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.
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Abbreviations
- ALD:
-
adrenoleukodystrophy
- BAEPs:
-
brainstem auditory evoked potentials
- CT:
-
computed tomography
- EEG:
-
electroencephalography
- HL:
-
hearing level
- IPL:
-
interpeak latency
- MLD:
-
metachromatic leukodystrophy
- SSPE:
-
subacute sclerosing panencephalitis
- VEPs:
-
visual evoked potentials
References
Brown FR, Shimizu H, McDonald JM, Moser AB, Marquis P, Chen WW, Moser HW (1981) Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy. Neurology 31:980–985
Davis SL, Aminoff MJ, Berg BO (1985) Brainstem auditory evoked potentials in children with brainstem or cerebellar dysfunction. Arch Neurol 42:156–160
Dyken P, Krawiecki N (1983) Neurodegenerative disease of infancy and childhood. Ann Neurol 13:351–364
Fujita M, Hosoki M, Miyazaki M (1981) Brainstem auditory evoked responses in spinocerebellar degeneration and Wilson disease. Ann Neurol 9:42–47
Fullerton PM (1964) Peripheral nerve conduction in metachromatic leucodystrophy (sulphatide lipidosis). J Neurol Neurosurg Psychiatry 27:100–105
Garg BP, Markand ON, DeMyer WE, Warren C (1983) Evoked response studies in patients with adrenoleukodystrophy and heterozygous relatives. Arch Neurol 40:356–359
Gloor P, Kalabay N, Giard N (1968) The electroencephalogram in diffuse encephalopathies: electroencephalographic correlates of grey and white matter lesions. Brain 91:779–802
Grimes AM, Elks ML, Grunberger G, Pikus AM (1983) Auditory brain-stem responses in adrenomyeloneuropathy. Arch Neurol 40:574–576
Hecox KE, Cone B, Blaw ME (1981) Brainstem auditory evoked response in the diagnosis of pediatric neurologic diseases. Neurology 31:832–845
Markand ON, Ochs R, Worth RM, DeMyer WE (1980) Brainstem auditory evoked potentials in chronic degenerative central nervous system disorders. In: Barber C (ed) Evoked Potentials, MTP, Lancaster, pp 367–374
Moloney JB, Masterson JG (1982) Detection of adrenoleucodystrophy carriers by means of evoked potentials. Lancet iii:852–853
Moosa A (1971) Peripheral neuropathy and ichthyosis in Krabbe's leucodystrophy. Arch Dis Child 46:112–113
Roach ES, Ford CS, Spudis EV, Riela AR, McLean WT, Gilliam J, Ball MR (1985) Wilson's disease: evoked potentials and computed tomography. J Neurol 232:20–23
Starr A, Achor LJ (1975) Auditory brainstem responses in neurological disease. Arch Neurol 32:761–768
Stockard JJ, Rossiter VS (1977) Clinical and pathologic correlates of brainstem auditory evoked response abnormalities. Neurology 27:316–325
Stockard JJ, Stockard JE, Sharbrough FW (1977) Detection and localization of Occult lesions with brainstem auditory responses. Mayo Clin Proc 52:761–769
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Chen, Y.J., Kurokawa, T., Mitsudome, A. et al. Brainstem auditory evoked potentials in children with neurodegenerative diseases. Eur J Pediatr 145, 471–474 (1986). https://doi.org/10.1007/BF02429045
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DOI: https://doi.org/10.1007/BF02429045