Brainstem auditory evoked potentials in children with neurodegenerative diseases

Abstract

We assessed the usefulness of brainstem auditory evoked potentials (BAEPs) for differentiating types of degenerative diseases in children. The findings of BAEPs were abnormal in one out of two with polioencephalopathies and in seven of the eight with leukoencephalopathies, showing prolonged interpeak latencies of waves I-III, I-V or disappearance of wave components. On the other hand, normal responses were observed in four with corencephalopathies and one with spinocerebellopathy. The serial BAEPs in eight patients had deteriorated with progression of the clinical symptoms. These data on 16 children suggest that BAEPs are indeed a useful tool for monitoring pathophysiologic processes of neurodegenerative diseases.

Host Response and Dysfunction in the CNS during Chronic Simian Immunodeficiency Virus Infection

“…The alterations in the latencies of BSAEPs confirm our previous findings in SIV-infected monkeys and extend them to relate these abnormalities to those in the cognitive and motor spheres. Abnormalities in BSAEPs can be early indicators of neurological disruptions and have been reported in HIV infection (Goodin et al, 1990;Pagano et al, 1992;Pierelli et al, 1993;Reyes-Contreras et al, 2002), as well as in other causes of neurodegeneration and dementia including Creutzfeldt-Jakob, Alzheimer's, and a number of childhood neurodegenerative diseases (Chen et al, 1986). Although the exact mechanisms resulting in the electrophysiological alterations induced by SIV, HIV, and the other disorders are unknown, the ability to identify preclinical signs of CNS dysfunction in these conditions can enable early detection of susceptible individuals and allow the testing of measures to prevent the development of clinical sequelae.…”

Detection of Early Abnormalities in the Mucopolysaccharidoses by the Use of Visual and Brainstem Auditory Evoked Potentials

“…Finally, as already suggested by ehen et al (2), this approach could also be effective in monitoring the progression of degenerative diseases such as MPS.…”