Machado-Joseph disease is a form of progressive spino-cerebellar ataxia with both bulbar and peripheral neurological manifestations. To date, anesthesia for patients affected by this disease has not been described. General anesthesia may be problematic because of the risk of pulmonary aspiration and hypoxia. We describe our experience with the successful use of combined spinal-epidural in a patient with Machado-Joseph Disease (MJD). A 38-year-old woman with MJD complicated by significant bulbar and peripheral neuropathy presented for an elective vaginal hysterectomy. She had no other medical history of note. After informed consent, subarachnoid block was performed by combined spinal-epidural anesthesia at the L2-3 lumbar intervertebral space with hyperbaric bupivacaine 12 mg, morphine 100 microg, and fentanyl 10 microg. Surgery proceeded uneventfully, with excellent postoperative analgesia. There was full recovery of preinduction neurologic function by the sixth postoperative hour. Central neuraxial anesthesia is an option for patients with MJD presenting for lower abdominal and lower extremity operations. Combined spinal-epidural anesthesia confers hemodynamic stability yet allows for augmentation of intraoperative anesthesia and postoperative analgesia.

Teo, Goy, Woon, , , , , , (). Combined spinal-epidural technique for vaginal hysterectomy in a patient with Machado-Joseph disease. Regional anesthesia and pain medicine, ;29(4):352-4. https://www.ncbi.nlm.nih.gov/pubmed/15305255