Ataxia-telangiectasia: somatosensory, brainstem auditory and motor evoked potentials in six patients.
Six patients with ataxia-telangiectasia were given neurophysiological examinations. The patients had progressive sensorimotor axonal neuropathy that had begun at about age eight years. Brainstem auditory evoked potentials (BAEPs) showed central alternations in two of four cases. Somatosensory (BAEPs) and motor evoked potentials (MEPs) were altered in four of five cases. In the advanced stage of the disease the neurophysiological findings, except BAEPs and MEPs, resembled those in Friedreich's ataxia. MEP and BAEP results may therefore be useful for differential diagnosis.
Scarpini
C
C
Istituto di Scienze Neurologiche, Università degli Studi di Siena, Italy.
Mondelli
M
M
Guazzi
G C
GC
Federico
A
A
D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000175:diagnosis* / Q000503:physiopathology; D002648:Child; D003937:Diagnosis, Differential; D016057:Evoked Potentials, Auditory, Brain Stem; D019054:Evoked Potentials, Motor; D005073:Evoked Potentials, Somatosensory; D005260:Female; D005621:Friedreich Ataxia / Q000175:diagnosis; D006801:Humans; D008297:Male; D009482:Neurophysiology; D015203:Reproducibility of Results; D012720:Severity of Illness Index