This 41-year-old female patient first experienced symptoms in 2015. She developed chronic overstimulation, fatigue, dizziness, and disturbed vision. Then, she developed unbearable brain fog and headaches. The most peculiar symptom was her intolerance to vertical movements. Since then, she has been unable to use an elevator, and the above-mentioned symptoms have increased to an unbearable degree. These symptoms are provoked by performing a Valsalva maneuver and by drying her eyes.

Currently, her main problems are a constant, debilitating pressure sensation, which is more pronounced on the left than right sides of her head; visual disturbances, including flashing sensations in the upper nasal quadrant of her left visual field; intolerance to postural changes; intolerance to slight body acceleration (she cannot tolerate elevators); left-sided tinnitus; and headaches. The headaches are pulsatile, extending horizontally around the skull, and are rated at 7/10. They occur almost daily. Sometimes, the patient vomits, but nausea is a constant feature.

Nausea is triggered by vertical body movement (e.g., getting up in the morning, using an elevator, or fast body movements with a vertical component), as well as by motor activity of the hands and exposure to noise and light. Headaches and dizziness are also provoked by defecation.

Lying down helps alleviate the symptoms. Food intake is limited due to nausea. The patient suffers from postural tachycardia syndrome.

A thorough workup by a cardiologist, neurologist, anesthesiologist, and ophthalmologist could not provide a specific diagnosis or relief for her condition.

Clinical findings:

The patient suffers from severe pressure and pressure changes inside her head, which she describes as a sensation resembling “the lack of oxygen.” She denies having symptoms in her abdomen or legs. Abdominal palpation reveals no localized pain or pathological lumps. There are no visible stretch marks, but substantial hyper-extensibility of large joints with a Beighton score of 5/9 is present. The family history is positive for Ehlers-Danlos syndrome. Auscultation of the abdomen reveals no vascular bruits and normal peristalsis. The skin is soft and shows no scars or excessive wrinkling.

Sonographic findings:

The patient had severe compression of the left renal vein, which fed the epidural plexus via a tronc réno-rachidien. This collateral route develops when the capacity of the left ovarian vein and left spermatic vein is insufficient to bypass the accumulating left renal venous blood in the event of compression of the left renal vein.

The epidural plexus becomes congested, primarily on its left side. Despite the valveless epidural veins, pressure is transferred with less resistance in non-branching trajectories since bending a tube reduces flow energy. Therefore, linear connections of veins are preferred to branching connections due to the high resistance to flow in such angular branches, even if they are valveless. This explains why the influx on the left side increases intracranial pressure more on the left than on the right. Consequently, drainage from the skull requires greater capacity on the left side in such a situation.

Unfortunately, the outflow of both internal jugular veins was compressed. However, the pressure gradient

at the compression site on the left side was much higher than on the right side, as demonstrated by the higher flow velocity on the left side. However, the left side had an additional outflow obstruction because the patient developed compression of the left brachiocephalic vein, which connects the neck vessels with the central thoracic vessels and the heart.

When the patient lies horizontally, gravity favors the influx of blood into the skull. As soon as the patient assumes an upright posture, the increased pressure is relieved by drainage via the jugular veins. Due to the double compression on the left side, pressure relief when assuming an upright posture is less pronounced on the left than on the right. This permanent pressurization of the left cranial hemisphere causes the left-sided headaches and other neurological symptoms described above. In the patient’s own words: ” I feel like I’m constantly under water, and also like on a wild sea because of a pulsating movement feeling on the left side of my brain”.

Combining abdominal volume flow measurements of relevant veins (mainly the left renal vein and the left common iliac vein), demonstrating congestion of the epidural plexus, and taking four-dimensional volume flow measurements of the jugular veins, as well as measuring pressure inside the jugular veins and the brachiocephalic vein, provides important clues for clarifying specific headache constellations, as in the patient described here.