2024 American Academy of Neurology Abstract Website

Objective:

- To appreciate atypical clinical features of severe spontaneous intracranial hypotension

- To appreciate radiologic findings of intracranial hypotension as well as false-localizing signs of cerebrospinal fluid leak

Background:

Spontaneous intracranial hypotension is a rare condition resulting from a leak of cerebrospinal fluid (CSF) which can affect individuals of all ages without a preceding trigger or risk factor. Presentation classically includes postural headache, but a variety of other neurological manifestations including tinnitus, vertigo, and ataxia have been described. Imaging findings typically help establish the diagnosis, but identification of the source of CSF leak and treatment using blood patch, surgery, or endovascular intervention can be challenging. Here we describe a case of severe spontaneous intracranial hypotension presenting with intractable hiccups.

Design/Methods:

N/A

Results:

36-year-old male with no prior medical history developed orthostatic headache, neck pain, and tinnitus during a military deployment. Initial concern for meningitis prompted a lumbar puncture. As symptoms worsened after unremarkable infectious workup, repeat imaging was performed which was suggestive of intracranial hypotension. He was transferred to higher level of care where he developed intractable hiccups. Imaging revealed progression of intracranial hypotension with subdural hemorrhages bilaterally and marked brainstem compression. Spinal imaging revealed extensive extradural CSF fluid collections including imaging signs falsely localizing for the CSF leak, which was eventually attributed to a spinal disc osteophyte. After repeated blood patches including a multi-level blood patch spanning C4-L4, the patient saw slow resolution of symptoms with eventual radiographic improvement.

Conclusions:

Intracranial hypotension is a rare condition resulting from CSF leak that is associated with a variety of cerebellar and brainstem signs. Hiccups are a rarely described finding that can indicate a more severe insult to the CNS and should prompt escalation of level of care. Urgent localization of the CSF leak and subsequent patching or repair is vital to prevent further decline in these patients.