FAMILIAL SYRINGOMYELIA AND STATUS DYSRAPHICUS

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Abstract
The authors have investigated sevefel members of a family comprising two generations, the majority of whom present structural anomalies and stigmata of degeneration of strikingly similar character. Genetic study of this group indicates the taint is dominant and is transmitted from a [male] to offsprings of either sex, other families reported in the literature suggest that it may be transmitted through the [female] as well. Many of the individuals had trophic ulcers of the feet, vasomotor disturbances and dissociation of sensation, particularly temp. and pain suggestive of lumbo-sacral syringomyelia. The relatively static nature of the disease, the dominant hereditary tendency and the proclivity towards the lower extremities is more often found in status dysraphicus than in syringomyelia. Fuchs in 1903 was the first to give the name "myelodysplasia" to a syndrome which, he said, is a picture of syringomyelia, but without progression. Twenty yrs. later Bremer differentiated this mylodysplasia from syringomyelia and since that time the entity has been known as status dysraphicus. The authors feel that a diagnosis of syringomyelia in an individual makes it manda- tory that his siblings be examined for similar taints, especially when the condition is more static than ordinarily seen in this disease, thereby reducing morbidity and possibly sparing of future generations.

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