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Brandon G. Rocque, Timothy M. George, John Kestle and Bermans J. Iskandar

C hiari malformation Type I is commonly associated with syringomyelia. The clinical presentation of CM-I with a syrinx varies from the incidentally diagnosed asymptomatic patient to the patient with severe neurological deficits. Three pediatric neurosurgical surveys have been previously conducted to determine if there is consensus regarding indications for operative treatment and choice of surgical procedure. 1 , 3 , 4 In this article, we report the results of a survey administered at the 2006 meeting of the ASPN to all members present. The survey was

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Marcy C. Speer, Timothy M. George, David S. Enterline, Amy Franklin, Chantelle M. Wolpert and Thomas H. Milhorat

In several reports the authors have suggested occasional familial aggregation of syringomyelia and/or Chiari 1 malformation (CM1). Familial aggregation is one characteristic of traits that have an underlying genetic basis. The authors provide evidence for familial aggregation of CM1 and syringomyelia (CM1/S) in a large series of families, establishing that there may be a genetic component to CM1/S in at least a subset of families. The authors observed no cases of isolated familial syringomyelia in their family studies, suggesting that familial syringomyelia is more accurately classified as familial CM1 with associated syringomyelia.

These data, together with the cosegregation of the trait with known genetic syndromes, support the authors' hypothesis of a genetic basis for some CM1/S cases.

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Timothy M. George and Lisa H. Fagan

and thoracolumbar syringomyelia presenting slow progressive muscular atrophy in the lower limbs.] Rinsho Shinkeigaku 34 : 918 – 921 , 1994 (Jpn) Fujimura Y, Kimura F, Ishida S, Shinoda K, Ohsawa N: [An adult case of tethered cord syndrome with lipoma and thoracolumbar syringomyelia presenting slow progressive muscular atrophy in the lower limbs.] Rinsho Shinkeigaku 34: 918–921, 1994 (Jpn) 11. George TM , Gabriel EM : Repair of meningoceles , in Rengachary SS , Wilkins RH (eds): Neurosurgical Operative

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Timothy M. George and Lisa H. Fagan

myelomeningocoele and tethered cord syndrome: good surgical outcome after abrupt neurological decline. Br J Neurosurg 9: 659–666, 1995 10. Fujimura Y , Kimura F , Ishida S , Shinoda K , Ohsawa N : [An adult case of tethered cord syndrome with lipoma and thoracolumbar syringomyelia presenting slow progressive muscular atrophy in the lower limbs.] Rinsho Shinkeigaku 34 : 918 – 921 , 1994 (Jpn) Fujimura Y, Kimura F, Ishida S, Shinoda K, Ohsawa N: [An adult case of tethered cord syndrome with lipoma and thoracolumbar

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Joseph L. Koen, Roger E. McLendon and Timothy M. George

transcripts in the splotch (Sp) mouse mutant. Proc Natl Acad Sci USA 90: 532–536, 1993 23. Friedman NB , van de Velde RL : Germ cell tumors in man, pleiotropic mice, and continuity of germplasm and somatoplasm. Hum Pathol 12 : 772 – 776 , 1981 Friedman NB, van de Velde RL: Germ cell tumors in man, pleiotropic mice, and continuity of germplasm and somatoplasm. Hum Pathol 12: 772–776, 1981 24. Gardner WJ : Diastematomyelia and the Klippel-Feil syndrome. Relationship to hydrocephalus, syringomyelia