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Electrophysiologic Study of Central Motor Pathways in Ataxia-Telangiectasia

Clinical Neurophysiology Unit

Ilaria M. Merlo, MD

Clinical Neurophysiology Unit

Virginia Monafo, MD

Department of Pediatrics, LR.C.C.S. Policlinico San Matteo, University of Pavia, Italy

Giovanni Lanzi, MD

Division of Child Neuropsychiatry, I.R.C.C.S. Fondazione Istituto Neurologico C. Mondino

Alberto Ottolini, MD

Division of Child Neuropsychiatry, I.R.C.C.S. Fondazione Istituto Neurologico C. Mondino

Pierangelo Veggiotti, MD

Division of Child Neuropsychiatry, I.R.C.C.S. Fondazione Istituto Neurologico C. Mondino

Arrigo Moglia, MD

Clinical Neurophysiology Unit

To date, corticospinal tract functional integrity in ataxia-telangiectasia has not been studied. Thorough evaluation of central motor pathways is also lacking in neuropathologic and clinical studies. Using electromagnetic stimulation, we assessed the integrity of the corticospinal tracts in eight patients with ataxia-telangiectasia. Cortical and peripheral compound motor action potentials were recorded from the abductor pollicis brevis muscle. Recordings of the shortest F-wave latency and of the compound motor action potential distal latency were made from the abductor pollicis brevis muscle after electrical stimulation of the median nerve at the wrist. A significant increase in central motor conduction time was observed in four patients, two of whom had clinical findings compatible with a pyramidal lesion. This study demonstrates involvement of the central motor pathways in ataxia-telangiectasia, which appears to be more frequent late in the course of the disease. (J Child Neurol 1997;12:327-331).

Journal of Child Neurology, Vol. 12, No. 5, 327-331 (1997)
DOI: 10.1177/088307389701200508


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