Neuropediatrics, Table of Contents Neuropediatrics 1998; 29(3): 124-126DOI: 10.1055/s-2007-973547 Original articles © Hippokrates Verlag GmbH Stuttgart Impaired Peripheral Somatosensory Function in Children with Prader-Willi Syndrome Brita Ryde Brandt1 , I. Rosén2 1Center of Child and Youth Habilitation, Lund, Sweden, 2Department of Clinical Neurophysiology, University Hospital, Lund, Sweden Recommend Article Abstract PDF Download Buy Article Abstract The Prader-Willi syndrome (PWS) is associated with a tendency to self-injury and a reduced sensitivity to painful stimuli. Somatosensory functions were studied in 5 children aged 11-13 years with PWS. Tactual perception in the hands (stereognosis) was apparently normal in 4 of them. Sensory nerve conduction velocities in the median nerve and latencies for sensory evoked potentials were similar in the PWS subjects and in 10 healthy controls indicating a preserved myelinisation of sensory nerve fibers in PWS. Sensory nerve action potential amplitudes in the PWS group were on an average only 40-50 % of normal size (p = 0.03), suggesting a reduced number of normal axons in the median nerve. The results may be relevant for the impaired pain sensitivity in PWS because similar neurographic findings and a low density of peripheral nerve fibers have been reported in patients with hereditary or congenital insensitivity to pain. Key words Prader-Willi syndrome - Somatosensory functions - Sensory nerve conduction velocities - Sensory nerve action amplitudes - Pain sensitivity - Self injury PDF (409 kb)
