Spontaneous Intracranial Hypotension Syndrome is associated with intracranial hypotension and/or CSF leak without identifiable cause. The diagnosis can be challenging since no single finding is diagnostic and some findings may be normal in different patients.

Common symptoms include:

(1) orthostatic headache, which may be disabling

(2) nausea

(3) neck pain or stiffness


Lumbar puncture opening pressure is typically low (< 60 mm H2O) but may be normal.


MRI is sensitive but may be normal in around 20%.

(1) Brain MRI may show diffuse pachymeningeal enhancement.

(2) Spinal MRI may show extradural CSF in up to 75% of patients.


Digital subtraction myelography and MR myelography with intrathecal gadolinium is sensitive for identifying the site of the CSF leak.


The diagnosis is supported if the findings respond to a large blood patch.


Differential diagnosis:

(1) CSF leak due to trauma or lumbar puncture


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