[A case of Larsen syndrome with severe cervical cord compression]

We reported a case of Larsen syndrome with cervical cord compression. She had a flattened face, bilateral joint dislocations of the elbows, hips and knees, and equinovalgus deformity on the feet. At first she had flaccidity of the upper and lower limbs, she gradually developed spastic. On laboratory examination, CSF protein was elevated. EMG showed fibrillation. In short latency somatosensory evoked potential (SSEP), N 20 was not detected. MRI showed a severe cervical cord compression. Cervical spine deformity has been often described in the previous reports on Larsen syndrome, but cervical cord compression demonstrated by MRI was reported in only one case. We should take into consideration this risk associated with Larsen syndrome.