• Ling Li, Wiebke Müller-Forell, Bettina Oberman, and Rainer Boor.
• Department of Pediatric Neurology, University Children's Hospital, Mainz, Germany.
• Brain Dev. 2008 Sep 1; 30 (8): 499-503.

AbstractChildren with achondroplasia may have high cervical myelopathy from stenosis of the cranio-cervical junction resulting in neurological disability and an increased rate of sudden death. To detect myelopathy we recorded somatosensory evoked potentials after median nerve (MN) and posterior tibial nerve (PTN) stimulation in 77 patients with achondroplasia aged 0.3-17.8 years (mean 2.7 years). In addition to the conventional technique of recording the cortical components and the central conduction time (CCT) we employed non-cephalic and mastoid reference electrodes to record the subcortical waveforms N13b and P13 (MN-SEP) as well as P30 (PTN-SEP), respectively, which are generated near the cranio-cervical junction. The findings were related to the MRI results. Thirty-four patients had abnormal MRI findings including spinal cord compression (n=28) and/or myelomalacia (n=24) at or below the cranio-cervical junction. The sensitivity of the MN-SEPs was 0.74 including all abnormal upper cervical cord MRI findings (specificity 0.98), and the sensitivity was 0.79 (specificity 0.92) for cervical cord compression, respectively. The sensitivity of the PTN-SEPs was 0.52 (specificity 0.93) for all abnormal MRI findings and 0.59 (specificity 0.92) for cervical cord compression. The subcortical SEPs N13b and P13 as well as P30 were more sensitive than the conventional recordings. The MN-SEPs, notably the subcortical tracings, are useful for the detection of cervical myelopathy in children with achondroplasia. The PTN-SEPs are less sensitive. However, the tibial nerve SEPs might contribute additional information from the lumbar or thoracic spinal cord, which was, however, not tested in this study.

26 keywords...

hide…