Development , validation , and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients
OBJECT Chiari malformation Type I ( CM-I ) is a common and often debilitating neurological disease .
Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes .
Chiari I malformation related syringomyelia: radionuclide cisternography as a predictor of outcome
Summary Background . This prospect i ve study , conducted in patients with Chiari I malformation ( C I ) related syringomyelia who underwent posterior decompression and duroplasty  , utilizes radionuclide cisternography in order to study the cerebrospinal fluid ( CSF ) dynamics at the foramen magnum and to predict the clinical outcome following surgery . Methods . 17 consecutive patients of C I with syringomyelia ( but without hydrocephalus or fixed atlanto-axial dislocation ) , underwent a detailed neurological examination and were assigned a clinical disability score based on the modified Klekamp and Samii score . A radionuclide cisternography ( using Tc99m-DTPA ) was performed via the lumbar route and the ascent of the tracer was followed utilizing a gamma camera immediately after injection and then sequentially after 1 , 2 , 4 , 6 and 24 hours . After posterior decompression and duroplasty , the modified Klekamp and Samii score was repeated at follow-up visits ( range : 3 months to one year ) along with radionuclide cisternography at 3 months , and MR imaging at 6 months . Findings . Three patterns of tracer flow were observed : a ) rapid flow ( n=7 ) ; b ) supratentorial subarachnoid delay ( n=7 ) ; and , c ) foramen magnum block ( n=3 ) . The patients having foramen magnum block had the poorest clinical scores on admission . At follow up , there was an improvement in the clinical scores so that the mean scores in all three categories reached nearly the same level . Following posterior decompression , the radionuclide cisternography performed in 10 patients showed a rapid flow of the tracer without any obstruction . The syrinx resolved in 4 of the 11 patients in whom an MRI was done . Interpretation . The patients with C I with syringomyelia may often have a free flow of tracer across the FM . Posterior decompression and duroplasty provides maximum clinical relief in patients with a demonstrable foramen magnum block on radionuclide cisternography while those with a normal flow have less relief . The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist
Development , validation , and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients
OBJECT Chiari malformation Type I ( CM-I ) is a common and often debilitating neurological disease .
Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes .