Purpose: Cough-associated headache (CAH) is the most distinctive symptom of patients with Chiari I malformation (CMI) and indicates clinically significant disease. We determined the clinical utility of simple 2D anatomic measurements performed on a PACS workstation by assessing their diagnostic accuracy in predicting CAH in CMI patients.
Methods: Seventy-two consecutive CMI patients (cerebellar tonsillar herniation > 5 mm) with headache seen by neurosurgeons over 6 years were included. Sagittal T1 images were used by two readers to measure: extent of tonsillar herniation, lengths of the clivus and supra-occiput, McRae and pB-C2 lines, as well as clivus-canal, odontoid retroversion, and skull base angles. Neurosurgery notes were reviewed to determine presence of CAH. Mann-Whitney test was used to compare measurements between patients with and without CAH. Predictive accuracy was assessed by receiver operating characteristic (ROC) curve.
Results: 47/72 (65.3%) CMI patients reported CAH. Tonsillar herniation with CAH (10.2 mm, 7-14 mm; median, interquartile range) was significantly greater than those without CAH (7.9 mm, 6.3-10.9 mm; p = 0.02). Tonsillar herniation ≥ 10 mm showed sensitivity and specificity of 51% and 68%, and tonsillar herniation > 14 mm showed sensitivity and specificity of 30% and 100%, respectively, for predicting CAH. Other 2D measurements showed no statistically significant differences.
Conclusions: Among the 2D measurements used, only the extent of tonsillar herniation is different between CMI patients with and without CAH. Although CMI is diagnosed with tonsillar herniation of only 5 mm, we found that a much higher extent of herniation is needed to be predictive of CAH.
Keywords: Chiari 1 malformation; Cough; Cranio-vertebral junction; Headache; Morphometry.