Valsalva-triggered pseudotumor cerebri syndrome: Case series and pathogenetic implications

Objective To report the clinical and radiologic features of 3 patients in whom prolonged vigorous coughing/Valsalva triggered the development of pseudotumor cerebri syndrome (PTCS) that persisted even after the coughing attacks had stopped and to discuss how this novel proximate trigger supports a “hydraulic model” of PTCS pathogenesis. Methods This was a retrospective case series of 3 patients seen between January 2011 and July 2017 in a tertiary care neuro-ophthalmology clinic. Results Three female patients (ages 13, 28, and 21 years) were asymptomatic until developing prolonged fits of profuse coughing. Two patients had upper respiratory tract infections, and 1 patient had a prolonged asthma exacerbation. Symptoms typical of PTCS began 2 weeks into the coughing attacks, and patients were examined 1 to 2 weeks after coughing had ceased. One patient was overweight and 2 were obese. All 3 patients had papilledema, and 1 of 3 patients had lateral rectus palsy. Two of 3 patients had visual field defects, and all 3 patients had dural venous sinus stenoses (DVSS) on venous imaging. Lumbar puncture (LP) confirmed elevated opening pressures in 2 of 3 patients and brought immediate and sustained relief of symptoms; LP attempts failed in the third patient because of body habitus (body mass index 68 kg/m 2 ). All patients were given acetazolamide and advised to work on weight loss. At follow-up 3 to 6 months later, all 3 patients had complete remission of their clinical syndromes and resolution of their DVSS on neuroimaging. Conclusions In susceptible individuals, prolonged coughing/Valsalva can trigger PTCS in the setting of collapsible (nonfixed) DVSS. This new observation supports a hydraulic model of PTCS pathogenesis.