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January 2022

Ataxia-telangiectasia: Somatosensory, brainstem auditory and motor evoked potentials in six patients

Author(s): Mondelli, M., Guazzi, G. C., Federico, A.

Journal/Book: Dev Med Child Neurol. 1996; 38: 40 West 20TH Street, New York, NY 10011-4211. Cambridge Univ Press. 65-73.

Abstract: 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 alterations 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.

Note: Article C Scarpini, Univ Siena, Ist Sci Neurol, Viale Bracci 2, I-53100 Siena, Italy

Keyword(s): FRIEDREICHS ATAXIA; MAGNETIC STIMULATION; INVOLVEMENT; NERVE; ABNORMALITIES; CONDUCTION; DISORDERS; ATROPHY


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