Pontine hyperperfusion in sporadic hyperekplexia

作者: R. Vetrugno , M. Mascalchi , A. Vella , R. D. Nave , L. Guerrini

DOI: 10.1136/JNNP.2006.113837

关键词: BrainstemStartle reactionNeurosciencePsychologyHyperekplexiaCingulate cortexCerebral blood flowPonsSuperior frontal gyrusNeuroimaging

摘要: Objective: To explore with neuroimaging techniques the anatomical and functional correlates of sporadic hyperekplexia. Methods: Two elderly women hyperekplexia underwent neurophysiological assessment, MRI brain proton magnetic resonance spectroscopy ( 1 H-MRS) brainstem frontal lobes. Regional cerebral blood flow was investigated single photon emission tomography (SPECT) during evoked startles at rest. Results: Both patients showed excessively large non-habituating startle responses. In both patients, impingement by vertebrobasilar artery, lack or abnormalities on H-MRS hyperperfusion in dorsal pons cingulate cortex, superior gyrus SPECT startles. Conclusions: our hyperekplexia, arteries were found to impinge brainstem. Neurophysiological findings neurofunctional imaging indicated a pontine origin movement disorder modulated activation cortical, especially frontal, areas. The human are similar those observed for circuit animals.

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