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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