Clinical and neuroimaging features of “idiopathic” syringomyelia
Abstract
In some adult patients with cervical syringomyelia, MRI studies do not identify primary disease within the foramen magnum or spinal canal. To identify the etiology of this idiopathic type of syringomyelia, clinical features and posterior fossa (PF) measurements from 17 of these patients, 17 patients with Chiari I-type syringomyelia, and 32 control subjects were compared. Idiopathic syringomyelia and Chiari I-type syringomyelia manifested central cervical myelopathy and a small PF with narrow CSF spaces, suggesting that they develop by the same mechanism.
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Received: February 14, 2003
Accepted: November 6, 2003
Published online: March 8, 2004
Published in print: March 9, 2004
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Our conclusion that the posterior fossa and CSF pathways are smaller than normal in Chiari I-syringomyelia and in idiopathic syringomyelia is supported by findings of significant differences between these groups and the control group in many comparisons. Of the nine continuous variables measured in this study, statistically significant differences were found in nine of nine comparisons of idiopathic syringomyelia versus control and in eight of nine comparisons of Chiari I versus control.
Use of the Bonferroni correction is appropriate when multiple independent variables are tested. In our study, variables (factors) were dependent and therefore not appropriate for the Bonferroni correction. Five variables indirectly measured the volume of the posterior fossa: 1) posterior fossa height; 2) Klaus index; 3) length of the subocciput; 4) length of the clivus; 5) Boogaard angle-three variables measured the size of CSF pathways; 6) distance c (cerebellum to opisthion); 7) ventral subarachnoid space; 8) dorsal subarachnoid space and 1 determined brainstem position; and 9) pontomedullary (pm) junction to foramen magnum.
To simplify the table, the usual standard of statistical significance (p <0.05) was used, although p values were in fact lower (p <_0.01 than="than" that="that" for="for" many="many" comparisons.="comparisons." variables="variables" indirectly="indirectly" measured="measured" posterior="posterior" fossa="fossa" volume="volume" p="p" values="values" were="were" _="_" _0.01="_0.01" height="height" ki="ki" and="and" boogaard="boogaard" angle="angle" in="in" the="the" idiopathic="idiopathic" syringomyelia="syringomyelia" versus="versus" control="control" comparison="comparison" length="length" of="of" clivus="clivus" chiari="chiari" i="i" comparison.="comparison." indicating="indicating" size="size" csf="csf" pathways="pathways" pm="pm" distance="distance" was="was" p0.01="p0.01"/>Gender distribution of the groups was similar (29 men and 3 women in the control group, 15 men and 2 women in the idiopathic syringomyelia group, and 13 men and 4 women in the Chiari I group), and statistical analysis confined to men gave similar results to those that we reported. Two neuroradiologists with 20 years of MRI experience measured the skull and brain structures by standard methods and the presence or absence of a syrinx did not affect their measurements. We believe that this information further supports the validity and methodology of our study.
Bogdanov et al conclude that idiopathic syringomyelia is due to short bones in the posterior fossa and narrowing of the CSF pathways despite an absence of cerebellar tonsillar herniation. However, gender/age of the control group were not provided.
Although the groups were of similar age, gender matching was not done. This could have been a confounder especially because of differences in measurements of areas within the posterior fossa (including bony structures) between men and women.
The study measured parameters on MRI but no information was given on the number or seniority of the radiologists taking such measurements or if they were blind to the type of syringomyelia. This could have been done by obscuring the area caudal to the foramen magnum on the image so that the presence or absence of syringomyelia would not be visible.
Areas for further research should have been addressed, including larger studies minimizing the above potential confounders and using alternative methods of assessing differences between the two groups of syringomyelia, including Cine-MRI. [2]
Finally, the statistically methodology did not take into consideration multiple testing of a small data set. Had a Bonferroni correction been done, the results would not have been significant. The study is important and interesting but we consider it to methodologically flawed.
References
1. Bogdanov EI, Heiss JD, Mendelevich EG, Mikhaylov IM, Haass A. Clinical and neuroimaging features of "idiopathic" syringomyelia. Neurology 2004;62:791-794.
2. Heiss JD, Patronas N, DeVroom HL, Shawker T, Ennis R, Kammerer W, Eidsath A, Talbot T, Morris J, Eskioglu E, Oldfield EH. Elucidating the pathophysiology of syringomyelia. J Neurosurgery 1999;91:553-562.