Background: Kernicterus is a relatively rare disease in Germany but more common in developing countries. The resulting dyskinetic movement disorder has a negative impact on the quality of life of patients and their families and can often not be controlled sufficiently by oral medication. An intrathecal baclofen therapy is an established therapeutic trial in patients with spastic and also dystonic movement disorders. There are no reports about the efficacy in kernicterus.
Patient: We report a 12 year old boy, born in Chechen, with a dystonic-athetoid cerebral palsy since birth. He was suffering from a severe physical disability (GMFCS V, MACS V) but had good mental abilities. Especially the unrelievable pain despite adequate medication led to a seriously impaired quality of life and depressive mood. The typical affection of the globus pallidus on both sides on cranial MRI allowed us to diagnose kernicterus. Several trials with oral anti-dystonic medication (Baclofen, Tetrabenazin, Trihexyphenidyl, Gabapentin) were unsuccessful and we therefore decided to implant a baclofen pump. Hoping to enhance efficacy in dystonic-athetoid movement disorder and to eventually allow a two-stage spinal surgery procedure for the advanced scoliosis, the catheter tip was positioned in the third ventrical.
Results: The intraventricular application of baclofen (487 µg/day) was able to clearly reduce pain and the dystonic-athetoid movement disorder. Additionally, an improvement of intended movements was seen. Also sleep and depressive mood were improved. As a side effect nausea and vomiting were observed dose dependently while increasing the dose too fast. Trunk and head control were insignificantly decreased.
Discussion: 1. The intraventricular baclofen therapy is an effective therapeutic option with only few side effects in patients with severe dystonic-athetoid movement disorder, including kernicterus. 2. The intrathecal catheter position might offer benefits compared with the common intraspinal catheter position.
