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of gait is usually present within 6 months, and half of the patients described by Aminoff and Logue were chairbound within 3 years; the average survival in the past was 5 to 6 years, but the disorder has rarely been fatal in our patients These lesions only infrequently give rise to intramedullary or subarachnoid hemorrhage The spinal uid shows high protein but little or no cellular reaction When viewed directly, the dorsal surface of the lower cord may be covered with a tangle of veins, some involving roots and penetrating the surface of the cord The progression of symptoms is due presumably to the chronic venous hypertension and secondary intramedullary ischemic changes, and the abrupt episodes of worsening have been attributed to the thrombosis of vessels all on uncertain grounds However, angiographic studies sometimes show only a single or a few such dilated draining vessels.

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Furthermore, there is not suf cient pathologic material to determine whether some of the more prominent venous anomalies represent true venous angiomas (probably they do not) As mentioned, these lesions rarely bleed Intradural Perimedullary and Subpial AVM The pial stulous arteriovenous communication that involves the super cial aspect of the cord to a variable extent is the least frequent but probably of a similar nature to the dural type, or it may be related to the vascular lesion in the earlier mentioned Foix-Alajouanine process In contrast to dorsal arteriovenous malformations, these stulas tend to involve the lower thoracic and upper lumbar segments or the anterior parts of the cervical enlargement The patients are often younger, and the sexes are equally affected.

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The clinical syndrome may take the form of slow spinal cord compression, sometimes with a sudden exacerbation; or the initial symptoms may be apoplectic in nature, due to either thrombosis of a vessel or a hemorrhage from an associated draining vein that dilates to aneurysmal size and bleeds into the subarachnoid space or cord (hematomyelia and subarachnoid hemorrhage); the latter complication occurred in 7 of 30 cases reported by Wyburn-Mason Diagnosis Any of these lesions, dural or intradural, may be revealed on CT myelography or MRI by the presence of one or more fortuitously discovered enlarged and serpiginous draining vessels in the subarachnoid space; just as often, they are not visualized by these methods (Jones et al).

Other features that have been emphasized include enlargement of the spinal cord at the level of the lesion and, particularly in the case of spinal dural stulae, with venous congestion and T2-bright enhancement of the swollen cord over several segments Because of the low- ow nature of the vascular lesion, the same region may be T1 hypointense Some clinicians have commented on the presence of peripherally located regions of T2-hypointense signal changes (Hurst and Grossman) Many of these changes reverse with appropriate surgical or radiological interventions that ablate the malformations The diagnosis is established through selective angiography, which shows the stula in the dura overlying the cord or on the surface of the cord itself, but the most conspicuous nding is often the associated early draining vein (Fig 44-8).

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As with other spinal cord malformations, demonstration of the stula requires the painstaking injection of feeding vessels at numerous levels above and below the suspected lesion, since the main vessel of origin is often some distance away from the malformation Also, blood ow through the nidus may be so rapid and brief that it is missed In rare instances the stula or high- ow arteriovenous malformation lies well outside the cord for example, in the kidney and gives rise to a similar myelopathy, presumably by raising venous pressures within the cord..

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