CHANGES OF BRAINSTEM AUDITORY AND SOMATOSENSORY EVOKED POTENTIALS IN LATE INFANTILE FORM OF METACHROMATIC LEUKODYSTROPHY

Yang J, Chen Q, Yang WM   

Department of neurology, Capital Institute of Pediatrics, Beijing, China

 

Objective: To investigate the characteristics and clinical value of evoked potentials in late infantile form of metachromatic leukodystrophy. 

Methods:  Brainstem auditory and somatosensory evoked potentials were recorded in 6 patients, and compared with the results of CT scan. 

Results:  All of the 6 patients had abnormal results of BAEP and MNSEP.  The main abnormal parameters in BAEP were latency prolongation in wave I, inter-peak latency prolongation in I-III and I-V.  The abnormal features of MNSEP were low amplitude and absence of wave N9, inter-peak latency prolongation in N9-N13 and N13-N20, but no significant change of N20 amplitude.  The results also revealed that abnormal changes in BAEP and MNSEP were earlier than that in CT. 

Conclusion:  The detection of BAEP and MNSEP in late infantile form of metachromatic leukodystrophy might early reveal the abnormality of conductive function in nervous system and might be a useful method in diagnosis.

 

 
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