The intent of this article is to propose the existence of the tethered cord syndrome in patients whose conus is in a normal position. The tethered cord syndrome has been a well-recognized entity, occurring in the pediatric and adult population. A central tenet to this syndrome is that the conus medullaris must be "abnormally" low, regardless of the tethering lesion. Our 12-year series of 73 patients with tethered cord syndrome included 13 patients in whom the cord terminated at or above the L1-L2 space. These patients otherwise displayed characteristics usually associated with the patient with an abnormally low conus. Most patients with progressive neurological deterioration responded to surgery. The preoperative radiographic evaluation and the operative findings usually demonstrated a thickened filum. Tethered cord syndrome may occur in the patient who has a conus in the "normal" position.

Tethered cord syndrome is a progressive form of neurological deterioration that results from spinal cord tethering by various dysraphic spinal abnormalities. The syndrome, treatments, outcomes, and current controversies are reviewed.

The matrin 3 family of nuclear proteins consists of members with potentially diverse activities. Matrin 3 and NP220 share RNA-binding domains, and NP220 has been shown to recognize and bind to the DNA sequence, CCCCC (G/C). We have isolated and characterized another member of the matrin 3 family, designated NP94, from a medulloblastoma. This protein, also named Ciz1, has previously been characterized for its ability to interact with p21(Cip1/Waf1) and contains 3 zinc finger domains and a matrin 3-homologous domain 3. Our immunofluorescence and Northern blot analysis data indicate that Ciz1 is localized in the nucleus and is expressed in a wide range of tissues, especially the pancreas and the brain; within the brain, the highest message levels are found in the cerebellum. A modified selected and amplified binding (SAAB) sequence method was used to identify DNA sequences recognized by Ciz1. From the analysis of the retrieved SAAB sequences and verification using electrophoretic mobility shift assays, we formulated a consensus DNA sequence, ARYSR(0-2)YYAC, recognized by Ciz1. The potential activities of Ciz1, including those involved in brain tumorigenesis, are discussed.