Strümpell's disease in a patient presenting for Cesarean section

The anesthetic management of a parturient with Strumpell's disease (hereditary or familial spastic paraparesis) who presented for Cesarean section is described. This neurological disorder is briefly reviewed and anesthetic implications of the condition are discussed.

A 30-yr-old woman in premature labour presented for Cesarean section. She had bilateral lower limb spastic paresis which had resulted in her being confined to a wheelchair from the age of 13 yr. A diagnosis of Strumpell's disease had been made in childhood. She was currently receiving thromboprophylaxis, having suffered a deep venous thrombosis four weeks after a previous Cesarean section. The patient was in mild respiratory distress. Despite a history of uneventful general anesthesia and the aforementioned complicating factors, epidural anesthesia was considered the most appropriate technique in these circumstances. An epidural catheter was sited at the L3-L4 interspace. Adequate anesthesia for the procedure was obtained after administration of 20 ml lidocaine 2% with 100 microg epinephrine and 100 microg fentanyl in saline. Postoperatively and at six month follow-up there were no neurological complications related to the use of epidural anesthesia.

Strumpell's disease is an inherited progressive spastic paresis predominantly affecting the lower extremities. Epidural anesthesia appears to be an appropriate technique when administering anesthesia for Cesarean section under similar circumstances.