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Updated: Jul 23, 2026
09:38
Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities
Published on: January 29, 2014
Impaired peripheral somatosensory function in children with Prader-Willi syndrome
1Center of Child and Youth Habilitation, Lund, Sweden.
Neuropediatrics
|August 26, 1998
Summary
Children with Prader-Willi syndrome (PWS) show reduced sensory nerve action potential amplitudes, suggesting fewer normal axons. This may explain their impaired pain sensitivity and self-injury tendencies.
Area of Science:
- Neuroscience
- Genetics
- Pediatrics
Background:
- Prader-Willi syndrome (PWS) is characterized by self-injury and reduced pain sensitivity.
- Understanding the neurophysiological basis of sensory deficits in PWS is crucial.
Purpose of the Study:
- To investigate somatosensory functions in children with Prader-Willi syndrome.
- To explore potential neurophysiological mechanisms underlying impaired pain sensitivity in PWS.
Main Methods:
- Somatosensory function assessment in 5 children (11-13 years) with PWS.
- Evaluation of tactual perception (stereognosis).
- Neurographic studies including sensory nerve conduction velocities and evoked potentials of the median nerve, compared to 10 healthy controls.
Main Results:
- Stereognosis was normal in 4 out of 5 PWS subjects.
- Sensory nerve conduction velocities and evoked potential latencies were comparable between PWS and control groups.
- Sensory nerve action potential amplitudes were significantly reduced (40-50% of normal) in the PWS group (p=0.03), indicating a reduced number of functional axons.
Conclusions:
- Preserved myelination of sensory nerve fibers in PWS.
- Reduced sensory nerve action potential amplitudes suggest axonal loss, potentially explaining impaired pain sensitivity.
- Findings align with reports on hereditary pain insensitivity, suggesting a shared neurophysiological basis.
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