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diastematomyelia

Thursday 9 February 2006

Diastomatomyelia is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra.

Diastomatomyelia is a rare congenital anomaly that results in the "splitting" of the spinal cord in a longitudinal (sagittal) direction. Females are affected much more commonly than males.

This condition occurs in the presence of an osseous (bone), cartilaginous or fibrous septum in the central portion of the spinal canal which then produces a complete or incomplete sagittal division of the spinal cord into two hemicords.

When the split does not reunite distally to the spur, the condition is referred to as a Diplomyelia (which is a "true duplication" of the Spinal Cord.)

Diastematomyelia is a rare spinal cord anomaly that usually occurs in a non-syndromal, sporadic manner. Few familial cases have been reported. The spinal column is divided by osseous or fibrous tissue.

Links

- Wikipedia

Synopsis

- variable expressivity
- X-linked dominant inheritance

References

- Sonigo-Cohen P, Schmit P, Zerah M, Chat L, Simon I, Aubry MC, Gonzales M, Pierre-Kahn A, Brunelle F. Prenatal diagnosis of diastematomyelia. Childs Nerv Syst. 2003 Aug;19(7-8):555-60. PMID: 12845458