To the Editor:
We report the case of a 37-yr-old parturient with Strümpell-Lorrain disease or hereditary spastic paraparesis presenting for the preanesthetic evaluation visit for planned cesarean delivery. Hereditary spastic paraparesis is a rare, inherited neurodegenerative disease characterized by weakness, hyperreflexia, and progressive spasticity affecting lower limbs (1). The patient's history included a previous cesarean delivery performed under general anesthesia, followed by worsening of her neurological symptoms.
We elected to proceed with spinal anesthesia. We placed a spinal needle at the L4-5 interspace with modest difficulty because of lumbar hyperlordosis. We injected a mixture of bupivacaine, sufentanil, and morphine. There was no paresthesia on injection. The block reached a T4 level. The cesarean delivery proceeded uneventfully, recovery was unremarkable, and there was no postoperative exacerbation of her disease.
The use of muscle relaxants is problematic in patients with paraparesis. Succinylcholine may induce hyperkalemia, and nondepolarizing muscle relaxants should be used with caution because of a possible exaggerated response (2). In addition, regional anesthesia has long been suspected to exacerbate neurological symptoms (3,4). However, prior reports have documented successful anesthetic management of patients with hereditary spastic paraparesis using general anesthesia (sevoflurane and N2O) (5) and epidural anesthesia (6). This case suggests that spinal anesthesia is also an option in these patients.
Stéphane Deruddre, MD
Martine Marie, MD
Dan Benhamou, MD
Hôpital Antoine Béclère
SOS Regional Anesthesia Hotline Service:
1. Fink JK. Advances in the hereditary spastic paraplegias. Exp Neurol 2003;184 Suppl 1:S106–10.
2. Brown JC, Charlton JE. Study of sensitivity to curare in certain neurological disorders using a regional technique. J Neurol Neurosurg Psychiatry 1975;38:34–45.
3. Dripps RD, Vandam LD. Exacerbation of pre-existing neurologic disease after spinal anesthesia. N Engl J Med 1956;255:843–9.
4. Aldrete JA, Reza-Medina M, Daud O, et al. Exacerbation of preexisting neurological deficits by neuraxial anesthesia: report of 7 cases. J Clin Anesth 2005;17:304–13.
5. Kunisawa T, Takahata O, Takayama K, et al. Anesthetic management of a patient with hereditary spastic paraplegia [in Japanese]. Masui 2002;51:64–6.
6. McTiernan C, Haagenvik B. Strumpell's disease in a patient presenting for Cesarean section. Can J Anaesth 1999;46:679–82.