Summary
The diagnosis of tethered cord syndrome (TCS) without typical conus medullaris symptoms and the radiological features such as a low set conus medullaris or dysraphic malformation is difficult. We report 11 year old identical twin brothers with TCS associated with the conus at the normal level. Their presenting symptom was progressive leg pain and both patients underwent surgical interruption of the filum terminale. The pain recurred in one patient treated surgically only after symptom became worse but resolved immediately in the other sibling treated promptly. We indicate the importance of early diagnosis and treatment of TCS to obtain excellent long-term outcome despite the absence of a low set conus or specific symptoms. Furthermore, when a twin or sibling of an affected person has neurological symptoms and the cutaneous signature of spinal dysraphism, radiological examination should be performed to establish the cause.
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Comment
This is a short report of tethered cord syndrome in a pair of identical twins. Dysraphic and paradysraphic malformations in identical and non-identical twins, and among non-twin siblings, have been well recognized. Their occurrences are considerably more common than might be inferred from the paucity of published reports. The malformations in each twin or sibling may be identical, as in this case (filum–filum), or different, e.g., split cord malformation (SCM)–lipoma, transitional lipoma–dorsal lipoma, lipoma–filum, or SCM–filum. If a twin or sibling of an affected person has compatible symptoms or cutaneous signature of spinal dysraphism, he or she should have an MRI, as the authors emphasized.
The location of the conus has not been refuted as an important indicator of cord tethering, so the authors’ point in this regard is well taken. Certainly, the presence of a fatty filum in face of unrelenting leg pain is a sound reason for surgery.
The leg pain in patient 1 is highly characteristic of chronic central pain. It is well known central pain of spinal cord origin takes a long time to recede after relief of the cause. Thus, this child’s arduous course is understandable.
Tethered cord syndrome in twin and non-twin siblings is relatively rare and this report is thus of important archival value.
Dachling Pang
University of California, Davis
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Mitsuka, K., Horikoshi, T., Watanabe, A. et al. Tethered cord syndrome in identical twins. Acta Neurochir (Wien) 151, 85–88 (2009). https://doi.org/10.1007/s00701-008-0170-8
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DOI: https://doi.org/10.1007/s00701-008-0170-8