Update on the Diagnosis and Treatment of Spontaneous Intracranial Hypotension

Abstract

SIH is an important cause of headaches caused by spinal cerebrospinal fluid (CSF) leaks, with an increasingly broad spectrum of clinical presentations and diagnostic findings. A simple conception of the condition as being defined by the presence of low CSF pressure is no longer sufficient or accurate. A number of etiologies for spinal CSF leaks have been identified, including the recent discovery of CSF-venous fistulas, and these various etiologies may require different diagnostic and therapeutic pathways in order to affect a cure. Familiarity with the spectrum of presentations and causes of SIH is critical to accurate and timely diagnosis and management. Challenges exist in both diagnosis and treatment, and require understanding of the underlying pathogenesis of the condition in order to appropriately select testing and treatment. Prospective studies are needed going forward in order to inform workup and guide treatment decisions.

Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions

“…22,38 A frequent but under-reported complication of CSF leak closure is rebound intracranial hypertension (RIH). 3 In our experience, some degree of RIH is present in over half of patients undergoing epidural patching, with approximately half of symptomatic patients requiring treatment for one week or longer. The most consistent clinical symptom exhibited in SIH is headaches change from orthostatic to those that are worse when recumbent or nonpositional, which allows discrimination between recurrent SIH and RIH.…”

“…19 Uncommonly, headache might be non-orthostatic or absent entirely. 3 Postural orthostatic tachycardia syndrome (POTS) has been well documented to cause orthostatic headache in some patients. 20 When headaches are absent (termed the "acephalgic" form), auditory symptoms, such as muffled hearing or tinnitus, and ear pain or pressure are common complaints.…”

“…3,25,45 While epidural patching usually causes rapid clinical improvement in postdural puncture headache, our experience has been that improvement in symptoms may take longer in cases of spontaneous CSF leak. 3,25,45 While epidural patching usually causes rapid clinical improvement in postdural puncture headache, our experience has been that improvement in symptoms may take longer in cases of spontaneous CSF leak.…”

“…3 Some early reports emphasized a high rate of immediate response with low recurrence rates, leading to a perception that SIH is easily treated with a single blood patch, with little to no after care required. 3 Some early reports emphasized a high rate of immediate response with low recurrence rates, leading to a perception that SIH is easily treated with a single blood patch, with little to no after care required.…”

“…Although historically perceived as rare, SIH is now being recognized more commonly; the incidence of SIH has been estimated at 5 per 100,000. 3 As a consequence, conceptions about appropriate diagnosis and treatment of SIH are sometimes based on outdated or insufficient information. 1 The true incidence of SIH is expected to be higher, as it is frequently misdiagnosed initially.…”