Consecutive index cases with trinucleotide repeat spinocerebellar ataxia (SCA) presenting from August 2006 to August 2008 to a tertiary care university department were studied clinically, radiologically and genetically (repeat expansions at SCA 1, 2, 3, 6, 7, 10 and 12). CAG repeat expansions were seen in 14 patients. Inverse relationship between CAG repeats and age of presentation was conspicuous. SCA 2 (10/14) was the commonest followed by SCA 6 (2/14), SCA 3 (1/14) and SCA 1(1/14). In one patient of SCA 6, ‘hot cross bun’ sign was seen on MRI Brain. Sixteen cases were negative for the genetic tests.

Spinal dural arteriovenous fistulae (AVF) are rare and can result in spinal cord dysfunction. We present one such case wherein the patient presented with a venous congestive myelopathy. Magnetic resonance imaging showed a syrinx formation, spinal cord edema, and flow voids. Digital subtraction angiography confirmed the dural AVF, which was treated with embolization. The syrinx disappeared, other spinal cord changes improved, and the patient had remarkable clinical improvement. The case is presented to draw attention to the rare formation of a syrinx in a spinal dural arteriovenous fistula and its disappearance after successful embolization.