Aarskog syndrome: severe neurological deficit with spastic hemiplegia resulting from perinatal cerebrovascular accidents in two non-related males.
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The results of somesthetic evoked response (SER) and electroencephalogram (EEG) records in 43 children with hemiplegic cerebral palsy have been compared. In all children there was a positive correlation between the SER changes and the affected side of the body. In 12 children the SER of the "damaged" hemisphere disappeared; in 31 children it was less than the response of the "healthy" hemisphere. On the other hand, a positive correlation between the EEG changes and the affected side was found in only 16 of the patients, and there was a dubious correlation in three cases. It is concluded that SER data are more accurate and reliable than EEG results. Moreover, SER findings allow cerebral damage to be suspected earlier than the EEG results and therefore are of greater prognostic value.
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To assess the impact of neurologic involvement on bone mineralization, dual-energy X-ray absorptiometry was used to quantitate bone mineral content (BMC), bone mineral density (BMD), fat and lean muscle mass in the limbs of 19 children with spastic hemiplegic cerebral palsy. The BMC in the affected limb was on average 26.5% lower (upper limbs) and 15.6% lower (lower limbs) than in the corresponding uninvolved limb. Lean muscle mass was reduced by 15% and BMD by 6% in the involved limbs and did not differ significantly between upper and lower limbs. The fat content of involved and uninvolved limbs did not differ. Children with poor hand function had greater reductions in BMC (39.3%), BMD (11.3%) and lean muscle mass (22.5%) than did children with better hand function. Thus, bone size and density decrease with increasing neurologic involvement, and weight bearing may slightly lessen the effect.
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