Abstract
Purpose
Olivopontocerebellar atrophy (OPCA), a variant of spinocerebellar degeneration (Shy-Drager syndrome), is a systemic degenerative disorder affecting the neurons of multiple nuclei. We investigated the sensitivity to vecuronium and the pupillary responses to various stresses in a patient with OPCA.
Clinical features
A 65-yr-old woman with a six-month history of OPCA underwent a left upper lobectomy for lung cancer under propofol-N2O anaesthesia. She had symptoms of dysarthria, bulbar palsy, cerebellar ataxia, Parkinsonism, myosis, pyramidal signs and muscular atrophy of the distal extremities. A cumulative dose-response curve for vecuronium was constructed, and pupillary changes in response to various noxious stimuli were evaluated with concomitant recording of the Spectral-Edge-Frequency 90% (SEF90; the frequency below which 90 percent of the EEG power is located). The dose-response curve for vecuronium and the estimated ED50 value (the 50% blocking dose of vecuronium) in this patient with OPCA were almost identical with those of five ASA 1–11 patients (27 μg·kg−1 vs 31 μg·kg−1). The pupil size and the SEF90 did not change after tracheal intubation or surgical stimulation in this patient, while in the control subjects (n = 3), these measures increased in response to both stresses.
Conclusions
The absence of pupillary and SEF90 responses to noxious stimuli suggests a sensitivity to propofol and/or central autonomic dysfunction in patients with OPCA. Although the dose requirement of vecuronium in this patient was similar to that of the control patients, the effects of neuromuscular blockers may vary depending on the severity of muscle atrophy.
Résumé
Objectif
L’atrophie olivo-ponto-cérébelleuse (AOPC), une variante de la dégénérescence spinocérébelleuse (syndrome de Shy et Drager), est une atteinte dégénérative généralisée qui affecte les neurones de multiples noyaux. Nous avons examiné la sensibilité au vécuronium et les réactions pupillaires à différentes stimulations chez une patiente souffrant d’AOPC.
Aspects cliniques
Une femme de 65 ans souffrant d’AOPC depuis six mois a subi une lobectomie supérieure gauche, pour traiter un cancer du poumon, sous anesthésie avec propofol-N2O. Elle présentait les symptômes suivants : dysarthrie, paralysie bulbaire, ataxie cérébelleuse, parkinsonisme, miose, signes pyramidaux et atrophie musculaire des extrémités distales. Une courbe dose-réponse cumulative pour le vécuronium a été élaborée et les changements pupillaires, en réaction à différents stimuli désagréables, ont été évalués à partir d’un enregistrement concomitant de fréquence spectrale limite à 90 % (FSL90; la fréquence sous laquelle se situe 90 pour cent de l’activité de l’EEG). La courbe dose-réponse au vécuronium et la valeur présumée de la ED50 (la dose de vécuronium provoquant un blocage de 50%) ont été presque identiques chez cette patiente et chez cinq patients ASA 1–11 (27 μg·kg−1 vs 31 μg·kg−1). Le diamètre pupillaire et la FSL90 n’ont pas changé après l’intubation endotrachéale ou la stimulation chirurgicale chez cette patiente, tandis que chez les patients témoins (n = 3), les réactions aux deux stimulations présentaient des mesures plus élevées.
Conclusion
Labsence de réaction pupillaire et FSL90 à des stimuli douloureux indique une sensibilité au propofol et/ou un dérèglement central autonome chez les sujets souffrant d’AOPC. Bien que la dose efficace de vécuronium soit similaire chez cette patiente et chez les patients témoins, les effets des bloqueurs neuromusculaires peuvent varier selon la sévérité de l’atrophie musculaire.
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Kuroda, M., Fukura, H., Saruki, N. et al. Vecuronium dose requirement and pupillary response in a patient with olivopontocerebellar atrophy (OPCA). Can J Anaesth 45, 979–984 (1998). https://doi.org/10.1007/BF03012305
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DOI: https://doi.org/10.1007/BF03012305