Familial adhesive arachnoiditis associated with syringomyelia

Abstract

Adhesive arachnoiditis is a rare condition, often complicated by syringomyelia. This pathologic entity is usually associated with prior spinal surgery, spinal inflammation or infection, and hemorrhage. The usual symptoms of arachnoiditis are pain, paresthesia, and weakness of the low extremities due to the nerve entrapment. A few cases have had no obvious etiology. Previous studies have reported one family with multiple cases of adhesive arachnoiditis. We report a second family of Belgian origin with multiple cases of arachnoiditis and secondary syringomyelia in the affected individuals.

Fig 1.

The pedigree of the Belgian family with spinal arachnoiditis and secondary syringomyelia. Squares and circles represent males and females, respectively. Solid symbols represent the affected family members.

Fig 2.

Sagittal T2-weighted MR image. Note enlargement of the spinal cord with a syringomyelic cavity extending from levels T4 to T8 (arrow). At the T5–T6 levels, posterior to the cord, a band-like low-signal-intensity structure represents a fibrous thickening of the arachnoid matter (arrowhead). The superior half of the cavity, just above the arachnoid thickening, is much more dilated than the inferior half.

Fig 3.

Intraoperative photograph confirming the presence of a thick and adherent arachnoid web (arrow), depicted by MR imaging.

Fig 4.

The pathologic examination demonstrated a thick membrane, rich in collagen limited by meningoepithelial cells. There is no evidence of inflammation, infection, or old hemorrhage (H&E stain).