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Study of Multimodal Evoked Potentials in Patients With Type 1 Gaucher's Disease

Department of Neurological Sciences, Federico II University, Naples, Italy, anperret{at}unina.it

Giancarlo Parenti, MD

Department of Pediatrics Federico , University, Naples, Italy

Pietro Balbi, MD

Salvatore Maugeri Foundation IRCCS, Rehabilitation Center of Telese (BN), Italy

Luigi Titomanlio, MD

Department of Pediatrics, Federico II University, Naples, Italy

Lucia Marcantonio

Department of Neurological Sciences, Federico II University, Naples, Italy

Mariangela Iapoce

Department of Neurological Sciences, Federico II University, Naples, Italy

Anna Rita Frascogna, MD

Department of Pediatrics Federico University, Naples, Italy

Generoso Andria, MD

Department of Pediatrics, Federico University, Naples, Italy

Lucio Santoro, MD

Department of Neurological Sciences, Federico University, Naples, Italy

To detect early subclinical nervous dysfunction in Gaucher's disease type 1, we carried out motor, brainstem auditory, visual, and somatosensory evoked potentials in 17 patients with Gaucher's disease type 1. Central motor evoked potential abnormalities were found in nine patients (69.2%), consisting of an increased motor threshold in all, with prolonged central motor conduction time in two patients. Brainstem auditory evoked potentials were abnormal in five patients (31.2%), and the most frequent abnormality was a bilateral increased I—III interpeak latency. Visual evoked potentials showed a delayed latency of the P100 wave in four patients (25%). Somatosensory evoked potential abnormalities were found in three patients (18.7%), consisting of an increased N13—N20 interval in two patients and a not reproducible N13 wave in one patient. Our findings suggest that the multimodal evoked potential approach provides information about nervous subclinical damage in Gaucher's disease type 1; transcranial magnetic stimulation proved to be the most sensitive tool. Early detection of subclinical neurologic dysfunction can be useful in view of more effective therapeutic strategies. (J Child Neurol 2005;20:124—128).

Journal of Child Neurology, Vol. 20, No. 2, 124-128 (2005)
DOI: 10.1177/08830738050200020801


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