Singing paraplegia: a distinctive manifestation of spinal dural arteriovenous fistula

Khurana VG, Perez-Terzic CM, Peterson RC et al. Neurology 2002; 58: 1279-1281

Case is included because of the extreme difficulty diagnosing spinal dural av fistulas. They may be worse with upright posture, ambulation, pregnancy and menstruation.

The case is a baritone who had a progressive myelopathy with recurrent paraplegia while singing. (He changed from singing while standing to singing while sitting). He had had insidious bilateral leg weakness while bending forward, without pain or bowel or bladder dysfunction. He did have chronic numbness and paresthesias in his feet. He had T2 signal change in mid to low thoracic area, later from T7 to conus with an enlarged cord. A spinal angiogram showed the fistula with an arterialized vein. The fistula was obliterated surgically and the patient became much better, ambulated better and could sing while standing.

The authors discuss that the slowly progressive myelopathy is due to chronic venous hypertension with postnidal venous engorgement and intraparenchymal cord edema and ischemia. Despite the location of the fistula itself, the MRI changes are almost always in the lower cord , attributed to orthostasis and exacerbated with ambulation. In this case, increased venous presure with Vasalva (respiratory exertion during singing) exacerbated venous engorgement and cord edema and ischemia (the pathogenesis involves "marginal" venous drainage).

Common misdiagnoses include disc herniation, canal stenosis, Guilian Barre syndrome, subacute combined degeneration, transverse myelitis, or spinal cord neoplasia. Many misdiagnoses lead to irreversible neurologic changes.