Schievink Wouter I, Raissi Sharo S
Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA.
J Heart Valve Dis. 2012 Nov;21(6):714-7.
Spontaneous intracranial hypotension, an important but frequently misdiagnosed cause of headache, is often associated with the presence of an underlying systemic connective tissue disorder. Bicuspid aortic valve (BAV) is a relatively common systemic connective tissue disorder that has been associated with other causes of headache, such as intracranial aneurysm and cervico-cephalic arterial dissection. The study aim was to assess the frequency of BAV among patients with spontaneous intracranial hypotension.
The medical records of a consecutive group of patients with spontaneous intracranial hypotension were reviewed for the presence of BAV. The control population consisted of a group of patients evaluated for face pain or headache not due to spontaneous intracranial hypotension.
The presence of a BAV was confirmed in three of 273 patients (1.1%) with spontaneous intracranial hypotension, but in none of 506 controls (p = 0.04).
Patients with BAV may be at an increased risk of spontaneous intracranial hypotension. The aortic valvular cusps, as well as the spinal dura, are derived from cells that originate in the neural crest; hence, a disorder of neural crest cell migration could explain the findings of BAV and spontaneous intracranial hypotension in the presently reported patients.
