Diagnosis and treatment evaluation in patients with spontaneous intracranial hypotension

Abstract

Spontaneous intracranial hypotension is characterized by an orthostatic headache and audiovestibular symptoms alongside a myriad of other non-specific symptoms. It is caused by an unregulated loss of cerebrospinal fluid at the spinal level. Indirect features of CSF leaks are seen on brain imaging as signs of intracranial hypotension and/or CSF hypovolaemia as well as a low opening pressure on lumbar puncture. Direct evidence of CSF leaks can frequently, but not invariably, be observed on spinal imaging. The condition is frequently misdiagnosed due to its vague symptoms and a lack of awareness of the condition amongst the non-neurological specialities. There is also a distinct lack of consensus on which of the many investigative and treatment options available to use when managing suspected CSF leaks. The aim of this article is to review the current literature on spontaneous intracranial hypotension and its clinical presentation, preferred investigation modalities, and most efficacious treatment options. By doing so, we hope to provide a framework on how to approach a patient with suspected spontaneous intracranial hypotension and help minimize diagnostic and treatment delays in order to improve clinical outcomes.

Don’t delay, but don’t despair: symptom duration, comorbidity and outcome after closure of spinal cerebrospinal fluid leaks

“…In Häni’s series, the duration is the only factor with a significant impact. They report, that a symptom duration below 87 days was correlated with a better outcome [ 9 ], promoting the recommendation to strive for a prompt diagnosis and therapy, ideally within 3 months if SIH is suspected [ 3 , 4 ]. In Brinjikji’s series, several factors influence the outcome, with symptom duration as the only modifiable variable [ 16 ].…”

“…The clinical presentation of spontaneous intracranial hypotension (SIH) is multifaceted and includes, among many other symptoms, headache, neck pain, nausea, tinnitus and cognitive symptoms [ 1 – 4 ]. Typically, there is an acute and well-remembered onset of clinical symptoms.…”

“…The first study that found a significant effect of the symptom duration on the postoperative outcome after surgery was by Häni et al [ 9 ]. Since then, a cut-off value of below 3 months as a predictor for a positive surgical outcome is frequently cited [ 3 , 4 ].…”

Reversible cerebral vasoconstriction syndrome following intracranial hypotension in a postpartum patient: a case report and literature review

“…In our case, intracranial hypotension may be the primum movens of RCVS. Most patients with intracranial hypotension respond to a short conservative management course followed by non-targeted patching of epidural blood ( 27 ). RCVS drug treatment with antagonists of calcium ion channels (nifedipine, verapamil, or nimodipine) positively impacts the clinical progression.…”

Intracranial hypotension in marfan syndrome

“…Intracranial hypotension syndrome (IHS) due to cerebrospinal fluid (CSF) leaks, causing orthostatic headache and other neurological disorders, has been more often described in adult females with Marfan syndrome (MS), or Ehlers-Danlos syndrome [1][2][3][4][5][6] . The prevalence of IHS is 1:5,000 to 1:10,000 population, with a non-consensual female predominance, at mean age of 42 years 2,4-6 , and presenting connective tissue disease [1][2][3][4][5][6] .…”