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Canadian Journal of Anesthesia 51:1049-1050 (2004)
© Canadian Anesthesiologists' Society, 2004

Correspondence

Central neural blockade in Charcot-Marie-Tooth disease

Hubert J. Schmitt, MD, Tino Muenster, MD and Joachim Schmidt, MD

Erlangen, Germany

To the Editor:

In fear of possible exacerbation of the basic disease by regional anesthesia there is some concern about its use in patients with neurological diseases. We report the uneventful course of spinal anesthesia in three patients and a prolonged effect of epidural anesthesia in a fourth patient, all four suffering from Charcot-Marie-Tooth (CMT) type 1 disease. The patients were scheduled for corrective orthopedic surgeries and preferred a regional anesthetic technique. On physical examination the patients presented in good clinical condition, were fully ambulatory, and showed the typical signs of CMT (impairment of tendon reflexes, sensory disturbances at the feet) at varying extents. Spinal anesthesia was administered by injection at the L3–L4 interspace, epidural anesthesia via an epidural catheter placed in the L3–4 interspace. Demographic and clinical data are shown in the TableGo. Remarquable was the significantly prolonged sensory block in the patient receiving epidural anesthesia. During a follow-up inquiry six months after surgery none of the patients reported any worsening of the underlying disease.


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  		TABLE Demographic and clinical data from the four patients
 
The use of regional anesthesia in patients with pre-existing neurological disorders is controversial. The main problem is lack of controlled studies in affected patients to evaluate potential risks for different neurological diseases. Besides known complications like hemorrhage, infection, nerve injury, or local anesthetic toxicity1 a theoretical risk of worsening of neurological symptoms secondary to regional anesthesia exists in this special group of patients. However, in the few published reports of regional anesthesia, its use was not associated with deterioration of the underlying disease.2,3 Administration and course of spinal anesthesia in three patients were uneventful in our experience. In contrast, complete regression of sensory block after epidural anesthesia was markedly delayed. In our opinion the concentration of the local anesthetic might be responsible for this very long duration of anesthesia. Maybe a lower concentration of local anesthetic would have been sufficient for the epidural block in this patient suffering from a disease characterized by demyelination of motor and sensory nerves.4 In neither patient did the use of central neural blockade cause worsening of pre-existing neurological symptoms. We believe that, if after thorough discussion, a patient suffering from CMT prefers central neural blockade, its provision should not be refused for fear of possible worsening of neurological function.

References

1 Auroy Y, Benhamou D, Bargues L, et al. Major complications of regional anesthesia in France: The SOS Regional Anesthesia Hotline Service. Anesthesiology 2002; 97: 1274–80.[Medline]

2 Antognini JF. Anaesthesia for Charcot-Marie-Tooth disease: a review of 86 cases. Can J Anaesth 1992; 39: 398–400.[Abstract/Free Full Text]

3 Scull T, Weeks S. Epidural analgesia for labour in a patient with Charcot-Marie-Tooth disease. Can J Anaesth 1996; 43: 1150–2.[Abstract/Free Full Text]

4 Krajewski KM, Lewis RA, Fuerst DR, et al. Neurological dysfunction and axonal degeneration in Charcot-Marie-Tooth disease type 1A. Brain 2000; 123: 1516–27.[Abstract/Free Full Text]





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