We present the possible causes, the proposed pathophysiological mechanisms, and the clinical and radiological development of this rare entity, with a review of the literature published to date. We propose that a preexisting weakness of the ventral dural fibers, combined with abnormal adhesion of the spinal cord to the anterior dural sleeve, leads to progressive herniation throughout life. Microsurgical treatment may halt the exacerbation of the neurological symptoms.

Sumatriptan, a selective 5-hydroxytryptamine (5HT1D)-receptor agonist, has recently been introduced in the pharmacotherapy of acute migrane attacks. The potential vasoactive effect of sumatriptan on human dural vessels in vivo, however, is still a matter of controversy. We investigated the effects of sumatriptan on dural vessels after subcutaneous or intra-arterial injection. During interventional angiography, the middle meningeal artery (MMA) of nine patients was catheterized with a microcatheter using the transfemoral route. Three MMA were entirely normal, two supplied a dural arteriovenous fistula (AVF) and four were transdural feeders to a brain arteriovenous malformation (AVM). Sumatriptan was injected either into the subcutaneous tissue of the right shoulder (6 mg, two patients) or into the catheterized MMA (2 mg, six patients). The substance caused a marked vasoconstriction of the three normal MMA, visible angiographically and confirmed by intravascular Doppler ultrasonography. Vasoconstriction was still present in the last angiogram obtained 15 min post-injection. Slightly hypertrophied feeders to dural AVF and to brain AVM showed some vasoconstriction in one and four patients, respectively. In two patients with markedly hypertrophied dural feeders to a dural AVF and to a brain AVM, respectively, rapid shunting probably prevented obvious vasoactive effects of sumatriptan. The data obtained by angiography and intravascular Doppler ultrasonography provide strong evidence that sumatriptan has a vasoconstrictive effect on normal as well as hypertrophied dural vessels.

X-ray examination of the intracranial ventricles with Dimer X made it possible to make the diagnosis of obstructive hydrocephalus due to a mass lesion of the midbrain and abnormal intrasellar recess of the third ventricle in a 34 year old female patient. Considering the location and ontogenetic and phylogenetic facts one can diagnose the second pathological finding as a persisting intrapituitary recessus infundibuli. A malformation due to inhibition during development is to be assumed.

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We report the endovascular treatment of a 40-year-old woman with bilaterally thrombosed transverse sinuses and a dural arteriovenous fistula (DAVF) causing cortical venous reflux by recanalization, angioplasty and stent deployment of the occluded sinus segment followed by occlusion of the DAVF by stent deployment in the fistulous segment. By recanalization of the occluded sinus we re-established normal anterograde venous drainage and eliminated the venous hypertension and cortical venous reflux. After the procedure, the patient was treated with aspirin and clopidogrel for three months. A follow-up examination showed total occlusion of the DAVF, patency of the sinus and a complete resolution of the clinical symptoms.