Spontaneous intracranial hypotension - Neurological symptoms, diagnosis, and outcome

Spontaneous intracranial hypotension (SIH) is a rare secondary headache disorder caused by spinal leakage of cerebrospinal fluid. Specialized treatment of SIH consists of epidural blood patches (EBPs), fibrin patching, endovascular sealing, and surgery. The aim of this paper was to characterize SIH patients identified at a tertiary headache center.

Eighty patients with SIH identified at the Danish Headache Center were included in this clinical cohort study. Data on demographics, clinical presentation, imaging findings, effect of EBPs, and invasive procedures were collected in a standardized manner. Prognostic elements of EBP treatment outcome were evaluated statistically with success defined as a minimum 30% intensity reduction sustained for at least 1 month.

The average age was 47 years and 53% were female. Peracute and subacute onset was noted in 35% and 36%, respectively. Orthostatic headache was noted in 85% of cases. The median Bern score was 4, and of identified leaks, 52% were type 1, 22% type 2, 22% type 3, and 4% type 4 (defined as peripheral leakage distally for the root pouch). Successful treatment response was 34% for EBPs and 90% for invasive procedures. Prepontine cistern effacement was more prevalent in patients with successful first EBP than without (p = 0.013).

SIH patients presented with a wide variety of symptoms with high prevalence of orthostatic headache. Peracute onset was noted in 35% and calls for more awareness in the acute setting. We found prepontine cistern effacement to be a potential predictor of a successful first epidural blood patch.