Occipital Neuralgia Secondary to a Spontaneous CSF Leak (P03.218)

Abstract

Objective: Spontaneous CSF leaks can cause occipital neuralgia. Background Spontaneous CSF leak is now recognized as a cause for secondary new daily persistent headache (NDPH). Although postural (orthostatic) headache is characteristic, multiple other headache types can occur with CSF leaks. We here report an occipital neuralgia case secondary to CSF leak, an unusual scenario not previously reported. Design/Methods: Case report. Results: A 51-year-old female diagnosed with NDPH for 3 years had bilateral, pressure-like, posterior headache which was exacerbated by Valsalva, alleviated by recumbency, and associated with muffled hearing. Neurologic exam, brain MRI/MRV/MRA, and cervical MRI were unremarkable. With the diagnosis of NDPH, amitriptyline was started but failed. With time, her headache changed to bilateral occipital, shock-like pains lasting seconds superimposed on steady, longer-lasting (20min to 5 hours) occipital pain, hypoesthesia, and dysesthesia without postural component. Greater occipital nerve block resulted in dramatic response, confirming a diagnosis of occipital neuralgia (ICHD-2 criteria), but later occipital headaches recurred. To exclude potential cranio-cervical junction structural pathology, brain MRI was repeated which showed slight dural gadolinium enhancement and “brain sagging”, features of spontaneous CSF leak. Subsequent complete spine MRI showed extradural fluid collections but no clear CSF leak location. CT myelogram confirmed a thoracic CSF leak but could not precise the level. Dynamic CT myelogram identified fast CSF leakage at T4-T5, secondary to a disc extrusion. Decompressive laminectomy with CSF leak repair completely resolved her occipital headaches. Conclusions: In addition to orthostatic and other headache types, occipital neuralgia is another manifestation of spontaneous CSF leaks. We suspect the mechanism may be traction of the C2-C3 nerve roots during cervicomedullary junction and brain descent from CSF leakage. This may be an underdiagnosed phenomenon which may account for some unexplained cases of occipital neuralgia. A transient orthostatic headache at some point in the clinical course may suggest the diagnosis. Disclosure: Dr. Ansari has nothing to disclose. Dr. Garza has received personal compensation for activities with American Headache Society as a speaker. Dr. Garza has received personal compensation in an editorial capacity for UpToDate and Current Neurology.