References:
Case-in Point (10/10/07)
http://3s.acr.org/CIP/ArchiveCaseView.aspx?CaseId=jXPJ9Xj0CXc%3d
Case-in Point (6/28/2011)
http://3s.acr.org/CIP/CaseView.aspx?CaseId=OIWcPd/OZRg=
Q: What clinical syndrome is associated with spinal dural AVFs?
A: Foix-Alajounine Syndrome (if you pronounce this correctly, you will have no problem at Le Bernadin)
from inservice 2007 rationale
Foix Alajouanine syndrome is a myelopathy associated with a spinal dural arteriovenous fisula.The entity tends to occur in males in their 40’s and 50’s. It begins with insidious onset of lower extremity weakness or sensory deficits and may progress to paralysis if untreated. The underlying pathology is chronic venous hypertension secondary to the vascular malformation. MR findings include a normal or enlarged spinal cord with increased signal intensity on T2WI. The spinal cord may enhance and prominent vessels are often seen on the dorsal surface of the spinal cord. These findings most commonly occur within the lower thoracic spinal cord or conus medullaris.
from the 2014 inservice questions
4.
Which of the following is a TRUE statement concerning spinal dural arteriovenous fistulas?
A. The vertebral body is frequently involved.
B. Patients most commonly present with acute hemorrhage.
C. They are the most common type of spinal vascular malformation.
D. Men and women are affected equally.
Rationales:
A. Incorrect. This is a feature of the Juvenile AVM also termed type III in Spetzler classification. (I like the term metameric AVM since it involves the metamere)
B. Incorrect. Patients, most commonly elderly males, present with gradual onset of lower extremity weakness. Myelopathy as opposed to hemorrhage is identified on imaging studies in combination with subtle dilatation of spinal surface vasculature.
C. Correct. SDAVF is the most common. There is usually a single feeding radicular artery draining into pial surface veins. In most cases, treatment is endovascular unless the anterior spinal artery arises from the same level.
D. Incorrect. Males are more commonly affected than females with age of onset somewhere in the fifth to sixth decade.
This entity is important in that it is probably under diagnosed. This is especially important since treatment typically stabilizes symptoms, so the earlier the diagnosis, the better.
Note:
Who were the radiologists who defined the anatomy and pathology of this entity?
Brian Ernest Kendall, Valentine Logue of the National Hospital for Nervous Disease, Queen Square, London. Dr. Kuo York Chynn (teacher of Drs. Kagetsu) the first neuroradiologist at Cornell Hospital and later St. Luke’s Hospital (now Mount Sinai Morningside), studied at Queen Square as well as the Karolinska Institute in Stockholm.
Radiologists were instrumental in defining the pathophysiology of this entity. In addition, interventional neuroradiologists can treat this entity as well.
Pearl for neuroIR folks
Case from fellowship, MR showed cervical vascular lesion, however pt had 2 negative spinal angios, what would you do?
A: Inject the occipital. This was what Dr. Berenstein did. He demonstrated and treated the fistula.
References:
http://emedicine.medscape.com/article/1160578-overview
Article from JAMA 6/8/2015 pt presenting with paraparesis
http://archneur.jamanetwork.com/article.aspx?articleid=2300275
Foix C, Alajouanine T. La myelite nécrotique subaigue: myelite centrale angio-hypertrophique a évolution progressive: paraplégie amyotrophique lentement accendante, d’abord spasmodique, puis 15 flasque, s’accompagnant de dissociation albumino-cytologique. Rev Neurol (Paris). 1926; 33: 1–42.
Kendall BE, Logue V. Spinal epidural angiomatous malformations draining into intrathecal veins. Neuroradiology. 1977; 13: 181–189
Our colleagues have written on the subject as well:
Niimi Y, Berenstein A, Setton A, Neophytides A. Embolization of spinal dural arteriovenous fistulae: results and follow-up. Neurosurgery. 1997; 40: 675–682