Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
December 4, 2018
Spontaneous intracranial hypotension (caused by posterior disc osteophytes successfully treated with epidural blood patch)
- Background
- Increasingly recognized syndrome associated with unique patient symptomatology and imaging features.
- Typically results from a CSF leak along the neuroaxis.
- Clinical Presentation
- 2:1 predilection for females over males.
- Majority of patients tend to present acutely with symptoms of intracranial hypotension.
- Symptoms can include postural headache (most commonly), but can also include neck stiffness, nausea, vomiting, vertigo, tinnitus, and cognitive abnormalities.
- In rare presentations cognitive symptoms can clinically mimic frontotemporal dementia.
- Increased risk for cranial nerve palsies due to anatomic distortion of the brainstem, as well as an increased risk of cerebral venous thrombosis from decreased cerebral venous flow.
- Key Diagnostic Features
- Typical imaging findings of intracranial hypotension include dural venous sinus dilation, subdural collections, brainstem and tonsillar sagging, and pituitary enlargement.
- Initial patient evaluation usually includes MRI and/or CT of the head based on presenting symptoms, which leads to suggestion of the diagnosis.
- MRI of the spine may provide non-invasive insight into the suspected site of leak.
- However, CT myelogram examination is more sensitive in potentially diagnosing a site of leak and may also lead to identification of a causal lesion (such as a posterior disc osteophyte/s, as in this case).
- On myelography, retrospinal spinal fluid collection at C1-2 can be seen incidentally and does not have to correspond to a site of CSF leak (false localizing sign).
- Treatment
- CT guided epidural blood patch targeting of observed or potential CSF leak sites has shown particular efficacy in treatment of spontaneous intracranial hypotension.
- In cases where multiple attempts of targeted epidural blood patch placement fail to result in adequate closure of the CSF leak site, more invasive surgical dural repair may be necessary.
- Improvement in patient symptoms and noted imaging findings are usually indicative of treatment response.