondary to trauma, surgery, or lumbar puncture. Spontaneous leaks may be due to dural tears related to perineural cysts or degenerative spondylopathy [1, 2].

B.-CLINICAL PRESENTATION

Main symptom is orthostatic headache which may be immediate or delayed. Other symptoms are: nausea, vomiting CN palsy, deafness, or cognitive impairment (often misdiagnosed as dementia) [2].

Diagnosis is frequently delayed by years especially when presentation is atypical. Correct diagnosis has important clinical implications, as most symptoms are reversible if treated [3].

C.-IMAGING PERSPECTIVE

Brain MR of intracranial hypotension: The “Saggy Brain”: [3, 4]
- Downward displacement of deep central brain and brainstem structures
- Herniated tonsils
- Engorged pituitary
- Pons draping (sagging) over the clivus
- Decreased pontomesencephalic angle (< 50°)
- Decreased mamillopontine distance (< 5 mm)
- Diffusely thick and enhancing pachymeninges
- Subdural fluid collections (haematoma or hygroma)
- Venous engorgement

Spinal imaging workup for CSF leak [3-6]
- CT-myelography is the initial imaging choice and often demonstrates the site of CSF leak (as in this case). It also identifies CSF leaks associated with perineural cysts or dural diverticula, extradural fluid collections, bony spurs and degenerative changes.
- For high-flow leaks, in which contrast spreads rapidly, the site of leak may be identified with dynamic CT-myelography or digital subtraction myelography.
- For low-flow leaks, MR-myelography with intrathecal gadolinium may be useful.
- Conventional MRI, especially heavily T2-weighted sequences, are sensitive for identifying extradural fluid collections and diverticulae and may be useful identifying high-flow leaks.

D.-DIFFERENTIAL DIAGNOSES

Other causes of meningeal thickening:
- Meningitis: Often lepto- and not pachymeningeal. Empyema restricts on DWI unlike hygromas.
- Chronic haematomas may restrict on DWI but contain blood product. Lack of brain “sagging”
- Lymphomatous meningitis.
- Granulomatous meningitis (TB, sarcoid)
- Meningeal metastases, often a more irregular and bumpy meningeal thickening. Lack of brain sagging.
- Dural sinus thrombosis. May simulate venous engorgement. Vascular imaging helps confirm patency.

E.-TREATMENT AND PROGNOSIS

Patients with mild symptoms may be treated conservatively with bed rest and avoiding upright position. Longstanding and severe cases benefit from epidural blood patching. Second-line management is directed to identifying leakage focus and directly treating the defect. CT-guided epidural blood patching of directly observed or potential leak sites has been described as such a technique [5-7].
Differential Diagnosis List
Spontaneous intracranial hypotension with spinal CSF leak
Meningitis
Meningeal metastases
Lymphomatous meningitis
Granulomatous meningitis
Dural sinus thrombosis
Final Diagnosis
Spontaneous intracranial hypotension with spinal CSF leak
Case information
URL: https://eurorad.org/case/15541
DOI: 10.1594/EURORAD/CASE.15541
ISSN: 1563-4086
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