Dae Chul Suha
aDepartments of Radiology and Research Institute of Radiology
University of Ulsan
College of Medicine
Asan Medical Center
Seoul, Korea
Kwang Kuk Kimb
bDepartment of Neurology
University of Ulsan
College of Medicine
Asan Medical Center
Seoul, Korea
Venous congestive myelopathy (VCM) often results from impaired venous outflow secondary to a spinal arteriovenous malformation.1,2 Foix-Alajouanine syndrome, also known as subacute necrotizing myelopathy or angiodysgenetic necrotizing myelopathy, is the term formerly used to describe this progressive myelopathy. One of the causes is known to be spinal dural arteriovenous fistula (SDAVF).3
McKeon et al. described the key diagnostic features that lead to the early diagnosis of SDAVF as follows: 1) a progressive rather than a subacute course; 2) the presence of abnormal, dilated vessels surrounding the cord on standard T2 sagittal MRI with subsequent confirmation of SDAVF with angiography; and 3) acute worsening even with corticosteroid therapy.4 Although they clearly described three features, at least two of them must be corrected because their wrong explanation may provide confusion leading to inappropriate management of this disease. The main reason of such inappropriate explanation seems to be derived from misunderstanding on the cause of VCM.
First, their description revealed confusion when describing the difference between SDAVF and VCM. SDAVF is the most common cause of VCM. However, SDAVF causes VCM as it is caused by regurgitation of fistular flow through the radicular vein to the perimedulary venous plexus. Therefore, VCM can be caused by any kind of spinal vascular malformation, even in pial (Fig 1) or epidural arteriovenous fistulas.5 Whatever spinal or paraspinal vascular malformations are related to VCM, disconnection of such regurgitation should be a primary target of the definitive treatment for VCM.
- Fig 1. A 30-year-old woman presented with progressive worsening of weakness in both legs during several months. (A) Sagittal T2WI showed a vascular lesion at the T11 spinal cord. Note the diffuse spinal cord edema (venous congestive myelopathy, VCM) with dilated perimedullary veins surrounding the cord. (B) Spinal angiogram showed a spinal cord arteriovenous malformation supplied by a pial feeder of the right L2 lumbar artery. (C) The VCM improved three weeks after embolization of the two pial feeders, as did the patient’s symptoms.
Second, they mention that acute worsening of neurological deficit with corticosteroid therapy occurs in SDAVF. Although such aggravation can be a feature of SDAVF, it is not always observed and sometimes there is improvement or no change in the neurological deficit.2 Once again, acute worsening with corticosteroid therapy occurs not only in SDAVF but also in VCM.
In the very late phase of VCM, there is diffuse spinal cord enhancement after gadolinium administration. In those patients, spinal cord atrophy associated with myoclonus can remain even after successful treatment, probably resulting from the venous infarction caused by prolonged VCM.6 Such venous infarction might differ in its extent and clinical presenting symptom pattern from those of other vascular territories of arterial infarction7 or from specific spinal cord lesion involvement due to other causes.8
References
1. Rodriguez FJ, Crum BA, Krauss WE, Scheithauer BW, Giannini C. Venous congestive myelopathy: a mimic of neoplasia. Mod Pathol 2004;18:710-8
2. Lee C-S, Pyun HW, Chae EY, Kim K-K, Rhim SC, Suh DC. Reversible Aggravation of Neurological Deficits after Steroid Medication in Patients with Venous Congestive Myelopathy Caused by Spinal Arteriovenous Malformation. Interventional Neuroradiology 2009;15:325-9
3. Mirich DR, Kucharczyk W, Keller MA, Deck J. Subacute necrotizing myelopathy: MR imaging in four pathologically proved cases. AJNR Am J Neuroradiol 1991;12:1077-83
4. McKeon A, Lindell EP, Atkinson JL, Weinshenker BG, Piepgras DG, Pittock SJ. Pearls & Oy-sters: Clues for spinal dural arteriovenous fistulae. Neurology 2011;76:e10-2
5. Suh DC, Choi CG, Sung KB, Kim KK, Rhim SC. Spinal osseous epidural arteriovenous fistula with multiple small arterial feeders converging to a round fistular nidus as a target of venous approach. AJNR Am J Neuroradiol 2004;25:69-73
6. Caviness JN, Brown P. Myoclonus: current concepts and recent advances. The Lancet Neurology 2004;3:598-607
7. Suh DC, Kim SJ, Jung SM, Park MS, Lee JH, Rhim SC. MRI in presumed cervical anterior spinal artery territory infarcts. Neuroradiology 1996;38:56-8
8. Suh DC, Yoo SJ, Park YS, Hong CY, Choi HY, Moon WY. MR features in patients with residual paralysis following aseptic meningitis. AJNR Am J Neuroradiol 1991;12:1234-7
