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Mivacurium in multicore disease.



Multicore disease is a rare, inherited myopathy also referred as minicore, multiple minicore or mini-core-multicore myopathy. It is commonly accepted that succinylcholine succinylcholine /suc·ci·nyl·cho·line/ (suk?si-nil-ko´len) a depolarizing neuromuscular blocking agent used as the chloride salt as an anesthesia adjunct and in convulsive therapy.  should be avoided in patients with myopathies1. However, to our knowledge the complete time course of neuromuscular blockade following the administration of a nondepolarizing neuromuscular blocking agent neuromuscular blocking agent
n.
Any of various compounds, such as curare, that compete with neurotransmitters for nerve cell receptor sites, thus preventing the contraction of skeletal muscles.
 has not been reported before in a patient with multicore disease. We report a case of a 31-year-old woman scheduled for elongation of both Achilles tendons. She had no history of any cardiac or pulmonary impairment and did not take any medication. Physical examination showed pes equinus, lumbar hyperlordosis and early rigid spine syndrome rigid spine syndrome(s) A heterogeneous group of early onset muscle dystrophies–eg, X-linked Emery-Dreifuss syndrome, in which muscular atrophy begins by early adolescence, often accompanied by multiple contractures of the spinal and other muscle groups  with scoliosis. Rough estimation of the muscle strength of the upper limbs was unremarkable. Anaesthesia was induced with 2.5 mg * [kg.sup.-1] propofol and 2 [micro]g * [kg.sup.-1] fentanyl fentanyl /fen·ta·nyl/ (fen´tah-nil) an opioid analgesic; the citrate salt is used as an adjunct to anesthesia, in the induction and maintenance of anesthesia, in combination with droperidol (or similar agent) as a neuroleptanalgesic, and  and maintained with a continuous intravenous infusion of propofol 6 to 10 mg * [kg.sup.-1] * [h.sup.-1] and bolus injections of fentanyl 1 [micro]g * [kg.sup.-1] titrated to effect. Neuromuscular transmission was monitored at the adductor adductor /ad·duc·tor/ (ah-duk´tor) [L.] that which adducts, as the adductor muscle.

ad·duc·tor
n.
 pollicis and corrugator supercilii muscles using acceleromyography (AMG) (TOF-Watch SX, Organon Teknika, Finland). Response of the adductor pollicis muscle was recorded with the transducer fixed over the distal interphalangeal joint of the thumb after train-of-four (TOF) of the ulnar nerve with supramaximal current (50 to 60 mA, 0.2 ms, 2 Hz) every 15s. In the same way the upper branches of the facial nerve were stimulated 2 cm anterior to the tragus tragus /tra·gus/ (tra´gus) pl. tra´gi   [L.] the cartilaginous projection anterior to the external opening of the ear; used also in the plural to designate hairs growing on the pinna of the external ear, especially on the tragus.  lobe and the response recorded with a specific AMG probe (eye adapter) at the medial part of the superciliary arch. According to published guidelines (2), we monitored the time course of neuromuscular blockade following intravenous administration of mivacurium 0.2 mg * [kg.sup.-1]. The results are shown in Table 1. Even though onset time and recovery index were similar at both muscle groups, complete recovery time ([T.sub.25]-TO[F.sub.90]) was prolonged at adductor pollicis compared to the corrugator supercilii muscles.

Overall, the time course of recovery and the finding of faster recovery of corrugator supercilii muscles in response to a standard dosage of mivacurium in our patient are within the range of published data from patients without myopathies (3). Remarkably, however, is the extent of difference in the recovery time between adductor pollicis and corrugator supercilii muscles. Compared with corrugator supercilii muscles, the markedly prolonged recovery of the adductor pollicis muscles may indicate possible involvement of these muscles by myopathy, though this was not apparent clinically. In conclusion, the differential involvement of different muscles by multicore disease indicates that the choice of site for recording of neuromuscular transmission is an important issue. Due to the difference of recovery time between adductor pollicis and corrugator supercilii muscles, and the well-known fact that the corrugator supercilii muscles respond like the diaphragm to muscle relaxants (3), we recommend monitoring of neuromuscular transmission at corrugator supercilii muscles in patients with multicore disease.

References

(1.) Gordon CP, Litz S. Multicore myopathy in a patient with anhidrotic ectodermal dysplasia anhidrotic ectodermal dysplasia
n.
The hereditary absence of sweat glands characterized by heat intolerance, finely wrinkled skin, sunken nose, malformed and missing teeth, and sparse fragile hair.
. Can J Anaesth 1992; 39: 966-968.

(2.) Viby-Mogensen J, Engbaek J, Eriksson LI, et al. Good clinical research practice (GCRP) in pharmacodynamic studies of neuromuscular blocking agents. Acta Anaesthesiol Scand 1996; 40:59-74.

(3.) Hemmerling TM, Schmidt J, Hanusa C, Wolf T, Schmitt H. Simultaneous determination of neuromuscular block at the larynx, diaphragm, adductor pollicis, orbicularis oculi and corrugator supercilii muscles. Br J Anaesth 2000; 85:856-860.

T. MUNSTER

H. J. SCHMITT

Department of Anaesthesiology an·aes·the·si·ol·o·gy  
n.
Variant of anesthesiology.


anesthesiology, anaesthesiology
the branch of medical science that studies anesthesia and anesthetics.
,

Friedrich-Alexander University,

Erlangen-Nuremberg,

Erlangen, Germany
TABLE 1
Time coarse of neuromuscular block after administration of
                mivacuriuna 0.2 mg.[kg.sup.-1]

Site of      Onset    [T.-      [T.-      [T.-     [T.sub.-    [T.-
Monitoring   Time    sub.10]   sub.25]   sub.90]    25-75]    sub.25]
             (min)    (min)     (min)     (min)      (min)    TO[F.-
                                                              sub.90]
                                                               (min)

Adductor
polficis      2.1      14.1      15.4      24.1       7.3        23.1
Corrugator
superatii     2.4       7.7      10.2      22.2       7.5        15.4

[T.sub.10], [T.sub.25], [T.sub.90] = time of recovery of the first
twitch.
[T.sub.25-75] = time between 25% and 75% recovery of first twitch
(recovery index).
TOF = ratio fourth to first twitch out of train-of-four.
[T.sub.25]-TO[F.sub.90] = time between 25% recovery of the first twitch
and recovery of TOF ratio to 90% (complete recovery time).
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Title Annotation:Correspondence
Author:Munster, T.; Schmitt, H.J.
Publication:Anaesthesia and Intensive Care
Date:Jun 1, 2006
Words:726
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