Somatosensory evoked potentials as a marker of disease burden in type 3 Gaucher disease.
作者信息
Garvey M A, Toro C, Goldstein S, Altarescu G, Wiggs E A, Hallett M, Schiffmann R
机构信息
Human Motor Control Section, Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA.
出版信息
Neurology. 2001 Feb 13;56(3):391-4. doi: 10.1212/wnl.56.3.391.
The authors compared stretch-evoked somatosensory evoked potentials (SEP) of 18 type 3 Gaucher disease (GD3) patients (two with progressive myoclonus epilepsy [PME]) with 22 age-matched normal controls and six patients with type 1 (nonneuronopathic) Gaucher disease (GD1). The mean P1-N2 SEP amplitude in GD3 patients was significantly larger than the SEP in controls and in GD1 patients, and there was a significant negative correlation between SEP amplitude and the IQ of GD3 patients. The authors conclude that abnormal cortical inhibition is a unifying feature of GD3 patients and correlates with the degree of cognitive deficit.
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