'Developmental impairment that is not immaturity'.
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Angelman Syndrome↗
Biomedical subjects
Guy Chéron
Publications and source records attributed to Guy Chéron.
Postural rhythmic muscle bursting activity in Angelman syndrome.
Postural impairment is one of the most consistent features of Angelman syndrome. Using multiple-channel electromyography, we studied a lower limb and an upper limb isometric postural task in 14 patients with Angelman syndrome and 18 unimpaired control subjects. Both tasks were associated with synchronous bursts of activity at frequencies of 6-8 s(-1) in all recorded muscles in all patients with Angelman syndrome and none of the control subjects. This pattern was not altered by extra-loading. Electroencephalogram recorded during the upper limb task showed no change in relation to the task. Burst-locked back-averaging of the electroencephalogram showed no spiking before or during the bursts. Various physiological and pathological rhythmic muscle activities have been proposed to be a manifestation of oscillations in the central nervous system and it has been suggested that such oscillations may have a role in the processing of motor commands. The mechanism of the rhythmic muscle bursting activity associated with maintaining posture in patients with Angelman syndrome is not clear, although it could be consistent with cerebellar Purkinje cell dysfunction, either as a pathological feature or as an adaptive process to overcome deficits in motor coordination.
Action Potentials↗
Gait control in spinal palsy.
Developmental motor impairment with lower limb spasticity most commonly corresponds to cerebral palsy of the spastic diplegia type. Here we describe a 4-year-old girl whose locomotor phenotype reflects early cortico-spinal lesion at the spinal level. This child has developmental spastic paraparesis secondary to D4-D8 cord compression. We analysed her gait using the ELITE optoelectronic system and compared it to that of six normal age-matched controls and six age-matched children with leucomalacic spastic diplegia. Gait characteristics of the patient included preservation of head orientation and arm swing similar to findings in normal controls and contrasting with children with spastic diplegia. She also had truncal instability and displayed lack of selectivity in lower limb movement as in spastic diplegia and in contrast with normal controls. This may reflect differences in locomotor control between developmental spasticity of cerebral and spinal origin. The latter might correspond to spinal palsy defined as abnormal movement and posture secondary to non-progressive pathological processes affecting the immature spinal cord.
Arm↗