关键词: 肯尼迪病, 麻醉, 全身麻醉, 神经阻滞

Abstract:

Kennedy disease is a rare X-linked recessive genetic disease with a low incidence rate. The main manifestations of motor neuron involvement include limb weakness, muscle atrophy, dysarthria, difficulty swallowing, and coughing after drinking water. The patient may die from pulmonary infection and respiratory failure, and there is currently no effective treatment available. There are few reports on anesthesia for such patients and no guidelines or expert consensus. This article reports on perioperative anesthesia management for a 69 years old patient who underwent lumbar spine surgery before with coronary heart disease. The patient was diagnosed with Kennedy disease through electromyography and genetic testing before surgery and underwent knee replacement surgery. After sufficient preoperative consultation and evaluation, femoral nerve block was performed with 0.25% ropivacaine under guidance with ultrasound and nerve stimulator, followed by induction of general anesthesia with sufentanil, propofol, and etomidate. A laryngeal mask was inserted without the use of muscle relaxants and breathing was controlled by machine. During the operation, propofol and remifentanil were used for total intravenous anesthesia. The patient had stable vital signs, well tolerated, and the surgical process was smooth. The time of recovery from anesthesia was short, and no anesthesia related complications, such as nausea, vomiting, aspiration, or suffocation was observed after the operation. Postoperative muscle strength recovery was good. After closely monitoring in the ICU for a day, the patient returned to the regular ward. A postoperative analgesia combination of nerve block and oral nonsteroidal analgesics was performed, and emergency pain rescue with pethidine was administered if necessary. The analgesic effect was satisfactory. The patient was safely discharged in the end and recovered well.

Key words: Kennedy disease, Anesthesia, General anesthesia, Nerve block