| Institution: | 1. Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, London SE5 9RX, United Kingdom;2. Complex Motor Disorder Service, Children’s Neurosciences Department, Evelina Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EH, United Kingdom;3. Department of Clinical Neurophysiology, King’s College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, United Kingdom;4. Moorfields Eye Hospital NHS Foundation Trust, 162 City Road, London EC1V 2PD, United Kingdom;5. University College London Institute of Ophthalmology, 11-43 Bath St, London EC1V 9EL, United Kingdom;6. F. Hoffmann-La Roche AG, Biostatistics, 4070 Basel, Switzerland;7. Department of Psychology, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, United Kingdom;8. Department of Radiology, Guy’s and St. Thomas’ NHS Foundation Trust, London, United Kingdom;9. Department of Neuroradiology King’s College Hospital NHS Foundation Trust, London, United Kingdom;10. Department of Functional Neurosurgery, King’s College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, United Kingdom |
| Abstract: | ObjectivesTo report Somatosensory Evoked Potentials (SEPs) and Central Motor Conduction Times (CMCT) in children with dystonia and to test the hypothesis that these parameters predict outcome from Deep Brain Stimulation (DBS).Methods180 children with dystonia underwent assessment for Globus pallidus internus (GPi) DBS, mean age 10?years (range 2.5–19). CMCT to each limb was calculated using Transcranial Magnetic Stimulation. Median and posterior tibial nerve SEPs were recorded over contralateral and midline centro-parietal scalp. Structural abnormalities were assessed with cranial MRI. One-year outcome from DBS was assessed as percentage improvement in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-m).ResultsAbnormal CMCTs and SEPs were found in 19% and 47% of children respectively and were observed more frequently in secondary than primary dystonia. Of children proceeding to DBS, better outcome was seen in those with normal (n?=?78/89) versus abnormal CMCT (n?=?11/89) (p?=?0.002) and those with normal (n?=?35/51) versus abnormal SEPs (n?=?16/51) (p?=?0.001). These relationships were independent of dystonia aetiology and cranial MRI findings.ConclusionsCMCTs and SEPs provide objective evidence of motor and sensory pathway dysfunction in children with dystonia and relate to DBS outcome.SignificanceCMCTs and SEPs can contribute to patient selection and counselling of families about potential benefit from neuromodulation for dystonia. |
